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pmc-6609788-1 | A 58-year-old man presented to us three years ago with a history of progressive anterior neck swelling and hoarseness of voice for the past one month. On clinical examination, there was palpable left anterior neck swelling with cervical lymphadenopathy. A core needle biopsy of the lesion revealed invasive poorly differentiated carcinoma. He defaulted our follow-up and opted for a left hemithyroidectomy at another institution. The histopathological report was intrathyroid thymic carcinoma. He refused adjuvant radiotherapy despite being counselled and subsequently defaulted follow-up.
Recently, the patient presented to us again with progressive hoarseness of voice and intermittent haemoptysis. He had shortness of breath, especially when lying flat, but no difficulty in swallowing. On clinical examination, there was a left supraclavicular mass measuring 2 × 2 cm. The mass was immobile and hard in consistency. The anterior aspect of the neck showed an irregular hard mass measuring 3 × 4 cm. An indirect laryngoscopy examination revealed a left vocal cord palsy, likely from his previous presentation. An intraluminal mass was seen over the posterior tracheal wall, occupying a third of the tracheal lumen. We proceeded with a contrasted computed tomography (CECT) of the neck and thorax, which revealed a lobulated hypodense soft tissue mass measuring 4.3 × 3.9 × 5.2 cm. It occupied the left thyroid bed from the C7/T1 to the T2/T3 vertebral level, with left retrosternal extension and tracheal deviation to the right. The soft tissue mass had displaced the left common carotid artery and internal jugular vein laterally. As the CECT showed (, ), the soft tissue mass had caused more than 80% of tracheal luminal narrowing. At retrosternal, multiple matted lymph nodes were seen.
Image-guided fine needle aspiration cytology (FNAC) of the soft tissue mass at the left thyroid bed showed malignant cells suggestive of intrathyroid thymic carcinoma (CASTLE). After a multidisciplinary meeting, we planned for curative resection and tracheostomy. Intraoperative tracheoscopic examination showed a soft tissue mass measuring 3 cm in length at the posterior tracheal wall, 2.1 cm from the vocal cord (). The mass was debrided to achieve an adequate diameter of tracheal lumen distal to the tracheostomy insertion. The left supraclavicular solid tumour densely adhered to the left great vessels. Due to a high risk of injury to the great vessels, we proceeded with tumour debulking only.
The histopathology report showed an unencapsulated tumour that displayed lobular architecture. The tumour lobules were composed of sheets of neoplastic polygonal cells rimmed by a variable amount of mature lymphocytes. The neoplastic cells were mildly pleomorphic, and lymphovascular invasion was present. Skeletal muscle bundles attached at the periphery showed focal tumour cell infiltration (, ). Immunohistochemical studies were immunoreactive for CD5 (membranous pattern) and CD117 but negative for thyroglobulin and TTF1 (, ). The proliferative index, Ki67, was approximately 20%.
Postoperative care was unremarkable, and no immediate complications were noted. The patient was counselled for adjuvant chemoradiotherapy, but he was not amenable to this course of action. He is currently under regular follow-up with no disease progression six months after the operation. |
pmc-6609882-1 | A 28-year-old woman without significant past medical history was referred to our institution for menorrhagia and 1 month history of progressive malaise (ECOG performance status: 1). She was treated with oral estro-progestative contraception at the time. Physical examination found fever, hypertension, asthenia, anorexia, dyspnea, abdominal discomfort, weight gain of 13 kgs, lower extremity edema, hepatosplenomegaly, and multicentric peripheral lymphadenopathy.
Laboratory investigations revealed normocytic, non-regenerative anemia (hemoglobin: 6.1 g/dL), thrombocytopenia at 24 × 109/L, leukocytosis at 14.3 × 109/L, with myelocytosis at 3% and dacryocytes on blood smear, blood urea nitrogen at 15.4 mg/dL, creatinine at 1.19 mg/dL (estimated glomerular filtration rate at 62 ml/min/1.73 m2 according to CKD-EPI formula), hypoalbuminemia at 33 g/L, cholestasis (alkaline phosphatase at 314 U/L, gamma glutamyltransferase at 369 U/L) without cytolysis, elevated fibrinogen at 7.8 g/L, elevated C-reactive protein level at 150 mg/L and, elevated β2 microglobulin level at 3.94 mg/L. Haptoglobin test and bilirubin levels were normal. Proteinuria was measured at 2 g/day including albuminuria at 1.5 g/day. Renal echography was normal. Urinary sediment examination found leukocyturia at 44,000/mL and hematuria at 645,000/mL but no bence-jones proteinuria. The blood protein electrophoresis showed an inflammatory profile without monoclonal bands on immunofixation test (total gamma globulins 13 g/L). There was no immunophenotypic aberrancy of lymphocytes by flow cytometry analysis (normal kappa/lambda ratio). Molecular studies were negative for breakpoint cluster region-abelson-1 fusion as well as, janus kinase 2 (JAK2), thrombopoietin receptor (MPL), and calreticulin (CALR) mutations. Auto-immune (complement, cryoglobulinemia, rheumatoid factor, anti-glomerular basement membrane, anti-neutrophil cytoplasmic, antinuclear antibodies) and viral [hepatitis C and B, Epstein-Barr virus (EBV), cytomegalovirus, parvovirus B19, Human Herpesvirus (HHV)-6 and -8, human immunodeficiency virus and syphilis] tests were negative. Vascular endothelial growth factor (VEGF) level was elevated at 640 pg/mL (normal range: < 500 pg/mL).
Computerized Tomography-scan showed diffuse lymphadenopathy, pleural and peritoneal effusion, and hepatosplenomegaly. Positron emission tomography found hypermetabolic lymph nodes, spleen and BM. The BM trephine biopsy evaluation revealed hyperplasia of the myeloid, erythroid, and megakaryocytic series without maturation anomalies, an overall myelofibrosis grade 2 using European consensus on bone marrow fibrosis grading, and polytypic plasmacytosis at 10%, without blast cells. Cytogenetic analysis of the BM aspirate demonstrated a diploid female karyotype.
A cervical lymph node biopsy found features consistent with the mixed histopathological subtype of iMCD (). Follicles were characterized by atrophic germinal centers with few lymphocytes and radially penetrating blood vessels (lollipop finding). The small lymphocytes of the mantle zones were arranged in concentric rings around the germinal center (onion skinning). There were also increased vessels and abundant plasma cells in the interfollicular region. We noted absence of Kappa or Lambda monotypic immunoglobulin and negativity for HHV-8 and EBV encoded RNA probe.
A renal biopsy revealed 32 glomeruli a MPGN pattern was observed with endocapillary proliferation (macrophages and neutrophils), mild mesangial proliferation, mesangiolysis, endothelial cell swelling and thickening of capillaries due to multiple double contours (light microscopy) (). There is also one focal segmental glomerulosclerosis lesion (tip variant). No thrombosis, no interstitial and tubular involvement, and no evidence of vasculitis were observed, but arteriosclerosis was present. Immunofluorescence found bulky endomembranous segmentary deposits for IgM, mild deposits for C1q, kappa and lambda, and no deposits for IgG, IgA and C3; this pattern was consistent with polytypic MGPN. An ultrastructural study revealed discrete electron-dense deposits without organization in the subendothelial space, partial podocyte foot process effacement, and absence of sub-epithelial or intra-membranous deposits. Considering (i) negative cryoglobulinemia, negative antinuclear antibodies, normal complement, (ii) mesangiolysis, endothelial cell swelling, mild mesangial proliferation, and multiple double contours, (iii) endomembranous focal deposits, the MPGN-like seems to occur in the settings of a chronic TMA.
Considering the clinical, laboratory, and histopathological findings, the diagnosis of the TAFRO syndrome [grade 3 severity, ()] subtype of iMCD () was made, and 1 mg/kg of prednisone was initiated. As we did not observe any laboratory improvement, we initiated 3 days of methylprednisolone pulse-therapy at 1,000 mg per day. We then observed a normalization of platelets, CRP, creatinine serum level (0.84 mg/dL), and proteinuria. Nine months later, the patient was in a complete remission and we progressively tapered the corticosteroid treatment. The patient remains in complete remission off of all therapy for 2 years, and she was able to give birth to a healthy child without recurrence of the disease. |
pmc-6610225-1 | A 30-year-old Arab female nurse was brought by family to the emergency department with a one-day history of epigastric pain, nausea and vomiting. She did not experience fever or altered bowel habits. Her past medical history revealed a laparoscopic one anastomosis gastric bypass operation for weight loss and diabetes mellitus 28 months prior, within a year her body mass index dropped from 53.6 kg/m [] to 41.5 kg/m [] with an estimated weight loss of 40% and was off diabetic medication, as her HbA1c fell from 10.8% to 5.9%. Physical examination showed that the patient was vitally stable but in pain. Abdominal examination revealed a non-distended abdomen with tenderness localized in the epigastric area. Routine laboratory results were unremarkable, and erect and supine abdominal X-rays showed nonspecific signs of intestinal obstruction.
The patient was admitted to internal medicine with an impression of gastritis. She was kept nil per os and given intravenous fluid along with omeprazole 40 mg once per day and metoclopramide 10 mg when necessary, but the condition showed no improvement. The patient was subjective to computed tomography which revealed evident of intraperitoneal free fluid, more significantly multiple dilated proximal jejunal loop. The distal small bowel is rather collapsed denoting small bowel obstruction which is due to jejuno-jejunal intussusception.
The general surgical team reviewed the patient and decided to proceed with surgery. The patient initially underwent a laparoscopic approach that was converted to laparotomy due to very large dilation of the small bowel performed by consultant general and a specialized metabolic and bariatric surgeon (Author 3). The intraoperative findings showed a small bowel tight intussusception of the biliary limb into the enteric limb with full-thickness necrosis (). The patient underwent resection, and primary end-to-end hand-sewn anastomosis of the biliary limb just distal to the previous gastrojejunostomy was performed.
The patient was in the intensive care unit for a total of two days observation, kept on antibiotics Piperacillin/Tazobactam 4.5 g and Metronidazole 500 mg, omeprazole 40 mg along with perfalgan 1 g and pethidine 75 mg when necessary all of which are given intravenous except the lateral given intramuscular for a total of 7 days. She was initially kept on Nasogastric tube with nil per so and was gradually proceed to soft diet discharged on the 6th day postoperatively.
Patient was followed up a week after discharge, two weeks then three month and was doing well, with no active complaint. |
pmc-6610228-1 | A 13-year-old boy was referred to our plastic surgery clinic with complaints of a slow-growing enlarged pruritic mass on his penis for over a year. He had had traditional male circumcision one year prior to his presentation. We asked the patient’s father to describe the process of traditional circumcision so that we could better understand the likely cause of the presentation. The procedure was done at a traditional initiation ceremony for boys which takes place around the same time every year. The process involved use of a razor blade to cut off the foreskin, without use of an anaesthetic. Haemostasis was achieved using pressure by wrapping material such as leaves and bamboo twigs around the wound. Finally a herbal paste was applied to the wound. He further went on to tell us that the recovery process had been slightly delayed compared to other boys in the same cohort. The patient’s past medical history was unremarkable. There was no family history of keloids according to the father. Examination showed a large irregularly shaped keloid along the circumference of the coronal sulcus, it measured approximately 6 cm broad and 5 cm thick in its widest dimensions (see , ) The patient also had other areas of keloids over his chest, both shoulders and back sustained from ritual ‘tattooing’ around the same period of the circumcision.
Consent for surgery and photography was sought from the patient’s father as the patient was considered a minor. The patient was scheduled for elective surgery under general anaesthesia. Surgery was performed by a consultant Plastic surgeon. The surgical procedure involved complete, circumferential excision of the keloid tissue to the level of dartos fascia. The circumcision-like wound was minimally undermined to allow a tension-free closure. Haemostasis was achieved with electrocautery. Single layer closure was done with nylon 5.0 sutures. Corticosteroid (1 ml of triamcinolone acetonide 40 mg/ml) was injected into the wound edges after skin closure. Standard dressings were used which included sterile Vaseline gauze and dry gauze. Corticosteroid was also injected into the keloids in other sites while patient was under anaesthesia. The peri-operative period was uneventful. The patient was sent home for review on the third post-operative day. With the view of his propensity for keloid formation he was given comprehensive advice to avoid all forms of cutaneous trauma.
Sutures were removed on the 14th post-operative day and a second dose of corticosteroid was injected. Wound healing was uncomplicated. The patient then returned for repeat injection every 6 weeks for a period of 6 months (see ). There was no recurrence after 1 year although the ventral aspect had a very mild degree of hypertrophic scarring. The keloids on his shoulders, chest and back also showed remarkable flattening of the scars. All injected area had minor degrees of hypo-pigmentation within the scar extending to a small surrounding circumference of normal skin. This did not bother the patient and he was assured that it was likely to improve after cessation of injections.
The patient was reviewed 3 years later with complaints of pruritus along the ventral surface of the penis (see ). There was no significant change in the size of the scar and the patient was treated with another course of 6 weekly corticosteroid injections successfully. He was discharged from clinic and for further follow up on request. He was grateful for the assistance. |
pmc-6610229-1 | We present a 60-year-old lady presented to the surgical consultation room complaining from painless mass in the right calf for the last 2 years.
The mass accidentally discovered 2 years before presentation which was diagnosed that time clinically as lipoma, the patient didn’t receive any treatment at that time.
In the last few months the mass increased gradually in size and the patient was aware about this change in the size.
The patient has hypertension for 10 years controlled with antihypertensive drugs, the past surgical history of the patient included history of laparoscopic cholecystectomy before 10 years and hysterectomy for large uterine fibroid before 5 years. The patient didn’t receive anthelminthic medication before surgery. |
pmc-6610232-1 | A healthy 53 year-old male presented with a 6-month history of non-productive cough. He had no significant past medical or surgical history and was a nonsmoker. Computed tomography (CT) demonstrated 7.4 × 5.2-cm right posterior mediastinal mass, abutting the trachea, superior vena cava, ascending aorta, and esophagus, as well as an incidentally noted retroesophageal SCA with a separate right carotid artery originating from the aortic arch (, ). CT-guided percutaneous biopsy revealed high-grade leiomyosaroma. Esophagoscopy did not demonstrate esophageal mucosal involvement. Metastatic workup was negative. After multidisciplinary discussion, four cycles of neoadjuvant chemotherapy with doxorubicin and dacarbazine were given in the hope that the tumor would decrease in size, permitting wider resection margins; however, there was no objective radiographic response.
He was subsequently taken to the operating room for resection and possible esophagectomy with planned substernal reconstruction and possible ligation of the right subclavian artery. Through a fourth intercostal muscle-sparing posterolateral thoracotomy, the large mediastinal mass was noted. Because the tumor was abutting the superior vena cava, ascending aorta, retroesophageal SCA, and trachea, it was not feasible to achieve wide margins without disrupting the vital structures. However, the mass encapsulated and was able to be dissected away from the surrounding vital structures. Extra care was taken not to injure the right subclavian artery, which was skeletonized away from the tumor. The mass was arising from the proximal esophageal muscular layer. Because there was no direct invasion of surrounding tissues, the mass was resected with partial resection of the esophageal muscular layer without violation of the mucosa or tumor capsule. A previously harvested intercostal muscle flap reinforced the exposed but intact mucosal layer of the esophagus.
The postoperative course was complicated by low-volume chyle leak, which resolved with conservative management. Adjuvant radiotherapy was offered, but he declined. The patient remained without evidence of disease for two years, until he was found to have a right 1.5-cm pleural-based metastasis, which was also resected (). He has no evidence of disease currently, now 30 months after resection of metastasis. |
pmc-6610239-1 | A 59-year-old lady with a BMI of 27 kg/m2 and no comorbid underwent an open appendicectomy via a Lanz incision for perforated appendicitis. There was a superficial surgical site infection which was treated by dressing followed by secondary suturing. Three years later, she presented to us with an incarcerated incisional hernia. We performed a laparoscopic intraperitoneal onlay mesh (IPOM) repair for her. Intraoperatively, standard port placement was done, followed by adhesiolysis of small bowel segments from the hernia sac. The fascial defect measures 6 cm in the largest dimension (). Prior to the mesh fixation, primary fascial closure was done using non-absorbable sutures (Prolene® 0) passed extra-corporeally with the help of an intravenous cannula BD Angiocath™ (14 gauge) instead of a transfascial suture passer. The rest of the procedural steps were the same as a standard laparoscopic IPOM repair. Post-operative recovery was uneventful, and during her follow-up six months later, she has no hernia recurrence or chronic pain. |
pmc-6610259-1 | A 32-year-old woman was referred to our hospital with severe psychosis. Three months previously, her family recognized emotional changes in the patient, such as euphoria and/or depression. She progressed to cognitive decline, insomnia, and whole-body edema developed one week before visiting another hospital. On admission to the other hospital, initial endocrine investigations revealed high ACTH (545 pg/mL) and cortisol (136 μg/dL) levels. Computed tomography (CT) showed an anterior mediastinal mass and multiple pulmonary nodules (Fig a,b). As ectopic ACTH-producing mediastinal tumor with pulmonary metastasis was suspected, metyrapone was administered at an initial dose at 250 mg/day, which was gradually increased to 750 mg/day. However, she lost the ability to communicate due to consciousness disturbance within one week of hospitalization. Therefore, she was transferred to the emergency unit of our hospital. Physical examination on admission revealed moon face and proximal muscle weakness. Body height and weight were 164 cm and 55 kg, respectively and her bodyweight had not altered during the previous few months. Blood pressure was 116/61 mmHg. She had emotional lability and impaired memory. Laboratory findings at our hospital demonstrated severe hypokalemia and metabolic alkalosis, in addition to the increase in serum ACTH and cortisol levels (Table ). She showed self-injurious behavior, and attempted to bite her tongue. Due to her severe psychosis and consciousness disturbance, intubation was required and she was sedated in the intensive care unit. Metyrapone was increased to the maximal dose of 4000 mg/day via nasogastric tube. In addition, potassium supplementation was performed at 200 mEq/day and spironolactone was used to control low potassium levels. On day 8, we added perospirone hydrochloride hydrate to propofol sedation to suppress restlessness (Fig ). Thoracic surgery was performed for the lung nodule. Hematoxylin and eosin staining of the resected specimen indicated irregularly-shaped sheets and nests of tumor cells, and a diagnosis of NET was made (Fig ). Immunohistochemical staining revealed tumor cells positive for ACTH, chromogranin A, and synaptophysin. The Ki-67 labeling index was <1%., indicating that cancer staging by the WHO classification was neuroendocrine tumor G1. The time course of changes in serum cortisol levels is shown in Figure . As cortisol levels decreased after initiation of metyrapone, her conscious disturbance improved. She became able to communicate and was extubated on day 14 of hospitalization. Somatostatin receptor scintigraphy was performed and showed abnormal accumulation in the anterior mediastinal mass (Fig a) but not in the pulmonary nodules (Fig b). There were no other metastatic organs, including the brain. Resection of the anterior mediastinal tumor and the largest metastatic lesion of the right lung were subsequently performed through a median sternotomy to decrease serum ACTH level as much as possible. The pathological findings, including immunological staining, in the mediastinal tumor were similar to the initially resected left lung tumor and confirmed that the tumor was thymic in origin. Thus, according to the WHO classification in 2018, a diagnosis of NET of the thymus (typical carcinoid) with multiple pulmonary metastases was made. Postoperatively, a combination of octreotide long-acting repeatable (LAR; 30 mg) every four weeks and everolimus (10 mg/day) therapy has been continued, and ACTH and cortisol levels have remained stable. There was no personal or family history of multiple endocrine neoplasia type I in the present case. |
pmc-6610285-1 | A 78-year-old male patient presented in April 2015 with suspected lung cancer. Chest CT examination revealed a pulmonary space-occupying lesion in the left upper lobe of the lung (Fig. ). Following fine needle biopsy, pathology confirmed the patient had lung adenocarcinoma and EGFR (Fig. ), ALK and ROS1 were negative (Fig. ). Due to the advanced age of the patient (78) together with a diagnosis of atrial fibrillation, EGFR-TKIs was recommended as the treatment of choice. From 23 October 2015, the patient commenced gefitinib 250 mg orally once a day until the disease progressed in July 2016 when CT scan of the chest indicated metastasis in the lung. The gefitinib, everolimus combined therapy was subsequently recommended because of disease progression. However, after a few months of combined treatment, everolimus had to be withdrawn because the patient experienced severe breathlessness, cough and mouth ulcers. At that time, the patient had been taking gefitinib for 16 months until drug resistance developed on 16 February 2017 (Fig. (a)).
The patient had second gene mutation testing at this time which indicated EGFR T790M mutation. Osimertinib 80 mg/day orally was subsequently recommended from March 2017. After only one month of treatment, chest CT scan revealed the disease had almost completely resolved (Fig. (b)). However, severe interstitial lung disease was also confirmed (Fig. (b)). The patient was found to have severe cough and difficulty in breathing at this time and the symptoms did not resolve, even after anti-infection and anti-cough treatments were administered. The patient attended our hospital for further treatment. After reviewing his treatment history and chest xray, we considered that he was suffering from severe drug-induced interstitial lung disease and advised him to cease taking the osimertinib immediately. Unfortunately, he still continued to take the osimertinib as he considered the medicine to be effective for his tumor, and refused to tell the doctor the truth until it was found that he was suffering from further dyspnea. After anti-infection and high dosage methylprednisolone (240 mg/day) and mechanical ventilation, the patient finally died of multiple organ failure after 2 weeks treatment for severe interstitial lung disease and resultant complications. |
pmc-6610670-1 | The described patient provided written informed consent. A 69-year-old male patient was referred to the Emergency department (ED) of our university hospital. The past medical history mentions pleuropneumonia and tuberculous meningitis 21 years prior to presentation. A follicular lymphoma was diagnosed 8 years later for which he was treated with chemo-immunotherapy and allogenic stem cell transplantation. Subsequent immune suppression therapy to minimize graft versus host disease after allogenic stem cell transplantation was complicated by an episode of meningitis and pneumonia. Because of this past medical history he was checked at the Haematology Department on a regular basis, during which laboratory analysis was performed to monitor renal and liver function and haematological parameters. At the most recent visit three months prior to presentation no clinically relevant abnormal laboratory results were obtained ().
The reason for patient presentation at the ED was suspicion of pneumonia with complaints of dyspnoea, without signs of coughing. Moreover, he experienced weakness in his legs since one week. During physical examination, the patient had a body temperature of 39.6 degrees Celsius and an oxygen saturation of 96% during three litres oxygen supplementation per minute, but was not in acute respiratory distress. Percussion dullness was noticed at the lower right thorax during pulmonary examination. The chest X-ray was markedly abnormal with flattening of the left hemi-diaphragm and pleural effusion and atelectasis in the right hemithorax was observed, but this was in accordance with previous observed pleurodiaphragmatic adhesions (). The abnormalities at the left were slightly increased compared to a previous observation.
To support the diagnostics, the phlebotomist collected blood samples and sent these to the laboratory for haematological and chemical analysis to assess electrolytes, renal function, liver function, infection and haematological parameters. The haematological parameters were measured on an automated XN-9000 haematology analyzer (Sysmex, Norderstedt, Germany). A Cobas C-8000 automated modular analyzer (Roche Diagnostics, Indianapolis, USA) was used to perform chemical analysis. No technical errors during laboratory analysis were reported. Strikingly reduced concentrations of potassium were observed (). Therefore, the clinician decided to supplement the patient with 40 mmol potassium orally to prevent serious rhythm abnormalities. In addition, 2.25 mmol calcium gluconate was supplemented intravenously based on the low concentration of calcium analysed from the same blood sample. To investigate whether the hypokalaemia and hypocalcaemia caused cardiac conduction disturbances an electrocardiogram (ECG) was performed. However, no abnormalities consistent with low potassium or low calcium were observed (). |
pmc-6610676-1 | A 59-year-old female patient has encountered a problem of unexplained weight loss over 10 kg within 6 months. Except for a nonspecific abdominal pain, there were no other GI symptoms. Her vital signs were within normal limit. On examination, she was pale. It was astounding for the patient that there was a palpable fist-sized mass at left upper quadrant during the physical examination. The routine laboratory tests revealed hemoglobin of 9 g/dL, and other blood tests were unremarkable. Computed tomography (CT) scan demonstrated a heterogeneously enhancing solid-cystic mass measuring 10 cm in maximal diameter at the pancreatic body and tail (). The provisional diagnosis of pancreatic mucinous cystadenoma was made and the patient was scheduled for distal pancreatectomy, lymphadenectomy, and splenectomy.
At the theatre, the exploration through a Chevron incision revealed that the tumor originating from the proximal jejunum just distal to the ligament of Treitz, in lieu of a tumor of the pancreas (). The tumor that was abutting the body and tail of pancreas, with meticulous handling, was dissected and removed. There was no spillage or rupture of the capsule while removing the tumor. End-to-end jejunojejunostomy completed the operation. There was an absence of adjacent mesenteric lymphadenopathy. Gross specimen, measuring 9 × 8 × 6.5 cm, was a soft, round-shaped, tan-grey colored mass with an irregular surface. There was large central necrosis and cavity. With a sequential serial section on histologic examination, the tumor was contained in the small intestinal segment in which the intramural mass consisted of interlacing bundle of spindle cells and interlacing bundle formation (). Nuclear atypia was presented. However, nuclear mitosis was scarcely found (0–1 /high-power field) (). The tumor margins were free from tumor cells. In the immunohistochemical study, the tumor cells were positive for tyrosine-kinase protein (CD117) and transmembrane protein (DOG1) which was consistent with GISTs. Post-operatively, there was no complication. The patient uneventfully recovered and discharged on postoperative day 8. Due to the large-sized tumor which entails an increased risk of tumor recurrence, adjuvant therapy was initiated with imatinib mesylate.
The patient was symptom-free during follow up at one month, three months, and six months after surgery. The CT scan was performed one year after the operation and no recurrence was detected. |
pmc-6610679-1 | A diabetic 90 years old woman with cough and diffuse abdominal pain was admitted into our hospital with an initial diagnosis of sepsis. Blood sample investigation revealed mild leucocytosis with PRC augmentation and normal AST, ALT and bilirubin levels. Preoperative ultrasound revealed thickened gallbladder wall suggestive of acute cholecystitis. She has had previous sigmoid resection for diverticulitis and ERCP for choledocholithiasis without any mention of biliary abnormality. She was scheduled for a laparoscopic cholecystectomy during which the incidental intraoperative finding of a left sided gallbladder was made. Standard trocar positions were used (A) without any variation of technique, simply following the Strasberg criteria for safe cholecystectomy []. Having seen the anatomical variation (B), a careful dissection of Calot’s triangle was carried out, the cystic duct and artery were identified and isolated, and subsequent complete dissection of the gallbladder from the liver was done (C). This manoeuvre allowed us to demonstrate a right sided positioning of the cystic duct. Furthermore, a safe clipping and transection of the cystic duct and artery was achieved (D), and the gallbladder was extracted in an endobag. The postoperative course was complicated by an aggravation of diabetes and basal pneumonia which were controlled postoperatively in the intensive unit. The patient was discharged on postoperative day 16, in good condition. Retrospective analysis of the clinical case was done and a previous CT scan was reevaluated. Even with the knowledge of a left sided gallbladder, the CT scan was unable to demonstrate its attachment to the left side of the liver. This was probably due to the presence of a hypertrophic left lobe mimicking only a contiguity with the gallbladder, which itself appeared to be in a physiological position (). A vascular and biliary reconstruction was then evaluated with evidence of agenesis of the right anterior sector of the liver () with the absence of the right hepatic duct and an intrahepatic variation with insertion of the right inferior sectorial duct into the left common duct () as type A3 of Huang Classification []. |
pmc-6610692-1 | A 42-year-old man was seen in our outpatient department complaining of moderate left upper quadrant abdominal pain. He was known to have a liver hemangioma diagnosed 1.5 years prior. Double helical CT scan revealed an 8.2 x 5.8 cm () vascular lesion within segments II and III of the liver with peripheral nodular enhancement. The surgery was performed according to the technique described above. The vascular pattern of the hemangioma could be differentiated from the one of the liver using ICG (). Patient’s outcomes are summarized in . |
pmc-6610692-2 | A 44-year-old man presented to our clinic with long-standing left-upper quadrant pain. CT scan revealed a 3.9 x 3.5 cm segment III hemangioma (). Positioning, trocar placement and surgical technique previously described. Outcomes are summarized in . |
pmc-6610692-3 | A 62-year-old man with a known diagnosis of liver hemangioma, presented to our outpatient department with right-upper quadrant pain. CT scan imaging showed a 9.1 × 7 x 2.2 cm mass occupying segments IVa and V. Prior to surgery, the patient underwent selective arterial embolization in the attempt to decrease the size of the lesion. Trocar placement was similar to the one showed in . Intraoperative US scanning showed that the deeper part of the hemangioma was very close to the bifurcation of the portal elements. Liver hilum was dissected, and a bulldog was applied to the common hepatic artery. After the falciform ligament was taken down, dissection started between the segments III and IVB, on the right side of the falciform (). The operation followed using the same technique previously described. Outcomes are summarized in . The patient had an uneventful post operatory course and was discharged on POD 5. One day after discharge, patient was readmitted with a pulmonary embolism which was successfully treated with subcutaneous low molecular weight heparin. |
pmc-6610708-1 | A 61-year-old woman was referred by another institution with a diagnosis of distal transverse colon invasive adenocarcinoma and resectable liver metastases. Her past medical history revealed type 2 diabetes mellitus, hypertension, and chronic kidney disease. No relevant family history was noted. The case was discussed at the multidisciplinary tumor board meeting, and the decision was taken for the patient to undergo surgery for the primary tumor and subsequently address the treatment of liver metastases. A laparoscopic-assisted transverse colectomy with primary anastomosis was performed with no intraoperative complications. On the fourth postoperative day, she developed tachypnea, fever, and leukocytosis. A CT scan of the chest and abdomen with contrast was carried out. This showed a right middle lobe opacification consistent with pneumonia. A small <5 cm localized pelvic collection was noted; otherwise, the intra-abdominal organs including the spleen were unremarkable (). Blood cultures were negative. Conservative treatment with intravenous ciprofloxacin was initiated. Her condition slowly improved. However, three days later, she developed severe metabolic acidosis due to acute renal failure. She was shifted to the ICU and underwent urgent hemodialysis. Four days after starting hemodialysis, she complained of acute left upper abdominal pain and developed hypovolemic shock.
Laboratory investigations showed normochromic normocytic anemia (hemoglobin 8.7 g/dL (12-17), white cell count 20.8 × 109/L (4-11), platelet count 517 × 109/L (150-400), blood urea nitrogen 12.9 mmol/L (2.7-7.2), and creatinine 342 μmol/L (53-97)). Liver function tests showed an albumin of 14 g/L (34-50), alkaline phosphatase of 225 μ/L (54-144), prothrombin time of 16.1 (9.6-12.6), partial thromboplastin time of 36 sec. (24.3-30.2), and international normalized ratio of 1.5 (0.8-1.2).
Blood gases revealed a pH of 7.15 (7.3-7.4), pCO2 of 27 mmHg (36-46), HCO3 of 18.5 mmol/L (21-28), lactate of 1.5 mmol/L (0.5-1), and anion gap of 24.2.
A CT scan of the abdomen and pelvis showed a large subcapsular splenic hematoma with gas formation. There was no evidence of bowel perforation or obstruction ().
An emergency laparotomy was performed, and this showed a ruptured splenic hematoma. Peritoneal lavage and splenectomy were performed. Histology showed a necrotic spleen with fibrin and focal fat necrosis with no evidence of malignancy.
The patient made a slow but uneventful recovery and was eventually transferred to the nephrology unit to continue treatment for renal failure. |
pmc-6610738-1 | The patient is a 41-year-old man, without any history of disease or medication. He is slightly obese and a current smoker (>50 pack-years) and reports moderate alcohol consumption. He was admitted in a regional hospital with sudden onset of hemoptysis and headache. There, the patient was diagnosed with hypertension (systolic blood pressure: 240 mmHg, diastolic blood pressure: 110 mmHg), and hemoptysis was confirmed. A brain and chest computed tomography (CT) scan was performed that showed no findings from the brain and alveoral hemorrhage, respectively (). In addition, the patient underwent a formal transthoracic echocardiogram (TTE), which revealed mild to moderate mitral valve regurgitation and prolapse. Blood tests were within normal rates. The patient was subsequently transported to the Pulmonary Department of our hospital for further investigation and treatment.
Upon his admission, the patient's temperature was 37.6°C, heart rate was 98 bpm, respiratory rate was 22 breaths per minute, blood pressure was 177/89 mmHg, and an arterial blood gas showed mild hypoxemia in room air (pH = 7.47; pCO2 = 34 mmHg; pO2 = 68 mmHg; HCO3 = 18 mmol/L; O2 saturation = 93%). Blood tests were normal again, except for elevated uric acid levels. Blood cultures were collected, and he was initially started on empiric broad-spectrum antibiotic treatment of piperacillin-tazobactam and oxygen supplementation due to hypoxemia. Chest CT was performed once again, which identified findings compatible with alveoral hemorrhage and consolidation in the right middle lobe. An abdominal CT scan resulted in findings such as slightly enlarged liver without focal lesions and bilateral, well-defined adrenal lesions, possibly adenomas. The patient refused to undergo a new brain CT or MRI scan. Pulmonary function tests showed obstructive lung disease, and bronchoscopy revealed blood clots in the right middle lobe. His HIV-screening test was negative, as was Mantoux tuberculin skin test (TST).
Two blood cultures revealed bacteremia by Serratia marcescens, and this finding was confirmed with one more blood culture, taken 5 days later. The Serratia marcescens isolate was susceptible to cefepime, meropenem, gentamicin, sulfamethoxazole-trimethoprim, and ciprofloxacin. An urgent transesophageal echocardiogram (TEE) was performed which demonstrated severe mitral regurgitation accompanying ruptured mitral chordae tendineae (Figures and ). The patient was transported to the Infectious Diseases Department, and antibiotic treatment was subsequently changed to meropenem 2 g tid, ciprofloxacin 400 mg bid, and gentamicin 80 mg tid intravenously, according to antibiogram. Of notice, he reported a diagnostic arthrocentesis in his left knee joint a few weeks ago.
Three days later, the patient was afebrile, hypoxemia and hemoptysis were resolved, blood tests remained within normal limits, including inflammation markers such as C-reactive protein (CRP) and WBC, and multiple subsequent blood culture results came back negative. A new transesophageal echocardiogram was performed 20 days after the previous one, without further deterioration.
The patient, after having stayed at our hospital for 4 weeks, was transported back to the regional hospital in order to continue his intravenous antibiotic treatment with meropenem and ciprofloxacin for a total of 6 weeks. He had already completed 2 weeks of gentamicin treatment.
Both cardiologist and cardiac surgeons suggested surgery for valve repair or replacement, but the patient refused. Therefore, close surveillance with repeat echocardiograms every 3–6 months was recommended. Moreover, pulmonary surveillance with a repeat bronchoscopy was advised after completion of therapy. After 24 months, he is currently asymptomatic, without limitations in physical activity. |
pmc-6610746-1 | The patient was a 30-year-old G2P1001 at 36 weeks and 4 days who was transferred to Loyola University Medical Center (LUMC) with a Type A aortic dissection. Her pregnancy was complicated by gestational hypertension that had been diagnosed one week prior to her initial presentation. Baseline HELLP labs (which include a CBC, CMP, and urine protein to creatinine ratio) were obtained and were normal at that time. The patient's blood type was O negative and antibody negative and she was Group B streptococcus positive; her serologies were otherwise unremarkable. She had had a prior normal spontaneous vaginal delivery at term without complications. Her past medical history and past surgical history were otherwise noncontributory. She had been taking her prenatal vitamins daily. She was a former smoker having quit one year prior; her social history was otherwise negative for alcohol or recreational drug use. The patient had a negative family history of connective tissue disease or aortic dissection.
She initially presented to an outside hospital two weeks prior to her admission to LUMC, complaining of left-sided chest pain; initial evaluation with EKG and CTPE was negative for acute coronary syndrome and pulmonary embolism and she was discharged home. Over the subsequent two weeks, her chest pain had slowly improved. On the day of her presentation to LUMC, however, her pain acutely increased, characterized as “sharp” and “tearing.” At the outside hospital, CTPE was negative, though the retrospective read of her second CT identified the aortic dissection. EKG showed mild ST-depression and her troponin levels were increased to 1.75. On physical exam, the patient was tachycardic with normal blood pressures and oxygen saturation; she was found to have a new continuous aortic murmur with bounding pulses. She was sent for a TTE with the final read showing evidence of dilation of the ascending aorta (4.2cm) with a dissection flap of aorta seen in the ascending aorta and aortic arch, severe aortic regurgitation, and severe diastolic dysfunction ().
She was transferred to our tertiary care center. On presentation to LUMC, her blood pressures were mild range and her HELLP labs and coagulation factors were normal. The fetus had a category I tracing. She was consented for cesarean delivery with repair of aortic dissection. She underwent a primary low transverse cesarean section under general anesthesia; total estimated blood loss (EBL) for this portion of the case was 1L. She was given Pitocin, misoprostol, and a B-lynch was performed for uterine atony. As there was concern for potential coagulopathy related to her aortic repair, a JP drain was left in place. She delivered a liveborn male infant with APGAR score of 9/9 and an umbilical artery pH of 7.34 with a 1+ base excess. After completing the obstetrics portion of the case, the patient underwent an emergency repair of the aortic dissection with resuspension of the aortic valve, primary repair of the left main ostial dissection, insertion of a hemishield graft, and cut-down of the left femoral artery. She was given 4L of cardioplegia, a sterile, isotonic crystalloid solution used to induce cardiac stasis and protect the myocardium during open-heart surgery, and was placed on cardiac bypass during the procedure with a pump time of 230 minutes and a cross-clamp time of 208 minutes []. The patient received 500cc of cell saver, 1U of platelets, 2U of FFP, 1U of cryoprecipitate, and 1U of pRBCs; total EBL for the second portion of the case was 800mL. Her postoperative course was uncomplicated, and she was discharged home on postoperative day seven with a hemoglobin of 7.4 and planning to use the copper IUD for contraception. |
pmc-6610752-1 | A 37-year-old Iraqi woman, living in the Netherlands, with no cardiac history, was brought to the emergency department with an out-of-hospital cardiac arrest (OHCA) due to ventricular fibrillation (VF). That evening, she had suddenly collapsed without any warnings. After two to three minutes, the police started with basic life support, and the paramedics arrived after ten minutes. On arrival, VF was documented, and after three direct current shocks, sinus rhythm was restored.
She was a nonsmoker, and there was no history of drug abuse. Current medications included diclofenac, mebeverine, and vitamin supplements. Her medical history consisted of a gastric banding operation one year ago, and prior to the event, she had only experienced some dizziness at times. The postoperative course of the gastric banding operation was uncomplicated. Her mother and four siblings lived in different countries around the world, so there was limited information about her family history. Her father passed away at the age of 55 due to complications of diabetes mellitus. The only thing that is known is that her family history was negative for SCD or cardiac disorders. Both of her sons had been diagnosed with autism but were in good physical health. The 12-lead electrocardiogram at presentation showed sinus rhythm with a frequency of 103 beats per minute and frequent premature ventricular complexes in bigeminy pattern with a QTc interval of 419 ms (). A computed tomography (CT) scan of the thorax and cerebrum showed no evident abnormalities. After therapeutic hypothermia of 24 hours, she regained consciousness with reasonable neurological recovery without apparent sequela. Furthermore, a transthoracic echocardiogram demonstrated a good left and right ventricular function and no valvular abnormalities.
Days after detubation, episodes of frequent premature beats, nonsustained ventricular tachycardias, and prolonged QTc intervals manifested, and treatment with propranolol was started. Furthermore, frequent episodes of torsade des pointes were observed (). Cordarone was given, which resulted in progression of the torsade des pointes into VF requiring electrical cardioversion. Cordarone treatment was discontinued. Laboratory tests showed a mild hyponatremia of 134 mmol/l, a hypokalemia of 3.1 mmol/l, and a normal calcium level of 2.38 mmol/l. However, after electrolyte corrections, ventricular tachyarrhythmias persisted. During hospitalization, the QTc interval varied between normal (<460 ms) and severely prolonged (552 ms) (). Due to bradycardias, treatment with propranolol was discontinued. Eventually, sinus rhythm was achieved with flecainide. Coronary angiography showed no coronary artery stenosis, and cardiac magnetic resonance imaging also revealed no abnormalities.
A dual-chamber implantable cardioverter-defibrillator was implanted for secondary prevention. Subsequently, genetic testing for the LQT1, LQT2, LQT3, LQT5, and LQT6 genes identified a heterozygous c.3092_3096dup (p.Arg1033ValfsX26) mutation of the KCNH2 gene (LQT2) and a heterozygous c.170T > C (p.Ile57Thr) unclassified variant (UV) of the KCNE2 gene (LQT6). The UV (missense mutation) of the KCNE2 gene is likely a pathogenic mutation, what results in the digenic inheritance of LQT2 and LQT6. Genetic screening revealed that both sons are not carrying the familial KCNH2 mutation. They have not been tested for the UV of the KCNE2 gene. |
pmc-6610913-1 | A 77-year-old male patient had a history of myocardial infarction for half a year, but without significant discomfort, lived a normal life at home. Two hours before seeing a doctor, he suddenly developed significant palpitations and shortness of breath and was admitted to the Second Hospital of Lanzhou University by ambulance. The patient’s expression was painful and a distinct murmur was heard in the precordium. Electrocardiogram showed old left ventricular anteroseptal myocardial infarction. X-ray showed enlarged heart shadow. Myocardial enzymes and other laboratory test results showed no significant abnormalities. Echocardiography showed that the interventricular septum was thinned near the apex and bulged toward the right ventricular side, exhibiting “paradoxical motion,” and a rupture opening of about 8 mm in diameter was visible on its top (Fig. ). CDFI: left ventricular blood shunted to right ventricle through rupture (Fig. ), the shunt velocity measured by CW is about 410 cm/s, PG:67 mmHg. Left ventricular enlargement (anteroposterior diameter about 61 mm), significantly reduced wall motion amplitude, left ventricular ejection fraction (EF) about 33%, moderate aortic and mitral regurgitation, pulmonary artery systolic pressure about 60 mmHg. Echocardiographic diagnosis: old myocardial infarction of ventricular septal apex with ventricular septal rupture. Due to the patient’s older age and reduced left ventricular function, surgical repair of the site of ventricular septal rupture was very difficult. After multidisciplinary discussion, it was agreed that the patient could not undergo thoracotomy and was not suitable for thoracotomy, and echocardiography guided interventional occlusion of the ruptured interventricular septum could be performed. Interventional occlusion was performed after obtaining the patient’s family’s consent and signing a written consent. Under the guidance of transesophageal echocardiography, the ventricular septal rupture was clearly displayed during the surgery. The size of the rupture was measured again. In order to prevent the occluder device from falling off, a VSD occluder device that is larger and can cover the normal myocardial tissue was selected. Under direct vision, the guide wire and sheath smoothly passed through the ventricular septal rupture (Fig. ). The left ventricular side of the occluder device was opened and the occluder device was gently pulled to determine the stability of the occluder device (Fig. ). Then the right ventricular side of the occluder device was opened (Fig. ), and then the occluder device was gently pushed to determine the stability of the occluder device again to ensure that the occluder device was stable and there was no residual shunt around the occluder device. The operation was completed. After repeated examinations within one year, the occluder device was in normal position without residual shunt(Fig. ), left ventricular diameter was gradually decreased (anteroposterior diameter was about 52 mm), left ventricular function was improved, and left ventricular EF reached 46%. The patient can live a normal life. |
pmc-6610916-1 | Patient 1 was a 27 month old girl (Fig. a) born at full term pregnancy but too small for gestational age: her birth weight was 2.12 kg (< 10th centile), length 48 cm, and head circumference 31 cm (< 10th centile). She presented with failure to thrive, dysmorphic features including microcephaly (z score: − 2.4), severe lumbar hyperlordosis, limited hip abduction and neuropathy. Shortly after birth the patient developed respiratory distress requiring intubation. Several attempts of extubation were frustrating and the patient underwent tracheostomy. She had episodes of seizures controlled by phenobarbital. Since EEG did not show epileptiform discharges, phenobarbital was discounted without recurrence of seizures. Cerebral MRI revealed atrophy with dilated ventricles and enlarged subarachnoid space, extensive periventricular leukoencephalopathy, and partial agenesis of the corpus callosum (Fig. ). Echocardiography revealed a patent foramen ovale and a patent ductus arteriosus. At age 1 month she developed gastrointestinal reflux due to intestinal obstruction secondary to jejunal stricture, managed by resection of the stricture and end-to-end anastomosis. Gastroscopy revealed a high-grade reflux disease. The index patient continued to have severe delay in all developmental aspects, did not gain any milestones, and continued to be ventilation-dependent. The further course was complicated by chronic lung disease. At 24 months she experienced an episode of apnea, and died from cardiac arrest at age 28 months. The karyotype was XX. |
pmc-6610927-1 | A 35 years old (height, 159 cm; weight, 85 kg; gravida 3, para 1) pregnant woman with SAS presented to our hospital and was scheduled to undergo elective CS under epidural anesthesia at 36 weeks of gestation. An echocardiographic examination at 34 weeks of gestation revealed that the diameter of the beginning portion of the aorta (under the aortic valve) was narrowed by about 17 mm. The diameter of the ascending aorta was normal, the average pressure gradient was 40 mmHg, and the ejection fraction was 67%.
In the operation room, her baseline vital signs were as follows: heart rate (HR), 82 bpm with normal sinus rhythm; noninvasive blood pressure (BP), 130/76 mmHg; and oxygen saturation (SpO2), 96%. Supplemental oxygen was administrated by a face mask at the rate of 3 L/min. Traditional epidural puncture was performed at the L1–2 and L3–4 interspaces in the left lateral position, and a catheter was inserted to a depth of 4 cm into the epidural space at the cranial and caudal ends of the surgical field. The patient was then placed supine in a left-tilt position; 3 mL of 2% lidocaine was injected as a test dose, and another 7 mL was injected 5 min later through the cranial catheter. Additionally, 3 mL of 1.5% lidocaine was injected through the caudal catheter. At 20 min after the lidocaine injection, the sensory block had reached T6. The patient had no discomfort, and her hemodynamic parameters were stable. The operating bed was adjusted from the left-tilt to horizontal position, and the operation was allowed. Two minutes later, the patient reported chest distress and difficulty breathing (HR, 110 bpm; BP, 80/69 mmHg; SpO2, 96%), and 3 mg of intravenous ephedrine was promptly administered. The patient immediately lost consciousness with no response (HR and BP were depressed to 40 bpm and 53/15 mmHg, respectively). The lowest BP at the time was not measured because the noninvasive BP measurement interval was set to 1 min. The obstetricians sped up the surgery, and in another 2 min, a 2750-g male newborn was delivered with an Apgar score of 10 at both 1 and 5 min. Spontaneous circulation of the patient was returned immediately after delivery, and stable vital signs returned with no other treatment. The postoperative course was uneventful. The mother and neonate recovered uneventfully and were discharged 4 days later. |
pmc-6610941-1 | A 62-year-old man was admitted to our hospital in March 2016 for control of massive hemoptysis of unknown cause. He had experienced hemorrhage for 10 consecutive days (maximum of 800 mL/day) despite conservative intravenous therapy and two bronchial artery embolization procedures at a local hospital. Upon admission to our hospital, the chest computed tomography (CT) findings obtained at his local hospital revealed complete obstruction of the left superior PV and stenosis of the right superior and left inferior PV, and bronchoscopic examination revealed hemorrhage from the left upper lobe. His medical history included radiofrequency catheter ablation for AF 5 months previously. Surgical left upper lobectomy was performed on an emergency basis and the hemoptysis was controlled for 2 days. However, on postoperative day 3, he developed another episode of massive hemoptysis (hemorrhage of 500 mL). Physical examination revealed severe moist rales over the bilateral thorax. His hemoglobin level was 73 g/L. The patient underwent bronchoscopic examination and multidetector row CT angiography. The bronchoscopic examination revealed hemorrhage from both the right upper lobe and left lower lobe. CT angiography revealed stenosis in the right superior PV (approximately 95%) and left inferior PV (approximately 90%) (Fig. ). When offered urgent surgical venoplasty or nonsurgical PV stenting, the patient chose minimally invasive catheter-guided PV stent implantation. Bilateral PV stenting was therefore performed to control refractory massive hemoptysis.
Procedural access was obtained through the right femoral vein, and a 12-Fr venous sheath (Cook Medical, Bloomington, IN, USA) was placed. Selective right upper, right lower, and left lower lobe pulmonary angiography was performed using a 4-Fr H1 catheter (Cordis Corp., Miami Lakes, FL, USA) to indirectly evaluate the location and narrowing of the PVs. An 8.5-Fr sheath (SL 1™; St. Jude Medical, Saint Paul, MN, USA) with a Brockenborough™ needle (St. Jude Medical) was used to cross the intra-atrial septum, through which direct right superior and left inferior pulmonary venography was performed using a 4-Fr H1 catheter (Cordis Corp.). Venography showed severe PV narrowing (Fig. a, c; Additional file : Video S1, Additional file : Video S3). From the catheter, a 260-cm Amplatzer guidewire (Cordis Corp.) was exchanged, and a 90-cm 7-Fr sheath (Flexor Check-Flo Introducer sheath; Cook Medical) was inserted into the distal side of the PVS. From the sheath, a 10- × 25-mm and a 9- × 25-mm balloon-expandable stent (Express™ LD Vascular; Boston Scientific, Marlborough, MA, USA) were placed in the right superior and left inferior PVs, respectively (Fig. b, d; Additional file : Video S2, Additional file : Video S4). The PV pressure was measured before and after PV stent placement. The pressure of the right superior PV (systolic/diastolic/mean) decreased from 44/23/32 to 16/3/10 mmHg and the pressure of the left inferior PV (systolic/diastolic/mean) decreased from 28/17/22 to 15/6/10 mmHg, respectively. The proximal part of two stents extended to left atrium about 8 mm.
This procedure resulted in control of the hemoptysis, and the patient had developed no further episodes of hemoptysis by the 30-month follow-up. During stent placement and after the procedure, anticoagulation or anticoagulant was continued. Before the procedure, 5000 IU of intravenous heparin was administered. Immediately after the procedure, the patient received an anticoagulant agent (low-molecular-weight heparin sodium), and this treatment was continued until discharge. After discharge, the patient received an oral anticoagulant agent (warfarin) and antiplatelet agent (clopidogrel bisulfate) until the 12-month follow-up CT examination, which showed widening of the PV stents. The 30-month follow-up CT examination revealed no restenosis (Fig. ). |
pmc-6610942-1 | A healthy 16-year-old Korean male presented to our clinic in September 26, 2018 with blurred vision in his left eye. He had neither past medical history nor trauma history.
On ocular examination, best corrected visual acuity (BCVA) was 0 logMAR in his right eye and Hand motion in his left eye. Intraocular pressures were 17 mmHg in the right eye and 13 mmHg in the left eye. The fundus was invisible due to massive VH in the left eye (Fig. a). His blood pressure and laboratory test results including coagulating factors were normal.
The VH partially decreased 2 weeks later and the BCVA improved to 0.4 logMAR in the left eye. No cause of VH other than the PHA was found (Fig. b). Slit-lamp examination demonstrated a Mittendorf’s dot located in the inferior nasal quadrant of the posterior lens capsule in the left eye (Fig. ). Optical coherence tomography (OCT) showed hyporeflective tubular structure of the PHA and elevated tissue structure of the optic nerve (Bergmeister’s papilla) (Fig. ). OCT angiography could not demonstrate the active blood flow of the PHA due to the technical limitation. However, we could find the blood flow in the Bergmeister’s papilla (Fig. ). The VH was spontaneously disappeared after 2 months. No serious disorders have been observed in the left eye during the follow-up period. |
pmc-6610948-1 | A 69-year-old man underwent radiofrequency catheter ablation for recurrent symptomatic premature ventricular contraction at a local hospital. The patient had no history of coronary artery disease. An echocardiogram showed no obvious cardiac lesions before ablation. The patient’s earliest activation site of premature ventricular contraction was located in the left ventricular inferior wall. Ablation was performed to the endocardial portion with 30 W (temperature limit, 43 °C). The patient developed shock the second day after ablation. A chest computed tomography scan showed pericardial effusion. Pericardiocentesis was performed, and the puncture fluid was a bloody pericardial effusion. The patient’s symptoms were relieved, but complained of recurring dizziness and chest pain. The patient was transferred to our hospital for further diagnosis and treatment. Transthoracic echocardiogram revealed an 9- × 4-cm giant pseudoaneurysm with a cystic structure in the left ventricular inferior wall near the mitral annulus along the left atrium, which was obviously compressed (Fig. ). The pseudoaneurysm was connected to the left ventricular cavity through a neck, and the lumen was filled with systolic and diastolic blood flow (Fig. ) (An additional movie file shows this in more detail, see Additional file ). In our hospital, the patient underwent three-dimensional transesophageal echocardiography. The pseudoaneurysm and the tract was clearly visible (Figs. and ), and the defect of the pseudoaneurysm was exactly at the point of radiofrequency energy delivery.
The pseudoaneurysm was surgically resected, and the defect was found in the inferior left ventricular wall near the posterior mitral leaflet. A bovine pericardial patch was placed on the neck of the pseudoaneurysm. The patient recovered well after the operation, and no premature ventricular contraction was observed. |
pmc-6610986-1 | We admitted a 38-year-old Kosovar Albanian man, an entrepreneur, to our emergency department in a critical state. He has never smoked and has no prior medical history or family history of illnesses. He reported enjoying an occasional alcoholic drink.
His symptoms had started after physical exercise 2 weeks before. He was then diagnosed at a local clinic with tonsillitis (lacunar angina) complicated with several retropharyngeal abscesses. He was prescribed first-line oral antibiotics; the treatment failed, and his clinical condition drastically worsened in the following days, which prompted the referral to our hospital.
On clinical examination, he was tachycardic with a heart rate of 130 beats/min and blood pressure of 100/70 mmHg, heavily somnolent with a Glasgow Coma Scale score of 10, and hypoxemic and cyanotic with a ratio of partial pressure of arterial oxygen to fraction of inspired oxygen (PaO2/FiO2) of 66 mmHg and a mean arterial pressure of 82 mmHg. His peripheral capillary oxygen saturation was 75% while wearing a 6-L O2 mask. We detected abnormal bronchial breath sounds and reduced airflow in the lower lobes.
Computed tomography (CT) indicated lymphadenopathy, inflammation, and fluid collection in the mediastinum accompanied by significant fibrin depositions (Fig. a, b). Abscesses were observed in the anterior and middle mediastinal regions (Fig. b, c) and the distal part of the neck, directly on the border with the left lobe of the thyroid gland (Fig. c, d). Other than weakness, the patient had no signs of any physical complications; moreover, neurological examinations did not reveal major abnormalities. The patient had slightly delayed motor responses and a normal pupil size and reaction to light, but his speech was incomprehensible.
The patient was admitted to the intensive care unit (ICU), where he was sedated (propofol, induction dose 2.0 mg/kg, maintenance dose 0.07–0.21 mg/kg/hr), intubated, and placed on mechanical ventilation with bilevel positive airway pressure FiO2 of 70%. We administered intravenous aminophylline 250 mg every 12 hr. As a gastroprotective therapy, we used ranitidine 50 mg every 8 hr. The continuous fluid therapy consisted of 200 ml/hr NaCl 0.9% and 50 ml/hr glucose 5% for the first 24 hr. We initiated parenteral nutrition using Aminoven 10% (40 ml/hr; Fresenius Kabi, Graz, Austria) and a fat emulsion (20 ml/hr; INTRALIPID® 20%; Fresenius Kabi).
We collected blood, urine, and tracheal aspirate samples for microbiological analysis (Additional file ). In anticipation of the definitive culture results, we administered broad-spectrum antibiotics (Fig. ). The patient was oliguric at this stage, with a urine output less than 50 ml in the first 2 hr in the ICU; furthermore, his creatinine (109.7 μmol/L), urea (10.6 mmol/L), and glomerular filtration rate (GFR) (69 ml/min/1.73 m2) pointed to mildly reduced kidney function (Fig. ).
On the basis of these findings, CRRT was indicated. We placed a 12-French double-lumen catheter in the right femoral vein to establish vascular access. Bearing in mind the likelihood of a systemic infection, we treated the patient with continuous venovenous hemodiafiltration with cytokine-adsorbing filters (CVVHDF-CAH) (PRISMAFLEX oXiris® filter; Baxter, Deerfield, IL, USA) set at a high hemofiltration rate of 35 ml/kg/hr. We exchanged the filters every 24 hr.
We next performed an urgent median sternotomy to remove the abscesses and purulent fluid. Tissue samples from the mediastinum and thymic area were collected for microbiological and histopathological analysis. We performed vacuum-assisted wound closure, and we replaced the drain every 48 hr.
The surgical intervention failed to revert his deteriorating condition. He became febrile (38.9 °C) and hemodynamically unstable (Table ). At this stage, his serum lactate was 6.3 mmol/L, and blood gas analysis (BGA) revealed acidosis. Because of his hemodynamic instability, we administered norepinephrine, but increasing the dosage from 0.2 mg/hr to 1 mg/hr over the next 48 hr was unsuccessful in reverting his poor systemic vascular resistance (FloTrac/Vigileo™ monitoring; Edwards Lifesciences, Irvine, CA, USA) of ≤ 700 dyn/s/cm5.
His clinical condition further deteriorated. He developed a high fever (40 °C) overnight, and his Simplified Acute Physiology Score II of 63 points suggested a poor prognosis.
Histopathological analysis revealed the presence of acute suppurative inflammation in the thymic and parathymic tissue (Fig. ), characterized by lymphocytic, neutrophilic infiltration and pus formation. Microbiological analysis of the wound samples revealed gram-positive cocci, identified as α-hemolytic Streptococcus; furthermore, the anaerobic cultures of the mediastinal tissue samples confirmed the growth of gram-positive bacilli of the Clostridium species.
We switched to a (rotating) antibiotic therapy according to the antibiogram or clinical observations. The detailed antibiotherapy timeline is shown in Fig. .
Collectively, the clinical manifestations and radiographic, biochemical (Fig. ), microbiological, and histopathological examinations indicated a severe case of anaerobic sepsis resulting from DNM [, ].
The patient remained in critical condition on day 3, with a fever of 40.1 °C, hemodynamic impairment, acidosis, and lactate level of 3.3 mmol/L (Table ). We commenced hyperalimentation to counter the patient’s low serum albumin level (14.6 g/L).
On day 4, the patient’s PaO2/FiO2 ratio was 114 mmHg. A transthoracic echocardiogram revealed pleural effusion in the right costophrenic space. Because of the patient’s worsening condition, we opted for extracorporeal blood oxygenation (EBOO) for 1 hr to improve the peripheral oxygenation of < 90%. EBOO was performed through the (same) right femoral vein catheter used for the CVVHDF-CAH. We coinfused low-dose O3, 10 μg/ml blood, and a bolus injection of 2 g of vitamin C to counter possible oxidative stress associated with hyperoxia. We also performed thoracentesis to remove 2800 ml of purulent fluid.
Our approach improved the patient’s clinical condition in the following 24 hr, gradually normalized his body temperature and biochemical parameters, and improved his BGA parameters; his PaO2 increased to 433 mmHg from an initial 80 mmHg (Table and Fig. ).
Consequently, we could decrease FiO2 to 50% while maintaining the PaO2 at 133.3 mmHg and O2 saturation at 98%. The sternum was closed on day 7, and the norepinephrine dosage was lowered to 0.06 mg/hr. The patient was extubated on day 11.
His recovery was short-lived. He once again became febrile on day 15. We observed a purulent secretion from the sternum that required an emergency median sternotomy, surgical debridement of necrotic tissue, and de novo vacuum-sealing drainage.
His overall clinical condition improved; nevertheless, he developed gastroesophageal bleeding on day 23. Via esophagogastroduodenoscopy, we identified a polypoid mass on the lateral wall of the esophagus measuring 10–15 mm in diameter. Pathological analysis of the biopsies confirmed squamous papilloma of the esophagus, inflammation, and ulceration.
The bleeding ceased upon the successful excision of the polypoid mass.
From day 24 onward, the patient’s condition steadily improved. He was kept in the ICU on continuous respiratory support and physical therapy until follow-up culture results were negative.
We performed the final vacuum sealing with definitive closure of the lesion on day 25 and extubation on day 27.
The patient was slightly dysphonic but otherwise in a decent health condition (Fig. ), and he was discharged on day 39. Control CT evaluation 5 days after discharge confirmed a complete regression of the abscesses (Fig. ).
The fully recovered patient presented for follow-up 1 year later in good mental and physical condition with no further complications. |
pmc-6611006-1 | The patient was a 35-year-old male presented to the ER with right craniofacial trauma due to an occupational injury caused by falling of a plastic pipe on his head. He was awake and aware at the time of arrival. The initial assessment revealed a GCS level of 14. Signs of traumatic injuries in the right side of his face included swollen eyelids due to trauma to the soft tissue of the right periorbital and frontal area along with right superior eyelid laceration, redness, and tenderness. The visual acuity in his right eye was almost no light perception. Right optic neuropathy was evident with a relative afferent pupillary defect (RAPD) of 4+. The eye movements of the right eye were restricted in all directions, but the other side was normal. Other examinations revealed no further abnormal findings.
CT-Scan depicted a small focus at the level of the right Sylvian fissure in favor of a pneumocephalus and a hyperdense structure in the right orbital cavity, posterior to the globe, suggestive of a bony material (Figs. , , and ). As we were not sure about the origin and material of hyperdense structure demonstrated on CT-Scan, use of MRI was waived in order to prevent subsequent complications in case of metal object foreign body. However, initial assessments of patient’s images amplified the suspicion of skull base fracture, regarding the foci of pneumocephalus, especially at the region of the right superior orbital wall. Based on these findings, the patient was admitted to the trauma section of the neurosurgery ward and received initial necessary supportive care. Antibiotic therapy was commenced for surgery preparation and preventing probable meningitis, with Cefepime and Vancomycin. A consult with ophthalmologists was performed, and regarding their evaluation, both ophthalmologists and neurosurgeons were agreeing with choosing a craniotomy approach. The procedure was performed by using the method of “extra-dural orbitocranial approach to the anterior cranial fossa” craniotomy. After a right brow skin incision with a soft tissue dissection with four burr holes trephination, a right frontal craniotomy was performed. Then, the roof of the orbital cavity was explored. No signs of fracture were detected in the superior orbital wall. To asses the orbital cavity, a supraorbital craniotomy was carried out, and the surgical field was extended to the right orbital cavity by removing the superior orbital wall, with a diameter of 1.5 × 2.5 cm. An exploration and dissection throughout the intraorbital muscles and structures were accomplished (Figs. , ). An intraorbital foreign body was successfully (a piece of plastic pipe) removed from the orbital cavity (Fig. ). Cranioplasty was carried out by titanium mesh and bone wax with wound closure as well (Fig. ). The patient was transferred to the neurosurgery ward to receive his post-op cares and subsequent follow-ups.
On microbiological culture of resected specimen colonies of Staphylococcus epidermidis were isolated which was sensitive to given antibiotics.
The patient was febrile, thus antibiotic therapy was continued with Cefepime and Vancomycin for 7 days based on the consult performed by an infectious disease specialist. In the patient’s initial evaluation after the operation, the overall condition was good, and his GCS level was 15. Eye-movements were normal on both sides. Regarding the patient’s visual loss and its effect on his quality of life in order to treat the consequences of traumatic optic neuropathy (TON) both ophthalmologists and neurosurgeons were agreeing with the administration of intravenous methylprednisolone (IVMP) with close monitoring of the patient. The patient received 250 mg methylprednisolone intravenously every 6 h for 3 days. Despite the foreign body removal and administration of high dose IVMP, patient’s visual acuity in his right eye was merely confined to light perception and did not improve, subsequently.
We presume that the patient’s vision loss occurred due to direct and high impact trauma to the optic nerve based on the location of the foreign body, which was embedded in the posterior part of the globe, just beside the optic nerve (arrow heads on Figure-3).
The patient was discharged from hospital 2 weeks after admission with a good general condition. |
pmc-6611010-1 | We are presenting the case of a 79-year-old Caucasian female with history of rheumatoid arthritis for 40 years and does not have prior history of established structural lung or cardiac disease. She is taking prednisolone, MTX, and hydroxychloroquine for more than 10 years. She presented to the emergency department with 2-day history of worsening dyspnea and palpitations. She was traveling across several states within the United States by a private motor vehicle; however, she did not travel to any high-altitude region. She also did not have any sick contacts. Past medical and family histories were noncontributory. In the emergency department, she was tachypneic, tachycardic, and her oxygen saturations were in the low 80s% in ambient air.
Physical examination revealed bi-basilar coarse crackles, decreased breath sounds over the right hemi-thorax, and hyper-resonant percussion note. An emergent chest X-ray (CXR) showed massive right-sided tension pneumothorax with collapsed right lung (). A right-sided 8-French surgical chest tube was inserted. A confirmatory computed tomography (CT) of the chest showed well-positioned chest tube, right pneumothorax, and no pulmonary nodules (). Initial laboratory workup showed neutrophilic leukocytosis (28 000, 95% neutrophils), arterial blood gas: 7.52/31/58 on 2 L of oxygen. Complete metabolic panel, procalcitonin, and 3 sets of troponin I were all unremarkable. Blood, urine, sputum, and fungal cultures were negative. Transthoracic echocardiogram showed pulmonary hypertension with right ventricular pressure of 50 to 60 mm Hg.
On admission to general medical floor, the patient had good oxygen saturations on 2 L of nasal cannula, and a repeated CXR showed 90% resolution of the pneumothorax. On day 2, the chest tube was removed due to slippage into the subcutaneous layer and the formation of subcutaneous emphysema (), and pneumothorax was confirmed to be mostly resolved. On the next day, however, a repeat CXR showed re-formation of right sided pneumothorax (occupying 40% of the right hemithorax), for which a 10-French pig tail chest tube was inserted to the right pleural space.
The chest tube under-water air leak persisted for 5 days following the insertion of the pig tail chest tube and the presence of BPF was suspected; therefore, an explorative thoracotomy was considered. A standard posterolateral thoracotomy and pleuritic exploration showed the presence of BPF at the apical segment of the right lower lobe (). The BPF was sutured, covered with pleural patch, and anterior and posterior chest tubes were also inserted to the right pleural space intraoperatively. The pathological examination of right lower lobe wedge biopsy revealed fibrosing pleuritis with acute and chronic histiocytic inflammation and subpleural septal fibrosis (). Postoperatively, the patient continued to improve, with remarkable decrease in air leak and chest tube aspirate and the chest tubes were removed in about day 10 postoperatively. |
pmc-6611056-1 | A 37-year-old man presented to the thoracic surgery department with a persistent consolidative lesion in the right lower lobe. He was a 14-pack-year smoker but denied a history of recent trauma or significant disease. A chest computed tomography scan revealed a 2 cm focal consolidative lesion with bronchiectasis in the subpleural region of the lateral basal segment of the right lower lobe. The patient had been under observation for approximately four months in the outpatient pulmonology department. The size of lesion had slightly increased over this time (Fig ). No lymphadenopathy or other mass lesions were identified. The patient underwent single port video-assisted thoracoscopic surgery. Wedge resection of the lesion was performed and showed an ill-defined fungating mass with focal cystic change, measuring 7.5 x 6.0 x 1.5 cm and weighing 410 g. Gross and microscopic features of the lesion are shown in Figure . These findings were consistent with the characteristics of PTL. The patient had an uneventful clinical course and was discharged without complication. |
pmc-6611083-1 | An 84-year-old male patient evaluated at the emergency department with low back pain, fever and malaise. The patient had past history of endovascular repair of an AAA, 8cm in diameter, with development of a type II endoleak. The abdominal computed tomography (CT) showed a 7cm diameter aneurysm sac, a long-standing hematoma in the retroperitoneum, and presence of peri-prosthetic fluid collection with gas (). Laboratory screening showed leukocytosis and increased inflammatory markers. Due to his high surgical risk, the patient was subjected to ultrasound-guided drainage with an 8.5F pigtail catheter, and 30mL of purulent material was drained. The post-procedure CT scan showed marked reduction in the collection volume ().
Cultures for aerobic and anaerobic microorganisms, and fungi, were negative. The patient had clinical and laboratory improvement, and was discharged in good general condition. Nine months of control CT scans did not show any new peri-prosthetic fluid collections. |
pmc-6611083-2 | A 68-years old male patient with chronic ruptured infrarenal abdominal aortic aneurysm treated endovascularly 6 years before, currently followed-up with CT angiography of the aorta. The patient had history of coronary artery bypass grafting (CABG), and was assessed at the emergency department with a history of fever and intense low back pain. The abdominal CT showed an enlarged aneurysm sac with heterogeneous peri-aortic infiltration, infiltration of the left psoas muscle, and reactive, enlarged retroperitoneal lymph nodes (). Embolization of the right internal iliac artery was performed, and a new graft was implanted through the common iliac artery, with the distal end in the external iliac artery.
Two weeks later, the patient developed hemodynamic instability, and a new abdominal CT showed increased periaortic fluid collection. The patient underwent CT-guided drainage, with placement of a 12F pigtail catheter (). The patient evolved with clinical improvement and reduction in the abscess volume, from 170mL to 35mL. Cultures were positive for Salmonella spp., and the patient was treated with vancomycin, based on the antimicrobial susceptibility testing.
Approximately 75 days later, the patient came back to the emergency department with fever and hemodynamic instability. The abdominal CT showed the abscess had increased in volume, from 35mL to 100mL. A new drainage was performed, with placement of a 12F pigtail catheter, with clinical and laboratory improvement. The patient was discharged with no further symptoms. After 18-month follow-up, no collections were found. |
pmc-6611083-3 | A 92-year-old male patient, evaluated at the emergency department with tachycardia and hypotension. The patient had a history of endovascular repair of AAA. The abdominal CT scan showed densification of fat planes adjacent to the right common iliac aneurysm sac, small fluid collections in the right psoas muscle and close to the distal segment of the iliac endograft. ()
The patient was not a proper candidate for open surgical repair and was subjected to CT-guided percutaneous drainage with aspiration of 15mL of purulent secretion and placement of a 10F pigtail catheter (). Cultures showed growth of Escherichia coli, and the patient received antibiotic therapy with meropenen, based on the antimicrobial susceptibility testing. Clinical and laboratory parameters improved, and he was discharged with no symptoms after 15 days. After 6-month follow-up, the images showed no recurrence of the collection. |
pmc-6611084-1 | A female, 18-year-old, Caucasian patient, born and residing in São Paulo. The patient has AD since she was 2 years old, with aggravation for the past 4 years, on 250mg cyclosporine (150mg in the morning and 100mg in the afternoon: 3.2mg/kg/day), 5mg desloratadine (morning), and 25mg hydroxyzine (evening), moisturizing lotion twice-daily, and clobetasol propionate cream twice-daily. History of allergic rhinitis, hypothyroidism and metabolic syndrome, on levothyroxine sodium 50mg/day and metformin hydrochloride 500mg/day.
The patient referred previous hospitalization for infection secondary to the skin lesions in late 2015, and five other subsequent episodes, treated in the outpatient setting. In November 2017, she was started on agomelatine 25mg/day and buspirone 5mg/day, due to depression and insomnia.
The disease remained poorly controlled, and the patient had very poor quality of life. After being on cyclosporine for more than 2 years uninterruptedly, without achieving proper control of the disease, the patient was started on mycophenolate mofetil, 1g every 12 hours, in the end of 2017. Cyclosporine was gradually tapered, and eventually discontinued. After switching medications, there were some side effects such as 12kg weight loss over 2 months, menstrual changes and telogen effluvium, but the regimen was maintained. However, with no clinical improvement.
The skin exam showed extensive eczema affecting 90% of the skin tissue, along with very intense pruritus and dry skin, and a Score for Atopic Dermatitis (SCORAD) of 45. The SCORAD is a tool to assess the severity of AD using a signs and symptoms scale ranging from zero (no lesions and symptoms) to 103 (maximum score). Over 40, AD is considered severe.(
)
After the dupilumab results were published and the monoclonal antibody was approved in different countries, including Brazil, it was indicated for this patient.(
) In March 2018, the patient received her first dose of 600mg dupilumab, subcutaneously. The loading dose was followed by 300mg every other week. Mycophenolate was discontinued one month after the biological agent was introduced. In the fifth dose, the patient was already showing considerable improvement. She is currently on 25mg hydroxyzine every evening. Desloratadine, the topical corticosteroid and the antidepressants have been discontinued. The skin exam showed major improvements, as well as dry skin and pruritus. The current SCORAD is 16. A to C (pre-treatment) and A to C (after three applications) show the level of improvement achieved between the first and third doses. |
pmc-6611129-1 | A 66-year-old male patient, with a history of smoking (30 pack-years) and no known medical or surgical history, was admitted in our department for a spontaneously resolved inferior ST elevation myocardial infarction (STEMI). The intra-hospital treatment included enoxaparin 0.6 ml twice a day, clopidogrel 75 mg once a day, aspirin 100 mg once a day, bisoprolol 2.5 mg once a day and atorvastatin 40 mg once a day. The coronary angiogram (performed at day 3 through the right radial artery) showed a severe thrombotic lesion of the distal circumflex. The patient underwent an ad-hoc successful angioplasty of the circumflex with a drug eluting (everolimus) stent. Initial laboratory tests at admission were normal except elevated troponin. Echocardiography showed a 65% left ventricular ejection fraction. The patient was discharged after 5 days of anticoagulation by low molecular weight heparin (enoxaparin). Laboratory tests were not controlled during the hospitalization. The discharge treatment included clopidogrel 75 mg once a day, aspirin 100 mg once a day, bisoprolol 2.5 mg once a day and atorvastatin 40 mg once a day.
One week later, the patient was referred again to our department for both chest and right lower limb pain. The electrocardiogram showed an inferior STEMI and the physical exam of the right lower limb found ischemic signs with absence of the femoral pulse. There was no history of aspirin or clopidogrel discontinuation. An urgent coronary angiogram (performed through the left femoral artery) showed total thrombosis of the circumflex stent (
). The patient underwent a successful primary angioplasty of the circumflex by simple balloon (
). Urgent lower limb contrast-enhanced computed tomography was performed immediately after the angioplasty, revealing total acute thrombosis of the right common femoral artery (
). The patient underwent an urgent successful thrombectomy with Fogarty catheter. Immediate evolution was favorable with total regression of coronary and right lower limb ischemic signs. Laboratory tests showed a marked fall in the platelet count (68,000/L) which was normal (364,000/L) in the previous hospitalization. A diagnosis of concomitant coronary stent and femoral artery thrombosis due to HIT was strongly suspected (4T score = 8). Our therapeutic strategy was immediate discontinuation of low molecular weight heparin (enoxaparin), aspirin and clopidogrel with strict daily control of platelet count. During this period, no alternative anticoagulation was initiated because of the unavailability of direct thrombin inhibitors in our center. Anticoagulation with a vitamin K antagonist (acenocoumarol 4 mg once a day) and dual antiplatelet therapy with aspirin 100 mg once a day and clopidogrel 75 mg once a day were initiated at day 3 once platelet count had recovered. The in-hospital outcome was favorable and the patient was discharged after 15 days on acenocoumarol 4 mg once a day, aspirin 100 mg once a day and clopidogrel 75 mg once a day. The 3-month follow-up, with controlled blood tests and lower limb contrast-enhanced computed tomography showing total reperfusion of the right femoral artery (
), was unremarkable. |
pmc-6611505-1 | A 70-year-old Asian female underwent a wide local excision and sentinel lymph node biopsy for a Stage 1A (T1c N0 M0), grade two, triple-negative invasive apocrine tumour of the breast with focal lymphovascular invasion and a Ki67 of 15% overall and 30% in hotspots.
Relevant comorbidities included well-controlled, non-insulin-dependent, type two diabetes mellitus; hypertension; and dyslipidaemia. She was a lifelong non-smoker.
She was commenced on adjuvant dose-dense AC (ddAC) regimen , consisting of a single dose of Akynzeo® (fixed-dose netupitant and palonosetron combination), dexamethasone 16 mg, doxorubicin 60 mg/m2, and cyclophosphamide 600 mg/m2 on Day 1, with pegfilgrastim on Day 2. Dexamethasone was continued at 8 mg on Days 2–3 and 4 mg on Days 4–5. The cycle was repeated every 14 days. She completed the first three cycles of treatment without incident. The day prior to her fourth cycle, she reported cough and low-grade fever. She was commenced on a course of oral amoxicillin after a review by her general practitioner. Routine blood tests performed on that day were unremarkable, apart from lymphopenia (lymphocytes 0.26 × 109/L, reference range 1.0–4.0 × 109/L). She received her fourth cycle as scheduled. Eight days later, she reported severe fatigue, peripheral oedema, generalized weakness, and a cough and was admitted to hospital for investigation.
On examination, she was hypoxic, with SpO2 by pulse oximetry of 88–90% on room air. Her other vital signs and physical examination were non-contributory. Baseline bloodwork demonstrated a raised C-reactive protein (CRP) of 151 mg/L (reference range, 0.0–5.0 mg/L) and lymphopenia (lymphocytes 0.31 × 109/L; reference range, 1.0–4.0 × 109/L). Chest X-ray showed non-specific patchy consolidation in the left lung and the right lung base (Fig. ).
Despite multiple antimicrobial agents for presumed community-acquired pneumonia, her respiratory function continued to decline, with repeat chest X-ray 36 h later showing extensive opacification in both lungs (Fig. ). Over the subsequent days, she remained lymphopenic, with a nadir lymphocyte count of 0.29 × 109/L. Simultaneously, progressive neutrophilia with left shift and rising CRP, consistent with toxic changes, were seen. On the fourth day of her admission, she was intubated for hypoxic respiratory failure. Until this point, extensive microbiology work-up—including sputum and blood cultures, atypical serologies, respiratory virus polymerase chain reaction (PCR), and urinary Pneumococcal antigen—and an echocardiogram were all non-contributory. Serum (1➔3)-β-D-glucan was not available at our institution. A bronchoscopy was performed shortly after intubation, which was macroscopically normal. In consideration of the possibility of PJP, empirical high-dose oral trimethoprim-sulfamethoxazole was commenced after bronchoscopy.
Nine days following her bronchoscopy, P. jirovecii was detected by PCR on her bronchial washings. The long turnaround time for this result was because of a 4-day laboratory closure over the Christmas holidays. Treatment was subsequently escalated to intravenous high-dose trimethoprim-sulfamethoxazole and methylprednisolone. Her condition continued to deteriorate, and she developed multi-organ failure. Despite multiple life support measures, her condition failed to improve, and she eventually succumbed to the illness in the intensive care unit. Human immunodeficiency virus (HIV) testing was never performed in this patient as it was felt she did not have the risk factors for it. |
pmc-6611516-1 | The patient was a non-smoking 85-year-old Japanese man. He underwent lobectomy of the left upper lung and was diagnosed with lung adenocarcinoma (pT2N1M0 stage IIA) in October 2014. His lung cancer recurred in the left lower lobe, second lumbar bone, and left pleura in June 2016. A pleural fluid specimen was obtained and examined for the presence of genetic alterations using a next-generation sequencer (NGS) and identified METex14 skipping mutations. Although the patient received four cycles of combination chemotherapy with carboplatin and pemetrexed (PEM), followed by one cycle of PEM as maintenance chemotherapy, in addition to pleurodesis with talc for malignant pleural effusion, computed tomography (CT) scan demonstrated progressive disease (PD) due to an increase in the number of multiple nodules in the lower left lobe and a left-sided pleural effusion (Fig. ). A CT scan also showed the presence of multiple nodules just below the left lower lobe and multiple bone metastases. Moreover, the patient had abnormal laboratory findings (haemoglobin: 9.2 g/dL, total protein: 6.8 g/dL, albumin: 3.9 g/dL, alkaline phosphatase: 322 U/L, blood urea nitrogen: 25 mg/dL, creatinine: 1.4 mg/dL, C-reactive protein: 0.25 mg/dL, carcinoembryonic antigen: 13.5 ng/mL, and sialyl Lewis X: 120 U/mL).
He was excluded from the clinical trials administering MET inhibitors because of a coexisting stable prostate cancer. As regulatory agencies did not approve the use of crizotinib in NSCLC with MET mutations, crizotinib (500 mg/day) was administered as secondary chemotherapy without health insurance coverage after receiving the patient's consent on 27 April 2017 and after obtaining approval from the ethics committee of our hospital. On 4 May, the patient experienced impairment in taste perception (grade 2), anorexia (grade 2), and photophobia (grade 1). However, these adverse events eventually resolved without any specific treatments. The dosage of crizotinib was reduced from 500 to 400 mg/day on 25 May due to the occurrence of renal dysfunction (almost grade 3). On 31 July, the target lesion was slightly reduced as shown on the patient's CT scan, and the patient's disease remained stable. On 20 November, the target lesion was slightly increased in size as shown on the patient's CT scan, but there was no evidence of disease progression. Although his disease was controlled with crizotinib for 7 months, the treatment was discontinued as the patient developed anorexia and experienced financial difficulties (Fig. ). Because the patient did not recover, he could not receive tertiary chemotherapy, and he therefore received best supportive care. He was transferred to the palliative care unit on 31 May 2018 and died on 16 June. Overall survival from the initiation of crizotinib was 13.5 months. |
pmc-6611530-1 | A 5-year-old boy reported to the Department of Pediatric Dentistry at Panineeya Institute of Dental Sciences, and parents give a history of multiple small swellings in child's mouth since 3 months which occurred during high fever and gradually increased in size even after fever subsides. The medical history, dental history, and family history were noncontributory. The extraoral examination did not reveal any specific finding, but intraoral examination did reveal the presence of soft, sessile papules varying 2–10 cm in dimension, two papules present on the right and left the side of the lower lip region, and one on the left ventral aspect of the tongue (). Based on history, clinical features and nature of lesion, a provisional diagnosis of focal epithelial hyperplasia was considered and differential diagnosis of squamous cell papilloma, condylomataacuminata, mucocele, focal dermal hypoplasia (Goltz–Gorlin syndrome) were considered. The patient was subjected to complete the hematological examination before the procedure and all the parameters were within normal limits. All the three lesions were excised under local anesthesia using diode soft tissue laser of 810 nm of 3–3.5W power for 3–60 seconds intermittently and specimens were sent for histopathology and polymerase chain reaction (PCR) evaluation (). The histopathology reports revealed the presence of benign parakeratotic hyperplastic mucosa with marked papillomatosis and acanthosis, and some of the cells showed isolated perinuclear vacuolization and the presence of occasional mitosoid cells. There was no evidence of dysplasia. These features were suggestive of squamous epithelial hyperplasia without atypical features of Heck's disease and further PCR was performed to confirm the subtype of virus associated with infection. Thus, this revealed the presence of HPV subtype 32 as etiology of this condition. Based on histopathology and PCR analyses, a final diagnosis as Heck's disease (focal epithelial hyperplasia) was established. |
pmc-6611532-1 | A 27-year-old male patient reported to the Department of Conservative Dentistry and Endodontics, SGT Dental College, with a chief complaint of severe pain in the right lower back tooth region since last three days. The pain was intermittent in nature and aggravated on taking hot food and beverages, and lasted for 2–3 hours.
On clinical evaluation, it was seen that there was secondary caries associated with restored right mandibular first molar. A diagnostic radiograph of mandibular first molar showed restoration close to pulp and presence of an additional root (). Another radiograph was taken at 300 mesial and distal angulation to confirm the same. Access cavity preparation was done under local anesthesia with an endo access bur (Dentsply, Switzerland). The first distal canal was located towards the buccal side indicating the presence of one additional canal on the lingual side.
The shape of the access cavity was modified from triangular to a trapezoidal form to locate the fourth canal. DG-16 endodontic explorer was used to locate the root canal orifices and 15 # K-file (Mani, Japan) was used to establish patency of the canals. Working length was determined using apex locator (Root ZX, J. Morita) and reconfirmed radiographically. Biomechanical preparation was done with rotary ProTaper Next (Dentsply, Switzerland) file system. During instrumentation, 1.3% sodium hypochlorite was used as an irrigant and 17% EDTA was used as final flush.
Obturation was performed with gutta-percha points using cold lateral condensation technique (). Restoration of access cavity was done with composite resin (tetric-N-ceram, ivoclar vivadent) and a post-obturation radiograph was taken. At 6-month follow-up, the patient was asymptomatic and radiographic evaluation showed no evidence of pathology. |
pmc-6611532-2 | A 22-year-old female patient reported to our Department with a history of pain in the lower right back tooth for 1 month. On clinical examination, right mandibular first molar was found to be carious and the diagnosis was established as irreversible pulpitis. Radiographic interpretation revealed the presence of the third root but not associated with any periapical changes ().
Root canal procedure of the tooth was planned and patient consent obtained. Local anesthesia was administered and after access cavity preparation canal orifices were negotiated with DG-16 explorer. The fourth distolingual canal orifice was negotiated more lingually, away from the rest of the three orifices. The canal lengths were determined radiographically with #10 K file, and cleaning and shaping of the root canal system were done with rotary ProTaper file system with apical preparation till F2. 1.3% sodium hypochlorite was used for disinfection of the pulp space and 17% EDTA was used as a final irrigant. After biomechanical preparation, temporary restoration was done and the patient was recalled after 4 days.
At subsequent appointment patient was asymptomatic. Master cone radiograph was taken, canals were dried with absorbent paper points, and obturation was done with gutta-percha cones using AH Plus sealer (Dentsply De Trey, Germany) (). |
pmc-6611535-1 | A 12-year-old male patient reported to the department of pediatric and preventive dentistry, Rajas Dental College and Hospital with a chief complaint of unesthetic appearance. Intraoral examination revealed a cone-shaped supernumerary tooth present in the place of left maxillary central incisor (). The left maxillary central incisor was clinically absent. The supernumerary tooth was caries free, responded to thermal stimuli (heated gutta-percha) and electrical pulp testing. Periodontal probing revealed healthy gingiva and no abnormal mobility was noted. IOPA () in relation to the supernumerary tooth revealed impacted permanent left maxillary central incisor above the root of the supernumerary tooth. An additional supernumerary tooth was found impacted above the roots of the permanent right maxillary central incisor. No pathosis was seen with the impacted teeth, and no radicular changes were noted.
Since the permanent left maxillary central incisor was above the root of the supernumerary tooth, it was decided to retain the supernumerary tooth and surgically remove the left maxillary central incisor and the additional supernumerary tooth. The permanent left maxillary central incisor and the additional supernumerary tooth were removed surgically, and sutures were placed ( to ). Root canal treatment was performed in the supernumerary tooth (). The crown preparation was done and Porcelain-fused-to-metal crowns (PFM's) crown luted (). |
pmc-6611542-1 | An 8-year-old girl reported to pedodontic clinics with mild pyrexia, lethargicness lower lip swelling, and a sore tongue. The lymph nodes were significantly enlarged. On oral examination, lips were found to be dry, cracked, red, and localized swelling was seen of the lower lip (). This swelling was accompanied by itching and subsided on its own. This painless swelling of lower lip has very rarely been reported in the oral manifestation of this disease, thus making this case report a novel presentation of Kawasaki disease. The patient first reported lower lip swelling and after few days strawberry tongue was seen. Her past medical history revealed that the child had developed Kawasaki disease at the age of 4 years for which she was hospitalized for uncontrolled fever.
The oral examination also showed the presence of a red bright erythematous tongue(strawberry tongue) with tongue tie (), dry erythematous fissured lips that bled easily, along with prognathic maxilla and protruded maxillary teeth (). Differential diagnosis of recurrent Kawasaki disease and scarlet fever was made because of the presence of strawberry tongue. As the patient was not having a history of sore throat or tonsillar exudate, the chances of scarlet fever were minimal. No oral exudates, ulcerations, or Koplik's spots were reported. The family history did not reveal any similar complaints in the family. The child was diagnosed with Kawasaki disease at 4 years of age. Lateral cephalometric radiograph of the nasopharynx showed enlargement of adenoid gland causing narrowing of the air passage ().
The patient was referred by us to a pediatrician for further checkups as there was suspicion of recurrence of Kawasaki disease. Investigations showed total white blood cells (WBC) count of 15,600/mm3 with 70% neutrophils, increased ESR (60 mm at the end of 1 hour) and serum C-reactive protein (96 mg/L) and low platelet count (80,000/mm3). Urine examination and culture study showed sterile pyuria. Tuberculosis test was negative. Electrocardiogram (ECG) reports showed cardiac changes. X-ray of the chest showed an enlarged heart and cardiac involvement ().
Generalized lymphadenopathy along with desquamation of the fingertips of the girl was seen within 2 days of reporting. Based on the investigations report and clinical picture, a diagnosis of recurrent Kawasaki disease was made by the pediatrician. She was treated with a single dose of 2 g/kg of intravenous immunoglobulin, and regular daily aspirin was adviced. The discharge was given after 3 days and the patient was free of the oral signs and symptoms within a month. |
pmc-6611545-1 | A 9-year-old female patient born to nonconsanguineous parents reported to the Department of Orthodontics and Dentofacial Orthopaedics, Subharti Dental College, Meerut, with a chief complaint of irregularly placed upper and lower front teeth. On extraoral examination (), she was having a straight profile, unesthetic smile with a prominent maxillary canine on the right side. Intraoral examination () revealed that she was in the late mixed dentition stage with Angle's class I molar relation on both sides. Maxillary midline was shifted to the right by 1.5 mm with respect to facial midline, and mandibular midline was shifted to left by 1 mm. All deciduous second molars (E's) were present. Maxillary right lateral incisor was in crossbite. Her radiological examination () revealed that all permanent second premolars with 2/3rd of their roots completed were developing in between roots of deciduous second molars. Orthodontic records were prepared. Her model analysis revealed 6.5 mm and 7 mm tooth material excess in upper and lower arches, respectively. Based on her dental age and model and cephalometric analysis, it was decided to use E-space for correction of crowding in anterior teeth and guided eruption of permanent second premolars. Nance's palatal arch and lingual holding arch were constructed and cemented in upper and lower arches, respectively (). Arch alignment was started with partial bonding in both arches. E's were sequentially reduced at the rate of 1 mm/month from the mesial side for distalization of first premolars and canines with tie backs in all four quadrants (). Alignment was improved significantly in both arches till the time of shedding of Es (). We were able to align both the arches completely without any significant proclination of incisors. Molars were maintained in class I relationship, and canines were brought into class I relationship (). There was perfect alignment as seen in occlusal photographs (). Profile of the patient was satisfactory after treatment (). |
pmc-6611545-2 | An 11-year-old male patient reported to the Department of Orthodontics and Dentofacial Orthopedics, Subharti Dental College, Meerut, with a chief complaint of irregularly placed upper and lower front teeth. On examination extra orally (), the patient had a straight profile and competent lips. Intraoral examination () revealed that he was in the late mixed dentition stage with Angle's class I molar relation on both sides. His mandibular midline was shifted to the right by 2.5 mm. Both maxillary right and left lateral incisors were in crossbite. The permanent upper left canine was missing. Deciduous upper left and lower right and left molars were present. His radiological examination () revealed that upper left and lower both right and left permanent second premolars with 2/3rd of roots completed were developing in between roots of deciduous second molars. Orthodontic records were prepared. His model analysis revealed 4 mm and 2 mm tooth material excess in upper and lower arches respectively. As done previously, based on his dental age and model and cephalometric analysis, it was decided to use E-space for correction of crowding in anterior teeth and guided eruption of permanent second premolars. Nance's palatal arch and lingual holding arch were made and cemented in upper and lower arches respectively (). Partial bonding was done, and arch alignment was initiated. E's space was maintained using Nance's palatal arch and lingual holding arch as during treatment the deciduous molars got exfoliated. Canines were tied back in all four quadrants. Alignment was improved significantly in both arches. We were able to align both the arches completely without any significant proclination of incisors. Molars were maintained in class I relationship, and canines were brought into class I relationship (). There was perfect alignment as seen in occlusal photographs (). Profile of the patient was satisfactory after treatment (). |
pmc-6611720-1 | A 2-year-old girl presented by abnormal passage of stools through her vestibule. She had a history of operation (anorectoplasty) during the neonatal period. The patient operative files revealed a limited sagittal anorectoplasty performed (without colostomy) for a rectoperineal fistula, with uneventful postoperative recovery.
The history would suggest an iatrogenic rectovaginal fistula, and, therefore, the patient was scheduled for examination under anesthesia with the possibility of performing a diverting colostomy. Examination under anesthesia revealed a well-positioned neoanus with good caliber; however, the vestibule showed a shallow common urogenital sinus with a septated vagina. The common sinus was short and wide that the anatomy was well-exposed for inspection upon labial retraction. Stools were seen emerging out of the vagina upon applying pressure on the lower abdomen. A decision was taken for fecal diversion as a first step for a staged repair. An oblique left lower abdominal incision was made to perform a pelvic (sigmoid) colostomy which revealed the presence of double colon sharing a common mesentery. A pelvic colostomy was done for the double colon that consisted of two proximal stomas and two distal mucous fistulae.
Diagnostic workup before definitive repair:
we performed contrast X-ray studies (
) and pelvic magnetic resonance imaging (MRI;
and
). Injection of contrast through the proximal stomas (each at a time) revealed complete colonic duplication up to a double cecum (with failure of contrast to pass into neither the ileum nor the appendix). Injection of contrast through the distal mucous fistulae revealed one posterior colon and rectum (R1) terminating by the neoanus, and another anterior colon and rectum (R2) terminating via a rectovaginal fistula (
). MRI studies revealed no associated spinal anomalies; and helped to complete the picture by defining the three-dimensional (3D) orientation of the double colon and rectum in relation to other pelvic soft tissue structures (
and
).
The sacral ratio was calculated in the antero–posterior X-ray film of the sacrum; its value was 0.53, which indicated a fair prognosis regarding the continence potential.
Operative management:
our surgical plan was to remove the distal colon and rectum (R2) communicating with the vagina, while keeping the posterior distal colon and rectum (R1) as a single exit for fecal stream after reanastomoses with proximal double colon (
).
As expected, separation of one colon from the other was unfeasible due to their common mesentery, so we went for just mucosal excision of the “antimesenteric” distal colon and rectum (R2). The distal colon to be excised was incised along its free antimesenteric border (
), followed by submucosal injection of adrenaline/saline solution (1/200,000) to control bleeding and elevate the mucosa (hydrodissection;
). Stripping the mucosa off this distal colon and rectum was then applied down into the pelvis; the stripped mucosa was transfixed and excised at its lower end communicating into the vagina (
). This was followed by seromuscular closure. The continuity of the fecal stream was then restored by anastomosing the proximal double colonic lumens with the retained distal colon and rectum in a
Y
-shape fashion (
and
). Before that, a 7 cm linear cutting stapler was applied to the distal end of the common wall between the proximal double colonic lumens (limited septotomy), converting it into a single wide lumen that could be anastomosed to the single distal colon (
). Postoperative recovery was uneventful, and the patient had normal defecation through a single neoanus. Few months later, the patient underwent partial urogenital sinus mobilization with division of the vaginal septum as a final step in the staged reconstruction. Partial urogenital sinus mobilization (PUM) is a procedure used to correct short common urogenital sinus anomalies which comprises dissection of the common urogenital sinus up to but without cutting through the pubourethral ligament.
The patient has been followed-up for the last 3 years, with a satisfactory functional and cosmetic outcome. She has voluntary fecal and urinary control; occasional constipation showed good response to stimulant laxatives. A follow-up MRI has been prescribed to check for postreconstruction pelvic anatomy (
and
). |
pmc-6611721-1 | A 14-year-old girl presented with aortocoronary fistula, which caused a decreased coronary flow during diastole. Her clinical condition worsened during exercise. Right three-port (3 mm) thoracoscopy was performed in upright positions. The fistulous tract was readily identified and dissected on arrival at the right atrium. The presence of this rare vascular anomaly was confirmed by fluorescence by immediate injection of ICG (dose of 0.2 mg/kg), allowing better visualization and secure ligature (
). |
pmc-6611721-2 | A 13-year-old boy was scheduled for varicocelectomy. He had a clinical history of asymmetry and testicular pain. Umbilical, and right and left flank trocars (5 mm) were introduced for lens and instruments, respectively.
After intravenous (IV) injection of ICG, the arterial vessels were initially visualized following by the venous vessels. Thereafter, ligation of the spermatic cord was performed in block, ensuring selection of all vessels and avoiding the section of lymphatics that are not filled in this phase (
). |
pmc-6611721-3 | A 13-year-old girl was admitted because of cholelithiasis and recurrent abdominal pain. She required two previous hospital admissions. Laparoscopic cholecystectomy was scheduled few days after admission. Fifteen minutes after ICG IV injection, the biliary tree was perfectly drawn, allowing clear identification of cystic artery, common bile duct, and hepatic duct. Safe dissection of the bile duct and artery was performed, completing cholecystectomy with total control of all surgical maneuvers (
). |
pmc-6611857-1 | A 56-year-old male was referred for evaluation of an intra-pelvic lipomatous tumour discovered incidentally on CT colonoscopy, performed for a recent weight loss of 10 kg. Unfortunately, no histology was available for this patient; however, radiological features are in keeping with a lipomatous lesion. Open mesh repair of the hernia was performed (Fig. ). |
pmc-6611857-2 | A 60-year-old female presented with an enlarging lump in the epigastrium, which had doubled in size over a period of 6 months. Consequently, she was referred to our centre by her general practitioner (GP). Morphologic histopathology findings were in keeping with an ALT (demonstrated by oedema and fibrosis present within the lesion) (Fig. ). |
pmc-6611857-3 | A 61-year-old lady presented with weight loss and abdominal pain. Cross-sectional imaging revealed a fatty mass in the left lower quadrant extending caudally to the level of the pubic symphysis, with internal septations and haziness within the fat, suggestive of an ALT radiologically. Histopathology findings did not confirm atypia or signs of malignancy (Fig. ). |
pmc-6611857-4 | A 63-year-old male presented with recurrent burping and dysphagia without an obvious cause. As part of his work up, a CT of the abdomen was performed which picked up a large intra-abdominal lipomatous lesion as an incidental finding. Excision was performed and histopathology findings were in keeping with a benign lipoma. No MDM2 or CDK4 amplifications were present (Fig. ). |
pmc-6611857-5 | A 53-year-old gentleman complained of a swelling over the left flank which appeared to be growing slowly over the period of a year. MRI confirmed the swelling to be an interparietal hernia secondary to a fatty tumour. Histology revealed this to be a benign lipomatous tumour (Fig. ). |
pmc-6611857-6 | A 58-year-old woman who had an incidental finding of a lipomatous mass on an MRI of the rectum which was performed for unrelated reasons, presented to our clinic with newly developed right-sided sciatica. Clinically, she had a palpable fullness in the right gluteal region.
On MRI, a herniating fatty tumour is seen extending through the greater and lesser sciatic foramina (Fig. ). |
pmc-6611857-7 | A 38-year-old lady presented to her GP with a history of intermenstrual bleeding. A pelvic ultrasound performed as part of the workup showed a mass, suspicious for an ovarian tumour. A pelvic MRI was then performed which revealed a large left ischiorectal fossa mass which extended into the perineum. Excision and histological examination followed which confirmed the presence of a lipomatous lesion with no MDM2 or CDK4 amplification (Fig. ). |
pmc-6611857-8 | A CT performed for increasing right groin pain in an 82-year-old gentleman picked up a large incidental lipomatous lesion in the right iliopsoas compartment. This was characterised on a dedicated MRI. Marginal excision was performed and cytogenetic analysis confirmed MDM2 amplification in keeping with ALT (Fig. ). |
pmc-6611857-9 | A 72-year-old lady had complaints of right groin and buttock pain since 3 months. She had no associated weight loss. This lesion proved to be an undifferentiated high-grade pleomorphic sarcoma (Fig. ). |
pmc-6611857-10 | A 49-year-old woman only recently noticed a lump in her right groin and anterior thigh and attributed this to a recent weight loss (2.5 stone) making the swelling more obvious (Fig. ). |
pmc-6611857-11 | A 16-year-old boy noticed an increase in weight and a significant enlargement of his left thigh which on clinical examination was 20 cm bigger in diameter than the right side. There was no associated pain and as he was a keen rugby player, he initially attributed these changes to a sports injury. When he finally presented to his GP, the mass in his left thigh was significantly large and he was referred for urgent imaging investigations. On MR imaging, the tumour contained small areas of fat intensity and was mostly hyperintense on fluid-sensitive fat saturated sequences, which pointed towards a (myxoid) liposarcoma. However, this case nicely illustrates that plain films are key to assess the presence of matrix calcifications in soft tissue tumours. After resection, histology was in keeping with a grade 3 periosteal chondrosarcoma (Fig. ). |
pmc-6612073-1 | A nine-month-old male infant, was admitted with an 8-day history of watery, non-bloody diarrhea, vomiting and decreased oral intake. The baby was previously healthy and had a negative medical history.
On physical examination, the patient was pale and irritable with generalized body edema, tachypnea (rate 36/min) and tachycardia (HR140/min) but no rales or murmurs were heard. No other abnormalities were noted.
Initial investigations revealed leukocytosis (19 × 103), anemia (hemoglobin 7.7 g/L, hematocrit 22%) and thrombocytopenia (platelets 62× 103). Serum creatinine was 2.5, BUN, 57 uric acid, 7.6 mg/dl respectively, LDH 2293 IU/L. (reference value 265 I U/L). Peripheral blood smear revealed evidence of microangiopathic hemolysis with schistocytes and helmet cells. Abdominal ultrasound showed echogenic but normal sized kidneys. The patient was admitted to the pediatric intensive care with the diagnosis of hemolytic uremic syndrome for possible dialysis.
During his stay, the patient had persistent diarrhea, decreased oral intake, oligoanuria and generalized body edema and hypertension. There was no response to high doses of intravenous furosemide (urine output less than 0.5 ml/kg/hour). Because of progressive deterioration in kidney function (creatinine reaching 5.2 mg/dl, blood urea nitrogen 88 mg/dl), persistent oligoanuria and worsening microangiopathic hemolysis and thrombocytopenia (hemoglobin of 5.4 g/dl and platelets of 23× 103) peritoneal dialysis was started after 48 h of hospitalization. The patient also received one unit of packed RBC transfusion.
Stool studies with Multiplex Qualitative reverse transcriptase PCR were negative for Salmonella, Shigella, Campylobacter, Yersinia, enterohemorrhagic E coli; enteropathogenic E coli (EPEC), enterotoxigenic E coli (ETEC), enteroinvasive E coli (EIEC) and Shiga-like toxin-producing E.coli (STEC) stx1/stx2, Rotavirus A, Adenovirus, Astrovirus, but were positive for Norovirus GI/G II.
Additional relevant laboratory studies included massive albuminuria with an Ualb/Cr 97 mg/mg (ref value < 0.2), low C3 and C4 complement of 0.56 g/L (ref value 0.9–1.8 g/L) and 0.07 g/L (reference value 0.1–0.4 g/L) respectively.
After 48 h of continuous peritoneal dialysis, the patient improved clinically with progressive decrease in edema and gradual increase in urine output. Peritoneal dialysis was continued for 5 more days and his kidney function improved steadily and normalized by the 10th day when his serum creatinine was 0.3 mg/dl, and albuminuria was down to 0.13 mg/mg creatinine. Three weeks after the onset of the disease, C3 and C4 had returned to normal levels at 1.16 g/L and 0.31 g/L respectively. The patient’s hospital course and laboratory data are depicted in Figs. a and b. The patient has been followed for 11 months since the onset of his illness and shows no evidence of residual damage with normal renal, hematologic and complement profile. He is normotensive and has no microalbuminuria. |
pmc-6612075-1 | A 21-year-old white woman presented to our emergency room with a 1-month history of fatigue, muscle aches, nausea, cutaneous rash, and 5 days of blurred vision in her left eye (OS).
She denied any double vision, pain with eye movements, flashing lights, floaters, or changes in color vision. She had no recent sick contacts or travel. She had no past ocular or medical history. Her family history for ocular or autoimmune disease was unremarkable. She used non-steroidal anti-inflammatory drugs as needed for her recent myalgia.
She presented with a malar rash and some violet raised papules erupting on her elbows and knees that were made worse by exposure to sunlight. Her arterial pressure was 115/70 mmHg. Best corrected visual acuity was 20/20 in her right eye (OD) and 20/100 in her OS.
Intraocular pressure (IOP) was normal in both eyes (OU). Extraocular movements were conjugate and full and she had no subjective red desaturation. Conjunctiva was white without dilated conjunctival vessels, cornea was clear, and anterior chamber showed no evidence of cell or flare, bilaterally.
On dilated examination she had bilateral retinal vascular engorgement, scattered foci of inner retinal whitening consistent with CWS, and some superficial hemorrhages in OU.
These changes were primarily around the macula and optic nerve and spared the peripheral retina (Fig. a).
Optic disks were pink with sharp margins and a cup-to-disc ratio of approximately 0.4.
Triton Plus® SS-OCT (Topcon Corporation, Tokyo, Japan) (Fig. b) showed light central macular edema in her OD and severe central macular edema in her OS with the presence of serous subretinal fluid. SS-OCT sections were obtained on the CWS highlighting the presence of hyper-reflective material affecting the nerve fiber layer and reaching even the inner nuclear layer in some sections. This is considered a sign of ischemia-induced edema. Consistent with this edema, inner nuclear and outer plexiform layers appeared wavy and were partially masked by the overlying CWS.
FA (Spectralis HRA+OCT; Heidelberg engineering, Heidelberg, Germany) (Fig. c) showed generalized vascular walls enhancement, veins dilatation, and capillary leakage whereas, consistent with CWS, vascular occlusion was observed.
Triton Plus® SS-OCTA (Topcon Corporation, Tokyo, Japan) (Fig. d) confirmed superficial and deep capillary occlusion in all areas affected by CWS, but these areas appeared more confluent than in FA. No alterations were visible in outer retina and choriocapillaris OCTA segmentations, but some shadowing effects due to the overlying CWS were present.
At presentation our patient had symptoms that indicated myositis including diffuse muscle pain, weakness, malar rash, and raised papules on elbows and knees. Her muscle enzymes were significantly elevated with a creatine kinase (CK) of 17,030 U/l (normal range 22–198 U/l) and an aldolase (ALDOA) of 106 (normal range 0.5–3 UI/l). Electromyography and nerve conduction study results were suggestive of moderate myositis and a subsequent right thigh muscle (vastus lateralis) biopsy was consistent with an inflammatory myopathy, showing vascular inflammation and a perifascicular atrophy. A myositis antibody panel was positive for an anti-Jo1 antibody. Given the constellation of findings our patient was finally diagnosed as having DM.
Despite the absence of a clear consensus over the most indicated retinal therapy [], in agreement with a rheumatologist who was managing the systemic disease, treatment was started with 1 mg/kg per day of orally administered prednisone, but she showed no improvement over the course of a week. Therefore, therapy was modified with the administration of a high dose of intravenously administered methylprednisolone (1000 mg daily), showing a slow favorable change after 1 week with a reduction in macular edema and subretinal fluid.
At this point, a decision was taken to start a steroid-sparing immunosuppressive drug, so the corticosteroids were progressively tapered and therapy with cyclophosphamide was started.
The protocol for cyclophosphamide that was used was 500 mg/m2 (maximum 500 mg) administered intravenously every 2 weeks for the first three doses and then 750 mg/m2 (maximum 1200 mg) every 4 weeks for a total of six doses, with no further cyclophosphamide infusions. Thus, a standardized non-continuous treatment protocol was used, with the cyclophosphamide treatment course completed within 4 months of initiation [, ].
After 3 months of therapy, best corrected visual acuity was returned to 20/20 in OU with subjective temporal pericentral scotoma in her OS.
A funduscopic examination (Fig. a) revealed a reduction in number and dimension of the CWS and some new hemorrhages in OU.
SS-OCT (Fig. b) showed a complete resolution of the cystoid macula edema and subretinal fluid. The ganglion cell layer and, to a small extent, even the inner nuclear layer were thinned in the areas previously affected by CWS, confirming the ischemic nature of the edema. The wavy appearance of inner nuclear layer and outer plexiform layer was unchanged at this stage.
FA (Fig. c) showed normal diameters of arteries and veins without wall enhancement. Consistent with ischemic areas previously affected by CWS, capillary and venule angioectasias were noticeable. However, no retinal neovascularization was visible in OU.
OCTA (Fig. d) confirmed the same capillary occlusions visible in FA and it detected venule angioectasias as well.
After 6 months of therapy, CWS were almost completely resolved and no new hemorrhages were seen (Fig. a,b). Best corrected visual acuity remained stable at 20/20 in OU. However, the visual field examination performed after disease resolution confirmed the presence of multiple and confluent scotomas in OU, with central involvement in OS (Fig. c,d). |
pmc-6612131-1 | A 74-year-old Caucasian male with an Eastern Cooperative Group performance status of 1 and a 150 pack -year smoking history initially presented with progressive right upper extremity weakness. Further workup revealed a 1.4 cm frontal lobe mass on magnetic resonance imaging. Computerized tomography (CT) of the chest identified a spiculated mass lesion measuring 1.6 × 1.1 cm in the right hilar region. Apart from hypermetabolic activity in the lung mass, a staging positron emission tomography (PET) identified avidity in the mediastinal and hilar lymph nodes. Biopsy of the lung mass and hilar nodes identified moderately differentiated adenocarcinoma of lung origin. Based on this tumor size and nodal involvement, his intrathoracic disease was staged as IIIA (AJCC 7th). His solitary left precentral gyrus mass was treated with gamma knife radiosurgery, and he was subsequently placed on steroids with improvement in his limb weakness. His intrathoracic disease was treated with four cycles of cisplatin and pemetrexed with concurrent definitive radiation therapy. During follow-up, a surveillance PET scan approximately 11 months later was notable for new metastatic liver, mediastinal, para-aortic and right lower lobe lesions. Due to his original biopsied tissue having PD-L1 expression of 50% (22c3 antibody), he was started on single agent pembrolizumab 200 mg every 3 weeks. His baseline blood counts before starting pembrolizumab were all within the normal range. Two weeks after completing the fourth cycle of pembrolizumab, he presented to the emergency department with fever, chills, and general malaise. He was noted to be neutropenic with an absolute neutrophil count (ANC) of 0, which previously was noted to be normal the day of his fourth dose of pembrolizumab (Fig. ). Hemoglobin was 12.6 g/dl, and platelet count was normal. The patient was hospitalized with febrile neutropenia and started on broad-spectrum antibiotics. As part of our programmatic approach, a serum C-reactive protein (CRP) level was obtained, which was markedly elevated at 175.4 mg/L (Fig. ). He was started on prednisone 80 mg daily and filgrastim dose of 5 mcg/kg daily for 4 days. All his infectious workup, including blood cultures, were negative. Bone marrow biopsy showed normocellular marrow with left-shifted trilineage hematopoiesis, with a predominance of early erythroid and myeloid precursors and no increase in blasts or significant morphologic dysplasia. Cytogenetics and myelodysplastic syndrome panel were normal. His ANC started to improve by day four of prednisone and filgrastim (Fig. ). From his serial blood samples, he also had T-cell counts and cytokines checked (Fig. ). He had complete recovery of his neutrophil count with ANC of 2400/ μL by day six of admission. Computerized Tomography imaging of the chest done during admission showed intrathoracic disease response compared to the CT performed 6 weeks earlier. His steroid taper schedule was 80 mg daily for week 1, 40 mg daily for week 2, 20 mg daily for week 3, 10 mg daily for week 4 and then stopping. His pembrolizumab was kept on hold.
However, eight weeks after being discharged, he was readmitted again with fever, cough, and shortness of breath. He was again noted to be neutropenic with ANC of 0 / μL (Fig. ). Other complete blood count parameters were within the normal range. His CRP had again increased to 43.5 mg/L, from 7.7 mg/L at the last clinic visit 6 weeks prior. He was started on broad-spectrum antibiotics, prednisone 1 mg/kg, and a single dose of peg-filgrastim. Viral studies (HIV, Hepatitis-B, Hepatitis-C, and CMV) were negative. EBV IgG and Parvovirus B19 IgG titers were elevated and thus consistent with prior infection. Antinuclear antibodies (ANA), antineutrophil cytoplasmic antibodies (ANCA), rheumatoid factor (RF), and neutrophil-associated antibodies were negative, and C3 and C4 levels were normal. His neutrophil counts recovered to > 1500 on day seven of the second hospitalization.
His neutrophil counts were 5440/ μL at his post-discharge clinic follow up a week later. A restaging PET scan showed continued response with some areas of complete metabolic response. He continued prednisone taper for 8 weeks. Although his ANC showed persistent recovery and remained > 5000 / μL after his second hospitalization, his pembrolizumab was kept on hold. Repeat PET scan 7 weeks after the second neutropenia showed the metabolic activity of a mass like consolidation with air bronchogram in the posterior right upper lung and moderate focal uptake in the enlarged portocaval lymph node and tiny periaortic lymph nodes suggesting recurrent malignancy with likely a post-obstructive pneumonia. Unfortunately, despite adequate outpatient management, he had recurrent episodes of bacterial pneumonia complicated by hospitalizations due to which his performance status declined considerably. Due to this, he could not be initiated on any further treatment. Three months after his second neutropenic episode, he died from hypoxemic respiratory failure secondary to bacterial pneumonia that was unrelated to ICI use or neutropenia. |
pmc-6612139-1 | A 60-year-old female presented to the emergency room for progressive exertional chest distress and fatigue. The patient experienced paroxysmal nocturnal dyspnea and orthopnoea 2 days before admission. On admission, the physical examination revealed body temperature of 36.8 °C, heart rate 105 beats/min with sinus rhythm, respiratory rate 35 per minutes, blood pressure 85/64 mmHg and fine rales can be heard at bilateral lower lobes. Laboratory examinations for systemic vasculitis and infectious diseases demonstrated no abnormalities and work-up for childhood and family history were negative. TTE showed a giant circumscribed echolucent mass with suspicious intra-cavity thrombus compressing left ventricle, although the global ejection fraction (EF) was maintained at 54%. An abnormal communication between the RCA and PA trunk was also identified (see Additional file 1), and the Qp/Qs ratio was 2.5. A coronary CTA scan performed with a 16-detector row confirmed an extensive right CAA (3.05 × 2.34 cm in short-axis) with RCA-PA fistula (Fig. a) and a partially thrombosed giant aneurysm (9.20 × 7.28 cm in short-axis) arising from the branch of the left anterior descending (LAD) coronary artery, oppressing the left ventricle (Figs. b, c and a). The results of invasive coronary angiography correlated with the previous findings by CTA and TTE (see Additional file 2).
Owing to the patient’s clinical status deteriorated continuously, the patient underwent surgical intervention under cardiopulmonary bypass to improve the hemodynamic instability due to restrictive cardiac dysfunction. Intra-operatively, a giant left CAA was found to be encasing the left and right ventricular wall and extending toward the apex (Fig. a). The fistulous vessel between the native RCA and PA was mobilized and ligated while we left the right CAA as it was before surgery. The left aneurysmal sac was opened and multiple hemorrhagic laminated thrombi were debrided completely. The orifice of the aneurysm connecting to one of the LAD branches was identified and closed with interrupted 5–0 prolene sutures (Fig. b). The patient weaned off bypass successfully and received lifelong antiplatelet therapy postoperatively. Postoperative TTE and CTA revealed no RCA-to-PA shunting or any flow in the residual aneurysmal sac and the compression of the giant left CAA imposed on the left ventricle had been obviated (Figs. d, e, f and b; Additional file 3). The patient experienced an uneventful postoperative course and discharged with improved heart function (EF: 68%, NYHA II). The pathologic examination of the aneurysmal wall revealed loss of smooth muscle cells in the media with local mucoid degeneration (Fig. c), no chronic inflammation, sclerosis and IgG4 were observed. |
pmc-6612164-1 | A 3-year-old previously healthy girl was seen in the rheumatology clinic for a persistent limp of 3 weeks’ duration. There was no known history of trauma. She was able to bear weight but reported right knee pain exacerbated by walking and climbing stairs. She had no pain at rest and no nighttime awakenings. She had no fevers, rashes, rhinorrhea, cough, sore throat, vomiting, or diarrhea. The patient was a “picky eater,” but she was growing appropriately and had met all of her developmental milestones. After the limp had persisted for 2 weeks, she had seen her pediatrician, who performed a right hip radiograph, complete blood count with differential, comprehensive metabolic panel, and erythrocyte sedimentation rate. These were unremarkable except for microcytic anemia with a hemoglobin level of 10.2 g/dL (mean corpuscular volume [MCV] 73 fL, red cell distribution width [RDW] 16%).
At the rheumatology visit, her weight was at the 46th percentile, and her height was at the 83rd percentile. Her vital signs were within normal limits. She had a normal examination of her eyes, mouth, throat, neck, lungs, heart, abdomen, and skin. Her musculoskeletal exam demonstrated normal strength and no tenderness to palpation of her lower extremities. She had intermittent guarding with movement of her right knee but no erythema, warmth, tenderness, limited range of motion or swelling to this joint; the remainder of her joint exam was normal. She had an antalgic gait with minimal right knee movement and favored the right side. Neurologic examination revealed normal mental status, cranial nerve exam, tone, and patellar deep tendon reflexes.
The differential at that time was broad and included infectious, mechanical, inflammatory, oncologic, neurologic, and intra-abdominal etiologies. Initial laboratory work-up included a complete blood count with differential, peripheral smear, and C-reactive protein, which was remarkable for a hemoglobin level of 10.2 g/dL (MCV 70.2 fL, RDW 14.8%) (Table ). Peripheral smear showed mild microcytic hypochromic anemia without blasts or abnormal cells. A radiograph of her right lower extremity demonstrated mild osteopenia, and a right hip and knee ultrasound were normal. She was discharged home on a non-steroidal anti-inflammatory medication with a plan to obtain additional imaging as an outpatient.
Over the next 7 days, the patient developed progressive weakness. Parental videos showed the patient exhibiting a Gowers’ sign and having difficulty ascending stairs. Given this acute worsening, she was admitted to the hospital. On admission her strength was abnormal; she would brace herself against furniture to stand and had a positive Gowers’ sign. Direct strength testing against resistance was difficult to obtain, but knee extension, plantar flexion, dorsiflexion and toe flexion were 4/5 bilaterally. She had a wide-based gait and had difficulty fully lifting her feet off the ground while ambulating. Otherwise, her neurological exam was normal regarding her mental status, cranial nerves, deep tendon reflexes, sensation, coordination, and upper extremity strength. Complete metabolic panel, creatine kinase, aldolase, lactate dehydrogenase, uric acid, thyroid stimulating hormone, vitamin D level, and acetylcholine receptor binding antibody were all within normal limits (Table ). A brain magnetic resonance imaging (MRI) was normal, and a spine MRI was remarkable for a small amount of fat within the filum terminale. Lumbar puncture showed normal cerebrospinal fluid protein, mildly low glucose (48 mg/dL), 1 white blood cell, no red blood cells, and negative enterovirus. MRI of the lower extremities demonstrated increased signal and enhancement of the right paraspinal musculature and bone marrow edema within the right sacral ala, posterior acetabulae, and distal femoral metaphyses. Electromyogram and nerve conduction studies were normal. Plasma amino acids, urine organic acids, acylcarnitine profile, ammonia, and homocysteine were normal. Iron studies demonstrated low iron (22 μg/dL), elevated total iron-binding capacity (481 μg/dL), and low transferrin saturation (5%) (Table ).
On hospital day 6, her vitamin C level, which had been obtained upon admission in light of her having a restricted diet, returned as undetectable at < 0.1 mg/dL. Upon further questioning, the patient refused to eat fruits or vegetables; her diet at home consisted of dairy products, bread, and crackers. She was started on ascorbic acid 100 mg 3 times daily and had improved strength within 24 h of her first dose. She was discharged shortly thereafter with a diagnosis of scurvy. She returned to her baseline within 2 to 3 weeks. At her follow-up neurology and rheumatology visits, she had a normal neurologic exam including strength and gait. Laboratory results 11 weeks post-discharge demonstrated improving hemoglobin level (11.3 g/dL; MCV 73.7 fL, RDW 13.8%) and normal vitamin C level (1.1 mg/dL). She saw a dietician, who recommended dietary and behavioral modifications and continuation of ascorbic acid supplementation. |
pmc-6612182-1 | A 33-year-old female presented to the emergency room with progressive abdominal pain. The patient underwent a LRYGBP two days prior to her admission. The primary operation was uneventful. Our technique involves double stapling of the intestinal jejunal-jejunal anastomoses using two 60 mm 2.5 mm staplers. No bleeding problem was encountered during this operation. Postoperative, she received subcutaneous low-molecular weight heparin for one week. She did not have a relevant past history. Her current medications were citalopram, pantoprazole and nadroparin.
The abdominal pain had a sudden onset and increased gradually. The patient had continuous severe abdominal pain localized in the left hemi-abdomen, intensifying from time to time (colic). Other complaints were nausea and vomiting. Since the LGBP, the patient did not have any stool. Flatulence was present. During physical examination, the patient experienced a lot of pain. Temperature, heart rate and blood pressure were normal. The bowel sounds were high pitched during auscultation. Palpation of the abdomen was mostly tender in the left hemi-abdomen. Biochemical analysis showed a C reactive protein (CRP) of 47 mmol/L, white cell count of 19.0 × 10 9/L, a glomerular filtration rate of more than 90 and a lipase of 47 U/L.
An abdominal CT-scan showed severe dilatation of the excluded stomach, filled with fluid. The whole trajectory of the proximal small intestines was distended, up to the Y-anastomosis. Remarkable was that the more distal small bowel was also distended, almost up to the distal ileum. A hyperdensity was seen in the excluded stomach and in the proximal small intestines (Fig. ).
A laparoscopy was performed. During laparoscopy, an intraluminal obstruction was found proximal of the jejunojejunostomy, causing dilatation of the excluded stomach and biliairy limb. Both, the alimentary limb as well as the common channel were distended due to a bulk found at the distal ileum. The bulk was movable trough gentle massage into the colon. The mass was a large blood clot, which appeared as a hyperdensity on the abdominal CT scan (Fig. , arrow). Another obstructing blood clot in the distal biliairy limb was removed by opening the blind loop of this limb and removing the cause of obstruction. The severely distended excluded stomach was decompressed with diathermic perforation and suction, followed by closing the defect with a V-lock.
Postoperatively, the patient had a persisting tachycardia, severe abdominal pain and elevated infection parameters (CRP 455 mmol/L; white cell count of 37.8 0 × 10 9/L) and a lipase of 207 U/L. Another laparoscopy was performed, which showed no signs of anastomotic leakage. A pancreatitis was seen: the pancreas was edematous and the pancreatic body was enlarged. Treatment was the standard management of pancreatitis.
The acute pancreatitis had progressed with a lipase of 697 U/L. The patient developed fever, for which intravenous ceftriaxone and metronidazole was given.-
An ultrasonography of the abdomen showed no cholelithiasis as a cause of pancreatitis.
Repeated CT-scan of the abdomen showed an edematous pancreas and peripancreatic fat infiltration, without any sign of pancreatic necrosis or intra-abdominal abcess.
The symptoms of the patient improved during the course of her admission and the inflammation parameters normalized. She was discharged after 16 days. |
pmc-6612210-1 | A 73-year-old woman suddenly developed right hemiplegia and severe aphasia and was transported to our emergency service 47 min after onset. Her medical history included hypertension and paroxysmal atrial fibrillation. Anticoagulants have been discontinued because of a few episodes of falls although she had previously received oral anticoagulation. Blood pressure was 105/75 mmHg without any significant difference between right and left limbs. No cardiac murmurs were audible. Twelve-lead electrocardiography (ECG) showed tachycardic atrial fibrillation with a heart rate of 150 beats/min but no significant ST-T changes, although the baseline was undulating due to patient movement (Fig. ). Glasgow Coma Scale score was 9 (E4V1M4). The patient was mute and could not follow any commands. She presented with right hemiplegia and showed left-ward conjugate eye deviation that could not be overcome with oculocephalic stimulation. The National Institutes of Health Stroke Scale score was 21. Blood glucose level was 189 mg/dL, serum creatinine level was 0.66 mg/dL, hemoglobin was 10.8 mg/dL, and platelet count was 16.9 × 104/μL. No abnormalities were evident on chest roentgenogram.
We immediately performed magnetic resonance imaging following the stroke management protocol in our institute. Diffusion-weighted imaging showed hyperintense lesions at the left basal ganglia and corona radiata (Fig. a, b). A susceptibility vessel sign was seen in the proximal portion of the left middle cerebral artery (Fig. c). Magnetic resonance angiography revealed proximal occlusion of the left middle cerebral artery (Fig. d). Alteplase at a dose of 0.6 mg/kg (the dose approved in Japan) was administered 43 min after hospital arrival [, ], immediately followed by endovascular thrombectomy as bridging therapy []. After thrombectomy with a stent retriever (Solitaire 2, 4 × 20 mm; Medtronic, California, USA), complete recanalization was obtained 95 min after hospital arrival (Fig. e, f). Cardiac rhythm monitoring during the endovascular procedure did not show significant findings other than the tachycardic atrial fibrillation.
Aphasia and right hemiplegia improved immediately after completing mechanical thrombectomy and she began to complain of chest pain. A detailed medical history was elicited and revealed that she experienced chest discomfort just prior to the development of weakness in the right extremities. No symptoms suggestive of angina pectoris had been present prior to this episode of chest discomfort. Twelve-lead ECG showed ST elevation at II, III, and aVF and ST depression at V2–V6 (Fig. a). Transthoracic echocardiogram showed decreased motion of the posterior wall and interventricular septum, and serum troponins were elevated. Coronary angiography revealed a filling defect in the right coronary artery, but distal flow was rapid and adequate (Fig. b). Distal occlusions were identified in the left circumflex small branch and the diagonal branch (Fig. c). No stenotic lesions suggestive of atherosclerotic pathology were identified. Because we considered that myocardial perfusion was sufficient overall, percutaneous coronary intervention (PCI) was not performed.
The occlusions of the left middle cerebral artery and coronary arteries were assumed to have occurred nearly simultaneously, with cardiogenic embolism due to atrial fibrillation considered as the most likely etiology []. No deep vein thrombosis of the lower limbs was evident on ultrasonography. On day 5 after admission, oral rivaroxaban was started at a dose of 15 mg/day (the standard dose in Japan) []. Bisoprolol (0.625 mg/day) was initiated for rate-control purposes. The patient was discharged for rehabilitation on day 22. The modified Rankin Scale score at 3 months after stroke onset was 2. The patient experienced no recurrence of cerebral or myocardial infarction at 6 months after discharge. |
pmc-6612217-1 | A 46-year-old Chinese woman, gravida 1 para 1, presented with lower abdominal pain for 5 days and dysmenorrhea for 2 years. Trans-vaginal ultrasound, magnetic resonance image (MRI) and computerized tomography (CT) indicated the presence of uterine adenomyosis and bilateral ovarian endometriotic cysts. There were no abnormalities were found in her bilateral lungs and other abdominal/pelvic organs. The preoperative serum CA-125, carcinomatous embryonic antigen (CEA), and CA-153 were 263.1 U/mL (normal < 35 U/mL), 13.9 U/mL (normal< 5 U/mL) and 33.1 U/mL (normal < 25 U/mL), respectively. She denied her personal and familial history of any cancers and related diseases.
After admission, an abdominal cystectomy of the right ovary was initially performed. The intraoperative frozen section was carried out. Unexpectedly, the frozen section showed an ovarian adenocarcinoma in the wall of the endometriotic cyst. The laparotomy exploration found no abnormalities in other abdominal/pelvic organs including stomach, colon, rectum, appendix, pancreas, and liver. There were no any visible tumorlets in the abdominal/pelvic cavity. The patient eventually underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy, pelvic and para-aortic lymphadenectomy and omentectomy. The patient was eventually assessed as a stage IC ovarian carcinoma because the tumor partly adhered to the surrounding organs. She recovered smoothly from her surgery. She received 5 courses of TP (paclitaxel + cisplatin) chemotherapy, but she had to abandon her last chemotherapy because of the severe myelo-suppression.
The patient was followed up regularly in our outpatient clinics. The serum CA-125, CEA and CA-153 gradually returned to normal levels within 22 days after her surgery. However, her serum CEA began to elevate (6.3 U/mL) at 6 months after her surgery and rose up to 62.1 U/mL at 7 months. MRI showed a right pelvic mass measuring 9.5*5.7 cm and no abnormalities in the abdominal organs. The patient was transferred to a tertiary hospital for further treatment. The gastrointestinal endoscopic examination with multiple biopsies showed no abnormalities in the stomach, terminal ileum, colon and rectum. A relaparotomy was carried out. The intraoperative findings included a large tumor adhering to the right pelvic wall and the ileum wall, and multiple small grey nodules along the small intestinal mesentery. Other abdominal organs, such as stomach, colon, appendix, pancreas and liver, looked unremarkable. The pelvic tumor and partial ileum were removed. The patient refused to adjuvant therapies including chemotherapy and radiotherapy. She was alive with disease for13 months after her first surgery at present.
Grossly, the right ovarian cyst for frozen section measured 18 × 15 × 14 cm. It had a smooth outer surface with occasional rough areas consistent with the adhesions to the surrounding organs. The unilocular cyst was filled with thick, chocolate-like fluids. The inner cystic wall was uneventful except the attachment of some blood clots. There was a small mural nodule with a size of l.0 × 0.5 × 0.2 cm protruding into the lumen. The nodule had a white and solid cut surface. The texture was soft. The right ovary was partly hemorrhagic due to the cystectomy. The left ovary measured 5.0 × 5.0 × 4.5 cm. It had some cystic areas containing dark brown hemorrhagic materials. The uterus measured 10*9*5 cm. The myometrium of the posterior wall was thickened. The cut surface was trabeculated and contained hemorrhagic foci. The endometrium measured 0.2–0.3 cm and looked unremarkable. The uterine cervix and bilateral fallopian tubes were grossly normal.
The mural nodule was histologically continuous with the endometriotic epithelium [Fig. a, b]. It showed an invasive growth pattern that were characterized by solid nests with occasional cribriform structures, crowded small irregular glands in sheets and scattered single cells [Fig. c, d]. The neoplastic cells in solid nests or in single harbored a typical signet-ring appearance as large mucinous vacuoles and atypical crescentic nuclei [Fig. c, e]. The glands were lined by columnar cells with abundant cytoplasmic mucin, significant nuclear atypia, and occasional mitotic figures. The intracellular mucin was Alcian Blue (pH 2.5) positive [Fig. f]. The stroma was desmoplastic and had occasional extracellular mucin deposits. There was no lymphovascular invasion or surface involvement of the ovary. The remaining cystic wall showed a typical morphology of an endometriosis that was composed of endometrial epithelium and stroma with hemosiderin-laid macrophages [Fig. a, b]. The epithelium adjacent to the tumor showed atypical features (“atypical endometriotic cyst”) that were characterized by cellular crowding, stratification, small papillary formation with eosinophilic cytoplasmic changes, and variable cytological atypia with increased nuclear to cytoplasmic ratio, enlarged hyperchromatic nuclei, and conspicuous nucleoli [Fig. g, h]. The surface epithelium was cytologically bland in most areas.
Immunohistochemistry demonstrated that the tumor cells were strongly and diffusely positive for CK 7 [Fig. a], CEA and p16, and focally positive for CA125, MUC-6 and p53 (DO-7). They were negative for phosphate and tension homology deleted on chromosome ten (PTEN), estrogen receptor (ER), progesterone receptor (PR), CK19, CK20 [Fig. b], PAX-8 [Fig. c], and CDX2. They did not show loss of hMLH1 and hMSH2 expression. The atypical endometriotic epithelium was positive for PAX-8 [Fig. d]. The final pathological diagnosis of the right ovary was poorly differentiated mucinous carcinoma with signet ring cells and concurrent endometriotic cyst with atypical features.
Other pathological findings included endometriotic cysts of the left ovary and uterine adenomyosis. The endometrium showed proliferating-phase alterations. The remaining right ovary, bilateral fallopian tubes, and uterine cervix were pathologically unremarkable. The omentum and pelvic lymph nodes were free of tumor.
The gross feature of the recurrent tumor from her second operation was unavailable in detail. Pathological review of the slides indicated that the recurrent tumor was consisted of poorly-differentiated adenocarcinoma in the fibrous stroma resembling to the original ovarian carcinoma. The tumor involved the outer intestinal muscular proper, but the intestinal mucosa remained normal after careful and extensive sampling. The excision margins were free of tumor. |
pmc-6612222-1 | A 26-year-old woman patient was admitted to Shanghai Pulmonary Hospital on December 13, 2013 for constant gastric pain for 1 month. One month before the hospital admission, the patient started to experience gastric pain accompanied with acid reflux for no apparent cause. The pain became worse on an empty stomach. The patient did not present swallowing difficulties, belching, nausea, vomiting blood, black stool, fever, fatigue, diarrhea, or tenesmus at hospital admission. The patient had no previous exposure to tuberculosis.Gastroscopy revealed a hemispherical bulge with smooth surface (Fig. a) and two small ulcers on the posterior wall of the lesser curvature of the stomach (Fig. b). Endoscopic ultrasound showed hypoechoic masses around the lesion (Fig. c), ununiform echo with some area of strong echo inside the lesion, and rich blood flow inside the lesion (Fig. d). The lesion was located in the fourth echo layer and showed a dimension of 26 mm × 21.5 mm (Fig. c). The patient was originally suspected to have gastric stromal tumor. The pathology of gastroscopic biopsy displayed acid-fast staining negative, reticular fiber staining negative, periodic acid Schiff staining negative, and ammoniacal silver staining negative. Haematoxylin & eosin staining of the gastroscopic biopsy specimen showed patches of caseating necrosis and granulomatous inflammation (Fig. e and f). Gene sequencing by polymerase chain reaction(PCR)analysis of the gastroscopic biopsy specimen found Mycobacterium tuberculosis (M. TB) deoxyribonucleic acid (DNA) fragments.
Chest computed tomography (CT) scan demonstrated: 1) patchy densities and calcified nodular shadows in the upper right lung (Fig. g), suggesting a possible previous lung TB; 2) calcified pleural nodules in the lower right pleural (Fig. h); 3) nodular shadows in the lesser curvature soft tissue of the stomach (Fig. i). Chest CT-guided percutaneous needle biopsy on the pleural nodules found necrotic tissue and inflammatory cells, The acid-fast stain smear was negative, Along with other findings in chest CT and gastric pathology and positive TB-PCR, these pathologic changes may be in favor of TB pleural involvement. Electronic Bronchoscopy showed normal lumen and mucosal membrane. The patient did not have a previous diagnosis of TB. She had normal routine blood and chemistry test results. Tuberculin skin test (TST) showed an induration of 22 mm × 18 mm. M. TB antibody test and T-SPOT.TB test (A30 and B10) were positive. Based on these clinical data, the patient was diagnosed as gastric TB. First acid-fast bacilli smear and culture tests were negative.
The patient was hospitalized and underwent the systematic antitubercular therapy(ATT) HREZ for 1 week (0.3 g once daily oral isoniazid., rifampicin 0.45 g once a day on an empty stomach, 0.75 g once daily oral ethambutol, pyrazinamide orally, 0.5 g three times a day. Oral protection of liver is also given). The patient insisted on an outpatient review every month, with routine blood tests and liver and kidney function tests. After 3 months of anti-tuberculosis treatment, the patient developed gastrointestinal reaction and blurred vision, and the adjustment programme was HRptZLfx,(Isoniazid 0.3 g 1 day orally, rifapentini 0.45 a week twice fasting, pyrazinamide 0.5 g a day three times, levofloxacin 0.5 g 1 day orally). The above side effects were alleviated.After 6 months of treatment, levofloxacin was discontinued and continued HRptZ treatment. She had normal monthly reexamination of the normal liver and kidney function, and acid-fast bacilli smear and culture tests were negative for every 3 months. The erythrocyte sedimentation rate and C reactive protein were negative before the drug withdrawal. Her gastroscopy showed normal results. Her chest CT scan revealed calcified nodules in the upper right lung (Fig. j), bilateral pleural thickening (Fig. k), calcified pleural nodules (Fig. l), and substantially smaller nodules with some calcification in the lesser curvature (Fig. l) compared with the chest CT scan results on the hospital admission (Fig. g-i). Her sputum acid-fast bacilli smear and culture tests in March and May 2015 showed negative results. The antitubercular therapy was ended in May 2015. Her total treatment course was 18 months.
Deparaffinisation and DNA extractionTissue sections 5 μm thick were cut from paraffin blocks, and 10 sections were placed in 1.5 ml sterile EP tube. The tissues were deparaffinised by adding 1 ml of xylene to each microtube and the samples were mixed gently for 20 min at 65 °C. The samples were then centrifuged at 13000 rpm for 2 min and the supernatants were discarded. Residual xylene was removed by washing the samples twice with 1 ml of 100% ethanol for 5 min and centrifuging at 13000 rpm for 2 min. The pellets were dried in a speed vacuum, and 200 μl of FTL and 25 μl of OB Protease (OMEGA). Genomic DNA was isolated using the FFPE DNA kit from Omega Biotek Inc. (Norcross, GA, USA) according to the protocol provided by the manufacturer. The contents and purity of the extracted DNA were assayed by measuring absorbance at 260 and 280 nm using a spectrophotometer.PCR amplificationIS6110 gene is specific for MTBC and 16SrRNA gene is specific for mycobacterium genus. The following primer sets were used for PCR: IS6110 (sense 5 ‘-CCTGCGAGCGTAGGCGTCGG-3′, anti-sense 5′- CCTGCGAGCGTAGGCGTCGG-3′); the product size of IS6110 was 123 bp a. Reactions were run in 25ul volumes containing 2ul of sample DNA. The remaining volumes was added using PCR master Mix (2×) from Thermo according to the protocol provided by the manufacturer. The amplification program was consisted of initial denaturation at 95 °C for 5 min, followed by 35 cycles of denaturation at 94 °C for 45 s, annealing at 60 °C for 30s, extension at 72 °C for 45 s, and a final extension at 72 °C for10 min. The PCR products were analyzed by 2% agarose gel electrophoresis [].
We searched PubMed for the keyword “gastric tuberculosis” and found that 22 cases of gastric TB were reported from 2000 to 2016 [–, –]. The summary of these cases is displayed in Table . The patients were 21 to 80 years of age, and the mean age was 38.6 ± 15.3 years. The average history of disease were 6.4 ± 6.06 months. The shortest is 1 month. Of the 22 cases, 45.5% (10/22) were men; 59.1% (13/22) were primary gastric TB; 31.8% (7/22) had gastric outlet obstruction (Table ). Two cases were gastric TB with concomitant cancer [, ]. PCR was commonly used to diagnose gastric TB since 2011 (Table ) [, , –]. Most cases presented caseating granuloma in endoscopic biopsy specimens or surgical specimens (Table ). Notably, a few cases did not show confirmed diagnosis of gastric TB but responded well to empiric ATT (Table ) [, ]. All the patients survived and respond well to routine ATT. |
pmc-6612226-1 | Subject VC06 is a 40-year-old, transgender woman from Rio de Janeiro, Brazil, who was diagnosed with HIV-1 infection at the end of 1999 and has been seen for routine clinical follow-up at the Instituto Nacional de Infectologia Evandro Chagas (INI-Fiocruz), Rio de Janeiro, Brazil since 2005. In 2009, VC06 signed an informed consent and was enrolled in the INI-Fiocruz LTNP/HIC cohort study, approved by the Brazilian National Human Research Ethics Committee (CONEP 840/2008) and by the FIOCRUZ Research Ethics Committee (CEP 1717.0.000.009–07). Due to study enrollment, individual VC06 was followed at least once every 6–12 months to perform specific infection-monitoring tests (such as HIV-1 RNA viral load quantification and CD4+ T cell counts) and routine clinical laboratory exams and to assess data related to clinical status and exposure to sexually transmitted infections. In addition, blood was collected at each visit to isolate plasma, whole blood and peripheral blood mononuclear cell (PBMC) samples for study. Subject VC06 was initially classified as an LTNP HIV viremic controller (< 2000 cp/ml dually infected with two HIV-1 subtype B viruses (de Azevedo et al. 2017) []. She carries a nonprotective HLA-B genotype (HLA-B*15:01/ B*48:02) but has heterozygosis for the CCR5-Δ32 mutation, which is considered a host-protective allele for disease infection and progression.
Subject VC06 displayed low-level viremia (< 500 copies/mL) in the absence of antiretroviral therapy until early 2013, when she started to show increases in the viral load, reaching approximately 10,000 copies/ml 1 year later (Fig. , V92014). The following months were associated with a spontaneous decrease in viral load, reaching 577 copies/ml in August 2015. Combination antiretroviral therapy (cART) with a scheme containing TDF, 3TC, and EFZ was prescribed in November 2015 but interrupted 1 month later by the patient due to intense dizziness related to the treatment. Side effects ceased, but the continuity of the therapy was refused by the patient in the following years. Transient recovery of viremia control was followed by intermittent viral loads above 2000 copies/ml and a new peak of approximately 8000 copies/ml in May 2017. This new peak of viremia was followed by a spontaneous decrease in viral load, reaching 1435 copies/ml in May 2018. The most recent available data indicated a viral load of approximately 3500 copies/ml at the end of 2018 (Fig. ). Despite increasing viremia, CD4+ T cell counts during the whole period were stable at high levels, suggesting no immunological commitment or disease progression.
In addition to the intermittence of the plasmatic viral load after a controller period, individual VC06 was diagnosed with latent syphilis in May 2010 based on positive VDRL results (1/32) in the absence of clinical signs or symptoms and a previous negative VDRL test in December 2009. The syphilis diagnosis coincided with unprotected sexual intercourse reported by the patient, and treatment with weekly benzathine benzylpenicillin 1,200,000 IU intramuscular injections were administered for 3 weeks starting in November 2010. Late latent syphilis was further diagnosed again at two additional timepoints: first in September 2015, based on VDRL titer of 1/8; second in November 2017, based on TPHA positive and a VDRL titer of 1/512. Both cases were preceded by a VDRL titer of 1/1 6 months before and were treated, as described above, in November 2015 and May 2018, respectively. No clinical signs or symptoms associated with syphilis infection were observed during follow-up. Another clinical event during the follow-up period included the diagnosis of an anal fistula at the beginning of 2014, which was surgically treated in the same year.
To assess the patterns of intrahost viral evolution and to investigate the cause associated with the loss of viremia control, PBMCs (1 × 107 cells) from selected visits (Fig. ) were thawed and used for genomic DNA extraction, as previously described []. The genomic DNA obtained was used for amplification by nested PCR single genome amplification (SGA) and sequencing of a ≈ 600 bp C2-C4 fragment of HIV-1 env, as previously described []. A neighbor-joining phylogenetic tree containing all sequences obtained from samples collected over time is shown in Fig. . Env sequences from 2009 (V12009; n = 29) and 2010 (V32010; n = 43) samples showed the presence of a single subtype B variant (called B1). Analysis of env sequences from 2011 (V42011; n = 32), 1 year after the diagnosis of syphilis infection, showed the presence of a second subtype B variant (called B2) in addition to the previous B1 variant. These variants branched separately and displayed a mean env genetic distance of 16.8%. Tropism analyses, realized through Geno2pheno tool using a false-positive rate (FPR) of 10% [], of env sequences, obtained at all time points, showed that both B1 and B2 variants correspond to R5-tropic viruses that present different predominant motifs at the top of the V3 loop (QPGR/QPGG for B1 and GPGR for B2). Env analyses of samples from subsequent time points revealed a shift in the proviral quasispecies proportion, with an increase of B2 variant frequency from 16% in 2011 (V42011; n = 32) to 93% in 2014 (V92014; n = 27). The majority of the B2 (93%) viral quasispecies was maintained even after the reduction of plasmatic viral load in 2016 (V142016; n = 14) as well as after a new peak of viremia (75%) in 2017 (V162017; n = 12) (Fig. ). For B2 quasispecies from all timepoints (n = 52), 80% of the sequences obtained were classified as R5 with FPR values greater than 45%, while the remaining presented FPR values between 11.5% and 18,5%.
Plasma sequences were obtained from the V92014 and V162017 samples, as previously described [], supporting that B2 was the replicating variant accounting for the increase in viremia observed at both time points (Fig. ). Moreover, the distribution of B2 sequences on the env phylogenetic tree indicates a pattern of increasing divergence along the follow-up period, with the identification of two distinct clusters harboring most sequences for the V92014 and V162017 samples. In additional analyses, all B2 sequences were used for estimation and reconstruction of the most recent common ancestor (MRCA), using the Phylip package []. The mean genetic distance between the MRCA and the B2 sequences for each visit was calculated to determine B2 viral divergence during follow-up. We observed increasing values of divergence during follow-up (0.9% for V42011, 1.7% for V92014; 3.5% for V142016; 4.4% for V162017), indicating a temporal evolution pattern. These results, in addition to the observation that sequences from the V142016 samples were distributed between the V92014 and V162017 clusters, with no clear predominance of any population, also indicate that different B2-related viral quasispecies accounted for the viral replication in each viremia peak.
To better characterize the SI and assess the divergence between B1 and B2 at a more conserved region of the viral genome, we conducted SGA of the int region from the V42011 sample, in which we first detected the B2 variant, and from the V92014 sample, in which B2 became the dominant variant. A neighbor-joining phylogenetic tree containing the int sequences is shown in Fig. . In the V42011 sample (obtained at the time of SI detection), a single int variant related to B1was found despite the detection of two variants in the env analysis from the same time point. The absence of a second int variant is probably related to the low number of sequences obtained at this time point (12 sequences for int vs 32 sequences for env). In the V92014 sample, however, we identified two int variants with a mean genetic distance of 4.2%. Although these data indicate the presence of B1 and B2, the frequencies of both int variants differ from those observed for env (40% vs 7% for B1; 60% vs 93% for B2). Even though different fragments have distinct PCR efficiencies, which could introduce a bias factor, the great divergence of representation could be indicative of recombination between int and env genes.
Finally, to evaluate viral integrity, we obtained the full-length HIV-1 genome from the V12009 sample, as previously described []. The overlapping fragment sequences obtained allowed the identification of the B1 variant full-length genome and the absence of deletions or frame-shift alterations related to genetic defects, indicating that B1 is a replication-competent virus. The full-length B1 genome also confirmed that the int variants obtained via SGA of the V42011 sample are related to this variant (Fig. ). Isolation of the full-length genome for the B2 variant was not possible due to the equivalent frequencies of B1 and B2 in some genes, as demonstrated by int SGA, which could lead to the generation of PCR artifacts.
To understand the potential impact of the SI on the host immune system, we analyzed alterations during the follow-up period in the frequencies of the T cell subsets and immune response to HIV peptides in PBMC samples collected at V32010 (prior to SI), V42011 (at the moment of B2 env variant identification after SI onset), V72013 (prior to the first peak of viremia), V92014 (at the first peak of viremia and detection of B1 and B2
env and int variants), V142016 (after viremia control) and V162017 (at the second peak of viremia). Briefly, T cell activation was evaluated by multiparametric flow cytometry by staining the cells with anti-CD3, anti-CD4 or anti-CD8, anti-CD38 and anti-HLA-DR antibodies to determine the frequencies of CD38+HLA-DR+ cells in both CD4+ and CD8+ subsets, as previously described []. In addition, cells were also labeled with anti-CD45RA, anti-CD27 and anti-CD95 antibodies to evaluate the frequencies of naïve (TN; CD45RA+CD27+CD95−), system memory (TSCM; CD45RA+CD27+CD95+), central memory (TCM; CD45RA−CD27+CD95+), effector memory (TEM; CD45RA−CD27−CD95+) and effector (TEFF; CD45RA+CD27−CD95+) T cell subsets.
An increase in the percentage of CD8+CD38+HLA-DR+ T cells was observed in samples from V32010 (6.81%) to V92014 (14%), followed by a decrease at V142016 (6.76%) and a new peak at V162017 (14.40%) (Fig. a). This higher values in the V42011 sample than in the V32010 sample, despite the lower plasmatic viral load, could be suggestive of an association between the SI event and an increase in immune activation. After the emergence of B2, the percentage of CD8+CD38+HLA-DR+ T cells followed plasmatic viral load levels, highlighting the relationship between the antigen viral load and CD8+ T cell activation. Although at more discrete levels, the same trend was also observed for CD4+ T cells (Fig. a). In relation to the CD4+ T cell subsets (Fig. b), we observed a decrease in the frequency of TCM cells between the V42011 and V142016 samples, with the recovery of those cells in the V162017 sample and an inverse pattern observed for TTM cells. The frequency of CD4+ TEM cells reached the highest levels at visits near the detection of the superinfection (V42011) and at both peaks of viremia (V92014 and V162017). For CD8+ subsets (Fig. c), the frequency of TEM and TEFF cells followed the viral load dynamics, which was in contrast with the expected TN cells expansion after the first viral load peak. Despite these variations, no statistical correlations between the frequencies of the different T cell subsets and plasmatic viral load were found during the follow-up.
In addition, we used Gag and Nef HIV-1 peptides matching CTL epitopes, based on the VC06 HLA-B genotype, to evaluate the HIV-specific T cell response by IFN-γ ELISpot assay and intracellular cytokine and CD107 staining. In general, very low or undetectable HIV-1-specific responses were observed, with the exception of the V92014 sample, when approximately 1% of CD107+CD8+ T cells showed detectable Gag- or Nef-specific responses (data not shown). No pattern of increase/decrease in the cytokine response was observed in consequence of the antigenic stimulation related to the viral load peak. |
pmc-6612227-1 | A 41-year old man was admitted to our Institute on July, 2014, because of a white blood cell count (WBC) of 100 × 109/l, a hemoglobin level of 116 g/l and a platelet count of 82 × 109/l. Cytological analysis of peripheral blood and bone marrow (BM) aspirate led to a morphological possible diagnosis of hypo-granular APL. The immuno-phenotype was positive for CD2, CD64, CD56, CD13, CD33, MPO, CD38. Cytogenetic analysis and nested PCR confirmed the presence of PML/RARα fusion gene (BCR3), which was also detected by using fluorescence in situ hybridization (FISH). Based on morphological, phenotypic, molecular and cytogenetic findings, the patient was diagnosed as having an APL “variant” and treated with AIDA 2000 protocol (ATRA and Idarubicin). After hematological and molecular complete remission (CR) were obtained, three following consolidation cycles were administered. On February 2015, however, the patient developed an isolated symptomatic EM relapse in the auditory canal. The patient received FLAG regimen (fludarabine, high-dose cytarabine and granulocyte-colony stimulating factor G-CSF), obtaining a new CR, that is currently maintained, 4 years after the first diagnosis. |
pmc-6612380-1 | A 43-year-old male patient attended a dental clinic with the prosthetic crown of tooth 24 in hand, with the remaining fractured root core, part of which was inside the root canal (Figures and ).
Clinical and radiographic examination revealed the absence of a root fracture, which might preclude maintenance of the tooth (). It was also observed that the root canal had not been submitted to removal of sealing material up to the adequate length of 2/3 of the dental remnant to the root apex [, ]; the cast metallic core was short, which impaired the intraradicular retention.
During clinical examination, it was observed that the dental remnant presented satisfactory conditions for a new rehabilitation with placement of an intraradicular core and a total prosthetic crown. It was proposed to remove the portion of the cast core that was inside the root canal for later accomplishment of a new intraradicular cast core, using the existing metal-ceramic crown as a reverse template for the coronal portion of this future core.
This alternative was possible because there was no need for additional preparation (wear) of the dental remnant at the cervical level, which would impair the adaptation and reuse of the original prosthetic crown. Initially, root canal preparation (buccal and palatal) was performed by instrumentation with Gates Glidden drills at the appropriate length (2/3 of the dental remnant in the largest (palatal root canal), 1/2 of the dental remnant in the other canal (buccal)) [, ].
Then, the prosthetic crown was internally ground with carbide drills to allow coronal adaptation of the new cast core. For that purpose, the prepared root canals were previously isolated with petroleum jelly and filled with fluid acrylic resin, supported by prefabricated resin rods (Pincanal®) in each canal. Polymerization of the acrylic resin was properly controlled to avoid adhesion of the resin pin inside the root canal ().
For preparation of the coronal portion of the core, the prosthetic crown was fitted on the coronal remnant and was internally isolated with petroleum jelly, filled with acrylic resin, and repositioned on the dental remnant with the core in place, thus serving as a reverse template for reconstruction of the coronal portion of this new core (Figures –).
After completion of polymerization, the finished resin core was sent to the prosthesis laboratory for alloy casting (Figures and ).
This new core was properly adjusted to the remaining root, and the adaptation of the metal-ceramic crown on it was also verified (Figures –).
Finally, both pieces (core and crown) were cemented with definitive zinc phosphate cement (Figures and ), and the occlusal contacts were adjusted (). |
pmc-6612390-1 | A 65-year-old woman with multiple liver masses presented for transplantation following a diagnosis of EHE made on biopsy. Preoperative imaging revealed an enlarged liver with multifocal disease throughout the hepatic parenchyma. The lesions showed peripheral enhancement with central hypoattenuation on arterial phase and appeared isodense to the liver parenchyma on delayed phase imaging. Some of the masses showed calcifications (). There was no significant interval change in size in the 10 months from initial diagnosis to transplantation. No chest or pelvic masses were identified elsewhere during the preoperative workup.
Pathologic examination of the liver explant disclosed multiple tan discrete nodules ranging from 0.5 to 3.0 cm in greatest dimension and involving the bilateral lobes. Low power examination revealed 2 main histologic patterns. A subset of the nodules contained well-formed vascular channels and scattered cords of tumor cells embedded in an abundantly sclerotic hyalinized matrix (), while other areas of tumor were characterized by small, irregular central hyalinized scars containing small subtle foci of neoplastic cells and a mild bile ductular reaction (). On high magnification, the tumor cells in both components exhibited mild cytologic atypia and were characterized by abundant pale cytoplasm, nuclei bearing open chromatin and visible though not large nucleoli. Scattered intracytoplasmic vacuoles were noted in the tumor cells arranged in a linear pattern (). Mitotic figures were not observed; however, focal vascular invasion by single tumor cells was noted. There was stromal lymphocytic inflammation, and focal calcification was noted in one of the lesions. The surrounding liver parenchyma had a nodular architecture, reminiscent of focal nodular hyperplasia ().
Immunohistochemically, the tumor cells showed an endothelial phenotype with strong expression of CD31 (clone JC70A; 1/350; Dako) and ERG (clone EPR3864; predilute; Ventana) (). The tumor nuclei were positive for TFE3 (clone MRQ-37; 1/50; Cell Marque) (), but CAMTA1 (polyclonal; 1/300; Novus Biologicals) was negative (). Glutamine synthetase (clone GS-6; 1/2000; Millipore) showed a map-like pattern of distribution in the hepatocytes surrounding small irregular areas of scar mimicking focal nodular hyperplasia ().
RNA sequencing with fusion gene analysis from formalin-fixed paraffin embedded tissue revealed an in-frame YAP1-TFE3 fusion gene joining exon 1 of YAP1 to exon 4 of TFE3 and a reciprocal TFE3-YAP1 joining exon 3 of TFE3 to exon 2 of YAP1, similar to the findings of Antonescu and colleagues [], using a published lab-developed protocol []. No other fusion genes, including WWTR1-CAMTA1, were identified. Fluorescence in situ hybridization (FISH) was performed with lab-developed split apart probes for the TFE3 locus (bacterial artificial chromosome clones RP11-416B14, CTD-2311N12, CTD-3009K20, and CTD-2173N15; all from Abbott Molecular, IL) and dual fusion probes for YAP1-TFE3 loci (bacterial artificial chromosome clones RP11-635D24, RP11-451F15, RP11-90M3, and RP11-750P5 along with the previously mentioned clones for TFE3; all from Abbott Molecular, IL) using standard techniques as previously reported by our group []. Interphase FISH revealed rearrangement of the TFE3 locus in all tumor cells and YAP1-TFE3 fusion at the genomic level (). An image of intact CAMTA1 split apart FISH performed using the same method and probes to the CAMTA1 locus is also shown (). Based on the combination of morphologic, immunophenotypic, and genetic findings, this case was classified as a YAP1-TFE3 rearranged EHE. |
pmc-6612391-1 | The patient #1509232 is a young male with a history of polyuria and polydipsia since he was 11 years old. This symptom was neglected. The patient had a normal academic progression and medical work-up (at age of 14 years and 17 years) excluding several times the diagnosis of diabetes mellitus, as no hyperglycemia has been detected. At the age of 20 years and 5 months he became polyphagic and his weight increased from 60 to 90 kg without any other complaint. Finally, 5 months later, a brain MRI was performed and a large hypothalamus mass (20x20mm) with thickening of the pituitary stalk and compression of the third ventricle was observed. A strong contrast enhancement was observed after administration of gadolinium (Figures , , and ).
The patient was admitted in the department of endocrinology and the biological explorations concluded to a panhypopituitarism associated with diabetes insipidus. At the initial assessment, the following was also discovered: (i) the patient presented stage I obesity (BMI=26.6 kg/m2), (ii) delayed bone age relative to chronological age (resp., 16 years for a chronological age of 20), and (iii) a growth delay with a deceleration of linear growth at age of 15 years.
A stereotactic biopsy of the brain mass was performed at age of 21 years (, M1) and immunohistochemistry showed positive staining for CD1a and PS100, supporting the diagnosis of LCH. Presence of BRAF V600E mutation was explored because this mutation in LCH is associated with more severe disease than did those with wild-type BRAF and irreversible damage, such as neurologic and pituitary injuries []. In addition, presence of BRAF V600E mutation may offer the possibility of a targeted therapy by BRAF inhibitor (vemurafenib or PLX8394, a second-generation BRAF inhibitor) []. Unfortunately, the mutation was not observed and this has limited the possibility to use BRAF inhibitors). Disease work-up failed to find any extra pituitary-hypothalamic extension.
The patient was referred to the oncology department to start vinblastine (one infusion of 10mg per week) and purinethol (100mg per day) according to the French guidelines HL2010 protocol []. He did not receive corticosteroids. The patient signed an informed consent to be enrolled in the French LCH registry according to regulation [].
One month after, the patient was admitted to the emergency room for altered consciousness (, M0). The blood tests showed hypernatremia at 164mmol/l and hyperglycemia at 25.8mmol/l without ketosis. Importantly, blood glucose levels were in the normal range 6 months before and at the time of the brain biopsy. After continuous administration of fluids and rapid acting insulin analogue (124UI/24hr), he was admitted to the diabetology department.
The search for type 1 diabetes (anti-GAD and anti-IA2 antibodies) was negative. C-peptide level was 9µg/l in the fasting state (normal range: 0.8-4.2 µg/l). The lipid profile showed hypertriglyceridemia at 5.7mmol/l (5,4g/l) and low HDL-cholesterol level at 0.41 mmol/l (0.16g/l). Blood pressure was normal at 125/68mmHg. The abdominal CT-scan found liver steatosis without any pancreatic abnormalities. The weight gain of 19kg during the last 3 months (body mass index: 32kg/m2) was linked to eating disorder (especially binge-eating disorder). It was decided to continue the first-line therapy up to 6 infusions of vinblastine.
Intriguingly, soon after the diagnosis of diabetes mellitus, daily insulin needs grew up to 400UI per day (4.2UI/kg), indicating a significant insulin resistance state. We did not observe acanthosis nigricans or loss of subcutaneous tissue and we excluded hypersecretion of hyperglycemic hormones. We tested the hypothesis of antibodies against insulin receptors as a cause of insulin resistance. The screening for serum anti-insulin receptor antibodies was performed using a radioreceptor assay as described [, ]. In summary, Chinese Hamster Ovary cells overexpressing insulin receptor (CHO-IR) were incubated or not for 90 min at 22°C with control and patient's serum at different dilutions, washed, and then incubated for 90 min at 22°C with a tracer concentration of 125I-insulin with or without unlabeled insulin.
The ability of patient's serum to inhibit binding of [125I]insulin was expressed as a percent decrease of insulin binding at 1:3 dilution as compared to the reference value. At the diabetes diagnosis, insulin receptor antibodies were positive (42% decrease in insulin binding) (), allowing the diagnosis of type B insulin resistance, a disease often associated with autoimmune disorders or hematological diseases. We then excluded systemic lupus erythematous, multiple myeloma, and Hodgkin's disease. Interestingly, insulin receptor antibodies levels decreased when first-line therapy was achieved (19% decrease in insulin binding) in parallel to a spectacular decrease in daily insulin needs (). Insulin therapy could be discontinued and replaced by a monotherapy with metformin (850mg twice a day). HbA1c remained stable at 6.5% (48 mmol/mol) until M8. In contrast, no improvement of binge-eating disorder was observed and body mass index kept increasing significantly during this period ().
Seven months after initial diagnosis of diabetes mellitus, intensification of hypoglycemic therapy with sulfonylurea and liraglutide LAR added to metformin was needed because glucose levels increased. The change in glucose levels was observed at the same time as a further increase of serum insulin receptor autoantibodies at M8 and M10 (43 and 42 percent decrease of insulin binding to its receptor upon incubation of CHO-IR cells with patient's serum at 1:3 dilution, resp.) (). Although a significant decrease of the size of the brain mass was observed when compared to initial evaluation, persistence of contrast enhancement after gadolinium administration suggested an incomplete response to therapy (Figures and ).
A second-line therapy was started, consisting in 6 cycles of cladribine (5mg/m2/24h, 5 days/week, each month) from month 12 to month 18 after the diagnosis. At M30, brain MRI showed a decrease of both tumor volume and contrast enhancement (). In addition, glucose levels were controlled with the association of metformin and liraglutide (HbA1c level decreased to 7.5% or 58mmol/mol). Diabetes improvement was associated with a decrease of insulin receptor antibodies levels until M31. Unfortunately, a new recurrence of uncontrolled diabetes (HbA1c: 11.2%, 99 mmol/mol) occurred at M32 needing insulin therapy. Insulin receptor antibodies were once again positive (36% inhibition of insulin binding by patient's serum at 1:3 dilution), while brain MRI did not reveal tumor recurrence.
It was decided to initiate new cycles of cladribine. The patient received a first cycle at M32 after diagnosis and a second one month later. During this period, insulin daily doses decreased from 300UI to 36UI at the end of cladribine cycles. Additionally, HbA1c level decreased from 11.2% to 8.6% (from 99 to 70 mmol/mol). The PET-MRI made during the relapse of diabetes at M32 showed a nodular thickening of the floor of the third ventricle and pituitary infundibulum with no [18F]-FDG-uptake (Figures and ). Control of diabetes was maintained until M35 and PET-MRI was unchanged at that time (Figures and ). The follow-up was marked by a very complex clinical disorder, with behavioural difficulties, polyphagy and agressivity; neurological difficulties, loss of memory; and very instable basal metabolic situation, hyperglycemia, and recurrent hypernatremia episodes. Four years after the diagnosis of LCH, the patients is actually frequently hospitalized in a psychiatric hospital. |
pmc-6612392-1 | The patient, a 71-year-old Caucasian female with prolonged smoking history and inconsistent medical care, presented to our institution via EMS following a mechanical fall she attributed to her chronic right hip pain. She had last been seen in our health system 1.5 years before and had intermittently received care in multiple area healthcare systems. She had known COPD with active smoking, periodontal disease, sick sinus syndrome status postpermanent pacemaker implantation, and urinary frequency. During her admission she was also diagnosed with type 2 diabetes mellitus and congestive heart failure. She had no history of industrial exposures. Her only medications included albuterol and occasional inhaled corticosteroids.
In the ED, workup revealed a large (6.1 × 5.5 cm) fungating right shoulder mass (), and the X-ray of her pelvis showed a pathologic fracture of the right iliac wing. She underwent CT of her pelvis, spine, and right shoulder without contrast, of her head with and without contrast, and of her chest/abdomen/pelvis with PO and IV contrast. These studies were remarkable for a pathologic fracture of the right iliac wing, multiple additional pelvic lytic lesions including the sacrum, right inferior pubic ramus, and left iliac wing, and lytic lesions in the right mandibular condyle, left occipital condyle, left posterior rib, right third rib, and L2 vertebral body, all concerning for metastatic disease. The shoulder CT suggested the overlying mass did not extend into underlying muscle but did identify several subcutaneous satellite nodules. The CT abdomen/pelvis showed left renal parenchymal irregularity suspicious for malignancy and bilateral adrenal nodules concerning for metastases.
On questioning, the patient reported she had fallen due to worsening of her chronic right hip pain. Her daughter reported that her right shoulder lesion had been draining, bleeding, and enlarging for two years but had been present for many years longer and that her mother had been told repeatedly to see a dermatologist but had never followed up.
She was seen by dermatology in the ED, who performed a shave biopsy of her right shoulder mass on hospital day (HD) 1. She was admitted to a medical service with orthopaedic oncology and medical oncology following. Medical oncology felt her most likely diagnosis was renal cell carcinoma but proposed that multiple myeloma or metastatic skin cancer also needed consideration. The pathology from her initial shave biopsy returned as nodular, ulcerated basal cell carcinoma extending to the base of the sections. Because of the small size of her adrenal masses and the inaccessibility of her renal mass, she underwent fine needle aspiration and biopsy of her right iliac crest bony lesion on HD2. The pathology for this was reported as “metastatic carcinoma morphologically consistent with patient's known basal cell carcinoma of the skin” (). Immunohistochemical staining showed the tumor cells were positive for p40, CK 5/6, focally positive for p63, BerEP4, GATA-3, and negative for CK 7, CK 20, CDX2, PAX-8, TTF-1, napsin, and uroplakin II, supporting the diagnosis [].
On HD6, due to the fungating nature of her right shoulder lesion and persistent bleeding, she underwent wide local excision of her shoulder mass. This pathology again showed ulcerated basal cell carcinoma with features of nodular and morpheaform subtypes invading to a depth of 1.0 cm, with negative margins. There was evidence of perineural invasion and one focus suspicious for lymphovascular invasion (Figures –).
In addition to the previously imaged osseous lesions, a technetium bone scan performed on HD4 demonstrated metastatic lesions in the right and left mid femoral shafts (). CT of the right femur without contrast performed on HD9 showed a 4.2 cm lytic metastatic lesion in the posterolateral middiaphysis with associated cortical thinning and endosteal scalloping, involving two-thirds of the cortical thickness. She underwent prophylactic cephalomedullary nailing of her right femur on HD13. She was treated by radiation oncology with 30 gray in 10 fractions to her right hemiplevis and 8 gray to the right femur postoperatively.
Additionally, she underwent biopsy of a suspicious right leg skin lesion on HD8. The pathology was consistent with melanoma.
On discharge from the hospital, the patient followed up with oncology and was schedule for a PET/CT to better assess her adrenal lesions and for wide reexcision of her right leg melanoma. She was to begin a hedgehog inhibitor (vismodegib) after her PET/CT. However, she was lost to follow-up and represented to our ED 7 weeks after discharge with shortness of breath. She was found to be in diastolic heart failure and to have a subsegmental pulmonary embolus and was started on therapeutic low molecular weight heparin. She was also found to have a new right scapula pathologic fracture. She was discharged to inpatient rehabilitation and expired 3 weeks later. |
pmc-6612404-1 | A 20-year-old Saudi male patient not known to have any chronic medical illness was referred to our urology department complaining of chronic left groin pain radiating to the left testis and the medial aspect of the left thigh for the last 6 years. Patient is also complaining of painful ejaculation with no history of lower urinary tract symptoms, hematuria, or trauma. Physical examination was unremarkable. Laboratory tests were ordered including complete blood count and renal function tests which all were within normal ranges. Ultrasound (US) report from the referring hospital mentioned that there is a left pelvic mass. Computed tomography (CT) and magnetic resonance imaging (MRI) showed diffuse distension of left seminal vesicle (9 X 7 cm) cyst () with ipsilateral left renal agenesis () which corresponds to Zinner syndrome. Left seminal vesicle (SV) cyst excision through a low midline incision was done. After general anesthesia and drabbing, a low midline incision was done with a transperitoneal approach, then retracting the intestine exposing and dissecting the right seminal vesicle cysts with release of the adhesion from the pelvic side wall reaching the level of the vas then ligations of both the distal end of SV and vas while preserving the contralateral vas and SV. Haemostasis was done with the insertion of a pelvic drain and closure of the wound in layers. The patient was asymptomatic during his follow-up in our clinic with disappearance of the pain. |
pmc-6612407-1 | A 7-year old girl presented to emergency room (ER) with bilateral elbow pain and swelling one hour after a fall from a swing on her outstretched hands. She had been previously healthy, and had no previous history of fractures. On examination, she had normal vital signs and body built. No dysmorphic features were noticed. She had bilateral elbow swelling with ecchymosis, but no wounds were noticed in her arms, and no features of compartment syndrome were observed. Distal pulses were palpable and sensory and motor examination of median, ulnar and radial nerves were normal.
Both arms were splinted in ER. X-rays showed bilateral isolated Gartland IV SCH (). She was taken to the operating room 4 hours after presentation for close reduction. The fracture was highly unstable; therefore, we decided to fix it with 4 k wires. For each side, we entered 3 wires laterally and 1 medially (). We applied a backslap for each side. Distal pulses and neurological examination postoperatively were normal. She was maintained on a good analgesic control. She was discharged home after 2 days, during which she had underwent serial clinical examinations for compartment syndrome and X-rays to ensure correct positioning of the 4 k wires and rule out fracture displacement.
Patient was seen at our Orthopedic outpatient clinic 3 weeks later. She had no local infection or fracture displacement, and range of motion was decreased. Therefore, the 4 k wires were removed ().
6 months follow up shows normal full range of motion of the bilateral elbow joints with completely healed fractures (). |
pmc-6612414-1 | Our patient, a Japanese woman aged 42 years and 6 months, visited our dental hospital with a chief complaint of impaired masticatory function due to anterior open bite. She also experienced pain in the TMJ while chewing and mouth opening. Her open bite had worsened gradually and she also had tongue thrust. She was previously recommended orthodontic treatment with orthognathic surgery by an orthodontist, but she did not want to undergo the surgery.
Her pretreatment facial appearance revealed a convex profile, and suggested hypermentalis activity associated with lip closure. The initial intraoral photographs revealed a − 6.0-mm anterior open bite and occlusal contact between only the second molars. The right molar occlusal relationship was class II, while that of the left was a more severe type of class II. There was crowding in either arch and the upper dental midline had shifted to the right by 4 mm. The lower midline coincided with the facial midline (Figs. and ). A panoramic radiograph revealed the existence of three third molars except the upper right third molar (Fig. ). A severe skeletal class II relationship of angle of point A-nasion-point B (ANB), 11.5° and a steep Frankfort-mandibular plane angle (FMA, 47.5°) with lingual inclination of upper incisors of upper incisor-Frankfort plane angle (U1-FH), 104.6° was noted in the cephalometric measurements (Table ). The DDwoR of both TMJs was evident in the magnetic resonance imaging (MRI) images (Fig. a, b). Schüller’s view showed flattening of both condyles, but there was no restriction of jaw movement (Fig. a, b). Based on this information, our patient was diagnosed to have a skeletal class II open bite with TMD. Informed consent was obtained from all individual participants included in the study.
A stabilization occlusal splint was used before the orthodontic treatment to reduce the TMJ pain on masticatory movement. Because our patient refused surgical treatment, it was decided to correct the anterior open bite and achieve an ideal occlusion with class I molar relationship via orthodontic treatment alone (without orthognathic surgery). The treatment plan was as follows.Extraction of the maxillary right and left first premolars Extraction of the mandibular right and left second premolars. Insertion of miniscrews into the palatal region and left alveolar bone of mesial part of first molars to intrude the maxillary molars and to avoid anchorage loss. Correction of crowding and distal movement of anterior teeth. Extraction of the maxillary left first molar to correct the midline and left molar relationship. Preparation and insertion of a retainer with tongue crib to avoid tongue thrust.
Before the orthodontic treatment, a stabilization occlusal splint was placed for 3 months to reduce the TMJ pain. After our patient confirmed alleviation of TMD symptoms, the four premolars were extracted and 0.018-inch (0.457-mm) standard edgewise brackets were bonded on the maxillary and mandibular teeth. This was followed by placement of two self-drilling titanium alloy miniscrews (2.0 mm in diameter and 6 mm in length, Dual Top Auto Screw; Jeil Medical Corp., South Korea) into the palatal region of the maxillary first molar, under local anesthesia, to intrude the maxillary posterior teeth. A transpalatal arch was also placed in order to maintain the maxillary molar width (Fig. ). The molars were connected by an elastic chain to intrude them and to avoid the loss of anchorage. Distal movement of the maxillary canines and mandibular first premolar was then initiated.
After 24 months, the extraction spaces of premolars had closed. The maxillary dental midline had shifted to the right by 3 mm. However, the left canine and molar relationship continued to be class II, while the right side had nearly progressed to a class I relation (Fig. ). Hence, the maxillary left first molar was extracted to correct the midline and the left molar relationship. The maxillary left premolars were moved distally using a miniscrew (1.6 mm in diameter and 8 mm in length) which was inserted into the maxillary left buccal alveolar bone.
After 36 months, the anterior open bite was corrected to 1.5 mm and the teeth had attained a class I molar relationship (Figs. and ). All the orthodontic appliances and miniscrews were then removed, and lingual bonded retainers were affixed on both arches. In addition, a Begg-type retainer with tongue crib was also affixed in the maxilla to avoid tongue thrust. Our patient did not have any TMD symptoms during the active treatment and the retention period. The occlusion continued to be stable at 24 months from the initiation of the retention.
After orthodontic treatment, the overbite increased to 1.5 mm, while the molar and canine relationships changed to class I on both sides. The maxillary and mandibular dental midlines coincided, and the arch alignment was well corrected. There was no major variation in the posttreatment facial profile compared with the pretreatment profile. Although, the posttreatment panoramic radiograph suggested minor root resorption of the maxillary incisors, all roots were aligned in parallel (Fig. ). On the cephalometric pretreatment and posttreatment superimposition, the upper and lower anterior teeth were distalized by 5 mm and 3 mm, respectively, and extruded by 2 mm and 3 mm, respectively. The maxillary molars were intruded by 1 mm (Fig. ). The ANB angle had changed from 11.5° to 10.1°, and the FMA had changed from 47.5° to 46.5° (Table ). Posttreatment Schüller’s view revealed no change in condyle shape and jaw movement (Fig. a, b). Our patient did not experience any symptoms of TMD, such as pain on mouth opening, during the orthodontic treatment. |
pmc-6612656-1 | A two year-old female patient underwent exploratory laparotomy with end sigmoid colostomy after a penetrating rectal injury. The intraoperative and early postoperative periods were uneventful. After ten days following the surgery, the child presented three times with a picture of intestinal obstruction. However, at each time, she was admitted for two days, treated conservatively, and improved. One month following the surgery, she developed diffuse severe colicky abdominal pain with vomiting, diarrhea and abdominal distension. Abdominal standing x-ray showed multiple air fluid levels with dilated loops. She was taken to the operating room and underwent a laparotomy and found an ileoileal intussusception. Manual reduction and resection of the necrotic part were performed. The child’s postoperative course was uneventful. |
pmc-6612656-2 | A five-month-old female underwent surgical reduction of idiopathic ileocolic intussusception (). Initially, the infant did not tolerate the slow advancement of her diet and this was managed as postoperative ileus. The infant developed bilious vomiting on postoperative day seven. Her abdominal x-ray showed multiple dilated bowel loops. Abdominal ultrasound showed ileoileal intussusception. At laparotomy, an ileoileal intussusception was identified and reduced manually, with resection of a short necrotic segment (). Subsequently, the infant did very well and was discharged home. |
pmc-6612656-3 | A seven month-old male with Hirschsprung’s disease, underwent a loop ileostomy at the age of 12 days due to intestinal perforation. At the age of three months, the infant presented with prolapse of his ileostomy necessitating revision of the ileostomy. Two months following laparotomy, the infant developed a prolonged course of watery diarrhea and malabsorption with poor weight gain. Cow's milk protein allergy was suspected and formula was changed with no improvement. At the age of seven months, the infant underwent pull through procedure. An ileocolic intussusception was identified incidentally and managed by manual reduction. The infant’s stool production and intestinal absorption normalized after the reduction. His postoperative course was uneventful. |
pmc-6612656-4 | A six month-old male with Hirschsprung’s disease, underwent laparotomy with leveling colostomy. Three days after the operation, the infant developed abdominal distention with bilious vomiting. Plain abdominal x-ray was performed and showed multiple air fluid levels with dilated bowel loops. The infant failed to improve with conservative therapy. The patient was taken back to the operating room five days after the initial laparotomy and an ileoileal intussusception was identified and reduced manually. The infant progressed well postoperatively and was discharged home at POD5.
, summarize the clinical details and symptoms and signs experienced by our patients. Unfortunately, we did not perform genetic testing for any patient. |
pmc-6612657-1 | A 63 years old lady carried out a complex re-laparotomy for a postoperative small bowel occlusion after a radical cystectomy because of urothelial carcinoma. The procedure lasted 3 h because of thick adhesions that needed to be cleared. At the end of the procedure, total blood loss was 2 litres, and two Units of Packed Red Blood Cells (PRBC) were infused. During the surgical procedure, a central venous catheter was placed in the internal jugular vein by ultrasound-guided puncture of the vessel, but an inadvertent puncture and cannulation of the right subclavian artery occurred before catheter placement. Because of the ultrasound-guided procedure, the anesthesiologist thought to have cannulated the carotid artery and applied local pressure for a few minutes. Two hours later, when the patient was in the Intensive Care Unit (ICU) around midnight, a chest X-ray to check the correct position of the central venous catheter revealed a massive hemothorax (), while the patient was hypotensive and responder to crystalloids and blood infusions. A chest drain was inserted without any substantial output but a small amount of clotted blood. The patient rapidly worsened, despite appropriate resuscitation with 10 U of PRBC, 8 U of Fresh Frozen Plasma (FFP), 3 U of cryoprecipitate and 1 U of platelets from apheresis. A second chest X-Ray to check tube position revealed a tension hemothorax (). The team in charge of the patient, comprising the anesthesiologist that did the general anaesthesia for the surgical procedure, made the diagnosis of suspected subclavian artery perforation and tension hemothorax with both hypovolemic and obstructive shock. The team decided to bring the patient in the hybrid room to control the likely bleeding for the right subclavian artery. While the patient has been positioning on the angiographic table, the ECG monitor showed severe bradycardia, with a heart rate of 30 and an impending cardiac arrest with a systolic blood pressure of 30 mmHg. An emergency thoracotomy to decompress the right chest was then performed via a V space incision, and five litres of blood under pressure were drained from the right pleural space, with a rapid improvement in the vital signs. During the endovascular procedure, the general surgeon left the thoracotomy open to allow a continuing suction in the pleural space to prevent the accumulation of clots. The endovascular procedure was conducted by the vascular surgeons and lasted 40 min, confirming the massive leak from a significant defect in the subclavian artery () and consisted in a 7 x 37 mm covered stent placement in the subclavian artery at the origin of the vertebral artery. The stent was expanded with a balloon taking care of not injuring the already damaged vessel (). The patient had a transient improvement, but in the next few hours, a hemodynamic instability again occurred, even if responsive to blood infusion. A CT scan revealed a leakage from the stent, because of retrograde revascularization of the vertebral artery (). A new endovascular procedure, with more pronounced balloon dilatation of the stent, definitely controlled the bleeding. ) The patient slightly improved after an open surgical debridement of the pleural space from clots and blood, given the absence of an output from the two large drains that were in place. In the next few days, the patient went back to the ward and made a gradual full recovery with no neurological or vascular defects (). |
pmc-6612668-1 | A 33-year-old morbidly obese female underwent an elective LC and was diagnosed with an iatrogenic BDI on post-operative day 22. She was taken for an exploratory laparotomy with washout and drainage and referred to our unit for further management five days later. Cross-sectional imaging confirmed a complete transection of the extrahepatic bile duct with 10 mm loss of substance. Due to uncontrolled sepsis the decision was made to defer definitive treatment. An ERC was performed that showed extravasation of contrast into the subhepatic space and no filling of the proximal bile ducts. After placement of a transhepatic drain an extraluminal rendezvous procedure was performed and a 10 × 80 mm SEMS placed, bridging the defect (). |
pmc-6612668-2 | A 40-year-old male presented with a trans-axial thoraco-abdominal gunshot wound. He was hemodynamically unstable, and a damage control laparotomy was performed. Gastric and diaphragmatic injuries were repaired and a grade IV liver injury was packed []. The packs were removed after 24 h and a closed suction drain was left in the subhepatic space. Six days after laparotomy, CT abdomen showed non-perfusion of liver segments 2 and 3, a large central intrahepatic hematoma and a subhepatic collection. A percutaneous ultrasound-guided puncture of the collection returned bile and an 8 Fr pigtail drain was placed. He subsequently developed a persistent bile leak and rising serum bilirubin (13–38 mmol/L). ERC demonstrated extravasation of contrast into the subhepatic space and no filling of the proximal bile ducts. MRCP showed complete disruption of the extrahepatic bile duct but an intact confluence. A PTC was performed noting a porto-biliary fistula and an 8 Fr PTBC was positioned into the subhepatic space. At extraluminal PTC/ERC rendezvous a 10 × 80 mm fully covered SEMS was placed, bridging the defect (). The patient developed haemobilia 48 h later. Angiography showed a bleeding right hepatic artery false aneurism successfully managed with an endovascular stent. |
pmc-6612705-1 | In November 2017, an asymptomatic 14-year-old boy referred to the authors’ hospital for management of right lateral chest wall mass discovered incidently one month earlier. There was no history of trauma. Clinical examination was unremarkable. Plain chest radiography showed a smooth-walled 6.0 × 3.5 cm homogenous right lateral chest wall mass (A). Computed tomographic (CT) scan revealed right lateral extrapleural soft-tissue mass 6.5 × 4.0 × 5.6 cm in size abutting 5th and 6th ribs and filling the 5th intercostal space (B). There were neither cavitation nor calcification within the mass, and the adjacent ribs were not eroded.
Magnetic resonance imaging (MRI) revealed right extrapulmonary and extrapleural chest wall soft tissue mass. Tl-weighted MRI demonstrated a solid mass with a low signal intensity (C), while T2-weighted MRI demonstrated a homogeneous mass with high signal intensity similar to that of fat tissue (D). Early phases after administration of contrast, showed characteristic heterogeneous enhancement of the tumor that became more uniform during the delayed phase (E and F).
The angiogram showed a sizeable vascular mass supplied by a branch of the internal mammary artery. That branch was subsequently embolized with Gelfoam pledgets with no further filling on the post-embolization arteriogram.
The patient underwent an exploratory video-assisted thoracoscopy (VAT) through the right 8th intercostal space anterior axillary line (A and B). Posterolateral thoracotomy revealed well-demarcated mass abutting the 5th and 6th ribs filling the right 5th intercostal space. En bloc resection of the mass involved ribs and the intercostal muscles were performed (C). Chest wall defect was reconstructed using polytetrafluoroethylene (PTFE) patch (GORE-TEX®, DUALMESH®, W. L. Gore & Associates) (D).
The surgical specimen measured approximately 6 × 4 × 3.5 cm, which included a hemorrhagic, highly vascular, mass that extended from the intercostal muscles to the extra-pleural space (E). Cut section revealed whitish homogenous material. Microscopically, there were homogeneous thick and thin-walled blood vessels with proliferating vascular spaces of capillaries of the cavernous type with intervening fibrous stroma (F). There was no evidence of recurrence at 3 and six months of follow-up. |
pmc-6612968-1 | A 28-year-old woman (1.65 m, 85 kg, BMI 31.2) was referred to our department five days postpartum with a bilateral femoral neck fracture.
During the last two months before the delivery of her first child (begin with the sixth month of gestation), she had complained about increasing weakness and pain in both hips and thighs. In the last two weeks of her pregnancy (begin of the 35th week of gestation), she had to walk on crutches, as she was unable to bear her full weight due to the severe pain in her hips. During this time she took paracetamol daily. Her treating gynaecologist did not initiate any clarification of the symptoms by radiologic imaging methods in this period. Considering her severe pain, it was decided to deliver the child per elective caesarean section (37th +4 week of gestation). Three days postpartum, after being released from the hospital, she had an epileptic seizure and fell. She was admitted to a neurological department of another hospital. During her two-day stay in this department, the woman complained of severe pains in both hips. X-rays and a computer tomography (CT) of the pelvis were performed, revealing bilateral femoral neck fractures. Following this diagnosis, the patient was referred to our department for further treatment.
Regarding her past medical history, she had childhood migraine and epilepsy. She suffered from her first epileptic seizure at the age of 15. She was treated with valproate over 11 years and remained seizure-free during that time. Her treating neurologist stopped the medication two years prior to her pregnancy. The patient did not have any other comorbidities. Particularly, she did not report on any past history of fractures, irregular menstruation, or family history of osteoporosis.
At presentation in our department, the clinical examination showed massively reduced hip movement on both sides because of severe pain. Serum laboratory examination was normal.
Based on the age of the patient, the limited bone quality, the age of the fractures, and their displacement (Figures and ), it was decided to perform a bilateral hip joint replacement with the use of short-stemmed prostheses (). There were no intra- or postoperative complications. Both femoral heads were sent for further histopathological examination. The results of both femoral heads revealed fracture areas with hemorrhagia, proliferation of fibroblasts in the marrow cavities, and formation of focal woven bone as a sign of the bone remodelling, being indicative for a TOH. Postoperatively, the patient was mobilized on crutches under full weight bearing of the operated extremities. After consultation with our Department of Neurology, the patient restarted her valproate medication. Due to this medication, the patient was advised not to breastfeed her child. Since the TOH is regarded to be a self-limiting disorder, no specific osteoporotic treatment was applied. The further course was uneventful, and the patient was dismissed after two weeks.
At 1-year follow-up, the patient is free of any complaints and has no limitation in the range of motion of both hips (extension/flexion 0°-0°-100°, abduction 50°). |
pmc-6612972-1 | A 36-year-old female had photopsia on her left eye for ten days. Furthermore, she noticed a dark spot on the nasal side of the left eye. Two weeks earlier, she had suffered from influenza-like symptoms. There was no history of previous general or eye disease, neither operations nor treatments. |
pmc-6612976-1 | A 20-year-old man with both upper limb disabilities admitted to our hospital for mandatory health screening before military service. He had no health complaints other than the skeletal disorder involving his upper limbs. In detailed questioning, he stated that he had four fingers in his left foot. He is the last of six children born to nonconsanguineous healthy parents. All of his brothers and sisters are completely healthy. There is no family history of any kind of congenital skeletal abnormalities in the extended family. His mother was at the age of 32 when she gave birth to our patient. There is no history of any drug, smoke, alcohol, or radiation exposure during pregnancy. Our patient was born uneventfully at full-term through normal vaginal delivery. No other significant health problem is present in the history of his childhood.
On physical examination, all skeletal elements beyond his left elbow and right wrist, and the fifth finger of his left foot were found to be absent (). He had oligodactyly of the left foot (). Anteroposterior radiograph of the right arm depicted the absence of the hand with well-developed radius and ulna. There was a rudimentary bone of about 1 cm in the medial neighborhood of the distal ulna (). Anteroposterior radiograph of the left arm demonstrated that the proximal forearm segment participating in the elbow joint structure was small but present, while the radius and ulna distal to this point were absent (). Anteroposterior, lateral oblique, and mediolateral radiographs of the left foot revealed absence of the fifth finger and the lateral cuneiform. The second to fourth fingers were short. The number, size, and joint relations of the other bones forming the foot were normal (). Magnified anteroposterior and lateral oblique radiographs of the left foot showed that the middle phalanges of the second and third fingers were shorter than the distal phalanges, and the middle phalanx of the fourth finger was absent ().
The patient was then referred to the Department of Orthopedic Surgery to identify the best prosthetic fitting option and to provide a convenient training regimen. |
pmc-6612987-1 | A 70-year-old woman presented to the emergency room of a university hospital with severe right upper quadrant abdominal pain without any radiation. It was associated with fever, nausea, and several episodes of vomiting for the past 4 days. There was no association of pain with the intake of food. She had not passed stool for the last 3 days and also complained of abdominal fullness for the past 2 days.
On examination, the patient appeared drowsy, ill-looking with a pulse of 78 bpm, blood pressure of 140/100 mmHg, respiratory rate of 18 min−1, a temperature of 100°F, and oxygen saturation of 74% under room air. The patient was anicteric. Voluntary guarding was present on abdominal examination and tenderness was present on the right upper quadrant. Murphy's sign was positive. Normal bowel sounds were audible. Systemic examinations were within normal limits. She had no other comorbidities.
Laboratory investigation revealed neutrophilic leukocytosis with total leukocytic count 12600 mm−3 and 82% neutrophil. Creatinine level was raised (260 μmol/l). Liver function tests, serum amylase and lipase, hemoglobin, platelets, and coagulation profile were within normal limits. Arterial blood gas showed metabolic acidosis (pH 7.21, HCO3 17.4, pCO2 43.5, BE -9.1, and Lac 0.6). Ultrasonography revealed multiple cholelithiasis with distended gallbladder, and a pericholecystic collection was noted.
She was admitted with the diagnosis of severe acute cholecystitis and started on ceftriaxone and metronidazole along with other supportive medications.
On the 2nd day of admission, her urine output dropped and she became oliguric. Her blood pressure and pulse escalated up to 170/100 mmHg and 100 bpm, respectively. She was started on amlodipine 10 mg and intravenous labetalol. Investigations revealed deteriorating renal function with creatinine 416 μmol/l along with worsening metabolic acidosis (pH 7.18). She was shifted to the intensive care unit and was started on imipenem/cilastatin.
On the 3rd day of admission, she was intubated for severe respiratory distress with worsening metabolic acidosis. Her blood pressure kept on rising (SBP up to 200 mmHg, DBP up to 115 mmHg) and was started on intravenous glyceryl trinitrate (GTN) infusion. Her hematological parameters were similar except for the declining level of platelets (93000 mm−3) on the 3rd day of admission. Urgent cholecystostomy was performed, and 100 ml of nonpurulent collection was drained and sent for culture. We identified purpuric rashes as well as few maculopapular rashes on the anterior chest and abdomen after she was admitted ().
On the 5th day, development of new onset rash and an epidemiological clue led us to the workup for scrub typhus. However, eschar was not present. A rapid IgM antibody detection test performed by an InBios Scrub Typhus Detect™ test kit was positive for scrub typhus. Meanwhile, culture report showed no growth. The patient was started on doxycycline 100 mg twice daily on the same day. She underwent one cycle of hemodialysis for raised creatinine level and worsening metabolic acidosis.
On the 6th day, the patient showed signs of improvement. Her hematologic parameters started improving. She was extubated and was shifted to intermediate critical care on the 6th day. Two days later, she was shifted to the general ward and was discharged on the 10th day of her admission. She underwent laparoscopic cholecystectomy 8 weeks later which revealed multiple calculi on her gallbladder (). Her follow-up was uneventful. |
pmc-6612990-1 | A 56-year-old man was referred to our internal medicine unit with abdominal pain, fatigue, and persistently elevated aminotransferases for 18 months. Three months earlier, he had been evaluated for numbness and weakness starting over the distal aspects of his four limbs and slowly progressing proximally over the last three years. Neurological examination performed at that time revealed walking difficulties and moderate muscle weakness in both lower and upper limbs (F = 3.5-4), generalized tendon areflexia, and mild sensory loss with stock and glove distribution. Electromyography/electroneurography (EMG/ENG) showed diffusely reduced motor and sensory nerve conduction velocity (mean motor nerve conduction velocity 22 m/s), with a dishomogeneous pattern, and absent F waves. Isolated hyperproteinorrachia (1.15 g/L) was found on cerebrospinal fluid (CSF) examination. The clinical, EMG/ENG, and CSF results were consistent with a diagnosis of CIDP, which was subsequently successfully treated with IV immunoglobulins.
On admission, the patient's temperature was 36°C, heart rate 125 beats per minute, blood pressure 110/80 mmHg, and oxygen saturation 94% while he was breathing ambient air. On physical examination, peripheral edema, bibasal pulmonary rales, hepatomegaly, and severe sensory and motor deficits located to the upper and lower limbs were noted. Laboratory data on admission showed the following values: aspartate aminotransferase (AST) 166 IU/L (normal range 17-59 IU/L), alanine aminotransferase (ALT) 64 IU/L (normal range 21-72 IU/L), gamma glutamyl-transferase 358 IU/L (normal range 15-73 IU/L), alkaline phosphatase 173 IU/L (normal range 38-126 IU/L), total bilirubin 1.28 mg/dL (normal range 0.20-1.30 mg/dL), albumin 2.8 g/dL (normal range 3.6-5.5 g/dL), lactate dehydrogenase 993 IU/L (normal range 313-618 IU/L), total serum calcium level 14.1 mg/dL (normal range 8.8-10.2 mg/dL), phosphorous 2.8 mg/dL (normal range 2.9-4.8 mg/dL), C-reactive protein 14.6 mg/dL (normal range < 1.0 mg/dL), and ferritin 1669 ng/mL (normal range 20-325 ng/mL). Viral markers for hepatitis B and C were negative. Serum intact PTH was low (4 pg/mL) (normal range 20-104 pg/mL). Moreover, anti-ganglioside antibodies (GD1bIgG and GM1IgG) were found in serum. Given the predominantly infiltrative pattern of altered liver function tests and the severe hypercalcemia with low serum intact PTH, imaging studies and further blood tests were performed to rule out cancer or metastases.
Whole body multidetector computed tomography (MDCT) scan showed a large hypoattenuating hepatic mass in the right lobe (maximum axial dimension 13 cm) characterized by heterogeneous peripheral enhancement, associated with secondary lesions located in the spleen and lung (more than 30 nodules); no evidence of bone metastasis was found (). The following magnetic resonance imaging, performed to confirm the diagnostic hypothesis from the previous CT study, showed typical findings of peripheral CHCC (). Alpha-phetoprotein was 247.8 ng/mL (normal value < 9 ng/mL), gastrointestinal cancer antigen (GICA) was 132 U/mL (normal value < 37 U/mL), and carcinoembryonic antigen (CEA) was 9.14 ng/mL (normal range 0-5 ng/mL). PTH-rP was markedly high (147 ng/mL; normal range 8.5-20.0 ng/mL).
Percutaneous ultrasound-guided biopsy of the hepatic tumor showed a mixed epithelial neoplasia comprising: (1) trabeculae and solid nests composed of large cells with pleomorphic nuclei and granular cytoplasm intermingled with (2) branching pseudoglandular structures composed of cuboidal/columnar cells with atypical nuclei. Nests and trabeculae showed strong immunohistochemical staining for cytokeratin 8/18 and HepPar1 while pseudoglandular structures stained selectively for cytokeratin 7 and cytokeratin 19 (). A diagnosis of “Stage IV CHCC associated to HHM and CIDP” was made.
The general status of patient rapidly worsened, and he became bedridden soon thereafter. After discussion with the patient about the prognosis of his disease, he declined further treatments and arrangements for hospice care were made prior to discharge. |
pmc-6613033-1 | An eight-year-old, spayed female Golden Retriever weighing 32 kg was presented to the University of Tennessee Veterinary Medical Center (UT-VMC) for evaluation of an intra-abdominal mass and hypercalcemia. The dog was spayed 6 years previously and reportedly normal until 10 days prior to presentation, when the dog was evaluated by the referring veterinarian for lethargy. A large cystic mass measuring 10 cm x 7 cm x 3 cm was noted on the right flank, which was drained by the referring veterinarian. This mass had reportedly been present for approximately one year prior to presentation. The dog was treated empirically with cephalexin at 22 mg/kg PO q12h (AmerisourceBergen, Chesterbrook, PA, USA). The lethargy resolved, and the dog was clinically normal at the time of presentation to UT-VMC, aside from reportedly licking and biting at its right flank. Physical examination revealed persistence of the reported subcutaneous cystic mass on the right flank as well as a firm, nonpainful intra-abdominal mass caudal to the right kidney.
Hematology was unremarkable. Biochemistry revealed mild total hypercalcemia (13.3mg/dL; reference range 10-12 mg/dL), normal phosphorus (2.8 mg/dL; reference range 2.5-5.9 mg/dL), mildly elevated creatinine (1.3 mg/dL; reference range 0.3-1.1 mg/dL), normal BUN (16 mg/dL; reference range 7-37 mg/dL), and mild hyperglobulinemia (4.1 g/dL; reference range 1.9-3.1 g/dL). Urinalysis revealed isosthenuria but was otherwise unremarkable. A hypercalcemia of malignancy profile was performed at the Michigan State University Veterinary Diagnostic Laboratory where these tests had previously been validated, revealing marked ionized hypercalcemia (1.75 mmol/L; reference range 1.26-1.39 mmol/L), a plasma PTH concentration below the reference range (0 pmol/L; reference range 0.5-5.8 pmol/L), and normal plasma PTHrP concentration (0 pmol/L; reference range 0.0-1.0 pmol/L). Serum concentration of 1,25[OH]2D was normal (97 nmol/L; reference range 60-125 nmol/L).
Radiographic (Super 80CP, Philips Medical Systems, Bothell, WA) findings included two large right caudodorsal abdominal soft tissue opaque masses in close proximity to one another, with evidence of mild fluid streaking of the fat surrounding both structures (). The first mass was located within the subcutaneous tissues of the right caudodorsal abdominal wall and was associated with focal medial deviation of the abdominal wall. The second was within the right caudodorsal abdominal cavity, caudal to the right kidney. It could not be determined whether the abdominal mass was associated with the body wall mass or whether these represented two distinct processes. Differential diagnoses for the intraabdominal mass included a granuloma, hematoma, or neoplasia, possibly originating from the mesentery or a regional lymph node. Differential diagnoses for the abdominal wall mass included benign or malignant etiologies such as a granuloma, abscess, or a sarcoma.
Abdominal ultrasound (Epiq 5, Philips Ultrasound, Bothell, WA, USA) was performed next to further characterize the identified masses. The sonographic examination was performed with the patient in dorsal recumbency using a microconvex 8 MHz transducer, a convex 9 MHz transducer, and a linear 12 MHz transducer. A heterogeneous, non-organ associated abdominal mass with strongly hyperechoic, shadowing foci within its center was identified in the right caudal abdomen (). This mass had multiple finger-like hyperechoic projections extending laterally and caudally, connecting it with the large extra-abdominal, cystic mass in the right lumbar region. The intra-abdominal mass was moderately vascularized when interrogated with color Doppler (), with evidence of multiple relatively large, slightly tortuous, branching intralesional blood vessels. Given the sonographic appearance of these lesions, differential diagnoses included malignant neoplasia, such as sarcoma or carcinoma, or a granulomatous inflammatory process (such as secondary to a chronic foreign body or fungal infection). Ultrasound-guided fine-needle aspiration of the abdominal mass was performed to obtain tissue samples for cytologic analysis, which was consistent with pyogranulomatous inflammation.
CT (Brilliance, Philips Medical Systems, Cleveland, OH, USA) of the abdomen was performed using a 40-slice helical scanner for further characterization of the relationship between the intra-abdominal and extra-abdominal lesions, in preparation for surgical excision. A submillimeter dataset of the abdomen was acquired and images were reconstructed in 0.9mm, 1.5 mm, and 5 mm slice thickness utilizing bone and soft tissue algorithms. The acquisition was repeated following intravenous administration of Ioversol 350 mgI/ml, a nonionic iodinated contrast medium (Tyco Healthcare/Mallinckrodt, Milwaukee, WI, USA) at a dosage of 2.2 mg/kg IV. A well-defined, heterogeneously contrast-enhancing, thick-walled, cavitary, soft tissue attenuating, abdominal mass was present caudal to the right kidney (). This mass was confluent with the distal tip of the right limb of the pancreas () and intimately associated with a small intestinal segment. Despite its close association with the small intestine and pancreas, this mass was not centered on these structures and therefore was most consistent with a non-organ associated abdominal mass with secondary involvement of adjacent abdominal organs. A few pinpoint mineral attenuating foci were noted within this mass. A large, rim enhancing, cystic subcutaneous mass was also identified in the right lumbar subcutaneous tissues, resulting in focal medial displacement of the abdominal wall (). In addition, ill-defined, peripherally contrast-enhancing tracts were seen extending through the right hypaxial musculature from the level of the midbody of the L4 vertebra to the level of S3 (). Both masses and the peripherally contrast-enhancing tracts in the hypaxial musculature were all interconnected by thick, peripherally contrast-enhancing soft tissue attenuating stalks (). Given the confirmed connection of the two masses, extra-abdominal extension of an intra-abdominal mass with multiple fistulous tracts led to a primary differential diagnosis of a granulomatous inflammatory process, such as secondary to a chronic foreign body. An aggressive soft tissue neoplasm (i.e., soft tissue sarcoma) was also considered.
An exploratory laparotomy was subsequently performed. The intra-abdominal portion of the mass was large (7.5 cm x 4.5 cm) and nonresectable because it was highly vascularized with extensive adhesions to the colon and omentum. The thick, fibrous capsule was incised to reveal fluid and a 4-inch x 4-inch gauze, most likely from the ovariohysterectomy 6 years earlier. A wedge biopsy of the mass was obtained for histopathological analysis and culture. The mass was lavaged, omentalized, and sutured closed. The abdomen was flushed with sterile saline and closed. A stab incision was made into the second subcutaneous cystic mass dorsolateral to the incision site. The content of this cystic mass was drained using suction. A Jackson-Pratt drain (Cardinal Health, Waukegan, Illinois, USA) was subsequently placed into this structure and secured in place for 24 hours. Approximately 240 mL of fluid was drained from the extra-abdominal portion of the mass.
Histopathology of the biopsy sample from the intraabdominal mass revealed chronic, fibrosing, pyogranulomatous and lymphoplasmacytic fasciitis with marked pancreatic atrophy and loss. This was consistent with a gossypiboma which had incorporated and replaced portions of the pancreas, likely the most distal aspect of the right pancreatic limb which was intimately associated with the mass on presurgical imaging. Aerobic and anaerobic culture of a portion of the biopsy sample revealed Streptococcus agalactiae from broth only, and 2 colonies of Staphylococcus sp. No growth was seen on fungal culture after 5 weeks.
Postoperatively, the dog was treated supportively with intravenous fluid therapy, a fentanyl/lidocaine constant rate infusion (AmerisourceBergen, Chesterbrook, PA, USA) at a dosage varying between 2 and 5 mcg/kg/hr, ampicillin (AmerisourceBergen, Chesterbrook, PA, USA) (705 mg, IV, q 8 h), and zoledronate (Novartis Pharmaceuticals Corp, East Hanover, NJ) at a total dosage of 4 mg IV given once. Ionized calcium normalized within 48 hours (1.34 mmol/L; reference range 1.26-1.39 mmol/L). The dog recovered uneventfully and was discharged 24 hours following surgery with tramadol (AmerisourceBergen, Chesterbrook, PA, USA ) at a dosage of 3 mg/kg PO q12h for 5 days, and amoxicillin (Zoetis, Parsippany, NJ, USA) at a dosage of 24 mg/kg PO q 12 h for 10 days.
The dog was reevaluated approximately 3 months following surgery. Clinically the dog was normal and normocalcemic (ionized calcium 1.32 mmol/L; reference range 1.26-1.39 mmol/L). Abdominal CT was repeated prior to and following intravenous contrast medium administration using the same imaging parameters as the initial examination. The soft tissue attenuating mass within the right caudal abdomen had markedly decreased in size and was less contrast-enhancing, with persistence of a non-contrast-enhancing hypoattenuating center (), and few pinpoint, mineral attenuating foci within it. The thick stalk previously extending from the mass through the right abdominal wall was no longer identified, and the stalk connecting the abdominal mass to the hypaxial musculature was decreased in thickness and no longer contrast-enhancing. There was however persistence of a thin, non-contrast-enhancing, soft tissue attenuating stalk connecting the cranial aspect of this mass to the distal tip of the right limb of the pancreas. The large cystic mass in the right dorsolateral abdominal subcutaneous tissues had resolved, with only a small, ill-defined region of mildly contrast-enhancing thickening of the subcutaneous tissues remaining. The fistulous tracts extending through the right hypaxial musculature had resolved. These findings were consistent with resolving granulomatous inflammation following surgical debridement of a gossypiboma. The residual stalks interconnecting some of these structures and the ill-defined, mild thickening of the right midabdominal subcutaneous tissues were most consistent with residual fibrosis, although persistence or progression of previously resolved fistulous tracts could not be entirely excluded due to lack of additional follow-up imaging. |
pmc-6613054-1 | We present the case of a 72-year-old male smoker presenting to the emergency department complaining of 4 months of progressive fatigue and dyspnea. He has a history of traumatic pneumothorax approximately 50 years ago during the Vietnam War and prior diagnosis of bladder cancer treated with cystoscopic resections and BCG (bacillus Calmette-Guérin) installations. More recently, he had 4 hospitalizations in the last 6 months for acute respiratory failure and was discovered to have an 8 cm mass in the right lung with multiple sites of suspected metastasis. Records of an outpatient positron emission tomography scan showed increased uptake in the left adrenal gland, contralateral lung, spleen, and multiple mediastinal lymph nodes. This lung mass was previously biopsied twice and failed to identify any malignancy; rather, these biopsies only showed cellular necrosis. Moreover, he was recently discharged after a 2-week-long hospitalization for hemorrhagic shock requiring multiple blood transfusions after a major bleed post-adrenal biopsy, which also failed to identify cytologic malignancy. During that hospitalization, bone marrow aspiration was performed, which was also nondiagnostic because it was a dry tap that failed to show any bone marrow cellularity.
On presentation to our emergency department, the patient was afebrile at 98.4°F, blood pressure was 125/66 mm Hg, heart rate 118 beats per minute, and oxygen saturation was 91% while on room air. On examination, patient appeared in moderate distress, tachypnic at 26 breaths per minute, there was no wheezing or crackles; however, there was decreased air entry bilaterally. White blood cell (WBC) count was noted to be extremely elevated at 69.3 × 103/µL, platelets 95, and hemoglobin of 6.9. Computed tomography angiogram identified a 10 cm right upper lobe mass associated rib erosion, multiple masses and pulmonary nodules bilaterally, a hemorrhagic mass in the right adrenal gland measuring 8.5 × 5.5 × 4.5 cm, multiple small masses in the left adrenal gland, and splenomegaly measuring 19 cm. Computed tomography scan of the head and neck did not show any lesions in the brain; however, right mandibular osteonecrosis with presumed adjacent dental abscess were seen.
He was admitted and sepsis protocol was initiated. He was resuscitated with crystalloid fluids and given 2 units of packed red blood cells. He was treated with broad spectrum antibiotics to cover postobstructive pneumonia. Despite aggressive therapy, his WBC count continued to rise without any improvement of his respiratory distress. On day 2, repeat chest X-ray showed worsening infiltrates and bilateral pulmonary edema. Tachypnea worsened and the patient was placed on bi-level positive airway pressure. His respiratory failure progressed, and he required intubation with mechanical ventilation. On day 3, the patient experienced an episode of supraventricular tachycardia and required amiodarone in addition to diuresis and vasopressors for hemodynamic support. On day 4 of his hospitalization, the WBC peaked to 196.1 × 103/µL. In light of his continued decline and poor prognosis, his wife expressed his wishes for comfort care only and for withdrawal of life support. He was extubated and was subsequently pronounced dead without having a definite diagnosis. Partial autopsy was performed, which provided light on his disorder.
On gross evaluation of the lungs, the tracheobronchial tree was patent except for the right lower lobe bronchioles, which was filled with thick mucoid material consistent with an infectious process. There were numerous apical blebs and thickened fibrosis diffusely spread throughout the lung parenchyma. The right lung had a large 8.8 × 6.6 cm poorly defined friable mass in the right apex infiltrating the chest wall with rib erosions. The dissected mass was chalky white and fleshy consistent with necrosis (). Similarly, the left lung had 3 masses appearing, the largest being 3.1 × 3.0 × 2.2 cm (). On dissection of the left upper lobe, we noted abundant amount of necrotic tissue with abscess formation walled by brown pigmented macrophages.
On hematoxylin and eosin–stained slides, the tumor cells had poorly differentiated pleomorphic cells with large vesicular nuclei, prominent red nucleoli, and binucleated to multinucleated bizarre shapes (). IHC investigations were used to help determine the lineage of the malignant neoplasm.
IHC staining of tumor cells showed strongly positive cytoplasmic staining with keratin CAM 5.2 (). Pancytokeratin AE1/3 was negative. Squamous differentiation marker p63 was negative. Glandular differentiation marker thyroid transcription factor 1 was negative. Myogenic differentiation marker desmin was negative. WBC markers CD45 LCA (leukocyte common antigen), CD3, CD20, and CD30 were negative. Histiocytic markers CD68 and CD1a were negative. Dendritic cell markers CD21 and CD23 were negative. Neuroendocrine differentiation marker synaptophysin was negative. Melanocyte differentiation marker HMB-45 was negative. IHC staining for epithelial membrane antigen, ALK-1, human chorionic gonadotropin, and S-100 protein were negative. A periodic acid-Schiff stain was negative for periodic acid-Schiff positive cytoplasmic inclusions. Furthermore, despite prior “dry tap,” the bone marrow was hypercellular with exuberant leukemoid reaction and metastatic large cell neoplasm ().
Interestingly, the presumed right mandibular abscess identified on CT scan was confirmed to be a large cell neoplasm with strongly necrotic tissue (). Despite multiple sites of necrosis blood cultures drawn from central venous line showed negative for microbiological growth. The left lung culture and pericardial fluid showed no microbiologic growth. His right mandible and broth from the right lower lobe of lung grew Candida albicans. Cytology from pleural fluid identified malignant appearing cells.
Furthermore, the submandibular and mediastinal lymph nodes were dissected. Submandibular and parabronchial lymph nodes on the right were positive for metastatic large cell neoplasm (); however, lymph nodes on the left were not consistent with metastatic disease. Four paraaortic lymph nodes from the arch of aorta and 4 abdominal aorta lymph nodes were positive for metastatic large cell neoplasm. Greater mesenteric lymphadenopathy was also noted to be consistent with metastatic large cell neoplasm. The spleen was large with markedly congested parenchyma with rare atypical cells but without infarction (). The right adrenal gland was pink-brown with a smooth surface measuring 12.1 × 7.1 × 4.7 cm and weighing 250 g. Left adrenal gland measures 8.1 × 2.5 × 1.5 cm and weighed only 70 g. Both adrenal glands had multiple metastatic nodules, hemorrhage, and diffuse necrosis (). |
pmc-6613258-1 | Sixty-three days before starting therapy for this infection, a 59-year-old female patient had been discharged on Day 22 post-surgery in our ICU following a mitral valve replacement. However, at 41 days after discharge, she developed pneumonia due to infection by P. aeruginosa, and thus returned to the ICU, where she had to be kept on mechanical ventilation (owing to respiratory failure) and continuous renal replacement therapy (CRRT; owing to AKI). A dosing strategy for doripenem was calculated to determine the continuous infusion to achieve a certain target serum concentration of the unbound drug, which was set to 32 μg/mL, i.e., four times higher than the actual MIC (8 mg/L) []. Here, if the maximal licensed dose in Japan (3 g/day) would be delivered by continuous intravenous infusion (1 g/80 mL of normal saline/8 h; 10 mL/h, every 8 h), the target concentration could only be achieved when the doripenem total clearance (CLtot) was < 3.6 L/h. However, previous reports have indicated a doripenem clearance of 2.7–5.9 L/h by the body (CLBODY) [–]; doripenem clearance by CRRT (CLCRRT) in this case was calculated to be 0.6 L/h based on the effluent flow rate of CRRT []. Therefore, the probability that the patient’s CLtot was < 3.6 L/h was low. Furthermore, Monte Carlo simulation computed by R (ver. 3.5.3, ) estimated that there was a 7.6% probability of achieving 32 μg/mL of unbound doripenem by using the population pharmacokinetic model for doripenem reported by Roberts et al. []. Therefore, therapeutic drug monitoring (TDM) was applied. The time course of the values for the concentration of unbound doripenem in the serum and the values from other laboratory tests during the patient’s stay at the ICU are presented in Fig. . The samples were prepared by ultrafiltration using a Nanosep Omega 10 K and the concentrations of unbound doripenem in the serum were quantified by high-performance liquid chromatography []. The concentration of unbound doripenem was 47.8 μg/mL at 20 h after the dose administration started. The concentration decreased to 33.6 μg/mL at 111 h, although these concentrations were maintained at levels that were four times higher than the MIC. Continuous infusion of doripenem was performed for 11 days, after which the patient was discharged from the ICU. After the end of the continuous infusion of doripenem, the MIC against P. aeruginosa detected in her sputum was 8 mg/L, which was not elevated. |
pmc-6613476-1 | A 20-year-old Sindhi female student with no known comorbid presented to the emergency department with a complaint of generalized weakness and shortness of breath over the previous 15 days. According to the patient herself, the generalized weakness was progressive and with increasing intensity to such an extent that it hampered her daily activities. On top of that she was also experiencing shortness of breath which was also progressive. However, she denied any orthopnea, paroxysmal nocturnal dyspnea (PND), fever, rash, altered bowel habits, cough, joint pain and any acute history of blood loss. According to the patient’s past medical history, she had on and off loose stools from 9 years of age, which resolved by age 16. Furthermore, 2 years back she was admitted to a nearby hospital with generalized weakness and jaundice. There is no official documentation but reportedly she was also transfused with 2 blood bags. Workup and diagnosis were not completed during her stay as she was non-compliant and left against medical advice at that time. All other tests were normal and her menstrual history was also normal. On examination, her vitals were blood pressure 110/60 mmHg, (reference, 120/80mm/hg); pulse 90 beats/minute (reference range, 70–100 beats/minute); temperature 98°F (reference range, 97–99°F) and respiratory rate 22 breaths/minute (reference range, 12–20 breaths/minute). Her general physical examination showed anemia, jaundice and clubbing, along with a raised jugular venous pulse. Her respiratory, cardiovascular system and central nervous examination were normal. However, her abdominal examination showed hepatomegaly (with liver palpable up to one finger) and splenomegaly (with spleen palpable up to 3 fingers below the costal margin) with the rest of the examination being normal.
Based on the history and examination, we ordered pertinent laboratory work up along with other tests. Her base line laboratory values were hemoglobin 2.3 g/dL (reference range, 11.1 – 14.5 g/dL); mean corpuscular volume (MCV) of 73.2 (reference range, 76 – 96); total lung capacity (TLC) 4.2×10
9/L; platelets 92000/mm
3 (reference range, 150 – 400000/mm
3); sodium 140 meq/L (reference range, 135–145 meq/L); potassium 3.6 meq/L (reference range, 3.5–5 meq/L); chloride 108 meq/L (reference range, 97–107 meq/L); calcium 8.5 meq/L (8.5–10.2 meq/L); magnesium 2.2 (reference range, 1.5–2.5 meq/L); total bilirubin 1.97 (reference range, 0.1–1.2 meq/L); serum glutamic pyruvic transaminase (SGPT) 59 units/L (reference range, 7–56 units/L); alkaline phosphatase (ALP) 123 IU/L (reference range, 44–147 IU/L); serum glutamic-oxaloacetic transaminase (SGOT) 40 units/L (reference range, 5–40 units/L); total protein 7.7 g/dL (reference range, 6–8.3 g/dL); albumin 3.5 g/dL (reference range, 3.5–5 g/dL) and prothrombin time to international normalized ratio (PT/INR) 11 seconds (reference range, 11–13.5 seconds).
As she presented with shortness of breath, we also performed an echocardiography and chest x-ray, both were within normal limits. At this point in time, we assumed that the patient’s symptoms and signs could be due to some autoimmune disorder (such as SLE, rheumatoid arthritis (RA)), immune mediated disorders (such as celiac disease), thyroid disorders (example immune thrombocytopenia purpura), Evan syndrome (due to thrombocytopenia and hemolytic anemia) and even chronic liver disease. Hence, in order to rule out the differentials and get to a possible diagnosis we conducted more tests. The patient iron profile showed ferritin 115.5 ng/mL (reference range, 12–150 ng/mL), serum iron 82 mcg/dL (reference range, 50–170 mcg/dL), total iron binding capacity (TIBC) 258 mcg/dL (reference range, 250–370 mcg/dL), transferrin saturation 37% (normal 25–35%) and her B12 levels were 2000 pg/mL (reference range, >200 pg/mL), both were in normal range. To rule out thyroid disorders, we checked her thyroid profile [thyroid stimulating hormone (TSH) 2.75 mU/L, (reference range, 0.5–4.0 mU/L); tri-iodothyronine (fT
3) 2.45 pg/mL, (reference range, 2.3–4.2 pg/mL); thyroxine (fT
4) 1.71 ng/dL, (reference range, 0.8–1.8 ng/dL)], which was within normal limits. She was also negative for anti-nuclear antibody, anti-Smith antibodies, anti-double stranded DNA antibody, anti-smooth muscle antibody and anti-mitochondrial antibody, thereby ruling out the possibility of autoimmune disorders. Furthermore, her blood Coombs test was also negative. To rule out secondary causes of idiopathic thrombocytopenia purpura (ITP), we tested for viral markers of human immunodeficiency virus (HIV), Epstein bar virus (EBV), cytomegalovirus (CMV) and performed a
Helicobacter pylori test, all of which were negative. For celiac disease, we took anti-tissue transglutaminase (anti-TTG) titers of IgA and IgG, which came out to be 353 U/mL, (reference, <20 U/mL); and 419 U/mL (reference, <20 U/mL) respectively. The elevated titers confirmed the diagnosis of celiac disease. However, at this point in time, in order to improve symptoms of the patient, she was transfused with 2 bags of blood. Her bone marrow biopsy showed a positive coombs test with immune mediated hemolytic anemia and immune mediated thrombocytopenia. Hence, we established a diagnosis of celiac disease with Evans syndrome. However, in order to confirm our diagnosis of celiac disease, endoscopy was scheduled, but the patient did not consent to the procedure. Moreover, we started oral prednisone therapy 40 mg once daily which was tapered off and stopped in four weeks and the patient was counseled about a gluten free diet. Within these four weeks, the patient felt better and was discharged. |
pmc-6613672-1 | A 29-year-old woman presented with a 2-year history of a slow-growing, indurated, and ulcerated skin mass at the anterior medial aspect of the left lower leg that was tender and mobile. The mass measured 5 × 6 cm, with skin surface ulceration, purulent drainage, and foul smell, probably as the result of wound superinfection (). The patient had no significant medical history. A routine blood count was within normal limits (WBC: 7,500/μL; hemoglobin: 13.8 g/dL; platelets: 311,000/μL). The patient initially consulted traditional healers, without improvement. A biopsy of the lesion was performed at Butaro Cancer Centre of Excellence (Butaro, Rwanda) and sent to Brigham and Women’s Hospital (Boston, MA) for additional work-up.
Tissue sections showed a deep skin incisional biopsy, extending to the subcutis (). The dermis and subcutis were diffusely infiltrated by a monotonous population of intermediate- to large-sized immature cells with round to irregular nuclei, dispersed chromatin, distinct small nucleoli, and scanty cytoplasm (). Frequent mitotic figures were observed. The overlying epidermis was not involved. An initial limited panel of immunostains was performed at the Butaro District Hospital Pathology Department, demonstrating that lesional cells were positive for CD45 (diffuse), terminal deoxynucleotidyl transferase (majority), and PAX5 (weak, small subset); lesional cells were negative for CD3, CD20, myeloperoxidase, and lysozyme. Given the inconclusive immunophenotype, the case was sent to Brigham and Women’s Hospital for additional immunostains. These additional studies revealed that lesional cells were positive for CD2, CD33, CD4 (weak), CD56 (), CD123 (), and TCL1 (); lesional cells were negative for CD10, CD19, CD34, CD7, and CD5. On the basis of morphologic and immunohistochemical findings, a diagnosis of BPDCN was rendered. A staging bone marrow biopsy was not performed before treatment initiation. A bone marrow biopsy performed after the induction phase of therapy revealed a hypocellular marrow (30% cellular) with maturing trilineage hematopoiesis and no morphologic evidence of disease. A 95-gene sequencing panel showed no pathogenic single-nucleotide variants or small insertions/deletions, although several variants of unknown significance were reported (ATM c.1810C>T p.P604S [in 58.9% of 440 reads]; CREBBP c.7306G>A p.E2436K [in 8.2% of 220 reads]; NOTCH3 c.4469_4472CGGA>GCGC p.1490_1491delTEinsRA [in 11.3% of 133 reads]).
Because of her young age and otherwise excellent performance status, our patient started receiving an intensive systemic chemotherapy regimen used to treat acute lymphoblastic leukemia (ALL), with intrathecal therapy for CNS prophylaxis. The treatment regimen we chose () is a modified treatment for ALL that was proposed by Hunger et al specifically for low-resource settings. It consists of initial and delayed aggressive phases of therapy (induction/consolidation and delayed intensification) with an intervening less intense phase of treatment known as interim maintenance. After completion of these blocks of more aggressive therapy, which generally takes between 6 and 9 months, there is a prolonged period of maintenance therapy that lasts 2 years. Our patient is currently in the interim maintenance phase and has responded well, showing complete healing of the skin tumor (). |
pmc-6613742-1 | This 31-year-old primigravida woman with a history of pulmonary embolism and multiple deep venous thrombosis during the index pregnancy was admitted to the obstetric department with pre-eclampsia with severe hypertension of 160/100 mm Hg at 26 weeks of pregnancy. She had been taking vitamin K antagonists, which were switched to low-molecular-weight heparin, and she was treated with magnesium sulfate and intravenous antihypertensive drugs. A primary caesarean section under general anaesthesia was performed at 30 weeks of pregnancy due to fetal distress, delivering a growth-restricted, premature girl of 920 g. Postsurgery the mother developed severe postpartum haemorrhage with a total of 5000 mL blood loss, and treatment was complicated by her anticoagulation therapy. She received blood transfusions and was discharged from the hospital with a haemoglobin level of 77.3 g/L. Three days later she was readmitted with complaints of fatigue, severe dyspnoea and fever. She had pleural effusion and thrombocytopaenia. She was later transferred to the cardiac intensive care unit (ICU) due to deterioration of her condition, and echocardiographic examination revealed severe left ventricular dysfunction with mild dilatation of the ventricles. ECG showed a sinus tachycardia with low voltages in the extremity leads and a QS pattern in V1–V3 (). Coronary angiography showed normal coronary arteries and she was diagnosed with PPCM. She soon went into cardiogenic shock with high lactate levels, and when high dosages of inotropes could not stabilise her extracorporeal membrane oxygenation (ECMO) was started. Heart failure medication was started, which stabilised her condition, and after 15 days the ECMO could be removed. ECMO was complicated by cannula-related severe ischaemia of her right foot, which had to be amputated. She was transferred to a rehabilitation clinic after hospital discharge.
Because of the combination of multiple venous emboli, pre-eclampsia with proteinuria, pleural effusion and the thrombocytopaenia, the patient was referred to an immunologist, who diagnosed her with antiphospholipid syndrome and systemic lupus erythematosus (SLE). Her last echocardiogram showed only a mild impairment of left ventricular function. |
pmc-6613742-2 | This 25-year-old primigravida woman with a history of childhood asthma and generalised anxiety disorder was admitted to a cardiology ward unit with progressive dyspnoea at 26 weeks of pregnancy. Her ECG revealed several abnormalities (), and echocardiography showed a DCM with severe left and right ventricular dilatation and a left ventricular ejection fraction of about 10%–15% (). She underwent emergency caesarean section the following day and was admitted to the ICU afterwards, where she was treated with heart failure medication and inotropes. Low-molecular-weight heparins were started because of a thrombus in the right ventricular outflow tract. ECMO was started due to refractory cardiogenic shock, and later a left ventricular assist device was implanted. There was no evidence of coronary artery disease. Due to severe confusion and drowsiness in the ICU, a CT of the brain was made, showing generalised brain atrophy. Therefore, a metabolic origin was suspected. Myocardial biopsy showed a myopathic image suspected for mitochondrial storage disease due to excessive glycogen storage. Morbus Pompe and Fabry were ruled out and viral tests were negative, as were autoimmune and paraneoplastic syndromes. Quadriceps femoris muscle biopsy showed a myopathy without specific characteristics. Blood and urine tests were performed, as well as genetic tests. Plasma carnitine was strongly elevated and a diagnosis of succinyl-CoA ligase deficiency was suspected, but could eventually not be confirmed as the results of further tests and analyses were inconclusive. No known mutations in the DCM genes were found, and the diagnosis of PPCM remained the working diagnosis. Her prematurely born son was diagnosed with (congenital) hypertrophic cardiomyopathy. One year later, the patient underwent a cardiac transplantation and is currently doing relatively well. |
pmc-6613742-3 | This 36-year-old woman with a history of unspecified palpitations and a family history of sudden cardiac death, who had once been assessed by a cardiologist because of suspected long-QT syndrome in her infant daughter, was admitted to the cardiology ward unit with complaints of dyspnoea and chest pain, 4 months after giving birth to her third child. Her N-terminal prohormone of brain natriuretic peptide (NT-proBNP) was markedly elevated, and echocardiography revealed a DCM with a left ventricular ejection fraction of 15% and moderate to severe tricuspid insufficiency and a moderate to severe mitral insufficiency. She was diagnosed with PPCM and was treated with heart failure medication on which she slowly recovered. She was discharged home and seen by a cardiologist for regular follow-up. A 24-hour Holter ECG showed frequent, non-sustained ventricular tachyarrhythmias, and as part of her outpatient follow-up an MRI scan of her heart was made, which showed distinct tubular dilatation of the right ventricle, with less distinct dilatation of the left ventricle (). Hypokinesia was most prominent in the right ventricle and there was severe tricuspid insufficiency. A diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) was made and a prophylactic internal cardiac defibrillator was implanted. Genetic tests revealed no known mutation. Her left ventricular function has almost fully recovered, but her right ventricular function remains moderately impaired. |
pmc-6613742-4 | This 26-year-old primigravida woman in labour with an unremarkable medical history was urgently admitted to the obstetrics department with failure to progress in the second stage and the suspicion of an intrauterine infection. She underwent an emergency caesarean section, which was complicated by a severe postpartum haemorrhage with a blood loss of 2 L. She was treated with sulprostone and intravenous fluids and a blood transfusion, immediately after which she became severely dyspnoeic. Chest X-rays showed congestion, and echocardiography revealed severe left ventricular dysfunction with an estimated ejection fraction of 15% and only moderate wall motility in the basal septal region. There were no signs of ischaemia or coagulopathy (thereby excluding amniotic fluid embolism). The diagnosis of PPCM was suspected and she was intubated and transferred to the ICU, where she was treated with conventional heart failure medication. She could be extubated very quickly after initiation of therapy, and repeat echocardiography showed normalisation of left ventricular function with normal ventricular dimensions within a few days. She had experienced the caesarean section as very traumatic and has visited a psychologist to help her cope. In the outpatient clinic, the question rose whether she had suffered from PPCM or whether her acute heart failure was caused by a takotsubo cardiomyopathy. The urgent and complicated characteristics of the delivery, as well as the echocardiographic images of akinesia affecting the whole ventricle except the basal segments and the very rapid recovery of the ejection fraction, are suggestive of a takotsubo cardiomyopathy rather than a PPCM. She returned to the cardiologist a few years later with the wish to become pregnant again. Due to her recovered left ventricular function, there were no objections to a subsequent pregnancy and she has had several uncomplicated pregnancies since. Her left ventricular function has remained normal to date. |
pmc-6614109-1 | The patient is a 74 years old, skin phototype III of the Fitzpatrick scale, right-hand-dominant female, who suffered a deep partial thickness burn with hot water in the right hand 14 years prior to admission. This burn healed by secondary intention. No debridement or skin graft was performed. There was no family history of skin cancer.
She presented to our department in January 2018 with an exophytic lesion in the right thenar eminence, about 2 cm of diameter, painful and bleeding, that had increased in size during the past several years ().
Her neurovascular exam was intact. She had no palpable axillary lymphadenopathy.
Laboratorial analyses and ultrasound revealed no relevant changes.
The treatment was local excision of the lesion with a margin of 1 cm of healthy tissue and then the defect was covered by a full thickness skin graft harvested from the internal surface of the right arm (), under general anesthesia.
The lesion was sent to microscopic examination ().
No lymph node dissections was carried out at that time.
The anatomophatological examination of excised tissue showed two nodular lesions with focal ulceration compatible with the diagnosis of a superficial spreading melanoma, IV level of Clark, 3,4 mm of the Breslow’s tumor thickness classification with high mitotic rate (13/mm2) and linfovascular invasion. pTNM: pT3bNXMX.
A thoracic-abdominal-pelvic computed tomography scan showed no evidence of metastatic disease or axillary lymph node involvement.
The patient was transferred to an oncologic institution where an enlargement of the margins and a sentinel lymph node biopsy was performed. This last one revealed intranodal metastases, so an axillary ganglionar emptying was performed.
In this case there are some negative prognostic factors, such as a chronic latency, presence of ulceration, high mitotic rate and linfovascular invasion. However, at 6 months post-operatively there are no signs of local recurrence or systemic dissemination. She must maintain follow-up for at least 5 years. |
pmc-6614111-1 | A 65-year-old male positive for hepatitis C virus antibody had been treated for HCCs in the liver segments 5, 6, 7, 8 in our hospital since October 2014. The patient was initially treated for the ruptured HCC in the segment 5 with TACE, while subsequent examinations detected other HCCs in segments 6, 7 and 8 which were similarly treated by 2nd line TACE, following the Japanese HCC therapeutic guidelines. After the treatment, the tumors in segments 6 and 7 were well controlled, while the pre-ruptured tumor in segment 5 and the lesion in segment 8 still remained viable. Hence, a 3rd line TACE was performed after 6 months: while the segment 8 tumor were well embolized after the treatment, the segment 5 tumor could not be embolized completely. Subsequently, it continued to gradually develop, as the caudal side of this tumor was perfused by the gastroepiploic artery, due to the ruptured tumor progression into the greater omentum. The collateral vessels from the omental branch into the tumor disturbed further TACE, as we suspected it could induce ischemia in the GI tract. Hence, the chemotherapeutical sorafenib was selected and administered repeatedly ().
Since this pre-ruptured tumor began and continued to involve the duodenum in the next six months, causing anemia and malnutrition, even further administration of sorafenib became difficult. At this point, the patient was referred to our surgical department for the first time. His critical situation compelled us to consider a surgical intervention.
A pre-operative examination by CT revealed a massive HCC in segment 5 of 6.5 cm in diameter, protruding from the liver and penetrating the duodenal wall (a), while other small HCC lesions appeared to be controlled. A gastroendoscopy revealed an ulcerative mass lesion in the posterior wall of the duodenal bulb (b), which was revealed to be HCC following histology analysis of a biopsy sample. Laboratory analysis revealed anemia (hemoglobin 9.2 mg/dl) and low albumin level (3.0 g/dl), while PIVKA-2 and α-fetoprotein levels were extremely high (2560 mAU/ml and 13,3000 ng/ml, respectively). The retention rate for indocyanine green until 15 min was 8.5%, revealing that his liver function was not compromised. During the preoperative evaluation, repeated blood transfusions became necessary, while communicating collateral vessels from the pancreatic and omental branch into the tumor disturbed hemostatic embolization. Since the disease condition aggravated by the tumor progression became a life-threatening oncological emergency, we were compelled to operate on the patient. However, considering his anemic and malnutritional preoperative conditions, we decided to exclude highly invasive surgical approaches such as hepatectomy with pancreaticoduodenectomy (HPD), and instead attempted to do HPPD to lessen surgical stress.
During surgery, we found that the ruptured HCC in segment 5 further developed to became about 10 cm in diameter, involving the duodenal wall, fortunately without any macroscopic peritoneal dissemination. This corresponded to a grade evaluation of S2 (invasion to other organs) and P0 (no peritoneal dissemination) according to the General Rules for the Clinical and Pathological Study of Primary Liver Cancer 2019 by Liver Cancer Study Group of Japan []. We started the surgical treatment with the partial hepatectomy of segment 5 using the Pringle maneuver, and removed the tumor with the liver parenchyma as surgical margin (a). We did not perform anatomical resection of the segment 5 considering the past multiple HCCs status and also to avoid a major HPD in case we would be forced to add pancreaticoduodenectomy during the operation. The gall bladder was also removed as it was attached to the tumor. After the resection of the tumor from the liver, we inserted a thin Nelaton catheter from the cystic duct into the common bile duct to investigate the approximate location of the major papilla of Vater and its relation to the tumor. The inspection suggested the possibility of choosing HPPD and avoiding HPD, even after taking en bloc resection into consideration. On the basis of this observation, we subsequently dissected the first and second parts of the duodenum along the pancreas head and cut the minor pancreatic duct after its ligation. Then the duodenal inner lumen was opened to directly confirm the preservation of the major papilla of Vater. We removed the liver tumor and gallbladder en bloc with the first and second parts of the duodenum, finally transecting directly oral side to the papilla using a surgical stapling device. The reconstruction was performed using the Billroth II approach as in distal gastrectomy (b). Blood loss amounted to two liters due to the bleeding from the fragile collateral vessels surrounding the extended tumor and from those developed in the adhesional planes that formed due to the previous TACE treatments. We did not perform further peritoneal resection since no peritoneal disseminated lesion was found. Therefore, we evaluated the achieved resection equivalent to grade R0 (no viable tumor remaining).
Post-operative course was uneventful except for a wound infection. He was discharged on the 45th postoperative day. Tumor markers were found to be in the normal range within three months after the surgery. Pathological examination was compatible with the pre-operative findings and revealed poorly-to-moderately differentiated HCC cells directly invading the duodenal wall and extending inside the duodenal lumen (). Approximately one quarter of the tumor was necrotic, demonstrating either the effects of repetitive TACE and the chemotherapy, although we cannot exclude spontaneous necrosis. Surgical margin was proved to be cancer negative.
After this surgery, the lesion in segment 8 remained stable. However, it was radio-frequency ablated twice as a preventive measure. Three years after the surgery the patient is still alive and leads a disease-controlled life. |
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.