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pmc-6528436-2 | The second case is a 67-year-old woman with PD in Hoehn and Yahr Stage II (bilateral involvement without impairment of balance) and a disease duration of 12 years. Soon after retiring as a financial consultant in the field of education, she became afraid that the decreased working load on her brain would result in memory loss. A few years into the disease, she indeed started experiencing memory loss, which motivated her to train herself in daily real-life situations. Specifically, she has developed several daily routines to train her memory. After waking up, she tries to remember all meetings for the upcoming day. She, afterward, checks her calendar to see if she was right. Also, if she is outdoors and plans a new meeting with a friend, she will note it in her calendar only by the time she comes home. Afterward, she will check to see if she remembered the correct date and time. Also, she manually enters frequently used phone numbers, even though she has saved them as contacts in her smartphone. Furthermore, when she plans on shopping for groceries, she makes a shopping list on paper that includes all the needed ingredients. In the store, however, she will not consult this list, but instead she will buy all products from memory. The shopping list is ultimately referred to as a checklist for completion. This type of real-life training requires multiple cognitive functions, including planning and memory. The patient feels that these self-invented routines keep her memory stable at an acceptable level. She is now confident that she is able to remember almost anything, and she has never heard from others that she forgot something. Importantly, compliance was again excellent, as she has been using these daily routines for over 5 years now. |
pmc-6528436-3 | The third case is a 68-year-old woman with PD in Hoehn and Yahr Stage III and a disease duration of 10 years. She has been living in South Africa volunteering as a community development worker for 28 years and has raised 5 children. After returning to the Netherlands, she was diagnosed with PD in 2009. In the following years, she started noticing cognitive problems, including concentration and memory deficits. She applied to a Dutch Web-based Bridge game service (called StepBridge, by StepBridge Foundation, see ), where she could play Bridge against gamers of similar difficulty levels at any time this would fit her schedule. This Bridge game requires several cognitive functions, including attention, reasoning, decision making, and memory. She reports subjective benefits in terms of both concentration and memory, which is also observed by her spouse. Compliance was again outstanding, as she has been playing StepBridge regularly for almost 10 years now. |
pmc-6528661-1 | A 75-year-old female with history asthma, diabetes and breast cancer status post chemotherapy was admitted for abdominal pain, watery diarrhea, and subjective fevers for four days. She had received antibiotics for pneumonia two weeks prior. On physical exam, she was febrile (103°), tachycardic (128 beats/min) and blood pressure was 100/70 mmHg. Abdominal exam revealed diminished bowel sounds and diffuse abdominal tenderness, without rebound or guarding. Laboratory data showed white blood cell count of 16,000 cells/ul with 2% bands, lactic acid was 7 mg/dL, chemistries were otherwise normal. Abdominal CT revealed diffuse wall thickening and fat stranding. Stool sample for C. difficile toxin was positive. Given the recent chemotherapy and antibiotic course, the initial concern was typhilitis or C. difficile colitis. Treatment was initiated with IV fluids, oral vancomycin 250 mg every 6 hours, IV metronidazole and ciprofloxacin. Typhlitis was excluded due to the lack of neutropenia; thus, ciprofloxacin was discontinued after one dose. Following the 7th dose of oral vancomycin the patient developed flushing, erythema, and pruritus of the face, neck and upper torso (). The adverse reaction resolved after the discontinuation of oral vancomycin and administration of diphenhydramine (). Oral metronidazole was continued for 14 days and patient had an uneventful recovery. The patient recalled having a previous episode of RMS with IV vancomycin, which had resolved after decreasing the infusion rate. |
pmc-6529042-1 | This is the case of a 57-year-old Caucasian male with a reported past history of major depressive disorder, adjustment disorder, anxiety disorder, and pedophilic disorder, referred by his primary care provider for “sad mood and irritability.”
The patient was released from prison two years prior to presentation after serving a sentence for first-degree sexual assault. This was his third incarceration for the sexual molestation of a child, the most recent being the three-year-old daughter of a family friend. There was no history of alcohol abuse or recreational drug use in our patient.
During incarceration in 2014, the patient was diagnosed with major depressive disorder and generalized anxiety disorder. This led to the initiation of fluoxetine which was eventually titrated to 60 mg daily and believed effective. In the fall of 2018, the patient informed his primary care physician of a progressive increase in anxiety, lack of motivation, low energy, and depressed mood, which began after his release from prison. The patient further relayed these symptoms complicated his adjustment to the outside world and led to social isolation. Both believed fluoxetine remained of partial benefit and bupropion was started at 150 mg daily as an adjunctive intervention to assist mood and anxiety.
Within weeks of this addition, the patient began to have an increase in sexual urges and fantasies. Both further affected his mood, anxiety, and level of isolation. After a referral to the resident mental health clinic, bupropion was discontinued and mirtazapine was initiated at 7.5 mg nightly as a replacement fluoxetine adjunct. Three weeks later, his sexual desires and urges were under better control and his mood and anxiety began to show significant improvements.
During the period between the cessation of bupropion and the improvement of elevated sexual desires, the patient reported improvement in social acceptance at his work. The reason for the decline in his active sexual urges was most likely due to the discontinuation of bupropion. |
pmc-6529043-1 | A 66-year-old male was previously admitted to our hospital with a subarachnoid hemorrhage (SAH) secondary to a ruptured anterior communicating artery (Acom) aneurysm. He underwent coil embolization of the aneurysm, which initially showed no residual filling; however, subsequent recanalization of a posterior 2-mm lobe was detected on one-year follow-up (Figure ).
The decision was made to retreat the patient.
The right femoral artery was accessed using the standard fashion. Next, with the catheter in the left internal carotid artery (ICA), a rotational angiogram was performed, revealing an aneurysm remnant, 2 x 1.9 mm in size, with one coil loop (Figure ).
A "Y" stent-assisted coil embolization was utilized for treatment. In the first patient, the operator deemed that there is no safe corridor or technique to place the coils in the recurrent aneurysm. In the second case, the initial case was initially thought that it would be stent assisted coil embolization. The coil was attempted to be placed; however, it kept prolapsing so the decision was to leave the stents.
The diagnostic catheter was replaced with a guiding catheter. A microcatheter was advanced over a microwire into the intracranial ICA, then the left A1 segment and finally across the Acom to the right A2. Then, the wire was removed, and a 2.5 x 23-mm LVIS® Jr. braided coil-assist stent was placed from the right A2 to the left A1. Subsequently, the stent pusher wire was removed and the microwire was replaced and advanced to the ipsilateral A2 segment. With the catheter in this position, the wire was removed, and an LVIS® Jr. 2.5 x 17-mm stent was placed. Follow-up angiographic runs revealed no filling of the aneurysm at this point (Figure ).
A femoral artery closure device was deployed at the end of the case.
Postoperatively, he was transferred to the neurological intensive care unit (ICU) and was continued on aspirin and clopidogrel. He was neurologically intact, and no aneurysm filling was noted on six-month follow-up (Figure ). |
pmc-6529043-2 | A 41-year-old female with a history of a ruptured Acom aneurysm treated with balloon-assisted coiling, presented with worsening headaches and a new Acom aneurysm was found in her three-year follow-up. She was noted to have intermittent residual right-sided weakness after her initial subarachnoid hemorrhage. Her home medications were aspirin and clopidogrel. Surgical and non-surgical options were discussed with the patient, including observation, and she opted for endovascular treatment of her new aneurysm.
The patient was brought to the neurosurgical operative suite. She was placed in the supine position on the operating table; both legs were prepped and draped in sterile fashion. The left common carotid and left ICA were sequentially catheterized, contrast injected, and a rotational digitally subtracted angiogram (DSA) performed, which revealed a wide-necked Acom aneurysm (Figure ), a suitable candidate for stent-assisted coil embolization.
At this point, the diagnostic catheter was swapped out for a guiding catheter and a Headway® microcatheter, (MicroVention Inc., Aliso Viejo, CA, USA) over a microwire, was advanced from the left A1 segment into the right A2. Next, a microcatheter was advanced over a microwire into the aneurysm. An attempt was made to place a 2 mm x 3 mm coil, but it kept prolapsing into the parent artery; therefore, a 2.5 mm x 23 mm LVIS® Jr. stent was placed into the right anterior cerebral artery, extending into the left A1. Again, coil placement was attempted, but it kept herniating this time into the left A2. Therefore, the Headway® microcatheter was reinserted into the guiding catheter and advanced into the A1 on the left and then the A2 on the left. A second 2.5 mm x 23 mm LVIS® Jr. stent was placed.
At this point, an attempt was made to place a 2 mm x 3 mm coil, but it kept prolapsing and the location of these prolapsed loops was unclear, therefore the coil was withdrawn. Next, we tried a 1.5 mm x 3 mm coil and had the same problem. Finally, we tried a 1.5 mm x 2 mm and encountered the same issue. While attempting to adjust this coil, the microcatheter came out of the aneurysm. We were unable to cross the two stents to return to the aneurysm.
A final angiographic run revealed slow filling in bilateral anterior cerebral arteries. Therefore, an intra-arterial dose of abciximab was given, and the patient was started on IV abciximab drip. Follow-up angiogram showed significant improvement, although not back to baseline, and the procedure was terminated.
Postoperatively, the patient was maintained on aspirin and ticagrelor. On postoperative day 2, she complained of worsening unrelenting headache, which she rated as 8/10 in intensity. Computed tomography (CT) scan revealed a 4.0 x 3.5 cm left frontal lobe acute intraparenchymal hemorrhage. She was managed conservatively in the neurologic ICU and made a significant recovery. She was discharged home on postoperative day 7, neurologically intact. A six-month post-operative angiogram showed complete obliteration of the aneurysm (Figure ). |
pmc-6529044-1 | A 56-year-old male with no medical conditions presented to a hospital emergency department with a one-day history of increasingly severe and constant abdominal pain. The patient described associated fecal urgency with small, frequent bowel movements, subjective fevers, and rigors. He also reported earlier dysuria and anuria for several hours.
On examination, the patient was obese, hemodynamically stable, and afebrile. Initial abdominal palpation elicited tenderness in the right iliac fossa and suprapubic area. Blood tests demonstrated a raised white cell count (11.8x10^9/L) with neutrophilia (90%) but identified no anemia or biochemical evidence of end-organ dysfunction.
Given the patient’s inability to pass urine, the treating emergency physician made a provisional diagnosis of acute urinary retention. Urinary catheter insertion drained only 100 mL of urine without symptom relief. Serial abdominal examination in the emergency department elicited increasing tenderness and the new development of lower abdominal peritonitis. The patient was transferred to a tertiary facility.
A contrast-enhanced computed tomography (CECT) scan of the abdomen was performed in the portal venous phase (Figure ), revealing mucosal thickening and severe inflammatory changes within the sigmoid colon. A 19 mm linear hyperdense focus was visible at the center of the most inflamed region of the sigmoid colon. This appeared to traverse the diverticular wall and was associated with a locule of free gas. These findings were consistent with foreign body perforation of a sigmoid diverticulum.
On further history, the patient recalled eating chicken for lunch but could not recollect swallowing a bone. Upon arrival at our tertiary facility, the patient had become tachycardic, tachypnoeic, and febrile, and showed signs of generalized peritonitis. The patient was taken for urgent laparotomy. Findings were of a perforation of a sigmoid colon diverticulum by a 30 mm animal bone. There was a pelvic abscess with local fecal contamination but no generalized peritoneal soiling. An intraoperative inotrope requirement and the gross fecal peritoneal soiling mandated the performance of Hartmann’s procedure and the removal of the extruding bone.
The patient made an excellent recovery and was discharged from hospital. A histological assessment of the resected sigmoid colon demonstrated diverticulitis without evidence of malignancy. He will undergo elective reversal of his Hartmann’s procedure after colonoscopy. |
pmc-6529047-1 | A 70-year-old man was admitted for an elective right hemicolectomy for a large bowel tumour. He was previously fit and well and lived independently. He smoked 12 cigarettes a day and drank 30 units of alcohol per week on average.
His operation was uneventful. He underwent a laparoscopic right hemicolectomy for stenosing cancer of the hepatic flexure. The anaesthetic record states a grade one laryngoscopy with dentures and few native teeth. He was extubated post-surgery and was admitted to the high dependency unit for 24 hours. He was then discharged to the surgical level 1 facility.
One day after discharge to the level 1 facility, the patient underwent further emergency laparotomy for an anastomotic leak. On the anaesthetic chart, it was noted that a left upper tooth was very loose. This was removed on induction and kept. Intubation proceeded uneventfully.
Postoperatively, he was kept sedated and intubated due to the high risk of further deterioration because of bowel contents soiling during the operation. The plain film chest X-ray taken showed no abnormality. As he was stable, he was extubated that afternoon.
The following morning, he desaturated and was reintubated. Flexible bronchoscopy was performed due to capillary oxygen saturation (SpO2) 70% with the fraction of inspired oxygen being (FiO2) 100%. There was an unexpected finding of a large tissue mass located 5 cm into the left main bronchus; it was difficult to pass the bronchoscope past this lesion. This lesion is shown in Figure . After suctioning the copious secretions, his oxygen saturation recovered to 100% on FiO2 50%. The ‘tissue mass’ would subsequently prove to be a tooth but, at the time, was thought to be a neoplasm.
A chest X-ray, shown in Figure , at post-intubation showed what was, in retrospect, a molar with a ceramic filling in the left main bronchus; however, this was not recognised at the time.
Advice was sought from a respiratory consultant who performed flexible bronchoscopy that afternoon. His findings were documented as ‘left main bronchus partially occluded by poorly vascular polypoid necrotic lesions fixed to bronchus’. Washings were sent and biopsies were performed.
A repeat chest X-ray that evening at 23:09 showed the tooth had moved from the left main bronchus to the right, as seen in Figure . Again, the tooth was not identified. The next morning, the patient was extubated. The tooth was found incidentally at the end of the endotracheal tube’s suction catheter. A post-extubation film demonstrated no tooth in situ. A subsequent review of the two previous X-rays revealed the tooth.
The patient recovered well, with no further complications, and was later discharged home. |
pmc-6529053-1 | Patient #1
The patient is a 70-year-old male with type-2 diabetes mellitus (DM) and chronic kidney disease (CKD) with a history of chronic abdominal discomfort who presented with a missed posterior congenital right Bochdalek hernia. Abdominal computed tomography (CT) revealed a posterior right diaphragmatic hernia containing loops of non-incarcerated bowel without obstruction. The operation was performed via a midline laparotomy and bowel loops were pulled from the hernia inadvertently creating a small enterotomy which was repaired primarily. A pleural sac covering the contents was identified but not opened. The hernia orifice was 8 x 6 cm with a muscular posterior rim. After reducing the orifice to 4 x 4 cm using sutures at the angles, the GORE® BIO-A® Tissue Reinforcement patch was used to close the defect without tension using multiple interrupted nonabsorbable 4/0 prolene sutures. The patient’s associated comorbidities and the enterotomy that was encountered increased the likelihood of surgical infection which led us to use the aforementioned technique and tissue reinforcement patch.
Patient #2
The patient is a 6-year-old male with no significant past medical history who was admitted with a strangulated Morgagni hernia, sepsis and reactionary pericardial effusion. A midline laparotomy was performed and bowel was pulled out of the hernial sac. The ischemic small bowel was resected with the primary end to end anastomosis. The rim of the defect was identified and dissected circumferentially. The defect was repaired using a GORE® BIO-A® Tissue Reinforcement patch with the same technique as mentioned above. In the setting of an infected field, this patient was deemed to be at increased risk of infection and therefore a good candidate for GORE® BIO-A® Tissue Reinforcement patch.
Patient #3
The patient is a 15-year-old male who presented with the recurrence of a posterior left Bochdalek hernia previously repaired ten years ago via laparotomy. The previous repair was done by direct suturing without patch placement. A left posterior lateral thoracotomy was done to avoid abdominal adhesions. The bowel was released from lung and pleura and the hernia rim was dissected and identified. After reducing bowel, two serosal tears were identified which were closed primarily. The 6 x 5-cm hernia was reduced to 3 x 3 cm without tension and the residual defect was closed using GORE® BIO-A® Tissue Reinforcement patch using the same technique as mentioned above. Multiple operations, which are associated with bowel perforation, placed this patient at high risk for surgical infection. |
pmc-6529055-1 | A 55-year-old female nonsmoker presented to the emergency department with progressively worsening seizures, left hemiparesis, and memory loss. Two years prior, she was diagnosed with right-breast triple-negative adenocarcinoma (TNBC). Her treatment regimen was as follows: 1.) neoadjuvant chemotherapy with doxorubicin, cyclophosphamide, and paclitaxel; 2.) right breast-conserving surgery by lumpectomy; and 3.) postoperative radiation. Overall, she completed 35 cycles of chemotherapy and three months of radiation. Her last documented therapy was administered approximately one year prior to the initial patient encounter, and her breast cancer was thought to be in remission.
In the emergency department, computed tomography (CT) scans of the head and magnetic resonance imaging (MRI) of the brain with contrast were obtained. MRI showed a large right frontal lobe lesion measuring approximately 4 cm with surrounding hemorrhagic necrosis. Additionally, a 1.2 cm right to left midline shift anteriorly, effacement of the anterior right lateral ventricle, mass effect, and herniation were evident. No leptomeningeal enhancement was noted. CT scans of the chest also showed a new 2-cm mass in the left breast. There was no CT evidence of lymphadenopathy or metastasis in the chest. Regional skeletal tissue changes were age-appropriate and without focal lytic or blastic lesions. Image findings, in conjunction with the patient’s clinical picture, were concerning for the development of metastatic central nervous system (CNS) disease. A frontal craniotomy was performed in an effort at tumor resection and helped provide a final pathological diagnosis. Properly controlled immunohistochemical stains for cytokeratin 7 (CK7), cytokeratin 5 and 6 (CK5, CK6), gross cystic disease fluid protein 15 (GCDFP-15), GATA binding protein 3 (GATA3), pan-cytokeratin (panker), estrogen receptor (ER), progesterone receptor (PR), human epidermal growth factor receptor 2/neu (HER2/neu), and glial fibrillary acidic protein (GFAP) were performed on brain tumor tissue. Malignant-appearing cells were noted and exhibited positive immunoreactivity for CK7, CK5-6, GCDFP-15, GATA3, and pan-cytokeratin. Tumor cells were negative for ER, PR, and HER2/neu (Figure ). This IHC staining profile supported metastatic TNBC.
The patient tolerated craniotomy well and her postoperative period was unremarkable. Her neurologic symptoms improved with anti-epileptic medications. She was discharged with recommendations to follow up with her prior oncologist near her home for systemic chemotherapy. Records lacked information regarding left breast lumpectomy. Also, there was no record of BRCA genotyping at the time of the right and left breast cancer diagnosis.
Two months after craniotomy, CT scans of the chest, abdomen, and pelvis were unremarkable for metastatic disease. However, subsequent positron emission tomography-CT demonstrated a small nodular area of fluorodeoxyglucose (FDG) activity in the right frontal lobe, suggesting possible tumor regrowth. Expected underlying right frontal lobe post-craniotomy changes were present. No additional FDG-avid lesions were identified. She underwent localized non-surgical, high precision stereotactic radiosurgery (SRS) to this region and the right frontal lobe post-craniotomy operative bed for the preventative management of brain tumor regrowth. She received a total of three SRS treatments to the right frontal brain lobe.
Three months following craniotomy, she presented to the emergency department with severe headaches, high-grade fevers, and nuchal rigidity. Physical exam was primarily benign. The workup was negative for infectious etiologies. Given the patient’s clinical history and new neurologic symptoms, concerns for leptomeningeal disease were raised. Brain and whole-spine MRI was performed, which revealed abnormal enhancement in the brain and spinal T6-8 meningeal enhancement. Lumbar puncture with CSF analysis was performed, which showed the presence of large atypical cells suggestive of metastatic adenocarcinoma (Figure ). Together with imaging and CSF analysis, LC was diagnosed. Treatment was palliative with intrathecal methotrexate (IT MTX) to improve her neurologic symptoms and prolong survival. However, she experienced a decline of sensorium and functional status despite three weeks of IT MTX. Subsequent lumbar punctures with CSF analysis showed the persistence of malignant cells. Also, repeat MRI of the brain showed new metastatic disease to the cerebellum, leptomeningeal enhancement in the right temporal lobe, and significant ventriculomegaly. IT MTX therapy was stopped, and she was transitioned to hospice care. |
pmc-6529490-1 | A 44-year-old male patient, described in this report, had previously undergone extraction of all upper teeth and was restored with an immediate full denture at the correct OVD and occlusal relation. His mandible was fully dentate, and all the remaining teeth had undergone non-surgical periodontal treatment and were periodontally stable. Following the appropriate healing period, a decision to restore the maxilla with a full-arch fixed prosthesis on six implants was taken. Radiolucent cone beam markers (Blue Sky Bio, USA) were attached to the patient’s existing complete upper denture which was then scanned with a ProMax 3D Mid CBCT appliance (Planmeca Inc., Finland). The patient was also scanned in the same CBCT appliance with the denture in situ. The denture scan was aligned to the maxillary CBCT scan (Figs. and ) in Blue Sky Plan (Blue Sky Bio, USA) to allow for prosthetically driven implant planning. Implant placement simulation was carried out in the software, and a pilot-guide surgical stent was designed (Fig. ) and printed in a desktop 3D printer (Lulzbot Mini, USA) using PolyLite PLA material (Polymaker, Netherlands). In order to facilitate correct implant placement, all teeth were removed from the surgical guide during the design process with the exception of the two central incisors and the two first molars (Fig. ). These four teeth were strategically kept to maintain the established OVD at closure.
Following surgical stent fabrication, six Straumann STL RN implants (Straumann AG, Switzerland) were inserted in the maxilla using a flapless approach (Fig. ). During the procedure, the surgical guide was firmly stabilized with finger pressure on the palate. After the implant placement, the maxillary denture was relined with Viscogel (Dentsply, USA) (Fig. ) and delivered back to the patient with instructions on oral hygiene and diet. A post-surgical panoramic x-ray was obtained (Fig. ).
Following 4 months of healing, intraoral scanbodies (Straumann CARES Mono) were hand-tightened on the implants with the angled surfaces facing buccally according to manufacturer’s instructions (Fig. ) and an intraoral digital impression of the maxillary arch was acquired using an intraoral scanner (Trios3, 3Shape, Denmark) and the official scan strategy as suggested by the manufacturer. A mandibular digital impression was also obtained.
To register the OVD already established with the immediate denture, the metal inserts were removed from the stent and the surgical guide was secured on the mesial implants using modified implant carriers and flow resin (Fig. ). The preserved central incisors on the stent served as an anterior stoppage for the correct anterior-posterior position of the mandible, and the first molars on the stent helped to reproduce the established vertical dimension of occlusion. Although the mesial portion of the molars had been removed from the stent to facilitate implant placement, the distal portion of the teeth was adequate in preserving the correct OVD. To aid in this process, a bite registration material (Prestige Bite, Vanini, Italy) was used to further stabilize the centric relation (CR) at the established OVD (Fig. ).
Following OVD recording, the guide was sectioned in half along the midline. Each half was firmly kept in place with the aid of the corresponding implant carrier and bite registration material in closure and used interchangeably together with the scanbodies of the contralateral side to register the bite using the intraoral scanner’s bite registration module and the scan strategy suggested by 3Shape (Figs. and ). The complete digital bite registration was finalized in the IOS software (Fig. ), and the case was inserted in a CAD design software (Dental Wings, Canada) (Fig. ). A milled PMMA prototype (Delta Techim, Italy) was constructed and used for the verification of anterior esthetics, and the minor adjustments in the established OVD and CR were finalized using a bite registration material. (Prestige Bite, Vanini, Italy) (Fig. ). A full-arch cement-retained metal-ceramic restoration on Straumann synOcta abutments (Straumann AG, Switzerland) was finally fitted (Fig. ). |
pmc-6529526-1 | A 72-year-old man presented with a 5-year history of paroxysmal, severe and electrical right facial pain in V2 and V3 trigeminal distributions, and complained of typical tic douloureux. Episodes of pain were triggered by washing face and brushing teeth with cold water. It was not relieved by over-the-counter medications. In the third year of the disease, magnetic resonance angiogram (MRA) showed vertebrobasilar dolichoectasia for this patient, and he underwent microvascular decompression (MVD) surgery. His facial pain completely resolved immediately postoperatively but had recurred at 5 months after surgery. Then he accepted the treatment of carbamazepine (600 mg per day) and gabapentin (900 mg per day), without complete pain relief, and the pain resumed every time when the treatment was reduced. The patient reported no alalia and visual changes, no numbness, or paralysis.
He was a patient with arterial hypertension (treated with indapamide), but without diabetes and any history of trauma, tumor or multiple sclerosis–related TN. He was a former smoker and drinker (40 pack-years). Physical examination revealed a well-developed, anxious male. The neurological examination revealed hypoesthesia and hypoalgesia in right V2 and V3 trigeminal distributions, and the right eye fissure was smaller than the left. The other vital signs and physical examination were normal.
Laboratory tests documented mild anemia (red blood cell count 4.22 × 10∧12/L, hemoglobin 124 g/L), hypokalemia (serum potassium level 3.44 mmol/L) with normal renal function. A slight reduction in above indicators may be associated with the patient's anxiety and poor diet due to the facial pain.
Magnetic resonance imaging (MRI) and MRA demonstrated a VBD compressing the right ventrolateral region of brainstem and the trigeminal nerve root entry zone (REZ) (). The contrast-enhanced MRI revealed a significant expansion of the basilar artery () and the widest part of the basilar artery was about 12-mm-diameter. Lacunar cerebral infarction was found in bilateral basal ganglia, left thalamus and right periventricular area, and hemosiderin deposition was found in right cerebellar hemisphere. The remainder of the brainstem, brain parenchyma, and cranial nerves appeared normal. Subsequent digital subtraction angiography (DSA) examination identified a basilar artery dissection indicating the delayed image of distal basilar artery and the stratification and retention of contrast agent (). The patient underwent partial basilar dissecting aneurysm embolization based on above evidences.
Immediately after surgery, pain attacks had adequate initial relief (barrow neurological institute, BNI Grades IIIa). The patient was discharged on postoperative day 7 without occurrence of other adverse events and continuous to take carbamazepine, which were stopped progressively eventually. The pain disappeared completely and did not need any more medications 6 months later. Three years after surgery, he had experienced no recurrence of the right facial pain. Physical examination including a neurological exam was normal. The final clinical diagnosis was VBD and basilar artery dissection. |
pmc-6529586-1 | A 64-year-old Caucasian female with a 5-year history of recurrent episodes of nephrolithiasis that required lithotripsy proceeded to our institution with a complaint of epigastric pain and nausea the last 2 weeks in addition to fatigue and joint pain. The clinical examination was unremarkable. Subsequent laboratory investigations detected hypercalcemia (2.8 mmol/L), hypophosphatemia (0.69 mmol/L) and parathyroid hormone (PTH) (15 pmol/L).
The patient had no family history of thyroid or parathyroid disorders or history of previous radiation. No previous surgical history or commorbidities existed, apart from mild hypertension. All these findings in conjunction with the patient’s clinical presentation lead to the assumption that the patient had primary hyperparathyroidism (PHPT). Then, an ultrasound and additional Tc-99m-MIBI scintigraphy where performed, and they detected a parathyroid adenoma located posteriorly the right lobe of thyroid gland. Following these, a parathyroid adenoma surgical excision was finally scheduled.
A standard thyroid collar incision of approximately 6 cm was performed and was extended over the sternocleidomastoid muscles, approximately 2 cm above the sternal notch. While the surgeons attempted to detect and retract both the carotid artery and the jugular vein, they incidentally detected that the RCCA emerged higher from the level of the right sternoclavicular joint due to an anatomical aberration of the trajectory of the BCT, from which RCCA arises.
More specifically, surgeons performed meticulous descending exposure of the RCCA and finally detected the bifurcation point of the BCT at the level of the third tracheal ring anterior to the trachea ( ). In particular, the BCT emerged as in usual fashion from the aortic arch and then ascended aberrantly to the left side of the trachea and finally divided into the RSA and the RCCA at the cervical level of the trachea. After the detailful exposure of the operative field and the gentle retraction of all the large vessels, surgeons continued as in usual fashion and the operation was uneventful, although such an anatomic variation may augment the potentiality of accidental injury and severe hemorrhage when it is not discovered preoperatively.
The patient was discharged with instructions the 2nd postoperative day, when the drainage placed was finally remo (). At the follow-up, the 7th postoperative day the patient had none complication. The histology of the mass confirmed the diagnosis of parathyroid adenoma that was composed predominantly of oxyphil cells without any malignant components. Serum calcium level was 2.69 mmol/L and iPTH 17.8 pg/mL 12 h after the operation. At the 6-month follow-up the patient had no complications. |
pmc-6529587-1 | A 22-year-old man, weighing 65 kg, was admitted to our hospital to evaluate a murmur in the routine examination. He had complained of mild shortness of breath on physical exertion for one month. Physical examination on admission revealed a systolic murmur in the left para-sternum, trans-thoracic echocardiography showed a subarterial VSD with a diameter of 8 mm, left to right shunting, pressure gradient via the defect was 70 mmHg. The left ventricle dilated mildly with left ventricle end-diastolic diameter was 57 mm and left ventricular ejection fraction was normal range. Pulmonary artery systolic pressure was 33 mmHg at rest. Mild aortic regurgitation was present.
The patient was conducted under general anesthesia with a single-lumen endotracheal tube and placed in supine position as for standard median sternotomy with two arms along the body. Defibrillation pads were placed on the right and left chest before sterile draping. The femoral artery and vein were dissected in preparation for cannulation with a 2–3 cm oblique right groin incision. A 4 cm left parasternal thoracotomy was used to enter the thorax via the third intercostal space (ICS). The left internal thoracic artery was preserved carefully. The third costal cartilage was divided close to the sternum, without resection, to increase exposure. The ribs were slowly spread with a mini-thoracic retractor. The pericardium was opened longitudinally and suspended with stay sutures. The femoral artery cannula was inserted directly into the common femoral artery. A multi-stage venous cannula was inserted using the Seldinger technique with the tip of cannula advanced to the superior vein cava under transesophageal echocardiography guidance. After femoral arterial and venous cannulation, cannulas were secured, and cardiopulmonary bypass (CPB) initiated. CPB was initiated with vacuum-assisted venous drainage and body temperature maintained at approximately 34 °C A long cardioplegia needle (Livanova, London United Kingdom) was utilized to deliver warm blood cardioplegia directly into the aortic root and repeated every 15–20 minutes. An aortic clamp was introduced through the thoracotomy incision as a standard median sternotomy. The aorta was cross-clamped after dissecting the main pulmonary artery from ascending aorta. The VSD was exposed through a transverse right ventriculotomy. A left vent was inserted via the VSD to evaluated the edge of the defect. The subarterial VSD was closed with a patch (Bovine pericardial patch, Edwards Lifesciences) by a continuous suture. The left heart was filled with saline to exclude air before tying the suture. Any remaining air was then vented through the original cardioplegia site. Ventriculotomy was closed, and two ventricular temporary epicardial pacing electrodes were placed before releasing the aortic clamp. The cardioplegia needle was removed after the de-airing maneuver was completed, and the patient might be weaned from CPB and decannulated. CPB and cross-clamp times were 58 and 42 min, respectively. The patient was ventilated postoperatively in the intensive care unit and extubated within 4 h without any complications. Echocardiography prior to discharge showed completely closed VSD, mild aortic regurgitation. The patient was discharged from the hospital on the fifth postoperative day. There were no complications after 3 months and 6 months of follow-up (, , ). |
pmc-6529784-1 | A 60-year-old man, with no comorbidities and living in a rural area free of Filariasis, begun a follow-up with the Body Contour Group (Plastic Surgery Department) of our institute in 2016. He reported that the scrotum began to swell in 2009, slowly and progressively, to a massive injury, depriving him of physical and sexual activities. Although he denied urogenital infections, he reported that he had recurrent episodes of scrotal erysipelas.
On physical examination, it was evident a massive lymphedema of the scrotum, approximately 20 cm x 30 cm x 40 cm, with a buried penis in the mass and non-palpable testicles. The skin of the scrotum presented with increased thickness and areas of peeling, hardened when palpated. A BMI of 27 was calculated. ().
The patient’s preoperative prepare was done with a cleaning of the inguinoscrotal region for three consecutive days with chlorhexidine, 3 times a day. The patient, when in decubitus, was maintained with continuous elevation of the lower limbs. Ciprofloxacin was used as prophylactic antibiotic. The preoperative surgical demarcation consisted of marking the midline and the transition between healthy skin and diseased skin, circumferentially, aiming to involve the entire areas with lymphedema. ()
In addition, an anesthetist and a cardiologist evaluated the patient in preoperative and considerated his surgical risk as moderate, indicating general anesthesia, thrombosis prophylaxis in postoperative and measurement of myocardial necrosis markers, as recommended in Novo et al. [].
An incision was made in the marked area, beginning with the supra-pubic region; a careful dissection was carried out within the infiltrated and hardened tissue that occupies the entire mass; as the dissection progressed, the skin incision is continued throughout the circumference of the mass. Meticulous dissection allows preservation of the penile body and the elements of the spermatic cord, resecting the skin of the penis superficial to the Buck's fascia.
During the resection of the lesion () (sent to anatomopathological study), we preserved two posterolateral healthy skin flaps in the perineal region, which were used for reconstruction of the scrotum. The tunica albuginea was opened, to avoid hydrocele, and bilateral orchidopexy was made to avoid testicular torsion. (). Subsequent coverage with the aforementioned flaps was then performed ().
For penile coverage, split-thickness skin graft (removed from the left thigh) was fixed between the glans and the base of the penis. To avoid contracture of the graft and retraction of the penis, a broken line suture (Z-plasty) was used in the topography of the median raphe.
The skin graft was kept occluded with a dressing adapted for medium compression and held for 5 days to avoid local traumas and consequent loss of it. The bladder catheter was maintained for the same period and the hygiene of the genital area was performed daily by the nursing team, avoiding manipulation by the patient. Graft opening was performed on the 5th postoperative day, with total graft integration. The patient was discharged on the 7th postoperative day, with local care guidelines. Anatomopathological exam confirmed chronic lymphedema. The mass weighted 9,9 kg.
The follow-up was kept for 20 months (). The patient regained sexual and physical activities and he had not had new episodes of erysipelas. |
pmc-6529786-1 | A 66-year-old man presented with bilateral direct inguinal hernia and benign prostatic hyperplasia. Patient received a TEP (totally extraperitoneal) procedure using mesh and TURP (transurethral resection of prostate) to resolve the problem. To minimize the risk of infection, transurethral resection of prostate was performed after laparoscopic transperitoneal access was obtained for repair of the hernia. Bilateral hernia repair with laparoscopy was done by reducing the hernia sac, followed by prosthetic mesh inlay. Mesh type used to close the defect was polypropylene. Preoperative and postoperative levofloxacin single doses were given. The laparoscopic procedures of TEP and TURP were done without any difficulties. The total operating time was 3 h with an estimated blood loss of 100 cc. The last pathological examination before the surgery showed atypical adenomatous hyperplasia with chronic inflammatory cell infiltration, but PSA level was 29 ng/ml. Postoperative follow-up found hematoma in the right inguinal in the third week. An ultrasound examination was performed, and fluid collection was found as shown in . At the three-month postoperative follow-up, the patient had bilateral inguinal abscess and sepsis. Then a CT scan was performed, and the results showed an irregularly shaped mass and prostate (, ). Inguinal abscess and post-laparoscopic surgery before are shown in . We conducted evacuation and debridement laparotomy (, ). At the next follow-up, no evidence of hernia recurrence after mesh evacuation was found. |
pmc-6529839-1 | An 8 year old intact female domestic shorthair cat was referred for evaluation of a reproductive tract anomaly identified during a routine ovariohysterectomy. The referring veterinarian discovered a large cyst-like structure of the caudal reproductive tract in close apposition to the urinary system and did not proceed with elective ovariohysterectomy. The owner reported the cat cycled regularly without noted abnormalities, but had never been bred. The cat never had abnormal vaginal discharge, pyometra, or any other significant reproductive history. The owner mentioned intermittent episodes of inappropriate urination over the past couple months, with the cat occasionally urinating outside of the litter box.
On presentation, the patient was in good condition and vital parameters were within normal limits. Physical examination revealed a non-painful abdominal distention. Evaluation of the external genitalia and mammary glands did not reveal any anatomical abnormalities. Transabdominal ultrasonography revealed a thin walled cystic structure (2.5 × 5.9 cm) containing echogenic fluid in the caudal peritoneum dorsal to the urinary bladder and ventral to the colon, with close association to the uterine horns (). The uterine horns contained a mild to moderate amount of echogenic fluid and the uterine body could not be identified. A hypoechoic structure (0.34 cm) was found on the right ovary, consistent with a follicle. The urinary system was normal. Differentials at that time included reproductive tract cyst vs. disorder of sexual development (i.e., intersex tissue with development of paraprostatic cyst). Percutaneous ultrasound-guided aspiration of the cystic structure was extremely difficult and produced a scant amount of viscous red-tinged material with low intact cellularity, abundant necrotic debris, and anucleate keratinized squamous epithelial cells. Culture of the aspirate yielded no bacterial growth.
Computed tomography of the abdomen confirmed the presence of a round, well-marginated, soft-tissue attenuating, peripherally contrast-enhancing space occupying cystic lesion (4 cm H × 7.8 cm L × 4.8 cm W) in the region of the uterine body (). Secondary effects of the space-occupying cyst included mild compression and right lateral displacement of the urinary bladder and marked ventrolateral displacement of the ureters. The kidneys were normal. The uterine horns were mildly distended with hypoattenuating fluid and converged along the dorsolateral aspect of the cyst where they then abruptly terminated along its periphery. An intramural cyst with concurrent hydrometra/mucometra was the suspected diagnosis at that time.
Blood samples were collected for preanesthetic hematological evaluation and serum biochemical analysis. Hematology revealed a mild stress leukogram (WBC 16.2 × 103; Seg Neutrophils 13.68 × 103) and a thrombocytosis (platelets 765 × 103). Serum biochemistry showed a mild stress hyperglycemia (glucose 273 mg/dL).
Based on diagnostic findings the patient was admitted for an ovariohysterectomy and removal of the cystic lesion. The patient was pre-medicated and anesthetized routinely and abdominal laparotomy was performed via a ventral midline incision. The uterine horns were hypoplastic bilaterally (6 × 1 cm) and mildly distended with fluid. The ovaries were grossly normal. The ovarian pedicles were ligated and transected bilaterally. The uterus was reflected caudally revealing the discrete, round (5 × 5 cm) fluid filled structure caudal to the bifurcation of the uterus (). The cystic structure encompassed the region of the uterine body and the cranial vagina. The cervix was not identified. Blunt dissection of the mesometrium was used to expose the cystic lesion, which had formed adhesions to the urinary bladder and was closely associated with the urethra and both ureters (). The uterine arteries were individually ligated and a partial vaginectomy was performed in addition to ovariohysterectomy to remove the entirety of the cystic structure. The bladder was expressed verifying the patency of the urethra and ureters were traced from the bladder to the kidneys bilaterally.
Following surgery the excised reproductive tract was examined grossly. The cyst contained opaque, viscous brown material (). A sagittal section of the uterus revealed diffuse cystic endometrial dilations (). The cervical canal was not present and a fibrous septum was identified in the region of the cervix (). The fibrous septum prevented communication between the uterus and vagina, there was no visible channel between the uterus and cranial vagina (). The cystic lesion was located caudal to the fibrous septum, and did not communicate with the uterus grossly.
The reproductive tract was submitted for histopathological evaluation. The ovaries contained several corpora lutea bilaterally. The right ovary contained a cystic structure formed of smooth muscle and lined by a single layer of cuboidal cells, probably consistent with a remnant of the male embryonic reproductive system. The mesonephric duct cyst did not appear to compromise ovarian function as the remaining ovarian stroma contained normal structures. The uterus had a moderate level of cystic endometrial hyperplasia with normal thickness and glandular development overall, scattered glands were cystic and contained small numbers of degenerate macrophages and occasional glands had tall columnar epithelium with papillary projections (). In the endometrial interstitium were occasional foci of lymphocytes and occasional neutrophils accompanied by a very mild inflammatory reaction (). The cystic structure of the caudal reproductive tract was formed of smooth muscle lined by a double layer of cuboidal cells, with minimal inflammatory changes (). The contents were hyaline eosinophilic material, usually seen with proteinaceous debris. The cystic structure was determined to be a blind portion of the cranial vagina. The blind structure provided no exit for normal secretions, which accumulated and inspissated. The secretions did not contain infectious organisms.
On follow up telephone communications as recently as 3 months post-operatively, the cat was reported to be in good condition with no reproductive abnormalities. The cat still had occasional episodes of inappropriate urination, which had not changed in character following surgery and are presumed to be behavioral in nature. |
pmc-6529841-1 | A 33-year-old Hispanic male who was brought to the emergency room with altered mental status. He was found by his coworkers to be incoherent and was walking naked outside his home. Blood glucose was low at 29 (74–106 mg/dl). He had persistent hypoglycemia despite receiving several ampoules of 50% dextrose. A 5% dextrose infusion was started which maintained glucose in the normal range. The patient did have some improvement in his mental status but not complete normalization. He denied any history of diabetes mellitus, alcohol abuse, or illegal drug use. History was significant for a 30lb weight loss over the last 4 months. The physical examination was significant for a stellate laceration in the occipital region and marked hepatomegaly. He was oriented to time, place and person. Ultrasound of the abdomen revealed a 15 cm mass in the left lobe of the liver concerning for malignancy. Elevated alpha-fetoprotein and liver biopsy both were consistent with the diagnosis of hepatocellular cancer. Further laboratory assessment for the evaluation of hypoglycemia showed low insulin, c peptide, proinsulin, and beta-hydroxybutyrate. Insulin antibodies and sulfonylurea screen were negative. Insulin-like growth factor 2 (IGF-2) was normal; however, the insulin-like growth factor-1 (IGF-1) was suppressed. The IGF-2/IGF-1 ratio was >10, consistent with the diagnosis of NICH (). Computed tomography (CT) of the chest and abdomen showed a large mass of 20 cm size in the liver and a solitary nodule in the right lung which was consistent with metastasis (). Since the patient continued to be hypoglycemic, he was started on glucocorticoids (initially hydrocortisone and then prednisone to a maximum dose of 40 mg). Despite this, the patient continued to have hypoglycemic episodes (). A bone scan revealed further metastasis in the right clavicle and scapula. Palliative debulking of the tumor was considered however was deferred per the patient's wishes. |
pmc-6529841-2 | A 54-year-old African American female patient who was transferred to our hospital for evaluation of hypoglycemia. Her past medical history was significant for diabetes mellitus on 10 units glargine nightly, end-stage renal disease on hemodialysis, hypertension, and uterine fibroids. Before the admission, she had hypoglycemic episodes for the last 2 weeks which persisted even after she stopped taking her insulin. These hypoglycemic episodes were characterized by sweating, anxiety, and confusion. A 20% dextrose infusion, but her hypoglycemia persisted. Initially, inadvertent intake of insulin or persistence of insulin due to renal failure were thought to be the cause of hypoglycemia. However, low plasma insulin, proinsulin, c peptide, and beta-hydroxybutyrate all pointed toward hypoglycemia secondary to a non-islet cell tumor (). IGF-1 was suppressed with a normal IGF-2 however the molar ratio was high confirming the diagnosis of IGF-2 induced hypoglycemia. CT abdomen revealed a 24 cm mass adjacent to the uterus (). Hypoglycemia was persistent and necessitated the use of high concentration of dextrose (D) solution (up to D 70%) and multiple administration of rescue D 50% ampoules (). A core biopsy was performed, and the pathology was consistent with a gastrointestinal stromal tumor (GIST). During exploratory laparotomy, a large vascular mass adherent to the sigmoid colon was observed. Excision of the mass along with a sigmoid colectomy with a colostomy, hysterectomy, oophorectomy, and an appendectomy was performed. Postoperatively she was hyperglycemic, and the dextrose infusion was discontinued. Pathology confirmed the diagnosis of GIST of the small intestine which stained positively for IGF-2 (). Upon follow up in the endocrine clinic after 2 months she was euglycemic on her original home insulin regimen. |
pmc-6529843-1 | The patient is a Caucasian 3-years old male child, late-preterm born (36 weeks) from vaginal delivery, after a pregnancy complicated by placental detachment. Birth weight was 2,490 g (26° centile). He was the first child of an unrelated couple. Family history was negative for cardiac or hepatic disorders. The main stages of psychomotor development were delayed (sitting position at 8 months with hypotonia; walking at 18 months; speaking at 3 years). At 20 months of age a systolic murmur was found at the cardiac auscultation and heart ultrasound was performed, showing a mild stenosis of the pulmonary branches. Screening for metabolic diseases was negative, except for the finding of hypertransaminasemia. Because of dysmorphic facial features, delayed neurological development and elevated liver enzymes, a genetic condition was suspected and the patient was referred to the Clinical Genetics Unit of the Giovanni XXIII Children's Hospital in Bari.
At referral, height, weight and head circumference were normal (>50° centile). He featured prominent frontal bossing, saddle nose with a bulbous tip, 2/VI systolic cardiac murmur, severe psychomotor retardation suggesting an autistic phenotype. His stools were hypocholic with remains of undigested food. Fundus oculi and brain resonance were normal. Karyotype and FRAXA analysis resulted negative. After patient's parents signed the informed consent, gene sequencing of JAG1 (NM_000214) was performed by Next Generation Sequencing. Target enrichment was done by TruSeq custom amplicon (Illumina, San Diego, CA, United States) according to the manufacturer's instructions. Template library was prepared and was sequenced using MiseqIllumina platform (Illumina, San Diego, CA, United States). Annotation and filtering of variants were performed with Illumina Variant Studio version 2.0, following recommended settings. To evaluate the completeness of the method for the screening of the targeted gene, the sequencing coverage of each amplicon was analyzed in detail using Integrative Genome Viewer version 2.3 (Broad Institute, Cambridge, MA). Variants and region with a depth coverage below 30x were confirmed by Sanger sequencing. The heterozygous sequence variant c.2026delT; p.Cys676AlafsTer67 in exon 16 was identified and confirmed using Sanger sequencing (), also because the coverage in exon 16 was very low (<30 x). Primers sequences for PCR amplification of the exon 16 were designed using Primer3 software: Forward primer CCTGTCGTGAATGGTCCTG, Reverse primer CCAGGCCCAGAGAAATATCA. The variant was absent in both parents, arisen as a de novo variant, which determines the formation of a stop codon. Variant was checked for previously reported causative mutations in published works and mutation databases: Human Gene Mutation Database (HGMD) and Leiden Open Variation Database (LOVD) and it has never been described before. Moreover, variant was searched indbSNP, 1,000 Genomes and ExACdatbases, to exclude common single nucleotide polymorphism.
The child started symptomatic therapy with ursodeoxycholic acid and multidisciplinary follow-up, in particular rehabilitative psychomotor follow-up. |
pmc-6530031-1 | A 2-year-old 38 kg (83.77-lb) intact male Labrador and a 14-year-old 42 kg (92.59-lb) neutered mixed breed female dog were referred to the Department of Surgery the Faculty of Veterinary Medicine at the University of Agricultural Sciences and Veterinary Medicine Cluj-Napoca, Romania. On physical examination, the Labrador dog had a dense, 3X4 cm well-defined subdermal mobile swelling on the craniolateral aspect of the tibia (Fig. a).
General condition of the dog was excellent and no lameness was observed on the affected limb. The dog had been evaluated by the referring veterinarian who also performed a biopsy. Histologic evaluation established a diagnosis of intermediate (grade 2) fibrosarcoma. Thoracic radiography and abdominal ultrasound revealed no metastatic foci. CT scan analysis was not available. No evidence of regional lymph node involvement was noticed. All results of CBC and serum biochemical analysis were within reference limits. A 3 cm surgical excision was performed, because histologic examination of tissue margins indicating complete tumor resection is predictive of nonrecurrence, and unaffected tissue margins of 2 to 3 cm in all planes are typically recommended to achieve this goal [] (Fig. b).
Cefazolin (22 mg/kg [10 mg/lb], IV) was administered 30 min prior to surgery, and the patient was premedicated with butorphanol (0.3 mg/kg [0.14 mg/lb], IM) and midazolam (0.3 mg/kg, IM). Anesthesia was induced with propofol (3 mg/kg [1.4 mg/lb], IV) and maintained with isoflurane in oxygen following endotracheal intubation. The patient was placed in lateral recumbency, and a hanging leg preparation was performed. The tumor was removed with 3 cm margins (Fig. b).
The 14-year-old 42 kg (92.59-lb) neutered mixed breed female dog was referred because of a chronic and 14X6 cm extensive skin defect on the lateral aspect of the distal tibia and tarsus (Fig. a). The dog was found on the street; probably a road accident was the main cause of cutaneous blunt trauma. No orthopedic damage was present. Neural motor function of the limb was adequate. General condition of the dog was very good, and score 2 lameness was present on the affected limb. CBC and serum biochemistry values were all within reported reference ranges at the time of surgery. The wound was surgically debrided and managed open with foam dressings containing dextrans, alginate and silver coating until a recipient bed of granulation tissue was achieved (Fig. b). Several millimeters of skin bordering the recipient bed were removed with a scalpel blade until a proper granulation bad was formed. Wound bed tissue samples were obtained for culture and sensitivity testing.
Cefazolin (22 mg/kg [10 mg/lb], IV) was administered 30 min prior surgery, and the anesthesia was performed in a similar way to the previous case. The patient was placed in lateral recumbency, and a hanging leg preparation was performed. The proposed incision lines and measurements of the flap length and width were done in a similar way to the previous case.
The genicular axial pattern flaps were marked with a sterile pen and the flaps were then developed on the lateral aspect of the limb. The base of the flap was located 1 cm proximal to the patella and 1.5 cm distal to the tibial tuberosity on the lateral aspect of the limb according to the recommendations [] (Fig. c). The incisions converged slightly so that the base was 2 cm wider than the tip []. Flaps were elevated in the loose areolar fascial plane below the skin according to recommendations []. Flap dimensions varied in length and width between the two dogs. The flaps were meticulously undermined, rotated distally to cover the cutaneous defects and sutured to the recipient site similar for both cases, using simple interrupted subcutaneous 3–0 monofilament polydioxanone and 3–0 monofilament nylon in interrupted pattern for the skin (Figs. c, d). The dogs recovered from anesthesia uneventfully. They were confined in cages having Elisabethan collars and the wounds were inspected every day for the presence of dehiscence and necrosis. Treatment with tramadol (3 mg/kg PO every 12 h, 5 days), carprofen (3 mg PO once daily, 5 days) and amoxicillin–clavulanic acid (18 mg/kg PO twice daily, 7 days) was established for both dogs.
Assessment of flaps viability was made 10 days, 4 and 8 weeks postoperative and was based on skin color and hair growth. A small dehiscence occurred in both dogs in the first two weeks postoperative (1X1 cm in the Labrador and 0,5X0,5 in the mixed breed dog). Due to the small size of the dehisced area it was decided to let the area heal by secondary intention for both cases (Fig. e). A small 1.5X1.5 cm skin defect remained in the Labrador (Fig. d) one month after surgery, but the owner was very satisfied with the outcome. At follow-up examination performed at 2 months, the surgical area in mixed breed dog was completely healed, covered by hair (Fig. f) and no lameness was observed. |
pmc-6530056-1 | A 68-year-old woman visited our emergency department (ED) because of a sudden attack of severe back pain. The pain developed so suddenly that the ED physician provided a tentative diagnosis of aortic dissection. Chest and abdominal computed tomography (CT) with contrast enhancement revealed no findings related to the pain and both aortic dissection and any aortic involvement were excluded. The ED physician prescribed non-steroidal anti-inflammatory drugs. The next day, the patient presented to the outpatient department and was admitted for further examination. She had medical histories of asthma and atrial fibrillation, was not receiving any medication, was a current smoker, and was not an alcohol consumer. She owned a corgi dog that lived in her house and she had been bitten and scratched by the dog daily.
Upon admission, her body temperature was 37.5 °C, with blood pressure 127/48 mmHg, heart rate 84 beats per minute and oxygen saturation 95% in ambient air with no accelerated respiration. She looked very ill suffering from the severe back pain. A physical examination did not note spinal knocking pain, neurological abnormality, or any other specific findings. A laboratory test revealed a white blood cell count of 13,360 cells/mm3 (normal value: 3900–9800 cells/mm3), platelet count of 17.0 × 104/μL, serum creatinine level of 0.66 mg/dL, and C-reactive protein level of 18.5 mg/dL (normal value: 0–0.5 mg/dL). Re-performed enhanced CT and plane magnetic resonance imaging (MRI) of the spine was not diagnostic.
On the second hospital day, Gram-negative bacilli were detected in her blood cultures. We started intravenous meropenem 1 g every 8 h, based on a provisional diagnosis of spinal epidural abscess or vertebral osteomyelitis/discitis. P. multocida was identified, and drug susceptibility was confirmed by the Vitek2 system with GN and AST-N228 card, bioMérieux (Table ) []. Then, the antimicrobial treatment was switched to intravenous ampicillin 2 g every 6 h.
On the eighth hospital day, MRI was re-performed with gadolinium-contrast to confirm the diagnosis. T2-weighted imaging and T1-weighted imaging with gadolinium enhancement revealed an epidural abscess at the Th5–6 level (Fig. ). We decided to withhold a surgery and continued the conservative treatment because of absence of a neurological deficit. On the twenty-fourth hospital day, the abscess had disappeared on the plane MRI. The antimicrobial treatment was switched to oral levofloxacin 500 mg every 24 h before hospital discharge. In total, 12-week antibiotic treatment was completed. She visits our hospital with another medical condition which is unrelated to this episode, and no recurrence occurred in 5 years after the treatment was completed. |
pmc-6530079-1 | Our patient is a 71-year-old Asian man with a history of rheumatoid arthritis (RA), type II diabetes, chronic kidney disease (CKD), and Hodgkin’s lymphoma, which was stage IV, diagnosed 6 years prior to presentation, status post six cycles of adriamycin/bleomycin/vinblastine/dacarbazine (ABVD), with sustained complete remission; he presented with progressive fatigue and malaise of 2 months’ duration. He was a former tobacco smoker with a 30 pack year history; however, he had quit smoking 20 years before presentation. He had no history of alcohol or illicit substance use. He was retired from a previous career as a farmer. He had no contributory family history. His vital signs were within normal limits with temperature of 36.3 °C (97.3 °F), blood pressure of 128/78 mmHg, and a heart rate of 70 beats per minute. An examination was most notable for new cervical lymphadenopathy. Other examination findings included finger deformities attributed to his RA and normal neurological examination. A laboratory evaluation was notable for pancytopenia with leukocyte count of 2.6 × 109/L, platelet count of 50 × 109/L, and hemoglobin of 11 mg/dL. His chemistry studies including calcium, creatinine, and liver function test were within normal limits. Positron emission tomography (PET) computed tomography (CT) demonstrated multiple enlarged hypermetabolic lymph nodes throughout his neck, chest, abdomen, and pelvis, the largest measuring 2.2 cm × 1.2 cm. This prompted concern for recurrence of Hodgkin’s lymphoma or de novo development of non-Hodgkin’s lymphoma. A tissue biopsy of an enlarged hypermetabolic left inguinal lymph node was obtained, with pathology notable for sheets of CD138+, MUM1+, MYD88-, and lambda restricted plasma cells, consistent with a plasma cell neoplasm. A bone marrow biopsy performed shortly afterward demonstrated largely unremarkable trilineage hematopoiesis without increased plasma cells, and with no monoclonal plasma cells detected. Our patient demonstrated normal renal function and electrolytes. Serum albumin, serum protein, and serum protein gap were within normal limits. His plasma and urine were negative for monoclonal protein, with normal findings on serum protein electrophoresis (SPEP), urine protein electrophoresis (UPEP), serum immunofixation, serum free light chain assay, and urine free light chain assay. Based on the above data, a diagnosis of multiple diffuse extramedullary plasmacytomas of the lymph nodes was established. On diagnosis our patient was on metformin 1000 mg daily, Januvia (sitagliptin) 100 mg daily, glipizide 5 mg daily, Nexium (esomeprazole) 40 mg daily, Vesicare (solifenacin) 5 mg daily, folic acid 1 mg daily, nebivolol 10 mg daily, and tamsulosin 0.4 mg daily.
Treatment was promptly initiated with subcutaneously administered bortezomib 1.5 mg/m2 and orally administered dexamethasone 40 mg weekly for 3 weeks on and 1 week off. Our patient’s platelet count improved considerably following initiation of treatment, allowing for the addition of thalidomide. He received four cycles of bortezomib/dexamethasone followed by two continuous 28-day cycles of thalidomide 100 mg orally administered with prednisone. Thalidomide was chosen rather than lenalidomide due to our patient’s CKD and some persistent thrombocytopenia. His initial presenting symptoms improved throughout the course of therapy. An interval PET-CT was then done to assess response, and demonstrated near complete resolution of prior hypermetabolic lymphadenopathy with no new areas of fluorodeoxyglucose (FDG) avidity noted. He now remains in remission over 18 months following completion of therapy. |
pmc-6530099-1 | A 57-year-old man presented to the emergency room with a several-day history of shortness of breath, nausea, dizziness, bilateral limb discomfort, and unilateral hearing loss. He also complained of cold intolerance and sluggish speech and movement that started a few weeks earlier, as well as 2-kg weight gain over the past year. He started rosuvastatin (5 mg, once daily) a year ago, with no dose adjustment; the patient was diagnosed with hypothyroidism at the same time. He was recommended levothyroxine treatment but did not initiate treatment because he had no related symptoms or discomfort in his daily life.
General physical examination revealed dry skin, neck vein distension, nontender diffuse goiter around the neck, and myxedema with puffy face, bilateral periorbital and lower extremities edema. His pulse rate was 52 beats/minute, blood pressure was 114/82 mmHg, respiratory rate was 20 breaths/minute, and his body temperature was 37.1 °C. Muffled heart sounds without fine crackle were also detected.
The initial results of laboratory tests (Table ) were as follows: BUN 19.0 (7–23) mg/dL, creatinine 1.5 (0.7–1.4) mg/dL, creatine kinase 9300 (43–198) IU/L, lactate dehydrogenase 1876 (238–422) IU/L, myoglobulin 636 (28–72) ng/mL, alanine aminotransferase 357 (3–45) IU/L, aspartate aminotransferase 278 (3–45) IU/L, creatine kinase muscle-brain fraction (CK-MB) 52.07 (0–4.87) U/L, troponin I 0.057 (0–0.014) ng/mL, total cholesterol 222 (130–240) mg/dL, low density lipoprotein cholesterol (LDL-c) 136 (50–160) mg/dL, high density lipoprotein cholesterol (HDL-c) 74 (40–85) mg/dL, and triglycerides 127 (35–200) mg/dL. The results of thyroid function tests showed TSH > 100 (0.17–4.05) μIU/mL, fT4 0.32 (0.89–1.79) μIU/mL, and triiodothyronine (T3) 55.6 (78–182) ng/dL. On chest radiography, heart shadow was not blunted without pulmonary infiltration, and cardiomegaly was seen (cardiothoracic ratio, 66%; Fig. a). An electrocardiogram showed bradycardia with normal sinus rhythm and low QRS voltage, and transthoracic echocardiography revealed a moderate amount of pericardial effusion (posterior 15 mm) with preserved left ventricular systolic function (55–60%; Fig. c). On further investigation, pure tone audiometry revealed that the patient had profound unilateral sensorineural hearing loss (Fig. a), and thyroid sonography showed diffusely enlarged gland with a heterogeneous echotexture and decreased vascularity (Fig. ); these findings are consistent with Hashimoto thyroiditis together with elevated thyroid peroxidase antibodies (> 2000 [0–5.61] IU/mL) and antithyroglobulin antibodies (9143.72 [0–4.11] IU/mL).
Upon admission, we started aggressive intravenous fluid resuscitation to treat rhabdomyolysis with acute kidney injury and levothyroxine replacement (oral, 150 mcg/day) to treat hypothyroidism. We also started systemic corticosteroids and intratympanic steroid injection to treat sensorineural hearing loss. The patient did not require pericardiocentesis because there was no ventricular dysfunction, and his vital signs were comparatively stable. Over the next several days, the patient’s clinical symptoms, such as shortness of breath, general weakness, and lower limb discomfort, improved substantially. On consecutive laboratory tests, creatine kinase as well as lactate dehydrogenase began to decrease on hospital day (HD) 3 and continued to decline to 1530 IU/L and 1508 IU/L on HD 7, respectively. Creatinine also started to decrease by 1.11 mg/dL on HD 7 accompanied by creatine kinase decline. Further thyroid function testing showed that his TSH was 53.65 μIU/mL and fT4 was 0.63 μIU/mL on HD 7. The patient was discharged home on HD 10 with instructions to continue levothyroxine (150 mcg/day, oral).
The patient was examined in the outpatient clinic 2 weeks after discharge. We observed recovered creatinine and creatinine kinase (Table ) as well as improved cardiomegaly (Fig. b). Except for hearing loss, his clinical symptoms continued to improve, and he was compliant with levothyroxine treatment. His thyroid function was normalized at 5 months (TSH 0.10 μIU/mL, free T4 1.60 μIU/mL) and remained euthyroid status until one year after discharge (TSH 0.54 μIU/mL, free T4 1.52 μIU/mL). No pericardial effusion appeared on transthoracic echocardiography (Fig. d). Regarding hearing loss, there was slight improvement in follow-up pure tone audiometry after 1 year; nevertheless, profound unilateral hearing loss remained in this patient (Fig. b). |
pmc-6530132-1 | A 68-year-old previously healthy male presented at a Rheumatology consultation with complaints of hand/feet arthralgia and oedema evolving for more than 6 weeks. He denied fever and there was no history of recent infection or past similar episodes. He worked as a young adult abroad (Iraq, Mozambique, South Africa, and Venezuela) and was natural of an endemic area in Portugal for familial amyloid polyneuropathy (FAP).
Examination revealed swollen and tender bilateral metacarpophalangeal (MCPJ), proximal interphalangeal (PIPJ), tibiotarsal and metatarsophalangeal joints, with pitting oedema of both hands and feet.
Laboratory evaluation revealed an increase in erythrocyte sedimentation rate (45 mm/h) and C Reactive Protein (2.04 mg/dL), with negative rheumatoid factor and anti-citrullinated peptide antibodies. There were no erosions on hand/feet radiography. Hand ultrasound revealed diffuse tenosynovitis of both extensor/flexor compartments, besides joint effusion with doppler sign of MCF and PIF.
Considering the global picture, the diagnosis of RS3PE syndrome was assumed. Symptoms subsided with prednisone 20 mg per day; however, peripheral arthritis relapsed whenever prednisone was tapered.
At this point, an extended workup was performed to exclude hidden neoplastic cause: trans-rectal prostate ultrasound, cervical ultrasound, serum prostate specific antigen, thoraco-abdomino-pelvic computed tomography scan, colonoscopy and upper endoscopy were all normal. The patient then started methotrexate 20 mg/week, with remission of articular complaints and normalization of blood inflammatory parameters.
He stayed asymptomatic for one year, when he started progressive hypostesia/dysestesia of both hands and feet, objectively with loss of sensitivity in glove and sock pattern. Electromyography showed a predominantly sensitive axonal polyneuropathy. No usual causes of polyneuropathy were identified (diabetic, hypothyroidism, alcohol abuse, human immunodeficiency virus, hepatitis B/C and vitamin B12/folate deficiency). Genetic study was performed, bearing in mind the patient’s background and possibility of FAP, revealing no transthyretin gene mutation.
Considering the sustained remission of articular complaints, methotrexate was stopped. Neurological symptoms progressively worsened, culminating in the execution of a sural nerve biopsy. Shortly after, erythematous/violaceous hypoaesthetic skin papules and plaques appeared at the trunk, face and limbs, with extensive patch lesions with hyperpigmented margins on both legs (Fig. ). Skin lesions’ biopsy showed numerous epithelioid granulomas without necrosis, negative for Mycobacterium tuberculosis complex DNA. Nerve biopsy, in addition to granulomas, also showed enlarged nerves, with lymphohistiocytic infiltrates and M. leprae bacilli in macrophagic vacuoles through Fite Faraco stain (Fig. ), allowing the definitive diagnosis of multibacillary Leprosy [].
The patient started treatment with rifampicin 300 mg/month, clofazamin 300 mg/month, dapsone 100 mg/day and clofazamin 50 mg/day. One year after, despite a complete skin lesions regression, there was no improvement in the peripheral neuropathy manifestations. |
pmc-6530164-1 | A 71-year-old man was scheduled to undergo total hip replacement surgery under general anesthesia to fix malunion of the right hip joint. Two months before the scheduled procedure, he had undergone left bipolar hip arthroplasty and right acetabular fracture fixation due to bilateral acetabular cartridge fractures. After the fractures, the patient had been prescribed oral polystyrene sulfonate calcium because of hyperkalemia. He was diagnosed to have PNH at the age of 60, and the oral administration of prednisolone was initiated. The therapy with eculizumab was not initiated.
The preoperative blood examination showed pancytopenia [white blood cells, 2.100/μl; hemoglobin (Hb), 12.7 g/dl; and platelets, 100 × 103/μl]. We suspected a hemolytic reaction due to the presence of a slightly increased aspartate aminotransferase, although bilirubin and lactase dehydrogenase level were within the normal limits. The hyperkalemia improved with the polystyrene sulfonate calcium. The irregular antibody screening was positive. Therefore, 6 units of packed RBCs and a blood salvage device (electa™; Sorin Group Italia, Italy) were prepared. No other abnormal results in the cardiac, liver, or renal functions were observed.
Figure a depicts the intraoperative progress course. The Hb and potassium (K+) levels after the anesthesia induction were 11.5 g/dL and 4.6 mmol/L, respectively. An hour after the operation started, the same levels became 9.6 g/dL and 5.4 mmol/L, respectively, due to unexpected bleeding and presumably intravascular hemolysis. We initiated blood salvage procedures and started transfusion of 2 units of prepared packed RBCs using a potassium adsorption filter. After that, 190 ml of the first salvaged autologous RBCs were re-infused. Blood examination results to check K+ concentration levels in the transfer bag showed a high level of 6.2 mmol/L in the salvaged RBCs. Because the patient’s Hb became 7.6 g/dL due to continuous bleeding, we transfused two more units of packed RBCs and re-infused 90 ml of the second salvaged autologous RBCs using a potassium adsorption filter. The value of K+ in the transfer bag of the second salvaged autologous RBCs batch was also high at 6.0 mmol/L. The patient’s Hb level recovered to 10.5 g/dL after the RBC transfusion. However, the hyperkalemia progressed to 6.8 mmol/L of K+, and we administered 850 mg of calcium gluconate and initiated glucose-insulin therapy. Although the operation was close to being finished, we transfused two more units of packed RBCs anticipating the possibility of intravascular hemolysis after the operation. The surgery was performed without complications. The value of K+ at the end of the operation was 4.9 mmol/L. The amount of bleeding during the operation was 1900 ml, and the infusion volume during the operation was 2400 ml of crystalloids, 6 units of packed RBCs, and 280 ml of salvaged autologous RBCs. The duration of surgery and anesthesia was 140 and 215 min, respectively. Postoperatively, the patient was transferred to the intensive care unit (ICU).
Figure b indicates the patient’s progress after the operation. Glucose-insulin therapy was continued until the postoperative day (POD) 1. The patient left the ICU and restarted the oral intake of polystyrene sulfonate calcium at POD 3 because the K+ increased again. At POD 18, the patient was transferred to another hospital for rehabilitation. |
pmc-6530166-1 | A 9 day old 3.5 kg boy was referred to our tertiary care hospital with diagnosed of PRS. Other than atrial septal defect (ASD), aspiration pneumonia and unilateral complete cleft palate with a maximum width of about 0.8 cm. There are no cleft lip or alveolar cleft or any other comorbidity. Upon admission, he presented with cyanosis with venous carbon dioxide pressure (PvCO2) 87.8 mmHg, multiple bedside direct laryngoscopy and GlideScope (UE Medical, China) attempts were made however none were successful. His saturation was improved to 95% by facial mask. The next morning he had thin sliced Computed Tomography (CT, Philips) with craniofacial as well as airway reconstruction (Fig. a, b).
The same night he deteriorated again. We attempted intubation with GlideScope which revealed grade 4 view. Next we tried a blind intubation with endotracheal tube loaded with stylet, however, this failed as well. Then we tried size 1 laryngeal mask airway (LMA, Well Lead Medical, China), however, we felt the LMA was blocked by an occupying lesion at the left side of tongue’s base so we decided not to force it through for fear it might further aggravate his airway. His respiratory distress was improved after we placed a NPA and saturation returned to 100%.
The third morning he was brought to operating room for MDO placement. After giving Penehyclidine to dry his secretion, we slowed dialed Sevoflurane to 6% then back to 3% to maintain his spontaneous breathing. Placement of a glidescope revealed no identifiable glottic structures. Fiberoptic scope (Olympus, Japan) revealed the epiglottis lying on the posterior pharynx, which could not be maneuvered beneath. Size 1 LMA and lighted wand (CLARUS Medical, MN) cannot be placed in the right place, multiple attempts with high-tech equipment failed to establish his airway. Since NPA could maintain his saturation, we decided to abort the procedure. Upon arrival in surgery intensive care unit (SICU), his PvCO2 was 119.4 mmHg. A TLA procedure was performed with sedation. The fourth night his PvCO2 was elevated to 183.8 mmHg. We reviewed his airway CT again with a different radiologist. We found he had large lesion with size of 21.1 mm X 11.7 mm occupying his base of tongue extending from left all the way to middle. Most likely it was thyroglossal cyst per the second radiologist. (Fig. a, b).
Knowing his hypercarbia could get even worse, on day 5 we brought him back to the operating room. After inducing patient with ketamine and sevoflurane, operator gloved then advanced nondominant middle finger along the tongue, once patient’s epiglottis was touched, middle finger was bent slightly to lift epiglottis, dominant hand then passed the lubricated and bent endotracheal tube based on 3D reconstruction right next to the middle finger into his trachea. Tube position was confirmed with capnography with endotidal CO2 of 120 mmHg. Once airway secured, patient had MDO procedure without any problem. He was sent back to SICU and successfully extubated there on postoperative day 5. |
pmc-6530200-1 | A 21-year-old healthy nulliparous female presented to a regional hospital in Australia in spontaneous labour at 39 weeks and 4 days of gestation. Her pregnancy was otherwise uncomplicated and she had no medical and surgical history of note.
The patient presented to the hospital with a cervical dilation of 4 cm. Her first stage of labour lasted 1 hr and 45 minutes and her second stage lasted 1 hr and 16 minutes, respectively. Analgesic treatment in the first stage included nitrogen oxide as required and 10 mg of intramuscular morphine. Analgesic used in the second stage was only nitrous oxide as required. An episiotomy was performed at delivery. She delivered a live born infant weighing 3690 g with APGAR scores of 9 and 10. The 3rd stage lasted 15 minutes with delivery of an intact placenta.
After delivery, she further requested nitrous oxide and had 10 mls of 1.0% lignocaine infiltrated into the perineum for an episiotomy repair.
Approximately 2 hours postpartum, the patient started complaining of sudden onset dyspnea and lower chest tightness. Her GCS was 15 at this time and her vital signs were oxygen saturation >95% on room air with a respiratory rate of 18 breaths per minute, pulse of 85 beats per minute, BP 130/78, and a temperature 37.2°C.
Physical examination at the time revealed swelling in the neck and jaw line and palpable crepitus in the anterior chest wall, neck, and jaw, consistent with subcutaneous emphysema.
Initial management included observation and pulse oximetry, as she was clinically stable and able to have a conversation without increase in chest pain or decrease in oxygen saturation.
Subsequently a chest X-ray (CXR) was obtained which demonstrated pneumomediastinum with air tracking superiorly into the pericardiac spaces (). Following telephone consultation with a cardiothoracic team at our major tertiary referral center, a computed tomography (CT) contrast study was ordered as recommended. CT contrast reconfirmed pneumomediastinum and subcutaneous emphysema and ruled out esophageal injury (). It also demonstrated extension of air in the cervical region ().
The findings were once again discussed with the cardiothoracic team who advised expectant management due to the stability of the patient's clinical state.
Her postnatal course was uncomplicated thereafter and she only requested routine oral postpartum analgesia such as Panadol and Ibuprofen. Subcutaneous crepitus receded and became minimal by postpartum day 2 for the remainder of her hospital stay. She remained clinically stable and all observations were within normal limits. She was observed for 48 hours postpartum and was discharged on day 2 with the extended midwifery service. A repeat CXR done 2 weeks postpartum was normal and showed resolution of all findings (). |
pmc-6530217-1 | The patient was an 18-year-old male who presented with a nasal mass. Preoperative imaging studies suggested a vascular lesion, and the patient underwent an incomplete excision of the mass. Initial histopathological findings documented a sinonasal basaloid squamous cell carcinoma, and subsequently, the patient underwent endoscopic craniofacial resection and reconstruction. Because of the complex tumor presentation, the tumor sample was sent for FISH cytogenetics: ESW-FLI-1 fusion analysis. |
pmc-6530233-1 | A 73-year-old woman presented with right hip pain and inability to ambulate after a mechanical fall in her home. The patient had a past medical history of congestive heart failure, atrial fibrillation, and hyperlipidemia. Three months prior to the fall, the patient had been hospitalized for 10 days due to a polymicrobial (Pseudomonas aeruginosa, Proteus vulgaris, non-beta-hemolytic streptococci, and Escherichia coli) urinary tract infection, was subsequently treated at a rehabilitation facility for a 6-week period, and was ultimately discharged home. Two days following discharge, she sustained a mechanical fall and presented to the emergency department. Prior to her fall, she was minimally ambulatory with a walker due to deconditioning from the recent hospitalization as well as a remote history of bilateral Achilles tendon injuries resulting in equinus contractures. The patient denied antecedent hip pain or pain in other joints on presentation. On physical exam, she was noted to be obese with a BMI of 36.56, her right lower extremity was shortened and externally rotated, and she had diffuse tenderness about the hip. She was neurovascularly intact distally. Radiographs including an anteroposterior (AP) pelvis and AP and lateral view of the hip demonstrated a displaced femoral neck fracture (). Due to medical comorbidities and limited functional status prior to the injury, the plan was made to proceed with a hemiarthroplasty as opposed to total hip arthroplasty. Two days following admission, the patient underwent a cemented hemiarthroplasty via the posterior approach to the hip without complication (). During her hospitalization, complete blood count and basic metabolic panel were monitored and within normal limits by discharge. She was diagnosed with osteoporosis on admission (left femoral neck T score of -2.8 on dual-energy X-ray absorptiometry [DEXA] scan). Her PTH level was slightly elevated (97.8 pg/mL), but both calcium and vitamin D levels were within normal limits (9.2 mg/dL and 41.8 ng/mL, respectively). An immunofixation test to detect multiple myeloma was unremarkable. She was ultimately discharged to a skilled nursing facility on postoperative day 4 with prescriptions for low molecular weight heparin for deep vein thrombosis (DVT) prophylaxis, narcotics for pain control, and vitamin D. She was also instructed to follow up with a metabolic bone disease specialist for further management of osteoporosis.
Two weeks after discharge, she presented with right posterior thigh and groin pain, which she reported had been constant since surgery and had prevented progress with physical therapy. She denied a new traumatic event. Physical exam was notable for tenderness over the proximal hamstring but was otherwise normal. Radiographs () and computed tomography (CT) scan demonstrated increased medialization of the femoral component into the acetabulum without evidence of loosening or hardware complications. Bilateral lower extremity duplex was negative for DVT. Infection workup revealed CRP 6 mg/L, WBC 11 (103/μL), and ESR 75 mm/hr, and it was determined that she did not have a periprosthetic joint infection (PJI) at that time. She was admitted overnight for pain management and discharged back to the skilled nursing facility the next day with a plan for restricted weight bearing with a walker.
At the 6-week postoperative visit, she continued to have limited progress with physical therapy, which she attributed to ongoing groin pain and weakness. Exam was within normal limits. Radiographs () and CT again demonstrated increased medialization of the femoral head, now with a fracture of the medial wall of the acetabulum. Infection workup revealed CRP < 5 mg/L, WBC 9.7 (103/μL), and ESR 71 mm/hr, and it was again determined that she did not have PJI at that time. She underwent conversion to a right total hip arthroplasty the next day () via a posterior approach. The fracture was treated with a jumbo cup and screws with impaction grafting technique utilized to restore medial bone loss. There were no complications. She was made partial weight bearing with instructions for walker-assisted ambulation at all times and discharged on postoperative day 4.
Two weeks after discharge, she presented with a 1-day history of incisional drainage. Her exam was significant for a fever of 101°F and an erythematous indurated incision with serous drainage. Infection workup revealed WBC 13.9 (103/μL), ESR 110 mm/hr, and CRP 89 mg/L. Radiographs did not demonstrate evidence of component loosening or fracture displacement (). She underwent right hip irrigation and debridement and revision arthroplasty the next day (). A cemented all-polyethylene cup was used in order to remove the bone graft and allow for the use of antibiotic cement. Intraoperative cultures grew Enterobacter cloacae, which was not one of the organisms of her previous UTI. The infection was treated with vancomycin and cefepime with a planned 6-week course as recommended by infectious disease, given that gram-positive involvement had not been entirely ruled out. She maintained partial weight bearing status and was discharged to subacute rehab on postoperative day 3.
She reported to the outpatient clinic for follow-up 3 weeks later. She continued to progress with physical therapy. Physical exam and imaging () were normal, and she was transitioned to weight bearing as tolerated. |
pmc-6530241-1 | A 30-year-old male with a history of cerebral palsy, autism, and scoliosis presented to the emergency department with complaints of left-sided pleuritic chest pain, shortness of breath, and fever.
Initial work-up revealed a white blood cell count of 19,300 and a chest radiography () showed left lower lobe consolidation, cardiomegaly, and a calcified mass in the left lung base. Electrocardiogram (EKG) showed sinus tachycardia with a rate of 114 with right axis deviation and non-specific ST-T changes (). He was initially treated for pneumonia and subsequently underwent an echocardiogram () due to findings of cardiomegaly on the chest radiograph. This revealed a large extra cardiac mass alongside the left ventricle with normal left and right ventricular size and function.
Computed tomographic scan of the chest () showed large mostly thrombosed proximal and mid left anterior descending artery (LAD) aneurysm measuring 7.7 cm in addition to a smaller calcified distal aneurysm which corresponds to the mass seen on the chest radiography. The LAD lumen appeared to be patent (asterisk). Coronary angiography () confirmed the presence of multiple aneurysms within the left main coronary artery and LAD with slow flow. The large mid LAD aneurysm was not well delineated on the angiogram due to the absence of calcification. No aneurysmal changes were noted in the right coronary or left circumflex arteries.
Due to the extensive and diffuse nature of the aneurysmal changes which involved the whole length of the LAD in addition to the substantial amount of organized thrombus, surgical intervention was not felt to be feasible. The patient was placed on Coumadin, atorvastatin, and aspirin and has not had a cardiac event over a 4-year follow-up period. |
pmc-6530244-1 | A 71-year-old Caucasian hypertensive, diabetic male with past medical history significant for critical coronary artery disease, hyperlipidemia, carotid artery stenosis, and subclavian vein thrombosis presented to the emergency room with chief complaints of high-grade fever of one day duration associated with chills and shortness of breath. Three months prior to presentation, the patient developed acute aortic dissection DeBakey type-1 for which he underwent emergent cardiothoracic surgery and dacron graft repair of the ascending aorta. Few days following his discharge, he started having low-grade fevers which was associated with night sweats, dry cough, and exertional dyspnea for which the patient did not seek medical attention until one week from the visit date. Prior to presentation, he was treated at an urgent-care facility with a five-day course of doxycycline for the same complaints with a working diagnosis of community-acquired pneumonia with only partial improvement of his symptoms. One day prior to presentation, he developed high-grade fever. He denied any chest pain, rashes on his body, and painful nodules on his hands or feet. On admission, his blood pressure was 137/68 mmHg, pulse rate 113 beats per minute, and respiratory rate 18/minute, and he was saturating of 99% on room air. He was febrile with temperature of 102.6°F. Physical examination was remarkable for tachycardia and early diastolic murmur in the aortic area and a pansystolic murmur best heard in the apex with radiation to the axilla. The sternotomy scar on his chest was well-healed.
Initial laboratory investigation results are presented in , along with reference range values. Notably, his WBCs, lactic acid level, and procalcitonin levels were within normal range. A chest X-ray as part of the routine work-up was ordered from the emergency room and showed widening of the superior mediastinum compared to his prior film (). Due to this concerning finding, a CT scan of the chest with contrast was ordered and showed a new fluid collection surrounding the ascending aorta and extending into the aortic arch measuring 5 cm in mediolateral dimension at the level of aortic arch (). The patient was admitted to the medical floors with working diagnosis of aortic graft infection. Blood cultures were drawn and vancomycin 1 gm every 12 hours and piperacillin-tazobactam 4.5 g every 6 hours was started. Cardiothoracic surgeons were called in to evaluate the patient for possible surgical evacuation of the abscess and explantation of the graft. A transthoracic echocardiogram (TEE) was recommended by the surgical team for better visualization of the valves and their involvement. The TEE showed an echolucent area () consistent with fluid or blood around the ascending aorta conduit graft concerning for periconduit leak or abscess. It also revealed moderate-to-severe aortic regurgitation () with global left ventricular hypokinesis, but no valvular or perivalvular vegetations concerning for endocarditis. At this point, a decision was made to take the patient to the operating room for drainage of the abscess. On surgical exploration, purulent material was drained from the wall of the aorta around the graft and microbiological cultures were obtained. Intraoperatively, it was found that there was a clear demarcation between the graft and the abscess collection; and therefore, a decision was made not to explant the graft. Mediastinal drains were placed, and patient underwent continuous irrigation with betadine. Microbiological cultures from the abscess remained negative. Patient was continued on intravenous antibiotics for 2 weeks and was eventually discharge to rehabilitation after completion of two weeks of inpatient antibiotic treatment following sternotomy. He started to defervesce postoperatively and remained afebrile upon discharge. Interestingly, both his blood and pus cultures were negative, most likely because of early initiation of potent broad-spectrum antibiotics. The patient was advised to follow up with cardiology for aortic valve repair at a later date as an outpatient. |
pmc-6530248-1 | A 27-year-old female without prior medical and family history presented with nausea, confusion, petechial bleeding, and anuric AKI necessitating admission on intensive care unit and immediate initiation of renal replacement therapy by continuous veno-venous hemofiltration (CVVH). Laboratory examination revealed thrombocytopenia and Coombs-negative hemolytic anemia (hemoglobin 3.7 mmol/L, platelets 118 × 109/L, haptoglobin < 0.1 g/L, fragmented erythrocytes 1%, lactate dehydrogenase > 13 mmol/L) but normal ADAMTS13 levels and activity (>50%) and absence of ADAMTS13 autoantibodies. Complement analysis yielded reduced levels for C3 (0.5 –0.7 g/l; reference range: 0.8 – 1.6) and normal levels for C4. Percutaneous kidney biopsy evidenced signs of acute and non-acute preglomerular and intraglomerular TMA (). The patient was initially treated with intravenous glucocorticoids and 6 weeks of plasma exchange. Only after addition of the C5-inhbitor eculizumab (4 weeks of induction followed by 6 months of maintenance) the patient’s condition slowly resolved with complete hematological and clinical remission, accompanied by gradual recovery of kidney function over several months, allowing to terminate hemodialysis (). Long-term follow up over 6 years showed no relapse and stable renal function at CKD stage 3 (CKD-EPI (Chronic Kidney Disease Epidemiology Collaboration) 40–50 ml/min/1.73 m2) without continued maintenance therapy.
Targeted next-generation sequencing using a gene panel consisting of 14 aHUS-associated genes (including ADAMTS13, C3, CFB, CFD, CFH, CFHR1, CFHR2, CFHR3, CFHR5, CFI, DGKE, MCP, MMACHC, and THBD) identified a novel heterozygous canonical splice site variant in the CFH gene (c.3134-2A>G; NM_000186.3), which was absent from SNP-databases (gnomAD). In addition, the following aHUS-risk alleles were detected: CFH-H3 (heterozygous), MCP-H2 (homozygous), and CFHR1∗B (homozygous). Copy number variations (CNV) of CFH, CFHR1-3 and CFHR5 were excluded by multiplex ligation-dependent probe amplification (MLPA). Segregation analysis yielded paternal transmission despite negative family history. The variant is located within the splice acceptor at the boundary between intron 19 and exon 20 ( and ). Sanger sequencing of patient cDNA indicated, that as a consequence of the variant c.3134-2A>G; r.3134_3160del, activation of an intra-exonic splice acceptor site results in an in-frame deletion of the first 27 base pairs of exon 20 transcript (). On the protein level, the variant leads to a loss of the very C-terminal amino acid of SCR17 and the eight N-terminal amino acids of SCR18 p.Asp1045_Thr1053del (). Importantly, the missing sequence includes Cys1048, which forms one of the two highly conserved intra-domain disulfide bonds with Cys1091 that is thought to be essential for correct domain folding (). In conclusion, this leads to a variant classification as pathogenic according to the American College of Medical Genetics (ACMG) ().
Written informed consent was obtained from the participant for the publication of this report. |
pmc-6530373-1 | A 66-year-old man with a history of infiltrative vesical carcinoma treated with intravesical immunotherapy 13 years previously was referred for recent-onset pain in the pelvic girdle. Pain was assessed by a visual analog scale (VAS) at 8/10 and was much increased with walking, which was limited to a short distance and required the use of a crutch. CT revealed extensive osteolytic metastasis involving almost all the right iliac wing and acetabulum together with the right sacral wing (
). Bone scintigraphy and 18F-FDG PET/CT revealed multiple other skeletal lesions. Biopsy of the pelvic bone revealed an epidermoid carcinoma. A vesical biopsy gave negative results, but because the prostate was clinically enlarged and the prostate-specific antigen level was increased (5 ng/mL), a prostate biopsy was performed and revealed epidermoid urothelial tumoral infiltration. The patient received a first course of systemic chemotherapy with carboplatine (AUC 5) and paclitaxel (80 mg/m2), a first infusion of bisphosphonates (4 mg acid zoledronic), and analgesics.
Given the pending risk of fracture and severe pain, the decision was taken in a multidisciplinary consultation to stabilize the pelvis before continuing systemic treatment and to attempt percutaneous osteosynthesis rather than open surgery because of the comorbidities and risk of complications with extensive surgery. Extension of the bone destruction to the sacral wing and anterior acetabulum precluded the use of the locked and perforated nail recently described to reinforce the arcuateiliac line []. Therefore, a combination of double screwing and cementoplasty was planned to restore weight-bearing force transmission from the spine to the hip.
The first step of the procedure consisted of percutaneous double osteosynthesis with two perpendicular screws. The optimal position and dimensions of the screws were determined from 3D CT-scan reformations of the pelvis. The percutaneous osteosynthesis procedure was performed under CT guidance with the patient in the prone position under general anesthesia. Skin incisions were made below the posterior superior iliac spine and laterally through the posterior iliac wing toward the sacroiliac joint. Two 11-Gauge trephine needles (TV Laurane Medical, Le Pradet, France) were used to penetrate the bone cortex and define the direction of the screws. The screw path was prepared using two flexible Kirschner wires guided by the pins, and two cannulated screws (DePuy-Synthes Spine system, Raynham, MA, USA) were inserted. The first screw was placed in the iliac bone along the arcuate line and the other from the posterior iliac crest to S1 through the sacroiliac joint (). So to insert the distal extremity of the screws into the remaining normal bone, a 7.5-mm-diameter and 135-mm-long iliac screw and a 7.5-mm-diameter and 80-mm-long sacral screw were selected.
The day after the procedure, with the patient under conscious sedation, the screws were anchored by cementoplasty under fluoroscopic guidance. An 11-Gauge trephine needle was inserted parallel to each of the two screws, and 47 cc of high-viscosity PMMA cement (Vertecem plus, DePuy-Synthes Spine, Raynham, MA, USA) was spread onto the bone alongside the screws (). Post-procedure CT showed correct filling of the osteolytic lesions around the screws, with slight cement extravasation but without any consequences for important structures. However, two large osteolytic areas remained, one in the superior acetabulum and one in the right posterior iliac wing, with no cement continuity between the two screws ().
Therefore, six days later, with the patient supine and under conscious sedation, 13 cc of the same cement was injected under fluoroscopic control into the acetabulum by an anterolateral approach (). One day later, 15 cc of the cement was injected under CT guidance into the posterior iliac wing to create a continuous bridge of cement between the two screws ( and ).
Immediately after the procedure, the patient reported pain relief (VAS 4/10) and was able to stand unaided and walk with one crutch. Analgesic treatment was decreased from 37 μg/72 h to 12 μg/72 h with a fentanyl patch. The follow-up included two courses of systemic chemotherapy.
After two months, the patient presented a superficial abscess at the site of the posterior skin incision around a slight cement extravasation into the subcutaneous tissue. No sign of bone infection was seen on CT scan. The abscess and the extravasated cement were excised. Escherichia coli was isolated on bacteriological examination. Cephalosporin antibiotics were administered and continued for 12 weeks. Three months after the procedure, the patient was able to walk without any help. Hip pain was minimal (VAS 2/10) and no pain medication was required. Local infection did not recur. At six months after the procedure, the patient was able to walk unaided almost without pain (VAS 1/10). Unfortunately, 18F-FDG PET/CT performed after two courses of chemotherapy revealed bone disease progression. The chemotherapy protocol was changed to a second-line regimen but was not able to control the cancer. The patient finally died from cancer progression eight months after the procedure. |
pmc-6530425-1 | A 69-year old male patient with initial diagnosis of a squamous cell cancer of the lower lip in 2013 underwent complete resection including plastic reconstruction of the lip and vermillionectomy including submental lymphnode resection on the left and right side. The histology demonstrated an infiltration of a moderately differentiated squamous cell cancer of the lip incorporating muscle invasion. The margins were negative. There were no signs of metastases. So the initial tumor stage due to TNM classification was pT1,pN0 (0/6),L0,V0, M0,R0, G2 (moderately differentiated). Due to the staging, no adjuvant therapy was administered.
In May 2015, the patient presented with a swelling in the right cheek. The subsequent biopsy confirmed a relapse consisting of a submandibular lymph node metastases on the right side. A neck dissection level I-V on the right and level I-III on the left side was performed followed by local radiotherapy (63 Gy on right, and 54 Gy on the left side) until August 2015. Therapy was well-tolerated without any relevant clinical symptoms. On routine follow-up in December 2015, a local relapse with new lymph node manifestations on the right side intra- and retro parotideal was confirmed. A whole body CT-scan showed no signs of metastases. A complete resection of the tumor manifestation was considered infeasible and, so, a systemic chemotherapy with cisplatin, 5-FU and cetuximab was initiated. After four cycles, a MRI-scan of the neck revealed progressive disease.
Immunohistochemical staining of the lymphatic node metastases from May 2015 revealed that 25% of the tumor cells were positive for PD ligand 1 (PD-L1), whereas PD-1 expression was negative. Based on these findings, therapy with nivolumab was started in April 2016 (3 mg/kg every 2nd week). After 6 administrations, restaging with CT and MRI revealed no change of the tumor extension (stable disease), and, therefore, nivolumab was continued. Therapy was well-tolerated and no side effects occurred. Unfortunately, in August 2016 after 11 administrations of nivolumab local progression was confirmed by routine follow up and radiologic scans (). There were no signs of metastases. In addition, no surgical approach was reasonable. Due to the lack of other therapeutic options, a combination with nivolumab and ipilimumab was initiated (nivolumab 3 mg/kg on days (d) 1,14,28, and ipilimumab 1 mg/kg on d1 following every 42 days). In January 2017, following two cycles, MRI of the neck confirmed a partial remission (size reduction of more than 50%) () and therapy was continued. This treatment was well-tolerated. Apart from a fatigue syndrome, no symptoms due to immunotherapy was reported by the patient. In the following clinical schedules, MRI examinations after 4 and 6 cycles revealed stable disease, without any change of tumor size. At this point, therapy was well-tolerated, the patient was able to carry on normal activity with minor symptoms of disease, such as fatigue syndrome (Karnofsky-Index of 90%).
After the 7th cycle (September 2017), the patient presented with progressive shortness of breath, weakness, and confusions. Laboratory findings revealed an acute renal failure grade III with hypercalcaemia probably associated with immunotherapy. Thus, therapy with nivolumab and ipilimumab was interrupted and oral steroid therapy was initiated with prednisolone 100 mg/d (1 mg/kg/d). After a few days, the clinical condition improved, the confusion disappeared, and the renal function normalized. Prednisolone was reduced stepwise to 75 mg after 7 days.
Three days after discharge, the patient was rehospitalized triggered by acute chest pain and shortness of breath. NSTEMI—constellation was diagnosed and a coronary angiography was performed, showing no signs of relevant coronary artery stenosis. A transthoracic echocardiography revealed a reduced cardiac ejection fraction of 36% (ejection fraction before start of immunotherapy was >60%) accompanied by apical balloning. A cardiac MRI detected the presence of a Tako-Tsubo cardiomyopathy, possibly caused by the immunotherapy with ipilimumab and nivolumab. Of note, the patient reported no history of cardiac co-morbidities.
Consequently, heart failure therapy and treatment with prednisolone 50 mg/d was initiated. Immunotherapy was discontinued until December 2017. In January 2018, echocardiography showed a normalization of cardiac function with an ejection fraction of >60%. At this time, steroid dosage was reduced to 5 mg per day. Accordingly, monotherapy with nivolumab was re-introduced and steroid therapy was stopped completely. In March 2018, after MRI showed on-going stable disease, immunotherapy was stopped according to the patient’s demand. Up to now, the patient remains in very good physical condition (Karnovsky of 90%). Follow up CT and MRI scans in July and October 2018 confirmed ongoing stable disease without any signs of tumor progression (). Consequently, therapy has not been reinitiated, yet. |
pmc-6530806-1 | A 23-year-old female presented to the emergency department with a two-day history of recurrent, severe, thunderclap headaches. Headaches were associated with focal motor seizures with secondary generalization. She had a known history of quadriplegia and autonomic dysreflexia from a cervical spinal cord injury. She also reported a blockage of the urinary catheter and experienced recurrent headaches when she attempted to flush the catheter. On our initial assessment, there was no change in the neurological examination from her baseline. Computed tomography (CT) head revealed a subarachnoid hemorrhage as shown in Figure . The CT angiogram of the head, as shown in Figure , was remarkable for multifocal narrowing of the anterior cerebral artery, bilateral middle cerebral arteries (MCA), right posterior cerebral artery, and pericallosal artery.
Magnetic resonance imaging (MRI) of the brain, as shown in Figure , revealed T2 fluid attenuated inversion recovery (FLAIR) hyperintensity in the bilateral frontal lobes and petechial hemorrhage in susceptibility weighted imaging (SWI). Initial workup, consisting of routine blood tests, erythrocyte sedimentation rate (ESR), comprehensive drug screening, antinuclear antibodies, anti-neutrophil cytoplasmic antibody (ANCA) panel, and angiotensin-converting enzyme, were within normal limits.
The diagnostic cerebral angiogram was remarkable for stenosis of the bilateral posterior cerebral arteries, callosal and pericallosal branches of the right anterior cerebral artery, and right middle cerebral artery as shown in Figure and Figure . During her hospital stay, her headaches recurred, which were associated with episodes of flushing, sweating, and elevated blood pressure. These events were precipitated by unclogging or flushing her foley catheter. Midodrine was discontinued and her headaches improved significantly at the time of discharge. Three months after discharge from the hospital, she underwent repeat diagnostic cerebral angiogram, which was unremarkable as shown in Figure and Figure . The patient denied a recurrence of symptoms during the subsequent follow-up visit. |
pmc-6530807-1 | A generally healthy 88-year-old female with a history of chronic sinusitis and dental decay developed a perforation in her left tympanic membrane with purulent drainage. A local otolaryngologist treated her with ototopical antibiotics and the perforation and drainage resolved. However, shortly after stopping antibiotics, the problem returned, treatment was repeated, problem resolved, but resolution was temporary. After repeating this cycle for one year, the patient presented to our clinic in October 2016. Her presenting symptoms included purulent otorrhea and mixed hearing loss without otalgia or vertigo. Otomicroscopy revealed a normal right tympanic membrane and purulent fluid filling the left external auditory meatus. Evacuation of the purulence revealed a 1 mm perforation in the anterior-inferior TM with surrounding erythema and purulence in the middle ear. Nasopharyngoscopy revealed normal nasal, nasopharyngeal, and eustachian tube anatomy. Her left ear was cleaned, and she was prescribed otic ciprofloxacin-dexamethasone drops as well as oral trimethoprim-sulfamethoxazole (TMP-SMX) due to history of amoxicillin-clavulanate allergy. Her otorrhea ceased after 48 hours and her antibiotic course continued for three additional weeks, during which time she had no drainage and examination showed closure of her perforation. However, one week after finishing the antibiotic course, her otorrhea recurred. Cultures were obtained and returned positive for TMP-SMX sensitive Staphylococcus aureus. A three-month course of TMP-SMX was initiated in conjunction with topical antibiotics, resulting in complete resolution of drainage. One month after finishing the second course, her symptoms returned; she was restarted on oral TMP-SMX and drainage once again subsided.
Computed tomography (CT) imaging was obtained and demonstrated left mastoid opacification and dehiscence of the bony septation between the mastoid opacification and the sigmoid sinus. At this time, the etiology of her symptoms was suspected to be secondary to chronic mastoiditis, and since durable resolution of otorrhea with extended antibiotic therapy could not be achieved, tympanoplasty with mastoidectomy was recommended. However, the patient was strongly averse to surgical treatment, and preferred to take TMP-SMX indefinitely rather than have the operation. In September 2017, a magnetic resonance imaging (MRI) was ordered to ensure that the bony destructive changes adjacent to the sigmoid sinus on her CT did not represent the effects of a cholesteatoma. The MRI revealed “a focus of restricted diffusion with imaging suggesting a small cholesteatoma,” and surgery was recommended. The patient again opted to continue treatment with chronic oral TMP-SMX rather than undergo surgery. In December 2017, her CSOM broke through and became symptomatic while on chronic oral TMP-SMX. Topical ciprofloxacin-dexamethasone was added and her suppuration resolved within a week.
In January 2018, a new temporal bone CT showed improved pneumatization of the left mastoid and middle ear without any opacification concerning for cholesteatoma. At this point, we began looking for another source of recurrent contamination of the middle ear and mastoid air cells that could explain her repeated relapses after discontinuation of systemic and topical antibiotics. All previous CT and MRI images had demonstrated ipsilateral maxillary sinus inflammation and/or air-fluid levels. Closer review of her CT imaging revealed a periapical lucency of tooth #15 with communication with the left maxillary sinus (Figure ).
Oral examination then revealed the tooth to be loose to gentle palpation without tenderness or purulence, a finding consistent with periapical abscesses that are freely draining into the maxillary sinus []. This provided evidence that the left maxillary molar was functioning as a nidus for recurrent infection of the left maxillary sinus, resulting in spread of infection through the eustachian tube into the middle ear. As a result, molar extraction was recommended and completed, and all antibiotic therapy was discontinued two weeks later. At a six-month follow-up the patient remained off of antibiotics with complete resolution of otorrhea and significant improvement in hearing. At the time of submission - nine months after molar extraction - the patient remained symptom free. The patient’s disease course is summarized and depicted in Figure . |
pmc-6531012-1 | A 29-year-old female patient was referred to our department with acute onset postpartum paraplegia with bowel bladder involvement after delivery of her third child by normal vaginal delivery. Her antenatal course had no significant history with any history of antepartum low-back pain, trauma, drug use, or any physical exertion. On examination, she was conscious, well oriented, and afebrile. Neurological examination showed complete paraplegia (power 0/5 with decreased tone), associated bowel bladder involvement, and absent deep tendon reflexes at knee and ankle joint with complete sensory deficit below the level of L1. All necessary blood investigations including coagulation profile came out to be normal. Patient was planned for magnetic resonance imaging (MRI) of dorsolumbar spine with screening whole spine, which revealed extradural broad based ellipsoid lesion, measuring 50 × 9 mm along T12 to L2 vertebra displacing the dura anteriorly and T1 hyperintensity (
), T2 hypointensity with minimal postcontrast enhancement suggestive of early subacute bleed causing mass effect on adjacent cord with areas of altered signal intensity at the level of lesion.
On the basis of clinical evaluation and MRI report, the patient was planned for urgent decompression in emergency operation theater. After preoperative marking of D12–L2 vertebral levels under fluoroscopic guidance, midline exposure done and paraspinal muscles elevated upto medial edge of transverse processes on both sides with the help of cobb's and broad osteotome. Intraoperatively, bilateral complete laminectomy of L1 vertebrae was done with removal of ligamentum flavum, a well-defined firm organized hematoma dark reddish black in color (5 × 1 cm) was seen over the dura which was removed (
) and checked for adequate decompression utilizing infant feeding tube. Sample removed was sent for histopathological examination reported as organized hematoma. Postoperatively, patient recovered rapidly in terms of neurological deficit and was discharged on 10th postoperative day with almost complete neurological recovery and improved bladder bowel incontinence. At the time of discharge (10th postoperative day), patient regained power of 4/5 from 0/5 preoperatively, improved tone, sensory improvement by 80%, and regained bowel bladder control. At last follow-up of 2 years, patient recovered completely in terms of functional and neurological evaluation. Patient was walking normally with normal bowel bladder control at the last follow-up. |
pmc-6531052-1 | A 48- year-old Chinese woman, gravida 4, para 2, was admitted to our hospital due to abdominal pain for 1 month. The clinical features of the patient at baseline were summarized in Table . The patient had a history of tubal ligation and denied asbestos exposure.
Fifteen years ago, the patient was diagnosed with a uterine myoma, which was about 8 cm in diameter. Instead of undergoing surgery, she opted for regular check-ups. One month ago, she developed abdominal pain and ultrasonography showed a 12 × 11.4 × 9.8 cm heterogeneous mass behind the uterus, with a cystic dark area of 3.4 × 2.3 cm (Fig. ). Ultrasound suspected as degeneration of uterine myoma. A further CT examination also considered it as a myoma fatty degeneration (Fig. ). Therefore, the patient underwent exploratory laparotomy.
Intraoperative observations were described as below: There was a small amount of viscous fluid in the pelvic cavity. The uterus was enlarged to the size of 2 months gestation. There was a mass about 12 cm in diameter behind the uterus with a pedicle attached to the uterus. The mass was multilocular cystic with myxoid fluid. The surface of the left ovary was dotted with minute neoplasms. No obvious metastatic lesions were observed elsewhere.
The mass was removed and a quick-freezing examination was performed. The results indicated that it was a mesenchymal tumor and adenomatoid tumor was possible. We treated her with a total hysterectomy and bilateral adnexectomy. The final pathology of paraffin confirmed it as localized well-differentiated benign mesothelioma (Fig. A). The results of immunohistochemistry showed that the 3 molecules Calretinin, D2–40, CK were positive, while p53, EMA were negative (Table ). The tiny neoplasm on the surface of the ovary was also thought to be mesothelioma involvement. In addition, a small nodule with a diameter of about 2 cm was found in the myometrium, and the pathological result suggested adenomatoid tumor (Fig. B).
The patient is now in stable condition, without any signs of recurrence during 1 year of follow-up. |
pmc-6531109-1 | A 38-year-old woman presented to a local hospital with a history of recurrent urinary urgency and dysuria and without macroscopic hematuria for 1 month. Urinalysis revealed leukocyturia (133/HP) and hematuria (25/HP). The patient was sexually active and was initially diagnosed with uncomplicated urinary infection. The patient received norfloxacin for 1 week. However, the symptoms remained unrelieved, and she was consulted for further examinations in the local hospital. As urinary ultrasound indicated thickening of the bladder anterior wall, further an abdominal contrast-enhanced computed tomography (CT) was carried out, through which more lesions were found, and malignant changes were highly suspicious (Fig. a). The cystoscopy from the primary hospital identified extensive basal mass in the bladder walls and the histological results of tissue biopsy revealed non-invasive urothelial carcinoma. Radical cystectomy was recommended by the provincial hospital owing to the extensive involvement of the bladder.
The patient considered the possibility of radical cystectomy to be devastating and presented at our hospital for consultation. A review of the patient's medical history revealed that the patient had undergone a Chinese IUD (Copper-bearing) placement 11 years ago after the birth of her first child. However, she became pregnant and underwent a painless induced abortion 3 months later, and the routine gynecological sonography revealed no IUD. After the following 2 accidental pregnancies, she underwent another IUD placement which worked well. Considering the above-mentioned history, our CT scanning revealed that a portion of one of the two implanted IUDs had migrated beyond the right uterus wall and was adjacent to the bladder (Fig. b). To verify the pathological diagnosis, diagnostic transurethral resection was performed (Fig. c, d), including the right, top, and trigone bladder wall, whereas histological examinations reported granuloma of the bladder right and top wall and glandular cystitis of the triangle wall. The result of the pathological analysis at the local hospital was sent to our pathology department for final confirmation, while result also revealed a benign granuloma.
As the results were controversial, a therapeutic (deeper and wider) transurethral bladder resection was performed in our hospital, and the pathology examination revealed the same benign conclusion. Finally, the uterus-IUD was removed at a gynecological clinic and the migrated IUD was removed by the cooperation of an urologist, gynecologist, and gastroenterologist. After the therapeutic transurethral bladder resection, the patient's lower urinary tract symptoms gradually disappeared. No recurrent lesion was noted in the bladder through computed tomography (CT) 3 months later (see Timeline, Supplemental Content, which illustrates the whole treatment process). |
pmc-6531111-1 | A 65-year-old woman was diagnosed with disseminated endometrioid adenocarcinoma of the ovary. Her past medical history was unremarkable. She was initially treated with right hemicolectomy and oophorectomy followed by 3 cycles of paclitaxel and carboplatin (Taxol/Carbo). Three months later, she underwent interval laparotomy and treatment with 3 more cycles of Taxol/Carbo. After a disease-free period of 23 months, progression localized to the pancreas led to the administration of 3 cycles of Taxol/Carbo. One month later, she relapsed with portal vein and celiac trunk metastatic lesions and was shifted to third-line chemotherapy with 8 cycles of liposomal Adriamycin and underwent cytoreductive laparotomy, achieving a disease-free period of 11 months. Then, she received fourth-line chemotherapy with methotrexate because of disease progression to the liver. She received fifth-line chemotherapy with gemcitabine. Positron emission tomography–computed tomography (PET-CT) demonstrated disease progression, leading to the administration of sixth-line treatment with topotecan (4 mg, total dose). Two days after beginning the fourth cycle, the patient was admitted to the emergency room because of tonic–clonic seizures and visual disturbance. Her blood pressure (BP) was 162/73 mm Hg, and blood tests showed no abnormal findings other than hyperglycemia (174 mg/dL). Physical examination revealed no abnormal findings. She had no medical history of hypertension or diabetes. Brain magnetic resonance imaging (MRI) revealed parieto-occipital hyperintensities on T2-WI and fluid-attenuated inversion recovery as well as restricted diffusion (Fig. ). Seizures were treated with diazepam and phenytoin (690 mg/d) and strict metabolic and BP control. She was discharged on the 2nd day because of clinical resolution of her symptoms. A new brain MRI was taken 9 days later showing disappearance of the lesions (Fig. ) leading to the diagnosis of PRES. Although no antihypertensive was prescribed, she did receive levetiracetam (1 g/12 h) for the next 16 months without any report of new seizures; also, she underwent another 3 cycles of topotecan without recurrence of PRES, and finally, eighth-line chemotherapy with Taxol/Carbo was administered with partial response. After 24 months of the diagnosis, she continues treatment under vigilant monitoring by the neuro-oncology unit. |
pmc-6531111-2 | A 66-year-old woman was diagnosed with bilateral breast cancer classified as infiltrating ductal carcinoma hormone sensitive (Luminal A). Her past medical history was unremarkable. She received first-line treatment with neoadjuvant fluorouracil, doxorubicin (Adriamycin), and cyclophosphamide (FAC) prior to surgery; she was, subsequently, consolidated with 4 cycles of vinorelbine (NVB) plus Adriamycin, followed by adjuvant radiotherapy and tamoxifen. One month later, she was diagnosed with uterine cervix carcinoma 1B1, for which she underwent radical hysterectomy. Two years later, she presented with a new mass in the right breast. Core needle biopsy showed infiltrating ductal carcinoma that was treated with neoadjuvant docetaxel plus carboplatin prior to surgery and radiotherapy, and at that time, she received anastrozole treatment. Three years later, her treatment was changed to capecitabine and exemestane because of liver metastases. Four months later, new liver metastases were documented, and NVB was added to capecitabine for another 4 months. Her treatment was changed to docetaxel plus capecitabine leading to partial response. As the patient showed progressive disease, treatment with paclitaxel (120 mg, total dose) and gemcitabine (1200 mg, total dose) was started. One month after the last dose of chemotherapy, she was admitted to the emergency room because of severe headache, altered mental status, tonic–clonic seizures (2 episodes), and hypertension (150/90 mm Hg). Physical examination revealed no abnormal findings. Blood tests showed a lactate dehydrogenase level (LDH) of 819, serum creatinine level of 2.47, and hyperglycemia (184 mg/dL). CT revealed diffuse hypodensities and edema within the parieto-occipital regions (Fig. ). During hospitalization, she was treated with 840 mg of phenytoin, IV steroids, hydration, and the antihypertensive drug nicardipine (20 mg/12 h). Two days after the event, she remained asymptomatic and was discharged on the 6th day with prescription for long-active nifedipine (20 mg/12 h) and phenytoin (100 mg/8 h); 2 weeks later, MRI and blood tests showed resolution of the abnormalities (Fig. ), leading to the diagnosis of PRES. Because of development of rash, phenytoin was stopped after 1 month. The patient died after 3 months of diagnosis due to cancer progression (outside the CNS). |
pmc-6531148-1 | A 37-year-old male patient was hospitalized for paroxysmal chest pain for 1 week with aggravation upon exercise in another hospital. Electrocardiography (ECG) showed no significant ischemic changes. Myocardial biomarkers were normal. Selective coronary angiography (CAG) showed only mild stenosis in the proximal segment of the right coronary (RCA) artery (see Fig. ). QCA was performed to check the severity of the stenosis. The mean diameter stenosis by QCA was 28%, and the mean area stenosis by QCA was 42%. The patient was told that the coronary arteries were basically normal, and the pain was not related to the heart. The patient was also advised to discontinue the antiplatelet drugs.
One month later, the patient was admitted to our hospital on an emergency basis because of persistent chest pain, lasting approximately 1 hour. ECG showed ST-segment elevation in the inferior wall lead, TnI 0.8 ng/mL. Coronary angiography showed acute occlusion of the right coronary artery. A 6F JR4.0 guiding catheter (Cordis, UA) was engaged, and a Runthrough NS guidewire (Terumo, JP) easily passed the occlusion. After repeated thrombus aspiration (EXPORT AP, Medtronic, UA), TIMI flow returned to grade 3; however, there remained residual thrombus in the middle segment of the RCA (see Fig. ). Optical coherence tomography (OCT) was checked to determine the etiology of the myocardial infarction and to measure the residual lumen area. As a result of the presence of residual red thrombus, the underlying structure was not well visualized in the corresponding OCT images. Nevertheless, there were representative OCT-erosion changes in various segments of the RCA (see Fig. ). Furthermore, there was no detectable plaque rupture or cavity formation. The minimum lumen area (MLA) of the middle RCA was 5.86 mm2, and the area stenosis (AS) percentage was 65% (see Fig. ). As plaque erosion was the etiology of the infarction, the residual diameter stenosis (DS) was <70% on angiogram, TIMI flow grade was 3, and the patient was stable without symptoms. The patient was advised to defer stent deployment. With the presence of heavy burden of residual thrombus, this can help to prevent no-flow phenomenon. The patient was then given intensified antithrombotic therapy (aspirin 100 mg QD, ticagrelor 90 mg BID, and GPI for 24 hours) postprocedure. Additional echocardiograms were checked to rule out embolism.
After 3 weeks, CAG and OCT were checked again, showing that the thrombus completely disappeared, and the endothelium was intact in the previous culprit lesion segment, confirming plaque erosion as the reason for the AMI. The lumen was large enough with only mild to moderate stenosis (MLA= 7.42 mm2 AS% = 49.2%), and there were no vulnerable plaques. The patient avoided stenting exclusively with medication. To guarantee the safety of the patient, fractional flow reverse (FFR) for the RCA was 0.93, meaning no functional ischemia (see Fig. ). The patient was advised to undergo 12-month dual antithrombotic and lipid-lowering therapy. At the current half-year follow-up, there were no symptoms such as chest pain, angina or chest tightness, and echocardiography showed normal cardiac function. |
pmc-6531191-1 | A 24-year-old nulliparous Nigerian woman presented with rapidly enlarging abdominal distention for 3 months. She also complained of anorexia and fatigue. She had regular menstrual cycles with hypermenorrhea and menstrual pain. There was no significant family history or regular medical history. Physical examination was unremarkable except for the presence of massive ascites. There was no abdominal tenderness, rebound tenderness, or muscle tension. The liver and spleen were normal, and we did not find Douglas nodules during anal palpation.
Blood analysis showed microcytic hypochromic anemia with a hemoglobin of 69 g/dL. Liver function, kidney function, iron level, coagulation, and tumor markers were all unremarkable except for mildly increase in the CA 12-5 level (41.54 U/mL). The human chorionic gonadotropin and purified protein derivative tests were all negative. The platelet count was highly elevated (804 × 109/L), whereas bone marrow smear and culture results had no abnormalities. Two hundred milliliters of dark brown fluid was drained out through paracentesis, and cytological analysis of ascites failed to find any evidence of malignant cells. An ultrasound scan of the abdomen and pelvis revealed massive ascites fluid with no evidence of intestinal and adnexal nodes (Fig. ). An abdominal contrast computer tomography scan further disclosed extensive ascites. In addition, omental peritoneum ultrasonography, gastrointestinal endoscopy, echocardiography, and ultrasound scan of superficial lymph nodes were all normal. Because of no definite evidence, laparoscopy was recommended, but the patient rejected the examination.
The patient insisted on a follow-up; the hemorrhagic ascites recurred several times; and the patient required repeated paracentesis to improve symptoms nearly every month. After 1 year, a limited omentum-like echo in the left lower abdomen was found by peritoneal ultrasound. The ultrasound image showed a thickened uneven echo peritoneum of approximately 1.86 cm (Fig. ). Then, a CNB of the thickened peritoneum was performed with a 14G needle under ultrasound guidance. The removed tissue was sent for pathological analysis. The pathologic diagnosis of endometriosis is based on the presence of 2 or more of the following features: endometrial glandular cells, surrounding stromal cells, and hemosiderin-laden macrophages.[ In our patient, we observed endometrial glandular cells and surrounding stromal cells in the omental tissues (Fig. ).
Considering age and fertility requirements, the patient chose conservative medical therapy. She received gonadotropin-releasing hormone (GnRH) analogs leuprorelin acetate for 3 months. The patient has been under contraception treatment of drospirenone and ethinylestradiol tablets for 8 months until now. She also added a polysaccharide iron complex for nearly 2 months. The patient's condition has greatly improved after taking these prescribed medications. Although ascites still exists, the rate of growth has significantly decreased. The hemoglobin level increased to 113 g/dL recently, and the CA-125 level became normal. This therapeutic effect is worth investigating, and we will continually focus on the patient's conditions. |
pmc-6531198-1 | A case of a 49-year-old man presented with lethargic, less communicative, and insomnia for 20 days was admitted into local hospital. The electroencephalogram showed a medium abnormality, while cranial computed tomography was normal. Therefore, wuling capsule, paroxetine (10 mg every day) were administrated to the patient, while 3 days later, the patient was presented with headache, nausea, and vomiting, then the medicine therapy was stopped and the patient was transferred to our hospital for further treatment on February 13, 2018. The patient had no history of drug or alcohol use, no medical history, and no drug allergies. The inpatient parameters were: body temperature 36.5°C, pulse 108 beats/min, blood pressure 153/97 mm Hg, and respiratory rate 20 breaths/min. On physical examination, no abnormalities were observed and neurological examination was normal. The results of cerebrospinal fluid (CSF) showed: the level of protein was 257.3 mg/L (normal range: 150–450 mg/L), glucose was 3.49 mmol/L (2.5–4.5 mmol/L), no white blood cell, and the culture of CSF was negative. Laboratory investigations including routine blood test revealed slightly increased white blood cell (9.6×109/L, normal range: 0–8×109/L). The thyroid function was normal. The manteaux test was negative. Examination and electroencephalogram were normal. Cranial magnetic resonance imaging was normal. ECG showed a sinus rhythm with a heart rate of 122 beats/min.
On admission, the body temperature of the patient reached to 37.8 °C, pulmonary computed tomography showed no signs of infection, so the diagnosis of encephalitis was suspected, and the empirical injectable acyclovir was started, along with 40mg methylprednisolone and CZX for antibiotic treatment. But the results of CSF were normal, thus the mental disorder cannot be excluded. Cipramil with a dose of 10 mg was started on 15th, Aricept with an initial dose of 5 mg was administrated on 15th, and Olanzapine with a starting dose of 2.5 mg was administrated on 18th to relieve the mental symptom but they did not work very well.
The symptom did not improve with consistent fever but no obvious infections were detected. The drug fever was considered while all intravenous medications were stopped on 20th, but the patient was still in hyperthermia and showed an increase in muscular tension. Sooner, the patient arose rigidity in extremities with trismus and shaking of 4 limbs, but he was still in consciousness and responsive. Due to the rigidity of extremities, a blood creatine phosphokinase (CPK) was checked on 24th, and it was 183 U/L (normal range: 55–170 U/L).
The symptom of the patient worsened day by day, he became more and more apathetic and exhibited a high fever (the heat peak reached to 39°C) coupled with photophobia, Estazolam was administrated to release symptom. After consulting psychiatry department, considering the medication history and clinical manifestation, the diagnosis of “serotonin syndrome” was considered. All the antidepressants were stopped on 22nd and Cyproheptadine with an initial dose of 12 mg a day was started along with supportive care. But the patient did not have any improvement in symptom but gradually accompanied by consciousness disturbance and further increased body temperature with diaphoresis and tachycardia. The patient was admitted to emergency intensive care unit for further treatment. He was sedated and paralyzed by intravenous Midazolam and Clonazepam along with physical cooling and supportive care. The patient was drowsy and appeared no more tremor and his body temperature decreased gradually. Oral Clonazepam was administrated 2 mg a day at first on 26th, then reduced to 1 mg a day, all of the patients’ symptom abated except slight fever and mild coma, then the patient was transferred to normal ward on February 28th. While on March 4th, the patient could get off the bed and be communicative, while the Cyproheptadine was reduced to 6 mg a day and Clonazepam reduced to 0.5 mg per night. On 9th, the patient made a full recovery and he was discharged from the hospital. The written informed consent was obtained from the patient for publication of the case report. |
pmc-6531213-1 | A 73-year-old man had a half-year history of left upper extremity pain and numbness, which was aggravated 6 months ago. In a local hospital, magnetic resonance imaging (MRI) was performed to reveal cervical degeneration and hypertrophic ligamentum flavum at the level of C5/6. The discs of C3/4, C4/5, C5/6, and C6/7 exhibited posterior bulge. He had received conservative treatments, including physical therapy, oral nonsteroidal anti-inflammatory drugs, and steroids; however, the pain and numbness were not alleviated. Subsequently, the patient visited our hospital for further treatment. He denied any history of trauma, fall, fever, chills, night sweats, or gout. However, he had a 5-year history of hypertension and a 2-year history of type 2 diabetes mellitus.
On physical examination, the left spurling test was positive. In addition, the patient had decreased sensation in the left upper extremity. His Japanese Orthopedic Association (JOA) score was 13, and the visual analog score (VAS) of the neck and limb pain was 8.
Laboratory examination revealed leukocytosis of 9.33 × 109/L (reference range 3.5 − 9.5 × 109), urate level of 549 μmol/L (reference range 202–417), CRP of 0.88 mg/L (reference range 0–4.0), ESR of 4.0 mm/h (reference range 0–15.0), BUN 14.53 mmol/L (reference range 2.86–8.21), and creatinine level of 176 umol/L (reference range 59–104).
Computed tomography (CT) of the cervical spine indicated spinal stenosis at the level of C5/6, mostly on the left (Fig. ). Owing to poor image quality, cervical stenosis was believed to be mostly related to the ossification of ligamentum flavum, with no suspicion of intraspinal gout.
Posterior percutaneous endoscopic surgery was performed in the prone position under general anesthesia with padding of all pressure points. Using fluoroscopic guidance, a left-sided longitudinal 7-mm skin incision was created above the facet joint at the level of C5/6. A dilator was bluntly inserted toward the lateral edge of the left C5/6 interlaminar space. After confirming the correct placement of the dilator by fluoroscopy, an operating sheath with a beveled opening was inserted through the dilator. Later, the dilator was removed, and the operating sleeve was inserted using the endoscope. The entire procedure was performed under direct visual control with neuromonitoring. The medial margin of the facet joint was removed by an endoscopic drill, and the ligamentum flavum was exposed. An abnormal mass with a white, chalky, cheese-like, and granular appearance was observed (Fig. A). The mass was removed with the aid of a grasper and bipolar radiofrequency. Then, the ligamentum flavum was resected with an endo-punch (Fig. B). Finally, the dural sac was exposed, and its pulsation was found to be good (Fig. C). The exiting nerve root felt free when palpated with a nerve hook. After removing all the instruments, the incised wound was sutured. The patient was advised to wear a neck collar for ≥weeks.
The patient experienced a progressive improvement in the left upper extremity pain after the surgery, and no signs of cerebrospinal fluid leakage, infection, or other complications were experienced. Postoperative images confirmed the removal of the abnormal ligamentum flavum, which resulted in a significant decompression (Fig. ). A histological examination of the material removed during the laminectomy revealed fibrous tissues with pools of amorphous debris having a foreign body giant cell reaction, which is typical of urate gout (Fig. ). The JOA score increased to 16, and the VAS decreased to 2 when the patient was discharged 3 days after the surgery. Clinical follow-ups after 3 and 6 months exposed no relapse of the symptoms.
This study was reviewed and approved by the Medical Ethics Committee of medical ethics committee of the Third Clinical Medical College of Nanjing University of Chinese Medicine. Written informed consent was obtained from the guardian of the patient for publication of the case report and any accompanying images. |
pmc-6531216-1 | A 45-year-old woman presented to medical attention for abdominal pain, episodic diarrhea, and a mild weight loss (<10% body weight). Informed consent was obtained from patients included in the study. All procedures performed were in accordance with the ethical standards of our institutional research committee. She underwent a colonoscopy that showed a 4 cm stenosis of the sigmoid colon, covered with pale mucosa that could not be passed by a conventional colonscope (13.2 mm) but could be passed with an enteroscope (9.2 mm) with which the cecum could be reached; the mucosa of the other colonic tract, as well as the mucosa of the terminal ileum, were unremarkable. Random ileal and colonic biopsies were obtained, which were unremarkable as well. Histology of the stenosis showed mild inflammation, mild stromal fibrosis, and architectural disruption. Subsequently, she underwent a computed tomography scan, which showed a mild thickening of the terminal ileum and sigmoid colon. The finding of thickened terminal ileum was further confirmed by a subsequent magnetic resonance imaging enterography, suggestive for Crohn disease (CD) localized at the terminal ileum. Due to the discrepancy of the radiological findings with the initial endoscopic examination, a further colonscopy with retrograde ileoscopy was performed, showing no visible endoscopic signs of inflammation and confirming the sigmoid stenosis. The repeated ileal and colonic biopsy sampling was again not diagnostic, showing a nonspecific pattern of inflammation affecting the sigmoid colonic specimens.
The physician in charge of the patient, in view of her clinical history and the radiologic finding of intestinal thickening, reckoned that the patient was affected by CD with terminal ileum and sigmoid colon localization and proposed a therapy immunomodulators: corticosteroids 1 mg/kg for 12 weeks together with antitumor necrosis factor (TNF) infliximab 5 mg at weeks 0, 2, and 6, followed by infliximab alone. After 6 months of treatment with infliximab (5 mg/kg every 8 weeks as maintenance), an endoscopic reevaluation was scheduled, showing a resolution of the stenosis in the sigmoid colon and a normal-appearing terminal ileum. The marked improvement of the sigmoid inflammation together with the finding of fibrosis on the corresponding histology further convinced the physician in charge of the patient in favor of a diagnosis of CD, and the anti-TNF treatment with infliximab 5 mg/kg was continued. After 3 months (9 months after the beginning of anti-TNF), the patient was admitted for obstructive symptoms. A computed tomography suspected neoplasia of ileocecal region. The patient underwent an uneventful ileocecal surgical resection. The surgical specimen consisted of a portion of 8 cm of terminal ileum with the cecum and the vermiform appendix. The ileal wall showed an increased consistency and a diffuse thickening, with cystic-hemorrhagic foci in the perivisceral fat. The ileal mucosa was corrugated and showed a polypoid structure with a wide base (1.5 cm maximum diameter) originating from the submucosa and determining ulceration of the mucosal layer. The ileocecal valve, the cecum, and the appendix were normal, except for a mild lipomatous filling of the submucosa. |
pmc-6531224-1 | Case A A 53-year-old Chinese female who had suffered ecchymosis in both legs for 3 days was admitted to our hospital on June, 2017. Pancytopenia was detected in complete blood count (CBC). Bone marrow (BM) aspirate revealed predominant blasts (Fig. ). Flow cytometry on the aspirate showed mostly positivity for CD33, CD117, CD34, CD13, MPO, CD64, and CD9, as well as partly positivity for CD123,CD99. Reverse transcription–polymerase chain reaction (RT-PCR) analysis detected STAT5b-RARα fusion transcripts. A routine chromosomal analysis was performed. An abnormal female karyotype 46, XX,+6q-,-11,14q-,?i(17)(q10); 46, XX was detected (Fig. ). ATO and ATRA were used for induction treatment. But the white blood cell (WBC) kept increasing out of control. The patient refused the following treatment and discharged on day 25. |
pmc-6531224-2 | Case B A 44 years old male suffered pain from lower limbs and hip. Abnormal CBC informed a high WBC (52.07 × 109/L), anemia (82 g/L) and thrombocytopenia (41 × 109/L). Bone marrow aspirate suggested an abnormal promyelocyte of 93.6% indicating APL (Fig. ). Flow cytometry was performed to confirm the diagnosis. Result of karyotype analysis showed 46, XY,?t(11,17)(q23,q21)/46, XY (Fig. ).PLZF-RARα was positive using RT-PCR detection. So ATO and chemotherapy with DA (daunorubicin 45 mg/m2/d for 3 days and cytarabine 100 mg/m2/d for 7 days) regimen were given at the same time. Four months later, the patient was admitted to hospital again for further management.BM aspirate still revealed predominant blasts. Chromosome analysis showed 46, XY,?11q-,17q+/47, idem,+17q+ (Fig. ). The coagulation got worse and finally he got the disseminated intravascular coagulation (DIC). |
pmc-6531224-3 | Case C 52-year-old male patient presented with fever for 3 weeks invalid to antibiotics and gingival bleeding for 1 week.CBC test indicated anemia and thrombocytopenia. Abnormal coagulation index showed the prothrombin time (PT) of 20.7 seconds, the fibrinogen of 0.6 g/L. Proportion of promyelocytes in the bone marrow was 19.6%, and the flow cytometry indicated a positive of CD33, CD117, CD13, CD123, CD9, CD64, MPO, and CD15. RT-PCR and chromosome analysis described a fuse gene of PLZF-RARα and 47, XY,+8/ 47, idem, t(11,17)(q23,q21)/46, XY. ATRA and ATO were used immediately. Subsequently, chemotherapy was added with DA regimen. He suffered hemoptysis, heart failure, and septicemia of methicillin-resistant staphylococcus aureus during the period of myelosuppression. In the following consolidation treatment, the patient received 3 courses of ATRA and CAG combination treatment (cytarabine 20 mg/12 hours for 14 days, aclarubicin 20 mg/d for 4 days, granulocyte stimulating factor 400 mg for 14 days). Then he got a complete remission (CR) without PLZF-RARα detected. Medium-dose cytarabine with daunorubicin were given (cytarabine 2000 mg per 12 hours for 3 days and daunorubicin 60 mg on the first day). The following treatment regimen was still under discussion. So far, the patient has survived for 7 months and remains CR.
The clinical characteristics of the 3 patients have been shown in Table . |
pmc-6531230-1 | A 56-year-old man, a heavy alcoholic beverage drinker for 20 years, was diagnosed with alcoholic liver cirrhosis for almost 3 years. He was previously admitted twice on May 2016 and November 2016, for bleeding esophageal varices. On March 23, 2018, he presented a hepatic encephalopathy coma with massive ascites and was treated accordingly. Thus, an LDTL was offered to the patient as the best treatment option available.
Preoperatively, his body weight was 78.8 kg with a body mass index (BMI) of 27.6. The Child–Turcotte–Pugh score was 11 (class C), and the Model for End-stage Liver Disease (MELD) score was 21.62.
The patient's 26-year-old son was found to be the only donor-compatible candidate for the LDTL. Preoperative evaluation included multidetector computed tomography (CT) scan, magnetic resonance imaging (MRI) including magnetic resonance cholangiopancreatography, and liver fibrosis scan. The segmental branches of the portal vein and hepatic artery were normal (Fig. A, B). In addition, large tributaries of the RHV from segments V to VIII were documented (Fig. C, D) and a trifurcation of the hilar hepatic duct was identified (Fig. E). In the CT volumetric study, the total liver volume was 1260 cm3. The right lobe had a total volume of 960 cm3, which occupied 76.2% of the total liver volume. The estimated residual liver volume was 23.8% and the graft-to-recipient weight ratio (GWRW) was 1.21%. The liver fibrosis scan was essentially normal. The right anterior section was considered the graft of choice with an estimated liver volume of 655 mL, an estimated GWRW of 0.83%, and adequate remnant volume of 605 mL. Table summarizes the volumetric assessment of the liver volume of the donor. Patients provided informed consent, and the institutional review board approved the study protocol with an approval number 4-2018-1168.
A right subcostal incision with extension to the upper midline was made to access the abdomen. The ligamentum teres, falciform, coronary, and right triangular ligament were divided. Cholecystectomy was then performed. Hilar dissection was initiated posterolaterally to identify and isolate the right hepatic artery (RHA), right portal vein (RPV), and right hepatic duct (RHD). Likewise, the anterior and posterior branches of the RHA and RPV were identified and isolated. The right anterior portal vein (RAPV) and right anterior hepatic artery (RAHA) were temporarily clamped to make a demarcation line on the liver for parenchymal dissection. Intraoperative ultrasonography was also used to precisely confirm the location of the RHV and middle hepatic vein (MHV). The liver transection was performed using the Cavitron Ultrasonic Surgical Aspirator (CUSA). The RHV tributaries from segment V and VIII were carefully ligated and divided for recipient graft reconstruction. The intrahepatic course of the MHV was followed up to its confluence with the left hepatic vein. The right anterior hepatic duct (RAHD) was isolated using the Glissonian pedicle approach, and was sharply transected leaving behind approximately a 3-mm hepatic duct stump for subsequent duct-to-duct reconstruction (Fig. A, B). The RAHA and RAPV were ligated and transected. The MHV was immediately secured using an endovascular staple. The intraoperative image of the liver after anterior sectionectomy is shown in Fig. C, D. An intraoperative cholangiography was utilized to visualize the biliary tree, particularly to define the structural integrity of the right posterior hepatic duct (RPHD) (Fig. E).
The right anterior section graft was flushed with a histidine–tryptophan–ketoglutarate solution. A vascular interposition graft was used to reconstruct the tributaries of the RHV from segments V and VIII (Fig. A, B). The proximal end of the interposition graft was conjoined with the MHV to form a common channel for venous outflow reconstruction (Fig. C).
A total hepatectomy with venacaval preservation was performed. A meticulous high hilar dissection was suitably performed. The stump of the MHV was made available for graft reconstruction. A venotomy was created from the orifice of the MHV to the inferior vena cava to enlarge the opening to ensure adequate venous outflow.
Next, we performed hepatic venous outflow reconstruction between the enlarged orifice of the MHV stump and the vena cava of the recipient and the conjoined MHV and interposition graft of the donor (Fig. D). The RPV of the recipient was anastomosed to the RAPV of the donor. Microanastomosis between the donor's RAHA and the recipient's RHA was performed. A successful flow was confirmed by an intraoperative duplex ultrasonography. Finally, we anastomosed the recipient's RAHD to the donor's RHD using a duct-to-duct method. Figure illustrates the transplanted anterior section graft of the recipient.
The total duration of surgery was 378 minutes with a total blood loss of 4100 mL. A volume of 1400 mL of ascites was noted intraoperatively with a grossly cirrhotic liver weighing 981 g. The right anterior section graft had an actual weight of 612 g, and the actual GRWR was 0.78. There was a 7% overestimation of the preoperative GWRW (0.83) compared with the actual GWRW (0.78) value.
The donor was discharged on the 7th day after surgery. There was no report of postoperative complications such as bleeding, bile leakage, or hepatic insufficiency. Although there was a small focal liver congestion on segment IV observed on the CT scan on the 7th postoperative day. Liver functions were within normal limits. Figure A, B shows the liver function recovery following right anterior sectionectomy. Notably, the postoperative CT scan showed no signs of biliary or vascular abnormalities (Fig. A, B).
Most importantly, the recipient had an unremarkable hospital stay. Liver function returned to normal levels at 2 weeks posttransplantation (Fig. C, D). There were no signs of SFSS such as ascites, coagulopathy, or renal failure. A serial duplex ultrasonography of the liver showed normal biliary and vascular findings. Moreover, CT scan findings revealed no remarkable complications on the 14th postoperative day (Fig. C, D). The patient was discharged after the 15th hospital day. |
pmc-6531267-1 | A 33-year-old female patient visited the Hand Department with complaints of mild pain and swelling. The little finger of her right hand was deformed because of an injury in a amateur basketball match 1 day prior. She requested for an emergency operation immediately. Upon inspection, she could not straighten the distal phalanx of her right little finger completely; the finger had assumed a fixed flexed position. The local general surgeon had used a splint to keep the finger extended completely. Unfortunately, the skin of wound bruised (Fig. ), and the incision may have turned necrotic after operation; therefore, open reduction was not a viable option. The patient was referred to the Hand Department. Her laboratory test findings were normal; however, further examination by X-ray imaging revealed that an intra-articular avulsion fracture of more than one-fourth of the base of the distal phalanx (Fig. ) that appeared to be type IV B injury, according to Doyle's classification.[ Considering the patient's condition, we decided to fix the bony fragment by K-wire fixation, which is a minimally invasive procedure with no skin incision. The surgical procedure was designed to first insert a 0.8 mm diameter K-wire from the dorsal side of the bony fragment to the middle phalanx, as close to the bony fragment as possible. The second 0.8 mm-diameter K-wire was inserted from the tip of the distal phalanx to the middle phalanx so that the finger is distally slightly hyperextended. We bended the first K-wire to cross the second K-wire to first reset the avulsion fracture. With the increase in angle, the avulsion fracture was able to undergo anatomy reduction gradually. When confirming fracture reset under the screen of the C-arm X-ray equipment we could tie up the two K-wires around each other using a steel wire at the end (Fig. ). The ends of the two K-wires protrude out of the skin (2–3 mm). A small splint over the metacarpophalangeal joint was used for 2 weeks followed by a finger plaster cast applied directly over the DIP joint but allowed active motion of the proximal interphalangeal and metacarpophalangeal joints. The suture line was removed 2 weeks after surgery. When the cortices of the fragment appeared to be joined on the radiographs, we confirmed that union of the fracture had occurred. Then both K-wires were removed under a digital nerve block 6 weeks after surgery.
The swelling in the surgical area was gradually reduced after the operation and returned to normal 3 days later. Six weeks after the surgery, X-ray scans both revealed no evidence of displacement. The patient also reported no finger pain or swelling symptoms. Six weeks after the surgery we pull out two K-wires and 3 months later operation follow up showed the finger returned to normal movement. Nevertheless, we continued to observe and follow up with the patient (Fig. ).
This study protocols were approved by the medical ethics committee of the Zhejiang Provincial People's Hospital. Written informed consent for publication of clinical details and images was obtained from the patient. A copy of the consent form is available for review upon request. |
pmc-6531273-1 | We present the case of a female Caucasian patient, aged 1 year and 8 months, without previous pathological conditions that was brought to the emergency room 1 week after onset of fever and cough, with accentuated respiratory manifestations, drowsiness, oliguria, and an intergluteal cleft lesion. At home, the patient received a treatment with amoxicillin-clavulanate, fever-reducing drugs, and oseltamivir for 2 days (in the context of a positive rapid influenza B diagnostic test).
At the time of admission, on physical examination, the following changes were noticed: the patient was febrile 38.2°C, pale, an ulceration covered by dry necrosis with hemorrhagic intergluteal border at coccyx level with a diameter of a 1.5/2 cm, accompanied by discreet local edema, bilateral bullous rales, respiration rate of 52/minute, oxygen saturation 94% to 96%, heart rate of 120 beats per minute, dry lips, palpable liver 1 cm under the coastal ribbon, and oligoanuria. The second day she presented afebrile tonic-clonic seizure, followed by choreic movements, drowsiness, neck pains, osteotendinous hyporeflexia, and plantar response in flexion. At skin level, there was noticed a rapid increase in the necrotic coccygeal ulceration (by 0.5/0.5 cm in 24 hours), with the transformation of the dry necrosis in sphacelus at the periphery and progression of necrosis in depth. On the same day, seizures repeated lasting about an hour, with spasticity of the lower limbs, eyeballs deviating upwards, and a Glasgow Coma Scale score of 7.
A lumbar puncture was performed and revealed no cytological or biochemical changes. Also an electroencephalography was performed and revealed a polymorphic theta-delta wave activity. A cranial magnetic resonance imaging (MRI) was also performed and revealed not pathological changes.
A chest radiography was performed and revealed enhancement of diffuse pulmonary interstitium, with a right infrahilar focal condensation. An thoraco-abdominal computerized tomograpohy (CT) scan was performed and revealed the following aspects: right and left posterior basal lung condensations, retroperitoneal lymph nodes with a short axis of max. 6.5 mm, agglutinated pelvic intestinal loops, rectum with thickened wall and minimal adjacent fat mass, small solution of skin continuity in the intergluteal fold in the neighborhood of the coccyx and minimal thickening of the subcutaneous tissue at this level. Bilateral inguinal adenopathy with a maximum diameter of 12/8 mm on left side were also noticed.
Considering the appearance of CT scan and the extremely rapid expansion of necrosis, it is decided that the patient to undergo surgical intervention of excisional debridement with necrectomy, lavage, and dressing. Intraoperatively, invasion of the fascia was excluded and tissue samples were harvested for microbacteriological examination (Figs. –).
Bacteriological examination of the tissues sampled intraoperatively was positive for Pseudomonas aeruginosa and Enterococcus faecalis confirming the diagnosis of diagnosis of ecthyma gangrenosum, strains that were identified using the VITEK 2 Compact analyzer (bioMérieux, Marcy-l’Étoile, France). The MICs (minimum inhibitory concentrations) were assessed according to the European Committee on Antimicrobial Susceptibility Testing breakpoints. The isolated strain of Pseudomonas aeruginosa was sensitive to ceftazidime, cefepime, amikacin, gentamicin, tobramycin, ciprofloxacin, pefloxacin, imipenem, meropenem, ticarcillin-resistant, colistin, trimethoprim/sulfamethoxazole, and intermediate sensitive to piperacillin-tazobactam. The Enterococcus faecalis strain was sensitive to ciprofloxacin, moxifloxacin, ampicillin, vancomycin, teicoplanin, tigecycline, tetracycline resistant, streptomycin, clindamycin, erythromycin, and intermediate sensitive to trimethoprim/sulfamethoxazole.
Although blood cultures were negative, with increased levels of procalcitonin (4.35 ng/ml, references values: 0–0.5 ng/dl) and C-reactive protein (200 mg/dl, references values: 0–10 mg/dl), concomitant pulmonary, cerebral disease, and renal failure supported the diagnosis of sepsis. At the time of admission and during hospitalization, the patient did not experience neutropenia, neutrophil counts ranging from 7550 to 9220/mm3, but the patient had anemia (Hemoglobin: 8.2–6.2–9 g/dl, references values: 13–17 g/dl) and thrombocytopenia (92,000–96,000/mm3, references values: 150–400,000/μl), which were normalized during the evolution. Upon discharge, acute renal failure was corrected under conservative treatment (creatinine 1.57 mg/dl, references values: 0.7–1.2 mg/dl; blood urea nitrogen 104.9 mg/dl, references values: 16.6–48.5 mg/dl). |
pmc-6531284-1 | A 3-year-old boy presenting with melena and jaundice for several days was admitted to our Pediatric Gastroenterology Department. His anthropometric measurements were appropriate for his age. Pallor, scleral icterus, petechiae, and ecchymoses on his legs were observed. Systemic examination revealed no lymphadenopathy, hepatomegaly, or splenomegaly. Peripheral blood counts revealed a hemoglobin level of 87 g/L, white blood cell count of 7.1 × 109/L, and platelet count of 484 × 109/L. A coagulation test showed the following results: prothrombin time, normal; activated partial thromboplastin time, markedly prolonged (131.1 seconds; normal range: 20.4–40.4 seconds); and plasma factor IX clotting activity level, 2.7% (normal range: 70%–120%). The patient's older brother had died of hemophilia B and ventricular septal defect several years ago. Based on these results, the patient was diagnosed with hemophilia B. However, he had no serious bleeding tendency and had never received a blood transfusion or any infusion of coagulation factors before admission. After admission to our hospital, his bleeding was stopped by transfusion of coagulation factors.
Because the patient presented with jaundice, we performed a series of tests. A liver function test revealed the following levels: total bilirubin, 72.9 μmol/L (normal range: 3–22 μmol/L); direct bilirubin, 66.4 μmol/L (normal range: <5 μmol/L); alanine aminotransferase, 192 U/L (normal range: <50 U/L); aspartate transaminase, 168 U/L (normal range: <50 U/L); γ-glutamyl transferase 791 U/L (normal range: <70 U/L); lactate dehydrogenase, 2,629 U/L (normal range: 300–600 IU/L); and albumin, 32 g/L (normal range: 35–50 g/L). A HIV serological test was negative. Other tests, including those for hepatitis markers, TORCH (toxoplasma, rubella virus, cytomegalovirus, and herpes simplex virus), serum ceruloplasmin, as well as Epstein–Barr virus nucleic acid and autoantibody indicated normal results.
After admission, we observed gradual abdominal expansion and hepatomegaly. An abdominal ultrasound indicated hydrops abdominis. Multiple blood routines and bone marrow examinations revealed no abnormalities. Magnetic resonance cholangiopancreatography (MRCP) (Fig. A, B) showed enlarged lymph nodes in the hilar region, in the areas of the hepatic ligament and round ligament, and in the space of the portal cavity. The lymph node enlargements resulted in hepatic hilar biliary duct obstruction and intrahepatic biliary dilation. Extensive peritoneal thickness and a small amount of ascites were observed. The patient was diagnosed with sclerosing cholangitis, abdominal infection, and hepatitis. It was also necessary to exclude cancer. None of the lymph nodes were superficially enlarged; we consulted the surgeon regarding whether biopsy of the abdominal lymph nodes could be performed. Considering the hemophilia, the surgeon advised against lymph node biopsy because the abdominal lymph nodes were small and as the patient was thought to have a high risk of bleeding.
We attempted treatment to protect the patient's liver by reducing enzyme activity, resolving jaundice, and providing anti-inflammatory and supporting therapy. However, all measures proved ineffectual and the patient's condition deteriorated. We found that the ascites had increased gradually. Abdominal contrast-enhanced computed tomography (CT) (Fig. C, D) on day 20 after admission showed a large amount of ascites, some intumescent lymph nodes with abnormal density around the hepatic hilar region or at the root of the mesentery, and diffuse incrassation in the peritoneum and mesentery. After obtaining parental consent, we decided to risk performing abdominal puncture. Tumor cells were found in the ascites (Fig. ). Flow cytometry of the ascites showed B lymphocyte-group cells with restricted light chain expression; these results were considered to indicate lymphoma. Using fluorescence in situ hybridization, both a c-myc gene translocation and a c-myc-IgH gene fusion were detected. Finally, the patient was diagnosed with Burkitt lymphoma, a type of non-Hodgkin lymphoma. He was transferred to the Pediatric Hematology Department of our hospital and treated with a modified B-NHL-BFM-95 protocol. Because of the patient's poor physical condition and malnutrition, he did not tolerate high-intensity chemotherapy. After 3 cycles of chemotherapy, he died from severe infection and bleeding. |
pmc-6531510-1 | The patient was a 66-year-old female referred to us for the management of pancreatic head carcinoma. At age 45, she had undergone a TG combined with a DP and a splenectomy for gastric cancer that had also involved the pancreatic tail (Fig. ). A tumor on the pancreatic head was discovered during a follow-up for repeated adhesive intestinal obstructions. A computed tomography (CT) scan revealed a 15-mm hypovascular tumor on the head of the pancreas. It had an irregular border and was observed to be infiltrating the superior mesenteric vein (SMV). The patient’s CA 19–9 and CEA levels were found to be 24.0 U/ml (normal range, 0–37 U/ml) and 5.0 ng/ml (normal range, 1.0–6.5 ng/ml), respectively. Considering the patient’s post-TG status, a diagnostic endoscopic ultrasonography, or an endoscopic approach for a biopsy could not be attempted. Therefore, the diagnosis of a borderline resectable pancreatic cancer was made based on the National Comprehensive Cancer Network (NCCN) guidelines []. Neo-adjuvant chemotherapy with tegafur, gimeracil, and oteracil potassium (S-1) as well as radiotherapy (dosage, 50.4 Gy/28Fr) was attempted preoperatively. However, the treatment did not produce a marked change in tumor size (Fig. ).
We planned a post-TG PD that would enable us to preserve the pancreatic body and thereby its functions, in order to prevent the sequelae of postoperative severe malnutrition and disorders of glucose metabolism, and which would also allow us to administer adequate postoperative adjuvant chemotherapy to the patient. However, as the splenic artery had been resected in the earlier procedure, it was necessary to identify the blood vessels perfusing the region including the pancreatic body. A preoperative angiography (AG) was performed, which showed that the dorsal pancreatic artery (DPA) supplying the pancreatic body, had branched from the replaced right hepatic artery (Fig. a). Although angiography via both superior mesentic artery (SMA) and celiac artery was performed, the great pancreatic, caudal pancreatic, and the inferior pancreatic arteries could not be identified (Fig. b). Based on these results, we concluded that the patient’s DPA was responsible for maintaining the blood flow to the pancreatic body.
A pancreatic body-preserving PD along with an SMV resection was performed. Using intraoperative deep manipulation, the DPA was carefully left intact. In order to prevent injury to the DPA, we only performed partial lymph node dissection around the SMA. We utilized intraoperative ultrasonography to accurately identify the tumor margin and thus delineate the line of resection on the pancreas. The pancreatic parenchyma was preserved to the maximum extent while ensuring that a safe, margin-negative resection was performed. Following the resection, the patient was administered an intravenous injection of 7.5-mg indocyanine green (ICG) to check the blood supply to the pancreatic remnant. The unimpaired blood flow was confirmed when the pancreatic body demonstrated an immediate fluorescence in response (Fig. ). A subsequent Roux-en-Y reconstruction (pancreatojejunostomy, cholangiojejunostomy, jejunojejunostomy) using a new Roux limb was performed (Fig. ). The operation was completed in 459 min, during which period, a 250-ml blood loss was recorded. Histopathological examination of the resected specimen showed an R0 resection with safe margins (Fig. ), no lymph node metastasis, and a moderate effect of the preoperative therapeutic measures. The tumor was staged grade IIa, as per Evan’s histological classification [], and T3N0M0 and stage IIA according to the 7th edition TNM classification published by the Union for International Cancer Control (UICC) for pancreatic cancer. Furthermore, the pancreatic cancer was diagnosed as a moderately differentiated tubular adenocarcinoma, ly0, v2, ne1, mpd0, and T3N0M0 Stage IIA according to the General Rules for the Study of Pancreatic Cancer (7th edition).
The course of recovery was uneventful, and the patient was discharged on postoperative day 13. The stable blood supply to the remnant pancreas was confirmed on an enhanced CT scan (Fig. ). The patient had required insulin therapy for a short duration postoperatively but eventually was able to achieve good glycemic control using an oral hypoglycemic agent. At discharge, the patient weighed 35.2 kg (body mass index, 15.4 kg/m2) and had lost 1.7 kg, as compared to her preoperative weight. Adjuvant chemotherapy with S-1 was then administered for 6 months. The transition in the levels of tumor markers and HbA1c values during the course of treatment have been graphically represented in Fig. . The patient passed away 21 months after the operation and 24 months after the start of preoperative therapy, due to a recurrence of peritoneal metastasis. |
pmc-6531558-1 | An 82-year-old Japanese woman presented at another hospital with right lower abdominal pain of 2 months’ duration. A colonoscopy revealed ascending colon cancer, and she was referred to our hospital. She had no previous history of abnormal bleeding. Her brother had pointed out any abnormalities in his blood coagulation test; however, we could not find the details because he had already died. Her family history except the brother was negative for bleeding tendencies, purpura, and hemophilia. She did not smoke or drink alcohol. She had no allergies. A total colonoscopy showed an ascending colon tumor with severe stenosis (Fig. ). Computed tomography revealed the wall thickness of the ascending colon (Fig. ). There were no distant metastases on the liver or lungs. Prolonged activated partial thromboplastin time (APTT 93.9 s) was found incidentally during a routine preoperative examination. However, the prothrombin time (11.2 s), platelet count (480 × 103/L), and liver function test results were all within normal limits. We conducted a cross mixing test, and she was diagnosed with a deficiency pattern (Fig. ). The activities of factors VIII, IX, XII, and von Willebrand factor were normal. The plasma activity of FXI was 3%, and the diagnosis of FXI deficiency was confirmed. It took about 2 weeks from confirmation of an abnormal APTT time to diagnosis of FXI deficiency. A total of eight units of FFP were transfused for 2 days before the operation. After the transfusion, APTT improved to 37 s, and FXI also increased from 3% to 25% as a result (Fig. ).
It takes about 3 days to obtain the result of FXI plasma activity in our hospital. We could not know the FXI plasma activity during operation; therefore, we used the APTT level instead of this and decided to do the operation. We performed laparoscopic ileocolic resection with our standard procedure. The operation time was 133 min and the intraoperative blood loss was 10 mL. We did not find the bleeding tendency in intraoperative findings, and we decided not to administrate FFP intraoperatively. The level of APTT on the day after surgery was 39 s. No complications including postoperative bleeding occurred in her postoperative course without additional transfusion of FFP. The final stage was T3N1M0 stage IIIa, according to the Japanese Colorectal Cancer Treatment Code 8th Edition. She was discharged on the seventh postoperative day. |
pmc-6531768-1 | A 24-year-old pregnant woman, at 28-weeks’ gestation, presented with bilateral visual impairment that started one day before. Physical examination and laboratory studies revealed hypertension (170/100mmHg), proteinuria (100mg/ dl), elevated transaminases and decreased platelet count. Visual acuity was 20/ 100 with -1.00-0.50x4 correction in the right; and 20/ 50 with -1.00+0.50x71 correction in the left eye. Intraocular pressure was 14 mmHg bilaterally. While the anterior segment examination was normal, fundus examination showed bilateral retinal detachments affecting the posterior pole ().
Spectral-domain optical coherence tomography (SD-OCT) demonstrated bilateral SRD involving the center of the macula accompanied by intraretinal fluid (,).
The patient underwent cesarean section the same day by obstetrician with patient approval. Following delivery, nifedipine 30mg po bid was commenced due to high blood pressure (150/ 80 mmHg). Due to the depressed consciousness of the patient, MRI was performed and T2-FLAIR sequences demonstrated hyperintense vasogenic edema, particularly, the white matter of the left occipital lobe (). On day 4 while confusion persisted, SRD resolved () and visual acuity improved to 20/ 25 bilaterally. On day 9 cranial MRI showed complete resolution of the vasogenic edema of the left occipital lobe (). A repeated ophthalmoscopy revealed complete resolution of SRD and normal visual acuity, bilaterally (,). At 6-weeks after delivery, blood pressure was 110/ 60mmHg, and the patient fully recovered. A final ophthalmologic examination was unremarkable and showed normal outcomes in terms of visual acuity, intraocular pressure, pupillary light reflex, anterior segment, and fundus examinations. SD-OCT revealed attached macula. The patient did not accept further etiopathological studies with FFA and ICG at the presentation and after the delivery. |
pmc-6531770-1 | We present the case of a 13-year-old female patient, known with severe visual field loss, who referred for another opinion regarding the ophthalmological diagnosis.
Anamnesis at presentation revealed that at the age of 9 years and 3 months, on a routine ophthalmological examination, papillary calcification and retinal hemorrhage were discovered in the left eye. At that moment, the suspicion of intracranial calcifications was raised. The patient underwent clinical neurological examination, EEG, and cerebral MRI, all of them revealing a normal aspect. The patient was also recommended fluorescein angiography, which showed papillary autofluorescence. The diagnosis established then was papillary drusen in both eyes, buried in the right eye and mixed in the left eye and the patient was recommended to keep it under observation, together with a periodical examination of the visual field.
The patient had had multiple examinations of the visual field over the time.
The first visual field examination of the right eye showed an arcuate nasal defect, in the superior nasal quadrant, sketching an aspect of nasal step, structure of the sensitivity defect that in 3 years time evolved into a quadranopsia.
In the left eye, the first visual field examination showed inferior nasal quadranopsia, extended superiorly with a nasal arcuate defect respecting 20° centrally, which after three years evolved into a paracentral diffuse defect with an island of central vision of 5°.
However, over the time, the examination of the visual field was made with different types of machines, and no correlation of the modifications could be made objectively.
The patient received several different diagnoses from several different ophthalmologists, among which optic nerve drusen; papillary oedema and hamartoma have to be mentioned.
At presentation, the patient’s visual acuity was 20/ 20 with correction for the RE and 20/ 20 without correction for the LE, with a refraction ROD: -1 DSf<> -0.75 DCyl, 179* and ROS: +0.50 DSf<>-0.75 DCyl, 167* and a cycloplegic refraction: OD: -0.75 DSf<> -1 DCyl, 168*, OS: +0.75 DSf<> -1 DCyl, 170*.
The intraocular pressure was 19 mmHg GAT in the right eye and 13 mmHg GAT in the left eye.
Slit lamp examination of the anterior segment revealed no pathological changes for both eyes, and the red-discrimination test was also normal.
Fundoscopy presented only with papillary pathological modifications.
The optic disc in the right eye was elevated, with relatively clear margins, pink color, and the absence of cupping. At 5 o’clock meridian, a nodular yellow mass, with irregular outline, could be noticed ().
In the left eye, the optic disc was also elevated, pale, of irregular outline, and the absence of cupping was noticed. Nodular, yellow, reflective protrusions, with irregular contour and brambleberry shape could be noticed ().
The retinal vessels, the macula, and the retinal periphery presented no pathological changes in either of the eyes.
The clinical examination suggested the diagnosis of optic nerve drusen in both eyes. B-scan ultrasonography and optical coherence tomography (OCT) examinations were used for the confirmation of the diagnosis.
B-scan ultrasonography is considered the gold standard method for the detection of optic disc drusen. In this patient’s case, it showed round, hyperechoic structures, observed at the optic nerves of both eyes. The A-scan mode, which was overlapped on the structure only for the left eye, showed hyperreflectivity at the anterior side of the optic nerve, of supraretinal intensity.
Optical coherence tomography is a useful examination in the assessment of the structure and the anatomical shape of the drusen, and in the analysis of retinal nerve fiber layer (RNFL) and GCL-IPL complex.
For patients under 18 years old, however, there is no normative database regarding the normal values of the analyzed parameters, therefore these analyses are useful only for patient’s follow-ups.
The OCT scan of the optic nerve showed a prominent aspect of the optic disc, with a lower value of average RNFL thickness in the left eye compared to the right eye ().
Macula was structurally normal, with an asymmetry of macular thickness, thinner in the left eye, compared to the right eye ().
There was also an asymmetry of thickness regarding the GCL-IPL complex, which was thinner in the left eye compared to the right eye ().
The investigations confirmed the diagnosis of optic nerve drusen in both eyes.
The differential diagnosis in the case of this patient took into consideration the following pathologies:
• Papilloedema – excluded by B-scan ultrasound;
• The existence of an intracranial expansive process – excluded by clinical and imagistic examinations;
• Optic nerve tumors
o Astrocytic hamartoma – the proliferation of astrocytic cells occurs above the optic disc, whereas optic disc drusen is located in the substance of the optic nerve.
o Optic nerve sheath meningioma – excluded by clinical and imagistic examinations.
• Leber optic neuropathy – it typically presents with severe loss of central vision.
• Infiltration of the optic nerve (leukemia, lymphoma) – excluded by normal laboratory tests.
The patient’s visual field examination at presentation revealed a superior nasal altitudinal scotoma at the right eye (), and at the left eye an important constriction of the visual field, with the preservation of a small 15* island of temporal paracentral vision ().
The patient was not recommended any treatment, but only periodical follow-up with visual field examination at every 4-6 months, and annual OCT.
The patient came back a year later for follow-up. At examination, there was no progression of the visual field alterations (,), but the intraocular pressure was at the superior level of the normal range, 21 mmHg GAT for the right eye and 20 mmHg GAT for the left eye.
Therefore, the patient was recommended the treatment with a prostaglandin analogue to prevent the exacerbation of the visual field loss in order to attenuate the mechanical compression on the ganglion cells axons and to improve the blood flow to the optic nerve head. |
pmc-6531822-1 | A 46 year old female G0P0 was referred by a gastroenterologist to our surgical outpatient consultation for management of a giant intraabdominal mass. The history dates back nine months prior to her consultation, where she noted a progressive abdominal distension associated with constant non-colicky abdominal pains, nausea and dyspeptic symptoms. Due to limited financial resources and lacking health coverage, she does not consult in a hospital, but turns to traditional healers for several months.
The medical history was remarkable for a sudden acute flaccid lower limb paralysis since the age of 5 years, with an undetermined aetiology, having nailed her since then on a wheelchair. Physical examination showed a grossly distended abdomen (), with a large regular abdomino-pelvic mass lateralise to the left size measuring about 38 cm on it largest dimeter.
In front of this unusual case and having knowledge of the financial difficulties of the patient, our hospital decided to take charge of her free of charge.
An abdominopelvic CT scan was then performed and showed an intraabdominal mesenteric cyst attached to the transverse colon (). She was then programmed for an exploratory laparotomy. Preoperative workups were normal. Under general anaesthesia, we performed a midline incision. Intraoperative findings consisted of a voluminous mesenteric mass attached to the transverse mesocolon between the posterior surface of the stomach and the transverse colon (). There was no ascites and no enlarged mesenteric lymph nodes. The mass was enucleated, with careful dissection of the transverse mesocolic vessels to which it was intimately bound.
Grossly (), the mass measured about 33 × 30 x 25 cm. The mass was and weighed 16 kg. The postoperative course was uneventful with a discharge on postoperative day five. Histopathology revealed a lymphangioma of the transverse mesocolon. |
pmc-6531828-1 | A 17-year-old female presented with a tender periumbilical mass three months post-laparoscopic cholecystectomy for symptomatic cholelithiasis. The mass was noticed two days prior to her presentation and appeared just deep to the infra-umbilical incision site utilised in her laparoscopic cholecystectomy. It measured 4 × 2 cm and was extremely tender and warm on palpation. The patient stated she was lethargic but denied any other symptoms. Her vitals were stable on admission (Temp 36.3 °C; RR 20; SpO2 100% RA; HR 62; BP 122/78) and she appeared systemically well. Biochemically, she had a raised white cell count (17.7) and C-reactive protein (65).
Focused ultrasonography of the region demonstrated a heterogeneous predominantly hypoechoic fluid collection in the anterior abdominal wall just below the infra-umbilical incision site. This appears to communicate with a hyperechoic structure just below the abdominal wall (). Discussion with the radiologist suggested that these findings may be due to post-surgical soft tissue changes or an incisional hernia, but these differentials were considered unlikely in the context of the patient’s clinical presentation. An abdominal and pelvic CT was performed for further clarification and this revealed an intra-abdominal collection draining into the umbilicus ().
The patient returned to theatres for exploration of her umbilical wound. A re-incision of her previous infra-umbilical port site resulted in direct contact with an abscess lying immediately deep to it. Significant pus and gallstones were noted in the cavity along with a piece of tissue that was later histopathologically confirmed to be a gallbladder remnant. The cavity was removed of all its content and washed with hydrogen peroxide and normal saline. Inspection of the underlying fascia was shown to be intact, so the intraperitoneal contents were not explored. The wound was left opened post-operatively and packed with saline soaked gauzes daily for delayed primary closure. On discharge, the wound appeared uninflamed and the patient was instructed to have daily dressing changes in the community.
Unfortunately, the patient re-presented to hospital 5 days following discharge. She complained about gallstones being expressed from her poorly healed wound (). A repeat ultrasonography of the abdominal wall revealed the presence of two echogenic masses representing gallstones (). The patient underwent an immediate diagnostic laparoscopy, entering the peritoneal cavity via her previous incisions utilised in her laparoscopic cholecystectomy. This revealed a retained gallbladder with numerous cholesterol stones (). A combination of sharp and blunt dissection delineated the retained gallbladder from adherent omentum and small bowel loops. All retained tissue and stones were collected and successfully removed. Postoperatively, the patient completed an one week course of amoxicillin/clavulanic acid 875/125 mg twice daily and made an uneventful recovery with delayed primary closure of her umbilicus.
The root cause of this patient’s presentation was traced back to her laparoscopic cholecystectomy performed three months prior to her initial presentation. The patient’s operative report noted difficulty removing the gallbladder retrieval bag from the infra-umbilical incision site but did not explicitly state any complications. On discussion with the primary and assisting surgeons who performed the laparoscopic cholecystectomy, it was clear that the retrieval bag used to remove the gallbladder from the peritoneal cavity ruptured as it was pulled from the infra-umbilical port site. This would have transected the gallbladder, causing its remnants and associated gallstones to be retained in the peritoneal cavity. |
pmc-6531847-1 | A 43-year-old woman presented in 2018 as an emergency to our psychiatric outpatient department. She reported depressed mood, sleep difficulties, and loss of energy, present for several weeks. Two hours earlier, she had been severely verbally offended by her colleague, which had induced emotional stress and led her to introduce herself to our clinic. Psychopathological findings on time of admission were anxiety, depressed mood, anger, loss of drive, anhedonia, and insomnia, consistent with major depressive disorder. Use of psychotropic substances was denied. The family history was positive for depressive disorder. She was a smoker. The somatic history revealed hypertension and neurodermatitis. The daily medication was diclofenac 75 mg once daily (OD). On admission, she additionally complained of persistent, non-respiration-dependent left-thoracic chest pain, lasting for about 2 h prior to admission; in addition, hyperventilation, symmetric leg tingling, and heaviness had started together with the chest pain; however, it had resolved about 30 min prior to admission. No past episodes of dyspnea or disturbances of consciousness were reported. Physical examination and resting ECG, performed immediately after introduction to our outpatient department, were unremarkable. Laboratory investigations revealed a significantly elevated troponin T (243 ng/ml; reference <14 ng/ml). We then transferred the patient to the department of cardiology, where another unremarkable ECG was performed (findings: sinus rhythm, heart rate 84/min, normal cardiac axis, the transition from S>R wave to R>S wave was between V3 and V3, no repolarization disorders). An emergency cardiac catheterization, which also included coronary angiography and ventriculography, was performed on the day of presentation in the department of cardiology and demonstrated severely reduced left ventricular function with typical apical ballooning; a coronary heart disease was excluded. The N-terminal (NT)-pro-brain natriuretic peptide (BNP) levels were elevated at 307.0 pg/ml (reference: <130.0 pg/ml). A cardiac MRI was performed about 47 h after the initial presentation in the psychiatry outpatient department and showed a nonhypertrophied left ventricle with mildly reduced systolic function with an ejection fraction of 52%, consistent with TCM; in the cardiac MRI, the right ventricle was not hypertrophied and its ejection fraction was 55%; there was evidence of a beginning diastolic dysfunction in the left and right ventricles and no evidence of myocardial scars or fibrosis. The patient was monitored for 5 days in the department of cardiology and was then discharged without any cardiac symptoms or complications; on follow-up 8 weeks later, the clinical investigation was unremarkable. |
pmc-6531863-1 | A 23-year-old male patient was referred to our public teaching hospital with two days history of colicky periumbilical abdominal pain, feculent vomiting and obstipation for one day. He had not had prior surgery, was HIV negative and had been generally well prior to this illness. He did however, admit to recurrent right lower quadrant abdominal pain throughout his life which had never been previously investigated. He had no relevant drug, family, psychosocial or genetic history.
Upon examination, he appeared dehydrated, lethargic and in mild respiratory distress. He had a temperature of 36.9 °C, a respiratory rate of 40 breaths per minute and a saturation of 82%. He had moderate abdominal distension, visible peristaltic movements as well as peritonitis with rigidity and rebound tenderness. Digital rectal examination and assessment of other systems were unremarkable.
Blood investigations revealed leucocytosis of 13.11/mm3 (4–11/mm3), haemoglobin of 19.9 g/dl (14–17 g/dl), reflecting dehydration. He also had a high urea: 97 mMol/L (2–6.7 mMol/L) and serum sodium was 131 mMol/L (133–146 mMol/L).
An acute abdominal series of x-rays was performed which showed distended loops of small bowel with multiple air fluid levels.
A diagnosis of distal small bowel obstruction was made. The patient was immediately admitted and catheterised; resuscitation was instituted with crystalloids, intravenous antibiotics and nasogastric decompression. Oxygen was administered per face mask and he was taken to the operating room.
At laparotomy, haemorrhagic fluid was noted on peritoneal entry. An axial 360° counter-clockwise volvulus of the midgut from terminal ileum to mid transverse colon was encountered around a very narrow mesenteric base (). There was gangrene of the involved intestinal segments which appeared to be grossly distended and the hepatic flexure was noted to be unfixed and mobile (c). The duodenum was normally situated as was the ligament of Treitz. Right hemicolectomy was performed with primary ileocolic anastomosis and peritoneal lavage. The rest of the bowel was normal, the splenic flexure was normally fixed in the left upper quadrant and there was no distal obstruction. The operation was performed by one surgical trainee with 4 years of specialised training and one general surgeon with 3 years’ experience. Post-operatively, the patient was admitted to the intensive care unit where broad-spectrum antibiotics were continued. He did not require inotropic support and was extubated on day 3 post-operatively. He resumed oral feeds on day 4 and was eventually discharged 10 days post-operatively. At follow-up in the outpatient’s clinic 1 week later he was having an uneventful recovery with no adverse events. At further review three months later, he had no recurrence of symptoms. The patient and his family were relieved that he had done so well. Histology results confirmed gangrenous loops of bowel with no other histologically evident pathology. There are no plans for continued follow-up. |
pmc-6532169-1 | A 50-year-old-Caucasian-female with 20-pack smoking years presented with a history of anorexia, lethargy, unintentional weight loss, and depression. Auscultation revealed absent air entry on the left chest. Laboratory workup demonstrated severe iron-deficiency anemia, low albumin, and normal serum alpha-fetoprotein (AFP) and beta-hCG. Pre-operative LDH was not included due to the low suspicion of nonseminomatous dysembryoma or lymphoma.
Chest X-ray and computed-tomography revealed a left-thoracic heterogeneously dense 20x25cm mass compressing the heart and lung with a major mediastinal shift (Figs. and ). A preoperative MRI was not performed as there was no evidence of invasion into any vital structures. Five IR CT-guided core needle biopsies from different regions of the mass yielded minute fragments of amorphous, acellular material which was insufficient for pathological diagnosis. Core needle biopsy has a diagnostic yield of 77% and can be inadequate for immunohistochemistry and flow cytometry evaluation []. The profusely dense material ultimately found during the operation largely contributed to the inability of the core needle biopsy to provide a diagnosis.
A preoperative clinical diagnosis of mediastinal teratoma was entertained. Her BMI on admission was 18.4 and she had a Karnofsky performance scale index between 10 and 20. Because of the severe deconditioned status of the patient and cachexia she was put on enteral feeding and sent to a rehabilitation program for 3 weeks. Thereafter the patient underwent left lateral thoracotomy and complete resection of the mediastinal tumor. Lateral thoracotomy was preferred over median sternotomy in this case because the mass did not cross midline and it extended below the level of the pulmonary hilum. A thoracotomy incision is standard approach to a middle or posterior mediastinal mass [, , ]. Intraoperatively the tumor was firmly adherent to the pulmonary artery and pericardium. It was necessary to resect the anterior aspect of the tumor first to obtain proximal control of the pulmonary artery before completely separating the tumor from the artery.
Macroscopic examination demonstrated yellow pasty-material with teratomatous components including hair and skin (Fig. ). Histologic description was consistent with mature squamous cells and occasional glandular cells.
The patient required prolonged chest tube drainage to allow for shift of the mediastinum and expansion of the chronically atelectatic lung. She continued physical therapy and enteral feeding supplementation due to severe deconditioning. There were no complications and she was discharged on the 17th postoperative day. At the first follow-up appointment 18 days after discharge she was regaining weight and strength without any clinical evidence of recurrence. Her BMI had increased from 18.4 to 21, and her Karnofsky performance scale index was between 80 and 90, quickly approaching 100. She was scheduled again for follow up in 2 months. |
pmc-6532186-1 | An 8-year-old male Mexican patient was referred to our Institution with a diagnosis of CF. He was the fifth child born from healthy non-consanguineous parents, without a family history of the disease (Fig. ). The mother previously had a spontaneous abortion after 16 weeks gestation by anencephaly. Clinical onset occurred at one month of age with steatorrhea, malabsorption, poor weight gain, and anemia. At 5 months of age, the patient had recurrent respiratory tract infections colonized by Pseudomonas aeruginosa. He was diagnosed with CF at 18 months of age, with elevated sweat chloride levels (88, 130, and 129 mmol/l). Currently, the patient’s weight is below the 5th percentile and his height is between the 5th and 10th percentiles.
Using samples from the patient, his parents, and his four siblings, we extracted genomic DNA from peripheral blood lymphocytes using the QIAamp DNA Blood Maxi kit (Qiagen, Valencia, CA, USA) following the manufacturer’s protocol. The index case was screened for the five pathogenic variants that are most frequent in the Mexican population (c.1521_1523delCTT, p.Phe508del; c.1624G > T, p.Gly542*; c.1519_1521delATC, p.Ile507del; c.1646G > A, p.Ser549Asn; and c.3909C > G, p.Asn1303Lys [–]), using PCR-mediated site-directed mutagenesis (PSM) as previously reported [, ]. Since none of the above-listed variants were identified, we performed complete sequencing of the CFTR gene using next generation sequencing (NGS; Illumina HiSeq 2500 sequencer). The NGS data were analyzed using the Genome Analysis Toolkit: UnifiedGenotyper (GATK) and Integrative Genomics Viewer (IGV) software. To predict the functional effect of the variant on the protein, we performed in silico analysis using Variant Effect Predictor (VEP) software [].
Data collection and molecular analysis were approved by the Research and Ethics Committees at INMEGEN. The parents provided written consent for the child participants, and all children assented.
Our initial screening of the patient revealed none of the pathogenic variants that are most frequent in the Mexican population. Analysis of the completely sequenced CFTR gene using GATK and IGV software revealed that the patient carried two different variants in trans. One was c.3266G > A (p.Trp1089*, W1089*), which is a previously report CF-causing variation, and the other was the novel variant c.1762G > T, p.Glu588* (, []). The novel variation involved the change of a G for T in codon 588 of exon 12. Functional effect prediction analysis revealed that this variant induced a premature stop codon (c.1762G > T, p.Glu588*).
Both variants were validated via automated Sanger sequencing. To identify their carrier status, we screened both parents for these variants. The patient’s mother carried only the c.3266G > A (p.Trp1089*, W1089*) variant, while the father carried neither of the variants identified in the patient (Fig. ). Since the father was not a carrier of any CF variant, paternity was tested using 23 STR genetic markers, which confirmed a paternal probability of > 99.99%. Thus, c.1762G > T, p.Glu588* was determined to be a de novo variant. The patient’s three sisters were carriers of the c.3266G > A, p.Trp1089* variant, while his brother showed a wild-type CFTR sequence. Additionally, two maternal uncles and the grandmother were also found to carry the c.3266G > A, p.Trp1089 variant. |
pmc-6532217-1 | Patient 1 was a 3-year-old female born at term by cesarean delivery with the history of aspiration of amniotic fluid and was not awaked easily for one day after birth. She was admitted to the hospital due to cough for 5 days and intermittent convulsions for 3 days. She had partial convulsions without fever while awake, she slept after remission, most convulsions were less than two minutes in duration. She had no movement or language disorders during the postictal period. She also presented with developmental delay, especially language delay. Cerebral MRI displayed symmetrical hyperintensity of bilateral basal ganglia and globus pallidus on T2-weighted image (T2WI) and hyperintensity of the bilateral parietal lobe white matter on fluid-attenuated inversion recovery (FLAIR) imaging. EEG revealed high voltage of delta and theta wave at approximately 2–4 Hz. Urinary organic acid analysis showed elevated GHB concentrations.
Molecular analysis of ALDH5A1 confirmed a compound heterozygous mutation of c.527G > A/c.691G > A (p.G176E/p.E231K) in exons 3 and 4. c.527G > A was inherited from her mother, whereas c.691G > A was inherited from her father. We have previously reported these two mutations [, ]. Bioinformatics analysis indicated that p.G176 and p.E231 are highly conserved among species. These mutations were predicted to be “Probably damaging” and “Possibly damaging” based on the Polyphen2 software. |
pmc-6532217-2 | Patient 2 is an 86-day-old male conceived through in vitro fertilization and born at 37 weeks by cesarean section due to cord around the neck. His mother had an abnormal reproductive history. Her first baby died of convulsion at 23 days after birth, while her second pregnancy was ectopic. This patient was referred to the hospital due to intermittent convulsions for more than 2 months and exacerbation for 2 days. Partial convulsions occurred while he was awake or asleep without fever. He was back to normal without movement disorders. He presented with decreased attention, poor head control, and thumb abduction. Cerebral MRI revealed patchy hyperintensity in bilateral frontal and parietal lobe on T2WI. Widened ventricle and the interval outside the cerebral were also noted in the cerebral MRI. EEG showed sleep spindle asynchrony. Blood gas analysis indicated metabolic acidosis. GHB in urine was significantly elevated according to urinary organic acid analysis. Unfortunately, this patient died of pneumonia despite rescue efforts.
A homozygous mutation variant, c.1529C > T (p.S510F), was identified in exon 10 of the ALDH5A1 gene in this patient. Subsequent targeted mutational analysis of exon 10 of his mother confirmed the segregation of the variant. The healthy father did not carry the sequence variant. p.S510 is highly conserved, its mutation is predicted to be “Probably damaging.” |
pmc-6532217-3 | Patient 3 was a 5-year-old boy born at full term with the history of hypoxia after birth. He was admitted to the hospital due to developmental delay complicated with lack of sleep. Urinary organic acid analysis showed a high level of GHB. EEG and cerebral MRI were unclear because both were performed by other hospitals.
This patient harbored a novel homozygous mutation of c.800 T > G (p.V267G) in exon 5, which he inherited from his parents. Bioinformatics analysis revealed that p.V267 is highly conserved among species. The mutation was predicted to be “probably damaging” and “disease causing” with a score of approximately equal to 1. |
pmc-6532217-4 | Patient 4 was an 8-month-old girl born at full term by natural delivery and referred to the hospital because of developmental delay. The clinical manifestations showed slow pupillary light reflex and hyporeflexia and hypotonia of all limbs. Cerebral MRI revealed widened ventricle, cistern, and sulci, which was considered as delayed myelination (Fig.). EEG was normal. Urinary organic acid analysis showed an increased excretion of GHB.
A compound heterozygous mutation of c.1344-2delA/c.1529C > T was identified in exons 9 and 10 in this girl. The mutation c.1344-2delA located in the intron splice site, and p.S510F (c.1529C > T) belongs to the NAD-binding domain. These mutations are separately inherited from both of her parents. |
pmc-6532225-1 | A 73-year-old Caucasian woman presented with a 3-month history of pain and fullness in the right lower quadrant of the abdomen, which had increased in recent days. The patient’s previous history included intermittent fever, tremor attacks, and use of multiple antibiotics. She was married and had two children (40 and 51 years old), both healthy. She did not smoke tobacco and consumed no alcohol. She had no history of surgery. Her father was 95 years old, and her mother had died at age 80 years of coronary artery disease. None of them had malignancies in their past history.
The patient appeared toxic; her temperature, pulse rate, respiratory rate, and blood pressure were 38.8 °C, 110 beats/min, 30 breaths/min, and 90/50 mmHg, respectively. A physical examination revealed that the patient had a palpable mass in the right lower abdominal quadrant.
The initial laboratory test results showed significant leukocytosis with a white blood cell (WBC) count of 37,100/μl, elevated C-reactive protein (CRP) of 218 mg/L, and mildly elevated creatinine of 1.2 mg/dl. Abdominopelvic computed tomography (CT) revealed heterogeneous and irregular minimal contrast enhancement in the pelvic ectopic kidney parenchyma. Radiologists reported that the images were consistent with chronic pyelonephritis (Fig. ). A Tc-99m dimercaptosuccinic acid (DMSA) renal scan demonstrated a nonfunctioning right pelvic ectopic kidney.
The patient underwent open simple nephrectomy via modified Gibson incision.
During surgery, a huge kidney specimen measuring 15 × 10 × 8 cm was obtained. The whole mass was a distended, saclike structure without any grossly visible renal tissue. Pathological examination showed renal pelvis SCC 8 cm in diameter infiltrating the renal capsule, in addition to perinephritic fatty tissue. Microscopic examination revealed SCC structures in well-differentiated areas and sarcomatoid changes in poorly differentiated areas. The nuclear grade of the tumor was 4, and it had focal sarcomatoid morphology (Fig. ). However, other microscopic findings supported the presence of concurrent chronic pyelonephritis. 18F-fluorodeoxyglucose (FDG) positron emission tomography/CT showed a hypermetabolic mass measuring 4 × 3 cm in the right parahilar area of the thorax, hypermetabolic nodules in both lung parenchyma, and increased FDG uptake in iliac, acetabulum, femur, head, neck, sternum, and costal bones. The patient’s diagnosis was accepted as T4N0M1 renal pelvis SCC, and she was admitted to the intensive care unit for respiratory distress on the seventh postoperative day. She had died of acute respiratory distress syndrome by the first-month follow-up visit. No autopsy was performed after the patient died. |
pmc-6532247-1 | An asymptomatic 44-year-old female with no medical history or history of trauma to the chest wall was admitted due to a right chest wall mass which was incidentally discovered in the shower. Chest computed tomography (CT) demonstrated a tumor, measuring 8.5 cm in diameter. Osteosclerosis was present on the top of the lesion along with calcification in different places and thickening on the nearby parietal pleura and diaphragm (Fig. a,b).
The laboratory investigation including serum tumor marker levels and routine hematologic, blood biochemistry results were normal. The patient underwent right lateral minithoracotomy in which a partial excision of the rib was performed, the intraoperative exploration showed the diaphragm was closely adhered to the giant mass. The large chest wall defect caused by rib resection was reconstructed by performing a polyester patch draft (Fig. a,b). When thoracic incision was closed, the right lung was insufflated by anesthetist to expel gas in the right thoracic cavity. No thoracic close drainage was placed to reduce postoperative pain and complications.
The patient had an uneventful recovery and discharged on the sixth postoperative day. Five months after the operation, she was doing well, without any evidence of local recurrence. A definite diagnosis of cavernous hemangioma was made based on histopathology examination results of the resected mass (Fig. ). The mass was composed of thin-walled blood vessels with dilated channels containing red blood cells and lined by a single layer of endothelial cells (Fig. ). |
pmc-6532299-1 | Our patient is a 58-year-old male with past medical history of hypertension and distant right femur fracture who was involved in a motocross accident suffering left- (1st-12th) and right-sided (7th, 9th, and 12th) rib fractures with bilateral hemopneumothoraces requiring bilateral chest tube placements. The patient also suffered a right intertrochanteric and a peri-implant femur fracture as well as a nondisplaced ulnar styloid process fracture. The patient was transferred from an outside hospital for further care. Upon arrival the patient was remarkably asymptomatic, in normal sinus rhythm, hemodynamically and respiratory stable, maintaining an O2 saturation between 95% and 100% with minimal supplemental O2 via nasal cannula. The patient denied significant chest pain or shortness of breath despite his significant injury burden. ECG findings showed some ST wave abnormalities suggestive of early repolarization. An initial troponin level of 0.15 normalized within 24h of admission. Given the patient's injury pattern and troponin leak in the absence of known coronary artery disease (CAD), congestive heart failure (CHF) pulmonary embolism (PE), or shock, a formal TTE was obtained. TTE revealed severe tricuspid regurgitation secondary to flail anterior tricuspid valve leaflet with preserved right ventricular geometry and systolic function. The cardiac surgery team recommended outpatient follow-up for elective repair in two months with repeat TTE.
The femur fracture was repaired on hospital day two under general anesthesia and the patient recovered well. The chest tubes could be sequentially removed without recurrence of pneumothoraces over the course of the admission. On hospital day three the patient experienced an episode of atrial fibrillation with rapid ventricular response that responded well to a single 5 mg intravenous bolus of metoprolol. After a few hours the patient converted back to normal sinus rhythm, in which he remained until the day of discharge on hospital day seven. The patient continued to deny any palpitations, shortness of breath, or radiating chest pain. By the time of discharge, the patient was able to ambulate, and his pain was well controlled with oral analgesics. Unfortunately, by one year after hospital discharge, the patient had not followed up with regard to his newly diagnosed tricuspid regurgitation. |
pmc-6532300-1 | A 65-year-old female patient was admitted to the hospital complaining of headaches and convulsions over the previous three months. The patient was conscious and alert with stable vital signs. Computer tomography (CT) scan showed a well-defined left-temporal extra-axial lesion of mixed density, predominantly hypodense (fat density), causing calvarian bone hyperostosis with a localized mass effect on the adjacent temporal lobe. Significant vasogenic edema with associated effacement of the left hemispheric sulci, compression of the ipsilateral ventricle, early left uncal herniation, and a rightward midline shift also were noted. MRI showed a dural-based focal mass in the left-temporal lobe exhibiting a mass effect on the adjacent parenchyma and a large area of perifocal edema (delete vasogenic) causing compression of the ipsilateral ventricle and approximately 6mm midline shift towards the right. Evidence of a high fat component (high T1 and T2 mass) showing avid enhancement with a dural tail sign was present (Figures and ). As a result, the patient underwent a craniotomy and a biopsy was taken. |
pmc-6532307-1 | A 73-year-old Caucasian male with a past medical history of coronary artery disease, heart failure, atrial fibrillation, chronic obstructive pulmonary disease, and type-2 diabetes mellitus was admitted after having a witnessed seizure at his nursing rehabilitation facility. When emergency medical services arrived, the patient was found to be hypoglycemic with blood glucose (BG) of 21 mg/dL. He was administered 25 g of dextrose 50% (D50) resulting in some improvement in his mental status. He was then transferred to our emergency department (ED).
In the ED, the patient was minimally responsive to both verbal and physical cues. The initial laboratory results were significant for hypokalemia with potassium of 2.9 mmol/L (normal 3.6-5.1 mmol/L), acute kidney injury with serum creatinine at 2.52 mg/dL (normal 0.7-1.3 mg/dL; baseline approximately 1.5 mg/dL), albumin of 2 g/dL (normal 3.5-5.7 g/dL), and hypoglycemia with a BG of 34 mg/dL (normal 70-105 mg/dL). Liver function tests (LFTs) were all within normal limits. The patient was immediately given 50 g of D50 resulting in a repeat BG of 134 mg/dL. Review of the patient's home medication list identified that the patient was prescribed levofloxacin 750 mg daily for pneumonia on discharge from a hospitalization three days prior with no other medication changes. Chronic medications were inclusive of aspirin 81 mg daily, atorvastatin 80 mg daily, glipizide 10 mg daily, losartan 25 mg daily, mometasone 220 mcg/inhalation three times daily, spironolactone 25 mg daily, torsemide 100 mg daily, and warfarin. The patient required four additional boluses of D50 and a continuous dextrose 10% (D10) infusion to maintain euglycemia before transfer to the intensive care unit (ICU).
In the ICU, the patient continued to experience multiple episodes of severe rebound hypoglycemia despite an additional four boluses of D50, increasing the D10 infusion rate to 100 mL/hr (see ) and withholding home medications. Glucagon 1 mg intramuscularly only led to a transient increase in BG. Given the patient's refractory hypoglycemia to standard supportive care therapies, octreotide 50 mcg subcutaneously was administered every six hours for a total of three doses. Within three hours of administration of the first dose of octreotide, there was a significant increase in BG levels (ranging from 170 to 237 mg/dL) and no further D50 boluses were required. Due to persistent hyperglycemia (BG >250 mg/dL), the D10 infusion was discontinued 5.5 hours after the second dose of octreotide. The patient eventually required an insulin infusion the following day and was transferred to the floor. The patient was ultimately discharged to his nursing rehabilitation facility in stable condition. |
pmc-6532322-1 | This is a 43-year-old white male patient with past medical history of hypertension and obesity. He was referred to the gastroenterology clinic from his primary care physician due to elevated liver function test found on routine annual physical exam. At the time of evaluation he had no complaint, and he denied any abdominal pain, nausea, vomiting, jaundice, weight changes, skin lesions, or discoloration. He also denied any prior history of illicit drug use, high risk sexual behavior, sexually transmitted diseases, or family history of liver or autoimmune diseases. His physical examination did not reveal any skin lesions suspicious for neurofibromatosis-1.
His initial workup revealed a random glucose level of 85 mg/dl, calcium level 9.5 mg/dl, total bilirubin 1.3 mg/dl, direct bilirubin 0.6 mg/dl, alkaline phosphatase 640 U/L, AST 220 U/L, ALT of 494 U/L, total protein 7.1 g/dl and albumin 4.6 g/dl. Further workup revealed negative viral hepatitis panel, antinuclear antibodies, anti-mitochondrial antibodies and anti-smooth muscle antibodies. Iron studies, ceruloplasmin and alpha-1 antitrypsin were within normal limits.
Abdominal ultrasonography revealed normal liver size and echotexture with no lesions, intrahepatic biliary duct dilatation, common bile duct dilatation 18 mm, contracted gallbladder with no cholelithiasis and no discrete pancreatic mass or ductal dilatation were identified. Magnetic resonance image/magnetic resonance cholangiopancreatography (MRI/MRCP) was performed and revealed moderate to severe intrahepatic and extra hepatic biliary ductal dilatation with mild enhancement of the distal common bile duct without choledocholithiasis or discrete mass, and pancreatic duct dilation with normal pancreas [].
An endoscopic retrograde cholangiopancreatography (ERCP) was performed and revealed a large ampullary mass distorting the anatomy, which precluded cannulation of the common bile duct []. During his ongoing evaluation the patient developed pruritus and his repeated liver function test revealed stable levels with numbers around his initial presentation. An advanced endoscopist repeated the ERCP, which revealed 2 cm ampullary lesion with upstream obstruction, pancreatic duct dilatation measuring 4.5 mm, intra- and extra-hepatic biliary dilatation with common bile duct measured 16 mm. A biliary sphincterotomy and balloon sweep was performed, and biopsies were taken of the ampullary tissue. Histopathology revealed congested mixed acute and chronic inflammation with reactive epithelial changes, negative for malignancy. The CA 19-9 level was elevated at 51.4 units/mL.
The patient underwent laparoscopic pancreaticoduodenectomy (Whipple's procedure) with regional lymphadenectomy. Histopathology was consistent with 1.3 cm ampullary neuroendocrine tumor infiltrating the mucosa, submucosa and muscularis propria with angiolymphatic invasion. Immunohistochemical stains were positive for somatostatin, and negative for gastrin, serotonin and pancreatic polypeptide []. There was no local invasion, the specimen margins were negative, and 1/18 lymph nodes were positive. The patient was diagnosed with stage IIb (T2 N1 M0) somatostatinoma.
The post resection surveillance at 3, 6, and 12 months revealed a normal history and physical examination, a fasting somatostatin level of 41, 28, and 19 pg/ml, respectively, and a CT scan abdomen and pelvis with no evidence of recurrence. He is still under yearly surveillance with normal history, physical examination and fasting somatostatin level for the last four years. |
pmc-6532328-1 | A 24-year-old male patient was referred for orthodontic treatment and orthognathic surgery in order to correct his anomalies of tooth position and jaw relations. According to his statements, previous surgical treatment offers to correct his appearance included tooth correction as well as chin augmentation. These treatment options had been unsuitable in the patient's assessment for adequately implementing his desire to change his profile.
The general medical history of the patient did not indicate any limitation of eventual orthodontic therapy combined with surgery. He expressly denied any serious illness. He reported that his limping walk was due to previous injuries.
Later, the patient acknowledged that he had deliberately made inaccurate statements about his health condition, because he had previously been refused treatment several times for his tooth anomalies when he had made known his underlying disease and medication.
The patient was of relatively short stature with an asymmetrically developed face and maxillary hypoplasia in the sagittal plane (Figure 1 ). His sclera had a slightly bluish tint, but this was only noticed in connection with the later diagnosis (Figure 2 ). Intraoral findings at first examination included a class III occlusion (Figure 3 ). Permanent dentition was complete, including wisdom teeth. The patient's teeth were of normal size and shape. The front teeth had increased translucency upon frontal illumination in the area of the incisal edges and in the proximal areas. There were no attritions of occlusal surfaces that exceeded age-appropriate wear and tear. In conjunction with the later diagnosed OI and respective medical reports on this subject, the dental findings did not indicate any clues for DI. |
pmc-6533102-1 | A 3-year-old boy with severe respiratory distress admitted to emergency department. He had pneumothorax at right chest and underwent tube thoracostomy (
). In his past medical history, he has free of symptoms and had no chronic disease. None of the family members had lung disease and none of them were smokers. At admission, his vital signs were within normal limits except high respiratory rate (45/minute). Total blood count, liver, and renal function tests were also unremarkable. On physical examination, a 2 × 1 cm palpable mass was noted at right side of neck. Computed tomography (CT) of chest revealed bilateral air cysts in varying sizes with ground glass appearance (
). Pneumothorax was also detected. The patient underwent biopsy form the neck mass and diagnosed as LCH confined to right salivary gland. The histopathologic features include polymorph nuclear leucocytes and atypical histocytes with oval nucleus with pale granular cytoplasm. Immunohistochemical staining with CD1a (cluster of differentiation 1 a) and S100 were positive. Chemotherapy including vinblastine and prednisone was initiated. During follow-up, pneumothorax persisted and another chest tube was inserted on the left side. Since the patient was unresponsive to medical treatment 2 weeks after admission, chemical pleurodesis with talc (2 cc) was performed. The pleurodesis was performed through tube thoracostomy. No intrapleural analgesics were used. The tube was clamped for 1 hour and unclamped at the end of procedure. During the clamped period, patients recommended to rotate on each side for 10 minutes. At the end of 10 days follow-up, patient developed bilateral pneumothorax with subcutaneous emphysema despite tube thoracostomy. Thoracoscopic bullae excision with pleural decortication was performed 10 days after talc pleurodesis (
). Histopathological evaluation of pleural biopsies obtained by thoracoscopy revealed inflammation and foreign bodies (talc). No lung biopsy was sampled during bullae excision. After surgical excision of subpleural bullae, the patient was symptom free for only 1 week and after developed respiratory insufficiency. Although total pleurectomy was considered for the patient, due to nosocomial pulmonary infection and severe respiratory distress, mechanical ventilation was initiated. Despite high-frequency ventilation, the oxygenation was inadequate and higher pressures are required. Five weeks after the bullae excision, extracorporeal membranous oxygenation (ECMO) support is initiated with vein-venous catheterization via internal jugular vein. At the 24th day of ECMO, he developed cranial bleeding and ventricular herniation and died of bleeding complications. |
pmc-6533103-1 | A 15 years old male presented with the compliant of progressive abdominal swelling since 10 years which had further progressed within the preceding 12 months.” Furthermore he experienced early satiety, unable to run, difficulty walking, shortness of breath in lying position, and significant subjective weight loss in 3 months duration. He was a grade VII student but discontinued school due to walking difficulty for the last 3 months. Two years prior to the current presentation he was admitted for with chylous ascites diagnosed by abdominal ultrasound at our hospital; however, a CT or MRI was not done at this time and a planned treatment with octreotide could not be performed as the patient left the hospital prior to that.
On physical examination he was looking chronically sick, the vital signs were within normal limits. The abdomen was significantly distended (
) with dullness on percussion over the whole abdomen with a positive fluid thrill. The right scrotum was empty. The complete blood cell count, renal and liver function tests, and serum albumin and stool examination were normal. Abdominal ultrasound and abdominal CT scan showed a 20 × 32 cm giant mesenteric cyst, absent right kidney and left moderate hydronephrosis due to the compression effect from the mass (
). On exploratory laparotomy through a midline abdominal incision a huge retroperitoneal cyst from duodenum to sacrum was found. The cyst was completely excised and right orchiectomy was done for intra-abdominal testis to prevent testicular germ cell malignancy. Gram stain, culture, and gene expert of the cystic fluid was negative with 7/mm
3
white blood cells. A postoperative abdominal ultrasound postoperative day 7 showed a mild left sided hydronephrosis. The patient recovered uneventfully and was discharged 1 day later. Histology of the resected testis showed an atrophic testis without sign of malignant transformation. Follow-up on 20th post surgery was unremarkable. |
pmc-6533329-1 | A 52-year-old woman was referred to the emergency room (ER) for a fever. She had undergone an extended hysterectomy for cervical cancer (stage IIb, pT2aN1M0) 7 years prior and developed a left uretero-vaginal fistula secondary to postoperative chemoradiotherapy managed with an indwelling ureteral stent (Fig. ). Following that, she suffered from repeated urinary tract infections occasionally treated by antibiotics and required routine ureteral stent exchanges. She also had type 2 diabetes mellitus treated with pioglitazone hydrochloride over 6 years and revealed no signs of a cancer recurrence during that period. Magnetic resonance imaging revealed a fistula formation between her sigmoid colon and left ureter (Fig. ). It was considered that a fecal leakage from the uretero-colic fistula worsened the urinary tract infection and an elective colostomy was planned.
One month after the diagnosis of the uretero-colic fistula while waiting for the colostomy, she visited the ER complaining of perineal bleeding. A physical examination revealed hypotension (systolic blood pressure 70 mmHg) and tenderness of her lower abdomen but without any bloody stool upon a rectal digital examination nor vaginal bleeding on colposcopy. The laboratory examination revealed an elevated C-reactive protein (CRP) level without anemia and microscopic hematuria and pyuria. She was diagnosed with urosepsis caused by a uretero-colic fistula and was admitted to the department of gynecology. Although she developed macroscopic hematuria after inserting a urinary catheter, the site of bleeding could not be identified by contrast-enhanced computed tomography (CT). Thirteen hours after the onset, her hypotension worsened resulting in hypovolemic shock (systolic blood pressure 40 mmHg) and massive genital bleeding became evident. She died 1 h later (14 h after the onset) despite intensive care.
The autopsy findings revealed a large amount of blood in the left renal pelvis, bladder, and sigmoid colon. In addition, the left internal iliac artery branch firmly adhered to the ureter and was found to form a UAF, which connected to the uretero-vaginal and uretero-sigmoid fistulas through the ureter (Fig. ). These findings suggest that bleeding from the newly developed UAF spreads not only into the urinary tract but also over the genital and intestinal tracts through the pre-existing fistulas resulting in sudden death. A histopathological examination of the UAF revealed extensive inflammation with necrosis and an infiltrative squamous cell carcinoma (nonkeratinizing type) in the surrounding tissue (Fig. ). Besides the previous surgery followed by chemoradiotherapy, her having suffered from repeated infections due to the indwelling ureteral catheter and the occult recurrent cancer tissue might have caused those histological changes that led to the fistula formation. |
pmc-6533445-1 | A 25-year-old female was referred to our clinic with double vision and restricted right eye abduction. In the ophthalmic examination, both pupils were round and reactive to light and accommodation. Extraocular movements were intact with the exception that the right eye was unable to abduct (Figure 1 , Figure 2 ). No nystagmus was detected and visual acuity was normal. Visual field examination revealed the enlargement of the blind spot and constriction of the peripheral fields. On fundus examination, bilateral papilledema with blurring of the disc margins was present.
Questioning her history revealed that the first symptoms were weakness and numbness in her lower limbs which had resolved spontaneously. Afterwards, she had developed an intractable headache associated with right gaze difficulty and diplopia and was referred from the emergency room to the neurology department. Magnetic resonance (MR) imaging revealed no pathology. After our finding of papilledema, despite the history of transient weakness in the lower limbs, the cause was firstly considered as idiopathic intracranial hypertension (IIH) and lumbar puncture was planned. But because she developed fever and neck rigidity at that time, she was referred to the department of infectious diseases to rule out meningitis.
The patient underwent a lumbar puncture, opening pressure was 255 mm H2O and the examination of the cerebrospinal fluid (CSF) showed 85 leukocytes/μl. Her Rose Bengal test was positive and the CSF culture yielded Brucella melitensis and diagnosis of neurobrucellosis was established. The patient was treated with a combination of doxycycline, rifampin, cotrimoxazole, and dexamethasone for 6 months. |
pmc-6533446-1 | A 31-year-old male presented with central scotoma of 9 months duration in the right eye and a similar complaint in the left eye, since a week. Best corrected visual acuity (BCVA) was 20/60 in the right eye and 20/30 in the left eye. His past history was unremarkable. Fundus examination revealed a circumscribed, flat, peripapillary, deep retinal lesion with a greyish, marginal opacification or demarcation line in both eyes; right larger than left (Figure 1 ).
Fundus autofluorescence (FAF) imaging revealed a normal autofluorescence in the area beyond the demarcating line (zone 1), a speckled hyper-autofluorescence within the AZOOR lesion (zone 2), and a speckled hypo-autofluorescence corresponding to the development of choroidal atrophy (zone 3). The delineating line was predominantly hyper-autofluorescent in the left eye and hypo-autofluorescent in the right eye corresponding to the stage of the disease; hypo-autofluorescence corresponding to atrophic stage, hyper-autofluorescent corresponding to active stage (Figure 1 ). Optical coherence tomography (OCT) was normal outside of the AZOOR line (zone 1). Inside the AZOOR line, multifocal material was present in the subretinal space resembling subretinal drusenoid deposits (zone 2). In zone 3, OCT showed a zonal loss of the outer retinal layers with a disruption of the photoreceptors, the ellipsoid line (formerly known as the inner and outer segment junction) and the interdigitation line (formerly known as cone outer segment tips, COST line) in both eyes with minimal sub-retinal fluid (SRF) in the left eye (Figure 1 ). Humphrey’s visual field (HVF; 30-2) showed enlargement of blind spot in both eyes; right larger than left (Figure 2 ). Multifocal electroretinogram (mfERG) showed a loss of foveal peak in the right eye and a reduced foveal peak in the left eye with normal parafoveal and perifoveal ring response in both eyes (Figure 1 ). Clinical features and supportive investigations were consistent with the diagnosis of acute zonal occult outer retinopathy (AZOOR). After discussing the management options, treatment was initiated with oral steroids (1mg/kg body weight), tapered over 6 weeks.
At 3-month follow-up visit, BCVA improved to 20/40 in the right eye and 20/30 in the left eye. Fundus examination revealed a circumscribed, flat, peripapillary, deep retinal lesion with a greyish, marginal opacification in both eyes; right larger than left (Figure 3 ). Autofluorescence revealed the demarcation line assuming an incomplete or interrupted pattern with disease progression (Figure 2 ). OCT showed a zonal loss of the outer retinal layers with a disruption of the photoreceptors, the ellipsoid and cone outer segment (COST) in both eyes (Figure 2 ). Humphrey’s visual field (HVF; 30-2) showed a slight reduction of scotoma size in the left eye, and stable in the right eye (Figure 3 ). |
pmc-6533447-1 | A 15-year-old boy was operated for traumatic endophthalmitis in the right eye in April 2015 with vitrectomy, lensectomy, and intravitreal antibiotics. After three months, he developed inferior retinal detachment for which a repeat vitrectomy, relaxing retinotomy, and SiO (1,000 centistoke viscosity) tamponade were performed. Both procedures were performed through 20 gauge sclerostomies which were sutured with 7-0 polyglactin sutures. The wounds were watertight at the end of the surgeries. He experienced persistent redness and foreign body sensation following the second procedure. He gradually developed outward deviation of the right eye and binocular horizontal diplopia for which he was referred to the strabismus clinic in October 2016. At this visit, his best corrected distance visual acuity in the right eye was 0.8 LogMAR (with +10.25 D contact lens) and near vision was N36 (with +3.0 D add). The conjunctiva was diffusely congested and boggy. Multiple shiny subconjunctival nodules of variable sizes were seen in all four quadrants. They were predominant in the superotemporal and inferotemporal quadrants (Figure 1a ). Other clinical findings were linear corneal scar superiorly, aphakia, attached retina at the posterior pole, and peripherally detached retina. The intraocular pressure was 10 mm Hg. Orthoptic evaluation revealed exotropia of 25 ΔD with unrestricted ocular movements. A diagnosis of sensory exotropia with sub-conjunctival cysts was made. Lateral rectus (LR) recession and medial rectus (MR) resection were planned which were delayed till May 2017 due to the patient’s unwillingness for further surgery.
During the surgery, LR muscle was approached under local anesthesia using a paralimbal conjunctival incision. The underlying sclera did not show any thinning or melt. Multiple clear thin-walled oil filled uni- and multi-loculated cysts, ranging from pin-head size to 5 mm, extending up to at least 14 mm posterior to limbus, were encountered below the conjunctiva. They were firmly adherent to the surrounding structures and ruptured on attempted dissection. Numerous cysts were found embedded within the tenon’s capsule, the muscle sheath, and under the LR muscle (Figure 2a–d ). This had rendered the tenon’s fascia thick and fibrosed (Figure 2e ). Dissection led to profuse bleeding making visualization and delineation of the muscle onerous and time consuming. The muscle sheath and portion of the tenon’s fascia surrounding the muscle had to be resected to remove the cysts and facilitate isolation of the muscle (Figure 2f ). The large cysts were removed by rupturing or resecting them. The SiO released from ruptured cysts was thoroughly washed from the ocular surface. After securing the LR with 6-0 polyglactin suture, it was recessed 6.5 mm using a sliding noose adjustable suture technique (Figure 2g ). Thereafter, medial rectus muscle resection (4.5 mm) was performed (Figure 2h ). The SiO cysts were sparse in the medial, superior and inferior quadrants.
In the immediate postoperative period, the patient was orthotropic and did not require adjustment. The conjunctival inflammation gradually reduced (Figure 1b–d ) eliminating the ocular discomfort. At the last follow-up thirteen months later, his BCVA was 0.8 LogMAR and central retina was attached under the silicone oil. He was diplopia free and was satisfied with the cosmesis. |
pmc-6533462-1 | An 81-year-old phakic female, a 69-years-old phakic male, and a 56-year-old pseudophakic female presented in our clinic with progressive deterioration of visual acuity, epiphora and photophobia of both eyes.
In all patients, preoperative slit-lamp examination revealed corneal edema with extensive cornea guttata in both eyes, caused by Fuchs endothelial dystrophy. A best-corrected visual acuity (BCVA) of 4/20, 6/20 and 2/20 was measured in the worst eye of each patient, respectively. In all patients, reliable measurements of endothelial cell density by corneal specular microscopy could not be obtained due to the advanced stage of endothelial dysfunction. Central corneal thickness (CCT) was 687 µm, 606 µm, and 832 µm, respectively, as measured by Oculus Pentacam® Scheimpflug camera.
The first two patients underwent Triple-DMEK (phacoemulsification and intraocular lens implantation combined with DMEK) while the third one underwent single DMEK as described by Melles and colleagues []. In all patients, an inferior peripheral Nd:YAG iridotomy was performed prior to surgery. Both endothelium and Descemet membrane were stripped off from the donor cornea immediately prior to the procedure. In recipient eyes, a descemetorhexis of about 9 mm in diameter was performed and the central portion of the endothelium with Descemet membrane was removed. Through a 3 mm clear corneal incision, the 8.5 mm diameter posterior lamellar corneal graft was inserted into the recipient anterior chamber through a glass injector, positioned in correct orientation onto the posterior stroma and secured by an air filling of the anterior chamber. No intraoperative complications occurred and all patients were asked to lie flat on their back postoperatively. The postoperative treatment included a combination of steroid (Dexamethasone), antibiotic (Ofloxacin), and miotic (Pilocarpine) eyedrops as well as an intravenous injection of Methylprednisolone 100 mg for the first three days after surgery.
The first and the third patient were discharged on the fifth postoperative day without presentation of any complications and with attached graft as documented by anterior segment OCT. In the second patient, re-bubbling was necessary due to incomplete attachment of the graft. During the re-bubbling procedure, the graft was accidentally partially folded. This patient was discharged after absorption of the air bubble.
Approximately one week following discharge, all three patients were referred to our clinic again with photophobia and increased blurry vision. Slit-lamp examination demonstrated diffuse corneal edema and corneal decompensation, highly likely as a result of graft detachment. Anterior segment OCT (Figure 1 ) confirmed an almost complete detachment of the donor graft from the recipient’s stroma.
We followed a “wait & watch” approach in all three patients and close follow-up visits were undertaken. Remarkably, gradual corneal clearance was observed within three months in all patients. Upon slit lamp examination three months postoperatively, the corneal stroma was more transparent in all three patients, despite the fact that it was covered neither from donor tissue nor from the patient’s Descemet’s membrane. Central corneal thickness demonstrated a significant decrease from 687 to 572 µm (17%) in the first patient, a moderate decrease from 606 to 556 µm (8%) in the second one while in the third patient a more significant decrease from 832 to 580 µm (30%) was documented (Figure 2 ).
Those changes in corneal pachymetry were accompanied by an increase of visual acuity from 4/20 to 8/20 in the first patient, from 6/20 to 12/20 in the second and from 2/20 to 4/20 in the third one within the same time period. Although a marked improvement of the CCT was seen in all three cases, BCVA has not been a reliable outcome parameter. The reduced visual acuity despite the more advanced corneal clearance in the second and third patient was attributed to a concomitant retinal disorder and central retinal vein occlusion, respectively. Furthermore, the “free-floating” Descemet graft was positioned within the visual axis, causing blurry vision.
However, despite the detached donor tissue, enlarged and irregularly shaped endothelial cells were found by noncontact corneal specular microscopy on the recipient’s posterior corneal stroma in all patients (Figure 3 ).
The above described positive findings were maintained over one-year follow-up, despite the still detached grafts. However, re-DMEK was performed after 14 months in the first patient, with successful graft attachment and a final visual acuity of 20/20. The second and third patient refused further interventions. |
pmc-6533542-1 | We present the case of a 15-year old girl with a history of B-cell acute lymphoblastic leukemia (t(1:19)) in clinical remission for two years. She was under maintenance chemotherapy with dexamethasone, mercaptopurine, and melphalan. In the past year, she was also suffering from pulmonary tuberculosis having completed 9 months of therapy. She presented in our ophthalmology emergency room with acute visual loss of the left eye (LE) and ocular redness for 5 days. Best corrected visual acuity in her RE was 20/20 and absence of light perception in her left eye (LE). Intraocular pressure upon presentation was 12 mmHg in RE and 22 mmHg in LE. In LE biomicroscopy, she presented with a very small hypopyon, small, inferior, keratic precipitates, anterior chamber cells 4+, vitreous cells 3+, and a dense white mass was visible in the vitreous cavity along with organized vitreous hemorrhage (Figure 1 ). The fundoscopy examination was made difficult by the dense vitritis. It was possible to perceive the presence of a white vitreous mass occupying most of the vitreous cavity and vitreous hemorrhage. Biomicroscopy and fundoscopy were normal in LE. B-scan ultrasound showed a multilobulated vitreous mass occupying practically the entire ocular globe with associated superior choroidal detachment (Figure 2 ). An orbital and cranial MRI revealed an intraocular mass with gadolinium enhancement and lacrimal gland enlargement (Figure 3 ).
The patient was medicated with an association of Timolol 1% and Dorzolomide 1% bid as well as topical Dexamethasone 1% qid. Two days later, she suffered clinical worsening with severe ocular pain, headache, and nausea. IOP was now 55 mmHg in LE and the biomicroscopy of LE showed a shallow anterior chamber. Acute angle closure was diagnosed, and we added topical Brimonidine 0.1% bid and Acetazolamide 500 mg qid to her therapeutic scheme. A flow cytometry analysis of the aqueous humor and vitreous revealed ALL-B cells sharing the immunophenotype with previously diagnosed leukemic cells (CD34–/CD10+/CD19+/CD20+ weak/CD38+). Microbiologic analysis was negative for bacteria and fungus as well as the PCR study for Mycobacterium tuberculosis. Ocular relapse of B-cell ALL was diagnosed. The patient was re-staged but there were no signs of relapse in the bone marrow, the central nervous system or any other systemic location. Radiotherapy (30Gy) and rescue chemotherapy (INTREALL HR) were started with symptomatic improvement and better IOP control even though the patient was still under topical hypotensive drugs. Final MAVC was absence of light perception and 8 months after presentation, the patient remained without any other signs of systemic relapse. |
pmc-6533544-1 | A 52-year-old woman came in our emergency room for consult due to sudden painful loss of vision of the right eye. The History of the present illness started 1 week prior to consult, the patient noted a sudden blurring of vision of the right eye described as curtain loss of vision. No history of trauma, pain, flashes, and floaters was noted, and neither consult done nor medication taken. Three days prior to consult, the patient noted a total loss of vision of the right eye associated with intractable pain, eye redness, headache, nausea, and vomiting, which prompted consult.
The patient had no known systemic diseases except for well-controlled bronchial asthma and hypertension. The patient was maintained on losartan 50 mg twice a day with poor compliance and control. The patient came in with no light perception for the right eye and 20/20 for the left eye. The blood pressure at the time of consult was at 240/100. Gross examination and extraocular muscles were normal. The right pupil was 6 mm non-reactive to light, and the left pupil was 3 mm brisk reactive to light with positive reverse relative afferent pupillary defect. Slit lamp examination of the right eye revealed shallow chambers with moderate corneal edema with microcyst and an intraocular pressure (IOP) of 60. On gonioscopy of the right eye, the angles on all quadrants were closed. Indirect ophthalmoscopy of the right eye revealed total retinal detachment with large subretinal hemorrhage. The examination of the left eye showed a formed chamber with an IOP of 12 and a cup-to-disc ratio (CDR) of 0.4 with arteriovenous crossing changes. On gonioscopy of the left eye, the angles on all quadrants were open to ciliary body band. The right eye had poor streak on refraction, while the left eye’s refraction was –0.50 D sphere. Ocular ultrasound of the right eye (Figure 1 ) revealed massive hemorrhagic retinal detachment and could not totally rule out the presence of an intraocular mass. The patient was assessed to have secondary angle closure from massive subretinal hemorrhage probably from intraocular mass metastasis of the right eye. Acetazolamide 250 mg four times a day and topical brimonidine one drop three times a day were started; however, the IOP was decreased only to 42. The blood pressure was controlled acutely with intravenous nicardipine to 170/90, then was shifted to amlodipine 100 mg once a day and losartan 100 mg twice a day. The plan was for enucleation of the painful blind eye for histologic diagnosis and for systemic work-up of the primary origin of malignancy. Since there was the clinical suspicion of a metastasis but no primary site of origin could be found, the histologic diagnosis would point to the right direction in finding a primary tumor and treating the patient for the presumed malignancy.
Systemic and metabolic laboratory work-up revealed normal results except for elevated glycosylated hemoglobin 7.6 (3.5–6.0%) and fasting blood sugar 8.0 (4.1–5.9 mmol/L), assessing the patient with newly diagnosed type 2 diabetes mellitus. The patient was started on metformin 500 mg once a day. Mammography and transvaginal ultrasound revealed normal results. Chest radiography also showed normal results.
Enucleation was done, and histopathology revealed subretinal hemorrhage and secondary angle closure glaucoma with no intraocular mass (Figure 2 and Figure 3 ).
However, cranial and abdominal contrast enhanced computed tomography scan was done post-enucleation revealing no intracranial and abdominal mass. Hence, we were arriving at a final diagnosis of secondary acute angle closure glaucoma from spontaneous massive hemorrhagic retinal detachment secondary to hypertension and diabetes mellitus. |
pmc-6533672-1 | A 49-year-old male patient sought treatment due to “repeated cough and sputum for one year and aggravation with chest tightness for one week.” Chest computed tomography (CT) in a local hospital revealed encapsulated effusion in the right thoracic cavity. Chest-enhanced CT after admission revealed a space-occupying lesion in the right chest. The pathological diagnosis according to percutaneous lung biopsy was a solitary fibrous tumor. Preoperative three-dimensional reconstruction showed that the blood supply to the tumor originated from the arteries and veins of the right lower lobe (Fig. ). After complete preoperative preparation, the patient underwent resection of the tumor with single-operation-incision thoracoscopy. Incision selection is one cm for the endoscope port (at the midaxillary line of the seventh intercostal space) and two cm for the operation port (at the preaxillary line of the fifth intercostal space). Intraoperative exploration revealed that the tumor had two tumor pedicles (Fig. ), and both were located in the right lower lobe. The tumor pedicles were intraoperatively separated using a linear stapling device. Because the specimen was large and the surface was smooth, bagging the specimen by the traditional method was difficult; therefore, the specimen was bagged by adjusting the operating table. The detailed procedure was as follows: First, the operating table was arranged with the head at a lower position, and the specimen bag was inserted into the thoracic cavity. Then, the operating table was adjusted such that the head was at a higher position to enable loading of the specimen into the specimen bag by gravity. Due to the large size of the specimen, extracting the specimen by the traditional method was difficult. Therefore, a special method named “pulling carrot” was applied to remove the specimen (Fig. ). The specific procedure was as follows. (1) The operation port was extended to five cm. (2) Several drawstrings were intermittently sewn at the smaller end of the longitudinal axis of the specimen. The area of suturing should be as large as possible, and if necessary, additional sewing should be carried out during the process of specimen extraction. (3) Two people synchronously pulled the drawstrings and the specimen bag, with up and down, left and right shaking. The size of the specimen was 18 × 12 × 6 cm (Fig. ). The surface of the specimen was smooth and the texture was soft and tough. Postoperative pathological examination revealed a solitary fibrous tumor immunohistochemistry: Vimentin (+), CD34 (+), bcl-2 (+), CD99 (+), SMA (−), S-100 (−), Desmin (−), P53 (−), CK (−), EMA (−), and Ki-67 positive rate of about 10%. |
pmc-6533677-1 | A 4-year-old Black African boy (from a rural locality in the Menchum Division of the Northwest Region of Cameroon), with no remarkable past medical and family history, consulted our emergency unit for a 3-day history of generalized abdominal pains, vomiting, and obstipation. We also noted an abdominal distention which his mother ascertained to have been evolving for 6 months prior to consultation at our health facility. In addition, the child had never been dewormed since birth according to the mother.
A physical examination revealed a conscious but asthenic patient with signs of malnutrition and some dehydration. His conjunctivae were pinkish and sclerae were anicteric. His abdomen was distended, soft but mildly tender, mobile with respiration, and dull on percussion. There was no palpable abdominal mass or shifting dullness. Bowel sounds were hyperactive, and the rectum was void of fecal material on digital rectal examination. Initial laboratory investigations revealed hypokalemia and hyponatremia. A full blood count was normal. A plain abdominal X-ray revealed discrete air-fluid levels. Based on the aforementioned clinical and paraclinical findings, a diagnosis of IO was arrived at. Further exploration of the cause of the obstruction was inaccessible mostly because our patient’s family could not afford the cost, and the nearest referral facility capable of performing these tests was approximately 76 km away, on poorly motorable and hilly roads. Taking these circumstances and the deteriorating clinical picture of our patient into account, we decided to do an exploratory laparotomy after receiving a verbal and signed consent from our patient’s carer.
He was admitted, rehydrated with 2 L of Ringer’s lactate and 1 L of glucose 5% per m2/day for 3 days, and intravenously administered paracetamol 15 mg/kg per 6 hours, ceftriaxone 50 mg/kg per day, metronidazole 15 mg/kg per 8 hours, and gentamycin 5 mg/kg per day. His legal guardian was immediately counselled on the need for a laparotomy and a signed informed consent was obtained after which an anesthetic consult was sought. He was operated on the third day of hospitalization after correction of the associated electrolyte imbalance.
The surgical approach consisted of the traditional midline incision. Perioperative findings revealed a dilated small bowel obstructed by bundles of live worms (Fig. ). An enterotomy of 2 cm in length which exposed the bundles of A. lumbricoides was done, followed by manual extraction and milking of the worms through the stoma (Fig. a, b). Postoperative management involved intravenously administered fluids with Ringer’s lactate, and intravenously administered paracetamol 15 mg/kg per 6 hours, ceftriaxone 50 mg/kg per day, metronidazole 15 mg/kg per 8 hours, and gentamycin 5 mg/kg per day. Progressive oral sips were started 8 hours after surgery and semi-solid food was introduced from postoperative day 3. Evolution was favorable with full restoration of bowel function on postoperative day 3. Our patient was discharged on postoperative day 7 with no fresh complaints. He and his entire household were dewormed with a single dose of mebendazole 500 mg administered orally. A follow-up visit 1 week after discharge revealed a healed abdominal wound and normal bowel functions. |
pmc-6533701-1 | A 78-year-old Japanese woman (gravida 10, para 2, abortion 8) had a 10-year history of uterine prolapse but she had no gynecological examination. She had no smoking history and her body mass index was 19.7. She visited our hospital because of a 1-month history of bleeding from an ulcerated lesion on the surface of the prolapsed uterus. Upon examination, the uterine prolapse appeared as complete eversion of the posterior vaginal fornix and was manually irreducible and classified as stage IV according to the Pelvic Organ Prolapse Quantification System []. On the surface of the completely prolapsed uterus, an ulcerated lesion of 3 cm in diameter was observed and a deep-seated induration was felt. A histological biopsy of a mass in the vaginal wall led to a diagnosis of keratinizing squamous cell carcinoma. Magnetic resonance imaging revealed the mass with a depth of 1.6 cm, but the endometrial cavity was not depicted (Fig. ). The mass was located approximately 3 cm from the cervical os with no sign of invasion to adjacent tissue. According to the International Federation of Gynecology and Obstetrics (FIGO) staging system, the tumor was vaginal cancer staged as I, and a fluorodeoxyglucose positron emission tomography/CT revealed no metastases to the lymph nodes or other organs. Although the diagnosis of this case was vaginal cancer, the main tumor was located in the uterine cervix because of the completely prolapsed uterus. Therefore, we considered that it is practical to develop the treatment plan according to the uterine cervical cancer. Because of the patient’s advanced age and the presence of arrhythmia (paroxysmal supraventricular tachycardia and paroxysmal atrial fibrillation), she was ineligible for a combination of surgery under general anesthesia and chemotherapy; therefore, RT alone was planned. If EBRT had been administered, radiation dermatitis of the genitalia would have been a concern because the prolapsed uterus would be irradiated. Neither a probe nor a tandem could be inserted because of severe intrauterine adhesions due to her history of multiple abortion procedures, also known as Asherman syndrome [, ]. The cervical canal was extremely narrowing due to advanced age. Thus, intracavitary BT (ICBT) could not be administered. In addition, the complete uterine prolapse inhibited fixation of an ovoid tandem to the vaginal fornices. Thus, ISBT was planned, in which plastic needles would be directly inserted into the margin of the ulcerated lesion on the surface of the completely prolapsed uterus under direct visual observation. In the BT treatment room, four plastic BT needles (LLA150-K; Eckert & Ziegler BEBIG, Berlin, Germany) were inserted into the margin of the ulcerated lesion to a depth of up to 3 cm under local anesthesia to sufficiently cover the lesion, the depth of which was 1.6 cm as previously measured by magnetic resonance imaging (Fig. ). Under only local anesthesia, direct insertion of the plastic BT needles caused very little pain. Next, a dummy source for X-ray imaging (LLH02–21 to 24) was inserted into the lumen of each of the four plastic BT needles, and X-ray imaging was performed. The patient was then transferred to the CT room. CT was performed with 2-mm slices, confirming that the BT sources had been inserted around the lesion (Fig. ). At our facility, HDR ISBT is administered using a 60Co remote afterloading system (RALS) (MultiSource; Eckert & Ziegler BEBIG). Thus, these CT scans were uploaded to the RALS treatment planning system (HDR plus; Eckert & Ziegler BEBIG). According to the guidelines established by the Groupe Europeen de Curietherapie and European Society for Radiotherapy and Oncology [, ], the gross tumor volume (GTV) and organs at risk (OARs) (i.e., the rectum and bladder) were contoured. The high-risk clinical target volume (HR-CTV) including the whole cervix could not be contoured because the endometrial cavity was not depicted and the whole extent of the cervix was not defined. The GTV was 19.4 cc, and treatment was planned with a dose of ≥6 Gy prescribed as D90 of the GTV (minimum dose delivered to 90% of the GTV) (Fig. ). On another day, the plastic BT needles and dummy sources were reinserted, and X-ray imaging was performed, followed by CT. A similar treatment plan was developed. At our facility, X-ray films alone are used to check the location of radiation sources for BT and develop treatment plans. In this case, X-ray films were obtained after insertion of each plastic BT needle and the dummy sources, and two treatment plans were developed (Table ). We selected the plan with the dummy sources placed closer to the lesion, according to which HDR ISBT was administered at 6 Gy per fraction twice weekly for a total of eight times. When the location of the dummy sources deviated from the planned sites, the plastic BT needles were reinserted to ensure administration based on one of the treatment plans. The total accumulation dose of HDR ISBT was estimated to be 65.0 Gy as D90 of the GTV (equivalent dose in 2-Gy fractions [EQD2]). One month after the completion of RT, the histological biopsy revealed remaining squamous cell carcinoma. Therefore, we performed additional HDR ISBT at 6 Gy per fraction twice weekly for a total of six times until the ulcerated lesion became soft and flat. The final total accumulation dose of HDR ISBT was estimated to be 113.8 Gy as D90 of the GTV (EQD2), 20.7 Gy as D2cm3 of the rectum (EQD2), 36.8 Gy as D2cm3 of the bladder (EQD2) with α/β = 10 for the GTV and α/β = 3 for the OARs (Table ). Because of the complete uterine prolapse, the GTV was remote from the rectum and bladder; therefore, the exposure doses to the OARs were extremely low. The only observed acute complication was grade 2 dermatitis in the genitalia (National Cancer Institute Common Terminology Criteria for Adverse Events of 4.03 []). One month after the completion of additional HDR ISBT, no residual squamous cell carcinoma was detected in the tissue biopsy. Two months after additional HDR ISBT, as late toxicities, local ulcer lesion with fibrositis was seen in the labium majus and tumor bed in the vaginal wall, and debridement was performed. Three months after additional HDR ISBT, no recurrence or rectal and urinary toxicity had occurred. |
pmc-6533729-1 | An 83-year-old Japanese man was diagnosed as having laryngeal squamous cell carcinoma (T2N0M0) at the Department of Head and Neck Surgery, Kyushu Hospital, in November 2016. He had been treated for pulmonary TB at the age of 18, but on examination, thoracic computed tomography (CT) and positron emission tomography (PET) showed no thoracic abnormalities. Radiotherapy for laryngeal cancer at 70 Gy (35 fr) was performed and the tumor disappeared. However, in April 2017, primary lesion recurrence with laryngeal edema and cervical lymph node metastasis were observed. While waiting for surgery, steroid (prednisolone) was administered for 1 month, with the dose starting at 60 mg and being reduced gradually, with the aim of alleviating the edema. In May 2017, a total laryngectomy and bilateral cervical lymph node dissection were conducted.
No thoracic abnormalities were observed on initial examination in November 2016, but a thoracic X-ray 2 weeks before surgery in May 2017 revealed a small amount of pleural effusion. The amount of pleural effusion increased immediately after surgery, and by the following day he had developed fever (39 °C). At first, the condition was considered to be pleural effusion associated with pneumonia due to general bacteria, and sulbactam/ampicillin and meropenem were administered, but alleviation of symptoms was not achieved. Pleural fluid analysis showed that lymphocytes were present, abating the concern for bacterial infection. The possibility of TB was considered; therefore, sputum smear tests, including rapid molecular diagnostic testing for TB using real-time polymerase chain reaction (PCR), were conducted five times, but the results were negative. Mycobacterium tuberculosis was detected in a solid medium (“Ogawa” medium) culture test after 3 weeks; our patient was considered to have TB pleural effusion, and TB treatment was initiated. He continued to receive anti-TB drugs, which were rifampicin (RFP), isoniazid (INH), and ethambutol (EB), but died 2 months later because his general condition subsequently deteriorated. Because he had a history of TB infection, the possibility of TB was considered unlikely; therefore, an interferon-γ release assay (IGRA) test was not performed. |
pmc-6533729-2 | A 60-year-old Japanese man was diagnosed as having mesopharyngeal squamous cell carcinoma (T1N2bM1 – lung) at the Department of Head and Neck Surgery, Kyushu Hospital, in April 2017. He had been treated for pulmonary TB as an elementary school student, but on examination, thoracic CT and PET revealed no signs suggestive of inflammation, despite multiple pulmonary metastases (Fig. a). The first treatment attempted was a combination of cisplatin chemotherapy and local radiotherapy at 60 Gy (30 fr). The pulmonary metastases increased in size; therefore, weekly administration of paclitaxel + cetuximab combination therapy was initiated in August 2017 and a steroid (dexamethasone 10 mg) was simultaneously administered weekly. In October 2017, CT revealed consolidation, suggesting inflammation at loci other than the pulmonary metastases (Fig. b). Although our patient reported no subjective symptoms such as cough or fever, sputum was collected. A sputum smear test and real-time PCR yielded negative results, but M. tuberculosis was detected in a solid medium (“Ogawa” medium) culture test after 7 weeks. He was admitted to our hospital for TB treatment, and treatment of head and neck carcinoma was discontinued. He underwent treatment with anti-TB drugs, which were RFP, INH, EB, and pyrazinamide (PZA), but the cancer progressed and he died 2 months later. Because he had a history of TB infection, an IGRA test was not performed. |
pmc-6533729-3 | A 69-year-old Japanese man was diagnosed as having maxillary squamous cell carcinoma (T4aN0M1 – lung) at the Department of Head and Neck Surgery, Kyushu Hospital, in August 2016. He had no history of treatment for TB, and on examination, CT and PET revealed nodules, suspected to be pulmonary metastases, with mixed infiltrative opacity in the surrounding areas (Fig. a). He was treated with a combination of cisplatin chemotherapy and radiotherapy at 60 Gy (30 fr). However, locoregional control was not possible, and the pulmonary metastases increased in size; therefore, weekly paclitaxel + cetuximab combination therapy was initiated in May 2017 and a steroid (dexamethasone 10 mg) was simultaneously administered weekly. In November 2017, CT revealed partial expansion of the original consolidation (Fig. b). Although he reported no subjective symptoms such as cough or fever, sputum was collected. A sputum smear test and real-time PCR yielded negative results, but M. tuberculosis was detected in a solid medium (“Ogawa” medium) culture test after 4 weeks. Because TB was considered unlikely, an IGRA test was not performed. Treatment of head and neck carcinoma and treatment of TB was continued. He is currently alive 2 months after starting anti-TB drugs (RFP, INH, EB, and PZA). |
pmc-6533763-1 | A 22-year-old woman was admitted to our hospital with a history of intermittent colicky right hypochondrial pain not relieved by any medications for the past 3 months. Initially, she was given proton pump inhibitors, but her pain was not relieved. She had no other symptoms. Her past medical and family histories were not significant. The result of her clinical examination was normal. Upper gastrointestinal (GI) endoscopy showed globular swelling at the medial wall of D2 (Fig. ). The ampulla was situated at the summit of swelling.
Computed tomography (CT) with oral contrast agent showed dilation of the intramural part of the common bile duct (CBD). A cyst of size 2.4 × 2.3 cm was noted in the second part of the duodenum (Fig. ). On the basis of the above findings, it was reported as type 3 choledochal cyst. The patient was further investigated with magnetic resonance imaging, which showed dilation of the intramural part of the distal CBD. A 2.4 × 2.3 cm cyst was noted in the ampullary region, again consistent with a type 3 choledochal cyst (Fig. ). Then the patient was planned for endotherapy, but owing to difficulty, it could not be negotiated into the ampulla. Finally, the patient was referred to the surgical gastroenterology department for operative management. After multidisciplinary team discussion, an abdominal ultrasonogram (USG) was done, which showed a clear thick wall cyst measuring 4.6 × 2.6 cm between the second part of the duodenum and the head of the pancreas with gut signature (Fig. ). Opening of the CBD into the duodenum was seen just distal to the cystic lesion. There was active peristalsis noted all around the cyst, suggestive of duodenal duplication cyst.
After the diagnosis was confirmed, the patient was taken for elective laparotomy. Intraoperative findings were a 5 × 3-cm cyst over the medial wall of the duodenum extending proximal and distal to the ampulla and displacing it posteriorly (Fig. ). The cyst was communicating with the ampulla by a small opening in its medial wall (Fig. ). So, cyst secretions were drained via the ampulla, which avoided retention of cystic fluid. Part of the cyst wall was shared with duodenal musculature. Upon needle aspiration, the cyst wall contained bile due to communication with the ampulla. The duodenum was Kocherized, and a longitudinal duodenotomy was made for about 5 cm. Partial excision of the cyst was done (Fig. ) because it was closely associated with the ampulla. Marsupialization of the remaining cyst wall was done. The duodenotomy was closed horizontally. Feeding jejunostomy (FJ) was done using a modified Witzel method. A flank drain was kept in place. The postoperative period was uneventful. The patient was started on oral medications on the third day after surgery. The FJ tube was removed after 6 weeks. Histopathology showed the cyst wall was lined by duodenal mucosal epithelium with focal areas of ulceration and composed of tall columnar cells with goblet cells on either side of a common (shared) muscular layer. The submucosa showed lymphoid aggregates with Brunner glands. The common muscular layer showed congested vessels. Histopathological features were suggestive of duodenal duplication cyst (Fig. ). At her 9-month follow-up visit, the patient had no complaints. |
pmc-6533767-1 | A 51-year-old unemployed Asian man sustained a thermal burn affecting 60% of his total body surface area (TBSA) in a gasoline fire. He arrived at our hospital 4 h after injury (day 0). Upon initial examination, he was in shock and found to have inhalation injury. He had third degree burns on his face, head, neck, chest, back, bilateral upper extremities, abdomen, and bilateral thighs. Fluid resuscitation and artificial ventilation with tracheal intubation were started. According to his family, he did not have any obvious comorbidities. The patient’s history of VZV vaccination and VZV infection were unknown. Despite fluid resuscitation and catecholamine support, he had persistent oliguria and hypotension. Continuous hemodialysis and filtration (CHDF) combined with polymyxin B-immobilized fiber column direct hemoperfusion (PMX-DHP) was started on day 1 to treat his renal failure, reduce cytokine storm, and remove endotoxins in an attempt to prevent development of multiple organ failure (MOF). Multiple eschar debridements and autologous and allogeneic skin grafting were performed. Almost complete escharectomy was achieved by day 13. However, throughout the treatment period, the patient’s general condition remained critical. He could not be weaned from artificial ventilation and CHDF, and he continuously needed catecholamine circulatory support. On day 10, he developed cardiac arrest that was treated by cardiac massage and intravenous adrenaline, with return of spontaneous circulation (ROSC).
Engraftment of skin grafts and epithelialization of the skin donor sites were poor, and on day 15, multiple-drug-resistant Pseudomonas aeruginosa (MDRP) was detected in specimens from his burn wound and blood. His burn sepsis was refractory to treatment, and he developed multiple organ failure (MOF). On day 31, compact aggregations of small vesicles appeared on the intact skin of his left knee and left buttock. The vesicles were located within the 4th lumbar (L4) spinal dermatome (Fig. ). From day 32 to day 34, similar new vesicles arose on his intact skin and epithelializing split-thickness skin graft harvested sites on both legs, both knees, entire abdomen, both buttocks, and entire face and neck. The vesicles ruptured sequentially and became multiple skin ulcers. On day 34, the patient was evaluated by dermatologists, and was diagnosed with disseminated zoster caused by reactivation of latent varicella zoster virus (VZV), based on the patient’s age, characteristic pattern of aggregations of vesicles initially limited to the left L4 dermatome, and positive Tzank smear (Giemsa-stained vesicular content positive for multinucleated giant cells). Because disseminated zoster can be transmitted via an airborne route, the patient was isolated in a negative-pressure room to avoid airborne spread of VZV. On day 36, the patient’s serum levels of anti-VZV immunoglobulin (Ig) G, anti-VZV IgM, anti-HSV IgG, and anti-HSV IgM antibodies were measured by enzyme-linked immunosorbent assay (ELISA) kits (Denka Seiken, Tokyo, Japan). The antigen in the measuring kits of anti-VZV IgG and anti-VZV IgM is isolated from Vero cells infected with VZV HS strain. The antigen in the measuring kits of anti-HSV IgG and anti-HSV IgM is isolated from RD cells infected with HSV H-S1 strain. The antibody titers calculated from a standard curve using ELISA OD values. The results showed a high anti-VZV IgG antibody titer of 128 (> 4.0 is defined as positive according to the manufacturer’s insert) and low anti-VZV IgM antibody titer of 0.46 (< 0.8 defined as negative by the manufacturer’s insert). The anti-HSV IgG antibody titer was indeterminant at 2.5 (> 4, positive; < 2.0, negative; 2.0–3.9, indeterminant by the manufacturer’s insert), and the anti-HSV IgM antibody titer was negative at 0.47 (< 0.8 defined as negative by the manufacturer’s insert). Intravenous acyclovir, 500 mg daily, was started. Despite intensive care and acyclovir for 14 days, the patient’s general condition worsened. On day 48, most vesicles had not healed and had become skin ulcers (Fig. ), and not much skin was available skin for skin grafts. Therefore, treating the burn was impossible. On day 52, the patient died of multiple organ failure. No cases of VZV infection occurred in other ICU patients or ICU personnel during the treatment period. |
pmc-6533936-1 | A 57-year-old man who had alcoholic liver disease and chronic hepatitis C-related Child-Pugh class C liver cirrhosis was brought to our emergency department by ambulance, exhibiting fever, short of breath and a localized wound pain over left ankle. The patient presented with dyspnea, lower extremity edema and some wounds over bilateral ankles area for 1 day before admission, but had no other symptoms, including a headache, sore throat, cough, and chest, abdominal, and back pain. He had alcohol and chronic hepatitis C- related liver cirrhosis, and took diuretics in the past 5 years. He drank approximately 60 g of alcohol per day for more than 20 years, but recently had been taking a bit more than usual.
His laboratory data 2 weeks before admission were 6.5 mg/ dl of total bilirubin, 2.1 g/dl of albumin, and 1.73 of international normalized ratio. In addition, he had moderate ascites, which was medically controlled. These findings were indicative of Child- Pugh class C liver cirrhosis. On arrival, he appeared to be in disturbance and distress that was classified as 13 (E3V4M6) on the Glasgow Coma Scale. His vital signs were 132/106 mmHg of blood pressure, 125 beats/minute of pulse rate, 24 breaths/minute of respiratory rate, and 38.4 °C of body temperature. Auscultation of the lung and heart revealed coarse breath sounds and rapid irregular heartbeats. His abdomen was soft and swollen. Two wounds with redness and swelling (measures 0.5 × 0.5 cm, left ankle; measures 1 × 1 cm, right ankle) were found on his lower extremities ( and ). Chest X-ray indicated a lower lobe infiltration and a mild blunting of C-P angle in the right side (). ECG showed atrial fibrillation.
Laboratory findings also included 8,990/ml of white blood cell count with 94% neutrophils and 3% lymphocytes, 11.1 g/dl of hemoglobin level, 104,000/ml of platelet count, 23 mg/dl of blood urea nitrogen, 1.42 mg/dl of creatinine, 121 mmol/L of sodium, and 4.6 mmol/L of potassium. Ascitic fluid analysis demonstrated 152 cells/mm3 of ascitic white blood cell count and 30 cells/mm3 of polymorphonuclear cell count 10 hours later after his arrival of emergency department. Arterial blood gas analysis demonstrated a severe metabolic acidosis (pH: 7.38, pCO2: 20.4 mmHg, pO2: 91.8 mmHg; base deficit: 11.2 mmol/L), which was indicative of septic shock.
Oxacillin treatment under the impression of cellulitis was administered 2 hours after his arrival; two sets of blood cultures were obtained. Rapid fluid resuscitation was performed and immediately developed by tracheal intubation and mechanical ventilation in the intensive care unit. Although there was no significant change of ankle wounds, antibiotic regimen with a combination of ceftriaxone and clindamycin was adjusted due to persistent hypotension unresponsive to fluid resuscitation and high dose vasopressor use and clinical suspicion of Vibrio vulnificus related necrotizing fasciitis.
One episode of upper gastrointestinal bleeding with manifestations of fresh blood in the nasogastric tube occurred on Day 2. Blood transfusion with 2 units of packed red blood cells was given. Despite intensive support efforts, the patient died on Day 3 after the sudden change. And after that, his blood and ascites cultures were positive for A. hydrophila. |
pmc-6533938-1 | A 30-year old male engineer of African descent, presented with progressive left upper limb and bilateral lower limb weakness over a period of 3 months, associated with an unsteady gait. He complained of altered sensation of his feet, which he described as ‘walking on cotton wool’. He denied any preceding trauma and there was no neck or back pain.
Clinical examination revealed that he had normal power in both upper and lower limbs. However, there was generalized hyper-reflexia in all muscle groups of both upper and lower limbs, bilateral inverted supinator jerk, positive Hoffman’s sign and clonus. Sensation was decreased in the left sole of the foot sparing the dorsum. At the time of presentation, patient had a Japanese Orthopaedic Association (JOA) score of 7 and significant gait disability (Grade 3 as per Nurick’s Grading) [].
Magnetic Resonance Imaging (MRI) of the whole spine showed stenosis with cord signal changes from C4 to C6 levels, secondary to impingement by a prominent bony growth arising antero-superiorly from the C6 spinous process and indenting the spinal canal (). Computed Tomography of the cervical spine further confirmed the bony mass in relation to the C6 spinous (, ).
In view of the above findings, cervical myelopathy resulting from spinal canal stenosis due to OYL was suspected. A posterior decompression laminectomy with excision of the ossified yellow ligament along with posterior instrumentation and fusion from C4-C6 was planned. Lateral mass screws were inserted into C4, C5 and C6. A wide Laminectomy was performed and the OYL was identified. With help of a burr, OYL was freed and removed in a piecemeal manner. OYL was found to be adherent to the dura and care was taken to ensure there was no dural tear and CSF leak (, ).
Histological examination of the surgical specimen showed endochondral ossification, lamellar bone and marrow formation within the bony tissue. This was consistent with our diagnosis of OYL. Postoperative period was uneventful, and rehabilitation was initiated as tolerated. Subjective weakness and unsteadiness resolved eventually, and patient recovered to his full functional status by 6 months. |
pmc-6533947-1 | A 76-year-old male patient previously diagnosed with systemic arterial hypertension
and stage-4 chronic kidney disease with indefinite etiology, only reporting a
previous history of kidney stones. He began follow-up at the oncology department for
marginal zone splenic lymphoma. Initially, no specific treatment for lymphoma was
instituted, and clinical follow-up was chosen.
Two months after diagnosis, two ulcerated lesions of approximately 10 centimeters in
diameter appeared in the posterior region of the legs, with peripheral erythema and
central hematic crust (). The patient
reported local pain and heat, worsening at the end of the day and in orthostatic
position. There were no lesions in other regions of the body, hyperpigmentation or
hypertrichosis. He had been under furosemide for 3 months, potassium citrate and
fluoxetine.
Due to the suspicion of skin infection, treatment with oral quinolone and topical
neomycin sulfate was started, without improvements, and biopsy of the lesion was
indicated. Histopathological examination revealed mild perivascular lymphocytic
infiltrate and moderate solar elastosis, with hyalinization of vascular walls of
capillaries on the superficial dermis with the periodic Schiff acid staining (), findings it compatible with porphyria.
Laboratory tests showed creatinine of 3.0 mg/dL, urea 144 mg/dL, calcium 11.5 mg/dL,
Hb 9.0 g/dL, ferritin 1117 ng/mL and normal liver biochemistry. Urinary levels of
porphyrin were normal - negative uroporphyrin at the 24-hour urine test - ruling out
the diagnosis of porphyria.
Based on the histopathological findings associated with normal urinary porphyrin
levels, the diagnosis of pseudoporphyria was established. The ailment was attributed
to furosemide and the medication was discontinued. Five months after its suspension,
there was a significant improvement in the lesions (). |
pmc-6533950-1 | D.M.S., female patient, 50 years old, Caucasian, single, pharmacist, initially went
to a hospital emergency room complaining of red eyes and body aches in the last
fifteen days as the first manifestations of the disease, which was treated as
conjunctivitis but without resolution. She had had a history of renal microcapsules
for two years, and bilateral renal parenchymal nephropathy, due to increased
medullary echogenicity found on kidney ultrasonography. Therefore, serial renal
function and metabolic tests were ordered; which reported anemia, hematocrit drop
from 25.5% to 24.9% (RV: 36-45), and progressive urea elevation of 75 mg/dL to 132
mg/dL (RV: 16-40); and creatinine from 1.2 mg/dL to 2.5 mg/dL (RV: 0.6-1.2) upon
seven days of follow-up, confirming an Acute Kidney Injury (AKI).
We started her on prednisone at 2 mg/kg daily for 3 months, with reduction of 5 mg
per week after that period, until complete suspension. In the meantime, serology for
hepatitis B and C, anti-DNA, C3 and C4, anti-streptolysin O antibody (ASLO) and
gamma globulin, all with negative results, besides normal albumin of 4.4 g/dL (RV:
3,5-4,8); ruling out several possible infectious and autoimmune etiologies. However,
there was a persistent increase in C-Reactive Protein (CRP) of 11 mg/L (RV: < 8),
which is an inflammatory marker; increasing proteinuria reaching 856.3 mg/24h in 60
days of follow-up (RV: up to 150), hypercalcemia of 13.1 mg/dL (RV: 8.5-10.2) and
hypercalciuria of 504.7 mg/24h (VR: 100-300) ().
Therefore, due to the acute renal damage associated with hypercalcemia and
hypercalciuria, the patient had an indication of renal biopsy. Histopathological
examination revealed inflammatory granulomatous reactions in the renal interstitium,
a finding that is not uncommon in cases of ruptured tubular lesion and exposure of
the content into the interstitium, suggesting a diagnosis of Renal Sarcoidosis.
However, adding such a finding to the clinical and laboratory data of the patient,
by ruling out other causes, and after evaluation by ophthalmologist and pneumologist
- reporting absence of other manifestations, a diagnosis of Isolated Renal
Sarcoidosis was confirmed.
High-dose corticosteroid therapy was completed and successful, and the patient
reported improved clinical status and laboratory stability. |
pmc-6533956-1 | A 13-year-old female arrived at the Emergency Unit complaining of blurred vision
and hyperemia, and pain in her right eye. She denied having fever, asthenia,
anorexia, arthralgia, myalgia, ulcers, abdominal pain, lower back pain or
urinary symptoms. The patient was diagnosed with bilateral nongranulomatous
anterior uveitis without additional complications (). She had hypertension, raised levels of
inflammatory markers (C-reactive protein: 2.6 mg/dL; erythrocyte sedimentation
rate: 96 mm/first hour), a glomerular filtration rate (GFR) of 48
ml/min/1,73m2, hypokalemia, metabolic acidosis, leukocyturia,
glucosuria, hematuria, non-nephrotic proteinuria, and raised urine
β2-microglobulin levels. Hepatitis B and C, toxoplasmosis, brucellosis,
Epstein-Barr virus (EBV) and cytomegalovirus (CMV) infection were ruled out. Her
chest X-ray images were normal. Her angiotensin-converting-enzyme (ACE) levels
were normal, and she was negative for antinuclear antibodies (ANA) and
antineutrophil cytoplasmic antibodies (ANCA). Ultrasound imaging showed her
kidneys were slightly enlarged. Kidney biopsy showed diffuse mononuclear cell
interstitial infiltrates consistent with acute tubulointerstitial nephritis
(). She was prescribed ocular
dexamethasone and mydriatics, oral prednisolone (5 mg/m2/day),
amlodipine, and potassium citrate; her blood pressure, serum creatinine, and
tubular function were normalized, and she was on remission from uveitis within
three months. Two months later she was started on methotrexate (10
mg/m2/week) on account of recurrent uveitis. She had two other
episodes of recurrent uveitis without renal involvement, one and three years
after being diagnosed; the dosages of methotrexate (12.5 mg/m2/week)
and topical corticosteroids were adjusted. Five years after being diagnosed, the
patient was asymptomatic and on methotrexate. |
pmc-6533956-2 | A 12-year-old female arrived at the Emergency Unit complaining she had been
suffering from photophobia and ocular hyperemia for four weeks. She was
diagnosed with bilateral anterior and intermediate uveitis (). Her blood pressure was normal;
she had iron-deficiency anemia, an ESR of 120 mm/first hour, a GRF of 47
ml/min/1.73m2, leukocyturia, glucosuria, hematuria, and
non-nephrotic proteinuria ().
Infectious and autoimmune diseases were ruled out. Her chest X-ray images and
kidney ultrasound examination did not show alteration. She was started on
mydriatics, topical corticosteroids, and oral deflazacort. Her renal function
recovered in six weeks and she was on remission from uveitis within two months
of treatment; she stopped taking systemic corticosteroids and was started on
methotrexate (10 mg/m2/week).
The patient was on methotrexate and asymptomatic 18 months after being diagnosed,
and has not had renal dysfunction or recurrent uveitis. |
pmc-6533956-3 | A 12-year-old female arrived at the Emergency Unit with asthenia, anorexia,
nocturia, polydipsia, normocytic normochromic anemia, and a GFR of 59
ml/min/1.73m2. She came in two months later with pain and
hyperemia in her right eye, and was diagnosed with bilateral nongranulomatous
anterior and intermediate uveitis with synechiae (). Her CRP was 3.19 mg/dL, the GFR was at 47
ml/min/1.73m2, and she presented with leukocyturia, glucosuria,
hematuria, non-nephrotic proteinuria, and raised urine β2-microglobulin levels
(). Infectious and autoimmune
diseases were ruled out. Her chest X-ray images were normal. Renal histology
showed lymphoplasmacytic interstitial infiltrates consistent with acute
tubulointerstitial nephritis ().
She was treated with ocular corticosteroids and mydriatics, oral prednisolone
(12.5 mg/m2/day), and methotrexate up to 15 mg/m2/week.
She was on remission from uveitis three weeks after being diagnosed and her
renal function improved. Two months later she had recurrent uveitis and was
started again on topical corticosteroids. She entered remission and was on
methotrexate. |
pmc-6533961-1 | In 2012, a 56-year old male received a cadaveric renal transplant, with 2 human
leukocyte antigen (HLA) mismatches, for chronic renal failure related to
hypertension. He was an ex-smoker who had been undergoing hemodialysis for four
years. There were no records of blood transfusions or previous kidney
transplantation. The cold ischemia time was 17 hours. The immunosuppressive regimen
for induction consisted of Basiliximab. Maintenance immunosuppression included
tacrolimus 0.1 mg/kg/day, mycophenolate, and prednisone. Immediately after
transplantation, he developed delayed graft function with the need for hemodialysis
for one week, later achieving a stable renal function; he currently has a serum
creatinine of 1.4 mg/dl, which translates to an estimated glomerular filtration rate
of 55 mL/min/1.73m. Four years after kidney
transplant, at an outpatient medical follow-up, he presented asymptomatic
microscopic non-glomerular hematuria confirmed by the absence of erythrocytic
dysmorphism in the phase-contrast microscopy of the urine. The physical examination
was normal. The serum creatinine was 1.7 mg/dL. A sonogram showed a solid mass on
the left native kidney. Using a 3.5 MHz convex transducer (Aplio 400; Toshiba;
Tokyo, Japan), a CEUS with Sonovue® (Bracco Int; Milan, Italy) bolus of
2.4 mL injected using a 20-gauge intravenous cannula, followed by a 10 mL saline
flush was performed. The examination was performed using contrast harmonic imaging
at a low mechanical index of 0,1. The exam was documented by digitally storing the
images over 60 s in DICOM format. The images showed a hypervascular mass in relation
to the remaining parenchyma of the native kidney with heterogeneous enhancement and
pseudocapsule sign (). Quantitative
analysis with time-intensity curve was used to calculate the amount of enhancement
in the mass and remaining parenchyma of the native kidney. Accordingly, in the
arterial phase, the mass was considered hypervascular when compared to the remaining
parenchyma ().
A CT scan confirmed the presence of a unilateral isodense renal nodule of the upper
pole ().
The patient underwent total removal of the left kidney. The 2.5 cm diameter tumor was
reddish-brown with well-circumscribed borders that mimic a capsule. It also revealed
multiple nodules of 2.5 cm, 0.7 cm and 0.5 cm in diameter and supported the
diagnosis of multicentric oncocytoma. The microscopic findings showed numerous
tubular cells with abundant granular cytoplasm and large nuclei ().
Following nephrectomy, the patient was followed-up for six months and showed normal
renal function and no more microscopic hematuria.
Since we began to perform CEUS in our facility as clinical research, the ethics
committee approved the study according to local legal requirements and informed
consent for contrast use and the report of the case was obtained from the
patient. |
Subsets and Splits
Exclude ER emergencies
Retrieves 100 descriptions that do not contain the terms 'ER' or 'emergency', providing a basic filter of the dataset.