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A 22-year-old female professional water-polo player presented to our Hand Department complaining of persistent pain on the dorsoulnar aspect of the right wrist (dominant hand) during the last 4 months, aggravated by sport activity, and not responding to medical treatment or rest. The patient did not report any acute trauma at that region and noted that her symptoms worsened while shooting. At that time a painful snapping sensation over the dorsoulnar aspect of the wrist followed the forceful forearm rotation. Physical examination revealed swelling, edema and tenderness over that area. A “clunk” and palpable dislocation of the ECU tendon along the ulna head with active or passive supination and ulnar deviation of the wrist was observed. Hand and finger function was normal, and neurovascular examination showed no abnormality.\nThe patient underwent radiological investigation with plain radiographs of the wrist, that were negative for any bone pathology ( and ), and dynamic Ultrasound (U/S) at rest (with the wrist in 0° of flexion-extension) and during pronation and supination with comparison to the contralateral ECU tendon. During supination, the unstable ECU tendon slided abruptly over the ulnar wall of the distal ulnar groove and dislocated volarly, while during pronation the tendon relocated into the groove ( and ).\nDiagnosis of recurrent ECU tendon dislocation was made on the basis of clinical history, physical examination and imaging findings. As the patient’s symptoms had not improved after a 4-month period of conservative management and in conjunction with a highly demanding sport, surgical intervention was opted.\nSurgical reconstruction of ECU tendon sheath was carried out under axillary block anesthesia using a high humerus tourniquet. A straight dorsomedial incision was made along the distal ulna starting 5 cm proximal to the wrist and ending 1 cm proximal to the joint (). The extensor retinaculum, found to be intact, was opened longitudinally over the ulnar aspect. Dislocation of the ECU tendon in ulnar-palmar direction was confirmed with the forearm in supination and the wrist in palmar flexion. The fibro-osseous sheath was found to be torn along its entire length from the ulnar retaining wall (). The distal ulnar groove was deep enough to retain the tendon in its normal position and it was not reconstructed. The ECU tendon was elevated, reduced into the groove and 4 mini bone suture anchors were placed along the groove’s ulnar margin (). The sutures were then passed through the ulnar border of the ECU sheath in horizontal mattress fashion and were tied, securing tendon sheath to bone. A piece of extensor retinaculum was used as a graft, to strengthen the reconstructed sheath (). Splinting of the humerus-forearm with the elbow flexed to 90° and the wrist in approximately 30° of extension, radial deviation, and pronation was applied for 6 weeks. A progressive rehabilitation program was prescribed after cast removal, including active and passive wrist flexion, extension, forearm supination and pronation, and progressive strengthening with gradual return to sport activities. The patient was allowed to fully participate in her team’s schedule 4 months postoperatively. Follow-up evaluation 12 months postoperatively revealed no recurrent dislocation of the ECU tendon and normal painless range of motion of the affected wrist.\nAll procedures concerning the management of the patient were in accordance with the Helsinki Declaration of 1975, as revised in 1983.
A 56 year old male with a past medical history of antiphospholipid syndrome, chronic kidney disease stage III (resulting from a renal infarct from an embolic thrombus), and essential hypertension presented to the emergency department after a near syncopal episode while he was uploading boxes. Upon emergency medical services (EMS) arrival, he was noted to be in monomorphic ventricular tachycardia (VT) (Fig. ) that needed immediate synchronized cardioversion with subsequent resolution of his symptoms. Upon arrival to the emergency room, the patient was hemodynamically stable with benign cardiovascular examination. Resting ECG (Fig. ) showed sinus tachycardia with T wave inversions over the lateral leads.\nLab analysis revealed sub-therapeutic prothrombin time and international normalized ratio (INR). Initial work up with coronary angiography revealed trivial coronary artery disease and possible congenital coronary anomaly. TTE on the day after admission demonstrated a LV apical aneurysm with mural thrombus formation. Chest X-ray showed a calcified nodule at the cardiac apex, which correlates to the calcified intramural thrombus. Subsequently, coronary CT angiography (Fig. ) revealed a potentially malignant, anomalous right coronary artery (RCA) with left coronary cusp origin just anterior to the origin of the left main coronary artery. This anomalous vessel coursed transmurally between the ascending aorta and pulmonary artery; thus, there is a significant risk for vessel impingement both in the transmural course during systole and between the aorta and the pulmonary artery.\nTTE with an ultrasound enhancing agent showed mid-LV hypertrophy and an apical LV aneurysm with an apical mass suspicious for a thrombus.\nSubsequent CMR showed mid LV hypertrophy consistent with HCM and LV apical aneurysm with intramural thrombus. LGE imaging revealed full thickness scarring in the LV apex. Ultimately, an automated implantable cardioverter defibrillator (ICD) was placed, and the patient was sent home with oral anticoagulation and antiarrhythmic therapy. Patient was counseled and recommended to undergo genetic testing for HCM.\nThe CMR bSSFP cine images revealed an LVEF of 60% with an apical LV aneurysm measuring 3.9 × 3.2 cm, with moderate hypertrophy of the mid-ventricular myocardium measuring 1.6 cm in maximal thickness consistent with HCM. A 1.2 × 1.0 cm lesion was noted within the apical aneurysm, consistent with intramural thrombus (Additional file : Movie S33, Additional file : Movie S34, Fig. ). Also, LGE images revealed near full thickness scarring of the apical LV segments (Fig. ).\nWe report a case of malignant VT with three plausible causes: anomalous RCA with a potentially malignant course, LV apical scarring with aneurysm formation and HCM. However, the apical scar is the most likely cause of the VT based on the VT tracing (Fig. ). This case emphasizes the role and importance of CMR in evaluation of new-onset VT as it helped determine the most probable source of this life-threatening arrhythmia and saved the patient from undergoing aggressive surgical intervention like: excision and re-implantation of RCA into the appropriate sinus of Valsalva, coronary artery bypass grafting or trans-aortic unroofing of the RCA since such surgical procedures have considerable morbidity and mortality. Hence, it was reasonable to only proceed to ICD implantation in addition to pharmacologic therapy.\nVT is a life-threatening arrhythmia, and along with ventricular fibrillation, is the most common cause of outpatient sudden cardiac death. Ventricular arrhythmia mostly occurs in setting of structural heart diseases like post-myocardial infarction (most frequently), coronary artery anomalies, dilated cardiomyopathy, HCM, arrhythmogenic right ventricular dysplasia, and infiltrative diseases like sarcoidosis in addition to non-structural heart diseases like in long QT syndromes. Hence, in patients with new-onset VT, it is vital to pinpoint the source of the aberrant rhythm to prevent the recurrence of potentially life-threatening arrhythmias. As was true in this case, multi-modality imaging plays a major role in evaluation and management of this arrhythmia.\nThe following will be a discussion of the three potential origins:Anomalous RCA While most of coronary artery anomalies are not associated with myocardial ischemia and clinical events, some anomalies can potentially result in myocardial ischemia depending on the location and course of the anomaly; thus, they can manifest with angina pectoris, syncope, myocardial infarction and VT; however, coronary anomalies are considered an under-diagnosed cause of sudden cardiac death (SCD) in young athletes []. Anomalies of RCA include ectopic origin from the right sinus of Valsalva, posterior sinus of Valsalva and left sinus of Valsalva. Our patient has ectopic RCA originating from left sinus of Valsalva which has an incidence rate of 0.03% to 0.92% on coronary angiograms []. In our patient, the long intramural course of the anomalous RCA in addition to its inter-arterial course between the aorta and pulmonary artery can potentially result in compression especially during exercise and decreased blood flow to myocardium potentiating ischemic ventricular arrhythmia. Coronary anomalies can be seen on echocardiography, angiography, coronary CT and CMR with coronary CT being the clinical standard due to its widespread availability, ease of use, high spatial resolution and very short acquisition time []. Left ventricular apical scar Ventricular scar or fibrosis most commonly forms as a consequence of myocardial infarction, but it can be also caused by non-ischemic insults like myocarditis. These scars are composed of fibrous tissue with intervening cardiomyocytes, and because fibrous tissue is unexcitable, it represents a substrate for re-entry circuits leading to ventricular arrhythmia. As was true in this case, LGE in CMR is the gold-standard imaging for diagnosis of myocardial scar. Normal and viable myocardium appears black on LGE imaging while myocardial scar appears bright or enhanced. Myocardial scar detected on imaging is considered a strong predictor for cardiac death and major adverse cardiac events []. In our patient, the apical scarring was thought to be related to either HCM or less likely thrombotic or embolic event causing infarction in the distal left anterior descending coronary artery (LAD) territory given patient’s history of anti-phospholipid syndrome in setting of sub-therapeutic anticoagulation. It is thus important that the patient be continued on anticoagulation as he would be at risk for further embolic phenomenon. Hypertrophic cardiomyopathy HCM is the most common cause of SCD in young athletes. It is mostly asymmetric with most cases having involvement of the basal inter-ventricular septum. However, hypertrophy can exclusively involve the apex, or mid-portion or posterior wall of the LV []. Diagnosis of HCM can be made by demonstrating a wall thickness > 15 mm in one more LV myocardial segments by any cardiac imaging modality, not explained solely by loading conditions []. Although the patient in this case has chronic kidney disease and hypertension, neither of these diseases can explain the LV hypertrophy demonstrated on the patient’s imaging as it is focal and asymmetrical (involving mid-segment of the inter-ventricular septum).\nAlthough TTE is considered a good assessment tool for LV wall thickness and LV outflow tract pressure gradient, CMR should be considered for accurate assessment of LV wall thickness in patients with poorly visualized LV regions on echocardiogram, and particularly in HCM cases that are confined to one or two segments of LV like apical or anterolateral variants because of its superior characteristics including lack of attenuation, great spatial resolution in addition to its ability to image in any plane []. Also, LGE CMR plays an important prognostic role in predicting adverse cardiovascular events among HCM patients [].\nThe CMR of Case 6 (Additional file CMR Link, ).
A 72-year-old left-handed man with past medical history of atrial fibrillation, congestive heart failure and mitral valve repair, but no history of malignancy, presented to the Emergency Department in 2016 for evaluation of right arm pain. The patient heard a crack in his arm while dressing and subsequently his arm pain worsened. His pain, when he was evaluated at the Emergency Department, was subjectively rated as 5/10, worse with activity and palpation and relieved with rest. No edema or erythema was noted. There was no axillary or cervical adenopathy. His pulses were normal and his sensation to light touch was intact. Radiographs obtained in the Emergency Department revealed a minimally angulated proximal right humeral fracture at the superior aspect of a linear sclerotic lesion in the proximal humeral diaphysis. The linear sclerotic lesion was thought to be likely a bone infarct (Figure and Figure ). No definite soft tissue lesion was noted. His fracture was treated conservatively with splinting.\nHowever, his pain progressively worsened, so repeat radiographs were obtained a couple of weeks later to assess healing of the fracture at his fracture follow-up clinic visit (Figure ). These subsequent radiographs demonstrated the development of a lytic lesion with surrounding periosteal reaction at the fracture site. No osteoid production/mineralization was appreciated. Magnetic resonance imaging (MRI) showed a T1-isointense (Figure ), T2 heterogeneously hyperintense (Figure ), heterogeneously enhancing lesion (Figure ) originating from the intramedullary cavity with osseous destruction of the humerus and a soft tissue component that measured up to 15 cm in superior-inferior dimension. A serpiginous, linear area of low T1 and low T2 signal consistent with a bone infarct was noted at the lesion, and this area of infarct extended more distally in the humeral diaphysis. This bone infarct corresponded to the linear area of sclerosis seen in the humeral diaphysis on prior radiographs.\nUltrasound-guided core needle biopsy of the soft tissue component of the lesion was performed. Histological analysis of the biopsy showed neoplastic spindle and epithelioid cells with focal osteoid formation (Figure ). A few giant cells were also noted. There were frequent mitotic figures (4 per high powered field). Areas of hemorrhagic necrosis were present. No chondroid material was noted. Immunohistochemical stains with adequate controls were performed. The epithelioid cells were negative for TTF-1, S100, AE1/3, PSA, HMB45, ERG, CD31, CAIX, HepPar, PAX8, and pancytokeratin. INI1 was retained. This immunoprofile combined with the histological findings supported a diagnosis of a tumor of bone origin and were most consistent with an osteogenic sarcoma (Figure ). Staging 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) showed localized disease (Figure ).\nHe was treated with reduced dose cisplatin/adriamycin given his age. He had a poor response to chemotherapy with enlargement of the mass through chemotherapy with the development of circumferential disease around the humerus. He underwent a right forequarter amputation. He developed lung metastases and an L4 spinal metastasis with pathological fracture two months following surgery. He received palliative radiation therapy to the L4 vertebra and died seven months after initial presentation.
A 40-year-old nulliparous woman with no past medical history, other than endometriosis, presented to the emergency room with severe chest tightness of one day duration. She described chest tightness while exercising on a bicycle after 10 minutes. The next day she developed a severe hacking cough at work during a conference call. The cough was associated with disorientation and severe chest tightness. This prompted her to present to the emergency department in February 2018. The patient is a pharmacist, nonsmoker, and denies illicit drug use or recent travel. She had endometrial laser ablation with myomectomy in 2006. Hormonal contraceptives have been used since 2004 and were stopped three months before presentation in hopes of conception. Her last menstrual period was four days before the onset of symptoms. On physical examination, she had chest tightness localizing to the right side and decreased right sided breath sounds. All the routine laboratory work and vital signs were normal. The CXR showed a large right spontaneous pneumothorax with what approved to be a 5.6 cm pleural mass at the right lung base ().\nFollowing the pneumothorax diagnosis, the patient underwent emergent right thoracostomy with pigtail catheter placement. A repeat CXR revealed marked re-expansion of the lung but persistence of the right cardiophrenic opacity of unclear etiology. A follow-up CTof chest showed a 33 mm diaphragmatic defect with a 5.8 x 4.6 x 3.9 cm area of herniated liver corresponding to the presumed pleural mass ().\nFollowing complete thoracic imaging the patient underwent video-assisted thoracoscopic surgery (VATS), mechanical pleurodesis, and open repair of the right diaphragmatic defect by Dr. Emily Cassidy (Figures and ). Intraoperatively, the lungs appeared grossly normal. An obvious diaphragmatic defect was noted in the posteromedial portion of the central tendon of the diaphragm with a sizable protrusion of liver in to the chest cavity. There was an attempt to dissect liver adhesions from the diaphragm. Extensive liver adhesions forced conversion of the right VATS to a posterior lateral muscle sparing thoracotomy. Electrocautery and blunt dissection were used to separate the liver and diaphragm. Once the liver hernia was completely reduced, the diaphragm was repaired using multiple interrupted prolene pledgeted horizontal mattress sutures. Later, right parietal pleurectomy was performed by scoring the pleura posteriorly, anteriorly throughout the entire chest cavity. At the apex as well as the base, where pleurectomy was difficult, mechanical pleurodesis using a Bovie scratch pad was perfomred. A 24-french chest tube was placed at the apex of the chest cavity and 24-french blake was placed at the level of diaphragm.\nIntraoperatively, an endometrial implant (blue berry spot) was noted on the chest wall (), but the endometrial implant was not successfully biopsied for pathology evaluation due to the extent of liver adhesions and due to conversion of procedure from VATS to open thoracotomy.\nOn postoperative day three, the patient began her menstrual cycle. She was evaluated by a gynecologist consultant who recommended hormonal therapy, leuprolide a Gonadotropin releasing hormone (GnRH) analogue, to begin 2-3 weeks postoperatively for a period of 6-12 months for hormonal suppression to reduce the risk of recurrent pneumothorax. GnRH analogs are highly effective at suppressing ovarian hormone production and inhibition of growth of endometrial tissue. Due to a persistent air leak, the patient's chest tube was transitioned to a Heimlich valve to facilitate home discharge. We believed that the persistent air leak indicated there was some minor defect in the visceral pleura that was too small to identify intraoperatively. The patient was discharged on postoperative day eight and was seen as an outpatient by the cardiothoracic surgeon. At this time, there was resolution of air leak and removal of the chest tube. Patient was also seen after two months in a primary care clinic for follow-up visit with no issues.
A 29-year-old woman sought orthodontic treatment, complaining about an unesthetic smile due to occlusal plane inclination and midline deviation. This was caused by absence of maxillary left lateral incisor and mandibular left second premolar, with ankylosis of deciduous molar in this region. Facial analysis revealed good symmetry and vertical balance of the facial thirds, a convex profile, and accentuated occlusion plane inclination in a smiling photograph (). Intraoral analysis revealed Angle Class II, Division 1 malocclusion, with absence of maxillary left lateral incisor, a peg-shaped maxillary right lateral incisor and the presence of mandibular left deciduous ankylosed second molar, which caused asymmetry on this side in both maxillary and mandibular arches ( and ). Maxillary midline was deviated 2 mm to the left while mandibular midline was deviated 2 mm to the right. Panoramic and periapical radiographs confirmed the absence of maxillary lateral incisor and mandibular second premolar and also revealed mandibular teeth greatly inclined towards the ankylosed deciduous molar. Initial lateral cephalogram and cephalometric tracing revealed skeletal Class II malocclusion, with upright maxillary incisors and well-positioned mandibular incisors ( and ).\nThe objectives of treatment were as follows:\nCorrect occlusal plane inclination. Obtain molar Class I relationship on the left side and Class II on the right side. Establish canine Class I relationship on both sides. Correct midlines. Extract deciduous molar and replace the tooth with implant-prosthetic rehabilitation. Open space in order to implant a prosthetic rehabilitation of the maxillary left lateral incisor.\nOrthognathic surgery was considered for occlusal plane correction, but the patient refused this option. Therefore, two other alternatives were considered to correct Class II malocclusion and tooth absences. The first option was to extract the maxillary right lateral incisor, replace lateral incisors with canines, and then replace canines with first premolars. This option was rejected in a meeting with the dentist responsible for the final rehabilitation. The dentist believed that the esthetic result would be better with implant-prosthetic rehabilitation of the maxillary lateral incisor, as maxillary canines had large crowns and were too different in color, so as to be used as lateral incisors. The second option was to extract maxillary right first premolar and insert a mini-implant or miniplate on the left side to move the maxillary right dentition posteriorly. This option was rejected by the patient due to longer treatment time required in comparison to that for first premolar extraction to distalize all teeth. Thus, in agreement with the patient and the other dentist, it was decided to correct the occlusal plane by means of a miniplate on the maxillary left side, extract the maxillary right first premolar and open space for rehabilitation of the maxillary left lateral incisor.\nTreatment began with the bonding of 0.022 × 0.028-in standard Edgewise brackets on both arches, followed by alignment and leveling with 0.012 and 0.014-in Nickel-Titanium archwires and from 0.014-in to 0.020-in stainless steel archwires. Thereafter, maxillary right first premolar and mandibular left second deciduous molar were extracted and maxillary anterior teeth were moved to the right, tooth by tooth, with elastomeric chains, in order to correct maxillary midline and open space, thus allowing the insertion of an implant in the space left by the maxillary left lateral incisor. On the maxillary left side, after correction of premolars rotation, a 2-mm space was created and both premolar and canine were distalized with elastomeric chains to increase the space for implantation of the maxillary left lateral incisor prosthesis and to partially correct Class II. On the mandibular arch, an implant was inserted into the space of the missing premolar to aid mandibular midline correction. That implant was positioned above the proper position, considering that after occlusal plane correction with maxillary intrusion and mandibular extrusion on this side, the implant would be in adequate vertical position. Likewise, the implant was positioned closer to the mandibular left first molar and away from the left first premolar, thereby allowing distalization of mandibular left molars and distalization of mandibular anterior teeth, thus correcting the midline. After that, a miniplate in the shape of an Y was inserted in left zygomatic buttress and used to intrude all maxillary left teeth, with elastics connected to 0.019 × 0.025-in wire segments inserted into a tube and connected to a miniplate, generating a force of 200 g/f each (). Furthermore, the miniplate was used to distalize all teeth on the left side, with elastomeric chains connected to a hook welded between the lateral incisor and canine, so as to correct Class II relationship. After correction on the maxillary arch, the mandibular arch was extruded with intermaxillary 1/8-in elastics connected directly to the miniplate and on the mandibular teeth and archwire (). In order to allow mandibular teeth extrusion, the mandibular arch was made bypassing the bracket of provisory crown over the implant. At that time, the space for maxillary left lateral incisor was already well defined and the implant was inserted. Maxillary right lateral incisor was provisionally restored with composite resin before appliance debonding, so as to precisely define the spaces on the anterior region. After 34 months of treatment, the appliance was removed.\nAt the end of treatment, we noticed an improvement in smile esthetics due to correction of occlusal plane inclination and because the midlines were coincident with the facial midline (). The profile remarkably improved as a result of counterclockwise rotation of the mandible, which reduced convexity, thus increasing the prominence of lips and chin (). Intraoral and dental casts analyses revealed that Class I molar relationship on the left side, Class II molar relationship on the right side and Class I canine relationship on both sides were all obtained, with good intercuspation ( and ). Panoramic radiograph showed good parallelism among roots, in addition to root resorption on maxillary left central incisor, which will be monitored after treatment. Post-treatment lateral cephalogram, cephalometric tracing and superimposition examinations confirmed accentuated mandibular counterclockwise rotation (). Furthermore, maxillary left molars were intruded while mandibular molars were uprighted and extruded. Maxillary and mandibular incisors were proclined after treatment. The patient will be monitored every six months in order to have root resorption and treatment stability controlled.
A 64-year-old male was referred to the Gunma University Hospital with complaints of a progressive oppressive feeling in the left thoracic region. His past medical history was unremarkable. At admission, general conditions were satisfactory, and a physical examination of the thorax was normal. Routine laboratory tests were within normal values, but a chest roentgenogram revealed an oval-shaped anterior mediastinal mass in the left hemithorax. An enhanced computed tomography (CT) scan of the chest revealed the presence of an ovoid mass about 9-cm at its greatest dimension in the left anterior mediastinal region. Its border, adjacent to the left lung parenchyma, was poorly demarcated. The mass showed a heterogeneous densitometric characteristic accompanied with areas of necrosis after contrast enhancement (Figure ). From the graphical specifications and laboratory studies, an invasive thymoma was strongly suggested, and we decided to perform a radical resection of the tumor.\nWritten informed consent concerning the operation was obtained from the patient before surgery, and the patient was admitted to the Gunma University Hospital for surgical resection of the tumor. The patient underwent tumor excision and thymectomy through sternotomy with continuous anterior thoracotomy of the third intercostal space. During the operation, the tumor, as seen on the CT scan, was 9-cm wide, elastic, and off-white in color. It was found to have infiltrated the upper lobe of the left lung. Based on this surgical finding, partial resection of the lung together with the tumor was performed with a sufficient surgical margin. On the basis of its morphologic and immunohistochemical features, the tumor was diagnosed as a mediastinal atypical meningioma. The patient had an uneventful recovery and was discharged on postoperative day 6. An ectopic pulmonary site of origin, such as the mediastinum in this case, should be accepted only after the possibility of spread or metastasis from primary intracranial or intraspinal origin has been excluded. One month after operation, the patient had an enhanced magnetic resonance imaging (MRI) of the head and spine, and no abnormal findings were observed, which eliminated the possibility of an intracranial or intraspinal origin. He has been monitored for 12 months as an outpatient without any symptoms of recurrence or metastasis.\nThe surgical specimens were routinely fixed with 10% formaldehyde and embedded in paraffin. Tissue sections (6-μm thick) were stained with hematoxylin and eosin (H-E), and periodic acid-Schiff (PAS) with and without diastase digestion. For immunohistochemical studies, tissue sections were incubated using the standard avidin-biotin peroxidase complex (ABC) method with the following antibodies: epithelial membrane antigen (EMA), vimentin, S-100, MIB-1, CD34, D2-40, αSMA, desmin, synaptophysin, and neurofilament. Macroscopically, the resected tumor measured 9-cm in its greatest dimension and adhered to a parenchyma of the left lung. The oval-shaped tumor had a smooth surface, and its cut surface was homogenously solid and yellow-white in color (Figure ).\nMicroscopically, the tumor was partially encapsulated and composed of bundles of spindle-shaped cells with elongated nuclei and syncytial nets of tumor cells with oval nuclei. A few tumor cells had an intracellular cytoplasmic inclusion. In some areas, many typical whorl formations for fibrous meningioma were observed (Figure ). There were several foci of small cells with a high nuclear/cytoplasmic ratio (Figure ). Spontaneous and zonal necroses were occasionally seen (Figure ). In addition, the tumor involved adjacent pulmonary parenchyma (Figure ). The mitotic index was four per 10 high-power fields (hpf) at the areas where the mitotic figures were most frequently observed, and the Ki-67/MIB-1 labeling index was approximately 7% in relatively higher positive-rate fields (Figure ). Immunohistochemical examinations showed positivity to EMA (Figure ) and vimentin, focal positivity to S-100, CD34, and D2-40, and negativity to αSMA and desmin. Some oval tumor cells containing large cell bodies were stained for synaptophysin and neurofilament.
A 17-year-old boy with history of fall from height of approximately 15 meters presented to our institution after receiving primary care at another hospital. At presentation, he was conscious, hemodynamically stable maintaining oxygen saturation at 98% on room air with no visible signs of respiratory distress. His Glasgow coma scale (GCS) was 15, and was able to move all four limbs. The patient had sustained open fracture both bone left leg along with fracture right ankle.\nChest radiograph showed no intrathoracic injury with normal lung parenchyma. Computed Tomography showed burst fracture of fifth lumbar vertebra with canal compromise () and ruled out any injury to head, cervical spine, thorax and abdomen. After primary care, patient was admitted to the orthopedic ward for spine stabilization surgery and surgery for lower limb fracture.\nOn day four post-admission, an emergency consultation call was sent to our intensive care unit (ICU) in view of patient’s deteriorating status. When seen, he was grossly pale and febrile at 101 F, pulse rate of 140 per minute, systolic blood pressure of 80 mmHg and respiratory rate of 32 per minute maintaining oxygen saturation around 90% on oxygen face-mask. Patient’s GCS was 15. Chest auscultation revealed bilateral diffuse coarse crepitation and he was immediately transferred to the ICU. Initial arterial blood gas (ABG) showed partial pressure oxygen (pO2) of 49mm Hg on oxygen by face mask. Patient was intubated, sedated, paralyzed and put on mechanical ventilation with initial settings of volume assist control and high positive end expiratory pressure (PEEP). Central venous catheter was secured in right internal jugular vein under ultrasound guidance. An arterial line was secured in right radial artery for invasive blood pressure and arterial blood gas analysis. Chest radiograph showed bilateral fluffy opacities (). Preliminary blood investigations were mostly unremarkable except for hemoglobin of 6.8 mg/dl and raised ESR of 44. Fundus exam specific for FES was normal and there was no petechial rash on general examination. Urine for fat globules was positive. Over the next few hours, patient’s hypoxemia worsened requiring higher fraction inhaled oxygen (FiO2) of up to 0.8 and PEEP of 16 cm water. A decision was made to turn the patient into prone position after discussion with the orthopedic surgery team in view of unstable lumbar spine fracture. Patient’s family were informed about the specific risks and benefits of prone positioning, particularly in a patient with pre-existing unstable lumbar spine fracture and a written consent for the same was obtained. Positioning was done with the help of 5 trained ICU staff, using logrolling technique for turning the patient lateral followed by a 6th member placing the spinal board under the patient. After securing the patient on the spinal board, he was then shifted to one edge of the bed while the head gel support, chest and pelvic roll were placed in position. Patient was then shifted to prone position and spinal board was removed. Patient’s hands were abducted and placed next to the head and all the pressure points were padded using pillows and cotton rolls.\nOver the next 16 hours, we kept our patient sedated and paralyzed in prone position during which he received targeted fluid therapy and remained hemodynamically stable. Wake up test and pupil examination were done at regular intervals. Serial ABG’s showed dramatic improvement with Fio2 requirement decreasing to 0.4 and PEEP of 8 following which patient was repositioned supine. Two units packed red cells were transfused while the patient was prone. Chest radiograph showed resolving lung infiltrates and we decided to electively mechanically ventilate our patient for next 24 hours in supine position. Weaning was started next day and patient was extubated a day after and transferred back to ward 24 hours later.\nThe patient later on went on to have multiple surgical procedures for injuries to spine and bilateral lower limbs. He was then followed for 8 weeks in the out-patient department following discharge during which he recovered well without any neurovascular deficit.
A 61 year-old man was admitted with a 103 ° F fever, confusion, weakness and slurred speech after hemodialysis. He had a history of viridans streptococcal mitral valve endocarditis, end stage renal disease on hemodialysis, atrial fibrillation not on anticoagulation due to GI bleeding, and monoclonal gammopathy of undetermined significance. He had a productive cough for a week without any identifiable sick contact. Physical examination was notable for an agitated edentulous man with a left central facial palsy, severe dysarthria, and a systolic murmur at the left lower sternal border. His lungs were clear to auscultation and there was no stigmata of endocarditis.\nThe patient was initially treated empirically for pneumonia and worked up for stroke. However, the treatment plan was quickly modified when a transthoracic echocardiogram on day two of admission revealed two echogenic structures consistent with vegetations: 0.4 × 0.4 cm on the anterior leaflet of the mitral valve, and the other 0.7 × 1.8 cm attached to left coronary cusp of the aortic valve (Fig. ). There was also thickening of the aortic root suggestive of abscess formation. Two sets of blood culture grew Gram-positive rods after 37.5 h incubating in anaerobic bottles (Fig. ), and after 86 h in aerobic bottles. The organism was identified as A. neuii by MALDI-TOF MS on day five of admission. Serial brain MRI scans revealed multiple bilateral infarcts on day two with increased number of infarcts and a small focus of hemorrhage on day five. The patient was diagnosed with infective endocarditis by A. neuii complicated by aortic root abscess and presumed cerebral septic emboli.\nThe patient was initially treated with vancomyin and piperacillin/tazobactam until A. neuii was identified. Subsequently, he was treated with ampicillin and gentamicin for two days, followed by ampicillin for the rest of his hospitalization. The choice of ampicillin was based on a large series that studied susceptibility to antibiotics of Actinomyces species [], and a previously successfully treated A. neuii endocarditis case []. Antibiotic susceptibility was not tested for our patient because he responded to the treatment well, and repeat blood cultures were all negative. A CT angiography of the brain and neck on day six ruled out mycotic aneurysm. It was concluded that the risk of further septic embolization outweighed the risk of intracranial hemorrhage, and the patient underwent aortic valve replacement, debridement of aortic root subannular abscess, mitral valve repair, and repair of a fistula between the aorta and left atrium on hospital day fourteen. A 2.5 × 0.6 cm vegetation on the aortic valve and a vegetation on the mitral chordae tendineae were removed. There was no microscopic evidence of bacterial elements on the aortic valve based on histopathology with Gram stain, and culture did not grow any organisms. The patient’s post-operative course was complicated by shock requiring intraaortic balloon pump, and a cardiac arrest from ventricular fibrillation 10 days after surgery. He recovered without further neurological deterioration, and was discharged to a nursing facility two months after heart surgery. He received 12 weeks of IV ampicillin followed by 11 months of oral doxycycline.\nOne year after the diagnosis of A. neuii endocarditis, while on chronic doxycycline, the patient had a fever and a bacteremia with coagulase negative Staphylococcus and group B Streptococcus. The bacteremia was sterilized after the initiation of antibiotic therapy and there was no growth from subsequent blood cultures. Transthoracic echocardiogram showed a small, mobile echogenic density on the non-coronary cusp of the bioprosthetic aortic valve. The patient refused to undergo transesophageal echocardiogram to further evaluate the prosthetic valve, so he was treated empirically for possible prosthetic valve endocarditis. The patient was cured from infection after two weeks of IV vancomycin and gentamicin, followed by four weeks of IV vancomycin. He had been taking oral doxycycline in addition to his IV antibiotics.\nThe patient eventually died of a sudden cardiac arrest after hemodialysis. This was 15 months after the diagnosis of A. neuii infective endocarditis, and four weeks after discontinuation of oral doxycycline. The family declined autopsy.\nPrimary infective endocarditis caused by Actinomyces spp. is rare. After PubMed (search term ((actinomyces spp) OR actinomyces) AND ((infective endocarditis) OR endocarditis)) and additional bibliographical search, we found 26 human cases dating back to 1939 (Table ), after excluding four reports, two with bacteria that have been subsequently reclassified to different genera [, ], one report with possible direct extension of pulmonary actinomycosis to the endocardium [], and one with primary IUD-associated actinomyosis and secondary endocarditis []. Cases were reported at all ages (6–87 years old). Two thirds of patients were men. The most commonly identified species were A. israelii (19%) and A. viscosus (15%). Twenty-two cases involved left-sided valves (mitral 9; aortic 5; prosthetic aortic 3; both mitral and aortic 4; undetermined 1). Risk factors included valvular disease (41%), poor dental hygiene or dental procedure (36%), and prosthesis (14%). All four right-sided cases were associated with intravenous drug use [, , , ].\nMost left-sided endocarditis patients had indolent courses. This did not seem to vary over time. However, the mortality and complications have improved significantly over time. Five of eight patients reported before 1990 died and five had embolic events (brain, spleen, kidneys, small bowel and skin), whereas only two of 14 cases reported after 1990 died, and only one had emboli to skin. Despite temporal courses of a subacute endocarditis, where stigmata of endocarditis are more common, only one report described Roth’s spots []. It is unclear whether this was related to virulence factors from Actinomyces spp., or simply the rarity of these complications []. Right-sided endocarditis cases had more acute and fulminant courses, and were all complicated by septic emboli to the lungs. Two (50%) of them had polymicrobial endocarditis [, ], which might have contributed to more complicated clinical courses. All four right-sides cases survived and all were reported after the year of 2000. Irrespective of the side of endocarditis, most patients were treated with a prolonged course of penicillin or β-lactam antibiotics. Four cases had surgery (three aortic valves [, , ] and one Eustachian valve [], an embryologic remnant of the valve of the inferior vena cava).\nTwo cases of infective endocarditis by A. neuii were previously reported [, ]. Both were in older men with preexisting aortic valvular anomalies (one had a bicuspid valve and the other a prosthetic valve). Both presented with subacute endocarditis, large aortic vegetations (2 cm) and root abscesses. The patient with a native valve underwent surgery []. Both patients were cured from the infection. One was initially treated with ampicillin, then ceftriaxone due to interstitial nephritis, and finally doxycycline for 9 months []. The other was treated with penicillin, followed by amoxicillin for 12 months [].
A 45-year-old man presented to our hospital in Belarus with the following history. He had been born in Belarus after an uncomplicated pregnancy. His family history was negative for congenital defects and the patient had no siblings. After birth, a systolic murmur was heard along the left sternal border leading to a referral to a pediatric cardiology centre in Ukraine where the diagnosis of tetralogy of Fallot was given based on physical examination, chest radiographs, and an electrocardiogram. Surgery was declined at that time. At no time in the patient's life were chromosomal studies undertaken. The patient had no children.\nAs a child, the patient had normal growth and mental development, but marked cyanosis, weakness, clubbing, and intolerance of moderate physical activity. The patient was referred to a medical institute in Moscow at age 18, after several episodes of syncope. A diagnosis of severe pulmonary stenosis with ventricular septal defect was considered at that time. A right Blalock-Taussig shunt was performed. The postoperative diagnosis was single ventricle type BIII with severe pulmonary artery stenosis and hypoplasia. After surgery, the patient was treated with digoxin, pentoxifylline, and spironolactone. The patient's condition improved significantly and he was able to walk several blocks without significant dyspnea.\nThe patient's condition remained stable for the next five years. He had shortness of breath with moderate exertion, but he was asymptomatic at rest. At 27 years of age, the patient reported an increase in dyspnea with minimal exertion. He was diagnosed with thrombosis of the Blalock-Taussig anastomosis and was treated with heparin for 4 weeks. He never returned to his improved, postoperative condition. He complained of palpitations, dull chest pain at rest, episodes of shortness of breath at rest, and abdominal pain. The patient had several documented episodes of ventricular tachycardia at age 35 years and was successfully treated with propafenone.\nAt 39 years of age, the patient presented to our hospital in Belarus. At the time of presentation, he complained of severe cyanosis, shortness of breath with minimal exertion, and chest pain. An electrocardiogram indicated sinus tachycardia of 120 beats per minute. The QRS axis was to the right (mean axis +130°). High QRS voltage suggestive of ventricular hypertrophy was noted. There were also ventricular extrasystoles in a trigeminal pattern and horizontal ST-segment depression in the inferior leads. A 24-hour cardiac monitor showed multiple episodes of non-sustained ventricular tachycardia with subjective feelings of palpitation and lightheadedness.\nThe patient was switched empirically from propafenone to mexiletine with better control of his ventricular tachycardia. His hematocrit was 58% to 64%. Transcutaneous oxygen saturation was 75% to 85% on room air. The patient could tolerate well most of his daily activities such as walking for two blocks, grocery shopping, and performing minor work at home. Over the next 6 years he had repeated hospitalizations for dyspnea, chest pain, and near syncope. He was treated with phlebotomies, saline and/or dextran infusions to improve viscosity, and medications including spironolactone, pentoxifylline, and aspirin.\nAt the time of the terminal hospitalization, physical examination was remarkable for a 3/6 systolic murmur heard at the upper left sternal border, edema of the right ankle and foot, and tachycardia of 140 beats per minute. The patient was noted to experience severe shortness of breath with even minimal exertion and was deeply cyanotic. The electrocardiogram is shown in Figure and demonstrated sinus rhythm with a mild tachycardia at a rate of 110 beats per minute, right axis deviation, and a non-specific intraventricular conduction delay with diffuse ST-T changes. Chest radiograph indicated a small pleural effusion on the right and increased pulmonary vascularity. The heart was moderately enlarged with prominence of the aorta. An echocardiogram was performed (Figure ). A single ventricle with unremarkable atrioventricular valves was seen. The end diastolic diameter of the ventricle was measured as 69 mm, the posterior wall thickness was 17 to 19 mm, and the left atrium measured 29 mm. The estimated ejection fraction was 45% to 50%. A vessel with a semilunar valve (the truncal valve) arising from the ventricle was seen. The cusps of the valve were hyperechogenic. Moderate regurgitation was noted. No pulmonary artery was seen. Doppler study of the lower extremities showed thrombosis in the veins of the right calf. Heparin therapy and intravenous fluids were initiated. The patient's condition deteriorated rapidly and several hours later he became comatose and died.\nAutopsy showed an enlarged heart that weighed 750 g, composed of two atria with an intact septum, and a single ventricle. The right atrium was enlarged to a diameter of 10 cm. The diameter of the left atrium was 3 cm. Both caval veins emptied normally into the right atrium. All four pulmonary veins entered the left atrium normally. Both atrioventricular valves had normal anatomy, free of vegetations. The single ventricle with left ventricular characteristics had a diameter of 12 cm with a wall thickness of 2 to 2.2 cm. A single artery, truncus arteriosus, arose from the ventricle and arched to the left. The truncal valve had three cusps that were moderately calcified. The coronary ostia and vessels were normal. The pulmonary artery trunk was located 3.5 cm from the origin of the truncus and divided to form left and right pulmonary arteries. At the hilum of the right lung the right pulmonary artery was surgically connected to the right subclavian artery. Red-gray masses were noted at the Blaylock-Taussig shunt anastomosis. Below the anastomosis the right pulmonary artery was almost completely occluded by a dark red adherent thrombus. The ligamentum arteriosum was a fibrous cord. Microscopically the lungs showed dilatation of the pulmonary arterioles and alveolar capillaries. Many bronchial and pulmonary arterioles contained recanalized thrombi or emboli.\nThe main anatomic diagnoses were: single ventricle with truncus arteriosus; status post Blalock-Taussig procedure; old thromboses of the established anastomosis; hypoplasia of the right pulmonary artery; congestive heart failure; recent thrombosis of the right pulmonary artery; and deep vein thrombophlebitis of the right calf.
A 65-year-old woman with controlled dyslipidemia and hypertension and a previous history of CABG 9 years previously was referred to our center because of the PSA. She had undergone coronary angiography due to symptoms which were suspected to be of ischemic origin in another hospital and during aortic root injection, the PSA was revealed. Coronary angiography showed that the left anterior descending artery and the right coronary artery were occluded at the proximal part; the left internal mammary on the left anterior descending artery was patent and the right coronary artery was filled retrogradely via the left coronary artery system. There was mild left ventricular systolic dysfunction. In addition, a large saccular aneurysm (25 × 55 mm) was detected in the ascending aorta (). These findings were confirmed by multidetector computed tomographic (MDCT) angiography, which illustrated a large pseudoaneurysm in the tubular part of the ascending aorta (maximum aneurysm diameter and PSA neck were 60 mm and 24 mm, respectively) with a calcified wall and without evidence of leak or pericardial effusion (). The diameters of the ascending aorta, arch, and descending aorta were 27, 20, and 17 mm, respectively, and the arch had 3 patent branches. In transesophageal echocardiography, the distance between the PSA and the aortic valve was 30 mm and the diameter of the oval-shaped orifice of the PSA was 20 × 29 mm. In addition, a thin layered clot was visible in the pseudoaneurysm cavity (). Cardiac surgery consultation was done, but the patient refused open repair owing to the high risk of surgery. At this point, we arrived at the decision to use an ASD occluder device in off-label way so as to seal the PSA orifice. Written informed consent, after explanation, was obtained from the patient.\nIn a hybrid operating room setting, under general anesthesia and with transesophageal echocardiography guidance, after open arteriotomy, a 30-mm Amplatzer-like ASD occluder device (Cardi-O-Fix, Starway Medical Technology, Inc. Beijing, China) was delivered via the right femoral artery using a 14-F sheath and pigtail catheter. To avoid the risk of perforation or rupture of the pseudoaneurysm sac by direct guide wire contact, we entered the pigtail catheter into the PSA cavity, with an approach similar to the insertion of the catheter for the left atrial appendage closure device (). Then, the sheath was introduced over the pigtail catheter through the PSA cavity. Finally, after the device was well-positioned in the orifice, aortic root angiography and transesophageal echocardiography showed no residual endoleak ().\nThe patient’s hospitalization course was eventless. Although dual antiplatelet therapy is not necessary for interventional procedures in the aorta considering the high flow pressure, our patient was low-risk for major bleeding and she was discharged healthy on Aspirin (80 mg/daily), off-label usage of Plavix (75 mg/daily for the first 3 months), Nitrocontin (2.6 mg/BD), metoprolol (25 mg/BD), atorvastatin (20 mg/daily), and ranitidine (150 mg/BD). At 3 months’ follow-up, MDCT angiography was repeated and it demonstrated that the device was in the appropriate position without endoleak (). Once more, we visited the patient 1 year after the procedure and she had been asymptomatic and healthy since hospital discharge. The patient refused our request for repeat CT angiography, which can be deemed a limitation in our follow-up period.
We present a case of a 58-year-old, right-handed gentleman with a medical history of Type II diabetes, obstructive sleep apnea, gastroesophageal reflux disease, asthma, gout, anxiety, and depression. He was an ex-smoker with a 40 pack-year smoking history. His oncologic family history was significant for his father dying from bladder cancer.\nIn the spring of 2014, the patient initially presented to his local emergency room with progressive frontotemporal headaches, a right visual field deficit, decreased appetite, weight loss, and a fall without sustaining traumatic injuries. On a review of systems, no respiratory, genitourinary, or additional neurological symptoms were described aside from increased daytime urinary frequency.\nPhysical examination revealed an obese gentleman (BMI of 41) with normal vital signs. The patient was alert, oriented to person, place and time, and ambulatory with a normal gait. Cranial nerve exam revealed right visual field deficits. Auscultation of the lungs revealed clear, equal air entry bilaterally without any adventitious sounds appreciated. No masses, organomegaly, or pelvic lymphadenopathy were appreciated on the abdominal exam, but this was limited due to his large body habitus. Digital rectal exam revealed a small, smooth, firm prostate without rectal masses.\nA CT and MRI of the head was completed with the latter revealing a heterogeneous mass in the left occipital lobe with solid and cystic components measuring 2.9 cm. There was associated cerebral edema with a 0.2 cm right-sided midline shift and no hydrocephalus identified (Figure ).\nThe leading differential diagnosis at the time was a primary brain malignancy, and accordingly, referral to neurosurgery at the same tertiary hospital was arranged. The patient subsequently underwent left occipital craniotomy and resection of the mass within five weeks of the initial referral. Pathology returned as metastatic adenocarcinoma, suggestive of a lung primary as it stained positive for cytokeratin 7 and TTF-1 and negative for cytokeratin 20. Adjuvant whole-brain radiotherapy was delivered to a dose of 30 Gy in 10 fractions, which was completed within six weeks from the time of surgery. The start date of radiotherapy was delayed by a couple of weeks as a result of a postoperative wound infection that was managed with oral antibiotics. During this time, the patient had a staging CT scan in the community encompassing the thorax, abdomen, and pelvis, which revealed a 5 cm left upper pole renal mass without other sites of disease. This report was not available in the patient’s record; however, the dictated notes from the medical team indicated that metastatic renal cell carcinoma was the provisional diagnosis.\nConcomitant with the planning and delivery of whole brain radiotherapy, the patient was also seen and assessed by the urology team at the same institution. Restaging CT imaging of the thorax, abdomen, and pelvis was completed two weeks after completion of radiotherapy, which revealed an exophytic solid renal mass arising from the upper pole of the left kidney measuring 4.9 cm. In addition, there were now two other areas of concern: a 3.1 cm heterogeneous pancreatic mass containing cystic components and a 2.1 cm pulmonary nodule in the lower lobe of the right lung. Both lesions, radiographically, were thought to be metastases from a primary renal cell carcinoma. Considerations for management included cytoreductive surgery, up-front Sunitinib, or enrolling the patient on a clinical trial (Figures -).\nHowever, before the patient could return to further discuss management options, an MRI scan of the head, completed two months after finishing radiotherapy to assess treatment effect, showed evidence of an intracranial abscess in the previous operative bed. Specifically, there was an interval development of a 4.2 cm rim-enhancing fluid signal in the post-surgical bed. Urgently, the patient was brought to the operating room for a left occipital craniectomy for evacuation and resection of the abscess. The patient recovered well without any additional complications, although did not wish to seek any further medical care at his local institution.\nThe patient’s primary care team in the community referred the patient to the multidisciplinary genitourinary oncology team at our center for a second opinion regarding management of the patient’s remaining disease. Following a review of the original imaging and pathology reports, the possibility of metastatic lung cancer (given the immunohistochemistry of the resected brain lesion favoring a lung primary) or synchronous primary malignancies was proposed. A PET-CT scan was ordered, which re-demonstrated the pulmonary mass in the lower lobe of the right lung, now measuring 3.0 cm with an SUV uptake of 9. A 1 cm non-18-FDG-avid subcarinal lymph node was also identified. The renal lesion measured 4.2 cm and was not 18-FDG-avid. A nodule in the pancreatic tail was thought to be a benign process, given its cystic appearance, stable size, and lack of 18-FDG uptake.\nThe case was also presented at our thoracic multidisciplinary team conference with two recommendations provided. First, it was advised to complete the diagnostic workup, including biopsies of both the lung and renal masses, in addition to sampling the mediastinal lymph nodes. Second, depending on the results of the investigations, treatment should be considered in accordance with the oligometastatic paradigm, which could involve sequential resection of the pulmonary and renal tumors.\nAn ultrasound-guided biopsy of the kidney mass confirmed renal cell carcinoma (clear cell). The sample stained positive for CKAE1/AE3 and vimentin, while staining negative for CK7, CK20, and TTF-1. Through an endobronchial ultrasound (EBUS)-guided biopsy, tissue was obtained from the right lower lobe mass and a paraesophageal (level 8) lymph node. The lymph node was negative for malignancy while the biopsy from the mass showed the presence of neoplastic cells. Given the limited sample, pathologists were unable to distinguish definitively between a well-differentiated adenocarcinoma and a neuroendocrine tumor at that time. It was evident, however, that the lung and kidney pathology were distinctly different.\nIn accordance with recommendations from both our multidisciplinary teams, the patient proceeded with radical intent treatment, first undergoing a video-assisted transthoracic surgery (VATS) right lower lobe lobectomy within two months of initial assessment at our center. Pathology revealed a unifocal tumor measuring 4.5 cm and was characterized as a Grade 2 papillary adenocarcinoma with negative margins. Eight lymph nodes were removed, and one peribronchial lymph node was found to be positive for malignancy with extranodal extension. Final lung pathologic staging was T2aN1.\nPostoperatively, the patient met and discussed with the medical oncology team regarding the benefits of adjuvant chemotherapy in the setting of N1 and oligometastatic disease. Given the unclear benefit systemic therapy might provide for completely resected oligometastatic disease in a patient with moderate comorbidities, adjuvant chemotherapy was ultimately declined by the patient despite it being offered.\nRestaging CT of the abdomen and pelvis was completed three months following VATS resection and demonstrated that the kidney mass had grown in size to 6.4 cm since previous imaging. The patient subsequently underwent a left partial nephrectomy three months later. Pathology revealed a unifocal 6.0 cm tumor in the upper pole of the left kidney, which was resected with negative margins. Histologically, the tumor was consistent with the previous biopsy, characterized as a clear cell renal cell carcinoma. Regional lymph nodes were not sampled. The final pathologic staging was pT1bNx, and no additional adjuvant treatment was felt to be indicated.\nThe patient was seen in follow-up three months following resection of the renal mass, which was 10 and 16 months from previous pulmonary and intracranial resections, respectively. He recovered well without surgical complications and was found to have no clinical evidence to suggest disease recurrence or progression. Repeat CT imaging of the head, thorax, abdomen, and pelvis revealed no evidence of recurrent or residual disease. Moving forward, the patient will continue with routine CT surveillance every three to six months.
We report on a 7-month-old boy with Downs syndrome who was born with an unbalanced left dominant atrioventricular septal defect and aortic coarctation. Initially he underwent aortic coarctation repair. However, despite good restitution of the systemic obstruction he could not be weaned from mechanical ventilation and subsequently underwent pulmonary arterial banding, which reduced pulmonary arterial pressure to 50% of systemic pressure and the pulmonary-to-systemic flow ratio to 1.5 : 1. Also a plication operation was performed for left-sided diaphragmatic paralysis. Supportive pharmacotherapy consisted of digoxin, captopril, spironolactone, and high-dose furosemide. In addition, a gastro-duodenal tube (G-Tube) was placed for feeding because of vomiting and poor oral intake. The hospital course was complicated by more than 10 endotracheal intubations for recurrent respiratory failure, which appeared to be triggered by exacerbations of an inflammatory disease process of unknown origin. During this time the infant underwent multiple courses of antibiotic treatment. Despite multiple diagnostic efforts, the cause of the recurrent sepsis-like exacerbations could not be determined.\nThe hemodynamic status was characterized by good cardiac performance of the functionally single ventricle despite persistent pulmonary hypertension (). There was clear evidence for ongoing pulmonary vascular disease with pulmonary hypertension and need for oxygen supplementation. In this situation, stage 2 palliation (superior cavopulmonary connection, bidirectional Glenn shunt) of his single ventricle defect was considered contraindicated. Since we hypothesized that the recurrent clinical symptoms of heart failure were caused by neurohormonal and inflammatory dysfunction we started a trial of low-dose beta-blocker therapy with 0.3 mg/kg/day propranolol (0.1 mg/kg per dose) by G-Tube in accordance with the German guidelines for congenital heart defects in children [, ].\nDuring the next fever episode the infant developed signs of hepatic failure and pulmonary edema and required intubation with mechanical ventilation as well as intravenous antibiotics and aggressive diuretic treatment. Since the hemodynamic and cardiac function improved, propranolol therapy was not stopped. He recovered from this initial episode of systemic inflammatory response syndrome (SIRS) (see SIRS I in ) within 3 days. He was extubated without dyspnea and began to tolerate oral feeding. However the fever continued unabated and when we stopped antibiotic coverage to collect blood cultures he decompensated with signs of an exacerbation of the SIRS II (see ).\nFurthermore we switched the medication to the selective β\n1-blocker metoprolol. The patient stabilized quickly again but the fever continued. Since all blood cultures remained negative, we hypothesized that the source of recurrent endotoxemia may be gastrointestinal. One week later we decided to remove as many potential sources of infection as possible. We removed not only the central venous catheter but also the G-Tube. Subsequent blood cultures and the central venous catheter cultures were negative, but resistant E. coli was recovered from the jejunal end of the feeding tube. The infant improved with oral treatment with cotrimoxazole. His supplemental oxygen requirements ceased during his oxygen saturation rose to 90% and the pulmonary arterial pressure decreased to the normal range. After six months of intensive care, including 12 intubations for respiratory failure, he was discharged home. Two days later he was readmitted for fever and decreasing oxygen saturations due to bronchospasm. An etiology of the bronchospasm could not be ascertained. Despite intensive therapy with systemic and inhalational β\n2-adrenergic agonists, theophylline, and high-dose prednisolone, the bronchospasm persisted and the infant's condition deteriorated. Metoprolol was discontinued and mechanical ventilation was reinitiated. A subsequent event of pulmonary hypertensive crisis ensued and unfortunately the patient expired (SIRS III). Post mortem the results of a bone marrow aspirate showed hemophagocytosis and the diagnosis of HLH was verified by a highly elevated ferritin and soluble CD25 receptor levels. Especially the cytokine profiles confirmed the diagnosis of HLH. Epstein-Barr virus infection could be excluded by polymerase chain reaction.
The patient was a four-year-old boy who was the third child of healthy Somali parents. They belong to the same tribe, but are probably not closely related. After an uneventful pregnancy, he was delivered at term without complications in Somalia in 2001. Birth weight, length, head circumference and Apgar scores are not known, but the mother described him as a healthy and normal infant until he was 5 months old. From this age he started to have seizure-like movements, which according to the mother's description, could have been myoclonic epilepsy or infantile spasms. At first, the seizures appeared at night, but soon they also occurred during the day, with increasing frequency and duration. The seizures stopped at 8 months after he was examined in a local hospital and put on antiepileptic drug therapy. Despite becoming seizure free, the mother described him as becoming markedly less responsive and alert.\nGrowth and motor development were normal for his age. He crawled at the age of 9 months and walked unsupported at the age of 12 months. Speech development was significantly delayed. The family moved to Norway when he was 1 1/2 years old. Although he could say some words at 2 years of age, verbal language skills improved only little after this age. At the age of 4 years he entered a day-care centre for autistic children, as it was observed that he had limited communication and social skills, gave poor eye contact and displayed a repetitive pattern of behavior.\nClinical examination at 5 years did not reveal any dysmorphic features. His muscle tone and strength and coordination and deep tendon reflexes were normal without signs of peripheral sensory deficit. His height and weight corresponded to the 25th centiles, and his head circumference was at the 75th centile. Eye examination with fundoscopy, hearing test, electroencephalography (EEG), echocardiography of the heart and magnetic resonance imaging of the brain were normal. Blood glucose, urea, creatinine, and liver transaminases were normal. Chromosome analysis showed a normal 46, XY karyotype. A Southern blot test for Angelman syndrome was negative.\nSince the time of the diagnosis of SBCADD our patient was on a trial with protein restricted diet (1 g protein/kg/day) for 5 months. The diet was reasonably well adhered to. He was tested by a psychologist using standard tests before and after 5 months of protein restriction treatment. No observable improvement was demonstrated during the period.\nWhen examined with ADOS-G (Autism Diagnostic Observation Schedule-Generic), he obtained a score of 13, just above the cut-off score of 12 for childhood autism []. ADI-R (Autism Diagnostic Interview-Revised) could not be administered, due to his mother's limited knowledge of the Norwegian language. When tested with Bayley Scale of Infant Development at 4 years, his cognitive ability score corresponded to that of 2 years. Like most autistic children, his visual-spatial abilities were much better than his language abilities. WPPSI-R (Wechsler Preschool and Primary Scale of Intelligence) at 5 years demonstrated visual-spatial abilities corresponding to 3 years of age. The verbal tests could not be administered due to lack of verbal abilities. Psychometric testing concluded with moderate mental retardation. This was also in accordance with the Vineland-2 Adaptive Behavior Scale, reported by his teacher. Motor development appeared normal when using the Movement Assessment Battery for Children test manual. During this examination, he was not able to follow verbal instructions.\nBecause mental retardation and autism sometimes can be caused by inborn errors of metabolism [], plasma and urine samples from the patient were analyzed by a metabolite screening system [] including gas chromatography-mass spectrometry (GC/MS) (organic acids), amino acid analyzer, tandem mass spectrometry (MS/MS) (purines, pyrimidines, acylcarnitines), and capillary electrophoresis (transferrin variants). Considerable amounts of 2-methylbutyryl glycine were identified in his urine. The excretion of 2-ethylhydracrylic acid was unremarkable. The patterns of amino acids and purines/pyrimidines were normal, except for a slight increase in glycine. MS/MS analysis of acylcarnitines showed increased plasma level of C5-acylcarnitine (1.43 micromoles/L, reference range 0.05–0.38) and an increased C5/C3-acylcarnitines ratio of 2.83 (reference range 0.16–0.73).\nDNA was extracted from blood and cultured fibroblasts by standard methods. PCR amplification of all exons and part of the flanking intron sequences of the human ACADSB (SBCAD) gene were carried out by use of intron-located primers under standard conditions in an automated thermal cycler (Thermal cycler 480, PE) []. The PCR products were sequenced in both directions using a 3100-Avant genetic analyzer and a BigDye® Terminator v1.1 Cycle Sequencing kit (Applied Biosystems). Sequence analysis of all exons, and part of the flanking introns of the human SBCAD gene from patient genomic DNA, revealed an apparent homozygosity for an A > G mutation, which changed the +3 position of intron 3 of the SBCAD gene (c.303+3A > G). No other changes were observed. Sequence analysis of the mother showed heterozygosity for the same c.303+3A > G mutation.
The patient was an 82-year-old male who was scheduled for a robotic left lower lobectomy for the resection of a biopsy-proven squamous cell lung carcinoma. He had a medical history of hypertension controlled with multiple medications; a 40-pack-year history of tobacco abuse, with a 20-year history of abstinence; and a prior history of melanoma of his left upper extremity, which was successfully resected.\nStandard American Society of Anesthesiology (ASA) monitors were placed and preoxygenation was performed; general anesthesia was induced with propofol, followed by the administration of muscle relaxant and the placement of a left-sided, double-lumen endotracheal tube. Additional venous access and arterial line placement occurred concurrently while the correct placement of the endotracheal tube was confirmed by bronchoscopy. The placement was then reconfirmed after the patient was situated in the right lateral decubitus position. The left lung was isolated and deflated, and the surgeon proceeded to place ports to facilitate the robotic resection as follows: the sixth intercostal space in the anterior axillary line, the third intercostal space in the anterior axillary line, and the ninth intercostal space in the posterior axillary line. The surgical procedure continued uneventfully from an anesthesia perspective with level 7 and 10 lymph node dissections, extensive lysis of adhesions and pericardial and pleural fat to facilitate visualization, and a left lower lobectomy. Surgical blood loss was estimated at less than 50 cc. Chest tubes were placed through the sixth and ninth intercostal port incisions. A bolus of 30 cc of 0.5% bupivacaine was administered via a catheter placed at the tenth intercostal space in the posterior axillary line for an elastomeric pump ball to infuse local anesthesia for post-operative pain management. Within 15 minutes of this, the patient began to experience hypotension, requiring incrementally increasing doses of vasopressors. Over the course of 10 minutes, escalating doses of phenylephrine were becoming ineffective at restoring the patient’s mean arterial pressures (MAP) to his intraoperative baseline (within 20% of the patient’s preoperative MAP). Vasopressin was administered, in 1-2 mg aliquots, as urgent assistance was summoned to the operating room. The hemodynamic instability was immediately reported to the surgeon. Based on the scant chest tube drainage, a thoracic source of bleeding was considered unlikely. A portable chest radiograph was ordered and point-of-care blood testing was performed. The hemoglobin was resulted at 6.5 g/dL, and transfusion was initiated while awaiting a confirmatory hemoglobin sent to the lab, which was resulted at 7.4 g/dL (preoperative hemoglobin 13. 5 g/dL). The chest radiograph did not show any areas suspicious for bleeding. The double-lumen endotracheal tube was removed and replaced with a single-lumen endotracheal tube to facilitate transport and the potential for prolonged mechanical ventilation, and the patient was transported urgently to the computed tomography (CT) scanner for chest, abdomen, and pelvis imaging. Upon review with the radiologist, the diagnosis was made of a subcapsular splenic hematoma with fluid extending to the diaphragm and tracking along the paracolic gutters, concerning for a splenic injury (Figure ).\nThe patient was then returned to the recovery room, where he continued to require ongoing blood transfusion and phenylephrine infusion to maintain satisfactory blood pressures. After receiving a total of four units of packed red blood cells, the phenylephrine infusion was able to be weaned significantly and serial hemoglobin values stabilized. Although both splenectomy and splenic embolization were considered in this patient, as his hemodynamic instability abated, the decision was made to observe him in the intensive care unit and to continue serial hemoglobin monitoring. On the first postoperative day (POD), he was weaned completely off of phenylephrine and was extubated. He was discharged to a rehabilitation facility on POD 15.
Mrs. S is a 28 year old married female who is an illiterate housewife. This patient came to our outpatient department with symptoms of decreased sleep and appetite and lack of interest in her usual household chores. The patient complained that her liver was "putrefying" and her heart was "altogether absent". She also reported that when she walked she could not feel her body and reported that her "stomach was missing". She was 6 months into a pregnancy with her first child.\nHer problems had started after she had left her joint family subsequent to frequent quarrels with her in-laws and had started to live along with her husband in a small rented accommodation. Initially she developed loss of sleep and palpitations and a feeling of loneliness which progressed to loss of interest in her surroundings and her daily household work. She began to have feelings of guilt about herself and felt that she had done something wrong in leaving the joint family. The patient's husband took her to a doctor who described her symptoms as 'vague', diagnosed her as having 'weakness' and prescribed multivitamin tablets. Over the next 6 weeks the patient's symptoms failed to improve. At that point, she consulted another doctor who, after an initial two weeks of unsuccessful treatment for her 'vague' abdominal complaints (missing stomach and putrefying liver), referred her for a surgical consultation. After examining the patient and carrying out various investigations, including ultrasonography and endoscopy, the surgeon continued the previous treatment and added an antibiotic for a week. Within the next 2 weeks the patient visited a faith healer and a practitioner of the Indian system of medicine. After this the patient consulted yet another doctor who referred her for a psychiatric consultation. In the meantime 3 months had passed since she had developed her symptoms. She had previously made an unsuccessful attempt at suicide by taking organophosphorus poison for which treatment included medications and observation. She had been advised to seek psychiatric consultation on discharge but had not complied. One month after the suicide attempt she had again thought of suicide but did not act on these thoughts because of her "fear of God".\nThe patient was dressed appropriately and was well groomed. Her speech was coherent and relevant. Her complaints included insomnia, a general loss of interest, lack of concentration and a feeling of hopelessness. She was found tohave depressed mood but would occasionally smile while answering questions related to her symptoms because the "doctors wouldn't believe her". She had partial insight into her symptoms. She was not concerned about her pregnancy and had ambivalent feelings of love and hate towards the unborn child. There was no disorder of content or possession of thought. Physical examination was unremarkable except the patient was anaemic with haemoglobin of 8.5 mg/dl. Other routine investigations, including thyroid function tests, were normal. A CT scan or an MRI was advised by a consultant to rule out any organic problem. The CT was not done because of her pregnancy and the MRI could not be done because of financial reasons. A DSM IV [] diagnosis of Major Depressive Disorder with Nihilistic Delusions was made.\nECT was suggested but the patient refused because of the social stigma associated with the procedure. Other possibilities were explored. Initially, the patient was reluctant to take drugs because of her pregnancy. After persistence and a few counselling sessions the patient agreed. The patient could not tolerate fluoxetine 20 mg because of persistent nausea. Escitalopram caused similar problems. Both drugs were discontinued within a week. Tricyclic antidepressants were not prescribed because of the side effects. The patient was put on mirtazapine 15 mg and haloperidol 5 mg. Both of these were increased to 30 mg and 10 mg, respectively, over the next five days. The patient showed remarkable recovery within the next 4 weeks. MADRAS [] scores came down to 18 from an initial score of 44. Over weeks four to eight the patient's mood and delusions improved. The improvement was sustained for four months and, at the time of writing, was in remission.
A 35-year-old Greek female patient, gravida four and para two, presented to our breast unit department due to a non-palpable breast lesion which was revealed via ultrasonic examination, with malignant ultrasonographical features. Her personal medical history and her psychosocial history were uneventful and Pap Smear tests were up to date and all negative. She was non-smoker and consumed alcohol only in social occasions. Her body mass index (BMI) was 30, 48 Kg/m []. Because of her mother’s breast cancer history (diagnosed with breast cancer at the age of 50, but never tested for BRCA), the patient was followed up via transvaginal ultrasonography and breast ultrasonic examination every six months since 2004.\nUpon arrival, physical examination of her breasts did not reveal any palpable mass. Breast ultrasonography showed a hypoechoic lesion of 0,9 × 0,8 cm located in the lower inner quadrant, while breast magnetic resonance imaging confirmed the suspicious and possible malignant finding on her right breast (MRM BIRADS IV) (). The chest x-ray test was normal. Her laboratory workup was all normal.\nUnder general anesthesia, a total excision of the lesion (which had been marked via a hook wire) was performed by a gynecologist specialized in breast surgery with 6 years’ experience in breast surgical procedures. The normal tissue of the breast, the nipple and the areola were conserved. The excised mass was almost 1 cm in diameter. The contralateral breast was normal. Ultrasound guided biopsy was not performed because BIRADS IV was overestimated (not necessarily malignancy). There were no peri-operative complications and the breast healed well. Post-operatively, the patient was followed up in the breast ward. She was administered antibiotics, fluids and painkillers intravenously. Histology confirmed the ultrasonic diagnosis, revealing a central low grade invasive ductal carcinoma and a peripheral in situ ductal breast carcinoma grade III (). The margins of the resected surgical specimen were negative for cancer cells.\nAfter almost a month from the first operation, the patient underwent a new surgical excision of unilateral right axillary lymphadenectomy of the first and second lymph nodes level. Twenty-two excised axillary lymph nodes were negative for metastasizing breast disease (0/22). Immunohistochemical analysis in cellular level showed ER (clone 6F11) stain positive in 100% of tumor cells, PR (clone 636) stain positive in 2% of tumor cells, Ki-67 (clone M1B1) stain positive in 60% of tumor cells and c-erb-2 (clone CB11)/HER 2 showing 3+ positive for cancer. Subsequently, the patient underwent treatment via chemotherapy, radiotherapy, herceptin and hormone therapy for the invasive ductal carcinoma Grade III (TNM staging: T1N0MO).\nGene evaluation for genetic mutations showed a BRCA 1 mutation; gene BRCA1 analysis was positive for mutations predisposing for breast or ovarian malignancy. More specifically the mutation p.Gly1738Arg (HGVS nomenclature)/ G1738R (BIC nomenclature) was detected. Thus, a prophylactic bilateral mastectomy was performed and followed by a successful plastic reconstructive surgery done by an experienced plastic surgeon, allowing for optimal aesthetic results.\nA year after the first operation, the patient underwent a prophylactic laparoscopic bilateral salpingoophorectomy and uterine diagnostic curettage at the age of 37. Before surgery, blood tests including tumor markers were all in normal levels (CEA: 1,3 ng/ml, CA15-3: 6U/ml, CA125: 8U/ml, CA19-9: 30 U/ml, AFP: 1,2 IU/ml). The chest x-ray test was normal and the preoperative magnetic resonance imaging of the upper and lower abdomen did not detect any pathological finding. The histological diagnosis of the surgical specimens was suggestive of a unilateral invasive high grade salpingeal cancer, mostly intraepithelial and minimal invasive (0, 1 cm) serous carcinoma of one salpinx. Immunochemical analysis was positive for P53 and Ki67 (). No other pathologic finding was detected.\nA further surgical evaluation was decided by the oncology council. Thus, the patient underwent an open total abdominal hysterectomy, omentectomy and bilateral pelvic lymphadenectomy by a gynecologist specialized in gynecologic oncology under general anesthesia. Postoperatively, the patient was followed up in a gynecology ward. She was administered antibiotics, fluids and painkillers intravenously. After a hospitalization of 5 days, she was discharged on the 6th postoperative day, in good condition. She was prescribed tinzaparin (u) for 8 days and cefuroxime (u) peros for 7 days. The patient was compliant with the therapeutic program which was well tolerated with no significant side effects. Histology was totally normal without any other pathological findings. Bone scanning was normal and did not reveal areas of increased radionucleide uptake. Similarly, the hip and pelvis x-ray were normal.\nThe patient is in excellent clinical condition and she is followed up via ultrasonography of upper and lower abdomen and breast magnetic resonance imaging every 6 months, without any pathological finding after the last surgery.
A 22-year-old female patient reported to the department of conservative dentistry with a chief complaint of pain and discoloration in relation to an upper front tooth. On examination, it was seen that tooth #11 presented with severe discoloration and considerable tooth structure loss on the palatal aspect []. The patient gave a history of prior attempted endodontic treatment a year back, and she could not follow up the treatment with the dentist as she had relocated. The onset of pain prompted her to seek immediate dental treatment.\nClinical examination revealed significant tooth structure loss in a failed attempt to gain access to the root canal resulting in thinning of dentin on the labial and palatal aspect of the crown and subsequent discoloration due to incomplete root canal treatment []. The tooth was mildly tender to percussion, absence of any swelling, and exhibited physiologic mobility. The radiograph revealed the presence of periapical periodontal widening along with dentin loss extending from middle third of the crown till the junction of the cervical and middle third of the root around the root canal [].\nAfter examining the case, the patient was informed regarding the present condition and the treatment plan was explained, with which she complied. The procedure comprised of root canal treatment, followed by restoration of iatrogenic tooth structure loss with a dentin substitute and intracoronal bleaching.\nAt the first appointment, the dentinal floor was explored to locate the root canal orifice, which had been obscured due to lodgment of debris since the access opening was exposed to the oral environment for a considerable time. After the canal orifice was located, a no. #15 K-file was used to scout through the canal to reach the apex. The working length was established at 21 mm using no. 30-K file and confirmed using an apex locator and radiograph. The cleaning and shaping was initiated, and thorough irrigation was achieved using sodium hypochlorite 5.25%. The canal was enlarged to size #50 using K-files only. After irrigation and drying of the canal, calcium hydroxide (UltraCal XS, Ultradent Products, Inc.) intracanal medicament was placed and secured with a cotton pellet and temporary restoration.\nThe subsequent appointment was a week later. There was no tenderness to percussion and patient-reported absence of pain. After removal of the temporary restoration, the canal was further irrigated with sodium hypochlorite 5.25%, normal saline, and final irrigation with chlorhexidine 2%. The canal was dried and prepared for obturation. AH plus sealer and gutta-percha were used for obturation using a lateral condensation method.\nAt the same appointment, the tooth was prepared for restoration of the lost tooth structure using Biodentine. The access cavity was flushed with saline and dried to remove any sealer and particles of gutta-percha. Biodentine was mixed in a triturator as per instructions and placed in the access cavity. Following this is a crucial step, which was carried out using an anterior Schilder plugger size 11 (1 mm diameter). The plugger was inserted into Biodentine projecting exactly above the coronal end of gutta-percha. It was moved in a circumferential motion to create a short groove of approximately 4-mm length uncovering the coronal portion of gutta-percha. The plugger was continuously moved making a circle of 2-mm diameter along with lateral flaring to form a triangular shape to simulate the pulp chamber. This was done to sustain the artificially sculpted orifice until the Biodentine had set (setting time: 12 mins) eventually replacing the lost dentinal structure as well as maintaining the natural canal anatomy [] []. Subsequently, the pulp chamber was sealed with temporary restoration until next visit.\nAfter a week, the patient was scheduled for intracoronal bleaching procedure. The patient was asymptomatic, and the radiograph revealed periapical healing. The procedure included an initial formation of an intraorifice barrier. Glass-ionomer cement (GIC) was mixed and placed in the artificially created orifice to form a 2-mm thick plug over the gutta-percha with a bobsleigh configuration []. Hydrogen peroxide-based powder and liquid bleaching system (Pola Office) were used for bleaching purpose. The powder and liquid were mixed to the consistency of wet sand and packed into the pulp chamber using a plastic instrument []. A cotton pellet was pressed over the bleaching agent, and a 2-mm thick plug of GIC was used to secure these contents into the pulp chamber [].\nThe next visit was scheduled after 2 weeks, at which the tooth bleaching was assessed. The patient was satisfied with the esthetic result of bleaching []. Hence, the bleaching agent was carefully evacuated from the pulp chamber and copiously flushed with water. The final restoration was done with fiber-reinforced composite (EverX Posterior) and final increment of conventional composite (3M ESPE Filtek Z350) [].\nThe patient was asymptomatic when called for a follow-up visit at 2 weeks, 3 months, 6 months and 1 year [].
The present case reports a 37-year-old man suffering from metastatic osteosarcoma originating in the distal part of the left femur. In March 2018, the patient entered the hospital with pain in the left leg as the major symptom. An MRI scan showed a large tumor with extramedullary parts and an intraosseous diameter of 13 cm. The histological examination of the biopsy showed a mostly epithelioid, in part osteoblastic, high-grade osteosarcoma. In the CT scans of the thorax and abdomen, there was no metastasis detectable. Before surgery, the patient was treated with a neoadjuvant regimen analog to the EURAMOS-1 trial () with two cycles of doxorubicin and cisplatin and four cycles of high-dose MTX. In the intermediate staging performed by a further CT scan before surgical resection of the tumor, there was still no sign of distant metastasis. In the restaging-MRI of the left thigh the tumor showed a decrease in size. Limb saving surgical resection of the entire tumor (R0) was performed in August 2018. The tumor showed regression with 30% vital tumor cells (grade IV Salzer-Kutschnik).\nSurgery was followed by an adjuvant chemotherapy analog to the EURAMOS-1-protocol containing two cycles of doxorubicin and cisplatin, two further cycles of Doxorubicin and eight cycles of high-dose MTX. The start of adjuvant chemotherapy was delayed for two weeks because of a wound infection.\nThe final staging after the last chemotherapy cycle showed two new pulmonary metastases in the CT scan of the lung. Hence, curatively intended surgical resection was performed in April 2019.\nIn September 2019, the patient had a seizure and in an MRI of the brain multiple cerebral metastases became visible. A neurosurgical resection of a symptomatic metastasis was performed, followed by a total brain irradiation with a boost on parafalcial and occipital metastases.\nIn a systemic restaging performed by a total body FDG-PET-CT scan and an MRI of the brain, the patient then showed a rapid systemic disease-progression with metastases affecting the lung, the mediastinum, the left adrenal gland, the brain, soft tissue, bones, and the skin. (, , )\nIn a molecular testing of the most recent tissue sample of the resected brain metastases, the tumor showed a high expression of PD-L1 (TPS 90% CPS 92%) but microsatellite stability (MSS). The patient was still in a good performance state (ECOG 1). A salvage chemotherapy containing the in osteosarcoma therapy established drugs ifosfamide and etoposide was not performed because of an acute kidney failure in the patient’s history and a high amount of cumulative neurotoxicity after the total brain irradiation. Benefit-risk ratio was not considered being favorable for this option. Referring to the case of a patient with advanced osteosarcoma reported by Nuytemans et al. (), who reached a stabilization of disease-progression undergoing immunotherapy with nivolumab and ipilimumab, an individual therapy attempt with the same treatment combination was conducted, as there was no further established therapy and no ongoing study available.\nStarting in December 2019, we exposed the patient to the immunotherapy combination of Nivolumab 3 mg/kg and Ipilimumab 1 mg/kg every 3 weeks for four times analog to the established treatment protocol for kidney cancer. In the following restaging performed by a PET-CT scan and an MRI of the brain 3 months after starting the therapy, the patient showed a clear response to the therapy with a profound remission of all tumor lesions (, , ). In some of the lesions, a minimally elevated uptake of FDG remained residually, whereas the lesions were not metrically measurable any more in the corresponding CT scan. In brain MRIs, minimal residual structures were interpreted as gliosis after total brain irradiation and immunotherapy. A definite distinction between inflammation or scar and minimal tumor residuals was not possible in PET-CT scans and MRIs.\nIn February 2020, the patient suffered from herpes zoster as a complication, which was treated with brivudine for 7 days.\nThe patient developed a mild facial palsy of the right side in March 2020, which can be considered as a side effect of the immunotherapy. In an examination of the cerebrospinal fluid, a slightly increased cell count of 9/nl could be detected but no signs of VZV encephalitis or meningeosis carcinomatosa, respectively.\nIn March 2020, the patient developed an immunotherapy-related pneumonitis with clinically mild symptoms but clear correlations in CT scans of the lung () and noticeably reduced diffusion capacity in a subsequent lung-function examination. Therefore, immunotherapy had to be discontinued, and nivolumab maintenance could not be started according to protocol.\nFor treatment of pneumonitis, the patient received prednisolone with an initial dose of 50 mg per day (0.5 mg/kg). Because of decreasing signs of pneumonitis in control CT scans () and an improving diffusion capacity in lung function, prednisolone could be quickly tapered to 7.5 mg, and re-exposure to nivolumab was feasible in June 2020. In the actual PET-CT scan and MRI of the brain, the patient still showed a profound remission of all tumor lesions, and there was no detectable sign of a relapse (, ). Currently, prednisolone is completely tapered, and the patient undergoes nivolumab maintenance (240mg) every 2 weeks. The performance state has further improved, and the patient is starting reintegration into work.\noutlines the patient’s history.
A 52-year-old man presented in the emergency department of our hospital with a history of typical angina and recurrent electrical storms with an in situ cardiac resynchronization therapy defibrillator (CRT-D) device, implanted 9 years ago.\nThe patient’s medical records revealed that an emergency coronary angiography had been performed 11 years ago for a transient posterior wall myocardial infarction. Interestingly no culprit lesion was discovered, with simultaneous left ventriculography showing no significant LV-function impairment. Two years later, the patient was readmitted with severe dyspnea and recurrent angina. A repeat coronary angiography could not establish an ischemic cause of the symptoms in the absence of any relevant coronary artery stenosis. Echocardiography showed a dilated left ventricle with severely reduced left ventricular ejection fraction (LVEF = 28%). Further investigation with cardiac-MRI, in the absence of any extensive myocardial scarring and specific tissue characteristics, excluded probable ischemic, dilated, hypertrophic, restrictive and arrhythmogenic aetiologies of severely reduced left-ventricular function. The precise pathophysiology governing the heart failure and ventricular remodelling remained in this case obscure. Hence, a provisional diagnosis of idiopathic nonischemic cardiomyopathy was proposed.\nIn view of continuing symptoms despite optimal heart failure regimen and without further improvement of left ventricular function, a primary prevention strategy with programmed ventricular stimulation was adopted. The patient underwent conventional CRT-D system implantation in the presence of a complete left bundle branch block (LBBB).\nRemarkably, the patient was a classic CRT-D responder with the improvement of LVEF up to 42% with no unusual electrical activity over the period. As part of a continuous heart care program, the patient had been attending regularly scheduled cardiology follow-up consultation. The patient showed to date no further deterioration of his cardiac function and had no clinical episodes of VT or shocks; however, he continued to have intermittent angina throughout. None of the further records revealed any ischemic cause of symptoms, except one of the angiograms showing the non-traceability of RCA.\nNine years after the implantation of CRT-D, the patient presented to us experiencing electrical storm for the first time, with a total of 22 malignant ventricular tachyarrhythmic episodes that were recorded in the tachycardia zone (Fig. ). After a few unsuccessful attempts at anti-tachycardiac pacing (ATP), the device delivered a total number of 25 appropriate maximal energy shocks at 38 J following ongoing tachycardia episodes.\nA 12-lead electrocardiogram (ECG) (Fig. ) at presentation showed ventricular tachycardia (VT). The patient was treated with 300 mg intravenous amiodarone bolus, which subsequently terminated the electrical storm. Later, 1000 mg/day in three divided doses, oral amiodarone was administered to establish the desired plasma level of the drug. An acute ST-Elevation Myocardial Infarction (STEMI) was subsequently excluded in a follow-up 12-lead ECG showing ventricular-paced rhythm without any relevant concordant ST elevation (“negative” Sgarbossa criteria) (Fig. ).\nFurther physical examination revealed that our patient was highly anxious and dyspneic with an initial fluctuating heart rate of over 200/min and blood pressure of 126/80 mmHg. The patient denied any recent episode of syncope. On pulmonary auscultation, bilateral basal crepitations were audible, which was later confirmed by chest X-ray as bilateral pulmonary congestion. The cardiovascular risk factors associated with the patient were hypertension, history of smoking (around 20 cigarettes/day) and elevated body mass index (BMI = 37).\nInitial laboratory test results showed normal levels of serum potassium, magnesium and thyroid stimulating hormone; however, the levels of creatine phosphokinase and creatine phosphokinase (MB) were elevated, i.e. 671 IU/L and 41 IU/L, respectively (optimal range CK 0–174 and CK-MB 0–25 IU/L). High-sensitivity troponin assays revealed a staggering increase from 41 pg/ml to 264 pg/ml within 2 h of the initial presentation (optimal range 0–14 pg/ml). The echocardiography and other laboratory investigations did not support left ventricular hypertrophy, acute deterioration of cardiac function (ejection fraction = 40%), electrolyte disturbances or any other triggering factors as an etiology for arrhythmic storm and acute coronary syndrome (ACS).\nBased on his history of continued symptoms and increased level of cardiac enzymes, the patient underwent immediate catheterizations. The diagnostic coronary angiogram excluded the presence of any culprit lesion, but revealed a non- significant 30–40% stenotic multivessel coronary disease of left anterior descending artery (LAD) and left circumflex (LCX) with normal TIMI III flow pattern. Anatomically, the left main coronary artery was observed to originate from the left aortic sinus. With the conventional angiogram using a right Judkins catheter after several attempts at cannulation, the course of the RCA was not traceable. Hence it was followed by the computed tomographic (CT) coronary angiogram, which revealed an RCA originating from the left coronary sinus (Fig. ) with an interarterial course in between the aorta and pulmonary artery leading to dynamic compression with evident decreased luminal diameter at the origin of RCA between the great vessels without any distinct narrowing of the remainder of the RCA (Fig. ).\nBased on the initial ECG morphology of VT (Fig. ) with a right bundle branch block RBBB-like morphology, an early precordial transition at V3 and predominant S in aVF; the wavefront of VT was therefore thought to exit and activate in the inferior aspect of the left ventricle. Subsequently, we urged for an electrophysiology study, to which the patient did not give consent. Cardiovascular surgical evaluation of the interarterial compression of RCA excluded the need for any immediate surgical intervention due to the lack of concrete evidence of any myocardial ischemia.\nOver the next 12 h, the patient’s clinical status improved without any recurrence of arrhythmia. The heart failure regimen was optimized with increased dosage of the β-blocking agent (metoprolol) and ACE inhibitor (ramipril) along with the addition of an aldosterone antagonist (eplerenone). We proposed the new ARNI (Angiotensin receptor- Neprilysin inhibitor) as an alternative to an ACE inhibitor as per the current heart-failure guideline, but the patient denied this therapeutic proposition. After close monitoring for any adverse effects, the patient was finally discharged on a maintenance dose of 200 mg/day of amiodarone.\nThe patient continued to experience persistent angina at rest, for which he was again hospitalized after 3 weeks. His condition prompted an ischemic evaluation to determine the extent of the myocardial perfusion. Radionuclide imaging using myocardial perfusion scintigraphy (MPS) was performed, which revealed extensive reversible perfusion defect involving the inferior and posterior wall coinciding with myocardial territory supplied by the RCA (Fig. ).\nIn the absence of any culprit lesion along with MPS based preliminary evidence of delineated ischemic area consistent with the VT exit site on 12-lead ECG, we hypothesised that the ventricular arrhythmia and severe angina episodes could be due to the atypical anatomy of the RCA with an interarterial course. Hence, we referred the case for surgical re-evaluation. After a thorough discussion, the patient was transferred to the cardiothoracic department for surgical unroofing, believing that the ischemia was due to interarterial dynamic compression of RCA. The patient was followed-up for 10 months after surgery; he remained asymptomatic without any further episodes of angina pectoris or electrical storms.
An 11-year old female came to the clinic after her parents complained of missing lower left teeth. No pain or previous discomfort was reported. The overall patient’s dental and physical health was good with non-specific general medical history and no contra-indication to dental treatment. A signed informed consent from the patient’s mother was obtained before the patient participated in the study.\nExtraoral examination revealed a symmetric face with no deficit in the lower left part of the face. Intraoral examination revealed a Class II incisor relationship and a Class II molar relationship from the right side in a late mixed dentition. At the left side, the first mandibular molar was clinically absent, and the overlying mucosa was normal in color and texture. The adjacent deciduous second molar had a large amalgam restoration with no signs of secondary caries.\nThe panoramic radiographic examination (PanRad) revealed the presence of six permanent molars in the upper jaw and five permanent molars in the lower jaw (). From the size of the teeth, the stage of the root formation, the location of the teeth buds, and the angulation of the impacted molar, it was assumed that the impacted tooth was the first mandibular molar, and the adjacent tooth bud was the mandibular third molar. A well-circumscribed unilocular radiolucent lesion in the body of the mandible was noticed, associated with the crown of the vertically impacted mandibular left first molar. The roots of the impacted molar were completely developed with closed apexes. The cephalometric X-ray confirmed a skeletal Class II malocclusion (). The clinical diagnosis was dentigerous cyst associated with the impacted molar.\nThe main objectives of the treatment plan were to eliminate the cystic lesion and establish a functional occlusion. The latter should include expansion of the upper arch, leveling and alignment of both arches, closure of any residual spaces of missing teeth, and establishment of a functional molar relationship. After taking into consideration the age of the patient, the missing mandibular left molar and her occlusal status, a combined surgical-orthodontic approach was decided upon. Both lower deciduous molars were extracted under local anesthesia. Two metal bands were cemented to the maxillary first permanent molars, and a Quad-Helix appliance was inserted in the palatal tubes of the bands. Eight months later, all mandibular permanent premolars had fully erupted, and a new panoramic examination showed that the impacted molar was still at the same height ().\nFixed orthodontic appliances (MBT, Dynaflex®, 10403 International Plaza Drive, St.Ann., MO, USA) were placed in the mandibular arch. After three months of active orthodontic treatment, initial alignment was achieved, and exposure of the impacted tooth was performed. A muccoperiostal flap was raised under local anesthesia, and a communication was established between the cystic cavity and the oral cavity. A specimen of the cyst was sent for biopsy. The crown of the impacted tooth was exposed, and a conventional molar attachment was bonded on the buccal side perpendicular to the long axis of the second molar with a self-etching adhesive. To upright the second molar, elastic traction was applied.\nHistopathological examination confirmed the diagnosis of a dentigerous cyst.\nAfter two months of orthodontic extrusion, radiographic examination showed a significant reduction in the size of the cystic cavity and a more favorable position of the first molar (). Orthodontic traction continued for 16 more months, while the upper dental arch was also treated with fixed orthodontic appliances. Eighteen months after the surgical procedure, the first molar had taken a very favorable position in the dental arch, thus debonding of the fixed appliances was decided due to the patient’s unwillingness to continue with the fixed appliance treatment. The previously impacted molar was left to erupt spontaneously, while the upper left molars were stabilized in order to prevent over-eruption ().\nThe patient reported for follow-up appointments every two months, and finally, 14 months after the debonding of the fixed appliances, the molar had fully erupted (). A stable occlusion was achieved, both arches were well aligned, and there were no residual spaces.\nRadiographic examination revealed an almost vertical position of the mandibular first molar and normal trabecular bone surrounding the previously impacted tooth ().
A 74-year-old Japanese woman was referred to our hospital with a history of several days of jaundice. She had no past medical history and no family history. On examination, her skin was jaundiced, and her abdomen was flat and soft. Her laboratory data revealed obstructive jaundice and cholangitis, and tumor marker levels were elevated with carcinoembryonic antigen at 3.6 and carbohydrate antigen 19-9 at 4573.9. Computed tomography (CT) revealed an enhancing mass in the biliary duct hilum and dilation of the intrahepatic bile ducts, and there was no evidence of lymph node metastasis or distant metastasis. Endoscopic retrograde cholangiopancreatography revealed a luminal filling defect in the biliary hilum (Fig. ). We diagnosed the tumor as a Bismuth type 1 cholangiocarcinoma, and performed endoscopic retrograde biliary drainage. Biopsies of the tumor revealed adenocarcinoma. The estimated volume of the postoperative liver remnant was less than 35%. Therefore, percutaneous transhepatic portal vein embolization (PTPE) of the right branch of her portal vein was performed (Fig. ). Twenty-one days later, the volume of the left lobe of her liver increased, and we performed right hepatectomy, caudate lobectomy, extrahepatic bile duct resection, and lymph node dissection. Node dissection included resection of hilar and pericholedochal nodes in the hepatoduodenal ligament, common hepatic artery nodes, and those at the celiac trunk, and posterior and anterior pancreaticoduodenal nodes.\nHistological examination of the tumor showed moderately differentiated tubular adenocarcinoma without regional lymph node metastasis, a pathological stage II tumor according to the Union for International Cancer Control classification of malignant tumors, 7th edition (Fig. ). Postoperative blood laboratory tests showed that liver enzymes were slightly elevated, but that total bilirubin was within normal limits. Resumption of diet started on postoperative day (POD) 3. Although the fluid in the abdominal drain had been serous until POD 4, the appearance of the fluid became milky on POD 5, and the amount of the drainage increased up to 1 L/day. CT showed a large amount of ascites with a small right pleural effusion. We placed our patient on total parenteral nutrition (TPN), and the ascites gradually decreased and became serous again. The abdominal drain was removed on POD 27, and an oral diet was restarted. She experienced sudden dyspnea on POD 42, and CT showed a massive right pleural effusion and a small amount of ascites (Fig. ). We performed a thoracentesis and placed a chest tube. One liter of chylous effusion was drained, and the triglyceride concentration of the pleural fluid was 1026 mg/dL. With a diagnosis of chylothorax, she was started again on TPN and given subcutaneous octreotide injections. Although the chest drained approximately 2 L/day for several days, both the output and the pleural effusion decreased on chest X-ray. On POD 46, the pleural effusion nearly completely resolved and the chest tube was removed. From then on the octreotide was stopped. Her oral diet was resumed on POD 57, and subsequent CT revealed recurrence of the massive right pleural effusion and a small amount of ascites, leading us once again to make our patient take nil by mouth (Fig. ).\nGiven that conservative management was ineffective in definitively treating the chyle leak, we performed lymphangiography through the inguinal lymph nodes with Lipiodol (ethiodized oil) on POD 62 to identify the location of the chyle leak and to develop a therapeutic strategy. Lymphangiography and post-procedure CT revealed that there was extravasation of the Lipiodol (ethiodized oil) near the right mediodorsal pleural space along the diaphragm, with an accumulation of Lipiodol (ethiodized oil) located near the staple line of the stump of the right hepatic vein; the exact location of the leak was not identified (Fig. ). CT also showed that there was neither obstruction nor dilation of the thoracic duct (Fig. ). An abdominal source of the chyle leak was not demonstrated. A chest tube was placed after the lymphangiography. The tube drained less than 500 mL/day for a week, and the pleural effusion resolved 10 days after the lymphangiography. She was discharged on POD 72. Two and a half years after the original surgery, she is doing well without evidence of recurrence of either chylothorax or cancer.
A 17-year-old female patient was referred to the Faculty of Dentistry of our institution for consultation. The patient was diagnosed with dental trauma in the maxillary central incisors and right lateral incisor. The patient's clinical history revealed that the patient has epilepsy, with a family history of diabetes and hypertension. On the 1st day, the patient received an emergency treatment with the restoration of central incisors and the right lateral incisor with glass ionomer cement.\nIn the right central incisor, we observed an oblique coronal fracture located below the middle third and pulp exposure. The upper right lateral incisor and left central incisor had coronary fractures extending from the mesial, almost to the level of gingival margin, to the distal end; all of the fractures had pulp exposure []. There was an active fistula in the vestibular region of the right central incisor and evidence of luxation in the same tooth [Figure and ]. Clinically, the patient still had a visible plaque index of 46.4% and gingival bleeding of approximately 31.2%. The residual roots of the #16 and #26 teeth were present as well as white spots in all molars and an active cavity at #46. Furthermore, there was a need for endodontic therapy in all incisors affected by the trauma [].\nThe treatment planning strategy for the treatment was carried out in two stages. In the first stage, endodontic procedures were performed on the three incisors. Next, the bonding procedure and restoration were performed. The teeth involved had irreversible pulp involvement, and because of the extent of the fractures associated with an occlusion overbite, the possibility of bonding failure was considered. This fact would provide the need for intraradicular pins for the reconstruction of the teeth. Thus, endodontic treatment was the choice of the professionals responsible for the patient.\nAfter the endodontic access of the incisors as described above, irrigation of the pulp chamber and cervical third with 5% sodium hypochlorite was performed with an endodontic instrument type K (Dentsply-Maillefer, Ballaigues, Switzerland). The mechanical chemical preparation was performed with irrigation, aspiration, and inundation with 5% sodium hypochlorite and 17% ethylenediaminetetraacetic acid. The canals were irrigated with saline, and a calcium hydroxide paste was added. The elements were sealed with the glass ionomer restorative cement, Maxion R (FGM Dental Products, Joinville, SC, Brazil), provisionally until the next clinical appointment for the definitive obturation of the three incisors.\nIn the third visit, after 16 days, tooth #11 displayed lower mobility than what was observed in the first clinical session. Under absolute isolation and removal of the provisional ionomer material, the removal of the intracanal calcium hydroxide and a final obturation of the root canal with mineral trioxide aggregate (MTA) (MTA-Fillapex, Angelus, Londrina, PR, Brazil) were performed []. In the next session, a dental prophylaxis was carried out. To bond the fragment in tooth #11, the composite resin Amelogen enamel B1 and A1 (Ultradent Dental, South Jordan, UT, USA) was chosen. The fragments were cleaned with chlorhexidine digluconate solution at 2% (FGM Dental Products, Joinville, SC, Brazil) for 1 min; a rubber dam (Dental Dam Hygenic, Coltène/Whaledent GmbH, Langenau, Germany) and the adjacent dental elements fracture were insulated with polytetrafluoroethylene tape (Polytubes, Pulvitec, São Paulo, SP, Brazil). The coronary fragment and the remaining tooth structure were etched with gel phosphoric acid 35% (Ultra-Etch, Ultradent Products, South Jordan, UT, USA) for 30 s on the enamel and 15 s on the dentin, followed by extensive washing with air/water spray and subsequent drying air jets.\nThe dental bonding adhesive procedures were technically similar to that performed on the three incisors. AdheSE bond (Ivoclar Vivadent AG, Schaan, Liechtenstein) adhesive was used. The clinical sequence followed an alternating pattern, starting first with the right lateral incisor [], followed by the right central incisor [] and then the left central incisor where there would be a need to complement with composite resin [Figures and ]. To facilitate the bonding, we used a securing device, OptraStick (Ivoclar Vivadent AG, Schaan, Liechtenstein), and photopolymerization for 40 s in the buccal and 40 s on the lingual surface using polymerizing halogen light with an intensity of 1400 mW/cm2 (Radii LED Curing Light, SDI, Australia).\nThe beveled end was used superficially on the tooth fragment of the vestibular external interface enamel of the teeth with the latest aid spherical diamond, FG 1012 (KG Sorensen, Cotia, SP, Brazil), and reinforced composite microhybrid Amelogen (enamel B1). The final polishing was performed using interface Sof-Lex disks (3M ESPE, St. Paul, MN, USA) and a high gloss polishing slurry (Opal L, Renfert GmbH, Hilzingen, Germany) with the aid of fur brushes mounted goat suede and cotton on the labial surface and palate of all external enamel.\nClinical and radiographic controls were performed 2 and 5 months after the adhesive bonding procedure []. The color disharmony that was encountered initially in the restored teeth resolved significantly on its own accord within the preservation period after the reattachment of the fragments.
A 45-year-old male was admitted to the hospital with a one-day history of abdominal pain and dyspnea. The abdominal pain started the day of admission; however, he noted worsening shortness of breath and a productive cough with clear sputum over the course of several days. He also noted a 20-pound weight loss occurring over a three-month period but denied fevers, chills, or night sweats. Physical exam was notable for tachycardia, distant heart sounds, and decreased breath sounds of the lung bases bilaterally. Vitals at presentation included a temperature of 99.2°F, heart rate of 104, respiratory rate of 20, blood pressure 182/106 mmHg, and an oxygen saturation of 95% of 2 liters via nasal cannula.\nOur patient has a past medical history significant for hypertension, chronic anemia, and end stage renal disease, due to focal segmental glomerular nephritis, on hemodialysis three times per week. He has required hemodialysis for approximately four years and unfortunately does not have an arteriovenous fistula due to financial restraints; therefore, his dialysis access is via a left jugular tunneled catheter. He has required multiple tunneled catheters over the past four years.\nInitial metabolic panel was within normal limits except for his creatinine of 7.99 mg/dL. Complete blood cell count revealed anemia with a hemoglobin of 10.6 g/dL and hematocrit of 33.7% but otherwise within normal limits. Chest x-ray was remarkable for bilateral pleural effusions and prominence of the cardiopericardial silhouette consistent with pericardial effusion (). Given his abdominal pain a CT of the abdomen and pelvis without intravenous contrast was performed showing large right and small left pleural effusions and a large pericardial effusion. Since the CT scan was able to capture a majority of both the pleural and pericardial effusions a dedicated CT scan of the thorax was initially deferred.\nGiven his dyspnea and pleural effusions our patient underwent a right-sided thoracentesis by interventional radiology with removal of 1.5 liters of cloudy amber-colored fluid. Body fluid studies revealed a total protein of 3.3 g/dL, LDH 110, glucose 106, RBC 45, WBC 993, lymphocyte predominance of 91%, and pH of 7.0. Serum LDH was 247 and serum protein was of 6.6 g/dL. Using Light's Criteria () our patient did not meet criteria for exudative effusion; however, results were borderline with a pleural fluid protein/serum protein ratio of 0.5 and pleural fluid LDH/serum LDH ratio of 0.445. Acid fast smear and cultures were obtained and resulted negative. Cytology was negative for malignancy but showed many small mature lymphocytes, mesothelial cells, and a few acute inflammatory cells.\nFollowing thoracentesis, he had significant improvement in dyspnea and oxygen saturation improved to the upper 90s on room air. Regarding the pericardial effusion, a transthoracic echocardiogram was obtained revealing a large circumferential pericardial effusion measuring 1.67 cm anteriorly and 1.29 cm posteriorly. The chambers of the right atrium (RA) and right ventricle (RV) were very small, with complete obliteration of the RV chamber during systole, suggesting high intrapericardial pressure. The IVC was collapsible, measuring 1.67 cm, with a 50% collapse during inspiration. No diastolic collapse of the RV or complete systolic collapse of the RA was noted. Cardiology was consulted and during their physical exam he displayed no jugular venous distention, negative Kusmaul's sign, and no clinical signs of tamponade. An EKG was obtained showing sinus rhythm and no electrical alternans; thus pericardiocentesis was deferred.\nOver the next four days he continued his regularly scheduled hemodialysis sessions and showed improvement in the pericardial effusion. Give hemodynamic stability and significant improvement there were no plans for intervention and the decision was made to closely follow with cardiology as an outpatient. However, on the planned day of discharge he developed supraventricular tachycardia and was kept another night for observation and evaluation by electrophysiology. During this time a repeat chest X-ray was performed revealing a reoccurrence of his pleural effusions; therefore, pulmonary medicine was consulted to further investigate.\nPrevious body fluid studies were reviewed, and, given significantly elevated lymphocytes of 91% and borderline Light's Criteria for exudative effusion, there was initial concern for common etiologies including tuberculosis, sarcoidosis, and malignancy. Our patient immigrated from Mexico to the United States approximately 20 years ago but denied any Tuberculosis exposure or previous diagnosis. He did have a 20-pound weight loss but denied other B like symptoms. We elected to further investigate the thorax for the presences of anatomical abnormalities, so a CT thorax was obtained. CT demonstrated a reaccumulation of the right pleural effusion and persistent moderate to large pericardial effusion () but surprisingly showed stenosis or occlusion of the superior vena cava (). This new finding was discussed with radiology that further commented on mediastinal collaterals and evidence of SVC stenosis particularly around the patient's tunneled catheter.\nA repeat thoracentesis was performed and 1.6 liters of milky fluid with a pink tinge was removed. Body fluid was remarkable for total protein of 4.6 g/dL, glucose 72, triglycerides 1,056 mg/dL, cholesterol 116 mg/dL, RBC 423,195, WBC 1,760, lymphocytes 86%, and pH 8.0. These findings were consistent with chylothorax likely form newly discovered SVC stenosis.\nOther labs to evaluate for autoimmune etiology including rheumatoid factor, c-ANCA, p-ANCA, ANA, and hepatitis screen were also obtained and were unremarkable.\nRepeat echocardiogram was performed and worsening pericardial effusion was noted revealing RA collapse and diastolic RV collapse suggestive of cardiac tamponade (). Hemodynamically the patient remained stable and a scheduled pericardial drain was placed with aspiration of 775 mL of bloody fluid consistent with chylopericardium.\nGiven recurrent pleural and pericardial effusions vascular surgery was consulted for SVC stenosis. He underwent angioplasty of the SVC and left brachiocephalic vein using a 12x4 angioplasty balloon and replacement of the tunneled hemodialysis catheter into the left jugular vein. Ultimately, after balloon angioplasty, high triglyceride diet, and draining both the pleural and pericardial effusion his symptoms significantly improved, and he was discharged home. There are plans to establish permanent hemodialysis access by creating an AV fistula in the near future.
A 39-year-old Caucasian man was referred to our emergency department due to a sudden loss of hot and cold sensation when taking a shower, followed by a progressive instability and weakness of his lower extremities. Our patient also complained of diplopia, dim vision and circumoral numbness. Our patient reported an upper respiratory tract infection for which he was being treated with an antibiotic (macrolide). Neurologic symptomatology began a few days later. His medical history was unremarkable apart from seasonal asthma. He used to smoke 20 cigarettes per day and drink alcohol only on social occasions. He was the father of two children. No high-risk sexual behavior was reported.\nOn physical examination, he had hot and cold temperature sensory disturbance under the T4 vertebrae level, symmetrically diminished muscle power mainly to his lower limbs, blurred vision and a loss of taste. Further neurological examination revealed paresis and diminished reflexes of his lower limbs; mainly the jerk ankle and patellar reflex. Cerebral pathology results following an MRI scan were negative. However, our patient reported a loss of balance, a failure to stand or walk and numbness. In summary, our patient showed progressive weakness of his lower limbs due to neuropathy, areflexia, sensory involvement and cerebella ataxia, and with the duration of the disease being less than four weeks, the required and some of the supportive diagnostic criteria for GBS were met. Other physical examinations were normal.\nTwo lumbar punctures were performed, the first on admittance and the second 14 days following commencement of the symptoms (Table ). The diagnostic tests are summarized chronologically in Table . Serology tests for neurotropic viruses were requested for differential diagnosis purposes. Hepatitis, human immunodeficiency virus (HIV) and varicella zoster virus (VZV) test results were negative, as were results for West Nile virus (WNV) with indirect enzyme-linked immunosorbent assay (ELISA) immunoglobulin G (IgG) and M antibody capture (MAC)-ELISA (IgM). However, CMV, herpes simplex virus (HSV) and EBV IgM antibody test results were positive. Blood CMV antigen and polymerase chain reaction (PCR) tests for CMV were negative, as was the PCR test for CMV from the cerebrospinal fluid (CSF). Blood, urine and CSF cultures were sterile. A tuberculin skin test and CSF culture for tuberculosis (TBC) were negative. Serology tests were repeated one month after hospital admittance and at a six month follow-up appointment. The comparative results are summarized in Table .\nResults of a chest X-ray were negative. A brain MRI scan showed no abnormalities of the cerebral parenchyma, but a slight inflammation of the ethmoid and right mastoid sinus was noted. In view of the neurological findings, his jugular and lumbar spinal column were scanned. No abnormalities except degenerative lesions and slightly slipped disks were noted, but these findings were irrelevant to the clinical symptoms. Brain, lung and abdominal computed tomography showed no abnormalities.\nElectromyography gave a mildly diminished activity of the examined muscles; the palpebra sphincter (right and left), first inter-osseus dorsalis muscle (right) and anterior tibialis (right). After infra-orbital nerve stimulation, no R1 responses were received bilaterally. Retarded R2 responses were received, while the R2 response of the left side was significantly lower. Electroneurography indicated absence of an f wave when his right median, ulnar and peroneal nerves were stimulated. Absence of an f wave was also observed when his left tibial nerve was stimulated, indicating a mixed demyelinative polyneuropathy with a conduction blockade in multiple nerves and mild axial damage.\nOur patient received 40 g intravenous Immunoglobulin for a duration of five days based on clinical suspicion. Notably, the CSF albumin became elevated (1 g/dL) only after the second lumbar puncture was performed two weeks after the onset of symptoms. Our patient received an additional 40 g of intravenous Immunoglobulin a few days later, for a duration of three days, in an attempt to ameliorate the final clinical outcome. Relapse can occur in patients who are treated early in the course of GBS and improve, and brief retreatment with the original therapy is usually effective in these cases [,]. On presentation with positive CMV IgM antibody test results, we applied an anti-CMV regime with ganciclovir intravenously 500 mg twice daily. The decision was taken to try to ameliorate our patient's neurological condition, which required him to remain resting in bed as any change in position caused inconvenience and a sense of falling. Intense physiotherapy was applied soon after diagnosis. Our patient markedly improved within two weeks. At that time, while still under anti-CMV treatment, he was able to sit up in bed and on a chair. Later on he was able to stand with support and also progressively take a few steps. After improvement of his clinical condition, our patient was discharged with appropriate follow-up.
A 46-year-old female presented in 2017 with symptomatic right leg pain and aesthetic complaints relating to the right calf. Medical history showed a previous head trauma (car accident) with brain hematoma drainage and a saphenous vein operation. Physical examination revealed edema in the perimalleolar area and painful varicose veins, in the right calf (with considerable aesthetic impact) and left calf (with minor aesthetic impact). Venous scores at the first visit to our clinic were the following: Venous clinical severity score VCSS 10 and Aberdeen quality of life questionnaire 27.7.\nDuplex examination conducted before the original venous operation (which had been performed in a different clinic in January 2016) had shown reflux in the left great saphenous vein and significant reflux in the right small saphenous vein. However, the operation actually performed was stripping of the right great saphenous vein. Both the left great saphenous vein and the right small saphenous veins were left in place untreated. After this procedure, symptoms had exacerbated progressively, and the aesthetics of the leg had deteriorated progressively.\nPreoperative evaluation was normal. We performed a complete duplex scan, according to our routine, as published elsewhere. The patient had type 1b+2a shunt in the right leg and 4+2d shunt in the left leg. We suggested operating to treat the small saphenous vein in the right leg and the great saphenous vein in the left leg. We treated the patient using the CHIVA technique to preserve the remaining saphenous veins.\nWe performed the CHIVA procedure on both legs during the same operation. Local anesthesia was provided with a solution containing 10 mg/mL 20 mL of ropivacaine and 2% lidocaine, using 20 mL and 60 mL of saline. We routinely have an anesthetist in the operating room to guarantee patient safety and comfort, who is always advised to avoid sedation as much as possible. When necessary, an opioid-free sedation technique is employed. In the right leg, we ligated the small saphenous vein at its junction with a calf vein and ligated two N3 collaterals, leaving the small saphenous vein draining through two perforators. In the left leg, we ligated a collateral draining to the great saphenous vein from the inguinal ligament and an N3 draining reflux from the great saphenous vein to the calf. A total of 5 small incisions were made. The patient was discharged two hours after the operation wearing compressive stockings and taking 40 mg enoxaparin per day for 3 days, according to our postoperative routine.\nOn the sixth postoperative day, duplex scanning was performed, showing minor continuous reflux in the small saphenous vein of the right leg and even less reflux in the great saphenous vein on the left. The right small saphenous vein had been 7.4 mm before the operation and was 3.8 mm after. The left great saphenous vein had been 4 mm before the operation and had not decreased in size during the initial postoperative period. The patient scored pain at 3 on a 0-10 pain scale and had taken one 750 mg paracetamol tablet during the entire postoperative period. We made a full photographic record before and after the operation ( \n ). There were no photographs or records of symptoms available from the original operation.\nIn relation to the wrong-site surgery, we comforted the patient and reported the case to both the previous surgeon and the patient safety surveillance team at the hospital where the operation had been performed.
Patient is a 58-year-old gentleman who presented to the VA neurosurgery clinic with complaints of neck pain and left arm pain. His symptoms started 4 months before his clinic visit. He had left-sided neck pain and severe pain down his entire left arm into all of his fingers. He described this pain as constant with slight improvement with hot showers and taking gabapentin 1200 mg 3 times daily. He also had pain and numbness along his left thorax and left ear and face numbness. He had noted some left arm weakness. He denied any recent trauma (prior MVC 15 years ago), falls, changes in balance, problems with fine motor skills, or any bowel or bladder incontinence.\nPrior medical history includes gastroesophageal reflux disease with Barrett’s esophagus, generalized anxiety disorder, recurrent major depressive episodes, hypertension, restless legs syndrome, cocaine abuse, hepatitis C, diabetes mellitus type 2, and diabetic peripheral neuropathy. He had no prior spine surgeries.\nLaboratories were unremarkable. On examination, he was full strength in his right arm and both legs and was three out of five strength in his left deltoid and four out of five strength in the rest of his left arm. Sensation was intact to light touch throughout, except decreased in the left arm, left hemithorax, and left jaw. He had diminished DTRs in his left arm. He had a negative Hoffman’s sign, no clonus and a negative Romberg’s sign. He had significant left arm muscle atrophy.\nHe underwent a MRI of his neuroaxis including MRI head without contrast that showed no acute pathology, incidental pineal gland cyst, and mild inflammatory changes along left mastoid. His MRI of the cervical spine with and without contrast [ and ] showed cord signal change and syrinx on the left spinal cord from C2 to C7 with syringomyelia from C7 extending into thoracic spine as well as multilevel degenerative disc disease and severe C5-C6 stenosis with cord compression. A MRI of the thoracic spine with and without contrast [-] showed syrinx extending to T7-T8 measuring 9.4 × 7.1 mm with no enhancement, severe compression of spinal cord at T7-T8 with anterior displacement of spinal cord. A MRI of the lumbar spine with and without contrast showed degenerative disc disease, severe L4-L5 stenosis with thecal sac compression, moderate L1-L4 stenosis, and facet hypertrophy. He underwent a diagnostic spinal angiogram which did not show any vascular abnormality or fistula and a cerebral angiogram which did not show any cervical feeders.\nThe patient underwent T7-T9 laminectomies with resection of arachnoid web, midline myelotomy with drainage of intramedullary syrinx, and use of intraoperative neuromonitoring []. Wide laminectomies were completed bilaterally at T7, T8, and the superior aspect of T9, with extra caution used to keep the facet intact. There was a moderate amount of fibrosis outside of the dura at the level of T7-T8 that had to be carefully reflected away to visualize the dura. Dura was opened in the midline and tacked up using 4–0 Nurolon. There was a large arachnoid web band at T8 with significant tethering and compression of the spinal cord with venous congestion noted superiorly. Microscissors and a micronerve hook were used to carefully dissect the arachnoid band off the spinal cord. The web encompassed nerve roots laterally and careful attention were taken to dissect the band away from the nerves. This was explored laterally on both sides to ensure that there was no further tethering. The spinal cord was obviously expanded due to the syrinx above the level of the web and a microblade was used to make a small midline myelotomy and the syrinx cavity was visible. A small angiocath was temporarily placed through the myelotomy to allow for further drainage of the syrinx. At the end of the resection, the spinal cord appeared very nicely decompressed and the dura was closed with 4–0 Nurolon in a watertight fashion. A Valsalva maneuver was performed and there was no evidence of a CSF leak. Tisseel was applied and a 7 flat JP drain was left in the epidural space and the incision closed in the standard fashion. Pathology revealed fibrosis and arachnoid web.\nThe patient was seen in clinic 2 weeks after surgery with significant improvement of his symptoms, especially his numbness and tingling. He continued to have persistent left arm pain, but only in a C6 distribution after surgery. His incision was well healed, staples were removed and the patient was full strength on examination. He had a new MRI of the cervical and thoracic spine with and without contrast 1 month postoperative that showed near-complete resolution of syrinx and no further spinal cord compression at the level of the arachnoid web, but remaining severe stenosis at C5-C6 [-]. He underwent C5-C6 anterior cervical discectomy and fusion []. He did well postoperative with improvement of pain, but continued to report some residual left arm numbness and pain.
A 37-year-old male presented with masticatory disturbance and aesthetic complaints. His facial profile was straight, and the frontal view was almost symmetrical, with long lower facial height. When the patient smiled, upper incisors could not be seen below the upper lip. He exhibited no phenotypes and medical and family histories about osteogenesis imperfecta and bone diseases.\nMolar relationships were Angle Class I on both sides. All erupted teeth showed severe AI without loss of congenital teeth (Fig. ). Anterior open bite of − 10.0 mm was observed between the edges of the upper and lower central incisors. The upper dental midline shifted 2.0 mm to the right relative to the facial midline, and the lower dental midline shifted 0.5 mm to the left relative to the facial midline.\nClinical and radiographic examinations revealed a stump lesion on the patient’s lower right second molar and caries lesions on his upper right first premolar, upper and lower left first, second and third molars.\nCephalometric analysis revealed a skeletal Class I jaw-base relationship (Table ). The mandibular plane and gonial angles were larger than historical values for Japanese control subjects [], indicating a high mandibular plane angle. The maxillary incisors showed an average degree of inclination, but the mandibular incisors were inclined lingually.\nThe patient was diagnosed as a skeletal open bite with severe AI of all teeth erupted, a skeletal Class I jaw-base relationship, and high mandibular plane angle. The treatment objectives were: (1) to correct the anterior open bite and establish ideal overjet and overbite; (2) to achieve acceptable occlusion with good functional Class I occlusion; and (3) to recover the shape of the collapsed teeth with AI by prosthodontic treatment in order to prevent further wear and sensitivity. The treatment was planned as follows:3.5 mm impaction of the posterior maxillary segments (bilateral second premolar, first and second molars) by compression osteogenesis Minimal extrusion of the anterior teeth to correct severe open bite Establishment of an ideal occlusal relationship through prosthetic restoration.\nAfter the upper and lower left third molars and the lower second molar were extracted, 0.018″-slot pre-adjusted edgewise appliances were placed on both arches. After leveling, corticotomy was performed under local anesthesia with intravenous sedation. Surgery was performed on an outpatient basis (in two stages to avoid bone necrosis) []. In the first stage, corticotomy was initiated at the palatal surface of the first and second upper premolars with a mucoperiosteum incision on the alveolar ridge, 3 mm above the tooth root apices (Fig. ). A fissure half the width of the desired amount was made with a round bar of 4 mm in diameter through the cortical plate of bone surrounding the teeth. The anchor plate was placed onto the center of the hard palate. The second corticotomy at the buccal site was performed 3 weeks after the first corticotomy (Fig. ). The mucoperiosteal flap was abraded beforehand to visualize the area of the corticotomy and to ensure that the procedure was carried out in accordance with previously corticotomized regions. The anchor plates were bilaterally fixed to the zygomatic buttress. Then, elastomeric chains were added to move the corticotomized bone/teeth segments 3.5 mm superiorly. After 1 month, posterior maxillary segments were moved superiorly, which ultimately resulted in correction of the skeletal open bite.\nAfter 8 months of postoperative orthodontic treatment, overbite was improved by − 2.0 mm, and molar relationships were maintained as a Class I relationship (Fig. ). Prosthodontic treatment was initiated to protect dentin and establish stable occlusion. After 5 months of prosthodontic preparation, all edgewise appliances were removed, and provisional crowns were set on all teeth with AI. The total active treatment period was 16 months.\nOn facial photographs obtained after treatment, anterior lower facial height was reduced, resulting in a balanced facial profile (Fig. ). The anterior open bite was corrected, and the occlusion was much more stable and acceptable, with Class I canine and molar relationships. The overjet and overbite were + 2.0 mm and + 1.5 mm, respectively. When the patient smiled, approximately one-third of the maxillary central incisors were properly exposed. Panoramic radiograph showed that root parallelism was achieved (Fig. ).\nPost-treatment cephalometric evaluation showed a skeletal Class I jaw base relationship (ANB 3.6°) (Table , Fig. ). The mandible was rotated 3.0° counter-clockwisely (MP-FH plane 37.3°). The upper first molars were intruded 3.5 mm toward the palatal plane. The maxillary and mandibular central incisors were extruded 3.0 mm and 0.5 mm, respectively. These factors contributed to the maintenance of an acceptable interincisal relationship.\nAfter 8-year retention, the occlusion was stable, and a good facial profile was maintained (Fig. ). Panoramic radiograph revealed no or less changes in alveolar bone level and root parallelism (Fig. ). Cephalometric analysis showed only minor changes in maxillary and mandibular position, which did not result in relapse (Fig. ).
A 41-year-old Asian female patient reported to the Department of Oral and Maxillofacial Surgery with a 4-month history of a gradually increasing swelling on the right side of the face. The swelling was largely painless with no other associated functional symptoms. On local examination, a diffuse swelling was observed measuring about 6 cm × 5 cm on the right side infraorbital region with regional lymphadenitis of the submandibular region of that side. The skin over the swelling appeared normal with no signs of infection or inflammation [Figure 1 ( and )]. The lump was diffuse, firm, and mildly tender. Fixation to the underlying structures was not present. Intraoral findings were unremarkable with no foci of infection. No other distant lymphadenopathy was elicited clinically. All her hematological investigation parameters were within normal limits. Chest X-ray confirmed the absence of any pathology including hilar lymphadenopathy. The clinical differential diagnosis comprised a wide spectrum of conditions such as lymphoma, fibrosarcoma, and neuroma.\nMRI scan of the head and neck showed a homogeneously enhancing infiltrative lesion seen involving the premaxillary space, buccal space, retromolar trigone, and lateral pterygoid in the masticator space on the right side. There was no significant bony destruction. Multiple enhancing solid lymph nodes were seen in the right submandibular, upper deep jugular group and the left submandibular group, largest measuring 18 mm × 12 mm in the right submandibular region. MRI report gave a differential diagnosis of neurofibroma [].\nFNAC was performed initially, but the result was inconclusive. Therefore, we proceeded with examination under anesthesia and biopsy. The incisional biopsy report suggested a nonspecific type of lymphoid hyperplasia. Hence, definitive surgery was planned with complete excision of the lesion. Intraoperatively, it was observed that the mass was well defined, capsulated, and firm. The histopathology showed dense fibrous connective tissue with some areas exhibiting sheets of numerous small well-differentiated lymphocytes interspersed along with reactive lymphoblasts that were infiltrating into the muscle tissue, blood vessels, and nerves, and in few areas, it formed germinal centers. Mixed type of inflammatory cells including plasma cells and macrophages were seen. The final histopathology report confirmed the diagnosis as reactive lymphoid hyperplasia with no evidence of malignancy.\nAdditional immunohistochemistry studies were performed to arrive at a more definitive diagnosis. It showed positivity for CD4, CD5, CD3, and CD20 and positivity for both kappa and lambda. This strongly suggested a pathology of inflammatory origin, ruling out malignancy. The patient was kept under close observation.\nOne year later, the patient reported with a recurrent swelling in relation to the right submandibular region. FNAC was performed again, this time from submandibular region. The cellular smear studies showed sheets of centrocytes, centroblasts, lymphocytes with epithelioid granulomas composed of round-to-oval cells with abundant cytoplasm, and sole-shaped nucleus admixed with necrosis and fibrous tissue suggesting granulomatous lymphadenitis.\nIn view of the earlier histopathological studies being inconclusive, persistent swelling, and FNAC showing granulomatous changes, we opted for a multidisciplinary approach to rule out other granulomatous diseases such as atypical tuberculosis and sarcoidosis. Fresh CT contrast studies were obtained. CT report suggested a diffusely enhancing soft tissue density lesion in the right buccal region involving upper and lower gingivobuccal space and retromandibular space. The region was closely abutting the right side of the mandible and obliterating the fat plane in the masticator space [Figure 3 ( and )]. Multiple enlarged lymph nodes were noted in the submandibular region and the upper deep cervical regions, the largest size being 2.1 cm in the submandibular region.\nLymph node biopsy was performed under general anesthesia, through a right submandibular approach. Intraoperatively, two huge solid lymph nodes, well encapsulated and firm in consistency, were removed []. The samples were sent for both histopathological studies and for microbiological tests. The culture test was negative for mycobacterium. The histopathology report this time suggested lymph nodes with multiple predominant follicles with prominent germinal centers with extensive fibrosis and inflammatory cells including multinucleated giant cells. Amidst these proliferating capillaries, prominent endothelial cells were evident. Based on the histopathology report, the patient was evaluated for serum immunoglobulin E (IgE) levels, peripheral eosinophils, and absolute eosinophilic count to rule out Kimura's disease. The IgE levels in this patient were considerably increased with 417 IU (reference value: <180 IU), absolute eosinophil count was marginally elevated with 445/cumm (reference value: <350/cumm), and differential count of eosinophils was 5%. The diagnosis of Kimura's disease was confirmed thereafter.\nBased on the available scientific evidence, the immunologist suggested that the patient is started on systemic steroid therapy with a loading dose of 60 mg of prednisolone in divided doses for 3 months. There was good response to steroid therapy, and the lump started to regress within 3 months which was confirmed by CT scan. Steroid dose was tapered to 2.5 mg OD over a period of 6 months. Complete remission of the lesion is observed for the past 18 months, and the patient is on a maintenance dose of 2.5 mg of oral prednisolone.
A 4-year-old male proband (Figs. , , ) presented with global developmental delay noticed by his family when he was around 11 months of age. The child was born at term from a vaginal delivery and an uneventful pregnancy. His parents were non-consanguineous and healthy. The mother disclosed she had consumed alcohol for social purposes before being aware she was pregnant. She reports having drunk small beverages of beer during the week, as well as vodka throughout the weekends, until the third month of gestational period. The exact daily amount of alcohol consumption is unknown. Despite the fact the mother had a small amount of vaginal bleeding at 42 weeks of gestation, the child’s Apgar scores were 8 in the first and fifth minutes. The patient developed a mild, asymptomatic, hypoglycemia due to suction difficulties, but he was discharged from the hospital at day three of life and had been able to be breastfed until 3 months of age only.\nThe child’s social and language skills were below the rest of his developmental domains. He attained independent walking by 23 months of age. Nevertheless, his verbal and non-verbal communication capabilities were so weak that, at that age, it was clearly observed that the quality of his eye contact, as well as social interactions, were in the autistic spectrum range. Moreover, significant motor and vocal stereotypic behaviors alongside difficulties in functional play and imitation had also ensued. The child’s growth curves for weight (centile 15–25), height (centile 15–25) and head circumference (centile 75) have been steadily unchanged over the course of his growth. No facial or corporeal dysmorphic features have been detected, that could be specific for FAS, or any specific genetic syndrome. In addition, there were no reports of clinical seizures in this patient. His physical and neurological examinations were unremarkable, except by the fact that he has developed a refraction error visual impairment. He was seen by an ophthalmologist who prescribed lenses accordingly.\nAt 3 years old, a brain MRI, which was undertaken to investigate the patient’s global developmental delays, showed no signs of abnormal patterns in myelination or in the setup of the structures comprising the supra and infratentorial brain compartments. However, we identified sparse increased signal in FLAIR and T2-weighted images in the white matter territories adjacent to the lateral ventricles bodies and subcortical zones (Figs. , , , ). In addition, around the same period of time, a v-EEG demonstrated signs of a non-specific slow background, but no other abnormal electrographic activity had been identified (Fig. ).\nFragile X DNA screening, as well as metabolic screening for Inborn Errors of Metabolism results, all came back negative. Chromosome analysis was carried out (please, see below).\nIn order to reinforce our thoughts on the diagnosis of ASD related to the genotype found in this patient, we revisited the ASD diagnosis at the patient’s age of 4 years, according to the Childhood Autism Rating Scale (CARS) []. The overall CARS score was 44, suggesting that the child was in the severe range of the autistic spectrum. This finding confirmed our primary developmental diagnosis of ASD, which has finally been aligned to the novel DSM5 criteria.\nA high resolution G-banded chromosome analysis of peripheral blood lymphocytes showed 46,XX,del(12p)(13.2) karyotype (550–600 GTW bands) in this patient.\nThe 12p13.2 deletion has not been identified in the parent’s karyotype, indicating a de novo terminal deletion in the short arm of chromosome 12.\nWe also used OMIM database to scrutinize scientific data related to all genes seated on the 12p13.2 region. Then, we selected the genes that could potentially be related to one of the etiologies of ASD.\nAside from the present patient, we found an additional number of 43 cases described in the medical literature since 1974, that have been related to the 12p region. However, to the best of our knowledge, none of the previous had been specifically linked to the 12p13.2 band. All cases were summarized in Table .
A 31-year-old gravida 4 para 0 African American woman at 22-weeks gestation presented with vaginal bleeding to an outside hospital. Her obstetric history was significant for 2 therapeutic abortions and 1 spontaneous abortion. During prenatal care, the fetus was noted to have a unilateral dysplastic kidney. The patient’s family history was significant for two family members with cervical cancer and two family members with endometrial cancer. She was placed on bedrest with inpatient admission. 48 h after admission she developed pelvic pain and uterine contractions. She expelled a mass vaginally measuring 11 × 9 × 5 cm with no fetal contents. The pathology from the outside facility showed a showed a highly cellular tumor composed of spindle-shaped cells and bizarre multinucleated giant cells with focal myxomatous change with mitotic count is greater than 50 per 10 high power field (Positive for CD10, SMA, ER, PR, EMA) with the differential diagnosis including endometrial stromal sarcoma and undifferentiated uterine sarcoma. The patient underwent examination under anesthesia, demonstrating a 3 × 3 cm defect of the posterior vaginal wall with active bleeding, which was sutured for hemostasis. The cervix was long, closed, and high with no evidence of bleeding and the fetus was intact with normal heart tones. She was discharged in stable condition.\nAt 27-weeks, the patient was referred to our institution for maternal-fetal medicine, and gynecology oncology consultation. On initial evaluation, she reported no prior abnormal gynecology history and no history of infertility issues. She denied dyspareunia and bulk symptoms prior to or during pregnancy, and her periods before pregnancy were normal. A pap smear at 27-weeks gestation was normal and negative for human papilloma virus. Her intake physical exam at our institution revealed a normal pelvic exam with no evidence of residual vaginal mass, and an ultrasound revealed no intrauterine myomas.\nGiven the differential diagnosis included an endometrial stromal sarcoma, an undifferentiated uterine sarcoma, and a primary vaginal sarcoma, the patient underwent magnetic resonance imaging (MRI) without contrast of the chest, abdomen, and pelvis at 27 weeks. MRI findings were notable for a peri-centimeter cyst in the cervix consistent with a Nabothian cyst, a multiloculated cystic structure in the fetal abdomen consistent with dysplastic kidney, and an anterior placenta. There was no evidence of metastatic disease.\nDue to the inability to ascertain whether the cancer had originated in the uterus, cervix or vagina, the patient desired definitive management for possible uterine sarcoma. In consultation with maternal fetal medicine and gynecology oncology specialists, the decision was made to proceed with a cesarean hysterectomy at 36 weeks. Betamethasone 12 mg intramuscularly was given for two doses 24 h apart with the first dose at 35 weeks and 6 days gestational age.\nAt 36 weeks and 1 day, she underwent planned exam under anesthesia of the vaginal canal with cesarean hysterectomy, bilateral salpingectomy, and peritoneal biopsies. She delivered a male infant weighing 2520 g with Apgars of 9 and 9. Operative findings were notable for normal appearing pelvic organs. There were no palpable or visibly enlarged pelvic/periaortic lymph nodes or evidence of metastatic disease throughout the peritoneal cavity. The neonate was admitted to the NICU post-delivery for prematurity. Neonatal US confirmed a right multi-cystic dysplastic kidney. He was transferred out of NICU on day 2 of life, and discharged home with his mother.\nThe final surgical pathology report was benign: no malignant or neoplastic cells were seen. The patient was seen at her 6-week postpartum visit and was doing well with a normal pelvic exam. At a follow-up surveillance visit 3 months postpartum, she was noted to have a 1 cm posterior vaginal wall lesion; biopsy results showed a high-grade sarcoma. The tumor was estrogen receptor and progesterone receptor positive. Computer tomography (CT) with contrast of the chest, abdomen, and pelvis showed no evidence of metastatic disease. The patient underwent an upper vaginectomy and proctoscopy. Operative findings were notable for 1.5 cm polypoid lesion in the posterior vagina wall 5 cm distal to the posterior vaginal apex. A 1 cm surgical margin was obtained circumferentially around this lesion where feasible, and surgical pathology showed high-grade sarcoma () of the polyp lesion with negative surgical margins. The tissue removed from this excision showed residual sarcoma, morphologically similar to the original pathology from the outside hospital pathology. All other vaginal biopsies were also negative for malignancy. Marker seeds were placed in the proximal and distal margins of the areas where the sarcoma was excised. She was treated adjuvant high dose rate vaginal brachytherapy using a multi-channel vaginal cylinder. She received a biologically equivalent 2 Gy dose (EQD2) of 45 Gy to the whole length of the vagina and 60 Gy to the post-operative bed (). A CT scan 36 months after surgery continue to show no evidence of disease. She remains disease free 58 months after completion of vaginal brachytherapy.
Mr. A is a 33-year-old married male, a pharmaceutical sales executive, presented with a history of consuming 200–300 ml/day of cough syrup containing codeine (10 mg/100 ml) for the past 28 months. Our patient reports having been married for the past 2 years and reports having ejaculation occurring with 5 min of penetration and difficulty controlling it during intercourse with his wife. He reports of not having problems with erection or pain during ejaculation and says he did not have this issue before marriage while masturbating. Three months after marriage, Mr. A suffered from acute bronchitis for which he was prescribed a codeine-containing cough syrup. He noticed his ejaculatory response after consumption of the syrup and started consuming 100 ml of the cough syrup (one bottle) each day after purchasing it OTC from different medical shops. Over the past 25 months, Mr. A found he had to increase the amount of cough syrup consumed to improve his time to ejaculation and consumed up to 200–300 ml/day (2-3 bottles). He would also have a craving for the syrup and would have episodes of headaches, nausea, and sweating upon abrupt cessation and subsided upon consumption of the syrup. Mr. A and his spouse did not report on any mood changes, increased sedation, or any other problems at work or family due to his cough syrup consumption. He decided to seek treatment for his problem after attempts by himself to abstain from consumption of the cough syrup were unfruitful during the past 3 months.\nThere was neither any history or family history of mental illness nor any conflicts with wife, other family members, and at workplace. There is no history of any medical illness, use of other medications, or other substance use disorders.\nOn mental status examination, he was adequately groomed with normal psychomotor activity. He was conscious with euthymic mood, and there were no ideas of guilt, depressive ideas/death wishes, formal thought disorders, psychotic symptoms, or cognitive impairment. As per the 10th revision of International Statistical Classification of Diseases and Health Related Problems, a diagnosis of mental and behavioral disorders due to use of opioids, dependence syndrome, continuous use, and premature ejaculation was made.\nHis physical examination and vital signs revealed no abnormalities, including normal pupil size and respiratory rate. He was admitted and laboratory investigations on the day of admission, including, hemoglobin, total and differential count, serum sodium and potassium, erythrocyte sedimentation rate, routine urine examination, renal and liver function tests, and random blood sugar were within the normal limits. No physical abnormalities of the genitourinary system were found upon consultation by a urologist.\nMr. A was started on oral clonidine of 0.1 mg three times a day and oral lorazepam 2 mg at night. The next day the patient reported having sweating, rigor, and mild muscle cramps of his legs following which he was given intravenous lorazepam and clonidine dosage was increased to 0.2 mg, three times a day. His symptoms gradually subsided and he did not report any withdrawal symptoms the next day. Oral naltrexone of 50 mg/day was started on the 4th day. He was discharged on the 14th day of admission after gradual tapering and stopping of oral clonidine and lorazepam tablets and motivational enhancement therapy sessions during his hospital stay. Liver function tests were normal on discharge. He was maintained on oral naltrexone of 50 mg/day over the next 3 months along with monthly monitoring of liver function tests. Sensate focus exercises along with stop and squeeze technique for practice were taught to Mr. A and his spouse following which Mr. A's premature ejaculation improved. After being abstinent from opioids for 6 months (confirmed by urine opiate screening test), oral naltrexone was stopped.
A 79-year-old male was referred to the surgical outpatient department with a 2-month history of altered bowel habit. Abdominal examination and digital rectal examination in the outpatient clinic were unremarkable.\nThe laboratory studies, full blood count and biochemistry were normal. A colonoscopy study identified four abnormal lesions, two of which were colonic subcentimetre polyps. More distally in the mid-rectum, at 15 cm from the anal verge, there was a sizeable, villous polypoidal lesion, which was proved on biopsy to be a tubulovillous adenoma (TVA) showing low-grade dysplasia. More proximally in the distal sigmoid colon at 25 cm there was a segment of a malignant-looking lesion associated with a stricture (). However, biopsy samples from this region showed hyperplastic colonic mucosa showing lamina propria fibrosis and mild chronic active inflammation with separate pieces of granulation tissue and ulcer slough but no evidence of malignancy.\nAs per local protocol for malignant-appearing lesions on endoscopy, a staging CT scan was arranged on the basis of the colonoscopy appearances to facilitate the cancer pathway. This demonstrated, at the site of the presumed malignancy in the distal sigmoid colon, a 7 cm segment of circumferential thickening on a background of diverticulosis (). This was associated with a few subcentimetre locoregional and inferior mesenteric lymph nodes. There was no suggestion of malignancy elsewhere; so the proposed CT staging was T2/3 N1 M0.\nAlthough the 5 cm rectal TVA could explain the patient’s symptoms it was felt at the multidisciplinary team discussion that either lesion could be responsible for the patient’s symptoms. Despite the lack of malignant cells from the biopsy samples the surgical team were still concerned about a potential sigmoid colon malignancy from the endoscopy and CT appearances. It was felt that the colonoscopy sampling was not truly representative of the malignant appearing sigmoid colon lesion seen at colonoscopy and CT. An anterior resection was planned for the patient to remove both the TVA and presumed sigmoid colon malignancy. An MRI scan of the rectum was performed in order to preoperatively assess the rectal polyp further. The sigmoid tumour was incidentally included in the MRI scan field owing to proximity to the rectal lesion (); however, this did not add any diagnostic value preoperatively.\nThe patient underwent an uncomplicated laparoscopic-assisted anterior resection with defunctioning loop colostomy. Post-surgical histology confirmed that the rectal polyp was a low-grade TVA. However, histological assessment of the presumed distal sigmoid cancer at 25 cm demonstrated mucosal ulceration with underlying fibrosis and diverticulosis. The ulceration was associated with a 5 cm jagged edged intraluminal dental plate containing a single front upper incisor prosthetic tooth found impacted at this site, with no metallic components (). The dental prosthesis was so impacted that endoscopic retrieval would be unlikely to have been successful. No malignancy was demonstrated in the post-surgical specimen. There were a few enlarged local lymph nodes present in the specimen, which were all benign. The patient had a good post-surgical recovery and went on to have an uncomplicated reversal of defunctioning colostomy several months later.\nRetrospective review of the patient’s previous imaging revealed that the patient had attended the accident and emergency department 2 years earlier, having accidentally swallowed “false teeth” during a meal. An abdominal plain radiograph had been taken at the time, which did not demonstrate any radiopaque foreign body. The CT scout view also demonstrates no radiopaque foreign body (). The patient denied any symptoms and had been discharged with no further follow-up. Interestingly, the patient claims he has always known that he had not passed the swallowed dental prosthesis.
A 62 year old gentleman came to the surgical out patient with complaints of abdominal discomfort, occasional left sided abdominal pain for the past 15 days. He had decreased appetite, projectile vomiting, no dyspepsia, no history of fever and bowel habits were normal. On examination, a large mass of 10 × 8 cm was palpated in the left hypochondrium, left lumbar, and umblical region. It was mobile, not moving with respiration and firm in consistency. On palpation of neck, the thyroid gland was found to be enlarged with palpable right lobe. Upper and lower gastrointestinal endoscopy was normal.\nContrast enhanced computed tomography (cect) abdomen showed large lobulated, heterogenously enhancing mass with internal necrosis and calcifications in the left hypochondrium in the region of distal body and tail of pancreas (). There were no other foci of metastasis in abdomen or chest. Chest X-ray of patient was normal. Ultrasound of neck revealed a suspicious nodule in right lobe of thyroid measuring 1*1 cm with no nodal enlargement. Pre-operative ultrasound guided biopsy showed features suggested of poorly differentiated malignancy (that was negative for gastro intestinal stromal tumor markers). His CEA and Ca 19-9 were normal. Fine needle aspiration cytology of thyroid nodule was done under image guidance which was suggestive of papillary carcinoma thyroid.\nAs image guided biopsy of abdominal tumour could not be done patient was planned for laparotomy. At laparotomy, patient was found to have a bilobed tumor arising from the lesser sac adherent to the pancreas and abutting the stomach, transverse colon, and left adrenal and splenic hilum (). The tumor was resected en bloc (distal pancreatectomy and splenectomy). The post operative period for the patient was uneventful. Oral feed started on 3rdpost operative day drain tube was removed on 5thpost operative day. Patient was discharged on the 12thpost operative day.\nThe histopathology showed the presence of very irregularly shaped cells with nuclei that exhibited a “wavy, buckled appearance”. Multiple sections studied showed pancreatic tissue with adjacent fairly circumscribed neoplasm composed of spindle cells arranged in sheets and fascicles. Pancreatic margin appeared free from tumour. There are areas showing alveolar and glandular patterns.There are areas showing hyalinization, necrosis and fibrosis. Tissue organization shows great variability, with hypocellular myxoid areas and areas of major cellularity diagnostic of Malignant peripheral nerve sheath tumour (MPNST). The histologic changes of MPNST include spindle cells with comma-shaped nuclei, tactoid bodies, nuclear palisading, hyaline bands, and schwannoma-like and curlicue foci ().The tumour showed focal S100 positivity,was strongly positive for ki67, was positive for vimentin ( and 5) and negative for cytokeratin,synaptophysin and all GIST markers(CD 117,DOG 1,PDGF) We re-examined the patient thoroughly and there were no cutaneous markers for neurofibromatosis and examination of the eye was normal. Patient was reviewed after a period of 2 months. Positron emission tomography () was done which revealed para aortic nodes and 2 mesentric nodes. It also revealed a metabolically active nodule in thyroid with cervical nodal metastasis. Patient was taken up for total thyroidectomy with functional neck dissection. Histopathology confirmed papillary carcinoma of thyroid with positive lymph nodes. Patient was advised to undergo a radioactive iodine scan which showed 0.3% uptake. Hence patient was started on chemotherapy for para aortic nodes with a regimen of paclitaxel, adriamycin, ifosumide and mesma. Patient has completed 3 cycles till date and is on regular follow up.
A 56-year-old man, with a history of B-related chronic hepatitis under antiretroviral treatment, was referred to our surgical department by the Infectious Diseases Unit, where he was under follow-up. The patient did not complain of any symptom concerning the gastrointestinal tract. During an abdominal ultrasonography, a hypoechoic round-shaped mass 4.7 cm in size, with regular margins, located between the left lobe of the liver and the anterior surface of the pancreatic body, was found. Magnetic resonance imaging confirmed the presence of a cystic mass with complex content, located anteriorly to the gastroesophageal junction.\nThe patient was then submitted to EUS, in order to better define the structure of the lesion and its relationship with the adjacent organs. EUS showed a hypoechoic mass with slightly heterogeneous internal echoes and regular margins, located just below the gastroesophageal junction (Figure ). The lesion measured about 4.5 cm and seemed to be contiguous to the fourth layer of the gastric wall (muscolaris propria). On the basis of the morphologic evaluation, a diagnosis of GIST was suggested. As is usual in a case of suspected GIST involving the upper part of the gastric wall, we tried to obtain diagnostic confirmation through EUS-guided fine-needle aspiration cytology (FNAC). The puncture was practiced using a 22 G needle, which, unexpectedly, penetrated very easily into the mass as it was cystic. We were able to aspirate only a few milliliters of a clear mucus-like fluid, and then a cytological sampling was made from the cystic wall. The collected material was judged adequate by an onsite cytopathologist. The cytological smear showed cylindrical cells isolated or aggregated in small groups with a palisade organization (Figure ). These cells showed long cilia and brush borders similar to the ciliated cells of the respiratory tract. These basal cells had oval nuclei with finely dispersed chromatin and small nucleoli, if any. The background consisted of proteinaceous material containing debris, crystal formations and engulfed hystiocytes. On the basis of these features, a diagnosis of duplication cyst with respiratory epithelium was made.\nLater on, the patient underwent a surgical intervention carried out by an open laparoscopic approach with a transhumbelical Hasson trocar (without a Verres needle to obtain the pneumoperitoneum) and four additional trocars (two of 10 mm and two of 5 mm). Once the lesion was clearly identified, the overlying serosa was cut by a harmonic scalpel (Harmonic Ace; Ethicon Endo-Surgery, Cincinnati, OH, USA). Through a cautious dissection performed under endoscopic control in order to keep the cyst intact, to prevent perforation of the gastric wall, the mass was totally exposed and then completely resected using a linear endoscopic stapler (Echelon™ 60; Ethicon Endo-Surgery). The surgical procedure was completed by performing a Dor fundoplication. The patient had an uneventful postoperative recovery and was discharged on the seventh postoperative day.\nPathologic examination of the surgical specimen revealed macroscopically a cystic lesion 5 cm × 3 cm × 3 cm in size with a mucoid content. Microscopically the cystic wall consisted of mucosa, subepithelial connective tissue, a layer of smooth muscle and an outer fibrous capsule. Focally the mucosa was lined by gastric foveolar epithelium with cardial glands but most of the cystic wall was lined by a pseudostratified columnar ciliated epithelium (Figure ). These features were consistent with a diagnosis of foregut duplication cyst of the stomach.
A 45-year-old Caucasian woman was admitted to our surgical department for treatment of a mass in her left groin area. From her past medical history, our patient was on treatment with levothyroxine after thyroidectomy for multi-nodular goiter and with amlodipine and valsartan for hypertension. She did not smoke cigarettes and did not report any history of trauma in the area.\nThe mass was discovered incidentally on a lower extremity triplex ultrasonography one month before her admission. Our patient was complaining of aching, soreness and heaviness of her lower extremities for two months and was advised to have her lower extremity venous system evaluated. On her right lower extremity, the triplex ultrasonography revealed insufficiency of the saphenofemoral junction and insufficient valves of the great saphenous vein. On her left lower extremity, the study was difficult to perform because of a mass in the groin area. It was a solid hypoechoic mass of heterogeneous texture, 50×55 mm in size, lying 11 mm under the skin surface and with minimal blood flow. It appeared to be in proximity with the femoral vessels but without compressing them, and there was no local lymph node enlargement.\nOn physical examination, a painless, fixed, solid mass was palpated in her left groin area. Both lower limbs were symmetrical with normal motility.\nOur patient was subsequently submitted for an MRI of the area. It revealed a mass lying in a space defined anteriorly from her pectineus muscle, posteriorly from her abductor muscle, laterally from her obturator muscle and medially from her innominate bone. The mass had a heterogeneous low signal intensity on T1-weighted images and heterogeneous high signal intensity with inner areas of low signal intensity on T2-weighted images. It was lobulate with dimensions 78×59×45 mm and relatively well-defined margins. No enhancement was marked after the intravenous administration of paramagnetic substance (Figures and ). Additional imaging studies (upper and lower abdominal ultrasonography, chest radiography) did not reveal any other pathology.\nOn laboratory examination, a full blood count, basic metabolic panel, liver and kidney function tests, electrolytes and amylase, and coagulation profile were normal. The thyroid function tests showed euthyroidism. Cancer- and tissue-specific markers (α-fetoprotein, carcinoembryonic antigen, CA 15.3, CA 19.9 and CA 125) were also tested. Of these, CA 19.9 was found elevated at 39.51 U/mL (reference range, 0-35 U/mL). To exclude possible laboratory error, the elevated value of CA 19.9 was repeated twice.\nOur patient underwent a surgical excision of the mass. A longitudinal incision over the femoral vessels was performed, and a mass measuring approximately 6 cm was identified. It was firmly attached to the adjacent structures; however, it was dissected without ligating any large blood vessel. The mass was resected en bloc and sent for histopathology study. The incision was closed with interrupted sutures.\nThe post-operative period was uneventful, and our patient was discharged on the third post-operative day.\nThe macroscopical specimen examination revealed an oval-shaped lobulated mass 80× 55×50 mm in size with residual striated muscle. On histopathology, it was found to be an IM.\nAt the follow-up six months later, our patient did not have any complaints, and there was no clinical evidence of recurrence. Ultrasonography of her left groin area revealed insufficiency of the saphenofemoral junction and no mass recurrence. Upper and lower abdominal ultrasonography results were normal. All laboratory test results were normal, and CA 19.9 was reduced to 11.34 U/mL. During colonoscopy, the sigmoid colon was found to be edematous and spastic, and first-grade hemorrhoid disease was present.
A 76-year-old man was referred to our hospital for having lower back pain for 5 months, which was suspected to be L2/3 spondylitis on magnetic resonance imaging (MRI). He had previously undergone cervical laminoplasty for ossification of the posterior longitudinal ligament and diffuse idiopathic skeletal hyperostosis 19 years earlier. He had a history of hypertension and diabetes. Although a history of BCG vaccination was unknown, he had no previous history of tuberculosis infection. He had also undergone transurethral resection of a bladder tumor (TUR-Bt) and had been treated with intravesical mitomycin C (MMC) for the bladder cancer 1.5 years earlier. Four months later, he again underwent TUR-Bt, received intravesical MMC, and started BCG therapy for the recurrence of bladder cancer. After the sixth course of intravesical BCG therapy, he was aware of systemic weakness and loss of appetite, and was unable to walk. He appeared to have septic shock and therefore was treated in the intensive care unit. In spite of systemic analyses, the source of his infection and the causative bacteria could not be identified. He was subsequently diagnosed with hypercytokinemia caused by BCG therapy. He showed clinical improvement without the administration of antituberculosis drugs, and was discharged 9 months before he came to our hospital.\nExcept for a temperature of 37.2 °C, his vital signs were within normal limits. Although there were no motor and sensory disturbances in the legs, he was unable to walk owing to lower back pain. Physical examination demonstrated vertebral tenderness at the L2/3 level. Laboratory analysis demonstrated a normal white blood cell (WBC) count of 6300 /μL, a high erythrocyte sedimentation rate (ESR) of 53 mm/h, and a high C-reactive protein (CRP) level of 2.7 mg/dL. There were no other abnormal laboratory findings regarding anemic changes, kidney function, or liver function. The patient did not receive tuberculin skin testing.\nThere were no notable findings on electrocardiogram or chest X-ray. X-ray of the lumbar spine displayed collapsed endplates of L2/3. Sagittal T1-weighted MRI displayed a decreased signal in the L2/3 disc and the vertebral bodies (Fig. ). Sagittal T2-weighted MRI displayed an increased signal in the L2/3 disc and fluid collection in the anterior part of the vertebral bodies (Fig. ). Axial T2-weighted MRI displayed an increased signal around the posterior area of the vertebral bodies, which extended into the left epidural space and reached the peripheral muscle tissue and the area near the aorta (Fig. ).\nOn the second day of hospitalization, the patient underwent an L2/3 disc biopsy, but the general bacteria culture was negative, and the smear was negative for Ziehl-Neelsen staining. The patient’s blood culture was negative, and the result of T-SPOT.TB® (T-SPOT), which is a type of interferon-gamma release assay (IGRA), were also negative; the number of spots for both 6-kDa early secretory antigenic target (ESAT-6) and 10-kDa culture filtrate antigen (CFP-10) was 5 or less. The causative bacteria remained unidentified and therefore he was started on empirical therapy with intravenous ceftriaxone. On the fifth day of hospitalization, a plain computed tomography scan, which was performed for systemic examination, showed soft tissue development in the adjacent abdominal aorta at the L3 level (Fig. ), which was suspected to be an infectious aortic aneurysm. A 2-stage operation was planned for the spondylitis with adjacent infectious aortic aneurysm, to prevent rupture of the infectious aortic aneurysm, obtain spine stabilization, drain the abscess and make a diagnosis. On the tenth day in hospital, because the risk of rupture was considered to be low, the patient underwent an L2/3 laminectomy followed by posterior fixation using percutaneous pedicle screws at T12, L1, L4, and L5 as the first stage. Specimen cultures of the lumbar vertebrae, yellow ligament, necrotic tissue, etc., were negative for general bacteria, specimen smears were also negative for Ziehl-Neelsen staining, and there were no pathological findings of caseating granuloma or necrosis. On the fiftieth day of hospitalization, because the infection had been controlled but the inflammatory response was sustained, he underwent replacement of the aneurysm with a synthetic graft by vascular surgeons, and lesion curettage and L2/3 anterior interbody fusion by iliac bone transplantation as the second stage. Spinal surgery was performed in the transabdominal approach owing to the risk of rupture. On pathological analyses, the L2/3 intervertebral disc, vertebral bone, and tissue surrounding the vertebral bone and aorta showed caseating granuloma and necrosis with multinucleated giant cells and epithelioid cells upon hematoxylin-eosin staining, and positive bacilli upon Ziehl-Neelsen staining. The tuberculosis-polymerase chain reaction (Tb-PCR) result of the tissue was also positive, using COBAS® TaqMan® MTB Test, which is a real-time PCR system targeting the 16S rRNA gene region of Mycobacterium tuberculosis complex DNA. Owing to the patient’s history of BCG therapy, negative T-SPOT, pathological findings, and positive Tb-PCR, the pathogenic bacteria of the spondylitis was considered to be BCG. We then started multidrug therapy with antituberculosis drugs, including isoniazid (INH), rifampin (RFP), and ethambutol (EB), because BCG is typically resistant to pyrazinamide (PZA). PCR-based genomic deletion analysis was performed using the specimens to distinguish BCG from the other M. tuberculosis complexes. Specifically, multiplex PCR was performed utilizing region of difference 1 (RD1), which is present in the DNA of other M. tuberculosis complexes but is deleted in the DNA of BCG []. Primers ET1, ET2 and ET3 bind and amplify a 190-bp region in BCG, whereas a 160-bp region is amplified in the other M. tuberculosis complexes, as observed by electrophoresis on an agarose gel. A clinical isolate sample from our patient was identified as BCG with a deletion in RD1 (Fig. ). Specimen cultures from the first-stage and second-stage operation were later identified as members of the M. tuberculosis complex using a mycobacteria growth indicator tube. After therapeutic intervention, the patient’s WBC count, ESR level, and CRP level were improved and MRI displayed no signs of active infection in the spine, epidural space, peripheral muscle tissue, or aorta. On the ninetieth day after hospitalization, the patient was discharged from our hospital and transferred to a different hospital for physical rehabilitation.
A 28-year-old male presented to the emergency department after exposure to an industrial blast injury, involving the chest and anterior abdominal wall. He was unconscious with labored breathing and pain. His Glasgow Coma Scale deteriorated to 8, and a definitive airway was placed without any incident. Positive pressure ventilation was instituted. The primary survey showed decreased air entry on the left side of the chest. All peripheral pulses were palpable and symmetrical on initial presentation.\nChest radiograph showed the left side chest opacification indicating hemothorax []. Thoracostomy was performed with an initial gush 450 ml of dark blood. Pericardial focused assessment with sonography for trauma was equivocal, and chest X-ray demonstrated a failure to fully evacuate the hemothorax.\nThe secondary survey showed superficial left hand, thigh, and leg cut wounds. Pieces of shrapnel were embedded in the left arm, anterior chest, and abdominal wall. He was stabilized and had CT of the brain, cervical spine, chest and abdomen. CT of the brain and cervical spine were normal. CT of the chest revealed fractures of the left first and second ribs. A hyperdense foreign body was seen penetrating the anterior chest wall soft tissue guarded by ribs without deep penetration to the heart []. There was evidence of retrosternal soft tissue hematoma and left-sided hemopneumothorax with underlying pulmonary contusion. Mild hemopericardium with air was seen within the pericardial sac but no significant major vessel injury. The left lateral chest wall and anterior wall surgical emphysema was also seen. CT of the abdomen [] showed pellets within the anterior abdominal wall with related surgical emphysema but no evidence of peritoneal penetration. A transthoracic echocardiogram demonstrated mild pneumohemopericardium without tamponade.\nRepeat examination of the lower limb revealed a palpable diminution in the right dorsalis pedis pulsation. Ankle-brachial pressure index was performed which revealed a significant discrepancy between the two lower limbs (right 0.4 vs. left 1.1). Review of the original CT revealed evidence of a foreign body in the right groin with no clear point of entry and a lack of subcutaneous air. With further deterioration in the status lower limb pulsation, he was shifted for CT angiography which revealed a metallic foreign body in proximity to the right common femoral artery (CFA) masking its bifurcation point with streaky artifact, just a few millimeters distally. A filling defect was noted within the superficial femoral with no pseudoaneurysm. Although there was no subsequent drop in his systolic blood pressure, an arterial line tracing revealed evidence of pulsus paradoxus.\nA joint decision was made to proceed with an emergency median sternotomy and right femoral artery exploration by the cardiac, trauma, and vascular surgery services. Intraoperatively, blood in pericardium with clots was evacuated. There was a large laceration about 4 cm in the inferior surface of right ventricle without active bleeding and just opposite, a 4 cm tear in the diaphragm tendon.\nCardiorrhaphy with pledgeted sutures and diaphragm repair was performed. The left pleura was opened, and clots were evacuated. Transesophageal echocardiography was performed intraoperatively, and no ventricular septal defect (VSD) was visualized. Shrapnel, lodged in the anterior chest wall muscles, was removed. Simultaneously, right groin exploration was performed. The proximal, distal, and profunda femoris were individually controlled. The foreign material was felt and removed from the CFA at the bifurcation by means of an arteriotomy []. A 4F Fogarty catheter was passed to 60 cm with good backflow and return of thrombus and debris. Right, lower limb pulses were regained. A primary repair of the artery was performed.\nThe patient was shifted to the Cardiac Intensive Care Unit in stable condition. His hemodynamics was normal without inotropic support. His lower limb pulses remained palpable. On the second postoperative day, upper limb numbness was noted, and a CT arteriogram of the head and neck was performed to rule out further embolic disease in the distribution of the carotid artery; this was negative. The patient did well in the following days and was discharged with no complications. He presented to the clinic for removal of symptomatic-retained foreign bodies from the hand and anterior abdominal wall which was successfully extracted and with further follow up he was recovered totally.
A 25-year-old healthy Caucasian woman presented with a metal ceramic restoration on the left maxillary central incisor, that did not satisfy her esthetic demands anymore. In addition, the patient was feeling a dull pain in the area. The patient did not smoke and was healthy. The radiographic examination showed an oversized cast post and core, which occupied three quarters of the root's length (). The clinical examination revealed that the metal ceramic restoration was fractured at the buccal marginal area, it had a surface texture that did not match that of the adjacent natural teeth, it was rather opaque, and its margins were placed on the underlying cast post and core instead of on sound tooth structure (). Soft tissue inflammation was evident around the crown's marginal area. An 8 mm pocket depth was found at the distal area of the tooth. The examination also demonstrated a discrepancy of the soft tissue contours between the two central incisors, sufficient width of keratinized tissue in the maxillary anterior area and a scalloped thin periodontium [–].\nTreatment options were presented to the patient after assessment of all clinical and radiographic data. The patient wished to restore the failing tooth with an implant-supported restoration.\nPrior to implant surgery, preliminary impressions were obtained by using irreversible hydrocolloid impression material. Diagnostic casts for both the maxillary and mandibular arches were fabricated with type III dental stone and they were mounted in maximal intercuspal position on a semiadjustable articulator. A diagnostic waxing was made and the maxillary cast was duplicated. A surgical stent was then fabricated with clear autopolymerizing polymethyl-methacrylate resin. The tooth's cingulum area was marked and an access channel was opened with a 261E-023 laboratory carbide bur (Brasseler, USA). A provisional restoration was also fabricated by using autopolymerizing acrylic resin (GC Unifast III, GC Tokyo, Japan). Two acrylic resin retainers resting on the palatal surfaces of the adjacent teeth were incorporated into the provisional restoration, in order to assist to the orientation of the provisional crown during the surgery. Finally, the provisional restoration was hollowed out and an access hole was opened palatally.\nOn the day of the surgery, local anesthesia with 4% articaine (1 : 100,000 epinephrine) (Ubistesin Forte, 3M/ESPE) was delivered and a full thickness flap was raised. An atraumatic tooth extraction was then performed with the use of a periotome, taking extra care to preserve the buccal plate (). The osteotomy started with a round bur which was placed into the channel of the surgical stent previously described, and progressed with the 2.3 mm twist drill, the pilot drill, and the 3.25 × 13 mm shaping drill at 500 rpm, with the drill torque test set at 50 Ncm. Attention was given to engage with the drills the palatal wall of the extraction socket. The osteotomy was performed under copious saline irrigation. A 4 × 13 externally hexed Osseotite NT implant (Biomet 3i, Palm Beach Gardens, FL) was placed using 20 rpm at 40 Ncm torque. The platform of the implant was placed 2 mm apically to the cementoenamel junction of the right maxillary central incisor () [–].\nA temporary metal cylinder was fastened with a titanium screw on the implant and marked in order to be reduced to the proper length. It was then removed, fastened to an implant replica, and cut with a silicon separating disc. It was further reduced and shaped with diamond burs. The final length and shape of the temporary cylinder was checked intraorally, with the provisional restoration in place. Furthermore, the clearance between the temporary cylinder and the provisional restoration was verified. Autopolymerizing polymethyl-methacrylate acrylic resin (Jet, Lang Dental, Wheeling, IL) was added intraorally between the provisional restoration and the temporary cylinder, using the bead-brush technique. After the polymerization was completed, the provisional restoration-temporary cylinder complex was removed and fastened on an implant replica. More autopolymerizing polymethyl-methacrylate acrylic resin was added to support the adjacent soft tissues and the restoration was placed in a pressure pot containing warm water. It was then shaped with laboratory carbide burs and polished with abrasive rubber points (Bredent, Senden, Germany) and pumice. The provisional restoration was fastened on the implant with a torque driver (Anthogyr, Sallanches, France) with a 20 Ncm torque. The palatal access hole was closed with gutta-percha and acrylic resin. Occlusion was checked and any centric and eccentric contacts were eliminated. The acrylic was further polished with abrasive points.\nA bone deficiency remained buccally and it was filled with bovine bone mineral (Bio-Oss Collagen, Geistlich) (). The flap was sutured in place. Postoperatively, the patient was prescribed 1 g of amoxicillin/clavulanate potassium (Augmentin; GlaxoSmithKline) per day for 6 days and the nonsteroidal analgesic nimesulide (Mesulid; Boehringer Ingelheim) (100 mg, twice a day for 6 days). A 0.2% chlorhexidine (Chlorhexil; Intermed, Greece) rinse was also prescribed and the patient was instructed to use it three times a day for 14 days. The sutures were removed after 10 days (). Patient was placed on a soft food diet for 6 weeks.\nTwo months after implant placement, the provisional restoration was removed and an impression coping was screwed onto the top of the implant. Light-polymerizing resin material (Liquidam; Discus Dental, Culver City, CA) was injected around the impression coping and was immediately polymerized with a UV polymerizing unit, preventing the soft tissues from collapsing onto the impression coping (). A plastic stock tray and polyether material (Impregum, 3M/ESPE, Seefeld, Germany) were then used for the impression (). A new screw-type provisional restoration was fabricated by using a temporary cylinder and light-polymerizing resin (Gradia; GC Tokyo, Japan) (). This provisional restoration was fabricated in order to support better the soft tissue contours mesially and provide a better esthetic result (). No centric or eccentric contact was given to the crown.\nRegular examinations were performed at 3-week intervals for oral hygiene and occlusion evaluation. After 6 months of uneventful healing, a periapical radiograph was taken, showing no areas of radiolucency around the implant. The provisional restoration was removed and a definitive impression was obtained with the method previously described. A definitive cast was fabricated from type IV dental stone and mounted in maximal intercuspal position with the mandibular cast on a semiadjustable articulator. A UCLA abutment with a gold hexed collar was used for the definitive restoration. This was fastened onto the implant replica and waxed to full contour. A silicone key was made and the wax-up was then cut back, invested and cast with high noble alloy. Porcelain was applied and baked in the traditional manner (). The restoration was tried-in, and proximal contacts and occlusion were adjusted. After patient's approval, the restoration was returned to the laboratory for the glazing procedure.\nAfter the delivery of the definitive restoration (Figures and ), the patient was followed every 4 months for the next year. She was then placed on a 6-month recall basis. Soft tissues around the implant supported restoration appeared stable with no signs of inflammation or recession. The interdental papillae looked normal enhancing the optimal esthetic result obtained by the definitive metal ceramic restoration.
An 80-year-old man presented with a 4-day history of swelling and pain behind the left knee with reduced mobility with very short distance intermittent claudication affecting the left calf and reduced range of knee movement. This patient in the preceding week was admitted under the care of medical specialty where a diagnosis of a spontaneous haematoma behind the thigh was made. Haematoma was thought to be a result of the patient being on oral anticoagulation (warfarin) for atrial fibrillation. The patient did not report history of trauma. He looked frail on the current admission on the vascular ward requiring support for some activities at home and had been on treatment for prostate cancer. He had previously also had coccygectomy for metastasis as well as a recent history of permanent pacemaker insertion for syncopal episodes and bradycardia. He also suffered from hypertension and was on a number of medications including warfarin. He was a lifelong nonsmoker and lived in a retirement home. There was no family history of aneurysms.\nExamination revealed extensive bruising at the back of the left thigh and knee with palpable femoral and pedal pulses. The popliteal pulse was difficult to feel due to a large haematoma. The left foot was viable with fully intact sensory and motor function. There was no clinical evidence of calf compartment syndrome on the left leg. The right foot was warm with all palpable pulses in the leg.\nAn initial ultrasound scan revealed PAA with no evidence of popliteal or femoral vein DVT. A CT angiogram performed on the same day showed generalized arteriomegaly and the ruptured 9.4 cm PAA involving only the above-knee popliteal artery () and relatively disease-free 3-vessel runoff to the ankle and foot. Options to treat this patient were either a surgical exclusion bypass with evacuation of haematoma or an endovascular treatment. For a number of reasons including patient frailty, associated comorbidities, and potential feasibility of successfully treating the PAA with a stent graft due to intact 3-vessel crural runoff, decision was made to treat the aneurysm by an endovascular approach.\nUnder general anaesthesia, the left common femoral artery was explored with a small open incision. An initial angiogram through a 5F sheath confirmed the CT findings of ruptured PAA with 3-vessel runoff (). The aneurysm sac was traversed using a curved catheter and a soft hydrophilic wire which was then exchanged to a stiff wire. A 5F sheath was exchanged to an 11F sheath to allow for insertion of 2 Viabahn (Gore®) covered stent grafts of 8-250 mm and 10-100 mm dimensions with a 4 cm overlap which were postdilated with 8 and 10 mm balloons. Postcompletion angiography revealed complete exclusion of the aneurysm sac with improved filling of the runoff vessels (). The procedure lasted for approximately 55 minutes.\nThe patient made a good recovery following the repair with a predischarge duplex scan demonstrating a successful exclusion of the aneurysm with good 3-vessel runoff. The patient was discharged on dual antiplatelet therapy which was continued for 6 weeks after which the patient continued on one antiplatelet and an anticoagulant treatment for AF. Subsequent 3-month surveillance duplex scans for up to a year showed a patent stent with intact distal circulation (). Duplex surveillance was discontinued after 1 year as per local protocol; a clinical follow-up after 18 and 24 months confirmed a patent stent graft with palpable pedal pulses.
A 69-year-old Japanese woman was referred to our department in August 2015, complaining of swelling in the right preauricular region. She had shown a mass lesion of the right lung on a chest x-ray and was referred to the university hospital. Fluorodeoxyglucose-positron emission tomography (FDG-PET) examination showed accumulation indicative of a pulmonary lesion and a temporomandibular joint lesion. The temporomandibular joint lesion had been enlarging but was otherwise asymptomatic. Her medical history included treatment for pulmonary tuberculosis at the age of 13 and surgery to remove breast cancer at the age of 53.\nOn examination, a tender mass in the right preauricular region was palpable. The chin of the mandible was deviated to the right side during mouth opening (mandibular maximum mouth opening, 41 mm). Facial nerve function and mandibular nerve were intact. There was no indication of cervical lymphadenopathy.\nA panoramic radiographic examination showed resorption of the right mandibular condyle to the ramus (Fig. ). Computed tomography (CT) showed destruction of the right mandibular condyle and a large mass lesion with enhanced margin in the masticator space; a cystic lesion was present inside the tumor mass. Three-dimensional CT was useful to understand the bone resorption findings of the mandibular condyle. There was no finding of metastatic cervical lymph node (Fig. ). T1-weighted magnetic resonance imaging showed an enhanced mass lesion in the right masticator space. Because some portions of the mass lesion showed high intensity in T2-weighted images, cystic lesions were suspected to exhibit changes of blood flow or retention of high-protein liquid. Tumor development was not observed in the articular disk of the temporomandibular joint (Fig. ). FDG-PET revealed abnormal FDG uptake in the right submandibular condyle and masticator space.\nA malignant tumor was suspected after analysis by various modalities. Therefore, we performed an incisional biopsy via preauricular incision. Histopathologically, the tumor was largely composed of proliferative, atypical, spindle-shaped cells. Some tumor cells showed increasing mitotic change and extreme atypia (Fig. ). The histopathological findings of biopsy suggested spindle-cell sarcoma. Tumor resection was performed with the patient under general anesthesia via a combined preauricular and transmandibular approach to the masticator space and infratemporal fossa.\nAfter preauricular temporomandibular incision, the superficial temporal fascia and temporal fascia were elevated. The facial nerve (temporal and zygomatic branches) was protected by the fascia (Fig. a).\nThe facial nerve trunk was identified, and its branch was traced according to the conventional method.\nA midline lip-splitting incision was connected to the submandibular incision and accessed the anterior mandibular ramus. The lip-split incision traversed to the periosteum of the mandible; the periosteum dissector was used to elevate the soft tissues of the mandible, and the lateral mandible was exposed. The branch of the facial nerve was traced continuously with the skin flap, and the masseter muscle and deep parotid gland were dissected on the tumor side (Fig. c). The mandible was resected to within ≥ 20 mm from the primary tumor on the anterior of the mandibular ramus. The masseter muscle was divided at the lower edge of the zygomatic arch; further, the temporal muscle was divided horizontally at the height of the zygomatic arch to reach the side of the temporal bone. Excision was performed on the temporal bone to the base of the pterygoid process, and an osteotomy was performed continuously from the maxilla to the pterygoid process. Osteotomy was performed via ultrasonic scalpel. Then, deep excision was performed on the skull base until the foramen ovale was reached. The mandibular branch (VIII) of the trigeminal nerve was resected at the foramen ovale, and bone wax was used to fill the foramen ovale when hemostasis was achieved. The tumor was excised in bulk with the surrounding tissue (Fig. d). Especially, the glenoid fossa was close to the primary tumor site; however, it was excised including the joint disc, and the glenoid and infratemporal fossa were excised, including the periosteum of the skull base. For that reason, the margin was considered complete, and we did not perform the histopathological evaluation intraoperatively.\nThe surgical defect was reconstructed with a free vascularized fibula with skin paddle (Fig. e). The occlusion was performed with intermaxillary wire fixation for 1 week postoperatively. There were no abnormal findings during the postoperative course with complete healing. Right-sided facial nerve dysfunction appeared immediately after surgery.\nThe resected specimen exhibited nearly identical histological findings as observed in the biopsy. Because it involved a periosteal reaction corresponding partially to Codman’s triangle, the tumor was thought to have derived from bone (Fig. ). Immunohistochemical analysis showed positive staining for vimentin, MIB-1 index (40–80%), desmin, α-smooth muscle actin, Bcl-2, neuron-specific enolase, and S-100; it showed negative staining for AE1/AE3, caldesmon, and CD34. Thus, the final diagnosis was osteosarcoma (fibroblastic). The resection margin was negative for tumor. After excision of the mandibular tumor, excision of lung cancer was performed under thoracoscopy by a respiratory surgeon in our hospital. Although we recommended adjuvant treatment after surgery, the patient refused this treatment.\nThere has been no evidence of local recurrence or distant metastasis through 30 months of follow-up. The chin of the mandible deviates to the right side during opening (maximum mouth opening, 40 mm). Centric occlusion has not changed. Facial nerve dysfunction gradually improved and became mild according to House-Brackmann Scale evaluation (Figs. and ).
The patient referred for invasive coronary angiography was a 42-year-old male, active duty US Army soldier, without significant cardiac history, admitted to Brooke Army Medical Center with chest pain. On the day of admission, the patient noted severe substernal chest pain along with lightheadedness, flushing, and nausea. Symptoms persisted for approximately 45 minutes and resolved spontaneously soon following presentation. Electrocardiogram obtained on arrival revealed diffuse ST segment depressions with an initial serum troponin T reported at 0.02 ng/mL. The patient was hemodynamically stable and free from chest pain at the time of admission. The patient was admitted to the Cardiology service for management of his NSTEMI, with plans to pursue an early invasive strategy. The patient was given aspirin 325 mg orally, clopidogrel 600 mg orally, and atorvastatin 80 mg orally and a continuous heparin infusion was initiated. Troponin T peaked at 0.42 ng/mL twelve hours following admission.\nThe following morning, the patient was sent for left heart catheterization with coronary angiography with arterial access via the right radial artery. The patient was normotensive and in normal sinus rhythm on arrival to the catheterization laboratory. Midazolam 1 mg and fentanyl 25 mcg were administered intravenously, with appropriate conscious sedation achieved. After standard preparation of the site and subcutaneous infusion of 3 cc of 1% lidocaine, arterial access was obtained without difficulty via modified Seldinger technique and a hydrophilic Terumo 6 Fr Glidesheath Slender was advanced into the vessel followed by administration of intra-arterial verapamil 2.5 mg and nitroglycerin 400 mcg. Continuous intravenous heparin drip was also started prior to initiation of the procedure. A 5 Fr pigtail catheter was advanced into the left ventricle over a J-wire in the standard fashion for evaluation of LVEDP prior to ventriculography.\nImmediately following catheter placement, the patient experienced probable acute radial artery spasm, preventing either antegrade or retrograde movement of the catheter. Manipulation of the catheter was discontinued for 5–10 minutes to allow for spontaneous resolution of the spasm; however, catheter entrapment persisted. Additional conscious sedation with fentanyl 50 mcg and midazolam 1 mg was administered intravenously, warm compresses were applied, and supplemental intra-arterial infusions of nitroglycerin and verapamil were provided, again without resolution of catheter entrapment. Escalation of vasodilator therapy was then pursued with initiation of a nicardipine infusion at a rate of 12.5 mg/hour. After sufficient time was expended for medication effect to occur, attempt at catheter removal was again unsuccessful. Local subcutaneous administration of additional lidocaine and nitroglycerin was ineffective as well. Right femoral access was then obtained and the procedure resumed, revealing normal coronary angiography and left ventricular hemodynamics ().\nWith multiple traditional interventions for relief of radial spasm exhausted, consultation with anesthesiology was pursued to assist with escalation of sedation. The patient was converted by the anesthesia team to general sedation, first with propofol infusion followed by a dexmedetomidine infusion. Catheter entrapment nonetheless persisted. At this time, surgical consultation with vascular surgery and cardiothoracic surgery was obtained, as surgical intervention appeared a distinct possibility for catheter removal.\nA final attempt at nonsurgical intervention was collectively decided upon, with suggestion made to attempt regional axillary nerve block as a means of reducing sympathetic tone attributing to the severe vasoconstriction. Under ultrasound guidance, 40 cc of 1.5% mepivacaine without epinephrine was infused slowly in the location of the right axillary nerve. Right upper extremity venous engorgement and vasodilation were appreciated on ultrasound during infusion. Approximately seven minutes after completion of the infusion, gentle traction was placed on the radial catheter, resulting in atraumatic removal of both the sheath and catheter.\nThe patient was monitored as an inpatient for 24 hours. He regained full function of his right arm without notable neurovascular deficiency. He has been followed up routinely as an outpatient without development of such deficiencies.
A 31-year-old woman with no significant past medical history presented to our emergency department complaining of a constant headache for the previous 4 days. The headache had begun approximately 6 h after receiving epidural anesthesia for labor. The documentation from the anesthesia service that day reported the use of a 17-gauge Touhy needle to enter the subdural space in the lower lumbar spine and the placement of a 19-gauge epidural catheter. No complications were reported with the procedure, and specifically, there was no mention of inadvertent dural puncture. The patient had an unremarkable delivery of a healthy infant at 38-weeks gestation later that day.\nThe patient described the headache as constant and occipital with some radiation to the frontal area. The headache was worse when upright and partially relieved in the supine position. She reported taking acetaminophen/butalbital/caffeine and ibuprofen with little relief. She had no associated vomiting, fever, or changes in her hearing or vision. She denied any photophobia or focal weakness or numbness. She was afebrile on physical exam, with pulse and blood pressure within the normal range. Her exam was notable for a normal neurologic exam including cranial nerves and no neck stiffness. The patient was tentatively diagnosed with a PDPH. After evaluation by the anesthesia service, she was admitted for pain control and possible placement of an epidural blood patch the next day. A computed tomography (CT) scan of her head was obtained prior to admission to evaluate for other possible causes of the headache (Fig. ). This CT identified bilateral parafalcine subdural hematomas measuring 7 mm in thickness on the left and 3 mm thickness on the right. There was no associated mass effect.\nThe patient was admitted to the intensive care unit and started on levetiracetam for seizure prophylaxis. Neurosurgical consultation advised observation, and a repeat CT scan of the head the next day showed no significant change in the hematomas. The patient also received an epidural blood patch the next day with no improvement in the headache. A head CT performed on hospital day 3 showed a decrease in the size of the hematomas, and the patient was discharged on levetiracetam for seizure prophylaxis for 1 week.\nISH occurring after dural puncture is extremely rare. Only sporadic case reports and a few small case series have described this condition [–]. Any procedure that results in spinal dural puncture will theoretically predispose to the development of an ISH. ISH has been described following epidural and spinal anesthesia, as well as lumbar puncture, myelography, epidural steroid injection, and after implantation of an intrathecal drug delivery device and a spinal cord stimulator [–]. The incidence of ISH specifically caused by epidural anesthesia used in obstetric practice has been estimated to be 1:500,000 [].\nThe same mechanism has been postulated for both PDPH and ISH []. The leakage of cerebral spinal fluid (CSF) from the dural puncture site may continue for several weeks, causing reduction in CSF volume []. This results in lower intraspinal and intracranial pressure, leading to relative ventricular collapse and caudal movement of the spinal cord and brain. As a consequence, the dura, pain-sensitive structures, cranial nerves, and subdural bridging veins are stretched. This may ultimately result in a tear of the bridging veins and consequently an ISH. Risk factors associated with ISH after dural puncture include excessive CSF leakage from multiple punctures in large needle use, pregnancy, coagulopathy, cerebral vascular abnormalities, dehydration, brain atrophy, and alcoholism [–].\nThe duration of time from dural puncture to the diagnosis of ISH ranges widely from 4 h to 29 weeks []. In one case series, 37% of cases were diagnosed within 1 week of dural puncture, and 85% were diagnosed within 1 month []. A headache, most often diagnosed as PDPH, is the main presenting symptom [, –]. Other reported symptoms and signs present at the time of diagnosis are listed in Table [, , ]. Reported rates of surgical intervention for ISH after dural puncture vary from 9 to 80% [, , ]. In general, surgical intervention for ISH is indicated if the hematoma thickness exceeds 10 mm, there is a midline shift of greater than 5 mm, or there is neurologic deterioration []. Furthermore, some have advocated for the use of epidural blood patching in the treatment of ISH caused by dural tears resulting in chronic CSF leaks [, ]. A full recovery is reported in over 80% of patients, with death reported in 7–10% of cases [–].\nHeadache in the postpartum period is common, occurring in 39% of women []. The majority of these headaches are benign primary headaches, such as migraine and tension type []. Secondary headaches in the postpartum period are typically due to obstetric or anesthetic complications, or the hypercoagulable state after delivery (Table ). Our patient was initially misdiagnosed as having PDPH, similar to many previous reports of this condition. PDPH is defined as a headache that develops within 5 days of dural puncture that significantly worsens soon after sitting upright or standing and improves after lying horizontally []. PDPH is more likely to occur in young women of low body mass as compared with other patients []. An epidural blood patch is considered the gold standard for treatment of PDPH, with a success rate of 70–90% []. Over 85% of patients report resolution of PDPH within 6 weeks regardless of treatment [].\nThe incidence of ISH after dural puncture is probably underreported since many of these patients are treated as PDPH with the eventual resolution of their symptoms. When to obtain brain imaging studies in the assessment of a likely PDPH is unclear. A reasonable approach would be to consider imaging in patients that (1) have a postural headache lasting more than 1 week, (2) do not improve or have worsening of their headache after an epidural blood patch, (3) report a change in the headache from postural to non-postural, or (4) develop other neurologic signs or symptoms with the headache [].
A 26-year-old Caucasian woman, gravida 2, para 1, with a spontaneous normal vaginal delivery 2 years ago was referred to our institution at 20.3 weeks of gestation after a sonographic finding of a sacrococcygeal mass of 26 × 24 mm in a male fetus.\nThe patient had no family history of birth defects or genetic disorders. She did not have any medical or surgical history, and she had no alcohol or smoking habit. She had no relationship with the father of her fetus and received no drug therapy while pregnant.\nShe had a normal gestational course with low risk of aneuploidies in the first-trimester screening and a normal first trimester scan at 13 weeks. Her sonographic examination revealed a single intrauterine pregnancy with an estimated gestational age of 20 weeks. The study revealed an exophytic, mixed echogenic mass arising from the sacrococcygeal region with high vascularization seen on Doppler flow (Fig. ). The examination showed adequate amniotic fluid, and no other abnormalities were detected.\nMagnetic resonance imaging was performed, which confirmed the diagnosis. There was no evidence of possible invasion of the fetal pelvis or abdomen. The spine appeared intact. The lower extremities, fetal kidneys, and bladder appeared normal. On the basis of these findings, a diagnosis of external variety, type I in the Altman classification, was confirmed (Fig. ).\nAmniocentesis guided by ultrasound scanning was done with normal karyotype and microarray results. The result of a fetal echocardiographic scan was normal.\nThe patient was scheduled for follow-up by ultrasound weekly (Figs. and ). These scans showed an increase in the size of the mass up to 190 × 150 mm with high Doppler flow and severe polyhydramnios (amniotic fluid index 37) (Fig. ). The patient developed gestational diabetes, which required insulin treatment.\nAt 33.6 weeks of gestation, the patient was admitted to the obstetric ward for preterm labor. Her vital signs were normal (body temperature 36 °C, pulse rate 90 beats/minute, and blood pressure 135/82 mmHg), as was her physical examination. Treatment with a corticosteroid (12 mg intramuscularly, twice) and atosiban was started. The tocolysis was effective, and an elective cesarean section was scheduled at 35 weeks of gestation in conjunction with the neonatal service and the pediatric surgeon. However, 1 day before the scheduled cesarean section, the patient had premature rupture of membranes. Fetal heart rate monitoring revealed recurrent late decelerations at that moment, and an emergency lower segment cesarean section was performed.\nA male infant was born at 35.1 weeks with an SCT of 200 mm. The combined weight of the baby and teratoma was 4030 g (Fig. ). His Apgar score was 9-10-10.\nExcision of the teratoma was performed at 36 hours of life, after embolization of the middle sacral artery. Surgery was done without incident, with an operative time of 3 hours. The reconstruction was done without any excess skin (Fig. ). Pathological findings revealed an immature teratoma and no evidence of yolk sac tumor or other malignant elements. The postoperative alpha-fetoprotein (AFP) levels decreased quickly, being 150,000 before surgery and 64,500 afterward. The neonatal AFP values were followed during the first months with values of 14,915 at the 14th day after birth, 4136 at 6 months after birth, and 1.3 at 12 months of life. Other blood test parameters (including liver function, blood cell count, and hemostasis) were normal in both mother and infant samples.\nThe baby was discharged at 25 days after birth with normal results of abdominal, cerebral, and kidney ultrasound scans. Neonatal follow-up was performed during the first 16 months, and no long-term neurological deficits have appeared.
A 37-year-old woman with LAM underwent bilateral lung transplantation on cardiopulmonary bypass for progressive hypoxic respiratory failure despite medical therapy with sirolimus over the preceding 5 years. Sirolimus was held for a total of 9 weeks prior to transplant (3 weeks prior to listing). She was extubated to room air on postoperative day (POD) 3, but developed a right sided chylothorax on day 4. Conservative measures, including no enteral nutrition, total parenteral nutrition (TPN), and subcutaneous octreotide were attempted with no clinical improvement. On POD 26, she required a sub-xiphoid window for a large pericardial effusion. An attempt at lymphangiography to seal the thoracic duct was attempted 6 weeks after transplant but was unsuccessful. She developed a chylous leak from the abdominal portion of the thoracic duct, resulting in chylous ascites. On POD 55, she underwent right sided video-assisted thoracoscopy with lysis of adhesions and mass ligation of the thoracic duct, but the chylothorax persisted. Both sirolimus and everolimus were tried after transplant without success. Whereas sirolimus was reasonable well tolerated pre-transplant, she developed anasarca after transplant. Everolimus was started at 5 mg, increased to 10 mg daily, and continued for 6 weeks. However, she developed ill-defined bilateral lung infiltrates and hypoxia not attributable to acute rejection or infection. These resolved after discontinuation of the drug.\nFive months after transplant she underwent thoracotomy with repeat ligation of the thoracic duct and two additional large lymphatic tributaries, as well as mechanical and chemical pleurodesis resulting in resolution of the chylothorax. A Denver shunt was placed to control chylous ascites. This shunt would require multiple revisions, manipulations, and replacements over the next 2 years and finally removed. She was referred to a second tertiary care center with a specialized lymphangiography unit for an opinion and possible intervention. Exploratory laparotomy was performed in an attempt to identify and treat culprit vessels. A Denver shunt was placed on the left side, which would continue to require manipulations and revisions and was removed and replaced with an external drainage catheter.\nThe patient was referred back to the same “specialized in lymphatic interventions” center for additional attempts at percutaneous treatment. Magnetic resonance (MR) lymphangiography demonstrated significant lymphatic leak from the left pelvic and lower retroperitoneal lymphatic masses (). Intranodal lymphangiography was then performed with oil-based iodinated contrast (Lipiodol; Guerbet Group, Princeton, NJ, USA) to verify the location of the lymphatic leak. Under fluoroscopy guidance, the retroperitoneal lymphatic masses were accessed transabdominally using a 21G Chiba needle (Cook Inc, Bloomington, IN, USA). The iodinated contrast was then injected through the needle to confirm its position in the masses (). The masses leading to the leak, were then embolized through the needle using N-butyl cyanoacrylate (N-BCA) glue (Trufill; Codman Neuro, Raynham, MA, USA) diluted 1:3 with Lipiodol. During embolization a small amount of glue leaked through an iliac vein into pulmonary circulation. The pulmonary emboli were then successfully removed from a right pulmonary vein using an endovascular snare through the right femoral vein approach. The output from the drain decreased over the next few days, and the drain was removed.\nHer symptoms would finally be controlled in the following 12 months.
A 65-year-old gentleman presented to the emergency department (ED) at night with a history of acute-onset atraumatic bilateral knee pain and swelling. His past medical history includes bicuspid aortic valve replacement and subsequent revision of his aortic valve replacement 4 weeks prior to the onset of knee pains. He was diagnosed with sternal surgical wound infection (clinically erythematous skin with possible discharge) 4 days prior to hospital attendance with associated left knee pain and swelling. A diagnosis of wound infection with reactive arthritis was made by his general practitioner (GP), and the patient was commenced on a course of clarithromycin due to penicillin allergy. The wound infection appeared to be responsive to oral antibiotic treatment. However, his left knee symptoms gradually worsened and greatly affected his mobility; 12 hours before ED attendance, he developed acute right knee pain and swelling with associated generalized fatigue. Clinical observation raised concerns of sepsis with a pyrexia of 39°C and tachycardia of 100 beats per minute. Both of his knees were diffusely swollen, warm, and extremely tender to palpate. He did not tolerate any range of movement of his knees. Hip and ankle joints were normal, and there was no appearance of cellulitis. He was also reviewed by the medical team, and other common sources of sepsis including chest or urine infections were ruled out.\nOn admission, his blood test showed raised inflammatory markers, with white blood cells of 15.9 × 109 L and C-reactive protein (CRP) of 288 mg/L. His knee X-rays revealed no bony pathology otherwise. Aspiration of both knees was performed under sterile technique on the ward prior to commencing antibiotics—cloudy thick pus was drained from both knees (Figures and ). Urgent microscopy and gram stain did not show any organisms.\nThe patient's initial presentation to his general practice with acute unilateral atraumatic knee pain and swelling raised suspicion of reactive arthritis secondary to a wound infection. His subsequent presentation to the emergency department with bilateral knee symptoms and inability to mobilize associated with systemic symptoms including pyrexia raised the clinical suspicion of bilateral SA. Raised white blood cell and C-reactive protein suggest SA as the most likely diagnosis, although both knee aspirations were negative.\nThis patient was started on combination of antibiotics treatment according to the local hospital guidelines. He then proceeded to have bilateral knee arthroscopies and washout which revealed more pus collections and active synovitis (Figure ). Synovial fluid samples were obtained and sent for culture. He was treated with a total 3-week course of intravenous antibiotics and underwent a total of four knee joint washouts. A total of six synovial fluids and two intra-articular soft tissues were sent for culture—none of which grew any active microorganisms. The possibility of infective endocarditis was also considered in view of the recent revision of his prosthetic valve. He had a total of four blood cultures collected at separate times, all of which were negative. Inpatient echocardiogram and subsequent transesophageal echocardiogram (TOE) showed no vegetation on his prosthetic heart valve.\nAfter repeated washouts and a prolonged course of IV antibiotics, the patient demonstrated full clinical recovery also evident with improvements in his inflammatory markers. He was then discharged with a further 2-week course of oral antibiotics along with an outpatient clinic follow-up. He underwent subsequent reviews by a cardiothoracic surgeon and cardiologist in view of his recent prosthetic heart valve revision surgery which was satisfactory.
A 61-year-old female presented with Gustilo-Anderson Grade 3C compound fractures of the distal femur and Shatzker five tibial plateau fracture. She underwent initial debridement and a spanning external fixator in the acute setting and subsequently definitive fixation with a retrograde femoral intramedullary nail and lateral and posteromedial proximal tibia plates, with a bipedicled gastrocnemius flap and split skin grafts inset over the large skin defect in the same setting. The healing was complicated by tibia plateau fracture and she underwent a planned bone grafting 3 months later.\nBone union was achieved at 5 months post operatively, but the knee range of motion was limited from 5 to 20 degrees as shown in . Aggressive physiotherapy was commenced but failed to improve the range of motion. A Judet quadricepsplasty and removal of implants was subsequently performed 12 months after the initial surgery and 9 months after the secondary bone grafting surgery.\nThe anaesthetic team inserted an epidural catheter for continuous epidural anaesthesia prior to surgery and was retained in situ for the first week. Intravenous Cefazolin was given 1 hour prior to initiation of surgery and continued on for 24 hours. In supine position under general anaesthesia, the leg was cleaned and draped above the level of the anterior superior iliac spine (ASIS) to the foot. A sterile tourniquet is applied and the initial skin incisions are marked out; along the lateral intermuscular septum to the lateral aspect of the patellar tendon and a medial parapatellar S-shaped incision as depicted in and .\nThe lateral incision was made first directly through the skin, fascia and muscle preserving the inter-connective tissue between the fascia and the skin to preserve dermal blood supply. The knee joint was entered via a lateral parapatellar capsular incision and the intra-articular adhesions between the femoral condyles, tibia and patella were released.\nA medial parapatellar approach was then utilized for adhesiolysis of the medial aspect of the knee. A simultaneous partial release of the medial collateral ligament (MCL) and medial capsule was performed. Under radiological guidance, the posteromedial tibial plate was removed to prevent possible impingement during flexion. At this point of time, the knee was ranged and flexion had improved to 60 degrees as shown in .\nThe tourniquet was then removed and the lateral incision was then extended to the level of the Greater Trochanter. The rectus femoris origin was released at its origin via the same lateral incision, a modification of the original Judet’s procedure.\nThe quadriceps muscle slide was then performed by elevating the quadriceps muscle off the femur along the intramuscular septum using an extra-periosteal approach as shown in . Careful cauterization and ligation of the perforating vessels was performed at this juncture to prevent significant blood loss.\nFractional lengthening of the fascia lata and anterior fascia of the thigh was then performed by making multiple transverse incisions at multiple levels. The intramedullary femoral nail was then removed. At this point, maximal knee flexion of 0 to 120 degrees was achieved as shown in . The medial and lateral incision’s subcutaneous tissue and skin were then closed over vacuum drains with the capsule intentionally left open to prevent recurrent capsular adhesion formation.\nAn epidural catheter provided analgesic relief for 5 days post-surgery before gradually being weaned off and converted to oral analgesia.\nContinuous Passive Motion (CPM) was initiated in the postoperative care unit and continued for 24 hours for the first week. Electric muscle stimulation of the quadriceps muscles, aggressive physical therapy and cycling exercises were started on the 3rd post-operative day and the patient was allowed to ambulate on the 6th day post-surgery, after the epidural catheter had been removed.\nHer drains were removed on post op day 4 and 5 when the drainage amount was minimal. Regular monitoring of her haemoglobin levels showed a drop of 1.0 g/dL on the first post-operative day requiring blood transfusion. The haemoglobin levels were subsequently maintained.\nRemoval of sutures was performed at 3 weeks where the wounds had healed well and there was no evidence of dehiscence or skin edge necrosis. At 3 months follow up, the patient was able to ambulate and had knee range of motion from 0 to 90 degrees without extension lag as seen in . This represented an increase in range of motion of 75 degrees and the patient was able to climb stairs and return to daily activities.
We report the case of a 3 year old Caucasian boy who first developed erythematous patches distributed over the whole body at the age of 5 months (Fig. ). A skin biopsy was performed, showing faint polymorphonuclear granulocytic infiltrates; a histopathologic diagnosis of probable autoinflammatory skin disease was made, which prompted further diagnostic evaluations into SAIDs (s. Table ). At 8 months of age, macroscopic haematuria, bloody diarrhoea and epistaxis were reported by the mother. Shortly after, recurrent episodes of fever up to 39.5 °C, 1–3 days duration, were reported to be occurring every 2 weeks, accompanied by an increase in erythematous skin lesions. From the age of 12 months, ulcerative skin lesions developing on previously intact skin primarily on the extremities, a sudden-onset unilateral facial swelling and transient anisocoria were seen (Fig. ). Deep skin defects developed after medical interventions such as bone marrow aspiration or immunizations, which were interpreted as representing a pathergy phenomenon. Subsequently, from the age of 18 months, palmoplantar bullous skin lesions occurred with rapid detachment of epidermal layers. One skin lesion on the foot healed with scar formation.\nThe boy was the youngest of several siblings of a family where both parents were part of the household and did not have connections to the health care sector, and who had no apparent psychiatric abnormalities.\nA diagnosis of SAID was one of the first diagnoses suspected, and was first proposed by the dermatologists, as fever and skin symptoms are typical findings for these entities. However, extensive testing yielded negative results (Table ). Autoimmune diseases and primary immunodeficiencies were ruled out by these investigations. Primary dermatologic disorders such as congenital or acquired epidermolysis bullosa, bullous pemphigoid diseases and other blistering skin diseases were ruled out by skin biopsy. In total, six skin biopsies were performed over a time span of 2 years. All biopsies showed faint perivascular lymphocytic infiltrates associated with rare infiltrates of polymorphonuclear granulocytes, possibly caused by mechanical or medical irritation of the skin. Several imaging studies were performed including abdominal ultrasound, brain MRI and echocardiography, all with normal results. Eye exams did not show any inflammatory changes and an underlying cause for the episodic mydriasis was not seen. Metabolic diseases were also ruled out by extensive testing. Experts for childhood metabolic diseases speculated on the presence of a leukotriene-associated disorder, but there was no specific evidence to support this suggestion.\nDue to suspecting a monogenic SAID like TRAPS or an undifferentiated SAID throughout the disease course, several treatments were implemented sequentially or in combination, including prednisolone up to 2 mg/kg/d, azathioprine (4 months of therapy), dapsone (3 months of therapy), anakinra, ibuprofen and/or hydroxychloroquine. However, most of the signs and symptoms described above persisted or recurred during treatment.\nSubsequently, clinical investigations shifted increasingly to the search for external and/or toxic factors. Contaminants in the household were considered and actively investigated, and screening for heavy metal poisoning was pursued. In total five hospital admissions for diagnostic purposes took place.\nAs the extensive work-up performed continued to yield only negative results, it became increasingly apparent that intrinsic disease was unlikely and MBPS was suspected. However, during a 3-week hospital stay with intensified surveillance, no maternal interference, that might explain the patient’s signs and symptoms was observed, symptoms remained the same. During this time two findings in particular aroused suspicion, (1) reversible unilateral (otherwise unexplainable) mydriasis, not reactive to topical application of pilocarpine 1% and (2) the sudden appearance of bullous skin lesions in previously intact skin. A North American expert panel on child abuse was consulted by telephone conference and it was felt that this case was highly suspicious for MBPS. Ultimately, both parents were confronted with the probable diagnosis with participation of the youth welfare service and an attending psychiatrist. As the mother was the person most consistently involved in the care of the child and was always present during hospital stays, we suspected her rather than the father to be the perpetrator. The mother and patient were separated immediately and the patient was admitted together with his father. Within 2 weeks all signs and symptoms of disease resolved.\nIn the meantime, the parents had contacted the police to report, that a crime had been committed by an unknown perpetrator. After 6 weeks of hospitalization, the patient’s mother confessed in writing to having performed multiple actions that resulted in different signs and symptoms. These actions ranged from (1) erroneous reporting (fever; medication not given), to (2) interference of specimens (blood in urine/diaper; manipulation of thermometer), and (3) induction of symptoms (mechanical irritation; application of capsaicin, anti-wart patches, mydriatic eye drops and saliva/stool application to induce skin infection) (Table ).\nRetrospectively, the primary care pediatrician reported abnormally frequent visits of the mother with her son to his medical practice, with up to 12 visits a month, possibly indicating secondary gain for the mother. However, no medical opinions beyond our center were accessed by the family. At the age of 3 years, a diagnosis of MBPS was firmly established with the evidence that separating mother and child led to spontaneous resolution of all disease symptoms and signs, along with the confession from the mother.
A 50-year-old Caucasian female visited the regional dental office due to uncertain pain in the right mandibular region. No significant diseases were mentioned in the anamnesis. The patient was diagnosed with gingivitis, and treated with a non-steroid anti-inflammatory drug and oral rinsing with chlorhexidine. The condition of the patient did not improve significantly. When she returned a week later, she had no alleviation, but labial paresthesia on the right side of her lip. As the cause of the new symptom remained unidentified, she was referred to the oral surgery group. Initially, the paraesthesia affected only the right side of the lower lip, but by this time it had spread to the skin of the right side of her chin. The oral surgeons decided to extract the lower right wisdom tooth, but the labial paraesthesia still persisted. Since there was an extended composite restoration in the lower right first molar, and translucency around its apical region was visible, the lesion was diagnosed as periapical granuloma. The lesion on tooth 46 was not close to the mandibular canal; this can be seen on Fig. . Therefore, root canal treatment was carried out, despite the uncertain result of the percussion, palpation and sensibility test. The transparent lesion around the apex and the negative sensibility test lead to the decision of endodontic treatment. Diagnosis was made by a dentist who is not a staff member of our Department. The treatment revealed that the pulp was vital. Our oral diagnostic team examined the patient, and we diagnosed the case as cemento-osseous dysplasia based on the panoramic radiograph showing apical translucency lesions around the anterior teeth with vital pulp (Fig. ). The paraesthesia has been continually present ever since. Because the lesion around the lower right canine had already involved the cortical bone by CBCT scan (Fig. ), a root canal filling and removal of the apical lesion were performed. On the CBCT image the involvement of the entire periapical region can be seen including the lingual region and the buccal cortical (Fig. ). It became clear during the planning of the surgery that the blood vessels and nerves of tooth 43 would be injured.\nThe root canal treatment was carried out on the lower right canine in a single-visit treatment using local anaesthaesia. We explored the entire root canal length using a size #15 hand instrument. The working length was determined using an apex locator, (Woodpex III, Guilin,China), then the length was also confirmed with radiographic imaging. Following the length determination, the root canal was shaped using Wave One (Densply Maillefer, York, USA) rotary instrument. The root canal was obturated using guttapercha and AH Plus sealer (Dentsply DeTrey GmbH, Konstanz, Germany) with lateral condensation technique. Glass ionomer cement (Fuji IX GP, GC Co., Tokyo, Japan) was then applied to seal off the access cavity, while the permanent restoration was done.\nWe prepared an intraoral mucoperiostal flap using an L-shaped incision and the surgery was carried out by using a surgical microscope (Aspheron, Schmidt and Bender Hungaria, Budapest, Hungary).\nWe opened up the buccal cortical bone using a surgical bur, thereafter we removed the lesion surrounding the apex of the root. The root apex was resected and a retrograde root canal filling was placed using mineral trioxid aggregate (MTA+, Cerkamed, Stalowa Wola, Poland).\nThe area of the lesion was augmented using gentamicin impregnated BoneAlbumin (OrthoSera Dental Zrt., Gyor, Hungary). We seeked to lower the chance of osteomyelitis occuring with the use of gentamicin. Sutures were then carefully placed to achieve tensionfree closure of the flap for optimal healing.\nThe 19-year-old Caucasian woman is the daughter of the first patient. CBCT showed a radiolucent lesion around the root of the lower right incisor (Fig. ). She is presently asymptomatic after a one-year follow-up, but she occasionally felt tension and moderate pain in the right side of the mandibular region eradiating to her ear, approximately 2 years earlier. The symptoms had no dental background, and ceased gradually.\nPulp tests and periapical pathology diagnoses were made by authors on teeth 33,32,41,43 with the use of percussion, palpation and sensibility test.\nPanoramic radiograph and CBCT scans of the first patient showed radiolucent lesions located in the periapical bone, specifically on lower incisors and canines – in the premandibular, and in the right molar region of the mandible with radiopaque parts showing the lesions inside (Figs. and ). The affected teeth were asymptomatic, CBCT proved the presence of apical pathology. The buccal cortical involvement was discovered with the aid of CBCT, which also affected the treatment plan because 2D imaging methods provide no information on bucco-lingual dimension. Prior to the surgical intervention the use of CBCT was motivated by the fact that only 3D imaging could ascertain the precise shape, location and involvement of surrounding anatomical landmarks of the lesion.\nFigure shows the postoperative condition (Fig. ). At the 6-months periapical follow-up, the bone healing is being processed (Fig. ). 18 months later it can be seen on the CT image that the buccal cortical bone surrounding the root of tooth 43 has regenerated (Fig. ). In the case of the second patient, there were no notable changes in the radiographic status 1 year later, and she has no complaint at present.\nThe removed tissue pieces contained both connective tissue and calcified areas. In the calcified area, beside the irregular trabecular – lamellar bony formations, oval and globular cementum-like structures were present. In the non-calcified area connective tissue was found and connective tissue filled out the centre of another bony sample as well. No elements or remnants of a capsule were visible. The histological diagnosis was cemento-osseous dysplasia (Fig. ).
H.C. presented as a medically and psychiatrically healthy 30-year-old Caucasian male. He denied a history of significant health and psychiatric conditions and denied a family history of psychosis. He endorsed prior cannabis use, denied a history of any significant adverse effects associated with prior use, and he disclosed that 6 years had passed since he last used cannabis. He reported weekly use of alcohol and caffeine and denied use of nicotine/tobacco products and illicit drugs. He endorsed use of over-the-counter medication as needed for seasonal allergies. During the first three study sessions, he smoked cannabis that contained 0, 10, or 25 mg THC through hand-held pipe. Dose-related subjective drug effects, cardiovascular effects, and impairment on cognitive performance assessments were observed as expected. On the fourth experimental session (as with other experimental sessions), baseline assessments were within normal limits and urine drug screening (for common drugs of abuse) and breath alcohol tests were negative. After consuming a standard low-fat breakfast, the participant self-administered vaporized cannabis that contained 25 mg THC within 10 min (per protocol). Acute drug effects escalated in magnitude for the first 20 min following inhalation. He had difficulty responding to staff inquiries, was unable to complete self-report questionnaires, had difficulty keeping his head up, and appeared to periodically fall asleep or lose consciousness despite encouragement by research staff to stay awake and continue. He was unable to maintain a balanced, steady gait when he walked.\nH.C. displayed behavior consistent with heavy sedation. The volunteer had difficulty maintaining consciousness and, at times, would not respond to verbal inquiries by study staff. He was under direct supervision of medical staff and neither his vital signs nor his behavior required medical intervention. He was able to complete a self-reported drug effect questionnaire, but had extreme difficulty completing cognitive performance assessments in the first 90 min following drug exposure. When he did speak, he reported feeling faint, dizzy, nauseated and that he was experiencing tingling sensations in his arms and legs and pain at the base of his neck.\nQuantitative analysis (LC/MS/MS) of whole blood collected 10 min after the completion of cannabis administration (peak level measured in this study) showed 16 ng/mL THC, 3 ng/mL 11-OH-THC, and 17 ng/mL THC-COOH for H.C. These are consistent with mean values (14 ng/mL THC, 2 ng/mL 11-OH-THC, and 7 ng/mL THC-COOH) observed for all participants in this study at that time point and dose of vaporized cannabis. Analysis of whole blood collected 10 min after the completion of cannabis administration in the smoked condition for the same dose level for H.C. (25 mg THC) showed 1 ng/mL THC, 1 ng/mL 11-OH-THC, and 4 ng/mL THC-COOH, and these values are consistent with mean values observed for all participants in this study at that time point and dose of smoked cannabis.\nThree hours after drug administration, his symptoms began to decrease in severity. He indicated that he had experienced a dissociative state and altered perceptions of auditory and visual stimuli at the time of peak drug effect. He reported a hypersensitivity to voices at that time, which he described as if he was more aware of conversations around him, but was unable to hear or understand distinct words. He described visual distortions in the form of the environment and floor sinking away and the appearance of patterns moving on the carpet and chairs in the room. Additionally, he reported an “out-of-body” experience characterized by the feeling of being removed from his body, existing above it in space, and feeling that his surroundings were sinking away from him, which was also accompanied by a feeling of paralysis. He reported having had a similar experience when administered ketamine before surgery for a broken leg. Four hours after drug administration, and after eating lunch, H.C.'s symptoms of nausea, faintness, dissociation, and auditory, visual, and perceptual alterations had almost completely subsided. Five hours after drug administration, he appeared more alert and was able to complete all study-related tasks.\nAt the end of the experimental session, H.C. was prompted to recount his experience. He reported feeling overwhelmed that it was an uncomfortable, scary, and unpleasant experience akin to what he would expect an overdose or anxiety attack may feel like, and he indicated he never wanted to have the experience again. He expressed the sense that he thought he would “never come out of this” and that he would always feel the adverse effects. Within 8 h of acute drug administration, measures of cognitive performance, subjective mood and drug effects assessments, and vital signs had returned to baseline levels. The study medical team determined that there was no significant health risk with continued study participation and H.C. completed two remaining experimental sessions (lower dose and placebo cannabis) without significant discomfort.
A 12-year-old boy was hospitalized with complaints of a headache and high fever accompanied by psychosis, delirium, and indistinct consciousness. He was diagnosed with limbic encephalitis, which is an autoimmune disorder characterized by inflammation of the limbic area in the brain. His symptoms became exacerbated, and he required intensive therapies including high-dose steroid and catecholamine administration.\nDespite the continuous therapeutic support mentioned above, he abruptly developed hypotension following diarrhea, fever, and abdominal distension on day 41 after admission. Metabolic acidosis (pH 7.34, base excess − 7.0 mmol/L) was confirmed by blood gas analysis, and highly elevated CPK 11800 U/L, AST 461 U/L, ALT 201 U/L, and LDH 1034 U/L values were revealed by a blood chemistry profile. An emergency CT scan revealed pneumatosis intestinalis localized in the ascending colon and rectum coexisting with portal venous gas (Fig. ). While the root of the SMA and the inferior mesenteric arterial (IMA) flow was maintained, the peripheral blood flow was attenuated adjacent to the non-contrast-enhanced ascending colon and rectum.\nAlthough intraabdominal free air was not detected in the CT scan, the massive ascites and progressing peritoneal signs with muscular guarding required an emergency laparotomy for suspected mesenteric ischemia and bowel perforation. Intraoperatively, skip ischemic lesions were observed in the ascending colon close to the hepatic flexure and the rectum without bowel perforation. Although SMA and superior rectal arterial pulsations were present, the marginal perfusion near the two lesions could not be confirmed. The patient was diagnosed with NOMI based on these operative findings and the rapid progression of the symptoms, which are unlike other vascular disorders or necrotizing enterocolitis. The remaining colon, from the transverse to the sigmoid colon, appeared intact. The color of the unaffected intestinal wall was restored, which suggested intestinal viability (Fig. ). We performed a distal ileostomy without bowel resection because a second-look laparotomy after 24 to 48 h was considered.\nAfter returning to the ICU, the patient required resuscitation for cardiac arrest, septicemia, and DIC. The scheduled second-look laparotomy was canceled, and intensive care including hemodiafiltration was continued. However, the gastrointestinal symptoms did not progress during the intensive treatment.\nOn the 16th and 60th postoperative days, colonoscopies were carried out, and they revealed rectal and ascending colon stenosis with ulceration (Fig. ). The patency was 5 mm in diameter at both strictures. However, normal findings in the transverse colon to the sigmoid colon were observed by colonoscopy.\nA lower gastrointestinal series by gastrografin contrast radiography also demonstrated the patency of the two lesions after laparotomy (Fig. a). Based on successful evacuation of the contrast media and intact mucosal findings around the mild stricture, we scheduled ileal stoma closure. For 1 month prior to the closure, approximately 100 ml of bowel contents that had collected in the ostomy pouch were injected into the anal side of the ileostomy to induce efficient bowel movement. We confirmed the continuous expulsion of feces from the anus and the improvement of transient bowel strictures; therefore, the ileal stoma was closed 14 months after the previous laparotomy.\nCurrently, the patient’s confusional state has prolonged, and he has received enriched liquid nutrition via gastrostomy. The two stenotic lesions are completely resolved, and defecation has been maintained after stoma closure (Fig. b).
This patient is a 56-year-old lady who presented to the emergency department on November 27, 2020, regarding on and off severe epigastric pain radiating to her back for one month associated with nausea. The patient did not report any feeling of abdominal mass, and she did not have any weight or appetite loss during the past months. She is post-menopausal with no complaint of vaginal bleeding, discharge, or lower abdominal pain. There were no other gastrointestinal or urologic symptoms, and her systemic review was unremarkable. Her past surgical history is significant for an open surgical intervention done due to a right ovarian tumor diagnosed in March 2012. The surgical intervention was cytoreductive surgery, including total abdominal hysterectomy, bilateral salpingo-oophorectomy, ascites cytology, bilateral pelvic lymph node dissection, para-aortic lymph node sampling, and omental biopsy. Her final histopathology diagnosis came as an adult-type granulosa cell tumor of the right ovary stage III-A.\nThe patient received adjuvant chemotherapy starting from May up to September 2012 composed of bleomycin, etoposide, cisplatin (BEP) protocol for one cycle; however, the protocol was shifted to paclitaxel and carboplatin due to severe neutropenia and gastrointestinal discomfort. The patient received six cycles of the second protocol with no significant side effects. She was followed closely for five years in the clinic, up to 2017, by physical examinations and scheduled imaging with no signs of recurrence and was then lost to further follow-up. Her family history is positive for breast cancer in her cousin and lung cancer in her father, and she also had multiple comorbidities, including diabetes mellitus type two, gastritis, and benign thyroid nodules.\nOn examination in the emergency department in November 2020 (nine years later), the patient was found to have mild tenderness in the epigastric area, no guarding or rigidity, and no masses or hernias could be felt. All her laboratory results, including complete blood count (CBC), liver enzymes, hepatitis serology, tumor markers, such as AFP (alpha-fetoprotein), CA19-9 (carbohydrate antigen 19-9), CA 125 (cancer antigen 125), CA 15-3 (cancer antigen 15-3), and estradiol, were within the normal range. Anti-Mullerian hormone (AMH), which is considered currently by many authors as an efficient hormone for the diagnosis and follow-up of granulosa cell tumors, was within the normal range as well []. On the other hand, her inhibin-B value, which is another tumor marker, proved to be efficient in detecting both primary and recurrent disease was elevated (120 PG/ML) [-]. The US done at presentation showed an ill-defined, mixed, echogenic, predominantly hypoechoic focal lesion with posterior acoustic enhancement and irregular margins measuring 5.7 x 6.6 x 5.3 cm in the left lobe of the liver (Figure ). No obvious vascularity was noted within the lesion with minimal peripheral and septal vascularity. Features suggested a complex cystic lesion. CT scan with intravenous (IV) contrast confirmed the US findings (Figure ).\nMRI done to further delineate the lesion showed a well-defined, well-encapsulated, rounded, multilocular, septated, and complex cystic lesion in the left lobe of the liver (segments III, IVb) (Figure ). It measured approximately 7.9 x 6.8 x 7.8 cm. It was heterogeneously hyperintense on both T1 and T2-weighted images with hypointense capsule and septa on both sequences. It showed fluid-debris levels within likely, suggestive of chronic hemorrhage/highly proteinaceous content. There were enhancing nodular and papillary projections with diffusion restriction in the soft tissue nodules, suspicious of malignant transformation. The lesion was seen stretching the liver capsule, abutting the pyloric region of the stomach, the duodenal bulb, the junction between the head and body of the pancreas, and the main portal vein; otherwise, the liver was normal in signal intensity and enhancement pattern. In the arterial phase, there was no appreciable enhancement, and in the venous and delayed phases, it showed enhancement of the capsule and the internal septa and no appreciable enhancement of the rest of the lesion. There was mild hepatomegaly; otherwise, the rest of the liver was normal. Preoperative MRI of pelvis done in 2012 was available for comparison (Figure ). Some features of the current hepatic lesion (complex cystic appearance, enhancing wall, papillary projections and multiple internal septations, and internal hemorrhage) were similar to those of the primary ovarian GCT. In view of the patient’s history, a primary diagnosis of hepatic metastatic recurrence of GCT was considered with a differential diagnosis of malignant transformation of a biliary cystadenoma. US-guided biopsy of the hepatic lesion was unsatisfactory. Hence, after discussion in a hepatobiliary multidisciplinary meeting, the decision was to proceed with a PET-CT scan, and it showed a low-level FDG uptake consistent with recurrence of the disease (Figure ). It additionally showed a small peritoneal deposit.\nThe patient was admitted for surgical intervention on the third of March 2021 to resect the liver metastasis, and surgery started with diagnostic laparoscopy to rule out disseminated metastatic disease. The lesion was cystic in texture, measuring around 6X7 cm, and it was occupying segments III and IVb of the left liver lobe. Another small peritoneal nodule measuring around 2X2 cm was found in the right hepatorenal recess. After ruling out a disseminated metastatic disease by diagnostic laparoscopy, we proceeded with the resection by an inverted T-shaped incision and opening the abdominal wall in layers. Adhesiolysis was done around the left liver lobe and then a modified left hepatectomy was done after marking our resection line, which was to the left of the middle hepatic vein to resect segments II, III, and IVb with preservation of segment IVa. The peritoneal nodule was also excised from the hepatorenal recess. The patient had a smooth recovery postoperatively and was discharged on the fifth day of surgery with no significant complications. Histopathological examination of the cystic liver lesion (7.2 x 5 x 4.8 cm) revealed a cystic neoplasm composed of tumor cells exhibiting the characteristic features of granulosa cell tumor (Figures -). The tumor nuclei had prominent grooves, imparting the classical “coffee bean” appearance (Figure ). Whilst Call-Exner bodies are usually seen in the microfollicular architectural pattern of granulosa cell tumor, this feature was not noted in our case, as it had a diffuse architectural pattern.\nImmunohistochemically, the tumor was positive for inhibin (Figure ), WT1 (Wilms' tumor suppressor gene 1), and calretinin (focal), confirming the histological diagnosis. Immunostains for cytokeratin CKAE1/AE3, CK7, and EMA were negative. Similar histological features were noted in the peritoneal nodule (2.2 x 1.5 cm), indicating another focus of metastatic granulosa cell tumor. Both metastatic foci were completely excised with adequate margins.\nThe patient has been following in the clinic for the past months with no new complaint. The gynecology multidisciplinary meeting plan is to follow the patient closely with AMH and inhibin, with no need for further hormonal or chemotherapy treatments.
We present a 20-year-old female with athletic constitution in good health condition. She suffered trauma caused by an accident during skiing, where she jumped over a small hill border and landed in a small pit on the slope. The traumatic mechanism of the accident was a ventral-lateral (right side) collision with the solid iced slope in knee and hip flexion at intermediate speed.\nAccording to the patient’s narrative, she was never unconscious, but immediately felt strong pain in her right upper and lower extremities after trauma. Clinical examination of the peripheral extremities showed no signs of neurological or vascular trauma.\nThe patient was hospitalized via flying ambulance to the closest surgical-trauma department shortly after. At admission to the hospital, clinical examination and conservative X-rays in two planes showed a posterior dislocation of the right hip (Thompson–Epstein Grade I), but no signs of fracture. Additionally, X-rays in two perpendicular planes of the right forearm were performed, showing a combined radial and ulnar fracture. The fracture was splinted with split plaster cast of the right arm. The dislocation was treated by joint reduction (Böhler’s technique) under general anaesthesia. The procedure took place around 2 hours after trauma and was performed by a trauma surgeon. A CT scan of the hip was performed afterwards (, ), indicating an acetabular fracture line without any dislocation and consequently needing no further surgical treatment. After one night of medical surveillance, our patient was discharged from hospital with treatment recommendations to use underarm crutches and to consult the nearest trauma department for surgical therapy of the forearm fracture after returning back home. Regarding pain management, the patient received a prescription for non-steroidal anti-inflammatory drugs as prn medication.\nSince it was not possible for the patient to handle crutches with her fully casted right arm, she visited the nearest trauma ambulance 3 days after the accident. After denial of two orthopaedic surgeons to perform surgical stabilization of the radial and ulnar fracture, the patient was provided with an arm splint for better handling of the crutches. Owing to unsatisfying success of this option, our patient was advised to hobble. Additionally, she was referred to a physical therapist for remobilization of the injured arm. According to our patient, she was not aware of pain in the hip during this period, but stated that she might have been distracted by the subjectively more severely felt symptoms of her injured arm.\nA few weeks later, our patient consulted her general practitioner and presented with continuing moderate pain in the right hip. The general practitioner decided to request MRI sequences of the patient’s hip, which showed several trauma-related injuries. On the images (), the articulation of the femoral head within the acetabulum was congruent. The posterior and inferior muscles around the joint were altered with severe tissue oedema, visible as hyperintensity on T2-weighted images. The origin of the superior gemellus muscle was not visible, the muscle belly being retracted and thickened (). The obturator internus muscle tendon was surrounded by moderate effusion in the subjacent bursa (). The tendons of the inferior gemellus, the quadratus femoris and the external obturator muscles were normal; there was however severe tissue oedema in the muscle bellies of the quadratus femoris () and the external obturator muscles (). Piriformis, gluteal and tensor fasciae latae muscles were normal. No abnormalities of vessels or adjacent nerves were observed.\nAfter 19 months of conservative treatment post trauma, our patient was still complaining about mild hip pain. A second MRI was performed to exclude avascular necrosis of the femoral head. The follow-up MRI () showed an undulated shape of the tendon of the superior gemellus muscle (). Although the obturator muscle itself was without pathology in the first MRI, it now was partially ruptured (). There was some scarring around the quadratus femoris muscle (). Scarring of the femoral head was present (). Additionally, we detected hypertrophy of the piriformis muscle (), which was without pathological findings in the first MRI report.
In 2013, a 24-year-old healthy female presented to our clinic 4 months after sustaining a martial arts twisting injury to her left knee. Continued pain and mechanical symptoms despite activity rest and conservative measures prompted an MRI study by her primary care physician and subsequent referral to our care based on the imaging findings of an ACL tear. The patient reported persistent instability and locking of her knee occurring with all activities and had failed trials of bracing, NSAIDs, and icing. She was unable to return to sport or her desired level of activity, and she desired surgical intervention.\nSurgical intervention was recommended given her age and activity level. The patient elected to proceed with ACL reconstruction with allograft after review of the surgical procedure and available graft options. Preoperative physical exam demonstrated a positive Lachman exam. Maximum knee joint arthrometer testing (KT-2000 MEDmetric Corp, San Diego, CA) was 7 mm on the right and 13 mm on the left. The patient underwent arthroscopically assisted ACL reconstruction with a 9 mm tibialis anterior allograft. Fixation included a 10 × 28 mm Arthrex BioComposite interference screw distally with an ACL TightRope RT for femoral fixation using outside-in tunnel retrodrilling technique. Button passage and confirmation of flip onto the lateral femoral cortex was visualized via the anteromedial portal through the 3.5 mm lateral cortical tunnel. Intraoperative findings were significant for ACL tear only without additional pathology noted. The procedure completed without complication.\nRehab consisted of knee immobilization and nonweight-bearing precautions immediately postoperatively. Range of motion was initiated on postoperative day one. Weight-bearing was advanced to as tolerated on postoperative day one. Jogging was initiated at three months. She completed postoperative rehabilitation without issue and returned to her desired level of activity by six months. Maximum KT-2000 testing at 6 months was 8 mm on the right and 9 mm on the left. Biodex testing at this time demonstrated 13.9% extension and 17.3% flexion deficit at 180 degrees/second.\nThe patient had an unremarkable immediate postoperative course marred only by a few brief periods of activity-related knee pain resolving with conservative measures including a single pes anserine steroid injection one year postoperatively for pes bursitis.\nEighteen months postoperatively, the patient presented to the clinic due to acutely worsening anteromedial knee pain after being kicked in the leg during martial arts practice. Her pain was worst with knee extension, but she denied mechanical symptoms or instability. Physical exam at this time was unremarkable and radiographs () demonstrated no evidence of complication of her reconstruction as compared with other imaging during her postoperative course. These issues subsequently resolved with conservative management strategies.\nTwo years postoperatively, the patient returned for worsening posterior knee pain. She reported a pinching sensation in the back of her knee that was most prominent at full extension and while walking. She had already begun to use crutches for ambulation due to these symptoms at the time of her visit. Radiographs at this time demonstrated a migrated TightRope button (). Physical exam revealed no ligamentous laxity and was remarkable only for pinching pain throughout passive range of motion. An MRI was obtained, and the migrated hardware was again visualized in the posterior knee joint, along with an intact-appearing ACL graft.\nThe patient was counseled on arthroscopic exploration and removal of the migrated hardware. Diagnostic arthroscopy revealed a stable-appearing ACL graft with probing that appeared well vascularized (). The TightRope button was located in the posterolateral knee, under the posterior horn of the lateral meniscus (). There was no TightRope suture attached to the button, and the suture was not visualized in the knee. The button was retrieved uneventfully. Postoperatively, the patient's pain and motion limitations associated with the displaced button resolved.
We report the case of a 63-year-old male with a history of chronic pulmonary obstructive disease (COPD), atrial fibrillation, peripheral arterial vascular disease, and aortic regurgitation, due to an ascending aortic aneurysm and annular dilation for which a valve-sparing aortic root replacement (David procedure) had been performed 12 years previously. His medication included rivaroxaban, metoprolol, digoxin, furosemide, lisinopril, and salmeterol inhalers for his COPD. A complete overview of his medication at the time of presentation is listed in Table . One week prior to the current presentation he had been admitted to our emergency department (ED) with severe pain in his right arm. Upon admittance by an emergency physician there were no signs of fever. His pulse was irregular and the rate was within normal ranges. His blood pressure was 144/89 mmHg. Saturation was 97%. Auscultation of heart and lungs was not documented. Also, there was no documentation on the presence of endocarditis stigmata. Laboratory investigations showed elevated inflammatory parameters (see Table ). An electrocardiogram (ECG) was not performed. No blood cultures were taken. After a CT-scan had revealed a thrombus in his right brachial artery the care of the patient was swiftly transferred to a vascular surgeon. Surgical embolectomy was performed within short notice. Elevated inflammatory parameters were attributed to ischemia in his arm and he was discharged the next day. A few days after discharge, it was realized that a source for his peripheral embolus had not been identified and our cardiology department was contacted to exclude a cardiac origin of the thrombus. We observed two minor Dukes criteria, i.e. the presence of an aortic graft after a David procedure and an arterial embolus. Because the patient had already been discharged he was contacted for further analysis. Since he was already on rivaroxaban for persistent atrial fibrillation and he reported no further complaints (e.g. no dyspnea or fevers), it was decided to perform ambulatory transthoracic echocardiography, possibly followed by transesophageal echocardiography, within two to three days to exclude a cardiac origin for his arterial embolus, such as infective endocarditis, left ventricular or left atrial appendage thrombus.\nBefore these additional investigations were performed, the patient was re-admitted with sudden dyspnea. Physical examination showed desaturations down to 89%, but no fever. His blood pressure was 136/83 mmHg. Cardiac murmurs, pulmonary rales, and endocarditis stigmata were absent. Laboratory testing revealed elevated inflammatory markers and n-terminal-prohormone B-type natriuretic peptide (nt-ProBNP), and no signs of cardiac ischemia. Arterial blood gas analysis showed a respiratory acidosis and elevated lactate (see Table ). An ECG showed atrial fibrillation with a ventricular rate of 110 bpm with a known left bundle branch block. Chest radiography showed consolidative abnormalities and pulmonary venous congestion. With a working diagnosis of pneumosepsis, with concomitant exacerbation of his COPD with secondary decompensated heart failure the patient was admitted to the pulmonology ward for treatment with amoxicillin and steroids. Blood cultures were taken. Within 24 hours, his condition deteriorated. He experienced severe dyspnea and he desaturated again to 88% while he was on oxygen therapy. His blood pressure was 155/92. He was transferred to the intensive care unit (ICU) for intubation, where he was stabilized. The antibiotic treatment regimen was changed to cefotaxime, ciprofloxacin in addition to steroids and diuretics. Transoesophageal echocardiography (TEE) was performed the next day. TEE revealed a 3 mm vegetation at the non-coronary cusp of the aortic valve (AV), indicative of IE, and a new, eccentric AV-regurgitation jet towards the anterior mitral valve leaflet (AMVL) (Figure , Video ). At the impact site of this aortic regurgitation jet, the AMVL showed severe regurgitation through a perforated aneurysm (Figure , Video ). This same day, the blood cultures revealed growth of Streptococcus salivarius, after which the dosage of the cefotaxime was raised to 12,000 mg/24h. Emergency cardiac surgery was scheduled but before it was started the patient developed signs of thrombo-embolic neurological complications. A CT-scan confirmed multiple extensive cerebral infarctions. Due to a now extremely poor prognosis palliation was initiated. The patient deceased the next day.\nIn summary, we present the case of a patient that was initially admitted with a peripheral arterial embolism without a raised suspicion of IE, despite the presence of a predisposing heart condition. He was discharged after surgical embolectomy. Upon second presentation with congestive heart failure a ruptured AMVL with severe mitral regurgitation was revealed upon echocardiography. Before surgery could be performed the patient succumbed as a result of an embolic stroke.
A 4-year-old boy was presented to our unit on referral with a three-week history of high-grade fever and a five-day history of generalized abdominal pain and abdominal distension. There was an associated history of headaches and body weakness. He had no history of jaundice. He had several episodes of vomiting which was initially none bilious, but later became bilious. There was an associated history of passage of diarrhea stools two days before presentation, although the patient had not passed stools on the day of presentation. He had no history of passage of melena or of hematochezia. His parents complained that he had been passing scanty urine for about five days before presentation. He had been receiving medications from the referring hospital for about two weeks before presentation.\nAt presentation, he was chronically ill looking with a toxic facie, febrile (temperature 38.5°C), pale, dehydrated, anicteric, and had no pedal edema. His respiratory rate was 28/minute; he had reduced air entry on both lung bases posteriorly. His pulse rate was 128/minute, which was regular but of small volume. Only first and second heart sounds were heard. Abdominal examination showed a distended abdomen which did not move with respiration. He had generalized tenderness with guarding. The bowel sounds were absent. A digital rectal examination showed an empty rectum with a full and tender rectovesical pouch. An initial assessment of a generalized peritonitis was made. The patient was placed on NPO with nasogastric tube for gastric decompression. He was commenced on IV fluid resuscitation and broad spectrum IV antibiotics (ceftriaxone and metronidazole) and was worked up for surgery. Initial laboratory investigations showed a hematocrit of 24% and hypokalemia (2.5 mmol/l) and a serum urea of 10 mmol/l. After an initial fluid resuscitation and correction of serum potassium, he had 300 ml of whole blood transfused. He had an exploratory laparotomy on the second day of admission. At surgery, about 600 ml of a feculent peritoneal fluid was drained, and a single perforation on the antimesenteric border of the terminal ileum was closed in two layers. An intraoperative diagnosis of perforated typhoid enteritis was made. Blood and tissue cultures were not done. He was continued on IV fluids and the same empiric antibiotics after the surgery.\nOn the third day of postoperation, the abdominal wound had a purulent discharge from the distal end. The wound was opened and stitches were removed from the site of drainage, with the institution of daily wound dressing. By the fifth day of postsurgery, a dark patch was noticed to have developed in the skin over the right iliac fossa and measured about 5 cm in its widest diameter. At about the 7th day of postoperation, the dark dry patch had extended to the right hypochondrium, and a similar patch had appeared along the edges of the abdominal incision and the left iliac fossa. A diagnosis of anterior abdominal wall gangrene was made. On the 8th day of postsurgery, the patient was noticed to have developed a complete wound dehiscence () and a fecal fistula. He had fluid resuscitation and was taken back to the theatre on the 10th day after the first surgery, for wound exploration and debridement of the anterior abdominal wall gangrene. A new intestinal perforation was seen at about 2 cm from the initial perforation. The intestinal perforation was exteriorized as an ileostomy, and the abdominal wound closed with tension sutures. Postoperatively, the patient's condition remained unstable, with intractable shock. He died six days after the reexploration of septic shock.
A 37-year-old woman purposely came to the outpatient department of our district hospital in the North West Region of Cameroon for a refill of her propranolol medication. She reported taking the drug routinely for palpitations. On further inquiry, she reported being well until 12 months prior to presentation when she suddenly became aware of her heartbeat, initially on moderate exertion and then even at rest. It had been recurrent and lasting several minutes each time it occurred. It progressively became associated with dyspnoea initially on moderate exertion and then on mild exertion, such that she had to quit farming which was her main source of income. After consulting at various health facilities in the North West Region of Cameroon, she was prescribed irregular and alternate daily regimens of 40 mg propranolol and 0.25 mg digoxin which she had been taking for 12 months at the time she presented at our hospital. Although the medications conferred her some relieve, she noticed a progressive weight loss over the 12-months period prior to consulting at our hospital, from 85 to 58 kg despite an abnormal increase in her appetite for food. She also experienced frequent watery stools (averagely six times daily). About 6 months prior to presentation, she complained of heat intolerance and profuse sweating resulting in sleeping with minimal clothing. In addition, she noticed spontaneous resting tremors of her hands which started at the same time as the heat intolerance. Three months prior to presentation at our hospital, she noticed a painless lump on her neck that progressively increased in size.\nOn reviewing her medical records, we noticed a number of investigations requested at various hospitals which she visited during the 8 months preceding her presentation at our hospital. These included electrocardiographs, echocardiograms, HIV tests, full blood counts, fasting blood sugar, thyroid hormone assays, which were all without particularity but for the electrocardiography results which always showed a sinus tachycardia. She has no known history of exposure to radiations or family history of malignancies.\nPhysical examination revealed a chronically ill-looking middle aged woman. Her eyes were normal (Fig. ). Her voice was clear. She had fine resting tremors of her hands with her arms outstretched. There was a left anterolateral neck mass measuring 4 × 3 cm (Fig. ). The mass was rubbery, mobile, non-tender, moved with swallowing, and not fixed to overlying or underlying tissue. No bruit was heard over the mass. There was no palpable cervical lymphadenopathy. Her vital signs were normal but for regular respiratory and pulse rates of 35 breaths/min and 104 beats/min respectively. Her BMI was 20.1 kg/m2. There was discrete bilateral pedal pitting oedema. The rest of the physical examination was without notable findings. In view of this presentation, a presumptive diagnosis of HT was made and we placed her on propranolol, 40 mg twice daily. Our hospital was not equipped with the necessary diagnostic tools, so we referred the patient to a regional hospital which is about 10 km from our locality. To confirm our diagnosis, we requested for a functional [serum T3, T4, Thyroid stimulating hormone (TSH)] and structural (ultrasound) assessment of the thyroid. The patient was counter-referred to us with the following results:Functional assessment of the thyroid: levels of serum T3, T4, and TSH (Table ). Structural assessment of the thyroid (ultrasound scan): The left thyroid lobe appeared enlarged, heterogeneous, with a fairly iso-echoic, well-defined homogenous solid mass (3.6 × 1.8 × 2.9 cm in size). The right lobe was without particularity. No cervical lymphadenopathy was observed.\nIn view of these findings, we concluded on a primary HT most likely due to a toxic uninodular goiter. Again, due to the limited resources in our hospital, we referred the patient to a hospital which is over 30 km from our locality for initiation of a course of methimazole. She was placed on 60 mg methimazole daily, 4 weeks after which she underwent a left thyroid lobectomy. The resected lobe (Fig. ) was sent for histopathology. The postoperative course was uneventful.\nThe lobe weighed 33.5 g and was received in formalin as two grey-tan soft tissue fragments: a large (6.5 × 5 × 3.5 cm) and a small (3 × 1.5 × 1 cm) fragment. Cutting demonstrated a red tan cut surface with a 3 × 2.3 × 2 cm nodule inside the bigger piece. Microscopic examination of representative sections of the large fragment with a full thickness section of the nodular structure revealed a nodular proliferation of enlarged pale cells with marginated chromatin and overlapping nuclei. Pink “bubble gum”-like colloid was focally noted. The lesion was partially encapsulated and displayed areas with fibrosis and more follicular appearance of the aggregates. Based on these, a histological diagnosis of PTC (pT2N0M0) was made.\nWe monitored the patient through scheduled regular visits and referrals. Figure shows the Incision site on the anterior aspect of the neck 2 weeks after lobectomy. We observed a progressive decline in hyperthyroid symptoms and signs: the palpitations regressed as well as the diarrhoea and polyphagia; the heat intolerance regressed and 1 month postoperatively, her weight increased from 58 to 68 kg.
The patient was a 74-year-old African American female with a past medical history of hypertension, smoking, sarcoidosis, and fibromyalgia. She presented with a painful swelling in her left neck and the symptoms did not improve with antibiotics and oral steroids for 4 months. She was referred for head and neck surgical evaluation. Physical examination demonstrated a 2-cm mobile tender mass inferior to the left earlobe. The overlying skin was unremarkable. Facial movement was intact and no cervical lymphadenopathy was identified. Contrasted CT of the neck revealed a 2.5×1.6 cm oval-shaped, cystic mass with irregular borders within the left parotid gland. The mass demonstrated peripheral enhancement and central hypo-attenuation. The top differential diagnoses by imaging included malignancy and infection. A fine needle aspiration was performed at the outside hospital and inconclusive.\nThe patient was transferred to the Veterans Affairs San Diego Healthcare System for surgery, due to the suspicion of a salivary neoplasm with malignant potential. Left parotidectomy was performed, during which a relatively well-demarcated cystic tumor was encountered within the superficial lobe of the parotid gland. There was no clinical evidence of extra-parenchymal extension, and no difficulty was encountered in dissecting the tumor from the facial nerve branches. The tumor and surrounding superficial parotid lobe were removed en bloc. Several slightly enlarged lymph nodes from Level II were also biopsied for histologic examination. No frozen section examination was requested intraoperatively.\nMacroscopically, the tumor measured 2.8×1.5×1.2 cm in size with an ill-defined boundary. There were cystic and hemorrhagic changes within the tumor. The vast majority of the tumor were sampled and submitted in six tissue blocks, in addition to one block of the uninvolved parotid gland. Microscopically, the tumor had an infiltrative growth pattern with centrally cystic areas and focal necrosis (). The neoplastic cells were exclusively oncocytic, characterized by abundant eosinophilic granular cytoplasm, uniformly round nuclei, and variably-sized nucleoli (). There were rare mucocytes, comprising less than 1% of the neoplasm (). No discernible populations of squamoid (epidermoid), or intermediate cells were noted. A focus of perineural invasion was present as shown in . No mitotic figures or significant lymphoid infiltrates were identified within the neoplasm. The uninvolved salivary parenchyma showed scattered small foci of chronic inflammation and otherwise unremarkable morphology. There were two lymph nodes identified with no involvement by the tumor cells.\nThe infiltrative pattern suggested a malignant neoplasm and the differential diagnoses included oncocytic carcinoma and oncocytic variants of other salivary malignancies, including OMEC. Mucicarmine stain confirmed the presence of rare mucocytes (). p63 immunostaining (Clone: BC4A4, monoclonal, BioCare Medical, Concord, CA, USA) revealed moderate-to-strong nuclear positivity in the oncocytes with a diffuse staining pattern (). p63 were negative in mucocytes (not shown). FISH was performed to detect the MAML2 translocation using a break-apart probe flanking the MAML2 gene at 11q21 locus (Leica DM 6000B, Mayo Clinic, Rochester, MN, USA). The result was nuc ish (MAML2x2)(5MAML2 sep 3MAML2x1) [89/200], i.e. 44.5% of the cells were positive for the MAML2 translocation (). Thus, the overall findings supported the diagnosis of OMEC with exclusive oncocytic morphology. Because of necrosis and perineural invasion, this case was graded as intermediate grade according to Armed Forces Institute of Pathology (AFIP) method.\nBased on the pathologic diagnosis of an intermediate grade MEC and the presence of perineural invasion, the patient received adjuvant radiation therapy postoperatively with a total of 6600 centiGray (cGy) to the parotid bed and 5040 cGy to the ipsilateral neck via volumetric-modulated arc therapy. She remained disease-free 16 months after the surgery.
Our patient was a 56-year-old postmenopausal African American woman with no past medical history who was previously treated at an outside oncology clinic for breast masses until 2010, when we first saw her. Her family history was negative for breast or ovarian carcinoma. She had a negative smoking history and endorsed drinking one drink per week. Per reports obtained, she first presented in 2006 with left breast lesions located in the upper inner breast that were documented as complicated cystic masses within the 9 o’clock and 9:30 positions on the basis of ultrasound (US). Subsequent US core biopsy in both areas revealed intraductal papilloma (IDP), and the patient was referred for a surgical consultation. No additional documentation of clinical visits was available until 1 year later. That documentation was in the form of a core biopsy pathologic report documenting the patient’s history of IDP at 9:00 and 9:30 positions as well as intraductal papillomatosis of the breast. A core biopsy taken at that time was from the left breast (location not mentioned) as well as the left axilla. The patient’s left breast showed fibrosis of mammary stroma including intralobular stromal sclerosis as well as microcalcifications in the lobular lumens. An axillary core biopsy confirmed IDP of the breast with stromal hyalinization as well as lymph node tissue adjacent to the papilloma. One month later, she underwent lumpectomy, with pathology reporting a 7-mm intracystic papilloma within a lymph node that was completely excised, as well as an epidermal inclusion cyst. The pathologist noted that the tumor was located near the periphery of a lymph node, probably arising in ectopic breast tissue in the capsular region. Approximately 11 months later, she developed another left breast mass. This was excised after a US-guided core biopsy once again revealed a benign IDP. The patient was then lost to follow-up at the outside clinic. She presented to our clinic 2 years later for an evaluation of a new left breast lesion. A bilateral diagnostic mammogram revealed two masses in the left breast, which were not well visualized, owing to heterogeneously dense breast tissue. Diagnostic US revealed a solid superficial mass measuring 0.81 × 0.76 × 0.81 cm corresponding to palpable findings also seen at the 6 o’clock position (Fig. a). Additionally, the patient had a large, complex cystic mass measuring 7 cm at the 1 to 3 o’clock position abutting the pectoralis muscle (Fig. a). A core biopsy of the 6 o’clock lesion was recommended. A US-guided, vacuum-assisted core biopsy of the 6 o’clock mass revealed an intracystic papillary neoplasm. Per the report, the patient denied nipple discharge, dimpling, thickening, redness of the skin, swelling, or tenderness at the time. A few weeks later, she underwent left breast lumpectomy with pathology revealing a complex cystic mass with fibrocystic changes at 1 to 3 o’clock and intraductal papilloma at 6 o’clock. The patient missed her 6-month follow-up mammogram. She returned 8 months later for a bilateral diagnostic mammogram, which showed a new 2.5-cm mass in the deep central aspect of her left breast at the 12 o’clock position. US showed a cystic mass measuring 3 cm and containing an intracystic solid component measuring 1 × 1 × 2 cm. No axillary or supraclavicular adenopathy was noted on the basis of imaging or physical examination. Her surgical team decided on left breast excisional biopsy with preoperative mammogram guidewire localization. Pathology revealed a benign papilloma measuring 1 cm, focally extending into skeletal muscle in the area adjacent to the previous biopsy site, but with negative margins and no signs of atypia. On the patient’s 6-month follow-up surveillance diagnostic mammogram, another new 3-cm density was noted at the 12 o’clock position. This was most consistent with a benign cyst and was aspirated. She was again lost to follow-up for more than 2 years until July 2015, when she presented with a 2-month history of a slowly enlarging left breast mass in the same region as her previous papillomas. A bilateral diagnostic mammogram with US showed a large mass at the 12 o’clock position measuring 7 × 2.5 cm. Her physical examination revealed that there were two areas of concern: first, a mass measuring 7.5 × 6.3 cm in the 1 o’clock position, and second, an area of nodularity measuring 4.6 × 3.1 cm in the 11 o’clock position. One month later, computed tomography (CT) of the chest and magnetic resonance imaging of the breast revealed a predominantly cystic mass with a solid component extending into the chest wall and approaching the pleural space (Fig. ). These tests also revealed a suspicious internal mammary lymph node (Fig. a). A positron emission tomographic (PET)-CT scan showed a hypermetabolic nodule located in the pretracheal space (Fig. b) with a corresponding standardized uptake value (SUV) of 6.1 and multiple associated hypermetabolic internal mammary lymph nodes with the highest SUV of 6.0 and nodular hypermetabolic activity along the inferomedial aspect of the cystic mass (SUV, 2.7).\nHer case was discussed at the multidisciplinary breast tumor board, and the recommendation was to proceed with a biopsy of the left internal mammary lymph nodes. Core biopsy revealed a papillary neoplasm with benign morphology with immunostains positive for estrogen receptor (ER) at 99%, positive for progesterone receptor (PR) at 85%, HER2/neu 1+, and a Ki67 proliferation index of 6%. An independent external pathologist agreed with the finding of histologically benign papilloma. The patient sustained a biopsy-related internal mammary artery injury and as a result developed a hemothorax requiring video-assisted thoracoscopic surgery.\nUpon recovery from the hemothorax, the patient was referred to the medical oncology department of our hospital. Given the malignant behavior of her tumor, a recommendation of aggressive local control was made. She was started on endocrine therapy with palbociclib and letrozole as a neoadjuvant strategy. Repeat PET-CT following 4 months of combination antiestrogen therapy demonstrated near-complete resolution of metastatic internal mammary lymph nodes (white arrows in Fig. a) and reduced size and avidity of the paratracheal nodes (arrows in Fig. b). A physical examination did not show any significant changes in the size of the left breast mass. She went on to complete 6 months of neoadjuvant therapy. The primary lesion demonstrated minimal clinical response after 6 months of combination endocrine therapy, and then she underwent a left simple mastectomy and sentinel lymph node biopsy. Once again, the pathology revealed a 7.1-cm papillary neoplasm described as microscopically bland and mitotically inactive, with a retained ME layer. Several similar-appearing satellite papillomatous lesions were also seen within the skeletal muscle and deep adipose tissue. Margins and all five sentinel lymph nodes were negative (Figs. and ).\nGiven the extent of her local involvement and history of recurrent disease, she underwent adjuvant chest wall radiation for 6 weeks, followed by adjuvant endocrine therapy with letrozole. Six months after mastectomy, a repeat PET-CT scan showed no evidence of disease. She continues to undergo surveillance CT of the chest and mammography of her right breast. One year after her mastectomy, she underwent left breast reconstruction (Fig. d). She remained without evidence of disease 2.5 years after mastectomy and continued on endocrine therapy during that time.
A 42-year-old woman presented with a 3-month history of bloody discharge from the urethra and lower back pain. Physical examination performed showed no remarkable changes on admission. She did not show urinary dysfunction. Laboratory data of the blood were within normal limits. Serum levels of tumor markers were not evaluated. Cytological examination of the urine sediment was highly suggestive of adenocarcinoma. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a tumor (diameter, 47 mm), which involved the urethra entirely []. There were no remarkable changes in the gynecological system and no swelling was observed in the intra-abdominal and intrapelvic lymph nodes. Cystourethroscopy showed two diverticula fenestrated in the urethral wall. Whitish papillary and villous lesions were observed in both diverticula []. Specimens of biopsy and TUR histologically suggested CCA, but were indeterminate for malignancy. The patient underwent total cystourethrectomy and partial resection of the vaginal wall. Grossly, the tumor extended around the entire urethra and anterior vaginal wall. Final diagnosis of the resected tumor was CCA stage III pT3N0M0.\nThe Papanicolaou-stained specimens showed a small number of papillary or spherical clusters of atypical cells with many benign urothelial cells and squamous cells in the background [Figure and ]. A few neutrophils and lymphocytes were seen, but no necrotic debris was seen. The nuclei of the atypical cells showed an increase in the chromatin content with fine to granular pattern and irregular contours []; the nucleoli were prominent. Most of the atypical cells had a moderate amount of cytoplasm that was lightly stained light green; however, some atypical cells showed clear, abundant cytoplasm that formed spherical clusters resembling “mirror balls” []. These findings suggested a malignant tumor of the urinary system and favored adenocarcinoma.\nThe biopsy and TUR samples showed that the tumor comprised papillo-tubular lesions []. The epithelial cells covering the tumor were cuboidal and single layered, and some of these cells showed a “hobnail pattern.” Most of the cells had eosinophilic cytoplasm, except for a few that had clear cytoplasm. The cells with a clear cytoplasm were positive for periodic acid-Schiff reaction. The epithelial cells showed relatively mild cytological atypia and did not invade the stroma. Mitotic figures were observed at a frequency of 10/10 high-power fields. Necrotic debris was frequently observed in the lumen of the tubular structures. In immunohistochemistry, the tumor cells were positive for cytokeratin (CK) 7 [SP52, ready-to-use (RTU) prediluted, Ventana Medical Systems, Tucson, AZ, USA], epithelial membrane antigen (EMA) (E29, RTU prediluted, Nichirei Bioscience, Tokyo, Japan), and carbohydrate antigen (CA) 125 (M11, 1:25, Dako, Glostrup, Denmark), and were also focally positive for CD15 (80H5, RTU prediluted, Immunotech, Marseille, France)\n[]. The tumor cells were negative for CK 5/6 (D5/16 B4, 1:50, Dako), CK 20 (SP33, RTU prediluted, Ventana Medical Systems), carcinoembryonic antigen (12-140-10, RTU prediluted, Leica Microsystems, Milton Keynes, UK), thrombomodulin (1009, 1:50, Dako), uroplakin III (AU1, RTU prediluted, Nichirei Bioscience), prostate-specific antigen (polyclonal, Dako), calretinin (5A5, RTU prediluted, Leica Microsystems), estrogen receptor (6F11, 1:50, Leica Microsystems), and progesterone receptor (312, 1:50, Leica Microsystems). Histological findings suggested diagnosis of CCA, although definite diagnosis of the malignancy could not be elicited because of absence of stromal invasion in both biopsy and TUR samples. In the surgically resected specimens, clear atypical cells with papillo-tubular structure invaded almost all layers of the urethra and vaginal muscular layer. The final definite diagnosis was CCA.\nThe Ki-67 labeling index of these tumor cells was approximately 20% [] and approximately 5% of the tumor cells showed strong p53 positivity in the nucleus.
A 68-year-old Caucasian man with severe chronic thromboembolic pulmonary hypertension and progressive decline to New York Heart Association (NYHA) functional class IV was admitted to our intensive care unit (ICU) because of respiratory failure and need for mechanical ventilation. His medical history included repeated episodes of deep vein thrombosis and two episodes of pulmonary embolism despite adequate anticoagulant therapy. Upon completion of the pulmonary hypertension investigation, his pulmonary thromboembolic disease was considered non-operable and was treated with bosentan and inhaled iloprost. Following the initiation of mechanical ventilation he was switched to intravenous iloprost and three months later our patient demonstrated a significant hemodynamic improvement, allowing him to undergo PEA. Details on his hemodynamic status and management are described elsewhere [].\nDuring his hospitalization, our patient had two episodes of ICU-acquired microbial and fungal infections including a catheter related candidaemia due to Candida albicans and Staphylococcus epidermidis (isolated from the catheter tip of a left internal jugular central venous catheter, treated with caspofungin for 15 days and linezolid 600 mg twice a day for 14 days), and an episode of ventilator-associated pneumonia (VAP) due to Acinetobacter baumannii treated according to the detected antimicrobial susceptibilities with imipenem (1 g every six hours) and colistin (3 × 106U every eight hours).\nImmediately prior to PEA, our patient was given 1 g of vancomycin as surgical prophylaxis with the induction of anesthesia. During the first post-operative week he was febrile without an apparent site of infection and received antibiotic treatment according to the surveillance cultures. On the eighth post-operative day an exudate drained automatically from the surgical wound; cultures grown in Sabouraud dextrose agar revealed a fast-growing isolate with cottony texture identified as Aspergillus spp. Microscopically, a potassium hydroxide mount confirmed the presence of septate and hyaline, and dichotomously branched hyphae. Conidiophores were uncolored producing spherical to ellipsoidal accessory conidia, covering the entire surface of the biseriate species. Further subcultures in potato flake agar (PFA) were prepared in-house and incubated at 35°C, and identified the presence of Aspergillus flavus. The isolate grew rapidly, producing olive-green colonies. When examined by tape mounts, a slide culture prepared on PFA cultured isolate revealed globose vesicles up to 20 μm in diameter, a biseriate arrangement with metulae and phialides, and globose conidia (3.0 to 6.0 μm in diameter); all features similar to those produced in Sabouraud dextrose agar and characteristic for A. flavus. A computed tomography (CT) scan of his thorax revealed no specific signs of mediastinitis. Serum and bronchoalveolar-lavage galactomannan tests were not performed, as they were not available at the time. No specific signs of mediastinitis were seen either.\nA combination of liposomal amphotericin B at a dose of 3 mg/kg daily and voriconazole (6 mg/kg twice a day for the first day followed by 4 mg/kg twice a day thereafter) were added because of the severity of the infection, and surgical debridement of the wound was performed. Despite all the therapeutic measures, the results of culture tests remained positive for A. flavus (surgical wound necrotic tissue test results were positive for hyphae on direct microscopy after the addition of KOH) and our patient died on the 26th post-operative day because of severe septic shock. Our patient underwent a whole body autopsy. Samples from the sternum, lungs and myocardial tissue fixed in a 10% buffered formol solution were embedded in paraffin; sections 4 μm thick stained with hematoxylin and eosin, PAS and Grocott's silver impregnation stains confirmed the presence of hyphae with morphologic characters pertaining to the genus Aspergillus [] (Figures and ).\nA detailed investigation of all patients undergoing surgical operation during the same period was performed. A total of 50 environmental samples were collected from various sites in the operating room. Four plates were left open for one hour, one of them on the surgical table and the other at various sites on the floor, focusing on the air conditioning system vents. Various surfaces were also sampled with cotton-tip swabs. The filters of the air conditioning system in the surgical room were replaced and samples culture tested. The central supply rooms that housed equipment used in cardiac surgery operations were all inspected for visible signs of mold contamination or damage that might lead to mold growth. Routine scheduled maintenance and replacement protocols for equipment and air filters were reviewed.\nNone of the patients undergoing surgical operation during the same period developed infection of the surgical site due to Aspergillus spp. and the results of environmental samples tested 17 days later were negative. In two of the tested samples hyphomycetes classified as Cladodosporium spp. grew on the 17th day, however these were considered as laboratory contamination. These positive samples derived from a storage area in the surgical room. No further isolation of fungi was noted from the plates, which were kept for one month after the environmental sampling inoculation. Additionally, a subsequent investigation was performed on the ICU staff using nasal cultures for possible nasal colonization with Aspergillus spp., also giving negative culture test results.
A 45-year-old man came to our outpatient clinic because of blood pressure level abnormality (200/100 mm Hg), which had persisted for >30 years. The patient was found with abnormal blood pressure when he was 10 years old. Maximal blood pressure level at 260/110 mm Hg was recorded during the physical examination for several years. The patient had a smoking history of 20 years and no similar family medical history was reported, but suffers no other significant clinical symptoms. In recent weeks, he suffered dizziness and was treated with oral antihypertensive medications. The treatment regime was nifedipine (controlled release tablets 30 mg Bid), irbesartan and hydrochlorothiazide (tablets 150 mg qd), and irbesartan (150 mg qd). The curative effect is limited and his blood pressure remains at elevated level. On physical examination, the patient had a pulse rate of 68 bpm, a respiratory rate of 18 bmp, a body temperature of 36.7°C, and a body mass index of 23 kg/m2. The first medical evaluation in our hospital revealed a regular heart rate of 68 bpm and a grade 4/6 systolic murmur at the position of T1-T3 on back. The murmur conducted to neck. His blood pressure was measured as 183/92 mm Hg in the left arm and 175/95 mm Hg in the right arm, 96/64 mm Hg in the left ankle, and 101/60 mm Hg in the right ankle (left ankle brachial index: 0.54, right ankle brachial index: 0.56). It indicated a significant differential blood pressure between the upper and lower limbs. Echocardiography showed the left ventricle did not present severe abnormal dimensions and impaired systolic function, the descending aortic arch could not be observed, and aortic valve regurgitated moderately. Computed tomography angiogram (CTA) revealed a type A interrupted aortic arch with complete discontinuity of the aortic lumen distal to the origin of the left subclavian artery, and a massive aorta arch which included 2 full-fledged collaterals network ensuring blood circulation to distal aorta (Fig. ). Angiography showed absence of anatomical continuity between the ascending aorta distal to the left subclavian artery and the descending aorta. There was no patent ductus arteriosus (PDA) and associated endocardial fibroelastosis. The descending aorta was fed exclusively by massive systemic collateral circulation (Fig. ). Cardiac magnetic resonance (CMR) found nothing abnormal. Carotid artery ultrasonography indicated bilateral carotid plaques. Lower limbs (right) ultrasonography indicated plaques on the arterial wall. Based on the available evidences, we have concluded that the patient suffered from secondary hypertension due to isolated interrupted aortic arch (IAA). We had discussed the advantages and disadvantages of the surgery option with the patient, but he refused. Therefore, we had prescribed antihypertensive medications: nifedipine controlled release tablets (Adalat) 30 mg bid; irbesartan and hydrochlorothiazide tablets (Coaprovel) 150 mg qd; irbesartan (Aprovel) 150 mg qd; doxazosin mesylate extended release tablets (Cardura) 4 mg qn; spironolactone tablets (Antisterone) 20 mg qd; carvedilol tablets 5 mg bid.\nAfter 10-day medication, 24-hour ambulatory blood pressure monitoring showed a mean of 24-hour blood pressure at 129/68 mm Hg. The day and night mean blood pressure was 139/79 and 103/54 mm Hg, respectively (Fig. ). The mean office blood pressure fluctuated between 140 to 160 and 90 to 100 mm Hg. The significant discrepancy between ambulatory and office blood pressure should be attributed to the white coat effect.
A 50-year-old male was admitted to our hospital complaining of slowly progressing weakness. He stated that when he was 4 years old, his mother noticed that he was not to be able to run as fast as his peers. He was the first of three children of his non-consanguineous parents, and both his siblings and his parents were healthy. No other muscular symptoms were noted throughout his childhood. In the 2 years leading up to his evaluation, he noticed mild weakness in his legs and difficulty getting up from a squatting position. These symptoms gradually became more severe, until he presented at age 50 with proximal-to-distal weakness in the muscles of the lower limbs. He has never experienced cognitive or cardiologic problems.\nNeurological examination revealed mild proximal muscle weakness in the lower extremities and the toe extensor muscles (MRC grade 4). The Gower’s sign test was positive and his tendon reflexes remained present but weak. There was no evidence of calf hypertrophy, scapular winging, or facial, and ocular impairment. Laboratory testing revealed slightly elevated creatine kinase levels (2× normal). Electromyography abnormalities of myopathic changes were found in both the vastus medialis and tibialis muscles. Echocardiograph and electrocardiogram results were normal. A magnetic resonance imaging (MRI) of the patient’s muscles revealed a preferential fatty infiltration in the posterior compartment of the patient’s thigh, with marked sparing in the gracilis, sartorius, and quadriceps muscles (). There was more focal involvement in the gastrocnemius muscles, and the remaining muscle groups were mildly involved (). The upper limb muscles demonstrate minimal fatty infiltration and no distinct involvement pattern (-).\nPeripheral blood samples were obtained with informed consent from three members of the family. Genomic DNA was isolated from these samples according to standard techniques. All known protein-encoding regions, including a total of 19,396 genes, were captured and enriched. The enriched libraries were sequenced on an Illumina NovaSeq 6000 (Illumina, USA), which generated data covering 99.9% of the sequence at 300×. The sequencing reads were aligned on a reference human genome (hg19) using Burrows-Wheeler Aligner software. The Genome Analysis Toolkit (GATK) was used to detect single-nucleotide variants and indels of the reads. Whole exome sequencing was performed at Chigene Medical Research Centre (Beijing, China). It turned out that the patient had homozygous deletion of a 2kb region, chr9.hg19: g.119460021_119461983del, in exon 2 of the TRIM32 gene (). The presence of this homozygous deletion was confirmed with quantitative real-time polymerase chain reaction (). The patient’s parents were heterozygous for the mutation. This novel mutation was neither observed in 200 healthy Chinese controls, nor four public databases, including dbVar, ClinVar, the Database of Genomic Variants (), and the Leiden Open Variation Database ().\nAfter written informed consent was obtained from the patient, a gastrocnemius biopsy was conducted. Muscle biopsy revealed marked variation of the muscle fiber diameters, internalized nuclei, as well as necrotic and atrophic myofibers. Small and irregularly slit-shaped vacuoles containing basophilic material were observed throughout the sarcoplasm in scattered myofibers. The connective tissue of the muscle was moderately increased. However, no inflammatory cell infiltration was observed (). Immunohistochemical staining showed that anti-dystrophin-N, -C, -R proteins, anti-α-, β-, γ-, δ-sarcoglycan proteins, calpain-3, and dysferlin levels were normal.
The patient was a 21-year-old woman, with an unremarkable family medical history. She had first undergone treatment for GISTs associated with an incomplete-type Carney’s triad 11 years prior, at the age of 10 years.\nAt the time of her first admission and treatment, the patient had presented with black stools as her chief complaint. Endoscopic examination revealed the presence of two submucosal tumors in the pyloric antrum (Fig. ) []. Magnetic resonance imaging showed multiple nodular tumors presenting as low signals on the T1-weighted image and high signals on T2-weighted image of the gastric body to antrum. In addition, computed tomography showed pulmonary nodular shadows in the S1 region in the right lung and lingular region in the left lung (Fig. ) []. Based on these findings, the patient underwent open partial gastrectomy with Billroth-I reconstruction. Histopathological examination showed a positive result for c-kit and negative for S-100 protein; thus, we provided a diagnosis of wild-type GISTs. There was no vessel or lymphatic invasion, and MIB-1 labeling index was below 10%. The GISTs were diagnosed to be of high risk because the pulmonary tumors were considered to be metastatic. Two months after this gastric surgery, imatinib was initiated for the treatment of the pulmonary tumors. As the pulmonary tumors grew slowly, the type of TKI was changed from imatinib to a combination of sunitinib and regorafenib. This change did not lead to a positive tumor response. As the pulmonary tumors in the right upper lobe increased in size, a right upper lobectomy was performed, 9 years after the index gastric surgery. The tumor was uncoated and displaced the surrounding lung tissue (Fig. ) []. Histopathological examination of the resected tumors confirmed the diagnosis of pulmonary chondroma. There was no vessel or lymphatic invasion. Based on the pathological result of pulmonary chondroma, a further histopathological re-evaluation of the resected gastric specimen was conducted that revealed a succinate dehydrogenase (SDH) deficiency of the tumors. Based on these findings, a diagnosis of an incomplete-type Carney’s triad was provided. The patient was followed up with yearly ultrasound examinations, with no indication of recurrent tumors in her stomach over the short-term follow-up.\nHowever, 2 years after her right upper lobectomy, at the age of 21 years, new gastric tumors were observed with ultrasound examination. Endoscopic examination of the upper gastrointestinal tract revealed the presence of multiple submucosal tumors on the residual side of the lesser curvature (Fig. a, b). Biopsy confirmed a pathological diagnosis of GISTs, with SDH deficiency. On computed tomography imaging, multiple masses were observed in the whole stomach, growing into the inner cavity. There was no obvious disseminated involvement nor metastatic lesions (Fig. ). The patient was referred to our center for further assessment and treatment.\nThere was no evidence of extra-adrenal paraganglioma. A clinical diagnosis of recurrent GISTs, as a component of an incomplete-type Carney’s triad, was made. We decided to proceed with laparoscopic examination and treatment, as appropriate. On laparoscopic examination, further multiple nodules were observed on the serous surface of her stomach, with mild adhesion around the gastroduodenal anastomosis that could be easily divided (Fig. a). Based on these findings, we proceeded with completion gastrectomy, with D1 plus lymph node dissection and Roux-Y reconstruction, performed laparoscopically (Fig. b). Macroscopic examination revealed that the sporadic gastric stromal tumorlets were multifocal subserosal polypoid nodules (n = 8), with the largest being 52 × 30 × 25 mm in size (Fig. ). The tumor cells showed an epithelioid pattern, and all eight tumors were diffusely immunoreactive on CD117 and CD34 antibody assay (Fig. a–c). Vessel or lymphatic invasion was not observed. The Ki-67 index was 10%. Tumors were SDH deficient, confirming the diagnosis of GISTs as a component of an incomplete-type Carney’s triad. We did not observe any benefit of chemotherapy on the harvested tumor specimen.\nThere was no complication after surgery, and the patient’s postoperative course was uneventful. She was discharged from the hospital in good condition and has been monitored carefully in follow-up, with no adjuvant chemotherapy treatment. Over the 7 months since the gastrectomy procedure, she has had no difficulty with oral intake, and there is no evidence of tumor recurrence.
A 52-year-old woman was referred to our hospital because of severe dyspnea and the presence of a neck tumor. She had experienced dyspnea for the past 6 months and had recently noticed bloody sputum. A computed tomography examination of the neck revealed a thyroid tumor about 8 cm in size. The tumor had invaded the trachea, obstructing 90% of the tracheal lumen (fig. ). An emergency tracheotomy was performed to maintain an airway through the tumor tissue. On biopsy, the tumor was diagnosed as CASTLE, suggesting a favorable prognosis if a complete resection could be performed. Magnetic resonance imaging and an esophageal fibroscopy examination revealed a low possibility of esophageal invasion (fig. ). Under general anesthesia, a thyroidectomy was initially performed via a neck incision. The invaded portion of the trachea was sharply dissected. A partial sternotomy was performed at the level of the 2nd intercostal space to identify the distal portion of the intact trachea and to mobilize the inferior tracheal segment. The trachea was transversely opened to find the intact portion. The length of the resected trachea was 6 cm (fig. ). Bilateral recurrent nerves were macroscopically preserved. We performed a suprahyoid release to mobilize the superior tracheal segment, but these mobilizations were insufficient to perform an anastomosis. Thus, to allow additional mobilization, we performed a clamshell thoracotomy and incised the pericardium 360° around the hilum, dissecting the pulmonary ligament bilaterally. These procedures allowed an anastomosis. An end-to-end bronchial anastomosis was carried out using running 4-0 PDS II sutures (Ethicon, Somerville, N.J., USA) for the membranous portion and interrupted 4-0 PDS II sutures for the cartilaginous portion. The bronchial anastomosis was wrapped with an omental pedicle flap that was lifted up through a retrosternal route via a laparotomy. A gastrostomy was also performed for postoperative enteral nutrition. For management after surgery, a chin suture was placed. Neck mobilization was restricted for the placement of the chin suture and airway fixation. After confirming an improvement in the laryngeal edema using a laryngoscopy, extubation was performed on postoperative day (POD) 17. The patient exhibited swallowing disturbances and underwent swallowing rehabilitation. Enteral nutrition was provided through the gastrostomy. The patient was discharged on POD 31, and per os feeding was started on POD 51. Radiotherapy (60 Gy in 40 daily fractions of 1.5 Gy) was performed as an adjuvant therapy. Throughout the clinical course, no major complications, including swallowing disturbances, hoarseness or anastomotic problems, were observed. A histopathological examination of the resected specimen revealed the characteristic morphology of CASTLE (fig. ); immunohistochemistry was positive for CD5 (fig. ) but negative for thyroid transcription factor 1 (TTF-1; fig. ).\nEleven months after surgery, lower back pain and right leg numbness developed and led to gait inability. Multiple lung and bone recurrences were observed, but no local recurrence. In order to ease the back pain, palliative radiotherapy (35 Gy in 14 fractions) to the bone metastasis was administered. Twelve months after surgery, drainage of pleural effusion was performed. She died of pleural metastasis 14 months after the initial diagnosis of CASTLE.
A 45-year-old male from the Netherlands presented with a painless right parotid swelling that was progressively increasing in size for the past 8 months. Though occasionally he suffered from jaw lock, other symptoms associated with neurologic deficit such as drooling, facial weakness, paresthesia, or auditory defects were absent. Apart from being a social alcohol consumer, there was no history of smoking, prior radiation, or significant family medical history, especially in regard to his present illness.\nPhysical examination revealed a tender right parotid swelling below the ear lobule, which extended inferiorly to the angle of the mandible (). The skin overlying the swelling was slightly erythematous, thickened, and nodular. The swelling was firm, diffused, and fixed to the underlying muscles, and there was no associated lymphadenopathy. Otoscopic examination of both ears was within normal limits.\nThe patient initially had a neck ultrasound and then a magnetic resonance imaging (MRI) to characterize the nature of the lesion. The neck CT scan revealed a well-defined altered signal enhancing mass measuring 3.5 × 2.2 × 2.0 cm at the posterior aspect of the superficial part of the right parotid gland (). The radiologist's impression was an altered signal enhancing mass lesion, likely to be a benign pleomorphic adenoma. The patient then underwent MRI of the parotid glands, which showed a well-defined focal lesion of altered signal intensity at the posterior aspect of the superficial part of the right parotid gland, measuring 3.5 × 2.2 × 2.0 cm along its maximum transverse, craniocaudal, and anteroposterior diameters, respectively. The impression was again benign pleomorphic adenoma of the right parotid gland. However, the radiologist could not exclude other diagnostic possibilities and recommend fine-needle aspiration cytology.\nFine-needle aspiration cytology of the mass was nonconclusive as the smears only showed polymorphous population of lymphoid cells in keeping with intraparotid lymph node. In view of these clinical findings, a superficial parotidectomy with facial nerve monitoring and preservation was planned.\nA modified Blair incision was done, with elevation of a skin flap and control of hemostasis during the surgery. The dissection was rather difficult! The superficial parotidectomy was done in piecemeal as the mass was unexpectedly adherent to the skin and underlying fascia (). The facial nerve was intact and was examined branch by branch. No frozen section was done during the operation.\nHistopathologic examination showed a widely infiltrative tumor involving the parotid parenchyma and extending into the surrounding adipose tissue and skeletal muscles (). The tumor was predominantly composed of bland spindle cells with wavy nuclear contours, embedded within a fibrillary, pale pink matrix, which resembled a neurofibroma (). Of concern, there were evidently distinct scattered more cellular foci of epithelioid tumor cells with nuclear atypia and increased mitotic activity (). The tumor was diffusely and intensely positive for the S100 protein and neuron-specific enolase (NSE) immunohistochemical stains, with a low Ki-67 proliferation index. In addition, the CD34 immunohistochemical stain was focally positive in tumor cells. On the other hand, the tumor lacked immunoreactivity for AE1/AE3, p63, EMA, Melan-A, and HMB-45 immunohistochemical stains, thus excluding carcinomas, clear-cell sarcoma, melanoma, and other salivary gland tumors. Based on morphologic and immunophenotypic features of the tumor, the diagnosis rendered was low-grade malignant peripheral nerve sheath tumor (MPNST) arising in a diffuse neurofibroma.\nGiven the rare presentation and the association of such tumors with NF1, the patient was evaluated clinically for features of the syndrome. Multiple café au lait macules were subsequently discovered on his trunk (). The patient was referred to a cancer center for further management and follow-up.\nFurther clinical examination of the patient and MRI revealed no distant metastasis. The patient underwent several courses of radiotherapy and is currently eighteen months' disease free.
A 65-year-old Asian woman was admitted with exertional dyspnea. She had a medical history of hypertension, hyperlipidemia, and coronary artery disease that had required transcatheter intervention. Her family history also included coronary artery disease. Her social history was unremarkable with respect to environmental and workplace exposures, and she did not smoke or consume alcohol. A physical examination upon admission revealed no signs of cardiac congestion or neurological deficits. Her vital signs were a systemic blood pressure of 143/86 mmHg, pulse rate of 89 beats per minute, and a body temperature of 35.9 °C. Chest x-rays at the time of admission showed cardiomegaly with protrusion of the right first aortic arch, and electrocardiography showed an incomplete right bundle branch block of normal sinus rhythm. Coronary angiography performed because of her history of coronary artery disease revealed no significant coronary arterial stenosis. However, a concurrent pressure study revealed oxygen step-up in gas sampling between the SVC and right atrium (RA) with a pulmonary blood flow/systemic blood flow ratio (Qp/Qs) of 2.07, even though pulmonary artery pressure was normal in the pressure study and the atrial septum was intact on echocardiography. Contrast-enhanced computed tomography (CT) revealed that the RUPV flowed into the SVC (Fig. ) without any other congenital heart conditions, including an ASD and a dilated RA and right ventricle (RV). Isolated PAPVR was diagnosed on the basis of CT imaging findings, and surgery was indicated on the basis of the patient’s symptoms and the hemodynamic pressure findings.\nThe surgical approach was via a median sternotomy. The SVC was cannulated near the confluence of the innominate vein, sufficiently above the RUPV. A cardiopulmonary bypass was established, and cardiac arrest was applied. The RA was incised longitudinally. The intact atrial septum was incised in the cranial direction from the superior aspect of the fossa ovalis, and this new ASD formed a hole with an approximate diameter of 15 mm after the right and left atrial endocardia were closed using continuous 5-0 polypropylene sutures (Fig. a). An intra-atrial baffle comprising an autologous pericardial patch was sutured from the inferior border of the new ASD to the cavoatrial junction to separate the cavity between the RA and the SVC (Fig. b). The SVC was divided above the confluence of the RUPV, and the cardiac side of the SVC stump was sutured closed. A new RUPV outflow route to the left atrium (LA) was subsequently constructed through the surgically created ASD. A cavoatrial pathway between the cranial side of the SVC stump and the right atrial appendage (RAA) was reconstructed using a ringed extended polytetrafluoroethylene (EPTFE) prosthesis with a diameter of 16 mm.\nThe patient tolerated all procedures well and was discharged with normal sinus rhythm after recovery from temporary sinus bradycardia. Anticoagulation therapy with oral warfarin was postoperatively prescribed for 3 months, which is standard procedure after bioprosthetic valve replacement. Postoperative echocardiography showed flow from RUPV to the LA through the new ASD without a significant pressure gradient or congestion. Postoperative contrast-enhanced CT showed that the RUPV flowed into the LA through the new pathway without intracardiovascular thrombus (Fig. ). The patient remains free of dyspnea, arrhythmia, and thrombotic events, and echocardiography at 4-year follow-up did not reveal evidence of stenosis or obstruction of the reconstructed pathway.
The patient is a 62-year-old right-handed woman with a past medical history of significant tobacco use with chronic obstructive pulmonary disorder, hyperlipidemia, and prior remote meningioma resection at 42 years of age with residual seizures on levetiracetam. She was diagnosed with stage IIB pT1aN1 left lung adenocarcinoma at 51 years of age and underwent left upper lobectomy with mediastinal lymph node dissection followed by adjuvant chemotherapy. Six months after her initial diagnosis, she was found to have brain metastases and underwent four Gamma Knife radiosurgeries to a total of 17 lesions over the course of 12 months. She did well over the next five years but then had progression of a right posterior temporal mass that was in the immediate area of a small lesion previously treated with Gamma Knife. She underwent a right-sided craniotomy and tumor resection, followed by adjuvant stereotactic body radiotherapy (SBRT) with 30 Gy in five fractions to the resection cavity. Two years later, she developed seizures and headaches, and she was found to have a growing focus of enhancement seen using MRI at the site of her previous surgery consistent with disease recurrence. Four months after this finding, she was taken back to the operating room for a redo right craniotomy for tumor resection.\nThe patient unfortunately once again developed local recurrence at the surgical site seen on fluorodeoxyglucose (FDG) positron emission tomography (PET) and gadolinium-enhanced T1 weighted MRI four months post operation. She was initially considered for laser interstitial thermal therapy (LITT) but was thought to have a tumor configuration and morphology that is not amenable for treatment using this modality. After multi-disciplinary tumor board discussion, she was ultimately recommended repeat resection and GT. Accordingly, the patient underwent a third right craniotomy with tumor resection and placement of GTs in the tumor bed, and titanium mesh cranioplasty overlying the surgical site. The patient tolerated the procedure well.\nA target was contoured on the preoperative MRI by the radiation oncologist. The prescription was to treat 60 Gy to 5 mm beyond the surgical cavity. The number of tiles needed was estimated by evaluating the circumference of the target in the axial, coronal, and sagittal views. Five tiles were estimated by a team consisting of the neurosurgeon, radiation oncologist, and medical physicist for the procedure. An extra sixth tile was ordered to account for any changes in the cavity on the day of the surgery. Three full and three half tiles were implanted, resulting in a total of 18 seeds with 3.51 U (5.51 mCi) per seed.\nThe patient had a thin slice (1 mm) CT of the head on postoperative day zero, which was unremarkable (i.e., no acute hemorrhage); MRI on postoperative day two showed a near-total resection. She also had a two-week follow-up wound check and was doing well at that time. The CT and MRI were fused, the postoperative cavity was contoured, and the 131Cs seeds were identified. A 5-mm expansion from the cavity (excluding bone) was used as a clinical target volume (CTV). The postoperative cavity volume was 14.36 cm3. Figure shows preoperative and postoperative MRI with cavity contours.\nTo model the GT dosimetry on a phantom CT, we created a spherical cap and a cube with the same volume as the postoperative cavity, expanded 5 mm (in all directions except for one flat surface), and acquired the CTV. Four and a half tiles (18 seeds in total) were each modeled in the spherical cap and the cube according to the tile configuration. The seeds were 3 mm from the cavity wall and 1 cm from each other. Figure shows the three-dimensional representation of the 18 seeds in the postoperative cavity for the patient and for the cube and spherical cap models.\nPostoperative plans for the patient and the two models were planned in MIM Symphony™ (MIM Software Inc., Cleveland, Ohio). Dose volume histograms (DVH) were compared for all three plans (Figure ). The cube model resembled the patient DVH better than the spherical cap model. Table shows the dosimetry parameters of the patient and the two models. Volume of the contours, mean dose, CTV volume receiving the prescription dose (V60Gy), and dose to 90% of the CTV (D90%) are reported.
A previously healthy 11-month-old girl presented with failure to thrive and dyspnoea secondary to cardiac dysfunction. She was found to be hypertensive. Blood pressure measurements demonstrated a persistent systolic pressure over 180 mm Hg. She was initiated on antihypertensive and diuretic medication. There was no family history of significance and no stigmata of autoimmune disease or neurofibromatosis. There was no syndromic phenotype. Biochemistry revealed marginally renal function (creatinine 25 mmol/L), but urinalysis was normal. Vasculitic screen was negative. Echocardiogram revealed left ventricular dysfunction and hypertrophy. Renal Doppler ultrasound demonstrated a reduced blood flow through a comparatively smaller right kidney (1 SD below mean) with abnormal spectral trace in the right renal artery. Divided renal function was estimated at 30% on the right and 70% on the left. No dysplastic changes were seen. Dimercaptosuccinic acid (DMSA) demonstrated a small right kidney and a divided renal function of 6% on the right and 94% on the left.\nAt this time, the patient was referred to our tertiary centre for a review at a multidisciplinary team (MDT) meeting to consider angioplasty versus nephrectomy as a treatment. The tests had not at this point identified any pathology in the left kidney (which DMSA had demonstrated to contribute to 94% of renal function), renal function was only slightly deranged, and bilateral RAS was thought to be usual. The decision was made to perform a right nephrectomy in the belief that the poorly functioning right kidney, and associated reduced renal perfusion, was driving a compensatory hypertensive response via the renin-angiotensin system. This was confirmed on biochemical assay of renin and aldosterone levels. At this point, she was taking 5 different classes of antihypertensive medication and the DMSA had shown only 6% function from the right kidney.\nThe nephrectomy was performed and the resected right kidney demonstrated no histological abnormality such as fibromuscular dysplasia (FMD). There was compensatory hypertrophy of the left kidney on follow-up ultrasound (>2 SDs above the mean), but the hypertension persisted and the marginally impaired renal function did not improve (thought due, in part, to the Angiotensin converting enzyme (ACE) inhibitor). Spectral analysis demonstrated abnormal waveforms suggestive of RAS of the solitary left kidney. Following further MDT meeting discussion, it was decided to attempt angioplasty of the left renal artery.\nPercutaneous transluminal angioplasty was performed via a 4Fr vascular sheath in the right common femoral artery. Angiogram demonstrated no aortic coarctation but a tight ostial stenosis of the left renal artery () suggestive of possible unifocal FMD. The stenosis was crossed and angioplastied with both 2 and 3 mm conventional balloons (Sterling, Boston Scientific, Massachusetts, United States), but there was failure to relieve the stenosis () despite high inflation pressures. As the use of a cutting balloon and its inherent risk had not been discussed or consented for the decision was made to stop and the use of a cutting balloon discussed in the paediatric urology meeting. A second attempt was scheduled for 3 months later. The MDT decision was to have a renal transplant surgeon available during PTA so that, should a complication, such as dissection, arise with loss of the solitary kidney, then the kidney could be auto transplanted and re-anastomosed in the pelvis. Using a 6Fr sheath, an over-the-wire 3 mm × 15 mm cutting balloon (Boston Scientific) was used over a 0.014 wire () to relieve the stenosis. The stenosis was post dilated with 3.5 mm × 20 mm balloon with excellent angiographic results and release of the stenosis (). There were no immediate complications.\nDuring the month following cutting balloon angioplasty, there was an improvement in blood pressure and antihypertensive medications were slowly weaned off. Three months following treatment, she had stopped 2 antihypertensives and 1 was reduced, leaving her taking 2 antihypertensives. The parents reported increased wet nappies, thought perhaps due to increased renal perfusion. Echocardiogram revealed improved left ventricular function. In addition, the biochemical profile demonstrated an improvement in renal function with a reduction in creatinine. At 1 year of follow-up, she had stopped all antihypertensives and remained with stable good blood pressure and normalised end-organ function.
A 47-year-old patient with an enormous uterine leiomyoma reaching beyond the navel and up to the costal arch was admitted. During the 14 years since its detection, because of the patient's extreme fear of an abdominal incision, the myoma was merely monitored and all suggested laparotomies thus far had been refused. At the moment of admission, the patient only agreed to a minimally invasive surgery. She was informed in detail about all risks, side effects, and alternatives as well as the potential risk for an emergency open abdominal surgery. Before surgery, we performed imaging diagnostics by means of computed tomography (CT) of the abdomen ().\nWhen performing a hysterectomy of a very large uterus (>2500 g), the anatomical changes in the abdomen caused by the size of the uterus need to be taken into account. The large uterus divides the abdominal area, and only 3 narrow spaces are left to manipulate surgical instruments: between the left uterine wall and left abdominal wall, between the right uterine wall and right abdominal wall, and between the fundus uteri and liver and diaphragm. Successful surgery is only feasible when both instruments (forceps and coagulator or scissors) are in the same space simultaneously. Hence, we performed LASH with the “changeover technique” as described previously []. As such, we inserted 6 trocars including 3 on the left: one trocar in the lower, one in the middle, and one in the upper abdomen, allowing access to the left narrow space ().\nThe 3 trocars on the right side were placed in a mirror-like fashion. Surgery was initiated at the patient's left side, using the upper left trocar to introduce the camera () and the other two left-located trocars to introduce forceps and coagulator or scissors.\nThe patient was slightly tilted to the opposite side to facilitate visualization and preparation of structures. For uterus manipulation and movement, we used blunt forceps and palpation probes. After parameterization and resection of the uterine adnexa on the patient's left side, the plica vesicouterina was exposed and the bladder was pushed caudally.\nNext, the surgeon and his team switched sides positioning to the right side of the patient, thus accessing the narrow space on the right side in between the right uterine wall and right abdominal wall (). Again, the patient was slightly tilted to the opposite side, enabling better exposure and preparation of organ structures.\nThe procedure continued analogically as on the left side. As was seen on the CT scan, multinodal, intraligamentary, and parametric myomas were found on the right side. Consequently, further exposure and protection of the ureter was done by visualizing followed by preparing or abscising the right adnexa, parametria, and blood vessels ().\nThe corpus uteri were removed from the abdominal cavity by power morcellation. The entire procedure lasted 4 hours and 53 minutes, of which surgery on the uterus took 2 hours and morcellation 2 hours and 53 minutes. No postoperative complications occurred, and the patient was taken to outpatient care 2 days after surgery. Before discharge, a vaginal and renal ultrasound did not reveal any conspicuous intra-abdominal findings. Because of the young age of our patient, a very slow growth of the leiomyoma (over 14 years), and negative Doppler test results before surgery, we assumed no presence of malignancy. Histological analysis of the removed specimen confirmed this assumption. Furthermore, to prevent morcellation-induced spreading of occult malignancy, the patient was alternately tilted from a head-down to head-up position, after which we extensively rinsed the abdominal cavity with Ringer's lactate solution, a routine postsurgical measure at our facility. The total weight of the uterus was 4065 g (Institute for Pathology PPO Berlin; Mona Tawfik, M.D.; 06.05.2014).
A 47-year-old previously healthy Sinhala female's right foot was bitten by a snake near the back door of her home in the Kegalle district, Sri Lanka. Within seconds, she felt burning pain ascending along that limb, and there was heavy bleeding from the site of bite. Within a couple of minutes, she felt dizziness, nausea, and numbness of the whole body, had profuse sweating and frothy salivation, and was screaming in pain from the site of bite. On the way to the nearby hospital, she started to clench her jaw tightly and limbs became rigid; she was frothing and was not responding for about 5 minutes, indicating a generalized seizure. She arrived at the hospital within 30 minutes. The doctor at the outpatient department decided to administer ASV and directed the patient to an internal medicine ward for that. Physical examination findings at the ward were a pulse rate of 100/minute and blood pressure of 150/90 mmHg, and lungs were clear to auscultation bilaterally with an arterial oxygen saturation of 95% whilst breathing air with no neurological deficit. By this time, the killed snake was brought in and doctors identified it as a HNV; thus, antisnake venom (ASV) was not administered. Even though there was bleeding at the site of the bite even on admission to the hospital, her 20-minute whole blood clotting time, platelet count, prothrombin time and international normalized ratio, and activated partial thromboplastin time and liver function tests were all normal. Urine sample obtained via the catheter showed 50–55 red cells per high-power field, arterial blood gases indicated a compensated metabolic acidosis, and serum sodium and potassium levels were normal. Her urine output was <100 ml for the first 24 hours and serum creatinine rose from 80 μmol/l to 277 μmol/l. She was transferred to the Teaching Hospital, Kandy, on day 2 for further management.\nOn day 2, a bulla developed at the site of the bite, and there was an edema and warmth at the right foot. Complete (full) blood count demonstrated neutrophilic leucocytosis, and the CRP level of the following day was 261 mg/l. Intravenous antibiotics was started to cover the wound infection. Serum creatinine was 377 μmol/l with oliguria on day 2. Serum sodium and potassium levels remained within the normal range from day 1–5. On the day 5, creatine kinase was 75.1 U/l. Regular hemodialysis every other day from day 2 to day 24 and fluid management were started. Oral sodium bicarbonate was started, and management of her acute kidney injury with collaboration of nephrology team continued.\nOn day 3, her blood pressure rose to 160/90 mmHg, and it was controlled by prazosin and nifedipine SR; however, it generally remained on or above 140/90 mmHg until her discharge. She developed bilateral lung crepitations on day 3 that remained for 7 days. She developed bilateral parotid swelling and edema of the right leg on day 3, and it lasted 3 days. Edema below her right knee persisted another 10 days. Her blood picture on day 2 did not show hemolysis and was suggestive of bacterial infection but blood picture on day 5 showed evidence of microangiopathic hemolytic anemia (MAHA), and same changes were there in a blood film taken on day 11, as depicted in .\nHer day 2 hemoglobin level of 10.8 g/dl dropped to 8.4 g/dl on day 5. On day 2, her platelet count was 104 × 109/l and that dropped to nadir of 29 × 109/l in day 6 and was <150 × 109/l until day 20. A consultant in transfusion medicine has assessed her, and blood transfusion and plasmapheresis was performed on day 7. Another four cycles of plasmapheresis followed. Local edema at the site of the bite increased with necrosis (); thus, wound debridement was done on day 7 and followed up by regular wound toilets.\nWe did an electroencephalogram (EEG) on this patient on the earliest available day (day 11) and that was normal. The 2D echocardiogram done on day 17 was also normal.\nThe offending snake's carcass was taken to the Peradeniya University, and an expert on HNV, Dr. Kalana Maduwage, has confirmed it as a Hypnale hypnale. is a photo of the offending snake.\nAs her daily urine output improved to >1000 ml, she was discharged on day 30 and asked to come for a review in five days. She defaulted treatment and was on alternative medication. After developing progressive bilateral ankle edema and exertional dyspnea, she came back again on day 46, and hemodialysis and supportive therapy were restarted at the nephrology unit. On day 49, she had an anterolateral non-ST-elevation myocardial infarction (non-STEMI), and she was managed at the cardiology unit. She had progressive impaired vision of the left eye starting from a few days after the snakebite and could not count fingers held 30 cm in front of that eye on the 46th day. She was referred to the eye unit, there was bilateral optic disc edema more on the left, the patient was diagnosed of left anterior ischemic optic neuropathy (AION), and steroid therapy was started. Her erythrocyte sedimentation rate and contrast-enhanced computed tomography (CECT) brain done on day 53 were normal. is a photograph of fundi of this patient.\nShe had two episodes of seizures on day 76, and we suspected a possible relationship to her envenomation. The opinion of the neurology team regarding three seizures was obtained. Repeated EEG and CECT brain were normal. Despite being on calcium carbonate 500 mg plus 0.25 μg 1-alpha-hydroxycholecalciferol daily from day 46, her serum calcium level was low (1.8 mmol/l). Last two seizures were attributed to hypocalcemia due to chronic kidney disease following HNV envenomation, and daily calcium carbonate dose was increased to 500 mg thrice daily. After three months, she was diagnosed of end-stage renal disease by nephrology team and on hemodialysis once in four days and was searching for a kidney donor at six months.
A 17-year-old right-hand-dominant male tennis player visited our hospital complaining of pain in the anterior aspect of the right shoulder. Four years before his visit, he had experienced sudden acute pain in the anterior aspect of the right shoulder while executing a forehand stroke. Before the injury, the patient, who was a member of a tennis club at his junior high school and a private tennis club, allowed himself only 1 day of rest each month. Despite the pain, the subject continued to participate in competitive tennis for 4 years and experienced the pain especially during the follow-through phase. The pain increased gradually. One month before his visit to our hospital, he was examined by his family doctor, who advised him to rest his shoulder. However, the pain in his right shoulder persisted even while performing daily activities. Hence, he was then referred to our hospital.\nPhysical examination revealed tenderness at the lesser tuberosity, with exacerbation of pain by horizontal adduction and/or by internal rotation during the elevation of the humerus. Clinical evaluation of the rotator cuff muscles revealed full muscle strength of the supraspinatus and external rotator muscles. The result of the lift-off test was negative for the subscapularis tendon. The range of motion of the right and left shoulders did not exhibit any differences in forward elevation and external rotation. The range of motion in internal rotation with the arm at the side, internal and external rotations with the arm abducted at 90 degrees, internal rotation with the arm at a 90 degrees flexion, and horizontal flexion were 60, 50, 100, 20, and 100 degrees, respectively. The patient showed negative results for joint laxity test. The results of joint instability tests, such as anterior apprehension and posterior jerk tests, were negative. The results of forced horizontal flexion test was positive. Moreover, the preoperative University of California, Los Angels (UCLA) score was 25.\nPlain radiographs (true anteroposterior and scapular views) of the right shoulder did not show any fracture or deformity (Figure\n). He was subsequently examined using magnetic resonance imaging (MRI). The T1-weighted images showed a low-intensity area at the lesser tuberosity, and the T2-weighted images showed a high-intensity area at the subscapularis tendon (Figure\n). Computed tomography (CT) was performed with the arm being elevated and in an internal rotation. Unfortunately, we could not confirm a small bony fragment and lesser tuberosity in the same slice because of the distinct position. However, conventional CT revealed an irregularity and osteosclerotic change at the medial edge of the lesser tuberosity. Three-dimensional CT (3D CT) revealed a small bony fragment located between the humeral head and the scapular glenoid in the position for pain provocation (Figure\n). On the basis of these results, his condition was diagnosed as an avulsion fracture of the lesser tuberosity. However, even though it was an avulsion fracture, we intended to remove the bony fragment, because it was very small and repair the tendon.\nIn the operating room, the patient was maintained under general anesthesia and placed in the beach-chair position for arthroscopic removal of the fragment. Diagnostic arthroscopy was performed through a standard posterior portal. The articular side of the subscapularis tendon was torn, and an avulsion fracture was identified (Figure\nA). We could not confirm the insertion site of the deep surface of the subscapularis tendon as the fracture site, because the area was very small and covered with fibrocartilage. However, the small bony fragment was originally inserted in the deep surface of the subscapularis tendon. Subsequently, internal rotation of the arm resulted in entrapment of the small bony fragment between the humeral head and the glenoid of the scapula (Figure\nB). The bony fragment was oval and approximately 7 mm in the major axis. The long head of the biceps showed no signs of subluxation or dislocation. No further intra-articular pathological features were detected. After the anterior and anterosuperior portals were established, the small bony fragment was removed (Figure\nC). A Fastin RC suture anchor with a no. 2 Ethibond suture (Mitek, USA) was used and inserted on the side of the fracture via the anterior portal. The 2 threads protruding from the area where the anchor was inserted were introduced through the only deep surface of the subscapularis tendon by the suture relay technique. A mattress suture was then used to repair the subscapularis tendon (Figure\nD). Histological examination of the lesion showed that the avulsed bone fragment was surrounded by fibrocartilage (Figure\n).\nThe patient’s arm was immobilized at his side in a sling for 2 weeks after the operation. During this period, only passive flexion was permitted. After 2 weeks, active arm exercises were initiated in all planes, ensuring avoidance of provocation of pain. After a month, the patient was almost completely relieved of pain and was able to perform all the daily activities. However, preparation for college entrance examination interrupted his sport activity for a while. He entered college 2 years after the surgery and began playing tennis again at a recreational level. The postoperative range of motion was almost improved, whereas the range of motion in the horizontal flexion remained to be 100 degrees. The postoperative UCLA score also improved to 33.
Mrs SS presented with secondary infertility for 6 months. She had a full-term normal delivery female baby aged 4.5 years alive and healthy, after which she used barrier contraception for 3 years and IUCD for 1.5 years for spacing. HSG done outside on February 7, 2007, showed right tubal block with the left tube filling poorly. Cannulation was done under fluoroscopic guidance, after which both the tubes showed free spill. Endometrium was positive for tuberculosis PCR. She had undergone four cycles of ovulation induction, two with clomiphene citrate and two with gonadotrophins. She ovulated in all the cycles but her endometrium was thin and lesser than 7.5 mm in all four cycles.\nHer first scan done at our clinic was on day 19 of the menstrual cycle. The uterus was normal, but the endometrium was only 7.8 mm thick. Both ovaries were normal with a volume of 4.96 and 4.20 cm3 and an antral follicle count (AFC) of seven. With this history and investigation report, the patient was planned for OI with IUI. Two cycles of IUI were performed with tablet Letrazole 5 mg given from days 3–7. The dermal application of EstroGel was done once the follicles were >16 mm for the treatment of thin endometrium. The ovulation occurred on days 13 and 15. The endometrial thickness on the day of IUI was 12.8 and 12.3 mm. We did two more cycles of IUI with gonadotrophins. Ovulation was documented on days 11 and 14 of the cycle and the endometrial thickness was 11.6 and 11.1 mm. As there was no pregnancy, the patient was counselled for in vitro fertilization (IVF). Hysteroscopy was done prior to IVF, which revealed adhesions at the fundus and both cornu. The endometrium was fibrotic and pearly white in color except for a small portion on the anterior wall. The cavity was smaller than normal in size. Adhesiolysis was done and the patient was put on conjugated estrogen (Premarin) 1.25 mg twice a day for 25 days with medroxyprogesterone 10 mg twice a day for the last 10 days. This hormone replacement therapy (HRT) was given for 3 months. A repeat hysteroscopy done after 3 months revealed reformation of adhesions at the fundus which were cut using scissors and HRT given again for 3 months. Before IVF, hysteroscopy did not show any adhesions but the endometrium was thin and fibrotic on the posterior wall.\nThe protocol used for IVF was long luteal down-regulation from day 21 with gonadotrophins started on day 3 of the cycle when the estradiol was 18 pg/ml and progesterone was 1.15 ng/ml. She was started on urinary FSH 300 IU and as the estradiol level on day 8 of the cycle was 6 pg/ml and the follicular size was 8–12 mm, the dose was increased by 75 IU and continued till day 13. On day 14, Rec. hCG 250 mcg was given subcutaneously when there were six follicles 16–18 mm in diameter. On the day of hCG, estradiol was 3265 pg/ml and progesterone was 2.8 ng/ml. Oocyte retrieval was done 35 h later. Luteal phase support was given with intramuscular inj. Gestone 100 mg (Ferring Pharmaceuticals, India). The luteal phase was monitored for ovarian hyperstimulation syndrome (OHSS). Ten days after ET, β-hCG was 53 mIU/ml and a repeat level after 7 days was 1030 mIU/ml. At that time, a single gestational sac was seen with a mean gestational sac diameter (MGSD) of 4.5 mm corresponding to 5 weeks and 1 day. Two days later, the patient complained of minimal bleeding per vaginum. The β-hCG level was 1258 mIU/ml, the MGSD was 4.6 mm and both adenexa showed multiple corpora lutea. No gestational sac was seen in the adenexa. One week later, the MGSD had increased to 8.5 mm which corresponded to 5 weeks and 5 days, but no fetal pole was seen and the bleeding had stopped. Despite the increase in the MGSD, there was no corresponding increase in the β-HCG levels which were 1567 mIU/ml. Two days later, the patient had three syncopal attacks. On examination, the vitals were normal but there was tenderness in the right iliac fossa and fornix. On TVS, the IU sac with yolk sac was seen, but there was also a complex mass of 90 × 70 mm in the right adenexa with free fluid in POD, which was turbid. Diagnosis of right EP with coexisting IU pregnancy was made. In view of an IU pregnancy and right adenexal mass, the decision for laparoscopy was taken. At laproscopy, right partial salpingectomy for ruptured EP was done. One week later, the TVS showed a irregular gestational sac with MGSD of 5.8 mm and the β-hCG level was 820 mIU/ml. The repeat hCG level after 5 days was 418 mIU/ml and the gestational sac had not increased in size. Decision for a curettage was taken. One week later, the β-hCG level was 5 mIU/ml. A frozen embryo transfer was done in a natural cycle, and a biochemical pregnancy was documented with the β-hCG level going to 378 mIU/ml.
An 87-year-old man was referred to our hospital with dyspnea. Based on the NYHA classification, his dyspnea exacerbated from class II to IV during 1 week. Although coarse crackle was not observed, pitting edema in the lower limbs was observed on physical examination. A 12-lead surface ECG showed bradycardia and revealed atrial fibrillation rhythm with left bundle branch block, and the width of QRS duration was 144 msec. Echocardiography demonstrated an ejection fraction of 30–40%. Despite medical therapy and cardioversion to sinus rhythm, his dyspnea still showed class III on the NYHA classification. After denying ischemic heart disease by coronary angiogram, we concluded the patient was a candidate for CRT. CRT implantation was performed without any complications (Fig. A). The patient's left ventricular dysfunction improved clinically. One week after CRT implantation, he was discharged with no significant laboratory data, chest X-ray, and echocardiography data.\nHowever, at 1-month follow-up, he complained of fatigue on exertion. Physical examination revealed no specific findings. Although the QRS morphology was not different from that at 1 week after CRT implantation, 12-lead surface ECG showed atrial fibrillation. In addition, heart enlargement was observed by chest X-ray. From the parameters of echocardiography, heart failure was negative. However, moderate pericardial effusion which was not observed at 1 week after CRT implantation was observed (Fig. B). Therefore, we suspected the fatigue must be caused by the increased pericardial effusion. Although computed tomography suggested atrial lead perforation (Fig. ), anemia was not observed from the laboratory data and the value of the atrial lead impedance and sensing threshold were not different from those at 1 week after CRT implantation. The pericardial effusion which was observed by computed tomography was located not at the ipsilateral side of the atrial lead, but at the opposite side. From above findings, we concluded that the pericardial effusion was not due to lead perforation. Signs of cardiac tamponade were absent by echocardiography. In addition, despite the 2-week observation period, pericardial effusion did not increase. Therefore, we decided to continue observation with no treatment. However, at 3-month follow-up, his pericardial effusion gradually increased (Fig. A and B) with low-grade fever. Laboratory data showed elevation of inflammatory marker (CRP 7.51 mg/dL) with absence of anemia. Although he had a fever, it was a low-grade fever of 37.3°C. The WBC from the laboratory data was within normal of 7900/μL with normal fraction. In addition, the atrial lead impedance and sensing threshold were not different from those at 1 month after CRT implantation. Therefore, pericardial effusion due to infection or lead perforation was not suspected. From the above findings, postcardiac injury syndrome was suspected as the cause of effusion. Although treatment using colchicine is not as common as NSAIDs or steroids , for the syndrome, colchicine is able to produces an anti-inflammatory effect with less side effects . Treatment using colchicine was deemed preferable, to drainage of the effusion. Therefore, colchicine intake (1.0 mg/day) was started. After 3 days, inflammation marker started to decrease. Furthermore, the effusion was diminished at 1 month after the start of colchicine therapy (Fig. C).
A 60-year-old female patient was admitted to the inpatient treatment section from the outpatient therapy wing of the physical medicine and rehabilitation department for the evaluation and treatment of impaired walking due to gradual weakening of the bilateral lower extremities that had lasted for three months. The patient seemed to have cognitive impairment, and did not have accurate memory of the trauma. However, according to the report of a family member, the patient had been medicated for cerebral infarction, hypertension, and osteoporosis, with no problems in ambulation and other activities of daily living before slipping down three months ago. After that, she had been in a bed ridden state and showed gait disturbance. Based on neurological examination, her mental status was alert, but her orientation, memory, calculation and other cerebral functions were generally impaired, whereas her speech was intact. In terms of the cerebellar function, there were no specific findings on both of the upper extremities, and the lower extremities could not be checked due to muscle weakness. Although an accurate evaluation of motor function was challenging during the manual muscle test, the upper extremities were found to be in normal range. For the lower extremities, the flexor and extensor of the hip and knee of both sides were in poor grade, and the dorsiflexor and plantarflexor of both ankle joints were degraded to trace grade level. There was no limitation in passive range of motion. In both lower extremities, the spasticity corresponded to the Modified Ashworth Scale I grade. Although accurate evaluation for sensory function was also difficult, the patient showed a relatively intact result in the pinprick test, but the proprioception and vibration sensation were impaired. Deep tendon reflex was normal for both upper extremities but increased in both lower extremities, and Barbinski's signs and ankle clonus were found on both sides. Results of the functional evaluation showed impossibility of independent ambulation. Although the activities of daily living using the upper extremities were possible, the patient needed considerable assistance in other areas. In terms of urinary function, urinary retention was observed, so we inserted a foley catheter since the patient was incapable of self-voiding. In addition, due to severe constipation, medication was provided and we helped defection with the use of glycerine enema, 2-3 per day as needed. The first lumbar vertebral body bursting fracture was found in the simple film taken at admission. From the magnetic resonance imaging (MRI) of the lumbar spine, the spinal dural arteriovenous fistula was found in the first lumbar vertebral body bursting fracture in addition to the shades of dilated vein in the periphery of the spines starting from the fifth thoracic vertebra to the cornus medullaris. As a result, the spinal cord edema was accompanied, and the specific findings of high signal, light shade of the spine were observed from the T2 weighted image (). Although electromyography and somatosensory evoked potential test were considered, we could not conduct them because of the patient's transfer to the neurosurgical department due to urgent need of arterial embolization. For more accurate location mapping of the spinal arteriovenous fistula, we conducted spinal vessel angiography and found that the arteriovenous fistula originated from the twelfth intercostal artery and first lumbar radicular artery. In addition, the spinal vein located on the anterior and posterior spine that continued to the cervical vertebra region was observed (). Since the artery of interest was not that large, we planned the embolization procedure employing glue (n-butyl cyanoacrylate) rather than the operation. After embolization of the number twelve right thoracic vertebrate intercostal artery and first lumber, the embolization of all fistula was followed. As a result, there was no venous plexus observed around the spine (), and the neurologic symptoms of the patient improved. The hip flexor and knee extensor improved to 'fair grade' and the ankle plantar flexor improved to 'poor grade'. Although we were not able to check the sensory function due to the patient's impaired cognition, big changes were not observed after the treatment. As a result of the post therapy follow-up using MRI to see the condition of spinal cord edema, we found that the spinal vein was dilated due to arteriovenous fistula not being found, as well as the spinal cord edema around the artery. Therefore, we were able to observe normal low signal intensity of the spinal cord from the T2 weighted image (). With the help of rehabilitation therapy including strengthening and endurance exercise of the lower extremities, gait training, and functional electrical stimulation to both tibialis anterior, the patient showed gradual improvement and could ambulate about 2-3 m. The urinary symptoms also improved and the patient was able to self-void after removal of the foley catheter following normal functioning results based on the urodynamic study. Bowel function also improved and the patient was capable of daily defecation.
A 45-year-old male motorcyclist with a history of hypertension, hyperlipidemia, and coronary artery disease was brought to the emergency department after being struck by another car on the highway at speeds of at least 40 miles per hour. Upon presentation, the patient was evaluated using Advanced Trauma Life Support (ATLS) principles. He had a patent airway on arrival and was breathing spontaneously on room air. His initial heart rate was 87 beats per minute, and his blood pressure was 124/63 mmHg without signs of significant hemorrhage. He had an initial Glasgow coma score (GCS) of 15 with equal and reactive pupils. The patient admitted to consuming alcohol and had a serum alcohol of 243 mg/dL. A later CT of the head demonstrated a subcutaneous hematoma without any intracranial abnormalities. His remaining physical examination revealed left lower quadrant abdominal pain without signs of peritonitis, ankle deformities bilaterally, pain with hip range of motion, and blood at the urethral meatus. Given his physical examination findings, subsequent imaging confirmed an unstable pelvic fracture with diastasis of the symphysis pubis of 6 cm, widening of the left sacroiliac joint, a left ischial pubic ramus fracture, and a urethral injury (). He also had a left ankle dislocation and a right compound fracture of the distal tibia and fibula. No intraabdominal injuries were identified on CT imaging of the abdomen. The pelvis was stabilized with a binder by the orthopedic surgeons with subsequent emergency irrigation, debridement, and open reduction and internal fixation (ORIF) of the open ankle fracture as well as reduction of the left ankle dislocation. He was extubated after the procedure and monitored in the ICU while the remaining preoperative medical workup was completed including X-rays and CT scans with 3D reconstructions of the pelvis reconstructions. A hydromorphone patient-controlled analgesia (PCA) pump was utilized for pain control.\nOn hospital day 2, the patient was deemed fit for surgery and was taken to the operating theater for a combined operation by the orthopedic surgeons for ORIF of the pubic diastasis, sacral fracture, and sacroiliac joint followed by the trauma surgeons to reconstruct the abdominal wall and inguinal canal. The trauma team performed the exposure of the pubic symphyseal region and the pubic diastasis. A Pfannenstiel incision was made, and the planes were dissected exposing the left spermatic cord. The orthopedic team then performed a gentle open reduction of the pubic diastasis taking care to ensure that the bladder and urethra were not incarcerated. The Asnis III cannulated screw system and a Matta pubic symphyseal plate (Stryker GmbH, Switzerland) were utilized under C-arm fluoroscopic guidance with appropriate alignment of the AP and inlet and outlet pelvis views. Once the Mata plate was in place and the orthopedic reduction was completed, we proceeded to reconstruct the anterior abdominal wall. Since the Cooper ligament was destroyed, it was dissected to allow direct visualization of the pubic rami. The abdominal wall defect was measured to be 10 × 12 cm. We then used a modified Stoppa technique by placing the 6 × 6 in Prolene mesh under the damaged internal inguinal ring, making sure the spermatic cord on the left side was not injured or pinched, securing it in place using sutures, including direct suturing to the periosteum of the repaired pubic symphysis and the plate as needed. The medial borders of the mesh were tucked inside the opened rectus sheath on the right side and secured laterally with fires of a 5 mm Covidien Endotack (Medtronic, MN, USA) to the remnants of the conjoint ligament. The midline was then repaired with sutures, including the mesh as reinforcement. The patient did well postoperatively with postreduction films demonstrating appropriate alignment (). He was discharged to rehab on postoperative day 5. There were no recurrences during the follow-up period of 10 years.
A 45-year-old man presented to our otorhinolaryngology outpatient department with the symptoms of persistent odynophagia and hoarseness for 1 week after ingesting a fish bone. There was neither dysphagia nor dyspnoea. At first, he sought medical consultation in a local clinic and cervical X-ray was done. However, it was not conclusive, and there was no sign of presence of foreign body in the pharynx. Due to the worsening of his symptoms, he presented to our clinic on 25 August 2016. Flexible pharyngolaryngoscopy was done, and it was noted that there was left vocal cord immobility, oedematous left aryepiglottic fold, and left pyriform fossa with secretions in it. We could not find any foreign body in his pharynx nor in larynx. However, based on his history, the presence of fish bone impaction in the larynx was suspected. The patient was then admitted to the hospital, and urgent computed tomography (CT) scan of the neck was done and revealed a 3 cm linear foreign body embedded in the soft tissue medial to the left thyroid cartilage with surrounding hypodense soft tissue swelling causing mild indentation of the left vocal cord extending into the supraglottic region up to the left pyriform sinus and downwards minimally extending to the infraglottic region causing mild asymmetric narrowing of the involved laryngeal segments and causing mass effect on the glottis and supraglottic airway causing mild asymmetric narrowing ().\nThe diagnosis of inflammation of the paraglottic region and vocal cord immobility secondary to fish bone impaction in the paraglottic space was made. The patient was then shifted to the operation theatre (OT), and rigid direct laryngoscopy was performed under general anaesthesia. It revealed oedema in the left pyriform fossa and left supraglottic region, and no foreign body was visualized. Also, rigid oesophagoscopy was performed, and it showed the same findings as the laryngoscopy. Therefore, foreign body retrieval endoscopically has failed.\nThen, we proceeded to the external approach, and thyroid cartilage window with a Skeeter drill window of 12 mm [] was made and the foreign body was retrieved (Figures and ). Then, the wound was sutured and a corrugated drain was inserted. The patient was then shifted to the intensive care unit (ICU) for observation, and he was put on mechanical ventilation. We started dexamethasone IV 8 mg TDS to reduce oedema. He was also given Augmentin (amoxicillin/clavulanic acid) IV 1.2 g TDS as a prophylactic, and analgesia was given when required.\nThe progress of the patient was well, and he was extubated. We started him on oral feeds in the ICU before shifting him to the ward. After his condition improved, he was shifted to the ward and laryngoscopy was repeated, and it showed improvement in the mobility of the left vocal cord and no oedema was noted. In the ward, the patient was started on normal feeds and he tolerated them well and was discharged.\nOn the following laryngoscopy two weeks later, the swelling of the left pyriform fossa and the left aryepiglottic fold has subsided, and the function of the vocal cords movement was recovered.
A 560 gm male baby was born at 23+6 weeks of gestational age by spontaneous vaginal delivery to a 41-year-old mother with prolonged rupture of membranes for 48 hrs. Mother was treated with clindamycin and received two doses of betamethasone prior to delivery. The baby was hypotonic at birth and was noted to have poor respiratory effort. He was intubated and received the first dose of surfactant and was placed on synchronized intermittent mandatory ventilation (SIMV) with FiO2 0.4 and transported to the neonatal intensive care unit. Initial chest roentgenogram (CXR) showed diffuse ground glass opacity suggestive of respiratory distress syndrome. He received the second dose of surfactant on first day of life. He was hypotensive and dopamine drip was started. He was treated with intravenous ampicillin and gentamicin for 48 hrs. Initial blood cultures were negative. He received a course of ibuprofen to facilitate PDA closure.\nOn the third day of life, he developed pneumothorax on right side of the chest, which resolved spontaneously in 48 hrs. On the fifth day of life, he had recurrent oxygen desaturations and repeat CXR showed pulmonary interstitial emphysema (PIE) in both lungs (). On eleventh day of life, his respiratory status worsened requiring increased inspired oxygen concentration. CXR showed right side pneumothorax and cystic lesion in the left lower lobe, 8 mm in diameter. A chest tube was inserted on the right side and the pneumothorax resolved in 72 hrs. The cystic lesion in the left lower lobe gradually increased in size to 33 mm in diameter in the next two weeks (). His oxygen requirements increased gradually to FiO2 0.9–1.0 on SIMV with high inspiratory pressures.\nOn nineteenth day of life, he was switched to high frequency oscillatory ventilation with inhaled nitric oxide 20 ppm to improve oxygenation and prevent further air leak. He was started on intravenous dexamethasone. Three hours after administration of dexamethasone, he suddenly became apneic and bradycardic with oxygen desaturation to 20% on HFOV with FiO2 0.8, mean airway pressure (MAP) 11 cm H2O, delta P 24, and frequency 14.5 Hz with inhaled nitric oxide 19 ppm. He had cardiopulmonary resuscitation (CPR) with chest compression. His blood gases showed significant respiratory acidosis. He developed tension pneumothorax on left side with pulmonary interstitial emphysema and pneumoperitoneum (Figures and ). His abdomen was distended but remained soft with bowel sounds. He had emergent chest tube placement. A Penrose drain was inserted into abdomen which drained minimal peritoneal fluid.\nDuring the next few days, he improved and HFOV was switched to synchronized intermittent mandatory ventilation (SIMV) with FiO2 0.4. He was started on enteral feeds and he tolerated them well. Next day, he had acute deterioration with oxygen desaturations, bradycardia, and hypotension. He had significant respiratory and metabolic acidosis on blood gases. CXR showed right upper lobe atelectasis. His SIMV was switched back to HFOV. He was noted to be anemic and was transfused with 15 mls/kg of packed red blood cells. He remained hypotensive with poor perfusion on dopamine and epinephrine infusions. He was treated with intravenous meropenem and linezolid to treat presumed sepsis. He had distended abdomen and abdominal X-ray showed dilated loop of transverse colon with no evidence of intra-abdominal free air. Blood cultures were negative at 5 days. He died from cardiorespiratory failure. Autopsy confirmed premature lungs appropriate for gestational age, pulmonary interstitial emphysema in both lungs, 4 mm cystic lesion lined by fibrous tissue in left lower lobe which may represent localized pulmonary interstitial emphysema (Figures and ), and congested and dilated loop of transverse colon with no macroscopic or microscopic evidence of perforation or pneumatosis intestinalis. Diaphragm was intact. Both bacterial and viral cultures of tissues and fluids at autopsy were negative.
An otherwise fit 16-year-old boy was referred for evaluation and treatment to our department (a tertiary Trauma and Orthopaedics center) by a general practitioner. The patient was suffering from pain on the lower right thigh with an onset of approximately four months before his initial evaluation. The pain was described as being initially dull and intermittent; later, it became continuous and significantly increased in severity. The pain was elicited on palpation at the affected area and aggravated by weight bearing. The ipsilateral hip and knee joints had a normal range of motion. Red and white blood cell count, C-reactive protein, erythrocyte sedimentation rate, biochemistry, and thyroid and parathyroid hormones levels, were all within normal range. Plain radiographs (Figures and ) revealed the existence of a rounded and well-defined lytic lesion at the right femoral diaphysis with evidence of surrounding mild laminated chronic-type periosteal reaction. The patient subsequently underwent a CT and an MRI scan. CT scan revealed a 13 × 24 mm eccentric lytic lesion with intact femoral cortices and no soft tissue involvement. The same lesion was depicted on the MRI scan (Figures and ) as rounded and well-defined, with intermediate MR sign in the T1 and hyperintensity in the T2-weighted images. The lesion demonstrated intense but relatively homogenous enhancement after the intravenous administration of contrast. On the short T1 reverse- (STIR-) weighted images, intense surrounding bone oedema and a linear periosteal reaction were present.\nThe patient underwent core needle biopsy under general anesthesia and C-arm X-ray image intensifier control (in order to accurately locate radiographically the exact location of the lesion) with a 6 mm trocar. Culture swabs were also taken from the lesion area and came back as negative. The histopathologic study of the specimen showed a mixed cellular infiltrate made up of macrophages, lymphocytes, eosinophilic granulocytes, multinucleated cells, and Langerhans cells. Further, immunohistochemistry showed positive staining for S-100 protein and CD-1a antigen and confirmed the diagnosis of LCH. Further imaging studies (plain radiographs of the thoracic and lumbar spine, skull, and pelvis) and a Tc99 bone scan verified the unifocal form of the disease, and a bone marrow biopsy failed to reveal any other pathology.\nTaking into consideration the solitary form of the disease, its location in a weight-bearing long bone, and the age of the patient and following consultation with a consultant radiologist, a pediatric-oncologist, and a pediatric-hematologist, we decided to perform RFA instead of open curettage or wide excision of the lesion followed by bone grafting and/or internal fixation.\nThe radiofrequency ablation procedure took place in the CT suite room under general anesthesia. A thin biopsy needle was initially introduced through the thickened femoral cortex via the track of the previously performed biopsy. A 15 cm/11-gauge RFA electrode needle (AMICA RF, HS Hospital Services S.p.A., Rome-Italy) was used. The RFA procedure was initiated following confirmation that the tip of the plain electrode was well placed in the center of the lesion. The electrode tip was heated up to 90-94°C for 8 minutes. After a cooling period of 1 minute, the electrode was safely retracted, followed by the probe and the cannula. The patient reported immediate relief from pain as soon as he woke up and was uneventfully discharged the day after. He was advised to walk without bearing weight and with the use of two crutches for one month.\nAt three months after the treatment, the patient was completely asymptomatic and able to walk with full weight bearing. The CT scan revealed a central zone with fluid density due to necrosis (). The MRI scan confirmed the central necrosis of the tumor, a moderate enhancement at the periphery of the lesion and a mild surrounding bone oedema, which was a consequence of the RFA procedure (Figures –). At six months post-RFA, he reported no pain at all, he was capable of performing all physical activities, and he was completely happy with the overall result of his treatment. The CT scan depicted the existence of callus at the cortex hole area (). The MRI scan revealed a more prominent central necrosis, while the post-contrast enhancement and the surrounding bone oedema were decreased (Figures –). During subsequent follow-up visits at 12, 18, 24, and 36 months post-RFA, the MRI characteristics depicting necrosis at the RFA area gradually subsided, until they became normal at the last follow-up visit at month 48 (). The patient remains still symptoms-free at his latest phone call follow-up visit six years post-RFA.
This case is about a 65-year-old female patient who developed bilateral lower extremity deep venous thrombosis (DVT). She had a medical history of right intradural internal carotid artery (ICA) aneurysm, status poststent-assisted flow diversion, and coil embolization. Anticoagulation was contraindicated due to recent coil embolization of the right ICA. An IVC filter was placed to protect the patient from pulmonary embolism. Ten months after the IVC filter placement, the patient had a complete resolution of lower extremity DVT, confirmed by ultrasound. She presented to our institution for elective removal of the IVC filter. The procedure was performed in the angiography suite under local anesthesia. Under fluoroscopic guidance, the IVC filter cap was dissected from the IVC wall, grasped, and then removed through a sheath. A cavagram was performed, and no complications were noted, showing patency of iliac veins and the IVC. However, on examining the filter, one of the secondary legs was missing, likely representing a fracture. The patient was evaluated at multiple sites with fluoroscopy to look for the retained foreign object. The procedural field was also inspected exhaustively. However, the missing piece was nowhere to be found. The patient remained hemodynamically stable through the entire case. At this point, the procedure was concluded, and the patient was sent to obtain computed tomography (CT) scan of the chest, abdomen, and pelvis to look for the retained IVC filer leg.\nThe imaging of the CT abdomen and pelvis was unremarkable. Chest CT scan revealed an ill-defined hyper-attenuating structure in the region of the tricuspid valve []. This was highly suspicious for the fractured strut of the IVC filter. Two-dimensional transthoracic echocardiography was obtained, which showed no changes compared to prior studies in the past.\nThe decision was made to retrieve the IVC filter strut under fluoroscopic guidance. Because of the possibility of tricuspid valve/heart injury and the need for sternotomy, general anesthesia was planned. Two large bore peripheral IVs (18G on each hand) were placed. Preinduction arterial line was placed in the left radial artery. The Standard American Society of Anesthesiologists monitors with five-lead electrocardiography were used throughout the procedure. After IV induction of anesthesia, the trachea was intubated with a 7.0 endotracheal tube, and a transesophageal echocardiograph (TEE) probe was inserted into the esophagus to assess cardiac integrity during the procedure. The interventional radiologist accessed the right common femoral vein and carefully advanced the guide catheter into the right atrium. After appropriate maneuvering, the filter fragment was snared and slowly pulled out of the heart. TEE study during and after procedure did not show any cardiac complications. It only revealed mild tricuspid regurgitation that was unchanged and already present from before as evidenced by prior echocardiographic studies. The fragment was secured and sent to pathology []. A completion cavagram was obtained. No complications were noted, the sheath was removed from the puncture site, and manual compression was held until hemostasis was achieved. The patient remained hemodynamically stable throughout the procedure. She was extubated successfully at the conclusion of the case and transferred to post anesthesia care unit where she remained. She was subsequently admitted to regular floor for overnight observation and was discharged home on the postoperative day 1 in stable condition.
A 48-year-old male presented in emergency with a history of abdominal mass since childhood. The abdominal mass was gradually progressive in size and rapidly increased in the last two weeks. He also complained of breathlessness for one week associated with abdominal pain. The patient never went to any hospital for the abdominal mass. There was no history of vomiting, altered bowel habits, trauma to the abdomen, melena, and passage of any membranes in the stools. There was no history of cough with sputum. On examination, the patient had tachycardia with normal blood pressure. There was no pallor or jaundice. Abdominal examination showed a large mass of almost 20cm x 20cm occupying the epigastrium, right hypochondrium, left hypochondrium, and umbilical region. It had varying consistency - soft in the upper part and firm in the lower and lateral parts.\nRoutine blood investigations, including complete blood counts and renal function tests, were normal. A liver function test revealed mild elevation (174 IU/L) of alkaline phosphatase (ALP). Hydatid serology was positive. Abdominal USG showed the presence of a large hypoechoic cyst with air and debris. Contrast-enhanced CT of the abdomen showed a 20cm x 18cm cyst, arising from the left lobe of the liver having an air-fluid level and few septations or membranes at the inferior aspect of the cyst with debris. The left portal vein was compressed, and the left bile duct was not visualized. The gallbladder had air within it. The common bile duct (CBD) was normal in caliber, and there was no air within it (Figure ).\nThe cyst's fistulous communication with the duodenum was demonstrated with oral contrast CT scan (Figure ). The cyst was closely related to the stomach and had displaced the stomach and spleen laterally. The pancreas appeared normal.\nAs the patient was breathless, a decision was made to put a percutaneous pigtail drain within the cyst to decompress. The percutaneous tube drained about 800 mL of bile-stained foul-smelling fluid with whitish jelly-like thin membranes. The microbiological analysis of the fluid showed the growth of gram-negative organisms, suggestive of an infected cyst. The cystic fluid's carcinoembryonic antigen (CEA) level was 230 ng/mL, and the carbohydrate antigen (CA19-9) was more than 1972 U/mL. However, serum CEA and CA19-9 levels were within the normal range.\nMagnetic resonance cholangiopancreatography (MRCP) showed a well-defined large cyst of 15cm x 15cm, probably arising from the left lobe of the liver with an air-fluid level in the epigastric region with pigtail inside. CBC could not be demonstrated clearly (Figure ).\nUpper gastrointestinal endoscopy showed a bulge along the lesser curvature of the stomach and fistulous opening in the first part of the duodenum (Figure ).\nThe patient was prepared for laparotomy and possibly left hepatectomy. After exploration, the abdomen was packed with cetrimide-soaked pads. Intraoperatively, a large cyst was found from the liver's left lobe and had dense adhesions with the stomach, diaphragm, and transverse mesocolon. During the adhesiolysis, there was an inadvertent opening of the cyst wall. Hence the cyst wall was opened for controlled drainage. The inside of the cyst wall was covered with a whitish germinal layer. Hence, the diagnosis of the HHC was confirmed. We proceeded with a partial cystectomy of the HHC. The germinal layer of the residual cyst wall was removed by scraping.\nA meticulous search for the CBC was done. A cholecystectomy was performed. The cystic duct’s cannulation was done, the catheter advanced into the hepatic duct, and CBD was blocked with a clamp. Propofol was injected through the catheter. Four CBCs were very well demonstrated with leakage of the propofol through the inner surface of the residual cyst wall. All the communications were primarily sutured with polypropylene. A drain was placed into the residual cyst cavity, and omentopexy was done. The cystoduodenal fistula was visualized during the cystectomy (Figure ).\nAs the size of the fistula was less than 5 mm, it was closed primarily with an omental patch.\nPostoperatively patient improved well without any complications. Orals were started on the fourth postoperative day and escalated gradually. The patient was continued with albendazole for three months. Repeat USG did not show any evidence of the recurrence. Postoperative histopathological features were diagnostic of hydatid cyst.
The patient was a 20-year-old male who had been a low-birth-weight infant, and had a history of Klinefelter syndrome and pulmonary valve stenosis. He was introduced to our hospital for further examination of a liver tumor that was increasing in size. The tumor had been found incidentally after laboratory findings in a health checkup showed impairment of liver function. The patient had declined treatment due to his employment situation, and had instead been followed up for 1 year.\nAt the first visit, he was completely asymptomatic with normal vital signs. A physical examination revealed a palpable right upper mass without tenderness. No symptom related to Cushing syndrome was observed. In blood tests, hepatitis B virus surface antigen and hepatitis C virus antibody were negative. Liver function tests indicated mild dysfunction. Regarding tumor markers, serum alpha-fetoprotein (AFP) and carcinoembryonic antigen (CEA) were normal; however, neuron-specific enolase (NSE) was elevated.\nUltrasonography showed a large low-echoic solid tumor with a vertical diameter of > 80 mm with partial calcification implied by an acoustic shadow in an anterior lesion of the liver. A computed tomography (CT) scan of the chest, abdomen, and pelvis revealed an 81 × 76 × 72 mm large, heterogeneously enhanced mass in the right lobe of the liver with dense partial calcification (Fig. a). Subsequent positron emission tomography (PET)/CT showed a large hepatic mass in the right lobe with a maximum standardized uptake value (SUV) of 22.4 and no extrahepatic metastasis. In magnetic resonance imaging (MRI), most of the tumor was weakly enhanced in T1-weighted images and strongly enhanced in T2-weighted images. Part of the tumor had early enhancement and washout in enhanced MRI. These findings suggested HCC, and especially fibrolamellar HCC, but without evidence of distant metastasis.\nRight hepatic lobectomy and cholecystectomy were performed 11 months after the initial detection of the tumor. The patient received no adjuvant chemotherapy or radiotherapy. The postoperative course was characterized by respiratory failure that required reintubation on postoperative day (POD) 2. X-ray and bronchofiberscopy showed pneumoniae due to pulmonary atelectasis and pulmonary edema. The subsequent hospital course was uneventful. On POD 7, a CT scan of the abdomen was interpreted as negative for hemoperitoneum and tumor recurrence, and the patient was discharged on POD 12.\nThe patient was followed up as an outpatient and received several examinations. On POD 62, a CT scan showed multiple, obscure, and circumscribed recurrent lesions in the remnant liver with contrast enhancement. The largest of these lesions had a diameter of 42 mm in segment 1 (S1) (Fig. b). In addition, a hypermetabolic para-aortic lymph node with possible metastasis was identified. On PODs 70 and 73, the patient underwent transcatheter arterial chemoembolization (TACE), but a second CT scan in the outpatient department on POD 84 revealed enlargement of recurrent tumors and the para-aortic lymph node. Chemotherapy (protocol for HCC) was started, but was unsuccessful because of side effects. At this time, there were no further surgical options and no other chemotherapy that was likely to be effective. Therefore, the patient received palliative care. The patient died 164 days after hepatectomy from tumor progression with development of progressive liver failure.\nGrossly, the tumor was confined to the right liver lobe. The resected specimen weighed 1180 g. The lesion had a maximum diameter of 100 mm, and was a well-circumscribed solitary mass with multiple small calcifications that were sharply demarcated from surrounding uninvolved liver parenchyma (Fig. ). The surgical margin was tumor-free. Microscopically, the tumor was characterized by an organoid arrangement of cellular nests of epithelioid cells and areas of sheet-like cell overgrowth (Fig. a). These cells had oval-like nuclei with no clear nucleolus and eosinophilic cytoplasm. Transition zones between epithelioid and spindle cells were observed, and a framework of spindle cells surrounded nests of epithelioid cells (Fig. b, c). Bile ducts were not intermingled with the tumor region. There were extensive regions of necrosis and calcification (or ossification) in the center of the tumor (Fig. d).\nIn immunohistochemical staining, epithelioid cells were positive for CD56, cytokeratin AE1/AE3 (focal), WT-1 (diffuse or dot-like in cytoplasm), β-catenin (diffuse in nucleus), vimentin, NCAM, and NSE (Fig. a, b). Spindle cells in mesenchymal components such as the septum were diffusely stained with α-smooth muscle actin (α-SMA) (Fig. c). The AFP level was within the normal range. Staining for glypican-3 was negative. The proliferation index on MIB-1 (Ki-67) immunostaining was < 5%. Staining was negative for hepatocyte paraffin-1, CK7, adrenocorticotropic hormone (ACTH), estrogen receptor (ER), and progesterone receptor (PR). The morphological and immunohistochemical features led to diagnosis of CNSET.
In October 2012, a 46-year-old woman was referred to our center for the presence of a large mass (50 × 70 mm) in the superior lobe of the left lung with homolateral pleural effusion. The patient was never smoker, without family history of cancer and without comorbidity. The cytological diagnosis was made using a CT-guided fine needle aspiration of the primary tumor and revealed an adenocarcinoma of the lung (TTF1+, CK7+) with the EGFR ex19del mutation. A PET-CT demonstrated the presence of liver and bone metastases and a nodule in the right breast, confirmed as a metastasis by fine needle aspiration. The patient received zoledronic acid 4 mg every 28 days and gefitinib 250 mg daily since November 2012 obtaining a partial response (PR). In August 2013, a disease progression (PD) was documented, with an increase in size of the primary tumor and size and number of liver metastases. A brain MRI revealed the presence of two cortical nodules, which were treated with stereotactic radiotherapy. The patient was enrolled in the IMPRESS trial and received 6 cycles of cisplatin and pemetrexed plus gefitinib obtaining again a PR that lasted until June 2014. Thereafter, a new lung metastasis appeared in the superior lobe of the left lung and the mammary nodule increased in dimensions. From June 2014 to December 2014 the patient received erlotinib 150 mg daily obtaining an initial stabilization of the disease (SD); however, within 6 months, she experienced again a PD with the increase of the mammary nodule and the appearance of a new bone metastasis in the sacrum. In December 2014, EGFR ex19del and T790M mutations were detectable in a new needle biopsy of the primary tumor; only at this time a digital PCR-based method was available for the analysis of circulating tumor DNA (ctDNA). Briefly, the method was optimized in order to recover a suitable amount of ctDNA for molecular analysis from 3 ml of plasma using the QIAmp Circulating Nucleic Acid Kit (Qiagen®, Valencia, CA). ctDNA was examined using the Prime PCR Probe Assay on a QX100™ Droplet Digital™ PCR System (BioRad®, Hercules, CA) for EGFR mutations (ex19del, T790M, and C797S) []. The ctDNA sample was considered as EGFR mutant when at least one droplet was above the fluorescence intensity threshold of 3000 and results were reported as copies/ml. The first plasma specimen was obtained in December 2014 and confirmed the presence of ex19del and T790M mutations (480 and 260 copies/ml, respectively; Fig. ). The patient was treated with atezolizumab from March to May 2015 and received stereotactic radiotherapy on the lung primary tumor and on metastases of the left superior lobe, breast and bone (sacrum and D10). Due to PD, the patient was given afatinib 40 mg daily from June 2015 to September 2015, but she experienced a further PD with an increase in size of the primary tumor and lung nodules, the appearance of new multiple bilateral lung metastases, lymphangitic infiltration and liver metastases. At this time, the presence of ex19del and T790 M was again demonstrated in a biopsy of a liver metastasis. Since osimertinib was available, the patient was enrolled in the ASTRIS trial and given 80 mg daily starting from October 2015 with an immediate clinical benefit. At the first evaluation a PR was documented with disappearance of most of the lung nodules and lymphangitic infiltration, reduction of the primary tumor and of liver metastases as well. A disappearance of ex19del or T790M was demonstrated in ctDNA in a blood sample obtained in October 2016. However, in April 2017, ex19del appeared again (400 copies/ml) and in July 2017 it increased to 1000 copies/ml, while T790M was also detectable (330 copies/ml, Fig. ). Then, ex19del and T790 M continued to increase to, respectively, 1600 and 510 copies/ml in October 2017, 2100 and 550 copies/ml in November 2017, and 6900 and 1900 copies/ml in December 2017.\nA radiological progression was demonstrated with increase in size and number of liver metastases in December 2017. The patient underwent a new liver biopsy that confirmed the PD and the presence of ex19del and T790M, whereas the ctDNA showed also the appearance of C797S mutation (170 copies/ml), in addition to ex19del and T790M. The patient started chemotherapy with carboplatin and pemetrexed and in February 2018 she obtained an SD associated with a drop of ex19del (800 copies/ml), T790 M (380 copies/ml), and C797S (90 copies/ml) and then a PR in May 2018, with disappearance of C797S and reduction of ex19del (760 copies/ml) and T790M (90 copies/ml). In July 2018, however, ex19del strongly increased to 2200 copies/ml, even though T790M and C797S were undetectable. Finally, in October 2018, when a PD was documented, ex19del increased to 5100 copies/ml, while T790M and C797S appeared again in plasma with 600 and 180 copies/ml, respectively (Fig. ). At this time a NextSeq 550 NGS platform (Illumina®, San Diego, CA) was available to analyse ctDNA by the AVENIO ctDNA Expanded Kit (Roche®, Pleasanton, CA). A plasma sample collected in December 2018 and analysed by NGS confirmed the presence of the ex19del, T790M and C797S and found, in addition, EGFR and c-MET amplifications, which were not present in tissue in the last re-biopsy of December 2017.
A 27-year-old female with primary infertility was seen in our unit in March 2011. She had regular periods and her body mass index was 25 kg/m2. Her baseline investigations were normal and ultrasound report revealed polycystic ovaries. Her husband's semen-analysis report revealed azoospermia. His clinical and biochemical findings were suggestive of obstructive azoospermia. After counseling regarding the treatment options available, a diagnostic percutanous epididymal sperm aspiration was performed which revealed presence of motile sperms. The couple decided to go ahead with ART.\nCycle programming was done using combined oral contraceptive pills and controlled ovarian hyperstimulation was started after onset of menstruation using with recombinant follicle stimulating hormone (FSH) and multiple dose flexible antagonist (0.25 mg) regimen was followed. Injection human chorionic gonadotrophin (hCG) 5000 international units (IU) intramuscular was administered once more than three follicles reached a diameter of 17 mm. Serum estradiol (E2) on the day of hCG trigger was more than 3800 pg/mL and expected number of follicles was 14-15. She underwent ultrasound guided transvaginal oocyte retrieval and a total of 13 metaphase II oocytes were retrieved. She received 100 ml infusion of human albumin (20% solution) over 4 h, prophylactically after oocyte retrieval. Intracytoplasmic sperm injection was performed using surgically retrieved sperms and 10 fertilized oocytes were obtained the next day. On day 2, three grade 2 cleavage embryos were transferred.[] Supernumerary embryos were cultured and eventually two blastocysts were cryopreserved.\nPatient received luteal support in the form of daily vaginal micronized progesterone 400 mg twice daily and was given intramuscular injection of progesterone 100 mg weekly twice. The patient was advised to take high protein diet and to maintain the total fluid intake/output records which was evaluated once in 3 days for signs and symptoms of OHSS.\nOn post-retrieval, day 9, the patient presented with complaints of severe abdominal discomfort, vomiting, difficulty in breathing and decreased urine output. Her abdominal girth had increased from 72 cm to 84 cm. Her blood test revealed hemoconcentration (hematocrit was 54%), raised liver enzymes, and low sodium (122 mEq/L). An ultrasound of the abdomen revealed bilateral enlarged ovaries (right ovarian size was 11 × 10 × 9 cm and left ovarian size was 10 × 8 × 8 cm) with significant ascitis []. Chest X-ray (an abdominal shield was used) showed bilateral pleural effusion. The patient was diagnosed with severe OHSS and admitted to the intensive care unit. Intravenous fluid administration, analgesics, anti-emetics, and prophylactic anti-coagulation with low molecular weight heparin 2500 IU subcutaneously were initiated. After stabilization, patient underwent therapeutic pleural tap and ultrasound-guided abdominal paracentesis. A total of 2.5 L of ascitic fluid was drained. The patient's symptoms were relieved temporarily. The patient's clinical and biochemical parameters were monitored on a daily basis. The patient was advised to wear elastic stockings and high protein diet intake. Infusion of human albumin was also given once daily.\nHowever, within 2 days the patient had recurrence of abdominal discomfort and difficulty in breathing due to collection of ascitic fluid. A repeat paracentesis was carried out and further 2.5 L of fluid was drained out. Barring low albumin levels (2.1 g/dL) rest of the biochemical parameters were stabilizing (hematocrit 34%). However, despite a high protein diet and daily infusion of albumin, the albumin levels kept deteriorating. Further she developed severe anaphylactic reaction after human albumin infusion on day 13 post-retrieval, which necessitated discontinuation of infusion. Despite all prophylactic measures prompt recognition and early aggressive treatment of OHSS, the patient's condition did not show any signs of improvement. She had rapid re-accumulation of ascitic fluid and due to worsening of symptoms, we had to resort to repeated paracentesis in order to relieve her of her discomfort. However, the serum albumin levels had fallen to alarming levels (1.7 g/dL) partly due to ascitic fluid tapping. The patient developed signs and symptoms hypoalbuminemia in the form of generalized puffiness of the body, face and limbs, and developed vulval edema. Her serum beta HCGs levels were 30 mIU/L (day 10, post-retrieval). With early pregnancy, worsening late OHSS, and severe hypoalbuminemia, rapid re-accumulating of ascites and drainage lead to loss of protein rich fluid, inadequate replacement of protein lead to further lowering of albumin, further contributing to rapid re accumulation of ascitis. Abdominal paracentesis was done 6 times and on day 20, after consulting the medical oncologist, who was familiar with treatment of rapidly accumulating ascitis, as a last resort we decided to transfuse the patient with thawed plasma.\nPatient was transfused four units of thawed plasma in 48 h. The transfusion volume was calculated (10-15 mL/kilo body weight). This resulted in a dramatic improvement in the patient's condition as reflected by significant increase in urine output and stabilization of abdominal girth (indicating no clinically significant re-accumulation of ascitic fluid). Most importantly her serum albumin level started rising (2.5 g/dL immediate post-transfusion). No further paracentesis was required and patient was discharged day 26 post-retrieval.\nThe patient's serum beta HCG levels on day 18 after oocyte retrieval was 522.4 mIU/L and after 14 days the transvaginal ultrasound revealed twin intrauterine gestation sacs with a yolk sac and a fetal pole in each sac but no cardiac activity []. Subsequently, a repeat ultrasound was done a week later and the patient was diagnosed with missed abortion and the pregnancy terminated. The patient was discharged and planned for frozen embryo transfer at a later date.
A 51 year-old male was diagnosed with chronic pancreatitis of seven months duration, having an obscure etiology. He was a non-drinker and a non-smoking. His chief symptom included several episodes daily of severe abdominal pain, and unable to stand without narcotic analgesics. The pain was mainly epigastric, but radiating to the rear. It was more severe after meals, and was relieved by leaning forward. Patient had lost weight (from 90 kg to 65 kg), and was diabetic.\nThe diagnosis of chronic pancreatitis was made based on the clinical presentation, radiological workups and percutaneous biopsy at another center. He had a metallic biliary stent for biliary stenosis. Endoscopic examinations revealed gastritis. A percutaneous celiac axis blockage was required for severe episodes of pain. Before referring to our center, patient had undergone laparotomy for pancreatic resection; however, because of the portal hypertension, the procedure had been capped off. The pancreatic tru-cut biopsy at this laparotomy confirmed the diagnosis of chronic pancreatitis without evidence of malignancy. He was then referred to us for surgery for pain relief.\nAt the time of admission, he was daily using narcotic analgesics, proton pump inhibitor, pancreas enzyme extracts and antidepressant drugs. He had mild anemia, slightly elevated liver function tests, and CA-19-9 was 202 U/ml. Computed tomography was compatible with chronic pancreatitis (heterogenous parenchyma, dilated pancreatic duct), and a 4 cm pseudocyst was seen at the tail of the pancreas ( and ). Positron emission tomography revealed the pancreatic activity as 5.1 SUVmax.\nWe intended to make a pylorus-preserving pancreaticoduodenectomy with portal vein replacement. Before surgery, the patient and his relatives were informed of the possible surgical methods. A reverse “L” incision was used. Adhesions of the prior surgery and venous collaterals complicated the procedure. A cholecystectomy was performed, and the common bile duct was transected for bilio-enteric anastomosis to replace the biliary stent. The metallic stent in the distal bile duct was checked, but was found to be stuck and could not be removed. Subsequently, the common bile duct stump was closed. The dilated venous collaterals, particularly around the head of the pancreas, resulted in substantial blood loss during the dissection, and 3 red pack cells were transfused during the procedure (). It was then decided to pursue with the Frey procedure instead of pancreatic head resection. The inflamed pancreatic head was excavated, and the cavity at the pancreatic head was expanded to the distal pancreas ( and ). The enlarged pancreatic duct became visible at the body and tail of the pancreas; however, the pancreatic duct at the head of the pancreas was not visible (). A Roux-en-Y pancreaticojejunostomy was created by 3-0 running polypropylene suture. Bilio-enteric anastomosis was done 10 cm distal of the pancreatic anastomosis with 4-0 polydiaxone interrupted sutures. Entero-enterostomy of the Roux limb was created in the usual way (). Totally, 40 ml absolute alcohol was injected around the celiac plexus for pain relief. Laparotomy was closed after insertion of two abdominal drains. The total operative duration was 490 minutes.\nDuring the early postoperative period, patient was delirious in the intensive care unit, but the attacks were relieved after medications. Fortunately, the remaining postoperative course was uneventful, except for prolonged abdominal ascites drainage and intractable diarrhea that commenced on day 11, and was treated conservatively (). The drain amylase levels were continually in the normal ranges. Oral liquid and semisolid nourishments were started on days 5 and 7, respectively. Patient required only one dose of 60 mg meperidine in the postoperative period. He was discharged on day 28. Unexpectedly, pathological examination of the pancreatic specimen reported chronic pancreatitis accompanied with pancreatic cancer. He was then referred to the medical oncology department.
A 25-year-old female patient was referred from an outside hospital in November 2011 for the management of dysphagia. The patient initially presented to a different hospital in 2008 with a 4-year history of heartburn and acid regurgitation and was diagnosed with GERD. The patient did not complain of dysphagia or globus symptoms at the time, and the esophagogastroduodenoscopy (EGD) performed at the previous hospital showed grade B erosive esophagitis according to LA classification. A 24-hour intraesophageal pH study showed a DeMeester score of 33.1 (normal value, <14.2), a total fraction time of pH <4 of 9%, and abnormal acid regurgitation when the patient was upright (upright fraction time of pH <4, 24%). Preoperative esophageal manometry showed normal lower esophageal sphincter (LES) relaxation during swallowing (resting LES pressure 14 mm Hg to LES relaxation 2 mm Hg), and no peristalsis was observed in the esophageal body (). A favorable response to medical treatment (proton pump inhibitor) was not achieved, and subsequent laparoscopic Nissen fundoplication was performed in September 2009 in a previous hospital. Thereafter, the patient developed postoperative complications such as solid and liquid dysphagia, a sensation of inability to belch, and a sticking sensation in her lower to mid chest. Approximately 2 to 3 weeks after the operation, the patient's symptoms showed improvement. However, regurgitation recurred and was soon aggravated to dysphagia. Dysphagia was worse with solids than with liquids, and these symptoms occurred whenever the patient swallowed food. Medical therapy with proton pump inhibitors and prokinetics was attempted in the previous hospital but was ineffective. The patient was then referred to our hospital. EGD performed at our hospital showed postfundoplication status, and the endoscope could pass through the gastroesophageal junction without any resistance (). The previously observed erosive esophagitis was improved. Esophageal mucosal biopsies ruled out eosinophilic esophagitis. Abnormal barium stasis in the esophageal body was found on barium esophagography (). A paraesophageal hernia was observed on abdominopelvic computed tomography (), which was performed to evaluate the patient for postoperative organic causes of dysphagia. Esophageal manometry showed aperistalsis in the esophageal body, and the resting pressure and percent relaxation of LES were 5 mm Hg and 81%, respectively, which were within the normal range (). In our hospital, medical treatment (prokinetics, mosapride 15 mg; proton pump inhibitor, esomeprazole 40 mg; calcium channel blocker, nifedipine 5 mg) was initiated and continued for 2 months; however, a favorable outcome was not obtained. Pneumatic dilatation with a 30-mm balloon was used for symptom relief, but the symptoms did not improve. Therefore, additional pneumatic dilation with a 35-mm balloon was performed 2 weeks later. Barium esophagography performed after the second pneumatic dilatation showed improved barium passage through the esophagus, but the solid and liquid dysphagia, and globus sensation symptoms were not improved (). We considered esophageal motility disorder or paraesophageal hernia that developed after fundoplication as the most likely cause of the dysphagia. Therefore, we recommended a revision operation that would involve taking down the wrap or Toupet fundoplication. However, the patient refused to consent to a second operation and is currently undergoing medical treatment.
The patient is a 47-year-old Caucasian female who presented to the Emergency Department of an academic tertiary-care hospital in the Midwestern United States with complaint of left-sided weakness of the upper and lower extremities and right gaze preference three weeks after a right pontomedullary infarct complicated by Posterior Reversible Encephalopathy Syndrome (PRES) [that initial infarct had been treated in a different state]. Imaging revealed an acute infarct in the posterior limb of the right internal capsule without hemorrhagic transformation and an acute punctate infarct in the right parietal subcortical white matter with corresponding diffusion restrictions, as well as remote evidence of subcortical chronic diffuse microhemorrhages (). The Psychiatry Consultation & Liaison service was consulted on hospital day 2 after the patient reported, “I want to strangle myself with my oxygen cord.”\nOn initial evaluation, the patient reported history of anxiety treated previously by her primary care physician (PCP). She reported she had been frustrated with her medical condition but really did not intend to harm herself. She reported fluctuating mood since her initial stroke and had “good days and bad days.” She denied prior history of inpatient or outpatient psychiatric care or prior suicide attempts. She was oriented to person and place, but not time, was able to state the days of the week forwards, but not backwards, and endorsed visual hallucinations during her hospitalization. This presentation was felt to be consistent with delirium, and she was started on quetiapine 25 mg.\nFollowing a six-day medical admission, the patient was discharged to the acute inpatient rehabilitation unit housed within the hospital. Extensive diagnostic studies did not reveal an underlying etiology for the strokes, which were thought to be due to uncontrolled hypertension.\nPsychiatry was reconsulted by the rehab physicians for management of problematic behaviors. The patient exhibited ego-dystonic behaviors for which she would later apologize including repeatedly climbing out of bed, shouting for nursing assistance without clear need for help, shoving her fist into her mouth to induce vomiting, and periodic, purposeless screaming. These behaviors were disruptive to staff and other patients on the unit. While initially conceptualized as residual hyperactive delirium, her behaviors persisted and continued testing for underlying causes of delirium including electrolyte derangement, occult infection, new or evolving cerebrovascular event, or excess medication burden which were unrevealing\nAfter 60 days of acute rehab, she had reached maximal benefit of that intervention and continued exhibiting behaviors incompatible with nursing home disposition. The patient was then transferred to the university's geriatric psychiatry inpatient unit on an involuntary mental health commitment for behavioral management.\nIneffective medication trials prior to transfer included quetiapine (25 mg at bedtime and 25 mg several times daily as needed), mirtazapine (7.5 mg at bedtime), olanzapine (initial trial of 2.5 mg at bedtime and 2.5 mg several times daily as needed and a second trial of 15 mg and 2.5 mg several times daily as needed), buspirone (15 mg TID), divalproex (initial trial of 750 mg at bedtime and a second trial of 500 mg TID with lactulose and levocarnitine for hyperammonemia), melatonin (9 mg at bedtime), propranolol (40 mg QID), trazodone (150 mg at bedtime), gabapentin (200 mg several times daily as needed), dextromethorphan (20 mg BID, given as Robitussin), and clonazepam (0.5 mg AM and 1 mg PM).\nThroughout this period, the patient remained intermittently apologetic for her behaviors. Orientation was typically attuned to person, sometimes place, and generally not to month or year. She consistently denied depressed mood, anxiety, visual hallucinations, auditory hallucinations, paranoia, suicidal ideation, or homicidal ideation. Thought process remained concrete and perseverative with limited spontaneous speech output and paucity of thought content. Language remained intact without evidence of aphasia. Recent and remote memory were difficult to assess formally due to behavioral disturbance, but she had difficulty remembering recent details of her hospital course and remote details of her life prior to moving to her current city. She required staff assistance for completion of toileting, dressing, and feeding. She had deficiencies in executing complex motor tasks, such as getting out of bed, and was frequently found diagonal in bed with a limb tossed over the side-rail. These deficiencies were in excess of the residual motor effects of her strokes and suggestive of alterations in visuospatial skills, executive function, and planning. Her aberrant vocalizations did not appear goal-oriented and were not ameliorated by staff presence. This presentation persisted and was thought to represent a new cognitive baseline meeting diagnostic criteria for major vascular neurocognitive disorder with behavioral disturbance.\nNonpharmacological strategies including music, sensory stimulation, one to one time with staff, and frequent repositioning were tried without improvement in her symptoms. Additional ineffective medication trials following transfer to inpatient psych included fluoxetine (60 mg per day), retrial of dextromethorphan with fluoxetine as an enzymatic inhibitor (again to 20 mg BID), retrial of quetiapine (up to 600 mg total per day), haloperidol (5 mg several times daily as needed IM), oxycodone (5 QID), lorazepam (up to 6 mg daily), carbamazepine (200 TID), and chlorpromazine (50 QID). Throughout these trials, the patient continued to exhibit frequent periods of severe psychomotor agitation requiring vest restraint and purposeless screaming alternating with periods of oversedation following medications. Other than providing intermittent sedation, no particular combination of medications proved effective in treating the target symptoms.\nAt this point, having exhausted all reasonable behavioral and pharmacologic options, the inpatient psychiatric team recommended ECT as a last intervention prior to pursuing a palliative approach. Medical Ethics was consulted and felt ECT to be consistent with her previously articulated beliefs and wishes.\nThe patient was formally evaluated by the ECT service and, given her incapacity to consent, a court order was obtained for the procedure. She underwent an acute course of bitemporal ECT using a MECTA Spectrum 5000Q machine. She received methohexital and succinylcholine as anesthetic and relaxant agent, respectively. A dose-titration method was used to determine stimulus intensity. She received treatments at 50% over seizure threshold with the following parameters: pulse width: 1 millisecond, frequency: 20 Hz, duration: 2 sec. Treatments were given three times per week. She was maintained on chlorpromazine (50 mg QID) and lorazepam (1 mg QID) during the treatments. Following the sixth ECT treatment, the patient rarely engaged in purposeless yelling, and remained quiet most of the day, experienced normalization of her sleep wake cycle, but still exhibited purposeless movements and psychomotor agitation requiring a vest restraint at night.\nFollowing the third week of ECT treatments, she was consistently having low scores on the Pittsburgh Agitation Scale (PAS) and had minimal requirements for as needed medications for agitation []. While she still required a vest restraint overnight, her psychomotor agitation had improved dramatically. She resumed feeding herself with her right arm and tolerated pureed foods for the first time in six months. Following an acute course of 16 treatments, ECT was tapered to twice weekly and she started sertraline 25 mg in preparation for further decrease in ECT frequency. She remained stable and was successfully discharged to a nursing home with continuation of ECT as an outpatient. Following the expiration of the original court order for ECT, outpatient ECT was discontinued and the patient's family chose to not pursue a renewal of the order for continued treatment. She received 29 treatments in total. Nursing home staff reported that her behaviors remained in control after stopping ECT and she was thereafter able to return home with her parents.
A 59-year-old gentleman with end-stage kidney disease due to hepatitis C with focal segmental glomerulosclerosis, on hemodialysis for seven years, underwent deceased donor renal transplantation. The donor kidney had a KDPI of 60%. There was a 4 antigen HLA mismatch with Class II panel reactive assay (PRA) of 62.31%. Class I PRA was 0%. His donor specific antibody testing was positive for an antibody to DQ7. The donor had died as a result of anoxic brain injury following cardiopulmonary arrest resulting from anaphylactic shock. The cold ischemic time was 12 hours, 7 minutes and the warm ischemic time was 51 minutes. Basilixumab was used for immunosuppression induction and the patient underwent early steroid withdrawal. His maintenance immunosuppression was tacrolimus (targeting trough levels 5-8) and mycophenolate sodium 720 mg BID.\nThe patient's postoperative course was complicated by delayed graft function, necessitating continuation of renal replacement therapy. He was hemodynamically stable throughout his hospital course. A renal allograft ultrasound showed good blood flow to the entire kidney with a resistive index of 0.64. A repeat flow crossmatch was negative, but he remained positive for a persistent low-level donor specific antibody to DQ7 (1000 MFI). He underwent a renal transplant biopsy on his 4th day postoperatively. This showed extensive acute tubular necrosis with associated peritubular capillaritis and interstitial nephritis (). Oxalate crystals were seen in several tubules. One large caliber artery showed active endothelialitis, but no tubulitis or glomerulitis seen. C4d staining was negative in the peritubular capillaries. Electron microscopy revealed minimal effacement of podocyte foot processes. The patient received methylprednisolone 500 mg x 3 doses to treat moderate acute cellular rejection. His tacrolimus dose was also optimized as his trough levels had been running low at between 3.5 and 6. He continued to take mycophenolate sodium at a dose of 720 mg BID. A decision was made not to treat for antibody-mediated rejection given that no glomerulitis was seen and that there was minimal capillaritis with a negative C4d stain.\nHe remained dialysis dependent with minimal urine output for three weeks after transplantation. A urine protein : creatinine ratio was elevated to 1070 mg/g when assessed after hospital discharge when his urine output started to gradually improve. His urine microalbumin : creatinine ratio was 450 mg/g. A decision was ultimately made to readmit the patient on day 12 after transplantation for IV thymoglobulin 1.5 mg/kg to treat his Banff Type IIa T cell mediated rejection, given his lack of response to pulsed IV steroid treatment. Given lack of improvement in renal function and urine output by day 16 after transplantation, a second renal allograft biopsy was performed. This revealed residual acute tubular necrosis with associated mild peritubular capillaritis and interstitial nephritis (). Extensive oxalate crystals were now visible in many tubules. His plasma oxalate level was concurrently elevated to 19.3 μmol/L (reference range ≤ 1.9 μmol/L). No signs of persistent acute antibody- or cell-mediated rejection were seen. Again, there was minimal segmental effacement of the podocyte foot processes seen on electron microscopy and no evidence of recurrent focal segmental glomerulosclerosis present.\nHe commenced calcium citrate along with dietary oxalate restriction to manage his hyperoxaluria and his serum creatinine improved to a nadir of 1 mg/dl (88 μmol/L), with a concomitant gradual reduction in his proteinuria and microalbuminuria to undetectable levels. Of note, the patient had no history of malabsorptive intestinal disease and denied any GI symptoms throughout this time period. He had never suffered from renal calculi. At three weeks after transplantation, his urine output and creatinine clearance had recovered sufficiently to enable him to become dialysis independent. A decision was made to continue low-dose oral steroids in the long-term given the presence of early acute cellular rejection on his first allograft biopsy. An interval renal biopsy performed 2 months later showed no ongoing evidence of oxalate deposition, tubular necrosis or cellular rejection. A concurrent repeat plasma oxalate level showed interval reduction to the normal range.
A 43-year-old male with a past medical history of heart murmur since the age of 11, obesity (BMI = 39.6 kg/m2), hypertension, and aortic valve disease was admitted for elective cardiothoracic surgery. The patient had previously been diagnosed with heart failure and severe aortic insufficiency via transesophageal echocardiography. The patient subsequently planned to undergo reconstruction of the ascending aorta and replacement of the aortic valve.\nOn the day of surgery, the patient was in his usual state of health with no signs of infection. The patient had no musculoskeletal symptoms and had never had shoulder complaints or undergone shoulder surgery. In the operating room, the patient was placed under general anesthesia using propofol as an induction agent and remained supine in a Skytron surgical table for the entirety of the surgery lasting three hours and 36 minutes. The surgery included reconstruction of the ascending aorta and the proximal arch with graft and prosthetic aortic valve replacement, followed by sternal closure with wires and a plate. There were no intraoperative complications and the patient’s mean arterial pressure was maintained at 70 mmHg throughout the surgery. The patient did not suffer any spikes in temperature or have any episodes of increased muscle rigidity.\nTwo days following surgery, the patient complained of bilateral shoulder pain and was unable to raise his arms above his head due to weakness and pain. He denied ever having experienced similar symptoms before. The patient had been restricted to bed rest and was continuously monitored via telemetry on the cardiac floor postoperatively. Upon orthopedic consultation, the patient was afebrile, normotensive, and in no acute distress. The patient’s chest, back, shoulders, and upper extremities displayed no bruising or break in the skin and were soft to palpation. The patient had a full passive range of motion (ROM) but displayed weakness abducting against resistance in the bilateral upper extremities. The patient denied focal tenderness to bilateral upper extremities and sensation was intact to light touch in the distribution of bilateral median, ulnar and radial nerves. The patient did not display tenderness upon palpation of the cervical spine and did not have radicular symptoms.\nThe patient’s creatine phosphokinase (CPK) level was checked two days postoperatively and noted to be 14,990 U/L (normal range male: 60-400 U/L; normal range female: 40-150 U/L) with a blood urea nitrogen (BUN) of 31 mg/dL (normal range: 6-23 mg/dL), creatinine of 0.9 mg/dL (normal range: 0.5-1.2 mg/dL), and potassium of 4.7 (normal range: 3.5-5.0 mmol/L). The patient subsequently received additional intravenous (IV) hydration. Magnetic resonance imaging (MRI) of the bilateral shoulders showed intramuscular hemorrhage involving the bilateral supraspinatus muscles (Figures -). There was also prominent intramuscular edema of the infraspinatus and teres minor muscles bilaterally, with mild intramuscular edema in the posterior deltoid and trapezius muscles bilaterally. These findings in addition to a markedly elevated CPK level were consistent with a diagnosis of RML. Of note, our patient was on Lovenox (Enoxaparin), 40 mg SQ daily which is standard of care for a patient after this type of cardiac surgery.\nThe patient was treated with a continuous IV infusion of normal saline for six days. His CPK levels consistently trended downward from 14,990 U/L on postoperative day 2 to 127 U/L by postoperative day 12; creatinine levels remained within normal limits and BUN levels were slightly elevated throughout the postoperative course (Table ). Physical therapy was ordered and the patient performed ROM exercises which aided in alleviating his functional restriction. Additionally, analgesics were given for pain. By postoperative day 12, the patient’s pain and weakness had completely resolved and there was no clinical or laboratory evidence of persistent RML.
A 64-year-old pure Japanese male with end-stage idiopathic chronic kidney disease, scheduled to receive a living ABO-incompatible renal transplant from his wife, was referred to our renal transplant center in April 2012. His past medical history included angina pectoris and cholecystitis. Physical and laboratory findings were normal, except for the abnormal findings related to end-stage renal disease. The protocol for antibody removal before the transplant consisted of double-filtration plasmapheresis and rituximab. The induction immunosuppressive regimen included a combination of prolonged-release tacrolimus, mycophenolate mofetil, methylprednisolone, and basiliximab. Renal transplantation was performed in December 2012. The postoperative clinical course was normal, and the patient was discharged 21 days after the renal transplantation, with a serum creatinine level of 0.78 mg/dl. Three months after the renal transplantation, the patient was admitted to our hospital with fever and general fatigue. Clinical examination, including chest and abdominal computed tomography, blood and urine culture, and cardiac ultrasound, revealed no abnormal findings, and the symptoms improved after antibiotic treatment. Three months later, the patient complained of fever and macroscopic hematuria. Cystoscopic examination revealed edema and bleeding in the left anterior region of the bladder (Fig. a). Because cold biopsy of the abnormal mucosa showed no malignant cells on hematoxylin–eosin staining (Fig. b), at that time, the bladder abnormality was suspected to be caused by viral hemorrhagic cystitis. The macroscopic hematuria stopped 3 days after dose reduction of the immunosuppressive drugs. Subsequently, the patient presented with intermittent fever, which resolved spontaneously within a few days. In May 2014, he presented with fever, general fatigue, and urinary retention and was re-admitted to our hospital. Ten days after admission, he required an artificial respirator and was transferred to an intensive care unit in circulatory shock. Because the precise cause for the aggravation of his general condition, frequent episodes of fever, and continuation of general fatigue was not clarified, his wife offered more details about the patient’s medical history. She stated that he had recurrent high fever since the age of 40 years and that his younger brother was suspected to have a familial autoinflammatory syndrome. After written informed consent, the patient’s serum sample was sent to the Department of Pediatrics at the Kyoto University Graduate School of Medicine for screening mutations and polymorphisms associated with hereditary autoinflammatory diseases. The ethics committee of the Akita University School of Medicine approved the use of human samples in the present study. During waiting the results of the genetic test, the patient continued rehabilitation at a related hospital. However, 2 months after discharge from our hospital, he presented with fever and heart failure and was re-admitted. At that time, he was found to be homozygous for the Mediterranean fever (MEFV) mutation (M694V/M694V) (Fig. ). Based on the presence of the MEFV mutation and the patient’s history, he was diagnosed with FMF. Treatment with colchicine was planned, but the patient died from heart failure in October 2014. We re-evaluated the pathological findings of the various tissue biopsies obtained during his treatment after the renal transplantation because FMF is known to be associated with systemic amyloidosis. Immunohistochemistry revealed amyloid deposits in the abnormal bladder region (Fig. c, d), transplanted kidney, and myocardium, which was diagnosed as AA amyloidosis associated with FMF.
The first case shows a 30 year old female. Clinical and radiographic examination showed a skeletal Class II pattern with an anterior open bite and a transverse maxillary deficiency with a lateral posterior crossbite on the right and the tendency to a lateral crossbite on the left (Fig. ).\nThe treatment plan involved a first stage of maxillary expansion with SARME to correct the transverse discrepancy followed by the leveling of the dental arches with lingual fixed appliances and finally two jaw surgery to correct the open bite as well as the Class II malocclusion.\nImpressions of the upper and lower arches were obtained for the lingual appliances.\nDuring the planning for the production of the lingual brackets, it was noted that a surgically assisted rapid maxillary expansion takes place. In the set-up, therefore, the transverse width of the upper jaw should be adapted to the lower jaw.\nFour Benefit mini-implants were inserted: two in the anterior area of the T-Zone and two 12 mm distally on each side of the midpalatal suture. A silicon impression was taken and the laboratory analogues were placed on the transfer caps. The maxillary expansion appliance was manufactured using a Hyrax screw anchored only to the four mini-implants, named the Quadhyrax.\nDuring the same appointment the lingual appliance was indirectly bonded using a dual cured composite (Fig. ) and the Quadhyrax was inserted and attached to the mini-implants using Benefit fixation screws. The first lower arch wire 14 NiTi was placed while the upper brackets were securely ligated with a continuous steel ligature in each quadrant to prevent accidental dislodgement during surgery (Fig. ).\nThe surgery for SARME was performed on all three patients according to the same procedure: First Le Fort I osteotomy with an oscillating saw. After that, the sutura palatina mediana was chiseled up for the midpalatal split. The tuber region was also mobilized with a chisel for the complete pterygomaxillary disarticulation. The appliance was activated intraoperative to evaluate the individual expansion of both sides of maxilla. After that the aplliance was resetted to reach a final gap of 1 to 1.5 mm.\nAfter surgery and a latency of a few days rapid maxillary expansion commenced with an activation rate of two quarter turns twice a day until the crossbite was corrected []. In all three cases one quarter turn corresponded to 0.2 mm. At four turns a day this was equivalent to 0.8 mm.\nA central diastema developed and expansion was complete two weeks after surgery. The Hyrax screw was then blocked for retention. Four weeks after surgery the first maxillary archwire 14 NiTi was placed to begin the alignement and leveling phase. The active closure of the central diastema started at about ten weeks post-surgery once enough bone had started to form for the incisors to move into. Because of the typical mushroom shape of the customized lingual appliances, the archwire has to be swiveled using tandem mechanics in front of the canines until the spaces are closed (Fig. ). The Quadhyrax was removed after six months. One mini-Implant was lost during removal of the expander and the remaining implants served as skeletal retention (Fig. ). The basal expansion of the maxilla worked well however the tooth-bearing segments of the maxilla showed some palatal tipping (Fig. ). After successful leveling and radiographic re-examination the second surgery was performed to correct the open bite and the Class II malocclusion.\nThe open bite could be closed. The patient has a positive overbite and overjet of 1.5 mm and shows a good transversal and sagittal occlusion.
A 38-year-old male complaining of recurrent episodes of melena for the past 2 weeks was admitted to the emergency department of our hospital. Two days before the hospital visit, the patient had complained of the presence of blood in vomiting and had a history of recurrent complaints of upper abdominal pain often radiating to the back. His previous medical history revealed that he had suffered from chronic pancreatitis for 3 years before present admission, possibly due to alcohol abuse. He was resuscitated with the blood and intravenous fluid for melena during present admission. Ultrasonography (USG) whole abdomen revealed a moderately enlarged liver (18.5 cm × 12.3 cm) with heterogeneous echotexture, with a prominent portal vein, of size 1.3 cm; hepatic and splenic hilar collaterals were suggestive of portal hypertension. The presence of mild ascites was documented. Transabdominal USG showed echogenic pancreas with multiple, small, hyperechoic, nonshadowing foci in the pancreas suggestive of fibrotic changes. There was evidence of irregular dilated main pancreatic duct with pancreatic and intraductal calculi. These features were consistent with chronic pancreatitis. The findings were suggestive of alcoholic liver disease with portal hypertension and chronic obstructive pancreatitis. After resuscitation, upper GI endoscopy was performed on two different occasions that showed bleeding from the second part of the duodenum with an abnormal tortuous vessel at the ampulla of Vater. This was diagnosed as hemosuccus pancreaticus, which might have caused the bleeding []. Serendipitously during the endoscopy, a worm was also found penetrating the mucosa of the duodenum. The worm was retrieved and referred to the laboratory for identification. It was a single intact live worm (size 12.3 mm × 0.3 mm) reddish pink, with head bent in the same direction of the body curvature with a spine at the posterior end []. The buccal capsule had six teeth (four hook-like on ventral surface and two knob-like on dorsal surface) []. The live worm was seen to lay eggs while examining under the microscope []. Three consecutive stool samples of the patients were examined. The stool sample was dark-colored, semi-solid in consistency without any parasitic elements. Wet mount examination of stool revealed nonbile-stained ova (60– 65 μm in length, 40– 45 μm in width) with transparent hyaline shell membrane containing blastomeres. The above morphological features of the worm and micrometry of the ova were consistent with Ancylostoma duodenale. For further confirmation and to exclude the probability of mixed infection with Strongyloides stercoralis, the modified Harada– Mori nematode larval culture method[] was performed, and larva of hookworm was retrieved after 1 week of incubation at ambient room temperature. However, duodenal biopsy could not be done to rule out this possibility.\nThe patient was a chronic alcoholic and had deranged liver functions. Other laboratory test revealed low hemoglobin (5.6 g/dL), elevated total leukocyte count (12,300/μl) with increased eosinophils (neutrophils - 54%, lymphocytes – 24%, eosinophils – 16%, and monocytes – 6%), hyperbilirubinemia (total serum bilirubin 12.5 mg% and conjugated bilirubin 11.7 mg%), alanine transaminase/aspartate transaminase (87.7/90 IU), serum alkaline phosphatase (827 IU/L), hypoalbuminemia (2.3 g%), and raised serum globulin (3.9 g%) with reversal of albumin:globulin ratio. Blood and urine cultures were bacteriologically sterile. Peripheral blood smear examination showed microcytic, hypochromic anemia, with the absence of any hemoparasites. The patient was planned for contrast-enhanced computed tomography (CECT) abdomen, but the course of illness was rapidly fatal, and he succumbed to the illness within 48 h of admission, hindering further investigative course. The cause of death was unexplained shock secondary to upper GI bleeding precipitated by chronic, alcoholic liver disease with chronic pancreatitis and portal hypertension.
A 67-year-old woman reported an accidental injury to the right eye from a falling chestnut. She visited an eye clinic nearby, and was treated with ofloxacin ointment. On the next day, cell infiltration was observed around the corneal wound, suggestive of deterioration of the corneal infection. Therefore, she was referred to our hospital.\nThe patient complained of mild pain. At first visit, her best-corrected visual acuity (BCVA) was 0.8 in the right eye and 1.5 in the left eye. Slit-lamp examination showed a perforation at the center of the right cornea; leakage of aqueous humor through the perforation was observed (Fig. ). The Seidel test, which evaluates aqueous leakage on the cornea using fluorescein, was positive, although the anterior chamber was deep (Fig. ). Cell infiltration was observed around the wound with conjunctival injection, and moderate cells were observed in the anterior chamber. No foreign body was found in the conjunctival sac, cornea, or anterior chamber. The iris was damaged, and the anterior lens capsule was opacified with pigments (Fig. ). AS-OCT showed a corneal wound involving the endothelial layer (Fig. ). The central corneal thickness was 603 µm. Additionally, slight cataract and early pterygium were observed. A gonioscopic examination showed no hemorrhage or foreign body at the angle of the eye. No fundus abnormality was observed. The patient was in good general health with no significant past medical history. Bacterial examination of corneal scrapings showed gram-positive cocci and a few neutrophils, but no fungi.\nThe patient was diagnosed with a corneal perforation and bacterial keratitis. Levofloxacin 1.5% and cefmenoxime administration every 2 hours, atropine administration once a day, and oral acetazolamide were initiated. A soft contact lens that was made of hydroxyethyl methacrylate was placed on her right eye in order to seal the wound.\nOn day 2 after the injury, the Seidel test was negative and the use of the soft contact lens was discontinued. There was no progression of cataract. However, there was an increase in cell infiltration with conjunctival injection.\nOn day 3, folds of Descemet membrane appeared (Fig. ). BCVA of the right eye decreased to 0.7. There was no improvement in the corneal cell infiltration. Slit-lamp examination and a repeat AS-OCT suggested inner wound closure (Fig. ). The corneal endothelial cell density was 2,778/mm2 in the right eye and 2,793/mm2 in the left eye. Steroid therapy was withheld because fungal infection had not been ruled out.\nOn day 9, the patient reported no ocular pain. Slight cells were observed in the anterior chamber with slight conjunctival injection. There was improvement in cell infiltration. A culture test revealed the presence of Propionibacterium acnes (P. acnes), which is sensitive to both levofloxacin and cefmenoxime. The test was negative for fungi. Because both antibiotics were thought to be effective, we continued the same treatment and decreased the frequency to 6 times a day. Atropine and acetazolamide were discontinued.\nOn day 14, BCVA of the right eye had improved to 0.9. The opacification of the anterior lens capsule decreased with slight cells in the anterior chamber. However, cell infiltration at the deep corneal stroma persisted.\nOn day 26, the patient was completely asymptomatic. The opacification and cell infiltration at the center of the cornea had improved (Fig. ). AS-OCT showed healing of the corneal wound with reduction in the central corneal thickness to 523 μm (Fig. ). The corneal shape analysis suggested that the wound did not affect corneal irregular astigmatism (Fig. ). No progress of cataract was observed and BCVA improved to 1.0.
A 16-year-old boy was presented to our clinic complaining of a painless mass on his penis that was increasing in size. The fist-sized mass had a firm consistency without inguinal lymphadenopathy. The patient had been unaware of the existence of the mass until his urinary stream weakened. However, the mass had recently been growing rapidly with intermittent pain.\nMagnetic resonance images revealed a 5×5-cm mass invading the left corpus cavernosum and corpus spongiosum (). Several inguinal lymph node enlargements were found; however, their diameters were less than 10 mm, which suggested that they were reactive lymph nodes. On cystourethroscopy, the penile urethra appeared to have been compressed by an external mass and mucosal erythemas were found around the mass. There were no abnormal findings on the chest computed tomography (CT) or bone scan. Laboratory examinations including urine analysis showed nonspecific findings. Core biopsy of the mass revealed a small round cell tumor.\nTumorectomy was performed because the patient and his mother wished penile preservation. A circular subcoronal and ventral penile longitudinal incision was made. Although the tumor had rich vascularity, dissection between the tumor and the overlying penile skin was not difficult. The corpus cavernosum and the corpus spongiosum that were invaded by the tumor were partially resected and repaired. A large mass was identified on the penile shaft (). Pathological examinations revealed small round cell sarcomas with neuroendocrine differentiation (). The surgical margin appeared most likely to be free of tumor and there were multiple lymphovascular tumor emboli with poorly defined mitotic figures. Immunohistochemical study showed that reactivity to CD56 and vimentin was positive and reactivity to CD34, CD31, factor-VIII, cytokeratin, smooth muscle actin, CD99, and c-kit was negative. The reactivity to epithelial membrane antigen (EMA) was weakly positive.\nA follow-up chest CT performed 1 month after the surgery showed multiple nodules of less than 1 cm in size in both lung fields. These lung nodules were removed by use of CT-guided biopsy, which revealed metastatic small round cell sarcomas identical to the penile mass. Therefore, chemotherapeutic treatment was planned.\nAccording to the surgicopathologic staging system of the Intergroup Rhabdomyosarcoma Study Group, our case belonged to Group IV, and according to the International Union Against Cancer staging system, his case was classified as stage 4. The patient underwent chemotherapy with etoposide (100 mg/m2/day) and ifosfamide (1.8 g/m2/day) and regular CT control every 3 months. Despite chemotherapy, however, the size of the nodules did not change. We then changed the regimen of chemotherapy to vincristine (1.5 mg/m2/day, maximum dose 2 mg/day, weekly), actinomycin-D (1.5 mg/m2/day, maximum 2.5 mg/day, every 3 weeks), and cyclophosphamide (2.2 g/m2/day, every 3 weeks) because his cumulative dose of ifosfamide had exceeded 69.4 g/m2. Until week 6 of the second-line chemotherapy passed, there was no evidence of a decrease in the size of the lung nodules. However, wedge resection of the two nodules on right upper lobe was then performed and the specimens revealed no residual tumor cells. After a total of 42 weeks of chemotherapy and 34 months of follow-up, the patient showed no evidence of distant metastases.
The patient is a 46-year-old male who presented to the neurosurgery clinic with a one-year history of progressive right eye blurry vision, which he first noticed while serving as an umpire in a little league baseball game. He denied any headaches, although he had recently developed a painless pressure sensation behind the right eye. He had no contributory medical history (e.g. endocrinologic, vascular) to explain an optic mononeuritis, and there was no personal or family history of neurofibromatosis. An evaluation by neuro-ophthalmology showed severe degradation of the right eye visual fields with sparing only of the inferior medial field. Right optic atrophy was also appreciated on examination. Physical examination in the neurosurgery clinic was unremarkable for any focal neurological findings, and both of his pupils were equal, round, and reactive to light and accommodation. A T1-weighted MRI with contrast demonstrated a 4 x 5 mm homogeneously enhancing mass at the right orbital apex within the bony canal and along the medial aspect of the right optic nerve sheath with resultant mass effect on the right optic nerve (Figure ).\nThe differential diagnosis at this point was optic nerve meningioma versus optic nerve glioma. Optic nerve signal was also consistent with neuritis on the right. After discussion of the operative plan in the clinic, the patient agreed to undergo endoscopic endonasal surgery for bony and dural decompression with resection of the tumor if it was felt to be amenable intraoperatively. Informed patient consent was obtained prior to treatment.\nThe neurosurgery team worked with otorhinolaryngology (ENT) for the skull base approach. The right-sided ethmoid air cells were opened to expose the medial orbital wall. A posterior septostomy was performed on the right, and the face of the sella was widely opened to expose the tuberculum sellae and planum sphenoidale superiorly. We removed the most distal portions of the lamina papyracea on the right. The right-sided optic canal and carotid prominence beneath were clearly visible. Image guidance with CT and MRI with contrast confirmed our location within these landmarks. Additional bone was removed from the proximal orbital apex and along the optic canal with a high-speed, diamond-tipped drill and irrigation. In this manner, we exposed the dura of the optic canal from where it opened into the orbital apex back to its intersection with the tuberculum sella. This portion of the procedure constituted the bony decompression. We then directly inspected the exposed dura and used neuro-navigation as well as visual landmarks to determine the location of the medial optic canal lesion. Directly over this, we made a linear incision in the dura and immediately encountered tannish-gray tumor material. It was somewhat tough in consistency and firmly attached to the optic nerve. At this point, the patient’s wishes to conserve vision in the right eye if possible was heavily taken into consideration, and given the previously mentioned difficult characteristics of the tumor, it was subtotally resected. Nevertheless, some of the central portions were acutely decompressed and samples were sent for permanent pathology. Although no CSF leak was frankly noted, there was concern the patient could develop such a leak in the future because of the small opening that was made in the dura. Therefore, we performed a direct repair using a free nasoseptal graft.\nFollowing surgery, the patient did well with no apparent complications and was discharged on postoperative day two. The final permanent pathology specimens revealed a classic palisading pattern of tumor nuclei typical of schwannoma.\nThe specimen also stained positive for S100 and was negative for EMA (endothelial membrane antigen), thus confirming the diagnosis of schwannoma (Figure ).\nThe patient was seen in clinic three months after his procedure and endorsed subjective improvement in his right eye superior quadrants; however, his preoperative visual deficits would return. Postoperative MRI at this time showed an approximately stable lesion compared to preoperatively (Figure ).\nHe was instructed to see the neuro-ophthalmology and radiation oncology services for a new baseline examination and adjuvant radiotherapy versus observation of expected growth of the residual tumor. The patient chose not to follow up with ophthalmology. He ultimately completed a course of external beam radiation therapy (EBRT) for a total of 50.4 Gy in 28 fractions with IMRT to reduce the dose to the optic nerve and other critical intraorbital structures. He successfully completed this regimen without any new visual deficits nor complaints and is scheduled for annual MRIs and follow-up with neurosurgery or sooner if he experiences an interim visual decline.\nInformed patient consent was obtained for reporting this case in the neurosurgical literature.
A 55-year-old female (height, 120 cm) with OI type III (Sillence classification) was referred to us 9 months after failed treatment of a spontaneous right femoral neck fracture (). Diagnosis was based on clinical history, radiological and genetic findings. Clinical diagnosis included short stature, multiple fractures due to minimal or no trauma, delayed healing and nonunion of fractures and progressive deformities. Radiological key findings included deformities of the long bones with multiple fractures, coxa vara, protrusio acetabuli and very thin, deformed and osteopenic bones. Genetic testing showed mutation COL1A1 c.2461G>A p.(Gly821Ser) (heterozygous).\nInitial treatment had consisted of two cancellous screws. In addition a small bone biopsy was done at the request of her treating internist (with consent of the patient). Postoperative radiographs noted an iatrogenic fracture at the site of the bone biopsy (). Initially this was treated non-operatively with bedrest. However, pain was difficult to control and there were no early signs of healing. At 2.5 months she could not tolerate bedrest any longer. She was taken back to the operating room and plate fixation with cerclage wires was done (). She was followed regularly for 9 months. She then requested a referral to our department where she had been treated many years prior for a left femoral shaft nonunion.\nWhen we saw her she was taking 70 mg morphine orally, with continuous pain in her hip and hardly the ability to stand for transfer. Prior to her femoral neck fracture she was able to perform a short transfer from wheelchair to bed. Since the age of 7 years she was wheelchair bound. The last years taking morphine for progressive and severe pain and neurologic spasms related to spinal cord injury due to many vertebral compression fractures and severe scoliosis. Plain radiographs and a computed tomography (CT) showed a femoral neck nonunion as well as a subtrochanteric nonunion at the previous biopsy site, with additional deformities of pelvis and distal femur. There was very close contact between the posterior wall and the femoral neck nonunion site (). We planned a Pauwels type osteotomy to address both the femoral neck nonunion as the ipsilateral subtrochanteric iatrogenic nonunion. In addition we planned a distal femoral osteotomy to obtain better alignment of the lower aspect of the femur. She was placed supine on a radiolucent table. A lateral approach was done. All hardware was removed. We did not do a formal debridement of both nonunion sites. We first addressed the varus deformity of the distal femur. A lateral closing wedge osteotomy was done using an oscillating saw under cooling. A pelvic reconstruction plate was used with standard screws. The distal screws were drilled eccentrically to provide compression across the osteotomy site.\nNext we addressed the proximal femur with the femoral neck and subtrochanteric nonunion. We anticipated a Pauwels osteotomy of 40°. At the subtrochanteric nonunion site a closing wedge was removed from the lateral cortex. Using K-wires as joy-sticks the osteotomy was aligned. The best available fit for this deformed femur was a titanium Philos plate (DePuy Synthes, Amersfoort, the Netherlands). The plate was customized to fit on the proximal femur. Screws were placed after drilling in the femoral neck using an oscillating to prevent a fausse route. A fibular allograft was wedged in between plate and bone to increase bone stock and stability. The plate was then secured to the shaft (). Post-operatively there were no complications. At 5 months a CT showed healing of the femoral neck nonunion. She was so pleased with the increased range of motion of her right hip that the Pauwels osteotomy had given her that she requested a similar procedure on the left side, which was done 9 months after the right side. This osteotomy also healed with good results for the patient (). Seven months after the left Pauwels osteotomy (and 16 months after the right osteotomy) she sustained a left distal femur fracture for which she was treated with a medial sided Philos plate and allograft bone chips.\nAt 2.5 years after the last surgery on her right hip a CT was taken because of increasing right hip pain when standing. The CT showed fully healed osteotomies and consolidation of the femoral neck nonunion. The most posterior screw in the right femoral neck was partly out of the neck (before re-entry into the femoral head) and was impinging on the ischium because of increasing protrusion of the femoral head into the acetabulum. This screw was removed in addition to removal of part of the greater trochanter to limit impingement. At four and a halve year she had new onset pain in her left groin during sitting. A CT showed a fresh fracture of the left ramus inferior os pubis. The nonunion of her right femoral neck was noticed to be solidly headed (). There was full incorporation of the allograft and healing of the subtrochanteric iatrogenic/pathologic fracture ().
A 10-year-old girl presented with a 2-day history of constant right-sided flank pain with intermittent episodes of increased intensity. Movement and laying supine exacerbated the pain, whereas some relief was found with paracetamol. There was no trauma to the area, but the patient reported playing netball shortly before the pain started. There was no associated nausea, vomiting, or change in bowel or urinary habit. There was no fever and no symptoms of cough or breathing difficulties.\nThe patient had no significant medical history and immunisations were up to date. On examination, she was apyrexial with a heart rate of 110 beats per minute, oxygen saturations of 99% on room air and respiratory rate of 22 breaths per minute. She was warm and well perfused with a central capillary refill time of <2 seconds. She appeared distressed when laying down reporting worsening pain; however, the abdomen was soft and non-tender to palpation. Systemic examination was otherwise unremarkable.\nUrine analysis showed no evidence of infection and blood tests revealed a C-reactive protein (CRP) of 23 mg/L and a normal full blood count with white cells of 13.4 × 109 cells/L. Liver function tests, urea and electrolytes, and venous blood gas were also normal.\nWith normal observations, examination and investigation results and a good response to analgesia in the department, the patient was discharged with a planned review.\nOn re-assessment the following day, she continued to complain of significant pain on the right side of the abdomen with similar features on examination. Owing to the persistence of symptoms, chest radiography (Fig. ) and abdominal ultrasound were performed. Both were reported as normal. With no surgical or medical cause of the pain identified, it was deemed musculoskeletal in origin, associated with playing netball. She was discharged with advice to take regular analgesia and to return if symptoms were not settling.\nThe patient represented the following day looking unwell. She appeared very pale with dry lips and walked slowly, hunched over. She was tachycardic at 130 beats per minute and tachypnoeic with a respiratory rate of 32 breaths per minute. Oxygen saturations, temperature, and blood pressure were normal at 98%, 36.80C, and 119/72 mmHg respectively. She stood with her right hip and knee slightly flexed, with her trunk deviated to the left giving her a marked scoliosis. Musculoskeletal examination of the spine, hips, and knees was unremarkable, but it was noted that abdominal pain worsened on flexion of the hip against resistance. Cardiovascular and respiratory examinations were normal. She was unable to lie supine because of severe pain and was examined in the semirecumbent position. Active distension of the abdomen and coughing further exacerbated the pain and she was found to have guarding with rebound tenderness in the right flank and hypochondrium. An intravenous cannula was inserted and intravenous fluids, paracetamol, and ibuprofen were commenced while awaiting a surgical review for her acute abdomen. Concurrent orthopedic opinion was also sought because of the new-onset scoliosis.\nAfter treatment the patients’ pain was significantly reduced, pallor had resolved and her observations normalised. Abdominal examination at the time of surgical review was normal, but because of repeated presentations with abdominal pain and a rising CRP (93 mg/L) and white cell count (13.9 × 109 cells/L) the patient was admitted and a computed tomography (CT) scan of the abdomen and pelvis was arranged. Orthopedic review did not yield any acute orthopedic concerns regarding the scoliosis and they agreed with the plan for abdominal imaging. The CT scan failed to reveal an abdominal cause for her pain, but to our surprise showed an organizing right lower lobe pneumonia with a small effusion (Figs. and ).\nThe patient was started on intravenous antibiotics for the thus far silent pneumonia. Despite treatment during the next few days, the pneumonia progressed and she developed a large parapneumonic effusion (Figs. and ). On the 6th day of hospital admission, she deteriorated rapidly with signs of sepsis and respiratory distress. This was accompanied by a further rise in CRP (351 mg/L) and white cell count (17.3 × 109 cells/L). She was stabilized and then transferred to a tertiary hospital for further management.\nAt the tertiary center, a chest drain was inserted and the patient was treated with intrapleural urokinase therapy and intravenous antibiotics. She was discharged after a 2-week stay.\nWith the complete resolution of the pneumonia and scoliosis, the patient made a graduated return to school and at 4 months post discharge was almost back to full-time attendance.
This case describes a 65-year-old Caucasian man who presented to our clinic with a six-year history of severe chronic lumbago with neurogenic claudication leading to the inability to walk more than a few steps. His past medical history included anxiety, atrial fibrillation after past ablation, coronary artery disease after percutaneous coronary angioplasty with three stents, gastroesophageal reflux disease, hypertension, obstructive sleep apnea, and hyperlipidemia. His pain was mainly axial and involved the lumbar spine with radiation to bilateral anterolateral thighs. He endorsed a history of over 100 falls during his years as an ice skater but described the onset of his pain as gradual. The patient had no history of any of the reported risk factors of OLF in literature, like nephrolithiasis, hypercalcemia, or kidney disease and his laboratory values for serum calcium, creatinine, and glomerular filtration rate were within normal limits. Prior interventions included physical therapy, acetaminophen, nonsteroidal anti-inflammatory drugs, pregabalin, and multiple caudal epidural steroid injections over the years with only marginal benefit. MRI revealed the etiology of his lumbar radiculopathy to be severe lumbar spinal stenosis.\nOn examination, the patient was sitting. He could only stand for a few seconds upright before leaning forward and taking just a few steps before he had to sit due to cramping in both legs attributed to neurogenic claudication. He exhibited 5/5 strength in his bilateral lower extremities with no signs of muscle wasting or atrophy. His deep tendon reflexes were 2/4 in the bilateral lower extremities. The facet loading test was positive bilaterally in the lumbar region. MRI of the spine revealed central canal narrowing and bilateral neuroforaminal narrowing from L3-5. At L4-5, there was a broad-based posterior disc bulge with annular fissuring of the nucleus pulposis and mild facet arthropathy. In retrospect, ligamentum flavum ossification was suggested with T2- and T1-weighted images (Figures -).\nThe patient underwent endoscopic intralaminar laminotomy with partial facetectomy and lateral recess decompression with immediate resolution of his symptoms. Intraoperatively, we identified OLF at the L4-5 that had not previously been noted on MRI.\nThe patient underwent uneventful induction of general anesthesia and neuromonitoring was performed via the quadriceps, anterior tibial, gastrocnemius, and hamstring on the right side. A true anteroposterior view of the lumbar spine at the L4-5 level was obtained by using fluoroscopy. A working channel was established and the spinal endoscope was introduced. The descending lamina was identified in the right L4-5 interlaminar window and a laminotomy was created over the right-sided descending lamina down to the descending facet. Once at the ligamentum flavum, the dorsal fibers were taken down, revealing ventral fibers of the ligamentum flavum that were unexpectedly found to be hardened and ossified across the interlaminar window at L4-5 (Figure ). The hardened ossified shell of the ligamentum flavum was carefully dissected. The dura was fully decompressed and freely mobile, without signs of ossification. Once adequate hemostasis was visualized, 40 mg of methylprednisolone was injected at the site and the incision was closed.\nThe patient reported immediate improvement of his radicular symptoms with no neurological deficits in the postanesthesia care unit. The patient presented to the clinic for a follow-up on postoperative day 9 with sustained improvement and was neurologically intact.
An 80-year-old woman presented with a history of intermittent, severe epigastric pain. Over the preceding 5 months, she had less severe and self-resolving epigastric pain 15–30 min after every meal. The full blood count, serum biochemistry values, and C-reactive protein level were within normal ranges. Additionally, arterial gas analysis was normal. Abdominal plain radiography showed free air between the liver and diaphragm, and PI (Fig. ). Abdominal computed tomography showed severe calcification of the SMA origin; SMA peripheral flow was reserved (Fig. ). Computed tomography (CT) revealed bubble-like intramural gas of the small bowel with the contrasted wall pneumoperitoneum (Fig. ).\nSince the patient did not have peritonitis, a conservative approach was performed. She was managed in the condition of intensive care unit, due to fears of the potential for acute mesenteric ischemia due to mesenteric artery occlusion or non-occlusive mesenteric ischemia. She was managed for bowel obstruction, which included fasting and intravenous fluid administration. She received heparin infusion to prevent SMA occlusion and maintain collateral flow. During admission, she reported abdominal pain relief. Seven days after admission, abdominal plain radiography showed improvement in PI and pneumoperitoneum; therefore, she was permitted to begin drinking fluids. There was no evidence of recurrent abdominal pain after the fluid consumption, so she was allowed to eat solid food.\nAngiography was planned to relieve the postprandial abdominal pain. The findings showed severe stenosis of the SMA origin with calcification, and the SMA had replaced the common hepatic artery (Fig. , ). ET, namely stenting to the SMA and common hepatic artery, was performed. The SMA trunk was engaged with a 6F Parent Plus 60 guiding catheter (Medikit, Tokyo, Japan) from the left brachial artery. Initially, 8000 units of heparin was infused, and additional heparin was added to keep the activated whole blood clotting time over 200 s. The SMA occlusion was traversed using a 0.014” NEO EVT Guide Wire Cruise (ASAHI INTECC J-sales, Tokyo, Japan). The SMA trunk to the hepatic artery was traversed using a 0.014” NEO EVT Guide Wire Cruise, which was engaged with a 6F SheathLess PV (Cardian Health, Ohio, USA) from the right brachial artery. Intravascular ultrasonography (IVUS) (Navifocus WR, TERUMO, Tokyo, Japan) revealed severe stenosis of the SMA trunk with calcification. Balloon dilation was performed with the kissing ballon technique using a 4-mm Coyote ES (Boston Scientific, Marlborough, MA, USA) to the SMA and 5-mm SHIDEN RX (KANEKA MEDICAL, Osaka, Japan) to the common hepatic artery. After dilation, two balloon-expandable stents (5-mm PALMAZ Genesis (Cardian Health, Ohio, USA) to the SMA and 6-mm PALMAZ Genesis to the common hepatic artery) were implanted with the kissing stent technique from the common ostium (Fig. ). The sizes of the stents were based on the IVUS measurements. IVUS showed good orifice expansion. Final angiography showed satisfactory circulation to the terminal organ from the orifice (Fig. ).\nEndovascular therapy relieved the patient’s symptoms. She required dual anti-platelet therapy (DAPT) with aspirin and clopidogrel post-intervention. Follow-up computed tomography showed patency of the orifice of the common hepatic artery and SMA, and there was no evidence of PI or pneumoperitoneum. No recurrent postprandial abdominal pain occurred for two years after the ET.
A 2-year-old male child was brought to our hospital by his father with complaint of a mass in right mid-axillary area for about 1-month. He weighed 12.5 kg and was 85 cm tall with head circumference of 48 cm. He was the last of two siblings without any previous family history of any congenital deformity or anomaly.\nMilestones were delayed as child was not able to walk or stand without support till presentation to the hospital. The mother denied any history of consanguineous marriage, drug or radiation exposure. The mother neither had problems during pregnancy nor during delivery. Immunization was complete till date. On first look, the child had intense hemipigmentation on the left side of the trunk [] and congenital deformity on the left side of the face and left limbs.\nThe physical examination revealed a mentally healthy and active boy. The patient's face was asymmetrical with an enlargement on the left side including the maxillary and mandibular region and large forehead []. It was clear that there was a unilateral enhancement of development on this side. The tissue surrounding the tooth when compared with the right side was enlarged. The skin and hair appeared normal. Tongue appeared normal. Speech was not impaired. The general examination revealed asymmetrical development of the extremities and the trunk. On examination, he was found to have grade 5/5 power on the both sides. However, there was visible hypertrophy of left lower limb including calf, thigh, and foot and left upper limb. His left lower and upper limbs were longer than right limbs significantly showing evidence of LLD [ and ]. There was no evidence of syndactyly, macrodactyly, café-au-lait spots, naevi, hyper extensible skin, vascular malformations. His mouth opening was adequate, and the modified Mallampati classification could not be assessed as child was uncooperative. Preoperative emergency tests revealed neutrophilic leukocytosis and hemoglobin was 8.0 g%. Viral markers were negative. Systemic examination appeared normal. Neck movements were normal, and spine was normal. High-risk consent was taken from the parents, and patient was accepted in American Society of Anesthesiologists Grade III.\nIn view of the difficult airway, parents were informed that the technique might fail, and tracheostomy consent was taken. A trolley for difficult airway was kept ready including laryngeal mask airway and tracheostomy set. On presentation, the child was already nil by mouth for the whole night that is, 6 h. We planned to go for general anesthesia under mask without intubation as patient could not be assessed for any congenital or cardiovascular anomaly in view of the emergent nature of the surgery. The child was crying and uncooperative. Hence, we premedicated with 60 mg ketamine with 0.2 mg glycopyrrolate via intramuscular route in the preoperative area in mother's lap. On loss of consciousness, the child was carried into operation theater, and all monitors attached. He was given injection ondansetron 1.5 mg and injection fentanyl 25 μg. Subsequently, he was taken on mask ventilation and maintained with oxygen and nitrous ratio 30:70 throughout the surgery. On completion of surgery, he was maintained on 100% oxygen till awake and shifted to the postoperative ward under close observation. The postoperative course was normal. He was referred to the pediatric and dermatology department for the establishment of diagnosis, but the child's father was not ready for any evaluation. He was subsequently discharged on 3rd postoperative day.
A 55-year-old man presented to the otolaryngology clinic on referral from his primary care provider for a chief complaint of tongue pain. He reported 30 years of right-sided tongue pain without an inciting factor or event, which over the preceding 10 years had developed into an open wound. This was accompanied by generalized radiation of his pain into his head and neck. He had no other significant medical history and was an active cigarette smoker with a 14 pack-year history. He reported heavy alcohol use as a young adult but had been alcohol free for several years.\nPhysical examination revealed a 2.5-cm ulcerative lesion of the anterior right oral tongue with central necrosis, with palpable deep extension of abnormal tissue to the midline (). Full motor and sensory functions of the tongue were intact. No floor of mouth involvement was detected, and there was no palpable cervical lymphadenopathy. The remainder of the head and neck examination, including flexible fiberoptic nasopharyngolaryngoscopy, was unremarkable.\nPunch biopsy tissue obtained at the first clinic visit was reported to be most consistent with moderately to poorly differentiated squamous cell carcinoma with moderate p16 immunostaining. A positron emission tomography (PET)/computed tomography (CT) scan was performed confirming the right tongue lesion (), and mildly hypermetabolic right level II nodes were visualized.\nThe patient underwent direct laryngoscopy with biopsies, bronchoscopy, partial glossectomy, and bilateral supraomohyoid neck dissection. Laryngoscopy, esophagoscopy, and bronchoscopy were normal. All intraoperative frozen section margins were free of disease. The glossectomy defect was closed primarily. His postoperative course was unremarkable, and he was discharged on postoperative day 5. At his first clinic follow-up on postoperative day 8, he was recovering well overall but had some minor dysarthria.\nAlthough the initial biopsy was most consistent with squamous cell carcinoma, the final specimen demonstrated findings inconsistent with this diagnosis, prompting further investigation with immunohistochemical staining. The final pathology analysis confirmed a 2.5-cm tumor with a thickness of 5 mm, consistent with MCC. The tumor itself comprised invasive mitotically active infiltrating nests of epithelioid cells, many of which had a rhabdoid appearance. These cells demonstrated a high degree of cellular atypia with eccentrically placed nuclei with prominent nucleoli and dense pink cytoplasm (), which combined with the infiltrative growth pattern confirmed a malignant tumor. There was little evidence of chondroid stroma or ductal differentiation, which assisted in ruling out pleomorphic adenoma. Immunohistochemical stains demonstrated positivity for pancytokeratin (), epithelial membrane antigen (EMA) (), calponin (), and glial fibrillary acidic protein (GFAP) (), consistent with myoepithelial differentiation. To differentiate between an MCC of soft tissue versus salivary gland origin, a fluorescence in situ hybridization (FISH) study using an EWSR1 break-a-part probe was employed. This study showed no evidence of an EWSR1 gene rearrangement, indicating that the tumor was likely of salivary gland origin. No perineural or lymphovascular invasion was identified. All margins were negative, and the bilateral neck dissection specimens showed no metastatic tumor. Final pathologic staging was T2N0M0.\nThe case was presented at multidisciplinary head and neck tumor board. Due to the early stage of the tumor and lack of high-risk features, no adjuvant therapy was recommended. At 3 months postoperative visit, he reported that his speech and swallowing were normal. He had resumed his pre-operative level of physical activity. Physical examination and indirect flexible nasopharyngolaryngoscopy were normal, with a well-healed surgical defect (). At his most recent evaluation, he had no evidence of disease 9 months after his surgery.

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