text
stringlengths 746
31k
|
|---|
A 13 years old male child reported to the department of pedodontics, PGIDS, Rohtak with chief complaint of difficulty in drinking water and change in voice since 1 month. Patient gave history of traumatic removal of his right upper back tooth (due to caries) 1 month back following which these problems started. On examination, his right maxillary permanent first molar was missing, there was slight opening on the buccal side of the alveolar process. There was nasal discharge of oral fluids along with nasal twang. Diagnosis of OAF was made ( and ). Patient was then referred to department of oral and maxillofacial surgery for further management.\nManagement: His maxillary antrum was irrigated with normal saline three times within a week. After taking informed written consent for the surgery and getting necessary blood investigations, closure with BFP under local anesthesia was planned. Right posterior superior alveolar nerve block and greater palatine nerve block was given with 2% lignocaine with 1:200000 adrenaline along with the right buccal vestibule infiltration. The mucoperiosteal flap was raised from mesial to 15 to distal to 17 with molt’s periosteal elevator after giving incision with no. 15 blade. The defect () was curetted with the help of Lucas curette. A incision was given over the periosteum on the undersurface of the flap and with pressure applied to the zygomatic arch region, the BFP easily extruded into the operative side. Dissection with a Metzenbaum scissors helped to mobilize as much BFP () as needed to obtain a tension-free closure across the communication. The palatal mucosa margin was freshened. The fat pad was then sutured with resorbable suture to palatal mucosa. The buccal flap was then positioned over the BFP and was sutured to palatal mucosa with 3-0 silk suture (). Antibiotics and analgesics were prescribed for 5 days. Patient was advised soft diet and to maintain oral hygiene. Sutures were removed after 2 weeks. The postoperative period was uneventful (). |
A 15-year-old boy who was referred from a peripheral hospital, with a history of penile amputation following trauma from a grinding machine which he was operating, was said to have sustained traumatic penile amputation, with severe bleeding and pain, and no history of loss of consciousness.\nHe was initially taken to the local hospital, where he was resuscitated and the bleeding vessels ligated; the amputated part of the penis was wrapped in gauze before he was referred to us and he presented to us about 30 h after the incidence.\nOn physical examination, he was found to be anxious mildly pale not dehydrated. There was a complete penile amputation through his penile skin to the cavernosal bodies and transaction of the urethra with bleeding from the dorsal vessels. His scrotum was lacerated exposing the testicles with devitalized tissues; however, the testicles were found to be intact with a spigotted catheter and the mummifying penile stump in between his thighs.\nAfter thorough irrigation with normal saline, parenteral antibiotic and analgesic was given. He was transfused with two units of whole blood and was taken to the theatre under general anesthesia. A rubber band was placed, as a tourniquet, around the proximal end of his penis for bleeding control and debridement was done, and the bleeding vessels were identified and ligated as shown in Figure .\nA 16Fr Foleys catheter was inserted transurethrally through the urethral stump followed by everting the urethral edges and suturing it using interrupted 4/0 vicryl sutures. The wound was dressed with Vaseline gauze and was found to be granulating well 5 days postoperatively as shown in Figure .\nEight days postoperatively, patient was prepared and had skin grafting using a split thickness graft from the thigh skin of the patient. The edges of the graft were sutured with vicryl 4/0, and the testicles were returned to the scrotum and scrotoplasty was done as shown in Figure .\nFour weeks post-split thickness, skin grafting patient did very well, wound had healed and he was able to pass urine with good flow. On follow-up examination, 5 weeks later, no necrosis was noticed on his skin Figure . |
A 70-year-old healthy woman presented at a private practice dental office, unsatisfied with her smile; this was due to excessive maxillary gingival display (a gummy smile), concomitant with short clinical crowns (). Intraoral examination revealed partial edentulism, rotated anterior maxillary teeth with incisal erosion, and enamel wear (). The mandibular molars were mesially inclined, resulting in the collapse of the occlusal vertical dimension (OVD) [, ]. The remaining mandibular teeth were severely worn. The right central incisor was rotated in the buccal position, with gingival recession and loss of gingival height ().\nDuring the next appointment, the anterior maxillary teeth were prepared with the BOPT, and the interim restoration was cemented. Next, two 3.8 mm diameter implants (WINSIX® BIOSAF IN Srl, Milan, Italy) were placed in the area of the mandibular first molar and left submerged during the osseointegration period. At the end of the session, two alginate impressions and a new centric relation record were obtained and mounted in an articulator to fabricate a mandibular full-arch restoration corresponding to the increased OVD.\nThe mandibular full-arch restoration was fabricated using a 3 mm OVD increment, and the curve of Spee was harmonized with the opposing arch. The mandibular teeth were prepared with the BOPT, and the complete restoration was placed in the mandibular arch from the right to the left second molar. The mandibular left second molar was mesially inclined, so it was prepared more on the mesial side than on the distal one using the BOPT with the POA to move the dental axis distally, thereby creating an adequate space for the first molar parallel to the path of prosthesis insertion (). The POA was also used for the mandibular right central incisor by preparing it more on the vestibular side to realign it with the adjacent teeth (). The interim mandibular full-arch restoration was cemented and corresponded to the new OVD. One month after soft tissue maturation, the definitive impression of the maxillary abutment teeth was taken, and zirconia ceramic full-crown restorations were fabricated in the dental laboratory. The gingival sulcus was probed again and found to be 2 mm on the vestibular side. The dental technician was instructed to position the crown margin 1.5 mm subgingivally only on the vestibular side, using all of the available gingival sulcus, thus improving the gummy smile (). In the interdental spaces and on the palatal side, the crown margin was placed 0.5 mm into the gingival sulcus. The patient expressed satisfaction with the results. Healthy gingiva, with an ideal scalloped architecture, was observed at the 2-year follow-up. No gingival recession or inflammation was noted. The restoration accomplished three goals: (1) correction of the deep bite, (2) realignment of the teeth, and (3) elimination of the gummy smile. The procedure did not affect the pulp vitality, and no endodontic complications were observed at the 2-year follow-up (). |
A 45-year-old Haitian American female with no significant medical history presented with a six-month history of progressive solid food dysphagia and a one-month history of odynophagia. The patient had lost 15 pounds and was only able to tolerate pureed food or liquids. She had experienced no symptomatic relief on omeprazole 40 mg twice daily for the past two months and did not use tobacco, alcohol, or illicit substances. Her initial blood count and metabolic panel were unremarkable. An esophagogastroduodenoscopy (EGD) revealed erythematous and friable mucosa with ulcerations in the proximal esophagus (). There was a stricture encountered at 15 cm from the incisors through which the gastroscope could not be traversed. Biopsies were taken from the inflamed esophageal mucosa and the proximal lumen of the stricture. Brush cytology was collected through the stricture as there was a concern for malignancy. A subsequent barium esophagram and upper GI series demonstrated 2 cm irregular narrowing in the cervical esophagus, but no abnormalities in the rest of the esophagus, the gastroesophageal junction, stomach, duodenum, or proximal jejunum. Contrast-enhanced computed tomography (CT) of the chest showed no acute esophageal, mediastinal, pulmonary, or cardiac pathology. The esophageal biopsy indicated acute and chronic inflammation with filamentous sulfur granules consistent with Actinomyces; rare fungal hyphal elements were additionally identified (Figures , , and ). The cytology was negative for malignant cells and the acid-fast bacilli (AFB) stain was negative, ruling out Nocardia as a potential pathogen. The patient was started on intravenous (IV) Penicillin for a diagnosis of esophageal actinomycosis and oral Fluconazole for a presumed Candida coinfection given concurrent fungal elements. Her human immunodeficiency virus (HIV) status was negative and her fasting blood glucose was within the normal range. She was discharged on Fluconazole 200 mg daily for 2 weeks and IV Penicillin G 3 million units every 4 hours for 6 weeks followed by oral Penicillin to complete a total of 6 months of antibiotics. Her esophageal culture eventually grew normal oropharyngeal flora and rare Candida albicans. The patient returned to the hospital 6 weeks later due to an acute right upper extremity deep venous thrombosis (DVT) associated with her peripherally inserted central catheter (PICC). Her odynophagia had improved but she was still not able to advance her diet. Repeat EGD was performed, which showed resolution of her esophagitis, but a remaining stricture in the proximal esophagus (). During an attempt to dilate the stricture, a small mucosal tear was induced. The dilation had to be deferred at that point pending mucosal healing. A repeat barium esophagram illustrated the previously identified stricture with no perforation. The patient's PICC was removed and she was discharged on oral amoxicillin 875 mg twice daily to complete the 6-month course. The patient was given a follow-up appointment in our GI clinic where she would be reassessed for esophageal stricture dilation. Unfortunately, she was lost to follow-up. |
A 35-year-old lady was admitted through emergency intake to medical ICU for altered sensorium for the 3 days progressing to complete unresponsiveness on the day of admission. Her symptoms were preceded by fever, vomiting and headache for 1 week.\nAt the time of admission, she was stuporous and febrile (temperature 37.7°C) but without any haemodynamic instability. Genital lesions were noted in various stages ranging from healed scars to active ulcerations (Figs and ). Her neurological assessment revealed anisocoria and facial nerve palsy on the left side. A paucity of movements in response to pain was noted on the left side and was hypotonic on passive movement. Tendon reflexes were exaggerated on the left side and plantar response was extensor in keeping with an upper motor neuron paralysis of the left side. Signs of meningeal irritation were absent. Other systems on clinical examination were normal. With this clinical picture, several possibilities including infection, various causes of stroke in young people including vasculitis and connective tissue disorders such as lupus were considered.\nLaboratory investigations revealed neutrophilic leucocytosis, elevated ESR and normal liver and renal function tests. Her blood glucose, serum electrolytes and calcium were within normal limits. Serological markers for common viral infections (HIV, HBV, EBV and HCV) were negative. Anti-nuclear antibody by immunofluroscence assay was negative and so was anti-neutrophil cytoplasmic antibody. A chest X-ray was normal. A CT scan of brain ruled out haemorrhagic stroke, but showed no other lesions. A study on cerebrospinal fluid (CSF) revealed markedly raised proteins with low sugar and normal cell counts. CSF gram stain was negative and culture did not yield any growth.\nShe was initially managed empirically with antivirals along with other supportive measures. However, on the third post-admission day, she was responding only minimally with minor improvement in sensorium. The possibilities of herpes simplex virus (HSV) meningoencephalitis or tuberculosis were considered as high. However, the acute onset was deemed against the latter possibility. On further questioning, the husband who had since returned from Middle East to accompany his ailing wife conformed to the history of ‘recurrent genital ulcerations’ in his wife which was being treated as herpes with little benefit and ‘recurrent oral ulcers’. In addition, he claimed not to be affected by the genital lesions himself at any point of time. These features raised the suspicion of an alternative diagnosis of BD to explain our patient's clinical picture and prompted us to specific neuroimaging.\nAn MRI study of the brain and whole spine showed multiple symmetrical non-enhancing foci of altered signal intensity in the brainstem, hypothalami, posterior limb of internal capsule and splenium of corpus callosum (Figs and ), and also the spinal cord in keeping with BD causing neurological involvement (neuro-BD). The later availability of negative CSF PCR for HSV, Japanese encephalitis and West Nile virus strengthened this diagnosis. She was then treated with five doses of intravenous methylprednisolone on consecutive days, along with colchicine and other supportive measures comprising care of the genital ulcers with topical agents, water bed to prevent bed sores, physiotherapy and nutritional inputs. A week later, she was conscious and was obeying verbal commands and was moving left limbs. Follow-up treatment consisted of oral tapering prednisolone and azathioprine. The patient was discharged home with neurorehabilitation and subsequent follow-ups have found her to be in good health with only minor residual paresis. |
An 83-year-old man presented to our university hospital because of uneradicated periprosthetic infection after TKA. He had undergone right primary TKA for incapacitating knee pain recalcitrant to conservative therapy at a previous hospital 35 months before visiting our hospital (). Eleven months after the first surgery, deep infection of TKA caused by methicillin-resistant Staphylococcus aureus (MRSA) occurred, so two-stage exchange arthroplasty (revision TKA following open irrigation and debridement of component retention) was performed 15 months after the first surgical procedure (Figures and ), and treatment with vancomycin was employed. The patient received a blood transfusion of 1,680 mL red cell concentrates during the revision TKA. The infection recurred 1 month after revision TKA, and treatment was repeated with implant removal, debridement, and insertion of an antibiotic-impregnated cement spacer (). During this surgery, a blood transfusion of 2,800 mL was required again. However, the infection persisted, so the patient presented to our hospital 22 months after the primary TKA. Antibiotic-impregnated cement spacer exchange and debridement were repeated 7 months after revision TKA and eradication of infection was confirmed with aspiration in the outpatient clinic of our hospital more than three times. In addition, after completion of antibiotic therapy, the ESR, CRP level, and total WBC and differential counts in the joint aspirates were obtained, and the patient was observed for 2 more weeks. This was the fifth surgery the patient underwent.\nThe patient, 60 kg in weight and 156 cm in height, with a body mass index (BMI) of 25 kg/m2, had a history of hypertension, hypercholesteremia, and liver abscess and a 40-year smoking history. There was no history of ischemic heart disease or cerebrovascular problems, and there had been no symptoms of peripheral vascular dysfunction. He could walk with two canes. Preoperative knee motion with articulating cement spacer ranged from −5° of extension to 85° of flexion, and lag was 10°. Synovial fluid cultured by agar medium more than 7 days was negative, and blood C-reactive protein (CRP) level of and synovial leukocytes level was less than 1.0 mg/dL, 1000 cells/μL, respectively, after the latest cement spacer prosthesis was implanted. All preoperative tests including liver function, renal function, complete blood count, and blood coagulation ability were within normal limits.\n12 months after the last surgery, the second revision TKA was performed under general anesthesia, using a rotating-hinge knee implant (NexGen RH Knee, Zimmer, Warsaw, IN, USA). Prior to surgery, the limb was exsanguinated and the tourniquet was inflated to 280 mm Hg. The total tourniquet time was 167 minutes. The tourniquet was released before closing the surgical incision, and a 1200 mL hemorrhage was observed. The source of bleeding was unclear but stopped with hemostatic techniques and the surgery was completed (). Immediately after surgery, the dorsalis pedis pulse weakened, and, four hours after surgery, the patient presented foot coldness, decreased sensation, and paresthesias; the dorsalis pedis pulse was not palpable. Emergency arteriography revealed arterial wall dissection and occlusion of the proximal popliteal artery (). Thrombectomy with a Fogarty catheter was unsuccessful, so femoral artery to posterior tibial artery bypass with large saphenous vein graft and relaxing incision was immediately performed. The resumption of the lower limb circulation was confirmed by arteriogram 12 hours after the second revision TKA ().\nAfter surgery, wound healing was delayed and additional debridement and exchange of insert were undertaken 1 month after the rerevision TKA, and a full-thickness skin graft was performed 7 months after the second revision TKA. The patient was discharged from the hospital 12 months after the second revision TKA. Therefore, amputation was avoided, but drop foot by ischemic lower limb paralysis remained. However, he could walk with a cane and achieved active range of motion of the knee from −20° to 100°. |
A 38-year-old female patient presented to the ED with a 12-hour history of sharp, pleuritic, right-sided chest pain. The pain was worse with movement and was not associated with any shortness of breath or respiratory symptoms. She was otherwise healthy and had no history of, or risk factors for coronary artery disease or venous thromboembolism. One day prior to her presentation, she had received dry-needling treatment through a physiotherapy clinic for an unrelated back pain.\nOn presentation to the emergency department, she looked well. Her heart rate was 101, blood pressure was 105/69, respiratory rate was 18 and oxygen saturation was 98% on room air. Physical examination revealed slightly reduced air entry on the right with auscultation. Electrocardiogram (ECG) showed moderate voltage criteria for LVH and no ST changes. Cardiac enzymes were not measured. Point of care ultrasound (POCUS) was used to investigate a possible pneumothorax and revealed normal lung sliding on the left (Video ), but absent lung sliding and the presence of a lung point on the right (Video ). There was no evidence of other lung pathology or a pericardial effusion on ultrasound. A subsequent chest radiograph showed a small to moderate-sized right-sided pneumothorax with 2.2 cm of parietal pleural separation (Figure ).\nUsing procedural sedation, a 9 French Cook catheter was placed in the right hemithorax. Placement was confirmed with a chest radiograph which also demonstrated re-expansion. The patient did have a transient episode of atrial flutter following chest tube placement. Her vital signs returned to normal and no treatment was required.\nThe patient returned to the ED two days after initial presentation for reassessment. Upon return, she was in no apparent respiratory distress. Her heart rate was 80, blood pressure 116/83, respiratory rate 20 and oxygen saturation 92% on room air. She had good air entry bilaterally and a repeat chest radiograph showed re-expansion with no evidence of pneumothorax. The chest tube was removed, and the site sutured with no further complications. |
A 76-year-old female was admitted to the emergency room of our hospital with the sudden onset of upper abdominal and back pain. At the time of presentation, blood pressure, heart rate, and O2 saturation were 133/70 mm Hg, 70 beats/minute, and 95%, respectively.\nAn emergent chest computed tomography was performed under the suspicion of an acute aortic dissection, which showed an acute stanford type B aortic dissection. The patient was then transferred to intensive care unit for strenuous supportive care. During the first 2 days of anti-impulse therapy and intensive care, abdominal and back pain improved. She was then transferred to the general ward.\nHowever, on the 8th day, she complained of general edema and indigestion, while her blood creatinine level was elevated from 0.6 mg/dL to 2.9 mg/dL. A follow-up chest CT revealed that the size of false lumen in descending aorta was increased and that of true lumen decreased significantly. We concluded that increased blood flow in false lumen significantly decreased the rate of blood flow in branched vessels of the descending aorta, which then developed malperfusion syndrome ().\nWe decided that emergent operation was very risky because of the patient's old age, poor nutritional state and acute renal failure. Therefore, the TEVAR was selected as an alternative. The primary goal of TEVAR was to cover the primary tearing site in descending aorta around the diaphragm to reduce the flow of the false lumen and expand the true lumen. The patient was transferred to cardiac intervention room. The patient then underwent general anesthesia, and the right common femoral artery was exposed. The guiding wire and diagnostic catheter was introduced through the right femoral artery. After the locating the true lumen and the primary tearing site, 30×26×150 mm SEAL aortic stent graft (S&G Biotech, Seongnam, Korea) was introduced through the right femoral artery. The primary tear at the level of distal thoracic aorta was covered by the stent graft, and the distal end of stent graft was then deployed proximal to the celiac axis. After the stent graft insertion was completed, the aortogram showed an increased flow of the true lumen and the perfusion of the branch vessels in the abdominal aorta was recovered ().\nAfter TEVAR, the general edema, abdominal and back pain subsequently resolved, and the blood creatinine level went down to 1.3 mg/dL. On the 8th day after TEVAR, she was discharged without any procedure-related complications. During the 1 year clinical follow-up, the patient had remained stable and had no evidence of a cardiovascular event. |
An 11-week-old boy was born at 36.3 weeks via spontaneous vaginal delivery with a birth weight of 3.2 kg and normal APGAR scores. He had an uncomplicated three-day NICU stay for mild respiratory support and feeding assistance and was discharged home where he continued to grow and develop appropriately. At 11 weeks of age, the parents noticed that the infant was not moving his right arm while crying. They brought the infant to the pediatrician's office early the next morning where it was suspected that he had a radial head subluxation. The pediatrician attempted to reduce it twice by twisting the baby's arm. However, he was not able to hear or feel it “pop” back into place and referred the patient to our hospital for orthopedic evaluation.\nUpon admission to the hospital, a physical exam revealed that the baby's right upper extremity was warm and swollen with grimace on manipulation. The remainder of the examination was unremarkable. A radiograph revealed a right distal humeral nondisplaced spiral fracture with soft tissue swelling as seen in . Given the concern for NAI, a complete physical exam to rule out signs of abuse was negative for retinal hemorrhage, burns, purpura, or ecchymosis. In addition, a skeletal survey and head CT ruled out any additional fractures or signs of intracranial hemorrhage. An in-depth psychosocial assessment revealed that the infant was the third of three children living at home with his parents and two older sisters (aged 3 years and 19 months). The parents were the primary caretakers and all family members were healthy. Ultimately, it was decided that the family was reliable and the overall presentation was not consistent with child abuse.\nThe workup then shifted to identifying an underlying metabolic bone disease. Biochemical investigation revealed normal levels of Ca (10 mg/dL) and phosphate (5.1 mg/dL), elevated alkaline phosphatase (595 U/L) and PTH (120 pg/ml), and low levels of 25 hydroxyvitamin D (<13 ng/ml) and 1,25 dihydroxyvitamin D (13 pg/ml) as seen in . A repeat physical exam and X-rays failed to show any craniotabes, widened sutures, rachitic rosary, or enlarged wrists. Further questioning revealed that the mother had inconsistently been taking prenatal vitamins during her pregnancy, was exclusively breastfeeding without any vitamin D supplementation, and had limited her own dairy intake in an effort to make the patient less colicky. The findings at this time were most consistent with rickets. |
Our patient is a 40-year-old Asian man with complaints of bleeding and discomfort in his anus of 2 months’ duration. He was an employee with average income who did not smoke tobacco or drink alcohol. He had no weight loss or urinary symptoms, and no substantial family history. He denied any significant medical or surgical history. His abdomen was soft, non-tender, and non-distended, with normoactive bowel sounds. In examination, a mass could be touched by finger tips. The mass was large and bleeding. In subsequent examinations, blood was detected in a stool sample. His vital signs were: blood pressure, 130.77 mm Hg; respiratory rate, 18 breaths/minute; heart rate, 83 beats/minute; and temperature within normal limits. Oxygen saturation was 98% on room air on admission. In colonoscopy, a large lobular tumor was diagnosed at 4 cm above the dentate line, which was suspicious for malignancy. Various samples were taken from the tumor. The rest of his large intestine did not show a clear pathologic lesion in the colonoscopy.\nHigh-grade adenocarcinoma was reported in pathological examinations. In subsequent diagnostic procedures, his carcinoembryonic antigen (CEA) level was normal. Computed tomography (CT) scans revealed that metastatic lesions were not detected in his liver, abdominal viscera, and chest. In CT scans with or without contrast, and magnetic resonance imaging (MRI) scanning, an ectopic kidney was detected incidentally on his right pelvis without any prior urinary symptoms. The left kidney was in its original location, and both kidneys were functional. Renal function tests provided normal results.\nIn subsequent investigations done by MRI scanning for staging the tumor, a pelvic rectum tumor was reported to be interfering with the T3 N1 mesorectal lymph nodes (Figs. , ). The case was discussed in a multidisciplinary cancer team; afterward, our patient was regarded as a candidate for neoadjuvant radiotherapy. He underwent 45 GY radiation in 25 fractions to the pelvis along with capecitabine. He underwent total mesorectal excision (TME) surgery to maintain the ectopic kidney 6 weeks later. After abdominal exploration, his abdominal viscera were examined. There was no metastatic lesion in his liver and abdomen (Fig. ).\nAfter mobilization of the left colon and the splenic flexure, and the closure of the inferior mesenteric artery (IMA), in the avascular plane, the mesorectum was separated from the fascia propria, and the mesorectal lymph nodes and hemorrhoidal vessels in the anterior and pelvic nerves were fully mobilized, and the distal rectum was removed by an appropriate margin (Fig. ).\nHis right kidney was completely inside the pelvis, and while the kidney was carefully protected by the retractor, an attempt was made to minimize the damage to the ectopic kidney because there was a possibility of damage to the pelvic nerve and nephrectomy.\nThe blood of the right kidney appeared to be supplied by the right superior iliac artery. During the surgery, hematuria occurred to our patient, which was resolved by hydrating him. Then, coloanal anastomosis and temporary ileostomy were performed on our patient. He was transferred to our intensive care unit (ICU). He underwent laparotomy again due to anastomosis leakage a week following the surgery. As a result, a colostomy was performed. Postoperatively, after the reappearance of symptoms, stabilization, and healing of the wounds, he was referred to medical oncology and started adjuvant chemotherapy with 5-fluorouracil, folinic acid, and oxaliplatin (FOLFOX). Follow-up testing (for a year) included routine medical history and physical examination (every 3–6 months), blood tests such as serum CEA, colonoscopy, and radiologic imaging. He was dissatisfied with the permanent colostomy after the end of the treatment. However, the satisfying result was that his kidney was preserved (Additional file ). Before the surgery, the potential risks and damage to his ectopic kidney and the possibility of its removal were explained to our patient and his consent was obtained. His general condition is appropriate after 1 year and his quality of life has been reported to be satisfying despite the permanent colostomy. |
Patient 43 y.o., male, high fall accident, with bilateral calcaneum fracture, both type III of Sanders’ classification. (-). Both legs were posed in anti declivous position and checked every day for two weeks. After this period the right foot showed a compromised conditions of the soft tissues with the presence of blisters but the left foot showed a recurrence of skin folds. For this reason we have chosen to treat the left foot with ORIF () and the right one with closed reduction and application of an external fixator (). Post-operatively, weight-bearing was not permitted for eight weeks for both legs. The fixator was removed after 10 weeks (-). Patient was followed up radiologically with radiographs at 2, 12 and 24 months, and clinically at 15 days, 1, 3, 6, 12, 18 and 24 months with the Maryland Foot Score, as suggested by Sanders et al. []. In our case report we obteined a good reduction of the fractures with both surgical techniques with a restoration of physiological angles of both calcaneum. Böhler’s angle before surgery was 6° in the left one and 3° in the right side, after surgery it was 27° in the left foot and 37° right one. The axial varus angle before surgery in the left calcaneum was 32° and in the right calcaneum was 28°, afther surgery it was 7° in the left calcaneum and 6° in the right one. The height before surgery in the left one was 23 mm and in the right one was 22 mm, after surgery it was 34.7 mm in the left calcaneum and 38,3 in the right one. The lenght before surgery in the left one was 68.3 mm and in the right one was 69.5 mm, after surgery it was 80 mm in the left calcaneum and 81 in the right one (Tab 1). The clinical outcomes showed, until one year’s follow-up, better clinical results in the foot treated with external fixator, (Maryland Foot Score 83 p), then in the foot treated with ORIF (Maryland Foot Score72 p.) (--). After one year the outcomes resulted good (Maryland Foot Score 87 p.) in the foot treated with ORIF and fair (Maryland Foot Score 69 p.) in foot treated with external fixation |
Our patient was an 83-year-old woman with a past medical history of type 2 diabetes mellitus, hypertension and microcytic anemia who lived in rural Maryland and worked at her family’s produce stand.\nThree weeks prior to her admission for weakness and hyponatremia, she presented with abdominal pain, and was found to have a urinary tract infection and hypontremia. Her sodium on the first admission was 121 mEq/L and she was discharged with sodium of 129 mEq/L. She did well for several weeks until she had sudden onset diffuse body pain followed by arm weakness one week prior to presentation. She presented to her primary care physician after the onset of proximal bilateral arm weakness, and during diagnostic work-up she was found to be hyponatremic.\nOn presentation on this admission, her vital signs were within normal limits, except for a mildly elevated blood pressure. The exam was grossly normal except for 3/5 muscle strength in the biceps, triceps, deltoids and quadriceps. There were no rashes present and the patient denied any recent history of rashes. She was hyperglycemic, with sodium of 121 mEq/L after correction. She was afebrile with no elevated white blood cell count. Imaging of chest was negative for masses or other abnormalities.\nLab values obtained during her hospitalization are displayed in and .\nThere were two problems we faced: hyponatremia and muscle weakness. To evaluate her hyponatremia, thyroid stimulating hormone, cortisol and computed tomography (CT) head were evaluated and found to be within normal limits. Medications which could have contributed to hyponatremia were discontinued (lisinopril). The physical exam suggested euvolemic hyponatremia, with urine and electrolyte studies confirming SIADH of unknown etiology. In following the management guidelines, she was fluid restricted to 1000 mL/day. After three days, sodium chloride (NaCl) pills three times daily (TID) were added as her sodium levels and symptoms were minimally improved with fluid restriction, alone.\nTo evaluate her muscle weakness, she was seen by nephrology and neurology. Magnetic resonance images of head, spine and brachial plexus were unrevealing. Lyme screening was performed given her residence in and endemic area and resulted positive with many immunoglobulin IgG and IgM bands appearing. Lumbar puncture was performed, demonstrating lymphocytic pleocytosis. Together with the Lyme serology, the lumbar puncture made neuroborreliosis the leading diagnosis in our patient and infectious disease specialists recommended prompt treatment.\nHer sodium was 137 mEq/L when we started treatment with ceftriaxone, having normalized two days prior. The day after antibiotics were started, her sodium trended down to 132 mEq/L. By the time of antibiotic initiation, the patient had been taking NaCl pills 1 g TID for eight days and her muscle weakness was largely unchanged. After 4 days of appropriate treatment with ceftriaxone, her sodium was 143 mEq/L and she was discharged on one NaCl tablet daily. Her muscle weakness had also markedly improved by discharge. Her strength was 5/5 in quadriceps bilaterally, 4/5 in the biceps/triceps, 3/5 in deltoids, and she was without any pain. One month after she was discharged, muscle weakness had completely resolved.\nImportantly, she decided to forego salt replacement or fluid restriction upon discharge and presented to her primary care physician after two weeks with completely normal sodium levels and no residual weakness. |
A 53-year-old Middle Eastern male with a history of type II diabetes complicated by peripheral neuropathy presented to the emergency department with numbness and tingling in hands and feet bilaterally nine days after a positive SARS-CoV-2 test. He reported that his paresthesias were different in distribution from his baseline. The patient was discharged with a referral to neurology. The following day he presented with paresthesias extending to the groin and bilateral face. Paresthesias were attributed to acute worsening of his chronic diabetic neuropathy with overlying hyperventilation due to anxiety, and he was admitted for observation.\nOn day one of admission, vitamin B12, folate, methyl-malonic acid, serum protein electrophoresis, and urine protein electrophoresis were within normal limits and SARS-CoV-2 IgG antibodies were reactive. Computed tomography of the spine revealed no acute fracture or deformity. On day three, neurology was consulted, and his examination did not demonstrate any focal weakness. On day four, his examination was significant for left-sided facial droop, dysarthria, bilateral grip weakness worse on the left, right foot drop, and absent bilateral biceps and patellar reflexes. At this point, continued, rapid progression of peripheral neuropathy resulted in the suspicion of GBS and initiation of IVIG 0.4/kg/day for four days. On day five, the patient required supplemental oxygen, and on day seven, he had increasing oxygen requirements due to progressive ascending paralysis involving respiratory and bulbar muscles. On day nine, the patient was placed on bilevel positive airway pressure due to respiratory distress and was transferred to the medical intensive care unit. On day 10, he was intubated due to diaphragmatic weakness. Five doses of plasmapheresis were given on day 17 through day 22. His oxygen demands improved, and he was extubated on day 22. The patient received two additional doses of IVIG on day 23 and 24 with improvement in symptoms. Nerve conduction studies on day 23 showed large-fiber sensorimotor polyneuropathy with findings of axonal demyelination, consistent with the clinical suspicion of GBS. The patient continued to improve clinically after treatment and was discharged to a long-term rehabilitation center. |
A 20-year-old unmarried girl presented to emergency with painful urinary retention since 8 h. She had history of similar episode 2 months back for which she was catheterized for 1 week. She also complained of frequency, poor stream, straining, incomplete voiding, and dysuria for 2 years. She noticed a mass occasionally coming out of vagina on straining. There was no history of hematuria, flank pain, urinary incontinence, menstrual disturbances, or sexual contact. Urinary bladder was palpable on abdominal examination and genital examination revealed a large non-tender cystic mass over anterior vaginal wall. She was catheterized and drained about 800 ml of urine. Routine blood picture and serum chemistries were within normal limits. Urine examination revealed 5-8 pus cells per high power field and sterile culture. Abdominal ultrasonography showed an infravesical mass with no dilatation of upper urinary tracts. Transvaginal sonography performed with broadband 9- to 5-MHz tightly curved array probe revealed a hypoechoic cystic structure posterior to urinary bladder and urethra with enhanced through-transmission and a small echogenic structure within it, possibly a stone. Communication with the urethra could not be delineated. A magnetic resonance imaging (MRI) of pelvis was done which showed a well-defined cystic structure posterior to urinary bladder measuring 7.3 × 6.2 cm displacing the uterus superiorly and vagina and cervix posteriorly with no obvious connection with urethra or urinary bladder seen []. Cystourethroscopy was performed using 15F flexible cystoscope with simultaneous digital compression over the bladder neck area and diverticulum. It revealed a large UD opening into the urethra in the right postero-lateral wall of the middle third. A rubber catheter tip measuring about 1 cm was seen inside the diverticulum, a retained foreign body from previous catheterization.\nTrans-vaginal excision of diverticulum was done through an inverted U-shaped incision over the anterior vaginal wall. Vaginal flaps were raised with careful preservation of the periurethral fascia and meticulous dissection of the diverticulum up to the neck. Diverticulum was excised and repair was done over 14F catheter in four layers, e.g. urethra, periurethral fascia, Martius flap, and vaginal tissue [Figures , ]. Suprapubic catheter was not placed and anticholinergics were given in the post-op period for three weeks. The urethral catheter was removed at 3 weeks, after voiding cystourethrogram (VCUG) revealed no extravasation [Figures , ]. She is presently asymptomatic three years after surgery. |
A 32-year-old female, graduate, married, was brought to the psychiatry OPD by her father along with complaints of suspiciousness toward her husband and family members, episodes of increased anger, and abusive behavior over the span of 10 years. Over the period of 10–12 years, the patient gradually became suspicious of her family members, she believed that her father and her brother are conspiring against her and were planning to assault her. Gradually her illness progressed and she was seen muttering and gesturing to self, her hallucinatory behavior increased, and was accompanied by episodes of increased anger and violent outburst toward her family. She was treated by multiple faith healers, and doctors but always had poor compliance. However for the past 4–5 years, she was maintaining well and was able to complete her education and got married. Immediately after few months of marriage, symptoms reappeared and she became suspicious toward her husband's fidelity, and later started becoming hostile against her in-laws. Detailed examination revealed that she was sexually abused by her brother from the age of 12 years. He used to make sexual contact with her when she went to sleep. This continued for 1–2 years, and subsequently, her brother was diagnosed with schizophrenia. The patient also reported that she always had increased sexual appetite; she even reported a history of hypersexual behavior for the past 6–7 years. After getting married, she used to demand for excessive sexual intercourse with her husband, so whenever husband used to deny having sexual contact with her, she will get irritable and started doubting his fidelity. On serial MSE, rapport was established with difficulty, she had multiple well-systematized delusion of infidelity, delusion of reference, and tactile hallucinations (she use to feel that someone is penetrating her repeatedly). She also had increased sexual urges. Her insight was grade 1 and her judgment was impaired. With a diagnosis of schizophrenia, she was treated with antipsychotics and reported improvement in symptoms. |
A fit 45-year-old man, with a background of Brugada Syndrome had an ICD inserted 10 years previously for secondary prevention. He was initially admitted for lead replacement and ICD box change. This procedure was attempted, however failed due to an occlusion of the brachiocephalic vein. Therefore, a laser-assisted lead extraction was undertaken. Immediately following the procedure, the patient showed sign of tamponade and a pericardiocentesis was carried out. This was unsuccessful and the patient became hemodynamically unstable. He was transferred immediately to the operating theatre, intubated, ventilated and proceeded for emergency sternotomy. On arrival to theatre, the patient developed pulseless electrical activity and external chest compressions were delivered for 30–60 s.\nFollowing sternotomy, the pericardium was opened and copious amounts of blood and clots were removed with prompt restoration of spontaneous circulation.\nA bleeding point was identified on the right ventricular acute margin, which was repaired using a single 3/0 pledgeted Prolene suture. A large volume of blood was also noted in the right pleura, with extensive infiltration of the extra-pericardial portion of the superior vena cava. Careful dissection revealed a 5–7 cm linear tear extending from the right subclavian vein to the pericardial reflection of the Superior Vena Cava (SVC; ). In order to repair this, the patient was connected to cardiopulmonary bypass and cooled to 25° without circulatory arrest. A patch of bovine pericardium was used to reconstruct the SVC and the right subclavian vein (). The patient was rewarmed and hemostasis secured.\nHowever, the patient remained hemodynamically unstable and the hemoglobin was noted to be low. The abdomen was very distended and an ultrasound demonstrated free abdominal fluid. The possibility of having a CT scan was dismissed due to the unstable condition of the patient. He proceeded for an emergency laparotomy with 4 L of blood drained from the abdomen. A deep laceration in the posterior part of the right lobe of the liver was eventually identified. The bleeding was difficult to control requiring mobilization of the liver and packing. The bleeding was eventually controlled. After 48 hours, a re-exploration of the abdomen was carried out and the liver laceration was still bleeding on pack removal requiring specialist repair by the hepatic surgeons. The patient eventually made a good recovery and remains alive and well. |
An 86-year-old Caucasian man was referred for management of a dislocated IOL in the left eye 15 years after cataract surgery. The IOL was in the posterior pole on initial examination with a BCVA of 20/40 and an IOP of 11 mmHg. He underwent a 23-gauge PPV with lens removal and secondary sutured IOL placement. The eye was found to be hypotonous, with pressures not exceeding 4 mmHg, during the first postoperative month. The patient also had a hyphema and vitreous hemorrhage with vision fluctuating from 20/400 to hand motions. He was found to have chorioretinal folds by SD-OCT () and received an intravitreal injection of triamcinolone acetonide 4 mg/0.1 mL with minimal improvement in IOP over the following 2 months. Contact B-scan ultrasonography demonstrated a cyclodialysis cleft possibly exacerbated by a subluxed IOL. He was treated conservatively with atropine 1% and prednisolone acetate 1% without improvement. He then underwent pupilloplasty and repositioning of the IOL and sequential rounds of diode laser to the cleft over the following 2 months with slow increase in pressure from approximately 1 mmHg to 10 mmHg. The maculopathy persisted for several months after the cleft closure with a near visual acuity of Jaeger 2. His pressure gradually decreased again, and he was treated with a 23-gauge PPV, membrane peeling, Healon injection, and cryotherapy to the cyclodialysis cleft to induce elevation in IOP. However, his IOP rose to 48 mmHg. A vitreous chamber tap was performed but rebound increases in IOP occurred, and the patient was taken back for a 23-gauge PPV and Healon removal 3 days after initial injection. Postoperatively, his IOP remained in the low-teens and vision stabilized. His chorioretinal folds by SD-OCT resolved () over the ensuing 2 months, and 17 months after his most recent surgery, the BCVA had improved to 20/40 with a stable IOP ranging from 9 to 12 mmHg. |
The patient was a 27-year-old lady referred by her orthodontist for restorative treatment of multiple anterior maxillary diastemas (). The unit internal review board approved the case report (2019-0923) on 23 September 2019. Clinical and radiological examination showed healthy incisors with no previous restorations and no signs of periodontal disease (). Measurements from the study models revealed a significant Bolton discrepancy and a 1.9 mm diastema between central incisors associated with 1.1 mm bilateral diastemas between central and lateral incisors.\nBecause of the young age of the patient, a noninvasive treatment with sectional veneers was recommended to close the spaces and restore the natural appearance of the smile. However, the size of the spaces to be closed was a possible contraindication for restoration with sectional veneers because the increased tooth width could result in altered width-to-length ratio and less than ideal tooth proportions. Ideal tooth proportions have been extensively discussed in the literature and many different ratios have been introduced to be used as esthetic guidelines for tooth dimensions [,,]. However, these ratios rarely occur in smiles deemed attractive by laypeople and dental professionals and their rigid application to predict ideal tooth size is controversial [,,,,]. An alternative approach to provide a guideline for ideal tooth dimensions is to use average tooth size in the human population as a reference. Anatomical values of tooth dimensions are well established in the literature and show that, although tooth width and length varies with gender, age and race, tooth width/length ratio in the upper anterior dentition is a stable, reference falling within a range of 72% to 85% [,,]. For the patient presented in this report, the diagnostic wax-up revealed a favorable 82% width-to-length ratio and confirmed that, despite the moderate size of the diastemas, restoration with sectional veneers could be completed without unbalancing natural tooth proportions ().\nOnce the preliminary measurements were completed and the treatment plan with sectional veneers was approved by the patient, the clinical procedure started with fitting the prefabricated veneers to the size of the diastema.\nThe prefabricated composite veneers for the patient presented in this case report (Edelweiss Veneers, Edelweiss Dentistry, Wolfurt, Austria) are fabricated with a nanohybrid composite cured with high temperature and high pressure to produce a veneer with improved mechanical properties compared to a conventional composite restoration [,]. In addition to the heat/pressure curing process, the tested veneers also feature a proprietary laser sintering technology that extracts the resin component from the composite shell and produces a veneer with high inorganic content and a highly esthetic, laser-sintered surface [,] ().\nEdelweiss Prefabricated Veneers are available in four sizes (XS, S, M, L) and a custom sizing guide is included in the system to select the veneer that best fits the deserving tooth. For the patient in this case report, small veneers were selected.\nAfter veneer size selection, the width of the diastema was measured with a digital caliper and transferred to the veneer using a thin marker (a,b).\nA diamond separating disc (ZR943, Komet, Lemgo, Germany)was used to cut the veneer along the marker line and produce a sectional veneer that exactly fits the space to be closed (a).\nAfter trying the sectional veneer in its eventual position to confirm the size and shape (b), a retraction cord was packed around the tooth (Ultrapack size 00, Ultradent, South Jordan, UT, USA) to prevent contamination from the crevicular fluid and to produce the gentle displacement of the soft tissue. The cord was positioned tooth by tooth only when the veneer was ready to be delivered and was removed as soon as isolation was no longer necessary. A thin retraction cord was used since the try-in showed how the emergence profile of the sectional veneer already produced effective soft tissue compression with no residual black triangle and no need for extra soft tissue displacement. Another reason for using a thin retraction cord was the thin scalloped gingival phenotype and the risk of stressing the delicate soft tissue.\nThe Edelweiss Veneer requires no sandblasting, no acid etching and no silane application for bonding. However, the manufacturer recommends internal conditioning with a dedicated resin primer (Veneer Bond, Edelweiss Dentistry, Wolfurt, Austria) to promote chemical adhesion and increase bond strength between the highly inorganic composite laminate and the luting composite.\nAfter Veneer Bond application, the prefabricated Veneers are ready for bonding using a complementary nanohybrid composite resin available in several dentin and enamel shades (Edelweiss NH, Edelweiss Dentistry, Wolfurt, Austria). Small- and medium-sized sectional veneers (i.e., diastema width <2 mm) are usually bonded with an enamel shade only. Larger sectional veneers are usually bonded with the combination of a dentin shade in the gingival area and an enamel shade in the incisal area in order to produce gingival–incisal color gradation and an increased background masking effect. For the patient presented in this paper, all the sectional veneers were bonded with an enamel shade only ().\nOnce the sectional veneer was loaded with the selected composite shade and was therefore ready for bonding, the tooth was etched with 35% orthophosphoric acid followed by the application of a single-step adhesive, according to the manufacturer instructions (Peak Universal, Ultradent, South Jordan, UT, USA). Then, the first sectional veneer was seated in position and gently pressed until it made contact with the adjacent tooth (). A thin spatula (IPC, American Eagle Instruments, Missoula, MT, USA) was used to sculpt the extra composite and achieve optimal adaptation between the veneer and the tooth (). Finally, the veneer was light cured for 20 s from the lingual direction plus 20 s from the buccal direction using a high-power curing light (Demi Plus, Kerr Corporation, Brea, CA, USA).\nOnce curing was completed, the margins of the sectional veneer were finished and polished with conventional composite instruments. The tested prefabricated veneers do not require special equipment and can be effectively polished with most of the commercially available polishing systems for nanohybrid composite resin. In the case presented in this report, a 50-micron needle point diamond bur (Brasseler DET3F, Brasseler, Savannah, GA, USA) was used to smooth the extra composite, followed by a silicone cup (Identoflex Composite Polisher, Ravelli, Milano, Italy) to remove the residual scratches () and a paper finishing strip (Sof-Lex, ESPE 3M) to polish the interproximal area (). Finishing and polishing is limited to the margins of the sectional veneer and no instrumentation is required on the buccal and lingual surfaces, since prefabricated veneers are pre-shaped and pre-polished to the ideal anatomic form, with accurate superficial anatomy and a high-gloss, laser-sintered surface.\nOnce all the veneers were bonded in position, the patient was dismissed and rescheduled for a post-operative evaluation two weeks later. At the recall appointment, a functional evaluation (absence of fractures, marginal adaptation), a biological evaluation (soft tissue response, post-operative sensitivity) and an esthetic evaluation (gloss, color matching) were completed and were fully satisfactory ( and ). As a final recommendation, oral hygiene instructions for the interproximal dental spaces were reviewed and the patient was rescheduled for a regular 6-month follow-up appointment.\nThe highly translucent and glossy surface of the Veneer produced optimal esthetics with invisible transition between the veneer and the tooth. The lack of tooth preparation and the single-appointment procedure with no impressions, no temporaries and no try-ons minimized the stress for the soft tissues and greatly contributed to the reduced post-operative discomfort and excellent acceptance by the patient. |
A 70-year-old white woman presented to my institution with severe pruritus. She had been diagnosed with stage IV adenocarcinoma of the ovaries with metastasis to the liver 1 year prior. She had undergone three rounds of carboplatin paclitaxel, followed by debulking surgery, followed by an additional six rounds of carboplatin paclitaxel therapy. After her last chemotherapy treatment, her carcinoembryonic antigen normalized. Four months later, she presented with jaundice, dark urine, and pale stools. A computed tomographic scan revealed the cancer had returned to the liver and was now obstructing her biliary duct. A stent was attempted several days later but was not successful. In collaboration with her oncologist, it was decided not to pursue further active treatment, and palliative care was initiated. She was otherwise healthy. The only medication she was taking was pantoprazole 40 mg daily for acid reflux. Her weight was 80 pounds.\nOne month later, she developed intense pruritus throughout her entire body. It was worse at night, and her sleep was significantly disturbed. She indicated her pruritus was 8/10 on a numerical rating scale (NRS), with a score of 0 reflecting no pruritus and a score of 10 reflecting the worst pruritus. She had no significant skin lesions or excoriations. She first tried over-the-counter moisturizers, with minimal effect. Benadryl was then suggested; it decreased her night waking, but she was too drowsy the following day. Loratadine was also attempted, to no effect. Cholestyramine was suggested, but she declined it due to the gastrointestinal side effects and longer time until onset of action.\nThe etiology of her pruritus appeared to be centrally induced, as opposed to prurioreceptive, neuropathic, or psychogenic []. My colleagues and I decided to try paroxetine on the basis of its preferable side effect profile, particularly in that it is less sedating. She was started on 5 mg nightly, with the intention to increase the dose as tolerated. The first night, her pruritus was 50% improved (NRS 4.5/10), and she was able to sleep better. The second night, her pruritus had fully resolved (NRS 0/10). She reported no nausea or vomiting. She was gratified with the effectiveness of paroxetine. She continued on 5 mg of paroxetine nightly, with no pruritus until she died 1 week later. |
A 54-year-old female patient presented with gradually progressive painless lump in the left breast for 3 months. She had no specific family history or any other systemic symptoms. On clinical examination, there was a firm, mobile, nontender breast lump in upper inner quadrant of the left breast measuring approximately 2.5 × 2 cm with overlying skin being normal and no axillary lymphadenopathy or swelling in the contralateral breast. Mammogram showed ill-defined branching soft-tissue density with scattered areas of calcification with a query of dilated duct in upper inner quadrant of the left breast. Ultrasonography revealed a cyst in upper inner quadrant measuring 3 × 2 cm containing echogenic debris along with a hypoechoic solid appearing lesion seen in continuity with its wall measuring 2.6 × 1.5 cm. Final impression suggested possibility of malignancy (BIRADS 4C) with a suspicion of intraductal lesion.\nCore needle biopsy was performed which showed fragmented cores composed of predominantly of fibrocollagenous tissue with benign ducts interspersed in the tissue. A separate small sheet of polygonal cells with eosinophilic cytoplasm was seen. A suspicion of malignancy was raised, and repeat biopsy from representative site was advised (Figure ). On repeat core needle biopsy, again a small focus of atypical cells of above-mentioned morphology was seen. Some extracellular keratin could also be appreciated (Figure ). Hence, a possibility of metaplastic carcinoma with squamous differentiation was considered.\nWide local excision was performed, the cut surface of which showed a cystic lesion filled with whitish material along with presence of a grey white well-defined firm area measuring 3 × 2.2 cm. Microscopic examination revealed a lesion filled centrally with keratinous debris and lined by atypical squamous cells, infiltrating the surrounding stroma as islands and clusters (Figure (C, D)). No other component was seen, and overlying skin was also not involved. Immunohistochemically, these tumor cells were negative for estrogen receptor, progesterone receptor, and her 2 neu with high-proliferation rate (Ki 67 index 60%, Figure ). An extensive workup ruled out possibility of other primary sites or metastasis.\nThe patient was later planned for radical mastectomy with no neoadjuvant chemotherapy. Histopathological evaluation of MRM specimen showed presence of a microinvasive focus. All the axillary lymph nodes were negative for any tumor deposits. The postoperative phase was uneventful, and the patient is in follow-up for last 1 year. |
A 51-year-old white man presented with left thigh pain and inability to bear weight after a ground level fall. This was an isolated injury, which he described as a twisting mechanism with his knee contacting the floor after slipping. Prior to this injury, he was independently ambulant. His past medical history was significant for type-1 diabetes mellitus, retinopathy, and hypertension. His past surgical history was significant for a left corneal transplant 10 years prior, as well as a successful cruciate-retaining TKA 8 years prior. He also had remote history of ACL reconstruction in his twenties; unfortunately previous operative details for this procedure were not available. Radiographic examination revealed a left distal femoral periprosthetic fracture with a well-seated and well-aligned cruciate-retaining implant. A short oblique supracondylar fracture line originated distally from one of his previous ACL ligament augmentation staples (Figs. and ).\nIn our operating room a direct lateral approach through the previous incision was utilized to directly visualize the ACL staple. The staple was visible through the fracture site, and was removed easily (Fig. ). The lateral femoral cortex was intact and had no significant cortical defects from the previous ACL tunnel. The total knee implant was then visualized using the original midline approach. After confirming the stability of the implant and reducing the fracture, cement in the intercondylar fossa was removed to establish the starting point for the intramedullary (IM) nail (Fig. ). The previous ACL tunnel could not be identified. An 11 × 360 mm IM nail was inserted in a retrograde fashion with three locking screws placed distally and one proximally.\nPostoperatively, our patient did well and achieved radiographic and clinical union after approximately 4 months. He underwent hardware removal of a symptomatic distal locking screw 5 months postoperatively without complication (Fig. ). At the final 6-month follow-up, he was ambulating independently and back to preoperative functional capacity. |
A 46 year old woman presented with generalized abdominal pain. Initial evaluation with abdomen/pelvis CT demonstrated wedge shaped areas of low attenuation in her spleen and right kidney consistent with infarction. She also had a dynamic ileus secondary to bowel infarction along with findings suggestive of an embolus in her superior mesenteric artery (SMA). CT also revealed a possible 2 × 4.1 cm “mass” within the LV. Empiric anticoagulation with heparin was started and she underwent urgent exploratory laparotomy, small bowel resection, SMA embolic material removal and patch angioplasty of the SMA.\nTTE on the day after admission showed an LVEF of 65% without regional dysfunction. It also confirmed an LV “mass” near the outflow tract which measured 3.2 × 2.3 cm (Fig. ). The mass was hypovascular and pedunculated with an attachment to the basal anteroseptal wall. Second look surgery on the following day showed that the areas of questionable viability from her first surgery were frankly ischemic, as a result of which she underwent ileostomy and jejunostomy, leaving only 20 cm of viable terminal ileum and the entire colon. She underwent a transesophageal echocardiogram (TEE) 2 days later, which corroborated the TTE findings, revealing an LV “mass” (Fig. ).\nInitial interpretation of the “mass” favored a myxoma but a thrombus was also suspected. A CMR performed a week after her admission showed findings consistent with a thrombus that had embolized from the LV into the aortic arch. On the following day a brain magnetic resonance imaging (MRI) revealed multiple cerebral infarctions in her internal carotid artery territory (Fig. ). Pathology results confirmed that the SMA embolic material was indeed thrombus. Due to difficulty managing her heparin, anticoagulation was changed to enoxaparin. The patient was found to suffer from left middle cerebral and posterior cerebral artery strokes when she had symptoms of right hemiparesis, dysarthria and word finding difficulty. Thrombolysis or thrombectomy were not undertaken as the patient was anticoagulated and had poor carotid access. She had residual right side hemianopsia with no other residual deficits.\nHer anticoagulation was then changed to fondaparinux, to be continued indefinitely. She was ultimately discharged to inpatient rehabilitation with plans to be evaluated later for intestinal transplant due to short gut syndrome. After rehabilitation, she was able to walk but used a wheelchair and walker for ambulating a longer distance.\nThe CMR revealed a LVEF of 60% with normal dimensions and regional systolic function without any LV mass (Additional file : Movie S10, Additional file : Movie S11, Additional file : Movie S12, Additional file : Movie S13).\nThe patient had a bovine aortic arch anatomy and was found to have a 2.8 × 1.9 cm occlusive thrombus in the proximal aortic arch extending into the proximal innominate artery. The thrombus had low signal intensity on bSSFP imaging, short T1 and T2 consistent with methemoglobin, and was hypointense on LGE (Additional file : Movie S14, Additional file : Movie S15, Additional file : Movie S16, Additional file : Movie S17, Additional file : Movie S18, Additional file : Movie S19).\nWe report a case of an LV thrombus that embolized in its entirety to the aortic arch, innominate artery and caused multi focal infarcts in the SMA and internal carotid artery distributions. Our report emphasizes the use of comprehensive CMR imaging in the assessment and differentiation of cardiac masses. In our patient, even though the “mass” had embolized by the time the CMR was performed, its tissue characterization abilities were critical in identifying that it was a thrombus. The mass had a high signal and a short T1 consistent with methemoglobin. Overall, due to a larger field of view, greater spatial resolution, lack of attenuation, and ability to image in any plane along with its superior tissue characterization properties, CMR is the gold standard for assessment of cardiac masses [].\nThere have been several case reports of thrombus mimicking cardiac tumors. LV thrombus formation is a well-known complication of a myocardial infarction, LV aneurysm, cardiomyopathy or a hypercoagulable state. It is uncommon for a thrombus to form in a normal LV in the absence of a wall motion abnormality. Initial interpretation of the “mass” favored myxoma due to the absence of these factors. The specific etiology for formation of thrombus is unclear as her imaging and hypercoagulable work up did not point to a specific cause. Notably, she was on medroxyprogesterone injections but her hypercoagulable work up was negative for anticardiolipin antibodies, antiphospholipid antibodies, lipoprotein A, beta 2 glycoprotein, and JAK (Janus Kinsae) 2 mutation.\nImaging plays a critical role in establishing a diagnosis and planning management. TTE, TEE, and CMR have all been used to detect the presence of cardiac tumors and thrombi []. CMR has a superior sensitivity and specificity of 88 ± 9% and 99 ± 2% in comparison to echocardiography and has enhanced ability to identify thrombi and differentiate from other cardiac masses because of superior tissue characterization []. T1 weighted CMR imaging has been successful in identifying acute/subacute thrombi due to the short T1 effect of methemoglobin [, ]. On CMR, recent thrombi have a shorter T1 than old thrombi due to the presence of methemoglobin and hence a brighter signal on T1 weighted images [].\nIt is important to establish whether the mass is a tumor, thrombus or vegetation due to their different complications and therapeutic implications. Treatment of thrombi includes adequate anticoagulation. In our patient, no intervention was performed as the thrombus had already embolized into the aortic arch.\nThe CMR of Case 3 (Additional file CMR Link, ). |
A 51-year-old male with a medical history of bipolar disorder type II presented to the clinic in June of 2019 with a three-month history of unintentional weight loss (25 pounds), bilateral knee pain, fatigue, exertional shortness of breath, and night sweats but no fevers. One month prior to this presentation, he was treated at a local walk-in clinic with doxycycline in a setting of a suspected tick bite while camping. In the clinic, he denied fevers or chills. Initial laboratory findings are summarized in Table . Since he had elevated inflammatory markers, he underwent autoimmune test and infectious disease workup including tickborne illness, flow cytometry on peripheral smear, and fungal serology, which were all negative. Fecal occult blood test was negative with subsequent normal colonoscopy examination. Computed tomography (CT) of the chest, abdomen, and pelvis showed mild splenomegaly but otherwise no acute pathology.\nIn December of 2019, the patient was seen in the clinic as a routine follow-up during which he reported gradual symptoms resolution and stabilization of his weight over months without any specific treatment. He denied fevers, chills, or night sweats. He still had mild knee pain but was otherwise functional without any deficit. His labs at this time are shown in Table .\nIn June of 2020, he returned to the clinic with similar complaints of weight loss, fatigue, and bilateral knee pain with occasional fevers and night sweats. At this time, he had lost up to 15 pounds since the last visit. Repeat labs are shown in Table . There was a suspicion for HLH even though the lactate dehydrogenase (LDH) was not elevated enough to support this. The patient was referred to the hematology service.\nA week later, he woke up with severe shortness of breath, lightheadedness, and confusion, and was brought to the emergency department (ED). His vital signs included a heart rate of 100 bpm, blood pressure of 98/60 mmHg, temperature of 37.5°C (99.5°F), and pulse oximetry of 98% in room air. Physical examination was notable for an ill-appearing middle-aged male who was alert, oriented but occasionally confused. Respiratory examination revealed clear breath sounds bilaterally. Neurological examination showed no deficit. Venous blood gas was normal, D-dimer was 1.21 ug/mL FEU (normal < 0.49 ug/mL FEU), and haptoglobin was normal; the remainder of laboratory findings in the ED are summarized in Table . Electrocardiography (EKG) showed sinus tachycardia with a heart rate of 104 bpm. Chest X-ray was normal. Computed tomography angiography (CTA) of the chest did not show evidence of pulmonary embolism. CT of the abdomen and pelvis showed mild hepatosplenomegaly (Figures , ). The patient was admitted to the hospital for further evaluation and management.\nFurther infectious disease tests including human immunodeficiency virus (HIV), COVID-19, tuberculosis, Epstein-Barr virus (EBV), cytomegalovirus, syphilis, hepatitis panel, and West Nile Virus (WNV) were all negative. Serum protein electrophoresis (SPEP) was negative for monoclonal gammopathy. Differential diagnosis included slowly growing indolent splenic marginal zone non-Hodgkin lymphoma (NHL), occult myeloproliferative disease such as chronic myelomonocytic leukemia, and hairy cell leukemia, even though his previous flow cytometry was normal. Angioimmunoblastic T-cell lymphoma related NHL or gamma/delta hepatosplenic T-cell lymphoma were also considered though less likely given long clinical course of the patient’s symptoms. Low-grade HLH was also on the differential given the patient met 4/8 diagnostic criteria (hemophagocytosis, splenomegaly, high ferritin, and mildly increased triglyceride (156 mg/dL; normal 50-150 mg/dL).\nThe patient underwent bone marrow biopsy with aspiration, which revealed hypercellular marrow and evidence of hemophagocytosis, though no evidence of B-cell, T-cell lymphoma or Hodgkin lymphoma (Figures , ) was found. The interleukin-2 receptor (IL-2)/soluble CD25 (sCD25) level was significantly elevated at 9,700 pg/mL (normal: 175-858.2 pg/mL). Magnetic resonance imaging (MRI) of the brain was obtained, which showed multiple abnormally enhancing lesions mainly in the right frontal lobe with a small hemorrhagic focus (Figure ). Cerebrospinal fluid (CSF) analysis was not suggestive of an infectious cause. A stereotactic needle brain biopsy revealed no evidence of malignancy or infection. Liver biopsy confirmed evidence of hemophagocytosis with no evidence of malignancy or lymphoma. A diagnosis of CNS- HLH was made, and the patient was started on high-dose dexamethasone 20 mg and etoposide 150 mg/m2 per the HLH-94 protocol for eight weeks alongside prophylactic BactrimÔ DS 800-160mg and acyclovir 400 mg. He had an excellent response with resolution of all his symptoms and normalizations of all HLH parameters (Table ). Follow-up brain MRI showed decrease/resolution of previous parenchymal frontal abnormalities (Figure ). HLH genetic screening test showed no abnormalities. |
In December 2006, a 31-year-old woman was referred to a neurologist because of consciousness disorder and fainting. Her main problems were obesity, snoring and waking up with a feeling of suffocation in the middle of sleep. The intraoral examination showed a large soft palate (). The soft palate was scored as class III according to the Mallampati classification (visualization of the soft palate and the base of the uvula) []. The electroencephalogram (EEG) showed focal dysrhythmia during hyperventilation with scattered sharp waves (). The patient was depressed and had sleep disorders such as sleep apnea and myoclonus, especially at the onset of sleep. She had experienced several occurrences of complete loss of consciousness during swimming and at work. The patient was on anticonvulsants and antidepressants (at first, she had been prescribed with Lamotrigine for 5 months, but later she was given 500mg Sodium valproate per day).\nOne of the best treatments for snoring during sleep is UUUP. The success rate of this type of surgery is reported to be between 16% and 83% [].\nWe chose a minimally invasive surgical procedure for the present case since the patient had a proper facial profile and a large soft palate (class III according to the Mallampati classification) [].\nIn May 2007, after analyzing the lateral cephalogram, we evaluated the craniofacial and pharyngeal airway morphology before the surgery. Under general anesthesia, 1cm of the soft palatal mucosa, from the right tonsil to the left tonsil, was removed. The patient’s tonsils were also removed during the surgery, and the anterior and posterior tonsillar pillars were sutured together ().\nThe symptoms were significantly decreased after the recovery. The patient no longer had sleep apnea, and antidepressants and antiepileptic drugs were discontinued. After the surgery, sharp waves were detected on the EEG at the level of the trachea (), but the patient was clinically asymptomatic. The 10-year follow-up showed no symptoms of sleep apnea or seizure. The patient did not lose any weight during the follow-up period. |
A 23-year-old female reported to department of dentistry with the chief complaint of dull intermittent pain on lower anterior gingiva for 8 years. She gave a vague history of surgery performed in the same region 3 years prior with no respite. She also gave a history of the application of mustard oil, turmeric, sensitivity paste, and other home remedies for it, with no relief.\nHer first dental visit showed gingival desquamation on her mandibular anterior gingiva []. She had numerous pitting on her lower anterior teeth and fibromas on gingiva and tongue [Figure , ]. No pus discharge, tenderness on percussion, or loss of clinical attachment was evident. Her radiographic examination revealed no significant findings [].\nHer medical history was contributory to a diagnosis of TSC when she was 10 years old. Her family history revealed that her elder sister also had TSC and was undergoing treatment for the same. She was very anxious and depressed about her chronic tooth-related pain to the extent of having aborted her studies. Her neurological history was negative for seizures and mental retardation. Axial sections of T2-weighted magnetic resonance imaging of the brain revealed hypointensities in bilateral periventricular and occipital regions suggestive of subependymal calcified hamartomas with a heterogeneous appearing lesion in left lateral ventricle about 1.1 × 1.1 cm, suggestive of a cortical tuber in right post temporal region [].\nShe also complained of constipation. Endoscopy revealed loss of folds of the stomach and video colonoscopy revealed colonic spasm up to terminal ileum. General examination revealed angiofibromas on the face and forehead []. An abdominal ultrasound revealed no kidney abnormality. Ophthalmic examination showed myopic fundus. Blood investigations were unremarkable.\nShe was advised to discontinue all local applications. The mandibular anterior gingiva showed satisfactory uneventful healing after 2 weeks but her unusual oral pain persisted. She was referred to Psychiatry and started on 25 mg amytrptalline tablet once daily. No dental/periodontal treatment was performed. After 2 months, she was found to be asymptomatic and has been happy and comfortable on regular follow-up for the past year.\nPatient's informed consent was obtained for the use of photographs/radiographs for academic purposes. |
A 19-year-old man underwent circumcision at a local urology clinic 3 months before visiting our department. A mass was incidentally detected on the glans penis during the surgery, which was suspected to be a cyst. The patient underwent punch biopsy at a local dermatology clinic and the results were consistent with hemangioma. The patient and his parents desired surgical treatment rather than multiple procedures and presented to the outpatient clinic of our department. The mass at presentation was 1.0 × 0.8 cm in size, painless, non-tender, blue-red colored, and tortuous (Fig. ). Since biopsy performed at the local dermatology clinic suggested a hemangioma, no imaging studies (such as ultrasonography or computed tomography) were performed.\nThe patient was placed in the supine position under general anesthesia. After the operative field was aseptically draped and the patient underwent Foley catheterization to prevent contamination, an elliptical incision line was drawn around the margin using a surgical marking pen. Local anesthesia was induced using 2% lidocaine mixed with epinephrine at a ratio of 1:100,000, which infiltrated the tissue around the mass. The mass was completely excised using a scalpel and bleeding was controlled using bipolar electrocautery (Figs. and ). Frozen biopsy was performed to completely rule out malignancy and showed a benign lesion. After confirming that there was no distortion of structures with the approximation of the skin flap, the resulting wound was closed layer by layer (Fig. ).\nPermanent biopsy showed classic features of cavernous hemangioma, including dilated blood vessels lined with endothelial cells encapsulated by fibrous tissue and containing red blood cells (Fig. ). Total stitch-out was performed 14 days after surgery, and the patient was discharged without complications, such as wound dehiscence or infection. Follow-up 14 months after surgery showed that the wound was well healed without recurrence and the patient was satisfied with the aesthetic result (Fig. ).\nThe patient provided informed consent for the publication of his clinical and radiological data. This study was approved by the Institutional Review Board of Chonnam National University Hospital (CNUH-2018-227) and was conducted in accordance with the principles of the Helsinki Declaration II. |
A 20-year-old Chinese woman, with anemia and thrombocytopenia, was admitted to the Hematology Department of our hospital due to progressive fatigue.\nThe patient presented with progressive fatigue three months ago, which had significantly worsened in the previous few days. Additionally, she had experienced intermittent knee pain with morning stiffness of both legs for almost six months. She had not seen a doctor until this hospital visit. She attended the emergency department of our hospital and initial laboratory tests showed anemia and severe thrombocytopenia. She was then admitted to the Hematology Department where further laboratory work-up was performed. On the second day of hospitalization, she was transferred to the ICU due to severe respiratory distress and shock.\nThe patient had no previous medical history.\nThe patient did not have a history of smoking, drinking or drug abuse.\nOn physical examination, the patient was pale, awake, alert, responsive to questions and in acute respiratory distress. There was some skin petechiae, indicating a bleeding tendency, but there was no skin rash, oral ulcers, alopecia or enlarged lymph nodes. Her heart rate was 140 bpm, blood pressure was 112/70 mmHg with norepinephrine continuously pumped (0.8 μg/kg/min), respiratory rate was 42 breaths/min, and temperature was 37.6 °C. The oxygen saturation remained at 80% on room air and increased to 94% on a high-flow nasal cannula with FiO2 of 40%. These findings suggested severe circulatory shock and respiratory failure. Heart auscultation showed low heart sounds without murmurs, and there were crackles over both lung fields, indicating heart failure associated with pulmonary edema or pneumonia. Her abdomen was soft and not tender, and the liver and spleen were not palpable. She had joint line tenderness in both knees and mild edema in both lower extremities.\nThe initial laboratory tests are shown in Table . Blood tests revealed mild leukocytosis 10.71 × 109/L with moderate anemia (hemoglobin 71 g/L) and severe thrombocytopenia (platelet count 33 × 109/L). Alanine aminotransferase (98 IU/L) and aspartate aminotransferase (301 IU/L) were increased, which may have been attributed to liver congestion induced by heart failure. Creatinine (1.54 mg/dL) was slightly elevated, indicating mild acute renal damage. Activated partial thromboplastin time and prothrombin time were roughly normal. Cardiac damage markers, including myohemoglobin (864.3 ng/mL) and hypersensitive troponin T (142.9 ng/L), were increased. Her plasma N-terminal pro-B-type natriuretic peptide level (> 35000 pg/mL) was significantly high. Autoimmune profiles showed hypocomplementemia with C3 of 36 mg/dL and C4 of 13.7 mg/dL, positive antinuclear antibodies with a titer 1:1000 (speckled nuclear pattern), positive SS-A antibodies (+++), positive SS-B antibodies (++) and positive Ro-52 antibodies (+++). An arterial blood gas on admission to ICU revealed a pH of 7.46, PaCO2 of 21 mmHg, PaO2 of 66 mmHg, lactate of 5.6 mmol/L, and HCO3- of 18 mmol/L with FiO2 of 40% on a high-flow nasal cannula, indicating respiratory failure and circulatory shock. An admission electrocardiogram showed sinus tachycardia with low voltage. Respiratory and blood samples were sent for culture, without positive results. Other laboratory investigations revealed normal thyroid function and urine analysis.\nComputed tomography of the chest showed an enlarged heart, increased pulmonary vascular diameter, ground-glass attenuation and interlobar fissure effusion, which suggested heart failure and pulmonary interstitial edema (Figure ). Additionally, consolidation was found in both lower lobes in the lung, indicating pneumonia (Figure ). Point-of-care ultrasound revealed an enlarged left ventricle (50 mm) (Figure ), global hypokinesia of the left ventricle and significant systolic impairment with a low left ventricular ejection fraction of 39.69% (Figure ), a distended inferior vena cava (22 mm) with loss of respiratory variation (Figure ) and diffuse B lines in both lung fields (Figure ). Based on these imaging findings, cardiogenic shock, pulmonary edema and pneumonia were confirmed.\nViral myocarditis, one of the most common causes of cardiogenic shock in young people, was considered in the primary differential diagnosis. However, the patient had no previous medical history of upper respiratory tract infection and further virological serum tests, such as influenza A and B, enterovirus, adenovirus and cytomegalovirus, were negative. Therefore, viral myocarditis was excluded as the cause in this case. SS-A antibodies and SS-B antibodies were positive; thus, primary Sjogren’s syndrome was considered. However, the patient did not have a dry mouth or dry eyes, and further Schirmer paper-strip tear tests were normal, with 12 mm/5 min and 13 mm/5 min for both eyes. Based on the 2016 American College of Rheumatology and European League Against Rheumatism classification criteria for primary Sjogren’s syndrome[], this was unlikely to be the cause in this case. |
A 37-year-old female Caucasian patient was referred to the periodontology department for evaluation of a gingival recession on the maxillary left second molar. The patient's medical history revealed no systemic diseases or allergies. The clinical () and radiographic () evaluation revealed 5 mm Miller Class II recession [] on the second molar buccal aspect. There was discomfort during tooth brushing, described by the patient, and permanent inflammation in this area. The probing depths were ≤2 mm, and apically to the recession, there was absence of keratinized gingiva. The proposed treatment was a connective tissue graft combined with a laterally displaced flap.\nPrior to the surgery, the patient underwent one session of scaling and polishing and received oral hygiene instructions in order to reduce the local inflammation. After explaining the objectives of the surgery, a written informed consent was obtained. The surgery started with the administration of local anesthesia and careful scaling of the exposed root surface, and a partial-thickness flap dissection was extended down into the vestibule, permitting passive coronal positioning of the flap. While preparing the flap, an oroantral communication was found apically to the first molar mesial buccal root with 3 mm diameter ().\nOroantral communication closure started by suturing the Schneider membrane with a 6 0' absorbable suture (monofilament polyglecaprone suture, Surgiclryl-Monofast ®SMI-Belgium) to close the perforation (). A connective tissue graft was harvested from the palate according to the technique described by []. The recipient bed was measured as well as the palate thickness. Using a #15 blade, oriented perpendicular to the palatal surface, a single incision was made 3 mm apical to the gingival margin of the maxillary teeth. The partial-thickness dissection was made as apically as needed to obtain a graft measuring 12 mm length and 7 mm width. The connective tissue was elevated from the palate, and the donor site was sutured with 4/0 silk sutures (Silk UPS braided, SMI-Belgium) applying pressure against the palate. The connective tissue graft was sutured to the recipient bed with 6 0' absorbable suture (monofilament polyglecaprone suture Surgiclryl-Monofast ®SMI-Belgium) (), and the flap laterally and coronally moved and sutured with 5 0' nylon suture (monofilament SERALON, SERAG Wiessner) ().\nA surgical stent was given to the patient to use during the following week (). The patient was instructed to the following: apply ice, in order to reduce the swelling; avoid suction and not to blow her nose; have a soft diet and not to brush the area. She was also recommended to refrain from physical exercise for a week. Chlorhexidine 0.12%, antibiotic, and anti-inflammatory drugs were prescribed (Figures and ). |
A 74-year-old female patient presented with a locally advanced tumor of the proximal pancreatic body. The patient showed no signs of obstructive jaundice and did not require biliary stenting. Initially, her tumor was noted to involve the major visceral vessels, including the celiac artery, portal vein, and splenic artery and vein (). The tumor was deemed unresectable and the patient underwent extensive chemotherapy with multiple cycles of gemcitabine, Abraxane, 5-fluorouracil, Alloxantin, Avastin, and Xeloda. After showing a favorable response, she was referred for surgical re-evaluation. The post neoadjuvant chemotherapy CT scan () showed a poorly defined infiltrative pancreatic neck and body mass measuring ∼25 × 15 mm with ill-defined soft tissue encasing the proximal splenic artery, common hepatic artery (CHA), distal celiac axis, and superior mesenteric artery (SMA). The mass also partially encased the portal vein and superior mesenteric vein (SMV). There was no radiologic evidence of hepatic metastasis and there was mild pancreatic duct dilatation. Pre-treatment and post-treatment serum CA 19–9 levels were 46 and 9, respectively. The patient did not receive any additional studies to assess GDA flow or the need for preoperative coiling/embolization. The absolute need for resection of the celiac axis was not determined until the time of the operation. The patient's functional status, perioperative risk, and likelihood of response were weighed and she was determined to be a candidate for an attempt at a modified Appleby procedure. After being properly informed of her various treatment options, she elected to undergo the operation.\nExploration of the duodenum and pancreas revealed a firm mass in the body of the pancreas with a soft pancreatic neck. No gross evidence of metastatic disease was present. There was a benign appearing lesion in the superior aspect of liver segment II, which was excised in its entirety and confirmed to be benign. Cholecystectomy was performed, followed by Kocherization of the duodenum and dissection of the pancreas and major vessels. We were fastidious in preserving the GDA. The splenic artery was controlled distal to the tumor, leaving a normal CHA pulse. The spleen and pancreatic body and tail were then serially elevated out of the retroperitoneum. The aorta was exposed, taking down the diaphragmatic crura. The celiac artery was identified at its origin, tied, divided, and oversewn with 5–0 polypropylene suture. Abnormal soft tissue was palpated along the proximal CHA. In light of this vessel involvement, we divided the distal CHA near the GDA. We then dissected the specimen off the SMA successfully and divided the pancreatic neck.\nThe inferior mesenteric vein was then clamped and tied. The inflamed pancreatic neck and proximal body were then dissected free from the right lateral aspect of the SMV and portal vein. The splenic vein was taken flush with the SMV, and its stump was oversewn, leaving good forward flow of the SMV to the portal vein. The proper hepatic artery (PHA) and the GDA were preserved throughout the resection. A Doppler ultrasound probe was used to test the GDA and PHA. Both arteries had adequate signal and in fact, the PHA had a faint palpable pulse. The liver parenchyma was also found to have a strong arterial signal. The specimen had two short stitches and purple dye placed at the neck margin and the rest of the specimen was inked per our Jefferson protocol.\nPathology of the resected specimen showed ductal adenocarcinoma with marked treatment effects with invasion of tumor into the peripancreatic soft tissue. The excision margins were free of neoplasia and the specimen had no regional lymph node metastasis (0/26). The tumor was within 2.0 cm from the pancreatic resection margin and within 0.1 cm of the circumferential margin in the posterior peripancreatic tissue. The maximum diameter of the tumor was 3.0 cm in size.\nIn the initial postoperative period, the patient had a transient transaminitis. A postoperative hepatic vascular ultrasound showed good hepatic arterial flow through the PHA and the transaminases normalized. The patient was discharged on postoperative day 9. She remains well without evidence of disease 7 months postoperatively, and has elected to receive no further chemotherapy. |
A 51-year-old male patient (R.R.), with good general health (ASA 1), nonsmoker, was referred to our department for rehabilitation of the first molar (). The tooth was periodontally compromised, with mobility of II degree and furcation interest. It was decided to extract it and in the second phase, after 3-month-healing period, make a single-tooth implant restoration with the bone volume augmentation.\nThe first attempt to extract this tooth was made by nonexpert oral surgeon and the palatal residual root was left inside ().\nAlso during a second session with a specialist, it was difficult and complicated to remove the entire root, which was fragile and fell apart.\nTo avoid a higher bone loss and oroantral communication (), it was decided to leave the last apical fragment in the alveolus. The primary purpose had not changed, and it was chosen to retrieve the residual root apex during sinus lift procedure, because antrostomy approach would help to remove it with a major visibility of the surgical field.\nAfter the healing period, a further accurate radiographic exam was necessary. As for any other sinus floor elevation case, reformatted computer tomography (CT) scans were required to examine insufficient bony support ().\nThis exam also revealed the palatal position of the residual root apex. Surgery was performed under local block anesthesia (posterior superior alveolar nerve, greater palatine nerve, and buccal infiltration from the canine to the first molar). An intrasulcular and crestal incision was performed and a full-thickness mucoperiostal flap was reflected to expose the maxillary and palatal wall (Figures and ).\nAn antrostomy with 5 mm diameter was made approximately from the distal root of first premolar to mesial root of second molar ().\nThe membrane was elevated from the bone and we could see the root fragment adjacent to the sinus floor. The residual was removed without difficulty with hemostatic clamps (Figures and ).\nAfterwards, implant was placed using a conventional approach: drills were used to prepare the fixture bed and the implant of 4,3 Ø in diameter and 11,5 mm of length (NobelReplace Select, NobelBiocare, Göteborg, Sweden) was installed protecting elevated sinus membrane. The insertion torque was of 35 N/cm, measured with a manual torque wrench by the operator. Particulate graft material (Geistlich Bio-Oss) was inserted between Schneiderian membrane and sinus floor and also between implant and vestibular tissue to fill the gap ().\nIn the lateral bony windows collagen membrane was installed to reinforce and keep stable graft material ().\nThe wound was closed primarily and detached sutures were positioned.\nPharmacological management was with antibiotic therapy (oral amoxicillin and clavulanic acid 875 mg + 125 mg every 8 hours) and nonsteroidal anti-inflammatory drugs (ibuprofen 600 mg, as needed by the patient).\nSutures were removed after fifteen days and no postoperative complications were present. No signs of infection, inflammation, or maxillary sinusitis were detected.\nAfter three-months, the second-stage operation was carried out to expose the fixture and cover screw was placed (). Radiograph examination showed () correct osteointegration, displaying good bone filling and the successful fractured apex retrieval (). |
A 69-year-old diabetic and hypertensive woman presented in July 2012 with a complaint of left arm numbness, weakness, and dysarthria. She had 2 previous left atrial myxomas resected and had presented both times with similar transient ischemic attacks. In June 1992, she underwent wide excision of a pedunculated 2.3 cm friable left atrial myxoma arising from the lateral left atrial wall above the right inferior pulmonary vein orifice, and autologous pericardium was used for reconstruction. In June 1997, she had a complete excision of a broad-based 2.0 cm friable left atrial myxoma arising between the left atrial appendage and the A1 section of the mitral valve. The resulting endothelial defect was closed primarily, and the margins were again negative. Her family history was negative for cardiac tumors. On physical exam she had a left facial droop, 4/5 motor power in her left arm, and no cardiac murmurs or visible skin lesions. A head MRI showed an acute right parietal stroke, and a CT angiogram () was consistent with two myxomatous fusiform aneurysms in the right middle (2.9 cm × 1.8 cm) and anterior cerebral arteries (10 mm × 5 mm). A transesophageal echocardiogram was obtained revealing a recurrent left atrial myxoma arising from the interatrial septum. Cardiac catheterization () showed normal coronary arteries and a tumor blush in the area of the left atrium. Preoperative chest CT scan demonstrated that the heart and aorta were not adjacent to the sternum thus allowing safe sternal reentry for the third time.\nA redo sternotomy was planned to resect the recurrent myxoma followed by cerebral angiography. Standard cardiopulmonary bypass and cardioplegic arrest were employed. A right atriotomy revealed an incidental smooth 1.5 cm flat right atrial mass that was excised. Frozen section confirmed the suspicion of a myxoma. A left atriotomy incision was next made, and the interatrial septum containing a gelatinous 2.0 × 1.5 cm friable mass, the lateral free left atrial wall extending to the right pulmonary veins, and the free right atrial wall were excised. Reconstruction was done using 2 porcine extracellular matrix (CorMatrix ECM, Roswell, Georgia) patches. The first patch replaced the interatrial septum and the anterior wall of the left atrium laterally to the orifices of the right pulmonary veins. The second triangular-shaped ECM patch closed the right atriotomy and extended from the medial aspect of the right atriotomy to the pulmonary vein-ECM suture line. The patient did well and was discharged on the 5th postoperative day. A review of all previous and current specimens showed that the 4 myxomas were completely excised and identical, corresponding to a polypoid benign myxoma extending from the endocardial surface.\nThe cerebral myxomatous aneurysms were approached by a multidisciplinary team consisting of neurologists, interventional radiologists, and neurosurgeons. A diagnostic cerebral angiogram was performed 5 months later, and the aneurysms were found to be fusiform, such that coil embolization could result in total occlusion of the affected vessels. The recommendation was to monitor with serial CT scans. The alternative, a bypass with coiling, was thought to be of high risk and would only be indicated if the aneurysms displayed growth on surveillance. Repeat head CT 3 months later showed the presence of a thrombus in the right MCA aneurysm without any significant increase in size, and the patient will be imaged again in 3 months. |
A 65-year-old man presented with a chief complaint of daily, persistent holocranial headaches and blurry vision. Upon review of systems, the patient manifested having a cranial mass located in the parieto-occipital region for the past 13 years (), which he believed had developed after head trauma he suffered in a motor vehicle accident.\nThe mass grew slowly and progressively over the years. It had never caused him any significant discomfort until approximately one year prior to presentation, when he started experiencing daily headaches which worsened with time and developed blurry vision. The patient's medical history also included essential hypertension, and he had no pertinent surgical, family or social history. On physical examination, an abnormally shaped head was noted due to a mass in the calvarium, as well as bilateral papilledema. There were no neurological deficits or changes in the skin overlying the cranial mass. The mass was not tender to palpation. Laboratory tests were within the normal reference range.\nComputed tomography (CT) of the head showed a large midline permeative calvarial lesion measuring 14 × 11.7 × 5.4 cm, with extensive periosteal reaction, subjacent dural thickening, extension into the subcutaneous soft tissues and intracranial extension into the bilateral parietal extra-axial space ().\nCT-Angiography revealed invasion and occlusion of a 9.5 cm segment of the superior sagittal sinus. There was no evidence of infarction. Digital subtraction angiography reported a highly hyper-neovascular destructive bone tumor with exuberant blood supply from the bilateral occipital, superficial temporal, and middle meningeal arteries and pial supply from the posterior internal frontal branches of the left anterior cerebral artery.\nMagnetic resonance imaging (MRI) post-gadolinium fat-saturated T1 sequence showed a large intraosseous mass with demonstration of intracranial extension, invasion into the superior sagittal sinus and regional dural thickening and enhancement (). The favored pre-operative clinical diagnosis was osteosarcoma.\nThe patient underwent super selective embolization of dural supply using n-butyl 2-cyanoacrylate (NBCA) and bilateral parietal circumferential craniectomy to excise the abnormal calvarium, followed by cranioplasty and reconstruction with titanium mesh.\nAn ovoid fragment of flat bone was received for pathologic examination. The outer surface of the specimen was nodular and had attached soft tissue, while the inner surface appeared flattened, irregular, and hemorrhagic. Sectioning revealed an ill-defined intraosseous mass expanding the bone. No areas of necrosis were grossly identified ().\nMicroscopic examination of H&E-stained sections revealed a predominantly intraosseous meningioma with overall features of meningothelial-type meningioma, WHO grade I, composed of cells with eosinophilic cytoplasm and regular, ovoid nuclei with fine chromatin and small nucleoli arranged in syncytia and whorls, with extension into soft tissue ().\nBrain invasion was absent. Few mitotic figures were identified reaching up to 2 mitoses per 10 HPF. Atypical features were focally present, including a minute focus of incipient necrosis and an area of small cell change, and some cells showed mildly enlarged nucleoli. However, the findings were considered insufficient for a diagnosis of atypical meningioma, WHO grade II. The Ki-67 proliferation index was estimated to be less than 5% in average; however, it was noted that it was higher than expected for a usual WHO grade I meningioma reaching up to 10% in the most proliferative areas. The gross and microscopic histopathologic features were those of an en plaque meningioma with extensive bone invasion, or possibly a primary intradiploic (intraosseous) meningioma. |
The second patient is a 72-year-old woman with a past medical history including hypertension, type II diabetes, and obesity, who presented with weight loss and steatorrhea. As her symptoms were suggestive of pancreatic exocrine insufficiency, and with the associated weight loss, she underwent a CT scan for additional evaluation. This demonstrated a suspicious 2.5-cm complex cystic pancreatic head mass with associated pancreatic gland atrophy and a dilated pancreatic duct. She was also noted to have fatty infiltration of the liver (although not overt cirrhosis) and ascites.\nShe was then referred for further evaluation. Magnetic resonance imaging (MRI) and magnetic resonance cholangiopancreatography demonstrated diffuse dilation of the pancreatic duct up to 7.5 mm with an associated cystic mass in the head of the pancreas, suggestive of main duct IPMN (). EUS was attempted, but was unable to be completed due to severe tortuosity of her esophagus. Positron emission tomography (PET) demonstrated heterogeneous metabolic activity within the pancreatic uncinate process and to a lesser degree within the body and tail. Additional workup included a paracentesis to evaluate the ascites, which was unremarkable and ultimately deemed secondary to poor nutritional status from her exocrine insufficiency. Laboratory results were within normal limits with the exception of tumor markers and a mildly low albumin (3.4 gm/dL). Carcinoembryonic antigen was elevated at 6.0 ng/mL, and CA 19-9 was elevated at 46.7 U/mL. She was placed on pancrelipase, which resolved her symptoms of exocrine insufficiency and helped improve her nutrition.\nDue to the concern for main duct IPMN, she was referred for surgical resection. Again, it was unclear if the diffuse dilation of the pancreatic duct was secondary to involvement by main duct IPMN or due to proximal pancreatic duct obstruction. The patient was planned for a Whipple procedure with possible total pancreatectomy if high-grade dysplasia was noted intraoperatively at the resection margin. Upon transection of the pancreatic neck, IPMN with focal high-grade dysplasia was indeed noted at the margin. Due to the abnormal preoperative PET scan, in addition to the high-grade dysplasia at the margin, a completion pancreatectomy was performed. Due to her poor nutritional status preoperatively, a gastrojejunal feeding tube was placed at the time of the operation. She had an unremarkable postoperative course and was discharged with feeding tube supplementation.\nHer final pathology demonstrated a 1.1 cm well-differentiated mucinous colloid carcinoma arising in a background of IPMN (intestinal type) with foci of high-grade dysplasia and intermediate grade dysplasia throughout the remaining pancreatic duct (). Staining of the tumor was positive for MUC1, MUC2, and MUC5. All margins were negative. Zero of 24 lymph nodes was involved with tumor. Her final pathologic staging was pT2N0Mx. She was referred to medical oncology for discussion of adjuvant therapy and will be initiating gemcitabine adjuvant chemotherapy in the near future. |
A 20-year-old female from Ethiopia presented with a giant skull-based tumor with a massive orbitocranial deformity distorting her facial anatomy and right eye.\nDiagnostic angiography, CT, and MRI revealed a giant, moderately hypervascular, multilobulated, and peripherally calcified expansile mass lesion which appeared to arise from the skull base and occupy the entire nasal cavity, maxillary sinus, and ethmoid sinus regions []. The intracranial extension of the tumor obliterated the sella turcica, bilaterally invaded the cavernous sinuses, and caused extensive elevation and splaying of the internal carotid arteries, anterior cerebral arteries, optic chiasm, and intracranial optic nerves. The mass measured approximately 10 cm × 8 cm × 11 cm. There was no evidence of edema or gliotic reaction in the adjacent brain parenchyma.\nThe patient underwent resection of the tumor through a subcranial transfacial approach. A bicoronal incision was carried down, preserving the galeal layer for possible use as a graft later. An incision was made over the face and nose to expose the front of the tumor. We broke through the thin bony shell and entered the multiloculated cystic tumor cavity. Fluid was drained and the operating microscope was brought into the field. This allowed us to work circumferentially to remove the entire solid tumor components all the way back to the circumferential bony shell. This left a massive cavity measuring over 15 cm in diameter. All soft tissues were removed and bone was drilled down. The lateral wall of the tumor was drilled, allowing for replacement of the globe to a more natural position. Bone was also drilled off the medial wall to decompress the lesion. The dura was never entered. Calvarial bone grafts were harvested by taking a large frontal parasagittal craniotomy.\nA craniofacial reconstructive surgeon with the help of an oculoplastic specialist completed a complex reconstruction. We used the bone grafts to reconstruct a new orbit and mid-face. Repair of the skull bone and scalp was undertaken in a second surgery by our craniofacial/plastic surgeon.\nHistopathological examination demonstrated an ossifying fibroma with a proliferation of plump spindle cells, mostly hypocellular, with focal hypercellular areas. Production of woven bone was seen intermittently with the spindle cells. The spindle cells showed focal areas of storiform arrangement with focal osteoblastic rimming. The final diagnosis was fibrous dysplasia.\nThe patient remained neurologically and physically stable throughout the follow-up period, with the exception of increasing headaches and occasional nosebleeds. Follow-up PET and MRI at 1 month and 10 months postoperative were stable and showed no evidence of tumor growth. The patient has been followed for 10 years with mild interval progression of bony changes but no clear evidence of tumor regrowth. During this time, she underwent one additional delayed cosmetic procedure for her left eye. |
The patient was an 80-year-old Japanese woman who had previously given birth four times by vaginal deliveries. She had been experiencing voiding difficulty and perineal discomfort for 5 years and had urinary stress incontinence for 2 years. Furthermore, she had not been previously followed up for POP. She visited her previous doctor because of the onset of general fatigue and urinary retention. Bilateral moderate hydronephroureters with dilated calyxes were identified by computed tomography. Therefore, a urinary catheter was inserted, and ∼1100 mL of urine was collected. Although no urogynecologists were available, she was admitted to the hospital to recover from her general condition. Subsequently, she went into a state of shock due to low blood pressure on the day of admission. Although she was administered antibiotics and dopamine hydrochloride for 2 days, she was transferred to our hospital for treatment because of worsening renal dysfunction and state of shock.\nA gynecological examination revealed total uterine prolapse (Fig.). In addition to hydronephrosis caused by chronic voiding difficulty and excessive residual urine, she was presumed to have pyelonephritis, which resulted in septic shock. She had high levels of activities of daily living and was generally healthy before suffering from this disease. According to laboratory tests, she had a high level of fibrin degradation products (23.4 μg/mL), high level of D-Dimer (9.8 μg/mL), and a low platelet count (4.9 × 104/μL), which also resulted in disseminated intravascular coagulation (DIC). Both blood and urine cultures were positive for Escherichia coli. Ring pessary and bilateral ureteral stents were temporarily inserted for urine drainage. Furthermore, ceftriaxone, nafamostat mesilate, and thrombomodulin were used for treating pyelonephritis and DIC. Serum creatinine, a measure of renal function, increased to a maximum of 6.09 mg/dL before ureteral stent insertion and then decreased to 1.10 mg/dL after 15 days. She recovered from DIC and septic shock 8 days after this multidisciplinary treatment.\nHer general condition completely improved 24 days later, and she was discharged from our hospital temporarily. We also suggested optional treatments (i.e., LeFort colpocleisis, vaginal hysterectomy with colporrhaphy, TVM procedure, and ring pessary insertion) to the patient, and she selected the TVM procedure without hysterectomy. She did not have any estrogen-related cancers or general diseases. Therefore, she was administered estrogen therapy for 3 months (i.e., 1 month before and 2 months after the operation) to prevent postoperative complications. She was readmitted to our hospital 3 months later, and an anterior and posterior TVM operation was performed. Surgery was performed under general anesthesia with the patient in a lithotomy position. We used the TVM surgical technique that has been previously described [], with a few modifications. A monofilament polypropylene mesh (25 × 25 cm; Gynemesh PS; Ethicon, Somerville NJ) was cut into a shape similar to the shape used in the Prolift system (Ethicon). The initial procedure for anterior TVM was an anterior colpotomy. The next step included posterior TVM to correct the rectocele and prolapsed uterus. The mesh was precut and adjusted according to the type of correction required. We exfoliated around the uterine cervix and combined the anterior and posterior mesh with another 2 × 7 cm rectangular mesh strip that was rolled around the uterine cervix using a 2-0 monofilament nylon suture on both sides. Correct positioning was confirmed by traction on the exteriorized sling arms. The operation time was 147 min, and the blood loss was 200 mL. The bilateral ureteral stents were removed 3 days after the operation. The patient had a favorable course, with relief from voiding difficulty and hydronephrosis and no recurrence during for 2 years following the operation. |
A 29-year-old male with a history of IV drug use, without any known comorbidities presented to the hospital with complaints of neck and back pain for three weeks. He denied any fever or chills, any radiation of pain, or any numbness or tingling. He was seen earlier in the emergency room during that week, given a shot of IV ketorolac for pain, and then discharged. The patient's physical exam was unremarkable for any focal neurological deficits. His illness did not subside, the pain worsened, and he subsequently presented later in the week with worsening neck and back pain. He also complained of an abscess on his right leg, located above the lateral malleolus with clean edges that he first noticed two to three weeks ago. At the time of presentation, he was alert and oriented, and vitals were within normal limits. The computed tomography (CT) scan revealed osteomyelitis at the cervical vertebra (C4-C5) level and prevertebral fluid collection. Neurosurgery was consulted, and magnetic resonance imaging (MRI) was recommended, which revealed C4-C5 osteomyelitis with an epidural abscess. The patient was started on IV vancomycin and admitted to the floor. During his hospital stay, neurosurgery performed an anterior corpectomy and posterior cervical instrumented fusion. The procedure was complicated by a cerebrospinal fluid (CSF) leak, and intraoperative cultures grew Serratia marcescens, as shown in Figure . The patient’s hospital course was complicated by the CSF leak and drainage from the surgical wound, for which he had to be taken back to the operating room (OR) for an anterior washout. A lumbar drain was placed that day for drainage. Infectious disease recommended adding aztreonam 2 g IV every eight hours for six weeks. Vancomycin was discontinued after the cultures did not grow methicillin-resistant Staphylococcus aureus (MRSA). Blood cultures were also negative during his hospital stay. The patient completed six weeks of treatment with IV aztreonam and was discharged home after completion of the antibiotics course. He was followed up in the clinic where his condition has significantly improved and he did not have any active complaints during that visit. |
A 44-year-old male, manual labourer by occupation, presented to our outpatient department with complaints of pain and swelling over the left wrist for the past two years. The swelling was initially small to begin with but gradually grew to the present size. Pain was initially intermittent and was present during strenuous activities, but now there was constant dull aching pain even at rest. There was no history of trauma or constitutional symptoms like fever, loss of weight, or loss of appetite or no associated swellings elsewhere in the body. Examination revealed a firm to hard oval swelling over the distal ulna measuring 5 cm by 4 cm (Figures and ). Skin over the swelling was normal. Tenderness was present on deep palpation. Terminal restriction of flexion and extension of wrist was noted. Routine serum biochemical studies were within normal limits. Plain radiography of the wrist in anteroposterior and lateral views showed a large expansile multiloculated lesion in the distal ulna with cortical thinning and no periosteal reaction (). No evidence of calcification was noted. CT scan of the wrist showed expansile lytic lesion with cortical thinning and few areas of cortical destruction (). MRI of wrist revealed 6.5 × 5.6 × 5 cm lesion isointense in T1 weighted and hyperintense in T2 weighted image in the distal ulna. The lesion showed enhancement on contrast MRI. Cortical break was noted (). Plain radiograph of the chest was normal. Fine needle aspiration cytology of the lesion showed a double cell population with stromal cells and multinucleated giant cells suggestive of giant cell tumour. Clinicoradiologically a provisional diagnosis of giant cell tumour of distal ulna Enneking stage III was made.\nAs per the staging system, we planned for wide resection of ulna. Anticipating the loss of long segment of ulna, ulnar reconstruction was planned. Reviewing the literature, extensor carpi ulnaris tenodesis of the stump was found to produce good outcome with limitations in pronation-supination movements. Some authors have tried ulnar buttress arthroplasty using iliac crest graft with limitations in movements. In order to overcome these limitations, we planned for reconstruction using proximal fibula and reconstruction of triangular fibrocartilage complex using palmaris longus tendon.\nPatient was taken up for surgery under combined supraclavicular block and spinal anaesthesia. Through a dorsal approach over the radial border of ulna, wide resection of distal ulna was performed (Figures , , and ). The resected ulna measured 8 cm (). Around 10 cm of proximal fibula was harvested in routine fashion. The harvested graft was trimmed to fit the distal ulna (). Care was taken to position the cartilage surface of fibula facing the radius while the raw surface facing medially as otherwise fusion of the newly constructed DRUJ could occur. The fibular graft was stabilised on the ulnar stump with a 6 holed 3.5 mm narrow dynamic compression plate with 5 screws. To stabilise the distal radioulnar joint, palmaris longus tendon free graft was harvested through two separate stab incisions, one at the level of wrist and the other in the proximal forearm on the volar surface. A drill hole was made across the joint. Palmaris longus tendon was passed through the hole and sutured back on to it (). To protect the palmaris longus tenodesis, two K wires were drilled additionally across the DRUJ. To augment the tenodesis, a slip of ECU was sutured to the palmaris longus tenodesis (Figures and ). Wounds were closed in routine fashion and above elbow POP slab was applied with forearm in supination. Sutures were removed on the 12th postoperative day. Histopathological examination of the resected specimen was consistent with giant cell tumour.\nStrict immobilisation was continued for 6 weeks. At the end of the 6 weeks, the K wires were removed and a full range of movements were initiated. Patient was followed up monthly for the first 6 months. Radiographic and clinical evaluation at the end of 1 year showed good union with no subluxation of the newly created DRUJ and ulna (). A near normal range of movements of the wrist including pronation and supination were possible and painless with a good hand grip (Figures and ). Patient was able to do routine activities since then. Patient had returned to normal work with no evidence of recurrence either clinically or radiologically. |
A 70-year-old woman was referred to our hospital because of complaints of pain in the MCP joint of her right middle finger over the course of the preceding year. She did not have a history of trauma, infection, or rheumatologic conditions by blood examination in which rheumatoid factor and anti-citrullinated cyclic peptide/protein antibody were 4 IU/mL and < 4.5 U/m, respectively, except for release of the tendon sheath of her middle finger due to stenosing tenosynovitis 30 years prior. Radiography of the right hand during the initial visit indicated osteoarthritis of the MCP joint of the middle finger with a slight narrowing of the joint-space and the presence of small osteophytes in both the basal phalanx and metacarpal bones; however, pathological findings were also remarkable in the index finger DIP joint (). Due to a mild complaint (DASH score, 8), the patient received anti-inflammatory drug, resulting in pain relief. Unfortunately, pain and discomfort of daily activities were worsened, with the patient receiving a 73/100 mm on the visual analogue scale (VAS) for pain and a DASH score of 49.1 at 3 years after her first visit. Upon physical examination, we observed swelling in the dorso-ulnar side of MCP joint, and a range of motion (ROM) at the MCP joint that was limited to 20–85 degrees and was inextensible. Furthermore, radiography indicated a bone tip from the ulnar side to the middle finger MCP joint and a growing osteophyte on the ulnar metacarpal head (). Radiography at 3 years after her first visit showed the presence of a small bone tip on the ulnar side of the metacarpal head (). The findings upon computed tomography (CT) and magnetic resonance imaging (MRI) also suggested that the bone tip was an intra-articular loose body, which produced the inextensible condition of her middle finger. Since the size of the loose body was large for the finger joint, we planned surgical resection under the arthroscopy. Surgery was performed under brachial plexus block and a pneumatic tourniquet was applied to the upper arm to control bleeding. The patient was placed in a supine position with her shoulder abducted and her elbow flexed to 90 degrees. A single Chinese finger trap was attached to the affected finger to apply 5 lb of longitudinal traction using a traction tower. Under the vertical traction, the MCP joint could be easily palpated and injected. Two portals were marked at radial and ulnar side to the extensor tendon. The distance between each portal and tendon was about 8 mm. A short blunt trocar and cannula system were inserted into the joint under steady gentle pressure after distension of the joint with saline and the introduction of an arthroscope with a 1.9-mm diameter (ConMed Linvatec, Largo, FL, USA) through the radial portal of the third MCP joint. Visualization was achieved and synovectomy was performed using a 2.0-mm full-radius motorized shave through the ulnar portal on the MCP joint. This maneuver allowed us to visualize and remove the loose body within the joint (), although the origin was not apparent. Radiography after surgery indicated the disappearance of the loose body (). Postoperatively, motion was permitted without immobilization the next day after surgery, while limitations in the motion of the MCP joint soon disappeared. The motion of the MCP joint of her middle finger was able to be actively and passively hyperextended to 15 and 40 degrees, respectively, without pain at 3 years after the surgery. |
The patient was a 74-year-old man who had previously been diagnosed with MDS. He was diagnosed with calculous cholecystolithiasis at our hospital. Laparoscopic cholecystectomy was planned, but we converted to open cholecystectomy with a right subcostal oblique incision because it was difficult to remove the impacted calculus in the cystic duct. The surgical wound was partially erythematous 4 days after surgery (Fig. ), so we opened the wound and cleanse with sterile saline and performed moist environment dressing because of surgical site infection suspected. Inflammation, however, spread to the surrounding skin with erosion (Fig. ). The condition of wound rapidly deteriorated, so we introduced intra-wound continuous negative pressure and irrigation treatment (IW-CONPIT) 10 days after surgery (Fig. ). In spite of IW-CONPIT, inflammation continued to further spread widely with a purulent coating (Fig. ). In addition, skin edema spread from the lower back to the lower extremities. Antibiotic administration and irrigation were not effective, either. Bacteriological examination revealed that Enterococcus faecalis and Pseudomonas aeruginosa were detected in the wound, although those counts were low. We decided to perform biopsy of the skin surrounding the wound to analyze pathological condition. Histopathologic feature showed that severe inflammatory cell, predominantly of neutrophils, infiltrated in the dermis, and no bacterial components were observed (Fig. , ). These clinical and histopathologic findings of the surgical site indicated that diagnosis of PG associated with MDS was most likely. After the addition of a systemic administration of 30 mg/day prednisolone to local care of the surgical site with moist dressing, fever had been rapidly alleviated, spread of rash had diminished, edema in the lower body had improved, and the purulent coating had disappeared on the eroded skin surface surrounding the open wound (Fig. ). Elevated CRP (18.1 mg/dL) and WBC counts (40.5 × 103 μL) before administration of prednisolone had been improved remarkably, and these data became within normal ranges after 2 weeks, so the dose of prednisolone was tapered to 25 mg/day. The dose had been reduced 5 mg/day per 1–2 months to follow up the skin and general condition, including laboratory data. Erosion had healed and epithelialization occurred. The surface of the wound was covered with satisfactory granulation tissue, and normal wound healing was achieved after administration of 5 mg/day prednisolone for 1 year (Fig. ).\nPG is an uncommon, chronic, recurrent, and painful cutaneous ulcerative disease with a distinctive morphologic presentation and uncertain etiology. The lower extremities are most common sites of involvement, though rare subtype of cases appears stoma site on ulcerative colitis increased recently []. More than half of patients with PG suffer from an associated systemic disease such as IBD, RA, and MDS [, ]. In this case, there was a history of MDS: it was to help diagnosis of PG.\nDevelopment of PG in a surgical and trauma site is rare and occurs mainly within 2 weeks after surgery []. The initial symptoms are surgical site erythema and extreme pain out of proportion to the physical examination [–]. PG is often initially diagnosed as a surgical site infection, though treatment with antibiotics and wound debridement fails to arrest rapid ulcer enlargement. Our case was also regarded as infection to treat with antibiotics, open drainage, and continuous wound lavage, but the condition had been getting worse. We performed excisional biopsy to clarify pathogenesis of the cutaneous lesion. Histopathologic findings revealed non-specific and neutrophil-dominant inflammation, but bacterial infection could be denied. The clinical and histopathological manifestations indicated that diagnosis of PG associated with MDS was most likely. Histological examination should be performed to exclude other disorders such as vasculitis, pyoderma, and vasculopathies, when the administration of antibiotic, debridement, and irrigation for inflammatory surgical site is ineffective and makes clinical exacerbation. A diagnosis of PG could be made when other diagnostic possibilities had been excluded, because no accepted diagnostic criteria existed. Su WP et al. proposed diagnostic criteria, including clinical and histopathologic findings and treatment response []. Post-surgical PG occurred after breast (25 %), cardiothoracic (14 %), abdominal (14 %), and obstetric (13 %) surgeries [], could deny infection and other possibilities, suspected PG, and performed experimental administration of steroid, following respond dramatically. PG has a good prognosis if appropriate therapy is immediately selected for it, but unless it is done, its prognosis becomes worse rapidly; it is likely to progress to death with consequent sepsis [].\nDefinitive guidelines for treatment of PG are lacking. Patients with PG are treated with local and/or systemic therapies. First, to make an optimal environment for wound healing, a moist wound environment after cleansing with sterile saline is required []. Local administration of corticosteroids could be used in patients with mild PG, though the efficacy of these drugs is limited to a few retrospective studies and case reports [, ]. In contrast, systemic therapy is necessary in patients with more severe PG. Glucocorticoids are mostly selected for systemic drugs [, ]. Zuo et al. reported that most patients were treated with oral prednisolone (0.5–1.5 mg/kg/day) or intravenous methylprednisolone (0.5–1 mg/kg/day) combined with/without immunosuppressants such as systemic or topical tacrolimus []. We administered oral prednisolone 30 mg/day (0.5 mg/kg/day) as initial treatment and by means of diagnostic administration. If this administration is not effective, the dose of oral prednisolone could increase or administrations of intravenous methylprednisolone and tacrolimus ointment could be selected. Topical tacrolimus is one of the effective local treatments for PG []. Cyclosporine exhibited equivalent effect compared with steroids as a systemic treatment in a randomized trial []. A wide variety of other systemic immunomodulatory drugs, including anti-tumor necrosis factor alpha agents could be also utilized as alternative or adjunctive treatments in patients with PG that fails to respond to glucocorticoids [, ]. |
A 35-year-old female with initial weight of 103.4 kg and body mass index of 35.8 kg/m2 was considered for bariatric surgery due to significant impairment of quality of life and failure of intensive lifestyle modification to maintain weight loss for 1 year. Her past medical history was notable for hypothyroidism, which was controlled with 0.5 mg of Levothyroxine daily. She did not have diabetes, hypertension, or any other obesity-related co-morbidities except fatty liver grade 1 with an increased liver span of 18.3 cm found on abdominal ultrasonography. Nutritional evaluation, including micronutrient measurements, was unremarkable.\nHer menstrual cycles had been regular and she had no history of infertility. Her obstetric history was significant for one full-term pregnancy ended by an uncomplicated elective cesarean section 7 years ago and one first-trimester spontaneous abortion 6 months ago. She was also found to have a negative serum β-hCG level 1 week before surgery.\nShe underwent successful and uncomplicated laparoscopic sleeve gastrectomy, creating a gastric tube over a 36-F bougie with the exclusion of 80% of the stomach, and was discharged on postoperative day two. She missed her first menses on the third week following surgery and was found to have positive serum β-hCG levels at that time. She discontinued oral hormonal contraceptives 3 months before surgery for personal reasons and her last menstrual period was precisely 11 days before surgery. Retrospectively, she acknowledged a history of unprotected sexual intercourse 3 days before surgery for which she took a dose of 1 mg of Levonorgestrel + Ethinyl Estradiol 100 mcg (2 pills of Contraceptive HD/Ovocept-HD) within 1 h after intercourse and then repeated it after 12 h. Two months later an intrauterine gestation of approximately 11 weeks (CRL = 41 mm) was identified by an obstetric ultrasound. Although adequate counseling was provided for the couple regarding the potential adverse effects of surgery and rapid weight loss phase on pregnancy outcome, they decided to continue with the pregnancy.\nShe was monitored throughout her pregnancy for proper weight gain, nutritional supplementation and surveillance, and for fetal wellbeing by a multidisciplinary team including high-risk obstetrics, bariatric surgeons, and nutritionists. She was also prescribed prenatal vitamins Naturemade® tablets daily (containing 27 mg ferrous fumarate, 11 mg zinc, 800 mcg folic acid, 5.2 mcg vitamin B12, vitamins A, B group, C, D, E, K, and biotin) and one Calcicare tablet daily (200 IU vitamin D, 400 mg calcium, 100 mg magnesium, and 4 mg zinc) up to the end of pregnancy. Her pregnancy continued uneventfully, and she underwent an elective cesarean section at 39-weeks to deliver a healthy full-term girl weighing 2900 g (between 10th and 25th percentile), and 51 cm in length with a head circumference of 33 cm. The infant reached all expected milestones during the first 6 months of her life, and the mother achieved 94.4% excess body weight loss. Her BMI at 12 months post-surgery were 25.6 kg/m2 (Fig. ). |
A 42-year old male farmer from Wayanad, admitted to the Institute of chest disease, with complaints of high grade fever, cough and chest pain of three months duration. Cough was associated with expectoration of scanty white sputum. Chest pain was initially pleuritic but later became dull, aching and diffuse in nature. There was exertional dyspnoea which progressed to grade III during this period. He also had bilateral pitting pedal edema. There was no history of hemoptysis, hoarseness, headache, vomiting, abdominal pain or OSA symptom. He was a known diabetic patient since 20 years and, on admission, had symptoms of multiple target organ damage like diabetic neuropathy, retinopathy and nephropathy. There was no history of CAD or ATT in the past. He was recently detected to have hypertension. He was a smoker with a smoking score of 900. He was treated as a case of pneumonia from a local hospital and referred as there was no improvement.\nOn examination he had pallor, bilateral pedal edema and a blood pressure of 180/100 mmHg. There were generalized hyperkeratotic skin lesions with central necrosis over both lower limbs and back of chest. Some of them were simulating diabetic kyrle and some healed pyoderma. Respiratory system examination revealed a diagnosis of non-resolving pneumonia right upper lobe. He had minimal ascites.\nInvestigation revealed hemoglobin 7.7 g %, ESR 138 mm/first hour and mantoux test was non-reactive. His diabetic status and renal function were fluctuating throughout the hospital stay. Retroviral screening and sputum examination for AFB were negative. LFT were within normal limits. Chest X ray [] showed a cavity in right upper zone with irregular inner wall, air fluid level and membranous projections from the wall. A lateral decubitus film demonstrated shifting of the fluid level with irregular cavity wall, and layers of air pockets in the cavity wall. As the X ray appearance was not typical of a lung abscess, possibilities like fungal infection, mycobacterial infection or hydatid cyst were considered in the differential diagnosis. CT thorax revealed fluid and solid attenuation areas mimicking a fungal ball as well as double layered cyst wall as in hydatid cyst []. USG abdomen showed changes of diabetic nephropathy, minimal ascites and mild hepatomegaly. There was no specific lesion in the liver to support a diagnosis of hydatid cyst. Echinococcal Ig G antibody was also negative.\nBronchoscopy revealed mucosal edema and narrowing of right upper lobe apical bronchus from which creamy white secretions seen spurting with each cough. BAL was taken and sent for investigations. During this period, he was treated with broad spectrum antibiotics. As there was no improvement a trans-thoracic needle aspiration was done which yielded 10 ml of yellowish white fluid which was sent for investigation. The BAL as well as aspirated fluid specimen was mounted in lactophenol cotton blue which showed fungal growth which when incubated in Brain Heart infusion broth at room temperature showed the growth of a rare fungus, Basidiobolus []. Since potassium iodide was the drug of choice for this fungus, he was started on oral potassium iodide with 1 drop thrice daily and gradually increased to 10 drops thrice daily. He was also given cotrimoxazole as some cases reported an excellent response to this agent.[] However his diabetic status worsened with the development of acute renal failure and hyperkalmia. KI was stopped temporally for control of renal failure and diabetes. The dose of insulin and parenteral fluids were carefully titrated. In order to achieve radical cure surgery was necessary as the patient's condition did not permit the use of the specific agent-KI. Hence patient was taken to cardio-thoracic surgery for resection after control of diabetes and renal function. Right upper lobectomy was done. The resected specimen showed a white ball of entangled mycelia []. Histopathological section showed filamentous aseptate fungi [] and the report came as zygomycetes group of fungi and fungal culture confirmed the diagnosis of Basidiobolus ranarum []. Spendre–Hoeppli phenomenon was absent in this case and peripheral eosinophilia also is not demonstrated. Post-operatively, he was started on itraconazole 200 mg twice daily. A repeat CXR [] six days after surgery showed near normal lung except for the presence of some amount of pleural thickening on the right side. There was good clinical improvement and he was discharged with itraconazole and other supportive measures. |
62-year-old woman with medical history significant for aortic stenosis and chronic atrial fibrillation presented to the emergency department with fatigue and progressively worsening shortness of breath with minimal exertion. She was asymptomatic at rest and denied chest pain, orthopnea, paroxysmal nocturnal dyspnea, leg swelling, presyncope, or syncope. She was incidentally found to have a systolic murmur during her pregnancy 20 years prior to this presentation. Notably, she had been offered aortic valve replacement in the past but declined.\nVital signs were normal with blood pressure of 110/60 mmHg, pulse rate of 79/min, temperature of 97.2°F, and respiratory rate of 16 breaths/min with normal oxygen saturation of 100% on ambient air. Physical examination was significant for irregularly irregular heart rhythm, and ejection systolic murmur was loudest in the aortic area with radiation to the carotids. Her lungs were clear to auscultation, and no pedal edema was noted. Electrocardiogram revealed atrial fibrillation with voltage criteria for left ventricular hypertrophy. Her most recent transthoracic echocardiogram revealed a thickened calcified aortic valve with decreased excursion with 4.6 m/s velocity suggesting a peak of 86 mmHg and mean of 36 mmHg suggestive of severe aortic stenosis. No other significant valvular abnormalities noted. Cardiac catheterization revealed widely patent coronary arteries.\nBased on the presence of worsening symptoms and the risk of sudden cardiac death, the decision was made to proceed with surgical aortic valve replacement. Given her history of chronic atrial fibrillation, she was also planned for left atrial appendage exclusion and Cox Maze IV procedure simultaneously with the aortic valve replacement. Intraoperative transesophageal echocardiogram revealed quadricuspid aortic valves confirmed during surgical exploration (). The native stenotic quadricuspid aortic valve leaflets were excised, and a 21 mm Saint Jude Medical Trifecta valve was implanted. The left atrial appendage was excised, and Cox Maze IV procedure was performed. Postoperatively, she remained in junctional rhythm and underwent uneventful placement of the dual chamber pacemaker on postoperative day 3. She recovered without further complications and was discharged on the eight postoperative day. |
A 91-year-old Japanese woman presented to our hospital with hypoxia of unknown origin. She had a medical history of hypertension and atrial fibrillation. Eight years before, when she was 82, an advanced atrioventricular block was diagnosed after a syncopal episode and she received a permanent dual chamber pacemaker implantation. She underwent coronary computed tomography (CT) and radioisotope examination at this time, but there was no evidence of cardiomyopathy or of ischemic heart disease. The tip of the pacemaker lead was observed to pass through a PFO at the left ventricular apex; she received a follow-up examination because of no serious adverse event. She had no history of lung disease or tobacco smoking. She had no subjective symptoms associated with cardiovascular disease after pacemaker implantation. There was no evidence of a decline in oxygen saturation levels at that time, which were measured by pulse oximetry.\nHer physical examination findings on admission were as follows: clear level of consciousness and severe hypoxia and cyanosis observed only in the right lateral decubitus position. These symptoms were immediately improved in the left lateral decubitus, the supine, and the upright position. Arterial blood gas values were consistent with her symptoms. Partial arterial oxygen pressure (PaO2) and arterial oxygen saturation (SaO2) decreased only in the right lateral decubitus position (Table ). These observations were reproducible. There was no evidence of lung rales or cardiac murmur.\nLaboratory tests, including complete blood count, thyroid function tests, and D-dimers were almost normal. A chest X-ray showed a significant elevation of her right diaphragmatic dome.\nAn electrocardiogram (ECG) showed an atrium-sensing, ventricular-pacing rhythm with a complete right bundle branch block (RBBB) pattern (Fig. ). In general, when the pacemaker lead is set on the right ventricle correctly, the pacemaker first stimulates the right ventricle, and next the left ventricle. Because of this, the expected ECG pattern with right ventricle pacing should show a left bundle branch block (LBBB) pattern. A RBBB pattern suggests that the pacemaker first stimulates the left ventricle, and next the right one. Only this ECG pattern could indicate the possibility of inadvertent left ventricle pacing. This patient’s ECG pattern also suggested left ventricle pacing. However, we could not assess a malposition of the pacemaker lead from only an ECG pattern because sometimes we find a RBBB pattern although the pacemaker lead is set correctly. Contrast-enhanced CT excluded the diagnosis of cerebral infarction, pulmonary embolism, pneumonia, and lung parenchymal abnormalities and revealed giant liver cysts and compression of the right atrium by one of cysts (Fig. ). A chest X-ray and CT scan revealed right hemidiaphragmatic elevation that was probably compressing the right atrium. This finding was suspected in the case of right hemidiaphragmatic paralysis []. A transthoracic echocardiogram (TTE) revealed concentric left ventricular hypertrophy, left atrial dilatation, and compression of the right atrium due to the giant liver cyst. In addition, the pacemaker lead probably ran abnormally to the left atrium and ventricle through PFO, but shunt flow across the PFO on color Doppler image was not apparently observed. Pulmonary perfusion imaging with 99mTc-macroaggregated albumin (MAA) was performed in the right lateral decubitus position. MAA accumulated in her kidneys and brain, which suggested the presence of a right-to-left shunt. The estimated shunt ratio was 26.3%.\nA transesophageal echocardiogram (TEE) with intravenously administered agitated saline contrast solution was performed in both right and left lateral decubitus (Fig. ). TEE showed a compressed right atrium and shunt blood flow in both directions: from the left to the right atrium and vice versa. The shunt flow was exacerbated by postural changes from the left to the right lateral decubitus (Fig. ). TEE also confirmed that the giant liver cyst was compressing the right atrium and the pacemaker lead was abnormally running to the left atrium through PFO (Fig. ).\nConsidering these results, we concluded that the cause of hypoxia was POS with right-to-left interatrial shunt through PFO. POS can be treated with the closure of the interatrial shunt by thoracotomy or percutaneous cardiac catheterization []. Surgical closure of PFO was not performed due to the age of our patient, surgical difficulties, and failure to obtain informed consent. For these reasons she was discharged after receiving medical advice about her posture. |
A male infant, who was term appropriate for gestational age, was born via repeat caesarean section to a healthy 26-year-old woman. No family history of bleeding disorders was reported by the mother. Pregnancy was unremarkable, and Apgar scores were 9 and 9 at 1 and 5 minutes, respectively. On initial physical examination, a localized hematoma to the left parietotemporal region was identified. A routine complete blood count (CBC) displayed normal counts. It was decided to repeat the CBC in 12 hours and, in the interim, to observe for dissemination of the blood collection. Prior to the next blood test, the physician was called to the bedside because the infant now had a change in his clinical examination: pale and tachycardic. His examination now revealed a large palpable fluid wave that extended behind the neck and left ear. Given the infant’s change in clinical status and concern for a subgaleal hematoma, the infant was transferred to the neonatal intensive care unit (NICU). The repeat CBC showed a hemoglobin level of 7.6 g/dL, with a hematocrit of 22% and platelets of 169 × 109/L. In the NICU, the patient received a transfusion of 20 cc/kg of O Rh-negative packed red cells. His coagulation profile disclosed a normal prothrombin time but an activated partial thromboplastin time (PTT) of 101 seconds (normal = 25-30 seconds).\nSubsequently, a FVIII level was ordered, which was very low at <0.01 U/mL. He was diagnosed with severe HA and was initially treated with 50 U/kg of recombinant FVIII. His repeat doses were adjusted to bring his FVIII level to 100%. A computed tomography (CT) scan of the head revealed a large subgaleal hematoma, beginning in the left parietal area, extending to the occiput and down the nape of the neck. The infant was discharged at 1 week of age following a repeat head imaging confirming complete resolution of the extracranial hemorrhage (ECH) without any evidence of intracranial bleeding. |
A 67-year-old female, with a typical presentation of carpal tunnel syndrome in the right hand, presented initially with numbness in digit IV and V a year later. A few months later, she developed numbness in all five fingers on the left side. Furthermore, the patient mentioned that she had had a burning sensation in both feet for a few years. Another few months later she complained of weakness in all four limbs and was sent for a neurological consultation with electromyography. Clinical examination revealed a Hoffmann-Trömner reflex on the left side, mild loss of strength in both hands, loss of vibratory sense and hypoesthesia in the distal end of all four limbs and loss of proprioception in both legs. The electromyography showed disturbed sensorimotor signals in the left hand and mildly disturbed sensorimotor signals in the right hand. The disturbed electromyography was attributed to carpal tunnel syndrome, which was presumably less severe on the right side due to treatment with long acting corticosteroid injections. The paresthesia in digit IV, digit V and both feet, however, could not be explained with the diagnosis of carpal tunnel syndrome. Therefore, magnetic resonance imaging (MRI) of the cervical spine was performed. Imaging showed an extra-dural soft tissue mass posterior to the odontoid process of the axis. The mass extruded through the transverse ligament of the atlas with severe compression of the myelum and myelomalacia at the level of C1 (Figures , and ).\nA plain radiograph of the cervical spine was performed to check for atlanto-axial instability. The radiograph during flexion shows a slightly widened atlantodental interval, measuring 4 mm (normal value: <3 mm) (Figure and ).\nPre-operative imaging of the cervical spine also revealed an anatomical variant of the craniocervical junction, atlanto-occipital assimilation of the massa lateralis bilaterally (Figure ).\nThe patient was treated with laminectomy of C1 to decompress the spinal canal followed by posterior fixation of C1-C2 for stability. |
We report the case of a 28-year-old man who presented with recurring episodes of unexplained fever (40°C). He complained of continuous pain in the right upper abdominal quadrant, not related to eating or drinking. He denied chest pain or cough and did not have vomiting or nausea. His intestinal transit was regular, and as were his stools and urine colour. He did not have any urinary symptoms but had noticed painful testicular oedema which resolved after he applied ice to it. Physical examination showed mild tenderness in the right upper quadrant without jaundice.\nThe patient was originally from Tunisia and had just returned from a recent visit there, where he had experienced an episode of fever and had been treated with levofloxacin for prostatitis. He was an active smoker, was not taking any other medication and was in good health before his trip.\nHe had been treated a year earlier in another city for pleural and pericardial effusions, thought to be of viral origin. Blood tests performed at the time showed a high C-reactive protein (CRP, 258 mg/l) with normal white blood cells at 7,110/mm3 with a slight neutrophil increase of 6,260/mm3 and lymphocytes down to 580/mm3. Haemoglobin was 12 g/dl and there was mild thrombocytopenia: platelets were 92,000/mm3. Renal function and hepatic tests were within the normal range. Testing for CMV, EBV, hepatitis A, B and C, and HIV came back negative. Thoraco-abdominal computed tomography (CT) revealed small bilateral pleural as well as pericardial effusions up to 12 mm in size. Hepatomegaly and splenomegaly were seen on imaging along with ascites. His echocardiography results were normal except for mild pulmonary hypertension. Scrotum ultrasound showed bilateral hydrocele without signs of epididymitis. Blood cultures and pleural effusion culture came back negative. His CRP decreased under colchicine, without any antibiotic treatment, as did all effusions. He had stopped taking colchicine during his journey in Tunisia because he ran out of medicine.\nGiven his country of origin and the recurring fever and symptoms without any cardiac or infectious causes found, familial Mediterranean fever (FMF) was suspected and the patient was put back on colchicine which ultimately resolved all his symptoms.\nThe patient presented 2 months later to our emergency department with another episode of fever, headaches and testicular oedema. He admitted stopping his colchicine treatment again.\nAs all symptoms including fever returned each time the patient stopped his colchicine treatment, and as he mentioned his sister had a similar medical history with remission during pregnancy, we decided to pursue genetic testing to confirm the suspicion of FMF. Unfortunately, our patient’s sibling refused to be tested for the mutations. However, genetic testing of our patient did reveal two variants of the MEFV gene: p.Pro369Ser and p.Glu148Gln. These two variants are rarely seen in the same person, and patients with these genetic variants presenting with severe FMF symptoms are seldom described in the literature. |
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)).\nThe 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. |
This is a case report of a 46-year-old female with a past medical history notable for depression, asthma, and uterine leiomyomas who presented to an urgent care with 5 days of progressive abdominal pain, bloating, nausea, and subjective fevers. The patient endorsed a several month history of gaining weight, though she attributed it to her lifestyle, accompanied with strong, intermittent, crampy right lower quadrant pain. The pain was random in onset and would dissipate very quickly. However, 5 days prior to presentation, her pain dramatically increased and was persistent in nature.\nHer surgical history included a myomectomy performed 5 years prior followed by a laparoscopic hysterectomy 2 years later. She was a regular drinker, consuming four to five alcoholic drinks per night, but stopped when her symptoms worsened and had no history of withdrawal. Family history was significant for a maternal grandmother with breast cancer, mother with skin cancer, and an uncle with colon cancer.\nUpon arrival to the emergency room, the patient was tachycardic, mildly hypotensive, and febrile to 103 °F. Her abdomen was soft, distended, and diffusely tender without peritonitis. Labs were notable for a leukocytosis of 15 K. CT of the abdomen and pelvis demonstrated moderate volume, complex fluid within the abdomen and pelvis with extensive amount of gas and peripheral rim enhancement, a thickened appendix filled with fluid, and a soft tissue/cystic lesion in the anterior abdominal wall (Fig. ).\nDifferential included pseudomyxoma peritonei with a ruptured appendiceal mucocele versus PMP secondary to an adnexal ovarian neoplastic pathology with an infectious component. Per the radiology report, the origin was unclear based upon imaging and stated a ruptured adnexal cyst should be considered given the markedly enlarged septated cystic lesions in the pelvis. The patient was resuscitated, and her blood pressure and heart rate normalized with 2 L of fluid. Given the patient’s hemodynamic stability, the decision was made to attempt conservative management with antibiotics and interventional radiology (IR) biopsy and drainage.\nOn the floor, the patient remained hemodynamically stable with intermittent fevers. Her abdominal exam also remained unchanged, with persistent pain that was under control with pain medication. It was unclear if the abdominal wall mass was neoplastic, and given our initial nonoperative approach, a biopsy was performed for tissue diagnosis to properly guide further treatment. A core needle biopsy of the abdominal wall mass demonstrated a uterine leiomyoma implant, and the cytology aspirate of the peritoneal fluid showed pools of acellular mucoid material. On hospital day 7, the patient’s leukocytosis rose to 23 K, and repeat imaging demonstrated a more organized collection with intraperitoneal air in addition to multiple thick, wall-enhancing, complex cystic multiseptated lesions. Given these findings on imaging as well as her clinical presentation, the patient was taken to the operating room (OR) jointly with surgical oncology and gynecology oncology for a peritoneal washout and bilateral salpingoopherectomy. Intraoperative findings included a large amount of mucin in the abdomen and pelvis with a pocket of purulent fluid in the mid-abdomen, a very large (12 cm) cystic left ovary, dilated right fallopian tube with enlarged right ovary, a very dilated and thickened appendix, and dense, diffuse small bowel intraabdominal adhesions (Fig. ). Her peritoneal cancer index (PCI) score was unable to be determined due to the degree of inflammatory adhesions. She underwent bilateral salpingoopherectomy given the amount of inflammation in the cecum and base of the appendix, the decision was made not to perform an appendectomy to avoid leakage at the staple line and further infectious complication. The patient’s abdomen was hostile, and no larger operation was deemed necessary at this time, as the patient was nonobstructed and the origin of PMP was not yet verified. The primary goal of the operation was to clear the infection, with the intent to return at a later date when her intraabdominal contents could be properly mobilized for a complete PCI score and an appropriate oncologic resection could be performed.\nHer hospital course was complicated by recurrent pleural effusions requiring multiple thoracocenteses, negative for cytology, and persistent need for supplemental oxygen. She also developed a secondary intraabdominal abscess requiring IR-guided drainage. After the patient clinically improved, she returned to the OR 2 months after her initial presentation for completion cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) with 40 mg of mitomycin-C at a target temperature of 42–43 °C over 90 min, as per our institutional protocol []. Her PCI at the second surgery was calculated to be 13. She had minimal peritoneal adhesions, and her intraabdominal infectious process had completely resolved. The patient underwent an appendectomy, omentectomy, and tumor debulking. The appendicular base was healthy and easily stapled across. The patient was discharged home with lovenox on postoperative day 5, saturating well on room air, tolerating a diet with oral pain medication, and with return of bowel function. Six months after the CRS/HIPEC, the patient had surveillance imaging with no evidence of recurrence (Fig. ). The abdominal wall mass was unchanged from prior imaging and, given the biopsy of leimyoma, presumed to be an implant at the port site from the patient’s prior hysterectomy.\nAfter the discharge from the second surgery, the patient’s postoperative recovery was complicated by new-onset shortness of breath after the completion of her 30 day course of lovenox. She was diagnosed with bilateral pulmonary emboli, with no evidence of deep vein thrombus on lower extremity duplex. Therapeutic lovenox was initiated, and her symptoms gradually improved. An interval angio CT of the chest demonstrated resolution of the clot. Anticoagulation has since been discontinued, and the patient will continue with 6-month interval surveillance imaging for PMP recurrence.\nThe pathology of the left ovary and tube following the first operation resulted as an ovarian cyst containing mucin pools and low-grade intestinal type mucinous glands, consistent with metastasis from appendiceal mucinous neoplasm. The right ovary and tube showed mucin, acute inflammation, and adhesions. Immunostains were positive for CK7, CK20, and CDX2 and negative for PAX8 (Fig. ). Following the completion cytoreduction, the appendiceal pathology demonstrated a low-grade appendiceal mucinous neoplasm (LAMN) with acellular mucin outside the appendix and associated fibroinflammatory response. The proximal margin, the appendicieal base, was negative for tumor. Additional specimens sent during the debulking included the falciform ligament, omentum, pelvic tumor nodules, and tumor deposits overlying the sigmoid, small bowel, left colon, and liver. All additional specimens consisted of acellular mucin. Pre-operative carcinoembryonic antigen (CEA) and CA-125 were elevated at 26.8 and 101 respectively. CA 19-9 was within the normal limits. Using AJCC (8th ed.) staging, the final staging was determined to be T4a NX M1a (stage IVA). Since the patient had a complete cytoreduction and the pathology was determined to be LAMN, no further adjuvant therapy was given. |
A 17-year-old girl presented to the surgical clinic with profound malnutrition with an albumin of 1.3 and BMI of 12.9 for evaluation for gastrostomy tube placement. Her medical history was notable for a prior drainage of perirectal abscesses and behavioral evaluations for anorexia nervosa. In addition, she had a two-year history of watery diarrhea about three times per day. On clinical exam, she was noted to have complicated and extensive perianal abscesses and fistulae. Due to her severe malnutritive state, she was admitted to the hospital for total parenteral nutrition, management of her refeeding syndrome, and work-up for IBD. Colonoscopic biopsies revealed Crohn’s disease. During her workup, a CT of her abdomen demonstrated opacities in the bilateral lower lobes of the lung, and a subsequent CT chest demonstrated extensive tree-in-bud opacities predominantly in the bilateral lower lobes of the lung (Figure ). Due to the unclear etiology of these pulmonary lesions and the need to start therapy for her Crohn’s disease, a biopsy of these lung lesions was recommended. However, given her significant malnutrition and asymptomatic pulmonary disease, the decision was made to improve her nutritional status prior to surgical biopsy. After a month of nutrition, a 20-pound weight gain, and an improvement in her albumin to 3.4, a thoracoscopic biopsy was performed and demonstrated necrotizing granulomatous inflammation and focal organizing pneumonia favored to represent pulmonary manifestations of IBD (Figure ). Infectious workup was negative. Her perioperative course was uncomplicated. Her chest tube, which was placed routinely for the treatment of potential pulmonary air leak, was removed on post-operative day one after no air leak was identified. A repeat CT scan was performed just prior to the biopsy (one month after the initial CT scan) to assist with operative planning. This demonstrated persistent, but significantly improved tree-in-bud opacities in the bilateral lower lobes without any treatment with steroids or anti-microbials and prior to initiation of any immunotherapy for her Crohn’s disease. She was subsequently started on infliximab without any repeat imaging of her chest. |
A 17-year-old Caucasian male presented to his orthopedist with a five-month history of lateral right knee and calf pain, with associated stiffness. There was no history of trauma, although he was an avid motorcross athlete. There was no family history of spondyloarthritis, chronic low back pain, or rheumatic diseases. Examination showed only tenderness over the right fibular head. Complete blood count, erythrocyte sedimentation rate, c-reactive protein concentration, antinuclear antibody, rheumatoid factor and Borrelia burgdorferi serologic testing were normal. A technetium bone scan demonstrated uptake of the lateral tibial condyle and fibular head. A subsequent noncontrast magnetic resonance imaging (MRI) showed only bony edema and soft tissue swelling of these same areas (Figures\nA & B). A Computed tomography (CT) scan performed 3 weeks later ruled out occult fracture and neoplasm.\nHe returned for orthopedic evaluation 8 weeks after the CT scan and an MRI with gadolinium showed a moderate TFJ effusion with persistent bony edema and surrounding synovial enhancement without synovial thickening. There was also a small subchondral erosion of the lateral tibia (Figure\nC & D). Upon review of his CT scan, the erosion was evident (Figure\nE).\nSoon after his second MRI, and eight months after the onset of pain, he presented for rheumatologic evaluation. At this point he had been inactive in athletics for several months while his knee pain and stiffness gradually worsened. His examination was notable only for tenderness to the area of his right fibular head and an antalgic gait. His modified Schober measured 5.2 cm. Based on the unique location of the inflammatory process, he had a biopsy of the proximal tibia, which was unremarkable, but did not include any synovium. Anaerobic bacterial culture of the biopsy site grew a corynebacterium species after several days, and was felt to be a contaminant. Aerobic, fungal, and acid fast bacterial cultures were negative. In follow-up 8 weeks later, he had persistent lateral knee pain and now right sacroiliac pain and stiffness of 4 weeks duration, and his Schober was 4.9 cm. Radiographs done soon after the onset of sacroiliac pain were normal. Further evaluation revealed positivity for the Human Leukocyte Antigen (HLA)-B27 haplotype and an MRI of his pelvis revealed early right sacroiliitis (Figure\n). He began treatment with etanercept 50 mg weekly and showed dramatic improvement of his knee and sacroiliac pain and stiffness. His modified Schober had improved to 6.1 cm. He required a single intraarticular corticosteroid joint injection of the right knee for recurrence of stiffness and a small knee effusion 3 months after initiating etanercept. The knee arthritis subsequently resolved. After one year on etanercept, with occasional use of ibuprofen for lingering lateral right knee soreness, he remained stable with no new peripheral arthritis, uveitis, or further decline of lumbar mobility.\nWhile this patient’s age at onset of symptoms exclude him from classification as enthesitis-related arthritis (ERA) or seronegative enthesopathy and arthropathy (SEA) syndrome, his presentation is relevant to the evaluation of younger patients\n[,]. He does not meet criteria for AS based on the modified New York criteria\n[], but these criteria rely on X-ray evidence of sacroiliac involvement that may not be evident early in disease\n[]. The Assessment of SpondyloArthritis International Society (ASAS) criteria are more appropriately applied to this patient. While he does not meet the criterion of > 3 months of inflammatory back pain, his axial imaging is strongly suggestive of sacroiliitis\n[], and his HLA haplotype and peripheral arthritis strongly support an AS diagnosis\n[]. |
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)).\nThe 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. |
A 52-year-old female patient with no obvious abdominal symptoms was admitted for liver mass found by ultrasound examination. A hypoechoic solid mass ranged 5.7 * 5.6 * 5.0 cm was detected in the left lobe, with a clear outline and abundant blood flow inside the tumor. Physical examination showed no jaundice in the skin and sclera, liver palms, and any spider angioma. The abdomen was flat and soft, with no varicose veins. No tenderness or rebound pain was induced by palpation. All investigations of chest and limbs were normal. Laboratory tests showed normal blood cell count and liver functions except a slightly increase of total bilirubin to 21.3 μmol/L. Liver tumor related cell markers, including alpha feto-protein, carcino-embryonic antigen, and carbohydrate antigen 199 were all within normal values. Hepatitis serological tests were all negative. No family or personal history of malignancy disease either.\nBoth CT and MRI examinations revealed the mass in the IVth segment of the liver with a multi-lobular appearance, clear boundary, and internal separations. The solid mass can be enhanced in the CT scan from 20Hu to 30Hu. Long T1 and T2 signal were showed in MRI, with only a weak strengthening in the delayed phase, suggesting the high probability of a benign hepatic tumor (Fig. ).\nPreoperative biopsy was suggested but denied by the patient and an open abdominal surgery was performed to exclude any risk of malignancy. The tumor was identified on the diaphragm side of the liver, with a size of 6 * 5 * 5 cm and white-yellow color, in an exophytic growth pattern (Fig. ).\nThe tumor was resected, and no intraoperative frozen sections were taken because the mass was recognized as a benign lesion. A smooth capsule with a clear boundary can be visualized outside the solid-cystic cortex, containing yellow colored jelly-like substance in the central portion with white fibrous septa (Fig. ).\nMicroscopy findings showed that the tumor was composed with different proportions of loose fibroelastic connective tissue, hypocellular mucus-containing stroma, and scattered bile duct epithelium, associated with partial hemorrhage (Fig. ).\nImmunohistochemistry (IHC) showed staining of Vimentin (Fig. ), SMA, CK, CK7, CD34, < 2% Ki67, but no CD117 and S− 100. Pathological diagnosis was collectively determined as the hepatic mesenchymal hamartoma by three pathological experts. According to AJCC classification, this mass belongs to T2bN0M0/IIb. Based on FNCLCC system, the hepatic tumor belongs to Grade 1 and the nodules of omentum metastasis belongs to Grade 2.\nThree years after the surgery, the patient was readmitted because of massive ascites. Severe peritoneal effusions and omental thickening were detected by both ultrasound and enhanced CT scan. An ultrasound-guided percutaneous biopsy of the omentum was performed and followed by H.E. staining. It was found that spindle-shaped cells were distributed in a bundled or interlaced pattern with a relative high density. Multi-sized nuclei were slightly enlarged and eosinohilic cytoplasms were transparent but lack clear boundaries with surrounding myxoid stroma (Fig. ).\nIHC staining of the biopsy specimen showed positive of vimentin, calretinin, and D2–40, < 5% Ki67, but weak for CK and negative for CK7, CK20, SMA, CEA, CA125, GLUT-1, WT1, CD34, and S− 100. Combination of the clinical progress with histopathological findings indicated that these metastatic lesions were developed from a malignant tumor with a mesenchymal origin, thus considered as low-grade MFS. Pathological consultation was carried out and a historical review of the original liver tumor slices indicated a high homology between these two lesions. The second onset was therefore considered as peritoneal metastasis of the primary liver tumor. This patient passed away 4 months later due to multiple organ failure. |
A 56-year-old man weighing 102 kg and 188 cm tall (Figure ) was referred to our hospital because of an evident thickening around the neck. He had had the enlargement for the last 10 years and lately, the condition worsened. He was also a heavy alcohol drinker for the last 30 years and had 50 pack-years of smoking upon presentation. He was employed as a farmer and construction worker and had never demanded health care for his condition in the past.\nMore specifically, during the last 3 years, the enlargement became a collar-like mass around the neck and dyspnea crises with cough and upper airway excretions appeared, especially when bending the neck. He was relieved after some minutes of neck extension. The patient reported progressive difficulty sleeping due to extensive bulk of the mass, while he never had a problem of snoring, sleep deprivation, or other sleep apnea-associated symptoms. Additionally, he complained of weakness and chronic fatigue during these last years. The mass presented with cervical pain upon weight lifting, while he did not have any signs of neuropathy.\nHe referred a known history of hypertension with frequent hypertensive crises with systolic blood pressures of 220-230 mm Hg after heavy drinking, for which he used an angiotensin II receptor blocker (ARB) and hydrochlorothiazide and was usually referred to the local hospital. The cardiac assessment was normal. He had suffered from hypothyroidism for the last 15 years.\nUpon examination, he had a circular mass of the neck, semi-hard on the anterior and hard on the posterior in a double oval-shaped configuration. The mass was not fixed to the overlying skin. His articulation was disturbed, and he had stridor while bending the head. Interestingly enough, we noticed that his legs and arms had lipohypertrophic along with lipoatrophic areas and that his supraclavicular regions were hypertrophic. The identification of the “pseudoathletic” type of Madelung's disease (type I), and hence, the diagnosis was delayed because the patient's physique was attributed to his occupation as a construction worker. Neurological examination was normal.\nHigh values of uric acid, cholesterol, triglycerides, CPK, and LDH were reported. A slight elevation of SGOT along with a double γ-GT price was compatible with alcohol abuse.\nA facial, cervical, and thoracic computed tomography (CT) showed a bilateral and symmetrical fat mass of the posterior and anterior region of the neck and of the upper trunk, without displacement or infiltration of the trachea (Figure ). Based on these findings, the diagnosis of Madelung's disease was made and we decided to treat the patient surgically.\nTwo different operations took place. In the first one, orotracheal intubation was impossible, so nasotracheal intubation was performed. A Gluck-Sorenson incision was performed, and fat tissue was excised in a similar fashion as in bilateral functional lymph node neck dissection with preservation of the accessory and marginal and cervical branches of the facial nerves. More specifically, we dissected levels I-V on the right side and levels I-VI on the left side. The dissection of the tissue in the carotid triangles was laborious (the fat tissue had septa that extended between the structures). The excised specimen's size was 27 × 12 × 18 cm, and its weight was 627 g (Figure ). A tracheostomy was performed before completion.\nIn the second operation, which took place 3 weeks later, the patient was placed in a prone position. The fat masses of the cervical, occipital, suboccipital, retro-auricular, and upper dorsal regions were removed surgically and with liposuction. The weight of the fat tissue was 114gram. We continued with removing the fat mass from the upper anterior cervical region. Part of the skin was removed because of its excess. The tracheostomy from the first operation remained and was finally removed 6 days after the second operation.\nHistological examination after both the operations confirmed the initial diagnosis.\nDuring the next 6 months, we suggested the use of a soft collar because we desired extra tension of the musculocutaneous tissue over the neck triangles for better healing. The patient did not follow our orders for alcohol abstinence.\nIn Figure , we can see the final result three months later. |
NO is a 70-year-old retired man. His health history is remarkable for a cholecystectomy in 2001 that was followed by confusion. An epileptic seizure was suspected and the patient was treated with carbamazepine for a short period. He did not have any new seizures after carbamazepine was withdrawn. The patient has a positive family history of AD with both his grandfather and mother having been diagnosed with the disease.\nIn 1995, NO started to complain of cognitive decline that progressed over the years with aphasia, apraxia, and agnosia. He developed spatial deficits with orientation difficulties and a strong tendency to misplace belongings. In 2002, he was diagnosed with AD. A magnetic resonance imaging (MRI) scan of the brain was normal with no atrophy or white matter changes. EEG showed slow postcentral rhythm (7 Hz), and single positron emission computed tomography (SPECT) of the brain revealed frontotemporal hypoperfusion with left-side predominance. CSF analyses showed slightly elevated albumin ratio of 12 (<10) as a sign of mildly impaired blood-brain barrier function, normal cell counts, elevated total-tau (T-tau) of 589 ng/L (laboratory reference limit <400 ng/L), and normal Aβ1-42 concentration 495 ng/L (>450). He was heterozygous for the Alzheimer-associated ε4 allele of the apolipoprotein E gene. Neurological examination was normal, and the neuropsychological profile and clinical evaluation were consistent with AD. The patient started treatment with galantamine. His cognition improved with the minimental state examination (MMSE) score rising from 22 to 28 points.\nIn November 2006, the patient was screened for a clinical trial of active immunization against Aβ. His cognition had been rather stable over the four years on treatment with only a minor decrease of the MMSE score from 28 to 25. He had no somatic complaints and his somatic status was normal. He showed no symptoms of infection and had no neurological complaints. An MRI scan of the brain showed slight atrophy with mild white matter changes consistent with AD.\nA lumbar puncture was performed at baseline before inclusion in the trial, and the biomarkers for AD, T-tau, phospho-tau181 (P-tau181), and Aβ1-42 were 520 (<400), 96 (<80), and 460 (>450) ng/L, respectively, which is consistent with AD. The albumin ratio was further elevated, and, surprisingly, CSF monocyte counts were high and so was the IgM-index (). Agarose gel electrophoresis of serum and CSF followed by immunoblotting against IgM supported ongoing intrathecal IgM production. This neurochemical picture is consistent with neuroinflammation. Anti-Borrelia titers were positive in CSF but negative in serum. The patient was treated with standard doxycycline 200 mg twice a day for 10 days. He was then followed with repeated CSF taps for 1.5 year during which his albumin ratio and cell counts were normalized. However, T-tau and P-tau181 remained pathologically elevated and Aβ1-42 turned low indicating an ongoing AD process. Accordingly, the patient has continued to deteriorate cognitively during the followup period.\nThe next case, BS, is a 68-year-old woman. She is occupied as a hairdresser, still working part-time. Her health history is unremarkable. In 2003, she started to suffer from progressive cognitive deterioration, and in February 2006 she fulfilled clinical criteria for AD and started treatment with galantamine. The diagnosis was based on the anamnestic record and supported by findings on MRI and SPECT, as well as neuropsychological test results. To further secure her diagnosis, a CSF tap was planned but she declined this investigation. The patient was terrified of her diagnosis and wanted to fight it the best she could. She had heard of ongoing immunization trials at the Karolinska University Hospital and was referred for being evaluated for eligibility. As a part of this evaluation, a baseline CSF tap was mandatory and she agreed on this. The results showed completely normal T-tau, P-tau181, and Aβ1-42 concentrations in CSF speaking against AD. However, the albumin ratio was strongly elevated, CSF monocyte counts high, and both the IgG- and IgM-indices elevated () with oligoclonal IgG and IgM bands selectively in CSF as a further sign of intrathecal IgG and IgM production. The anti-Borrelia titer in serum was negative but positive in CSF. She was treated with standard doxycycline 200 mg twice a day for 10 days. She was then followed with repeated CSF taps for 1.5 year showing normalization of the blood-brain barrier function and cell counts. Her cognitive status slowly improved during this period and the AD diagnosis was disposed. |
A 55-year-old Caucasian male was referred from another clinical department as he had undergone a routine chest radiograph that revealed multiple mediastinal and diaphragmatic nodules, raising concern over possible malignancy. The patient was employed as a Chemical Engineer at various chemical plants and exposed to a large array of chemical fumes, including arsenic. He was a smoker until 1 year before presentation and was previously exposed to asbestos related to his occupation as engineer in the mining and construction industry. His surgical history included abdominal gunshot wounds suffered in the 1970s while he was enrolled in military service, one which led to an emergency splenectomy. His medical history included admission for severe malaria in the mid-1980s, which required admission to an intensive care unit.\nThe absence of Howell–Jolly bodies on the peripheral blood smear at presentation (Fig. , left) was a possible clue to the presence of ectopic splenosis as their presence would be expected in a fully splenectomized patient. A CT scan of the chest (Fig. ) was requested without contrast due to impaired baseline renal function. This revealed pleural plaque formation, as well as enlarged mediastinal lymph nodes together with multiple pleural- and mediastinal-based soft tissue nodules. Some of these nodules were calcified. Basal ground-glass opacification was also noted. Differential diagnosis of these lesions includes both primary and metastatic malignancies (e.g. malignant mesothelioma, lymphoma and metastatic adenocarcinoma) as well as non-malignant tumours (e.g. lipoma) and various infectious and non-infectious causes (e.g. asbestosis and sarcoidosis). Granulomatous infection due to M. tuberculosis was an important consideration as South Africa is a country burdened by a very high prevalence of disease due to this pathogen. Due to his increased occupational exposure asbestosis was a possibility and this would also explain the early ground-glass opacification. Sarcoidosis was considered, although no systemic or extra-pulmonary manifestations were present. The inability to perform a contrasted CT scan limited our ability to narrow the differential diagnosis in this patient.\nThe patient therefore underwent formal thoracotomy and excision biopsy at multiple sites. Histological examination of the relevant sites revealed splenic tissue with white and red pulp, secondary germinal centres, lymphoid follicles and granulocytes, surrounded by a fibrotic ring. These findings were consistent with splenosis in all the relevant biopsies. No evidence of malignancy was observed (Figs and ).\nAt 1-year follow-up, the patient remained well and the appearance of Howell–Jolly bodies was noted on repeat blood smear (Fig. , right), following the previous excisions. |
A 38-year-old female was admitted in the gynecology outpatient's clinic due to secondary infertility. She was Gesta I Para I, with one previous uneventful pregnancy and a cesarean section 7 years before. She had hypothyroidism and was medicated with levothyroxine 150 μg. As soon as she left the combined pill in order to conceive, she started complaining of severe dysmenorrhea (pain score: 8/10). At physical examination, a hard and painful nodule was palpable at midline, under the cesarean section scar. She had a nonmobile uterus. A painful hard mass was palpable at rectal digital examination, 3 cm away from the anus.\nAbdominal wall soft tissue ultrasound revealed a nodule of heterogeneous echogenicity with 41 mm × 34 mm × 17 mm located medially at the level of the rectus abdominis muscle, under the cesarean section scar [Figures -]. Abdominal magnetic resonance imaging confirmed the presence of a nodule with 4.9 cm × 3.8 cm × 2.0 cm on the rectus abdominis muscle and its fascia [Figures and ]. Pelvic transvaginal ultrasound revealed normal uterus and right adnexa and a cyst at the left ovary with 30 mm × 26 mm, with no septa or solid components and with ground glass echogenicity.\nThe patient was submitted to laparotomy. During surgery, a hard median nodule with 5 cm of length was found located in the rectus abdominis muscle and its fascia [Figures and ]. Pelvic anatomy was completely distorted with several adhesions between the uterus, the adnexa, colon, and pelvic wall. The left adnexa was involved in adhesions, the left tube was enlarged, and there was a 3 cm cyst in the left ovary. The abdominal wall cyst was removed, and ovarian cystectomy, left salpingectomy, and contralateral tubal patency test were performed. The ovarian cyst had a dark brown thick liquid inside. Despite the adhesions, the right adnexa was normal and the right tube was patent. |
A 29-year-old male with a history of right tibial shaft and lateral malleolus fractures following a fall injury underwent open reduction and internal fixation with a locking plate for the fibula fracture and minimally invasive percutaneous osteosynthesis for the tibial shaft fracture at another hospital. Postoperatively, the patient was followed up at our hospital for 1 year due to residential issues, and the removal of the plate was scheduled after confirmation of bone union. According to the previous hospital’s medical records, a 9-hole locking plate (APIS®, Gwangju, Republic of Korea) was used for the distal tibia, with three 5.0-mm locking screws for proximal fixation and six 3.5-mm locking screws for distal fixation. Under spinal anesthesia, a plate removal surgery was performed, following which the fibular plate was easily removed. However, five of the six distal locking screws were damaged, and they could not be removed with a screwdriver. This situation was explained to the patient during the operation, and we could confirm that the patient wanted to remove the plate irrespective of the amount of time required. At the time, a tourniquet was used for approximately 1 h, and screw removal was attempted using a conical extraction screw, but only one of the six screws could be removed. Previously proposed techniques requiring additional skin incisions were not considered because there were multiple damaged screws that could not be removed, these techniques would require extended amount of time for removal, and the surrounding soft tissues could be damaged. Hence, we decided to use a screw extractor (IRWIN®, Huntersville, NC, USA), a non-medical instrument, following sterilization with ethylene oxide. In brief, 6-mm drill bit of the extractor was used, four screw heads were drilled, the locking screws and locking plate were separated, and the plate was removed. Of the remaining four screws, one was removed using a vice grip; the remaining three screws could not be removed with the vice grip owing to a short extruded portion. A hollow reamer could not be used because the three screws were adjacent to each other and the reamer could cause a large bone defect; hence, we created sufficient space around the screws by forming small holes around them with a 1.8-mm K-wire, following which all screws could be removed using the vice grip. After saline irrigation, debridement of the soft tissues contaminated with metal debris was performed. The sizes of the metal debris were found to be relatively large, which facilitated easier debris removal. With 2-h use of a tourniquet, all implants could be removed and the skin could be closed (). Postoperatively, no complications were noted during physical and radiological examinations. Remarkably, no problems were noted at the final outpatient visit at 3 months after discharge; therefore, follow-up was terminated. The patient had not visited an outpatient clinic for more than a year since the last follow-up, thus we thought that he had no specific complications including infection and re-fracture. |
An 80-year-old woman presented with a history of intermittent, severe epigastric pain. Over the preceding 5 months, she had less severe and self-resolving epigastric pain 15–30 min after every meal. The full blood count, serum biochemistry values, and C-reactive protein level were within normal ranges. Additionally, arterial gas analysis was normal. Abdominal plain radiography showed free air between the liver and diaphragm, and PI (Fig. ). Abdominal computed tomography showed severe calcification of the SMA origin; SMA peripheral flow was reserved (Fig. ). Computed tomography (CT) revealed bubble-like intramural gas of the small bowel with the contrasted wall pneumoperitoneum (Fig. ).\nSince the patient did not have peritonitis, a conservative approach was performed. She was managed in the condition of intensive care unit, due to fears of the potential for acute mesenteric ischemia due to mesenteric artery occlusion or non-occlusive mesenteric ischemia. She was managed for bowel obstruction, which included fasting and intravenous fluid administration. She received heparin infusion to prevent SMA occlusion and maintain collateral flow. During admission, she reported abdominal pain relief. Seven days after admission, abdominal plain radiography showed improvement in PI and pneumoperitoneum; therefore, she was permitted to begin drinking fluids. There was no evidence of recurrent abdominal pain after the fluid consumption, so she was allowed to eat solid food.\nAngiography was planned to relieve the postprandial abdominal pain. The findings showed severe stenosis of the SMA origin with calcification, and the SMA had replaced the common hepatic artery (Fig. , ). ET, namely stenting to the SMA and common hepatic artery, was performed. The SMA trunk was engaged with a 6F Parent Plus 60 guiding catheter (Medikit, Tokyo, Japan) from the left brachial artery. Initially, 8000 units of heparin was infused, and additional heparin was added to keep the activated whole blood clotting time over 200 s. The SMA occlusion was traversed using a 0.014” NEO EVT Guide Wire Cruise (ASAHI INTECC J-sales, Tokyo, Japan). The SMA trunk to the hepatic artery was traversed using a 0.014” NEO EVT Guide Wire Cruise, which was engaged with a 6F SheathLess PV (Cardian Health, Ohio, USA) from the right brachial artery. Intravascular ultrasonography (IVUS) (Navifocus WR, TERUMO, Tokyo, Japan) revealed severe stenosis of the SMA trunk with calcification. Balloon dilation was performed with the kissing ballon technique using a 4-mm Coyote ES (Boston Scientific, Marlborough, MA, USA) to the SMA and 5-mm SHIDEN RX (KANEKA MEDICAL, Osaka, Japan) to the common hepatic artery. After dilation, two balloon-expandable stents (5-mm PALMAZ Genesis (Cardian Health, Ohio, USA) to the SMA and 6-mm PALMAZ Genesis to the common hepatic artery) were implanted with the kissing stent technique from the common ostium (Fig. ). The sizes of the stents were based on the IVUS measurements. IVUS showed good orifice expansion. Final angiography showed satisfactory circulation to the terminal organ from the orifice (Fig. ).\nEndovascular therapy relieved the patient’s symptoms. She required dual anti-platelet therapy (DAPT) with aspirin and clopidogrel post-intervention. Follow-up computed tomography showed patency of the orifice of the common hepatic artery and SMA, and there was no evidence of PI or pneumoperitoneum. No recurrent postprandial abdominal pain occurred for two years after the ET. |
We present a 57 year old gentleman with CKD 5 who had an autogenous brachiocephalic fistula 4 months prior to presentation to us. During their last surgical clinical visit, the fistula was noted to be poorly maturing and then referred to interventional radiology for fistulogram and possible endovascular intervention to assist with fistula maturation.\nThe patient had a fistulogram which demonstrated a high grade juxta-anastomotic stenosis which was successfully balloon dilated. After a 6 week follow up clinic visit the fistula was still immature and a duplex scan, a second fistulogram with possible intervention were requested.\nFistulogram was performed via an antegrade approach from an access just proximal to the swing point. There was an “apparent” stenosis (Fig. ) which was angioplastied then followed by severe spasm (Fig. ). which was perceived by the operator to be recalcitrant stenosis. In the light of this perceived recalcitrant stenosis, a decision to stent the area was taken. After measuring the vessel diameter based on the immediate post-plasty images a 6 mm diameter × 5 cm length Viabahn stent (Gore & Associates, Flagstaff, AZ) was selected and deployed in the standard fashion.\nFollowing stent deployment, the stent migrated and stopped at the confluence of the cephalic vein and the subclavian vein (Fig. ). At the time the operator thought the stent was stable and unlikely to cause harm to the patient in this position. However, after reviewing the images with colleagues including vascular surgeons a decision was made to attempt to retract the stent into the arm which would be easier for the surgeon to retrieve the stent surgically, if required. The patient was subsequently brought back 24 h later to the interventional radiology suite.\nInitial fluoroscopic image of the left shoulder region demonstrated the stent was absent from the final position documented the previous day indicating the stent had migrated further (Fig. ). Fluoroscopic scanning of the chest identified the stent to overlie the left lower lobe (Fig. ).\nSubsequent Pulmonary angiogram confirmed the stent to lie within a segmental pulmonary artery of the left lower lobe (Fig. ).\nAfter discussion of the options, risks and benefits with the patient and a multidisciplinary team, a decision to attempt stent retrieval was made versus leaving the stent in situ.\nAfter appropriate informed written consent, the right groin was prepped and in the standard fashion. Right common femoral vein access was then upsized to accept a 16 F sheath (Cook, Bloomington. IN USA). Main pulmonary access was then performed with an APC pulmonary catheter (Cook, Bloomington. IN USA). The APC catheter was then removed over a Storq wire (Cook, Bloomington. IN USA) wire and subsequently a 12 F 70 cm braided sheath was advanced into the main pulmonary artery and then left lower lobe pulmonary artery. Pulmonary angiograms performed identified the optimal projection to identify the vessel to access. After accessing the appropriate vessel the 12F sheath was advanced just to the origin of the branch above the stent. Subsequently a 15 mm Amplatz Gooseneck snare (ev3, Plymouth MN, USA) was manipulated until the stent was lassoed at about half way along the stent. Given the flexibility and potential collapsibility of the Viabahn stent it was over-sheathed carefully collapsing and gently retracting the captured stent to minimize potential vessel injury (Fig. , Additional file ). Once the stent had been totally ensheathed, the 12F sheath was retracted through the outer 16F sheath coaxially. The stent was retrieved intact (Fig. ).\nThe procedure was performed under moderate sedation using Fentanyl and midazolam with continuous monitoring of the patient’s vitals by a dedicated nurse. Throughout the procedure the patient remained hemodynamically stable with normal respiratory function. Post procedure the patient was observed for 6 h post procedure before being discharged home in a stable condition.\nThe patient has so far been followed up for 3.5 years and has not developed any adverse pulmonary or cardiac condition. Interval CTPA done at an outside facility showed normal pulmonary vasculature with no evidence of pulmonary vessel injury. |
A 66-year-old right-handed man was referred to the cognitive neurology clinic following 4 episodes of transient amnesia over a 6-month period. Each episode occurred within hours of strenuous physical exercise. In the first episode, he returned home from a bicycle ride confused about the route he had taken. The second event occurred following a walk up a steep incline. The third event occurred the day after a strenuous bike ride when the patient awoke in the morning confused as to where he was and what the plan for the day was. This confusion recurred the same day following a post-prandial nap. All the events were witnessed by the patient's wife who noted repetitive questioning to be a feature in each. All lasted between 30 min and 2 h with complete recovery. No other accompanying focal neurological symptoms were noted during the attacks.\nThe patient's past medical history was unremarkable: he had undergone two hip replacements but was otherwise fit and well. There was no history of recent head injury or epilepsy. He consumed 12 units of alcohol per week and was a lifelong non-smoker. He had previously worked as a project manager for a bank and regularly enjoyed cycling, golf and walking. There was no family history of any cognitive disorder.\nNeurological examination in the clinic was unremarkable. On cognitive testing, he scored 0/28 on the Six-Item Cognitive Impairment Test (normal) and 28/30 on the Mini-Mental State Examination.\nA diagnosis of TGA, concordant with suggested diagnostic criteria [], was thought likely on the basis of the history, at least for the first 2 episodes. However, because of the recurrent nature and brevity of the attacks and events emerging on waking from sleep, all features more suggestive of TEA [], further investigations were undertaken.\nMagnetic resonance (MR) brain imaging showed some very subtle but unequivocal signal change in the right amygdala (fig. ), the nature of which was uncertain. Diffusion-weighted imaging was normal with no restriction of diffusion and no disruption of limbic white matter tracts or adjacent temporal fibre bundles. Single-voxel MR spectroscopy of the affected area showed elevated choline resonance (NAA:Cho ratio = 0.64). Repeat MR brain imaging performed 2, 6 and 12 months later showed unchanged appearances. The imaging findings were thought most likely to indicate low-grade neoplasia, or possibly dysplasia or gliosis. Initial concerns that the imaging findings might reflect limbic encephalitis or frequent epileptic seizures (‘epileptic crisis’) were refuted by the clinical phenotype and the stability of the imaging changes, without evidence of focal atrophy.\nThe standard electroencephalogram (EEG) was within normal limits, but sleep-deprived EEG showed excess slow waves over the right temporal region and one prolonged run of slow waves followed by brief high-amplitude sharp wave bursts.\nBecause of the clinical and radiological stability of the patient, a biopsy of the lesion was not thought to be indicated. The patient continues to be followed up with regular surveillance MR brain imaging and has had further occasional amnesic episodes on waking from sleep. For these episodes, prophylactic anti-epileptic drug therapy has been offered but the patient has declined this on the basis of the infrequency of the events and concerns about possible adverse effects of medication. Repeat Mini-Mental State Examination 12 months after initial presentation was stable (score 29/30).\nIn light of our experience with this case, we undertook a systematic review to identify reported cases of TGA and brain tumour. (Although the diagnosis of tumour was not definitively established in our case, it was our provisional diagnosis since it was the most likely on the basis of the investigation findings, and the system of surveillance initiated was that employed at our centre for suspected tumour cases.) A systematic literature search was undertaken using several abstract databases (Medline, from 1966 until the end of 2013; PsycINFO, from 1887 until the end of 2013, and Embase, from 1980 until the end of 2013). Five full-text collections (Science Direct, Ingenta Select, Ovid Full Text, Blackwell Online and Wiley Interscience) and the abstract database Web of Knowledge (4.0, ISI) were searched. The following search terms were used: ‘transient amnesia or TGA or TEA’ and ‘tumour or glioma or meningioma or cancer or dysplasia or gliosis’.\nThe findings, including the current case, amounted to 20 examples (table ) [, , , , , , , , , , , , , , , , , ]. This has extended the previous narrative reviews of TGA and tumour by Dinca et al. [] and Daniel []. No cases of transient amnesia associated with either dysplasia or gliosis were identified, although pathological evidence of gliosis may be found in some cases of epilepsy-related amnesia; however, the latter is persistent rather than transient [].\nThe only study reporting data which might address the question of the frequency of TGA associated with brain tumour was that of Agosti et al. []: in a series of 130 patients meeting diagnostic criteria for TGA and undergoing investigation with modern brain imaging modalities, 13 patients had structural brain lesions, of whom 2 (1.5%) had falx meningiomas. No case of brain tumour was encountered in the series (n = 114) of Hodges and Warlow []. In a consecutive series of 24 TGA cases seen in our clinic [, , ], this is the first case encountered in which a brain tumour has been found.\nMany of the reports [, , , , , , , , , , , , ] predated widely accepted clinical diagnostic criteria for TGA [], and for this reason, some cases might be excluded as not conforming to criteria. For example, in one case, the amnesic episode lasted more than 24 h [], and in another, progressive memory problems followed a generalised tonic clonic seizure []; in yet another case, the patient was reported to have 6 episodes of TGA and on examination had bilateral papilloedema [] (criticised as unlikely to be TGA by Hodges []). Caplan [] previously criticised the case reported by Meador et al. [] on the grounds that the reported clinical features (two short-lasting and unobserved episodes of loss of awareness) did not suggest TGA.\nThe clinical features of TGA cases with and without neuroimaging evidence of structural brain lesions are said to be the same []. However, in the current case and one previous report [], both associated with medial temporal lobe pathology, there have been clinical features suggestive of both TGA and TEA.\nVarious tumour locations were reported. Some were located distant from memory-eloquent structures, such as falx meningiomas [, ] and pituitary tumour [], one associated with pituitary haemorrhage []. On the other hand, tumours located within or in proximity to memory-eloquent structures, such as the temporal lobe [, , , ], limbic system [], hippocampus [] or amygdala (current case), were also reported.\nMost reported cases of TGA and brain tumour had either histologically proven primary brain tumours [, , , , , , , , , ] or were presumed to be primary brain tumours where histological analysis was not performed. Two cases were secondary tumours, from a bladder transitional cell carcinoma [] and a lung primary [], respectively.\nIf the absence of EEG abnormalities is accepted as a TGA diagnostic criterion (EEG is very seldom recorded during an amnesic event) [], 6 of the reported cases might be excluded on the basis of abnormalities on interval EEG studies [, , , , , ]. (EEG was not reported, presumably not undertaken or normal in at least 6 other cases [, , , , ].) It is possible that some of these cases might have been instances of TEA rather than of TGA, although EEG abnormalities are only seen in about one third of TEA cases on interval EEG []. In his review, Daniel [] stated his belief that the cases reported by Hartley et al. [], Shuping et al. [] and Honma and Nagao [] are likely to be instances of TEA rather than of TGA, and possibly also the case of Meador et al. []. |
A 67-year-old male patient presented with painless jaundice, dark-brown urine and acholic stools. The general practitioner had diagnosed onset of a diabetic condition with a HbA1c of 8.5% and a weight loss of 4 kg, corresponding to a weight loss of 5%. His antecedent history showed prostate cancer treated by prostatectomy 6 years before and a malignant melanoma on his back 5 years before. Both malignant conditions were followed periodically and did not show any signs of recurrence. Furthermore the patient had had borreliosis 30 years earlier and polymyalgia rheumatica for 2 years. The latter had been treated with low-dose steroids for one and a half years. During the first 6 months, 5 mg of prednisone was given daily, then prednisone was tapered to 2.5 mg twice a week.\nA CT scan showed a double duct sign without clear delineated tumor, no calcification of the pancreas nor infiltration of the vessels (fig. ). A sonography was performed, which showed a mass of 15 × 23 mm in the pancreatic head. Blood tests showed normal values of the tumor markers CEA (2.4 μg/l) and CA19-9 (<3 kU/l) as well as total bilirubin of 50 µmol/l. Surgery was indicated since we suspected pancreatic cancer due to the clinical and radiological findings.\nInfiltration of the mesocolic artery found during surgery made the planned Whipple procedure impossible. Biopsies were taken from the pancreatic head. A cholecystectomy and a biliodigestive anastomosis were performed. The histological results showed chronic inflammation with fibrosis and fat necrosis, but no signs of malignancy. After discussion of the case on a multidisciplinary tumor board, a secondary percutaneous biopsy was taken. This one showed chronic periductal inflammation with infiltration of plasma cells, sclerosis, venulitis, parenchymal atrophy (fig. , fig. ) and strong positive immunohistochemical staining for IgG4 (fig. ). This led to the diagnosis of type 1 autoimmune pancreatitis in the absence of granulocytic epithelial lesions.\nWe determined IgG4 in the serum, which was elevated (3.4 g/l, normal range 0.03–2.01 g/l), although the other autoimmune rates (rheumatoid factors, antinuclear antibodies, c-ANCA, p-ANCA) were normal. A follow-up CT scan 6 weeks later showed a 15% reduction in the craniocaudal dimension, resulting in a less prominent pancreas with inhomogeneous alteration of the structure. Nevertheless, neither calcification nor tumor mass was found (fig. ). As the patient was meanwhile asymptomatic, further steroid therapy was omitted. |
A four-year-old female patient was admitted to the pediatric infectious ward due to a fever of unknown origin in January 2020.\nShe was a known case of Marfan syndrome with a family history of this syndrome in her mother. Both of them had a history of anterior\nlens dislocation that led to surgery.\nShe also had a history of congenital heart disease, including atrial septal defect type 2 (ASD2), mitral regurgitation (MR),\nand MV prolapse regarding fever, headache, and meningeal signs a lumbar puncture was performed. She was treated for probable\nbacterial meningitis since the result of her cerebrospinal fluid (CSF) test was positive and active; moreover, the white blood\ncell was observed in CSF fluid.\nAfter five days and secondary to the changing of heart murmur intensity that revealed progressive severe MR, she was referred to\na tertiary referral center for the evaluation of cardiac involvement. Transthoracic echocardiography (TTE) revealed a large dense\nheterogeneous and oscillating mass (18 mm×12 mm in size) attached to the atrial septum closed to the hinge point of the anterior\nleaflet of MV with independent movement towards the heart valve (an argument for the fungal endocardial vegetation)\n(Figures ,,).\nResults of color Doppler flow imaging revealed an abnormal regurgitation flow at the areas of A3 anterior leaflet of mitral suggestive\nof perforation of MV. In addition, valvular destruction, perforation of the anterior leaflet at the site of the lesion, as well as\nthe existence of perivalvular infection and severe eccentric MR reduced left ventricular ejection fraction. Sub-systemic pulmonary\nhypertension was found with mild pericardial effusion due to acute heart failure, previous history of congenital heart disease, and ASD2.\nNeurologic physical examination for the evaluation of meningeal involvement contains clinical examination and paraclinical data.\nThe cerebrospinal fluid analysis showed meningeal irritation and brain magnetic resonance imaging revealed a small subcortical\nfocus of involvement probably due to post-endocarditis embolic lesions.\nInfective endocarditis (IE) was diagnosed according to the DUKE criteria. Regarding the clinical and paraclinical criteria,\none major criterion and two minor criteria were observed. Persistent fever and the presence of refractory heart failure due to\nsevere acute MR led to surgery for vegetation removal, MV repair, and ASD closure. Surgical findings in the operation room\nindicated large fungal vegetation with perforation in the infection site. She was discharged after daily slow intravenous (IV)\ninjection of liposomal amphotericin B dose 0/3 mg/kg for four-week. Moreover, the daily consumption of oral antifungal Voriconazole\n200 mg film-coated tablets was continued for one month at home.\nThe results of the present study showed in vitro resistance to fluconazole (minimum inhibitory concentration> 16 µg/mL).\nThe TTE in the outpatient clinic after two weeks, as well as one, three, and six months after discharge showed no recurrence\nexcept mild eccentric non-significant MR.\nsummarize the results of the hematologic indices. Biochemical test results revealed\ncreatinine 0.3L mg/dl (NR: 0.6-1.4); AST(SGOT) 70H IU/L (NR 5-40); ALT(SGPT) 83H IU/L (NR 5-40).\nThe peripheral blood sample was taken and inoculated into an anaerobic blood culture medium (manufactured in BC, USA)\nat 30ºC for five days in an automated blood culture system (manufactured in BC, USA); at 30ºC for five days; however,\nno growth was observed. The resected vegetation was inoculated into formalin and normal saline solution. The pathology result\nrevealed a fibrohyalinized tissue with an area of fibrinoleukocytic exudate, mixed inflammatory cell infiltration,\nand neovascularization consistent with endocarditis. Furthermore, the hematoxylin and eosin staining showed a few\nthin hyphae-like elements. To confirm the fungal infection, the second portion of the specimen was sent to the Medical\nMycology Laboratory of the Center for Research and Training in Skin Diseases and Leprosy in Tehran, Iran for further evaluations.\nInitially, the biopsy specimen of the MV was taken and a sub-culture was made on Sabouraud Dextrose Agar (SDA)\n(manufactured in Merck, Germany) at 35ºCfor seven days. After seven days, the colonies of Aspergillus spp.\nwere grown in the SDA medium. The PCR for the amplification of the β-tubulin gene was conducted to identify the species precisely.\nGenomic DNA was obtained from the colonies cultured on the Czapek’s Agar (manufactured in Merck, Germany)\nforseven days at 35ºC using the genomic DNA extraction kit (manufactured in Roche Life Science, Germany).\nIn this study, the amplification of the β-tubulin gene was performed using the β-tubulin forward and β-tubulin reverse primers\n( ). The PCR amplicon was subjected to sequence using the same primers.\nSubsequently, the sequences were compared with reference data available from the Gene Bank database using the BLAST\nsequence search tool ().\nThe antifungal susceptibility test was performed by the broth microdilution methodusing four antifungal agents,\nincluding voriconazole, itraconazole, fluconazole, and amphotericin B (manufactured in Sigma-Aldrich, USA).\nBased on the Clinical and Laboratory Standards Institute (CLSI) M38 3rd ed ( ),\nCandida parapsilosis (ATCC 22019) was chosen as a quality control strain in every run.\nThe culture on SDA revealed green and powdery surface colonies. The microscopic examination of the colonies was compatible\nwith A.flavus. The sequencing result was interpreted and deposited in the GenBank under accession no: MW195498.\nThe A. flavus strain showed resistance to fluconazole with minimum inhibitory concentration (MIC>16 µg/mL).\nFurthermore, susceptibility to voriconazole (MIC 0.313 µg/mL), amphotericin B (MIC 0.313 µg/ML), and itraconazole\n(MIC 0.25 µg/mL) was noted in this study.\nThe patient was first treated with vancomycin and ceftriaxone which was\nlater switched to the combination of meropenem, ciprofloxacin, and vancomycin when the fever continued and the clinical\nstatus deteriorated. The patient was treated with liposomal amphotericin B regarding the result of cultures and the diagnosis\nof IA. She was discharged after four weeks of treatment with IV amphotericin B. It is worth mentioning that the antifungal\ntreatment was continued with oral voriconazole for an additional four weeks after the performance of the susceptibility test.\nThis case report was performed in compliance with the Declaration of Helsinki and written informed consent was obtained\nfrom the legal guardians of the patient. The details will be included in the manuscript for publication. |
A 30-year-old female with a past medical history significant for total thyroidectomy on levothyroxine developed a sinus infection, cold-like symptoms, and a severe headache so she decided to see an outpatient neurologist. She had a brain MRI which was normal. She was told she was having a complex migraine and was not prescribed any medications. She then developed severe nausea and emesis, and therefore went to the ED for evaluation. She was ultimately told that she had gastroenteritis and was discharged home from the ED. One week later, she began to experience muscle weakness which began in the upper and lower extremities but progressed to her facial muscles. She again presented to the ED due to the increasing severity of the weakness. In the ED she was unable to ambulate, get out of bed, or speak more than a few words at a time. The cold-like symptoms, nausea, and vomiting had subsided by this time, but she still had headache and had developed right-sided jaw pain, tongue swelling, very mild neck pain, and paresthesias in her toes and fingers. She revealed in the ED that two weeks ago she had returned home to Florida from a trip to North Carolina where she spent time in a suburban setting. She did not go camping or spend any time in a rural setting. She did not notice any tick bites, although she admits to having one to two mosquito bites.\nNeurology was consulted due to the weakness, headache, paresthesias, and jaw pain. In the ED she was afebrile without leukocytosis. On physical exam, she had symmetric 4/5 strength in the upper extremities and symmetric 2/5 strength in the lower extremities. She was hyporeflexic in the upper extremities and areflexic in the lower extremities. She was admitted to the hospital, and her initial workup included a complete blood count, basic metabolic profile, chest X-ray, and electrocardiogram which were all unremarkable. Additional blood work was ordered including thyroid-stimulating hormone, rapid plasma reagin, hepatitis C, HIV, vitamin B1, B6, D, E, B12, and folate which were also all unremarkable. An MRI of the brain with and without contrast was ordered and was negative for meningeal enhancement (Figure ). An magnetic resonance angiography (MRA) head without contrast and an MRA neck with and without contrast were ordered and were negative for vertebrobasilar insufficiency and carotid stenosis. After preliminary testing was deemed unrevealing, a lumbar puncture was performed.\nCSF studies showed an elevated protein level of 298 mg/dL (range 15-45 mg/L), white blood cell (WBC) count of 6/mcL (range 0-4/mcL), and glucose of 56 mg/dL (range 40-75 mg/dL) (Table ). CSF infectious screen for syphilis, cytomegalovirus, and Epstein-Barr virus were all negative. The CSF study values were indicative of albuminocytologic disassociation, which along with the clinical presentation were consistent with a diagnosis of GBS. Electromyography (EMG)/nerve conduction velocity (NCV) studies were not available in the inpatient setting. Treatment with a two-day course of IVIG 1 g/day was initiated along with a corticosteroid regimen consisting of an initial 20 mg dose with a three-week taper of 8 mg BID, 4 mg BID, and 2 mg BID. The patient had significant motor improvement after IVIG treatment.\nAfter treatment with IVIG had concluded, polymerase chain reaction results from the CSF became available indicating a possible Borrelia burgdorferi infection in the CSF, 1.20 Lyme Index Value (normal high ≤0.99). This lab finding along with the recent history of travel to the Northeastern United States was highly suspicious for Lyme disease. Infectious disease was consulted. At this point, the patient had not regained full recovery of motor function and thus treatment for Lyme disease was initiated. The patient was given a 14-day course of ceftriaxone 2 g/day. Two days after initiation of ceftriaxone, the patient was discharged home with a peripherally inserted central catheter line to continue the antibiotic treatment. At the time of discharge, the patient was able to ambulate on her own, with only minor residual weakness in all limbs. She had regained her ability to speak and had only mild paresthesias in her lips. She was given instructions to continue physical therapy, follow up with infectious disease, and follow up with neurology for outpatient EMG/NCV. |
A 15-year-old boy who was treated for optic neuritis for 4 years was referred to our clinic on November 17, 2011, because the vision in his right eye was still poor in spite of steroid therapy. At the initial visit, the visual acuity of his right eye was counting fingers, and the critical flicker frequency and visual field were nonmeasurable. The relative afferent pupillary defect test was positive, and the pattern visually evoked potential (VEP; , upper half) was nonrecordable. The optic disc appeared slightly pale, and the fluorescein angiography showed hyperfluorescence of the disc. The left eye was completely normal. Tests for the aquaporin-4 antibody and mitochondria DNA 11778 were negative. The blood cell counts and serum biochemical findings were within normal limits. Computed tomography (CT) and magnetic resonance imaging (MRI) of the brain were also negative.\nWith a tentative diagnosis of right optic neuritis, we started steroid pulse therapy, but the visual acuity did not improve significantly. Humphrey visual field (HVF) 30-2 tests showed a central scotoma whose density increased with increasing time (). Subsequently, he complained of headaches and urinary incontinence. However, urological tests showed no abnormalities. He was regularly followed up in the neurological clinic.\nIn February 2013, he reported that he had severe headache with nausea, and he was referred to our Neurosurgery Department for a detailed investigation of the pituitary gland. The neuroimaging examinations showed a RCC (), and a microscopic transsphenoidal resection of the cyst was performed on March 26, 2013. Most of the specimens obtained by surgery composed of pituitary tissue with inflammatory cell infiltration. In a part of these specimens, epithelial cell linings which include goblet cells and microvilli were observed. These pathological findings were consistent with RCC.\nOn April 11, 2013, the right visual acuity had improved from 0.03 to 1.2, and no scotoma was found in the HVF. In addition, the VEPs returned to normal size and configuration (, lower half).\nHis vision has remained at 1.2 up to this final examination on January 12, 2014. |
A 62-year-old male was admitted to Shandong Provincial Hospital due to hematemesis and melena for 4 days. The patient also reported an 11-year history of mitral inadequacy, without chest pain or shortness of breath.\nThe patient had experienced melena for twice during the previous 4 years. Neither esophagogastroduodenal endoscopy (EGD) nor colonoscopy was conclusive.\nThe patient was severely anemic with an initial hemoglobin concentration of 3.9 g/dL. A complete physical examination revealed no pertinent findings. After blood transfusing with 4 U red blood cell and 500 mL serum, the patient underwent urgent EGD, which demonstrated curious dark blood in the second portion of the duodenum. We thus assumed that the bleeding lesion might locate at the duodenum or at the proximal loops of the jejunum. Colonoscopy was herein used and showed a 2.0 cm × 2.5 cm submucosal mass at the proximal loops of the jejunum. There was an ulcerative pit on the top of the mass, within which was an adherent clot (Fig. ). Fresh blood was extruded at the center of the pit when the patient felt nausea. The patient was then suspected as stromal tumor with active bleeding.\nConsidering that the patient still had massive bleeding and the blood pressure kept going down, urgent endoscopical sclerotherapy was performed. A 5 mL test dose of polidocanol was administered by injecting into the mass body with a 23 G injection needle, followed by 2 mL aliquots were injected circumferentially surrounding the center of the pit. The total volume of polidocanol was 15 mL. The sclerotherapy was done till the surface of the mass bulged and the active bleeding terminated (Supplementary Video). Restriction from oral intake was required for 24 hours after the sclerotherapy procedure. Red blood cell (2 U) was transfused in the operation. The hemoglobin level was determined again 6 hours after the treatment, and it increased to 8.1 g/dL, indicating the bleeding stopped successfully. The patient felt much better than before and was then transferred to the department of surgery. The hemoglobin level increased to 10.8 g/dL 5 days after the sclerotherapy.\nSeven days after the procedure, the patient received the surgery to remove the mass completely. The operation revealed a mass, locating at the jejunum, approximately 20 cm to Treiz ligament, without bleeding, which had already invaded to the greater omentum (Fig. ). The immunohistochemical studies showed positive staining of tumor cells for CD34, CD117, and Dog-1, and negative staining for smooth muscle actin (SMA) and S-100 protein (Fig. ). The mitotic activity was 1 mitosis/50 high-power field (HPF). These findings strongly supported a diagnosis of low risk of GIST. The patient was discharged at day 9 after operation.\nWritten informed consent was obtained from the patient, and the consent procedure and study protocol were approved by the Medical Institutional Ethical Committee of the Provincial Hospital Affiliated to Shandong University. |
A male patient aged 5-year-and-1-month was referred for genetic evaluation of development and speech delay, intellectual disabilities at the genetic counselling clinic in Shenzhen Maternal and Child Healthcare Hospital. The parents described that an affected brother also presented the same clinical phenotype but was not available for the clinical examination. The chromosome karyotype and chromosomal microarray analysis (CMA) in the proband were normal. The mother was pregnant again and pursued genetic counseling. The proband was subject to comprehensive neurological testing including the Gesell Developmental index. Molecular genetic tests and biochemical and neurochemical analysis were performed on the proband. The present study was approved by the hospital’s Institutional Review Board and written informed consent was obtained from their parents.\nThe proband was the second boy of healthy nonconsanguineous parents (pedigree in Fig. ). He was born at 39 weeks of gestation from an uneventful pregnancy and delivered by Caesarean section (weight, 3600 g; length, 50 cm; head circumference, 36 cm). He showed head control at 12 months, ability to sit by himself at 15 months, and walking with aid at 20 months. His verbal language was nearly absent and he made no visual contact. He suffered from seizures from 6 months old. He had no craniofacial dysmorphism. Gastrointestinal problems such as chronic constipation or nausea were noted in the proband. The physical examination on the proband showed 95 cm height, weight 18.2 kg and developmental and language delay. The proband also had an electroencephalogram (EEG) test, which showed sharp and slow waves in sleep during 24-h EEG monitoring. A brain stem auditory-evoked potential (BAEP) test showed mild abnormality. The proband had a Children’s Autism Rating Scale (CARS) score of 33, which indicated mildly autistic characteristics. The Gesell developmental scale test was used to evaluate the proband. Both the development age (DA) and developmental quotient (DQ) data showed extremely low grades which suggested severe development delay (adaptability, DA = 14.23mo., DQ = 23; gross motor, DA = 26.37mo., DQ = 43; fine movement DA = 15.87mo., DQ = 26; vocabulary DA = 13.07mo., DQ = 21; personal-social skill DA = 13.3mo., DQ = 22). The test results are depicted in Additional file : Figure S1A. The affected brother of the proband (II:1) was not available for the physical examination. The parental description of the clinical phenotype of the brother was mostly the same as the proband. The parents were physically healthy and indicated no significant past medical, surgical or family history.\nDNA samples were provided from the index patient and other family members, which were extracted as previously described []. The present study used the TruSight One Sequencing Panel and NextSeq 500/550 Mid Output v2 kit (300 cycles) with high depth of coverage for 4813 target genes (approximately 62000 target exons) that are associated with clinically relevant phenotypes. An average sequencing depth of 136.88x was achieved and 98.25% of targeted variants were covered at least to a 10x depth, and 97.04% of targeted variants were covered at least by 20x. The total detected variants numbered 24594, which included 21,733 SNPs, 1,182 insertions and 1,679 deletions respectively. The data were analyzed on the TGex (Translational Genomics Expert) platform featuring with the VarElect scoring system []. A missense mutation, c.1181C > A (p.Thr394Lys), in the SLC6A8 gene was called with high probability as a candidate mutation.\nSanger sequencing was performed to confirm the SLC6A8 gene c.1181C > A mutation (forward primer 5’ ACGGAACTTGTCAGATTGT3’, and reverse primer 5’CAACAGCATGAAGAAGAACA3’). The father (I:1) was wildtype and the mother (I:2) was heterozygous for the c.1181C > A variation. The affected brother (II:1) and the proband (II:2) both carried the hemizygous variation of c.1181C > A. The pregnant mother had an amniocentesis at 22 weeks and Sanger sequencing targeting the SLC6A8 gene c.1181C > A was performed. The result showed a wild-type allele (II:3) and the mother gave birth to a healthy baby girl (Fig. ). In silico variant prediction analysis methods, including SIFT, PolyPhen2, PROVEAN, and Mutation Taster demonstrated this variant had probably damaging or diseasing-causing effects.\nBiochemical screening was performed with blood and urine samples from the proband and his mother. The creatine/creatinine (Cr:Crn) ratio was determined by liquid chromatography-mass spectrometry with deuterated internal standards in two urine samples taken on different days. A urine creatine test of the proband showed significantly elevated levels of creatine (0.805 mg/ml, normal control 0.160 ± 0.177 mg/ml) (Additional file : Figure S1B), and the creatine/creatinine ratio was significantly elevated compared to controls. Proton magnetic resonance spectroscopy (MRS, Magnetom Skyra 3.0-T, Siemens Healthcare GmbH, Erlangen, Germany), examination using a 3.0-T system at the brain left parietal lobe, right parietal lobe and genu of corpus callosum all showed marked reduction of the brain creatine peak (Fig. left part). Brain MRI showed a thin corpus callosum in the proband (Fig. right part). The MRS and MRI examination of the mother (I:2) showed normal results (Additional file : Figure S1C). |
A 66-year-old woman presented at a hospital with chest pain. Her history included diabetes, hypertension, and hyperlipidemia. On examination, the patient had a pulse of 100 beats/minute and blood pressure of 150/80 mmHg. Her electrocardiogram, echocardiogram, and blood test results were normal. Multislice computed tomography (CT) showed a saccular LMCA aneurysm and significant stenosis in the LAD artery (Fig. ). Coronary angiography revealed a saccular LMCA aneurysm measuring 9.8 × 7.5 mm with 75% stenosis in the proximal portion of the LAD artery. The operation was performed under general anesthesia. A median sternotomy was performed, and after a longitudinal pericardial opening was made, the heart was inspected. The LITA was removed from the inner chest wall in a skeletonized fashion using electric cautery. A distal segment of 1.5–2 cm was procured and reserved for use as a patch repair. Before aortic cannulation, the ascending aorta was dissected from the pulmonary artery. Under cardiopulmonary bypass, coronary artery bypasses of the left internal thoracic artery to the LAD artery were constructed in the beating heart. After aortic cross-clamping, the LMCA saccular aneurysm was exposed without main pulmonary artery transection. The saccular LMCA aneurysm was carefully dissected and completely excised. There was no thrombus in the lumen. Then, the LITA was longitudinally divided and trimmed to fit the incised LMCA. The small internal thoracic artery patch was sutured to the normal and firm lateral coronary arterial wall with a continuous 7–0 Polypropylene suture. Resection of the saccular aneurysm and closure using a small internal thoracic artery patch was then complete. The aortic cross-clamp time was 120 min, and the CPB time was 147 min. The patient had an uneventful hospitalization and was discharged on aspirin therapy. Follow-up multislice CT 10 days after the operation revealed the complete disappearance of the aneurysm and a successful repair with no luminal stenosis by the internal thoracic artery patch. The LITA graft was also found to be patent (Fig. ). The patient has been followed up yearly since 2009. Fortunately, at the 9-year follow-up, the patient was still asymptomatic, and there were no changes in the ECG and UCG. The patient included in the follow-up had preserved preoperative left ventricular function, and there was no coronary incompetence. Pathology of the aneurysm revealed that the aneurysm wall was very thin due to a lack of trilaminar arterial structure from the remarkable atherosclerotic changes (Fig. ). |
A hypertensive and diabetic 72-year-old man with 3-vessel CABG surgery, SVG to the first diagonal branch of the left anterior descending artery (LAD) and to the obtuse marginal branch of the left circumflex artery (sequential grafting) and the left internal mammary artery (LIMA) to the distal LAD, performed 25 years earlier, presented at our hospital complaining of recurrent chest tightness over the past week. On physical examination, there were no remarkable findings. An electrocardiography showed a normal sinus rhythm at a rate of 78/min with a complete right bundle branch block, but no significant ST-T change was found compared with the findings of his previous electrocardiography. Regional hypokinesis of the post-lateral wall with moderate left ventricular dysfunction, which had not been previously confirmed, was also observed. Cardiac enzymes were not elevated. Coronary angiography showed the obstruction of SVG with a large thrombus burden (Fig. , Movie 1). Although LAD was chronically occluded from the ostium, there was no significant change compared with his condition 3 years ago. LIMA to LAD was patent, and other natives were the same as they had been 3 years ago. He was diagnosed with unstable angina due to acute SVG thrombosis. Instead of PCI, he was treated with apixaban 5 mg twice a day for 3 weeks in addition to aspirin 100 mg once a day, which was already being taken. We added only apixaban and not a P2Y12 inhibitor considering the high risk of bleeding and the mechanism of thrombotic occlusion. After coronary angiography had been performed and PCI was selected, we planned to load a P2Y12 inhibitor. The angiography 3 weeks after starting apixaban showed a considerable resolution of the thrombus and an opening SVG (Figure , Movie 2). The coronary flow of the final angiography almost reached TIMI grade 3 flow, and there was no significant stenosis in SVG. Therefore, we did not move on to PCI due to the fear of distal embolization. The rest of the patient’s stay in the hospital was uneventful, and he was discharged 1 week after the procedure. |
A 65-year-old male patient with non-ischemic dilated cardiomyopathy, left ventricular ejection fraction of 20%, intra-ventricular conduction delay with a QRS duration of 148 ms, and NYHA class 3 was scheduled for upgrade of an implantable cardioverter defibrillator (ICD) to a cardiac resynchronization therapy - defibrillation (CRT-D) system. The ICD had previously been implanted for primary prevention. Echocardiography prior to the BiV upgrade demonstrated myocardial scarring of the LV lateral wall and showed no evidence of mechanical dyssynchrony. Unfavorable coronary venous anatomy required epicardial LV lead placement in the lateral position via minimal lateral thoracotomy. Four weeks later, the patient was admitted with multiple ICD discharges due to incessant ventricular tachycardia (VT) (). He was sedated and Amiodarone was initiated, whereupon VT recurrences were no longer observed. In the following months his clinical condition deteriorated despite continuation of BiV pacing.\nAn invasive hemodynamic pacing study was performed to evaluate the hemodynamic effect of conventional BiV pacing and to explore whether LV endocardial pacing could effectuate hemodynamic improvement. For this purpose, a temporary pacing electrode and a RADI pressure wire were positioned within the LV cavity, another temporary pacing electrode was placed in the right atrium (). The RADI wire allowed determination of maximal rate of LV pressure rise (LVdP/dtmax ) as a measure of LV function. The pacing protocol consisted of a baseline without ventricular pacing (atrial pacing) alternated with AV-sequential BiV, LV and right ventricular (RV) pacing using the implanted system and LV endocardial pacing using the temporary LV pacing electrode. To exclude hemodynamic variability induced by spontaneous changes in heart rate, pacing was performed at a faster rate 10 beats above intrinsic heart rate. Average LVdP/dtmax was measured during 10 seconds of pacing for each pacing configuration.\nNeither LV, RV or BiV pacing with the implanted system nor LV endocardial pacing at different sites improved LVdP/dtmax as compared to baseline (). Furthermore, the number of premature ventricular complexes (PVCs) increased upon LV stimulation. This was confirmed by 24 hour Holter monitoring, which demonstrated 40,000 PVCs during BiV pacing versus 4000 PVCs when ventricular pacing was programmed off (). Considering the unfavourable hemodynamic and pro-arrhythmic effects of LV pacing, placement of a permanent endocardial LV lead was not performed and the ICD was programmed to ventricular pacing off (AAI-DDD mode). Our patient was scheduled for left ventricular assist device placement. |
A 12-yr-old female presented to our outpatient department with a right winged scapula. Her height was 139 cm and her weight was 38 kg. This condition abruptly developed after climbing a mountain for 2 hr with a rucksack/back-pack weighing about 20 kg at 2 months previous to her hospital visit. At a certain moment, she felt the drooping shoulder with a popping sound. She was born via spontaneous full-term vaginal delivery with a normal APGAR score. She had no specific family history of nerve or muscle diseases. Her growth and developmental history was nonspecific before the onset of winged scapula. Upon the physical examination, a winging deformity of the right scapula was noticed with the lateral deviation and upward rotation of the inferior angle, as observed on the erect neutral posture (). This abnormality was not detected when both shoulders were abducted (), but it was aggravated by shoulder flexion (). The motor and sensory functions of the upper extremities were normal and no side to side differences were revealed. The deep tendon reflexes of the biceps and triceps muscles were normoactive and symmetrical. No significant muscle atrophy in the shoulder girdles and the upper extremities was observed. On the laboratory tests, the routine complete blood count with differential counting, erythrocyte sedimentation rate, C-reactive protein, rheumatoid arthritis factor and urinalysis were normal, and the muscle enzyme tests, including serum creatine kinase, lactate dehydrogenase and transaminase, were also normal. Simple radiological studies, including the chest posterior-anterior view, both the shoulder anterior-posterior views and the scapular view, were nonspecific except for the slight scapular asymmetry.\nWe examined the nerves by conducting an electrodiagnostic examination that included the long thoracic nerve, the dorsal scapular nerve and the spinal accessory nerves and the muscles including serratus anterior, levator scapula, trapezius and rhomboideus and so on; there were no definitive evidences that indicated any neuropathy or myopathy on electrodiagnosis.\nWhen the pain and functional impairment persisted, surgical intervention then became appropriate. An explorative operation was done. A posterior longitudinal skin incision was made parallel to the vertebral border of the scapula. The subcutaneous tissue was divided. We can observed that the rhomboidius major was ruptured and trapezius lower thoracic bundle displayed thinning (). The right scapula was unstable to the chest wall. Rhomboideus major and trapezius muscle repair and reefing were performed. After the muscle repair, the scapula was stable to chest wall at the time of the operation.\nPostoperatively, she achieved the anatomical restoration of her right shoulder and she received rehabilitative treatment for 2 weeks including range of motion exercise of the shoulder and strengthening exercise of the rhomboideus muscles. On the follow up examination, at 2 months after the operation, there was no weakness or deformity of the affected shoulder and no pain nor limitation of shoulder motion. She was satisfied with the recovery of her shoulder function (). |
A 22-year-old right-handed Caucasian woman with a known diagnosis of SCA8 since the age of 10 years was admitted to our university medical center with encephalopathy and left-sided hemiparesis of unclear cause over the last 3 months. She had been diagnosed with SCA8 at 10 years of age after presenting with ataxia and gait difficulties that progressed rapidly. She was diagnosed at the Children’s Hospital of Nebraska after genetic testing confirmed the diagnosis. She continued to see the geneticists there for management of her condition. Her family was also tested and was found to be negative for genetic mutations, confirming the patient as the only affected family member, probably from a sporadic mutation. Neuropsychological testing was not performed at the time, but the patient’s family reported that she had an average IQ and was able to speak normally and perform daily functions without difficulty.\nThe patient’s physical examination on admission at our center revealed encephalopathy with left hemiparesis without obvious visual field deficits or other cranial nerve deficits. Magnetic resonance imaging scans revealed leptomeningeal contrast enhancement and edema over the right hemisphere (Fig. ). The results of lumbar puncture and resulting cerebrospinal fluid studies were unremarkable. A routine electroencephalogram (EEG) revealed independent slowing of both hemispheres, with the right hemisphere showing greater focal slowing and attenuation as well (Fig. ). Right posterior quadrant epileptiform discharges from an O2 electrode were occasionally seen in a quasiperiodic manner (Fig. ). Given these findings, the patient was started on levetiracetam therapy to treat potential epileptogenicity from the right posterior quadrant.\nTwo days later, she was noted to have frequent spells of confusion and decreased awareness. Owing to concern for ongoing seizures, she was connected to a long-term video EEG monitor for diagnosis. Video recordings captured multiple spells, each lasting 2–3 minutes, of loss of awareness with left gaze deviation and oromanual automatisms and staring with postictal lethargy and confusion consistent with clinical seizures. EEG captured posterior quadrant onset from both left and right hemispheres consistent with electroclinical seizures. A clear lateralization of onset was not seen with many of these seizures, owing to rapid bilateral involvement of both posterior quadrants (Figs. , , and ). Many of these seizures occurred frequently over a 2–3-hour period, meeting criteria for status epilepticus. She was started on lacosamide therapy, and her dose of levetiracetam was increased. Her seizures resolved within a few hours of increasing her antiepileptic therapy.\nHer family revealed that she had been having similar spells since the age of 12 years and that they had witnessed at least 15–20 similar spells in the past that were not previously recognized as seizures. Many of these spells were associated with nausea and vomiting, findings that were not captured on our video EEG recordings.\nShe continued to show improvement in mental status, and her left hemiparesis showed progressive improvement over the next few days with antiepileptic therapy and physical therapy. She was discharged to a rehabilitation facility on lacosamide and levetiracetam, continues to return for follow-up as an outpatient, and is currently doing well without any further seizures. When she was seen 3 months after her admission, her left hemiparesis had improved, and she had returned to her previous mental baseline. |
A 12 years old male patient presented with a swelling on the medial aspect of the right thigh. There was complaint of pain in the right thigh for the last one week with history of fever since three days. There was also complaint of difficulty in walking. No other relevant history was present. Patient was totally vegetarian. On local examination, temperature was raised with tenderness on the right thigh. There was a single swelling of size approximately 4×5 cm in the right medial aspect of thigh (). Multiple small tiny spots were present over the thigh. There was difficulty in extension of thigh of about 20 degree. Rest of the clinical examination was normal. Routine investigations were within normal limits. Patient was sent for the ultrasonography of the whole abdomen and thigh.\nUltrasonography (USG) was done on Logiq 500 Pro machine (GE Medical Systems, USA) with convex probe at 3.5 MHz frequency and with a linear probe at 9.6 MHz frequency. On ultrasonography there was a well defined isolated cystic lesion of size 3.3 × 2.5 cm intermuscular area (). There was small cystic lesion in the right vastus medialis muscle with surrounding inflammatory phlegmon of 4 × 3 cm. and leaked echogenic scolex was also seen with linear probe. Therefore, on sonography, the diagnosis of cysticercosis in the right vastus medialis muscle was made with surrounding inflammatory phlegmon (). Rest of the abdomen was normal. The patient was managed conservatively with short course of tapering steroid, prednisolone 2mg/kg/day and on tablet albendazole 15mg/kg body weight/day for 21 days. After three weeks of conservative treatment, on follow up, pain and tenderness completely disappeared and patient was well. On follow up sonography, there was no evidence of phlegmon or cysticercosis in thigh. Patient was followed again after three months and there were no complaints.\nDiagnosis made as cysticercosis in the right vastus medialis muscle of size 3.3 × 2.5 cm with surrounding inflammatory phlegmon. |
A 28 year old woman presented in year 2000 with an ischiorectal abscess at age 17. This was drained after which she developed a fistula, which was managed with a cable tie seton before resolving. She re-presented in 2002 with obstructive symptoms and a small bowel series revealed stenosing ileocaecal Crohn’s disease. From 2002 and 2008 she was managed with azathioprine 150 mg PO daily with intermittent steroid courses and one induction dose of infliximab (prior to funded access to therapy).\nIn June 2008, she was commenced on induction and maintenance therapy with adalimumab for recurrent perineal fistulae, remaining on concomitant azathioprine 150 mg PO daily. In Feb 2009, she ceased azathioprine as she was in clinical remission. However in June 2009, a flare requiring a course of prednisolone occurred and azathioprine 150 mg PO daily was recommenced. Prednisolone was ceased, but she remained mildly symptomatic. Metabolite testing at this time revealed a 6TGN of 181 and a 6MMP of 4558 (6MMP:6TGN ratio of 25). Her dose was gradually increased to azathioprine 225 mg PO daily, however, repeat testing revealed 6 TGN 253 and 6 MMP 8093; a ratio of 32, consistent with shunting. Whilst liver function tests, iron and inflammatory markers were all normal, the patient complained of lethargy and nausea. She was placed on concomitant allopurinol 100 mg PO daily and the azathioprine dose was reduced to 50 mg PO daily, together with continued adalimumab. A repeat metabolite testing revealed 6TGN of 493 and 6MMP of < 250 with resolution of nausea and lethargy. In March 2011 she reported feeling the best she had ever felt. She subsequently conceived in October 2011. She decided to cease adalimumab at that stage, but remained on azathioprine and allopurinol throughout her pregnancy with no further flares. She delivered a healthy baby boy by elective caesarean section at 38 weeks, and has remained in clinical remission on last review in September 2012. |
A 47-year-old man complained of scapular crepitation whenever he moved his shoulder. He had a motorbike accident 28 months prior that resulted in a claviclasterum dislocation that was treated with open reduction. The sound became increasingly irritating over time, with no improvement despite the use of medications and physical therapy. He was stressed because of the noise and was unable to carry out his daily activities. The physical examination revealed abnormal scapula motion accompanied by snapping sounds during active shoulder movement. CT scans reveal an abnormality in the scapular superomedial corner () Massive bursitis and thickness of the superomedial scapular corner can also be seen on MRI ().\nUnder endotracheal anesthesia, the patient was set up in the chicken-wing position . The anesthesia machine and its tubes were put at a position that allowed the robot arm to rotate 360 degrees around the operating table ().\nAfter calibrating the robot system with the operating table, the patient was covered. The following 2 portals were used in our procedure: ()Portal 1: between the scapular spine and the inferior angle of the scapula Portal 2: at the level of the superomedial angle (Ejnisman portal) ,\nThese portals were located 2 cm medial to the medial margin of the scapula.\nThe arthroscopic probe was inserted through the Ejnisman portal to dissect layers. The serratus anterior muscle served as the roof of this cavity and was also the first anatomical landmark used for orientation. The posterior chest wall, the ribs, and palpable intercostal spaces comprised the floor of this space. We found the serratus anterior space filled with fibrous tissue ().\nAfter the debridement of inflammatory tissues was carried out, we revealed the abnormal superomedial angle (). Two needles serving as landmarks for the inferior margin and lateral margin of the intended resection zone were inserted under arthroscopic visualization.\nA 3D-CT scan of the scapula was performed with Artis Pheno Robot (Siemen) after covering the robot arm (). Another surgeon from outside assisted with the 3D reconstruction of the scapula and checked the position of the needles in relation to the margin of the intended bone area for resection. We aimed to make the distances between the suprascapular notch and the lateral edge of the resection about three centimeters and the distances between the scapular spine and the inferior edge of the resection about two centimeters (). Then, the two marked needles were repositioned to the proper position.\nThe scapuloplasty procedure was then carried out by using an acromionizer within the level of these needles. It is necessary to thoroughly clean the cavity then, leaving no debris left.\nFollowing the scapuloplasty, a second 3D CT scan should be conducted to ensure that the proper amount of bone was removed (). Some minor repairs with acromionizer could be required to removed neglected debris.\nFor comfort, the patient was placed in a sling for two weeks. Maximal protection passive range of motion was performed immediately following surgery. He had a smooth recovery and was back to his normal activities within a month of surgery. After 16 weeks, the patient resumed sports activities. There was no recurring symptoms at the 6-month follow-up (). |
A 72-year old woman was admitted to our hospital with palpitations, dizziness and fatigue from a few hours ago. The patient had a history of heart failure (NYHA II) from three years ago and also an episode of paroxysmal atrial flutter two years ago. She was free of angina and had no cardiovascular disease risk factors. The family history was unremarkable with respect to cardiac arrest, unexplained syncope, ventricular tachyarrhythmias, or cardiomyopathy. At the time of presentation to the emergency department the patient was receiving carvedilol, valsartan, and furosemide. The baseline 12-lead ECG revealed a sustained monomorphic VT with RBBB morphology and left axis deviation (Fig. ). Since tachycardia was poorly tolerated with systolic blood pressure of 80 mmHg an electrical cardioversion was applied and the rhythm was restored to sinus (Fig. ). The ΕCG, physical examination and laboratory tests after the cardioversion were unremarkable.\nThe chest X-ray revealed an increased cardiothoracic index. The transthoracic echocardiogram showed dilated left ventricle with a lateral wall aneurysm, left ventricular ejection fraction of 40% and mild mitral regurgitation (Fig. ). The coronary angiography revealed normal coronary arteries, whereas the left ventriculography further confirmed the lateral wall aneurysm (Fig. ). Magnetic resonance imaging further confirmed the presence of the LVA (Fig. ).\nThe patient was started on amiodarone per os and after two weeks an electrophysiology study was performed. The programmed ventricular stimulation in the right ventricular apex with 500/230-240 msec basic drive cycle and two extrastimuli reproducibly induced sustained monomorphic VT of 160 bpm associated with hemodynamic instability. That tachycardia, however, had different characteristics from the one at the emergency department as it was associated with left branch bundle block (LBBB) morphology and normal cardiac axis and was terminated by overdrive pacing (Fig. ). The LBBB morphology was consistent with septal origin of the VT. Of note, during the inducible VT a 1:1 ventriculoatrial conduction with intermittent Wenckebach block was recorded (Fig. ). A cardioverter defibrillator (ICD) was implanted for the secondary prevention of VT and the patient was discharged 2 days after ICD implantation on treatment with carvedilol, amiodarone and angiotensin converting enzyme inhibitor. During a follow-up period of six months no ventricular arrhythmias occurred. |
The 62-year-old German Caucasian woman presented with a nonresectable adenocarcinoma of the right lower lobe with infiltrated lymph nodes of the mediastinum and malignant pleural effusion, staged by tumor size and dissemination (T3N3M1a) as IV.\nThe first CT scan 12 months AD showed a stable disease; a positron emission tomography/computed tomography (PET/CT) scan 20 months AD exhibited a marginal but definite progression of the disease, disintegration of the central lymph nodes without enlargement, pleural effusion but no distant metastases. A PET/CT scan 26 months AD, performed because of swallowing difficulties, revealed an enlarged lymph node that had caused a severe obstruction of the esophagus, which required treatment with a stent. A histological analysis revealed tumor infiltration, which had caused the lymph node enlargement. Neither a second radiation nor chemotherapy was performed.\nThe patient received a combined treatment of radiation and chemotherapy for three months starting two weeks AD. Four cycles of cisplatin and pemetrexed were administered together with a total of 64Gy.\nACT with the CAPRI cells started after radiation and chemotherapy, that is, three months AD (Figure ). In the first six months, the patient received 40 to 60 million CAPRI cells twice weekly; after improvement of the cell count, the patient received 80 million CAPRI cells twice weekly, two-thirds intravenously, one-third intracutaneously, with a total of 260 injections over a period of 35 months. The growing pleural effusion, diagnosed by PET/CT scan 26 months AD, was first treated for several months with thoracentesis and later successfully combined with thoracic CAPRI cell injections eight times using 100 to 300 million immune cells. After the first thoracic injection of 300 million CAPRI cells, the patient felt very weak and had a low-grade fever for two days. Consequently, the dose of CAPRI cells was reduced to 100 million, which was well tolerated. The patient is still alive after 40 months (Figure ). |
A 55-year-old woman visited the emergency clinic of Hanyang University Seoul Hospital with chest pain that had persisted for one month and had become aggravated two days earlier. Ten years before this admission, the patient had undergone double valve replacement with aortic and mitral mechanical valves due to aortic regurgitation and mitral steno-insufficiency.\nAn electrocardiogram revealed myocardial ischemia in the inferior wall. However, no abnormalities were observed in laboratory findings, including cardiac markers. Echocardiography revealed normal motion and function of the mitral and aortic valve prostheses, and coronary angiography findings were also normal. However, coronary computed tomographic angiography revealed three large lobulated aneurysms with calcified walls bulging from the base of the left ventricle (). The leak in the left ventricle was located in the sub-mitral valve prosthesis area. Based on this finding, we diagnosed the patient with a pseudoaneurysm of the left ventricle, and surgical treatment was planned.\nSurgery was performed under general anesthesia using double-lumen endotracheal intubation. A left lateral thoracotomy was performed via the fifth intercostal space. Arterial cannulation via the left femoral artery and venous cannulation via the left femoral vein and left pulmonary artery were performed. Three communicating aneurysmal sacs were observed in a single plane, identical to that observed in coronary computed tomographic angiography. The neck of the first sac was located at the base of the left ventricle. Under cardiopulmonary bypass and fibrillation, the aneurysmal sacs were incised and the thrombi in the sacs were extirpated. A defect measuring 15 mm in diameter was identified in the sub-mitral left ventricular wall with calcification but with no infection. The pseudoaneurysm of the left ventricle was determined to be caused by myocardial discontinuity. Closure of the defect was performed with a Supple Peri-Guard Pericardium Patch with APEX Processing (Synovis, St. Paul, MN, USA) using pledgeted horizontal mattress sutures. The walls of the aneurysmal sacs were closed, and the patient was weaned from cardiopulmonary bypass without difficulty. The total cardiopulmonary bypass time was 70 minutes.\nThe vital signs of the patient remained stable, and she was extubated six hours after the operation. Follow-up echocardiography was performed on postoperative day eight, and no abnormal findings, including cardiac wall motion, were noted. The patient underwent coronary computed tomographic angiography on postoperative day 14. No leakage at the closed aneurysmal neck was observed, and there was no evidence of recurrence of the pseudoaneurysm (). The patient was discharged on postoperative day 16, without any complications. She has remained disease-free for six years postoperatively, with regular Coumadin anticoagulation therapy due to the presence of the prosthetic aortic and mitral valves. |
A 20-year-old previously healthy man, a student by occupation and a non-smoker not on any medications, presented to his primary care physician with a history of swollen glands for a 'couple of months'. On further review it was found that for one month prior to presentation, our patient had noticed multiple enlarged cervical, occipital, and right inguinal lymph nodes. No constitutional symptoms were reported. Our patient was of Middle Eastern heritage, but was born and raised in the USA. He had not travelled recently, nor had he had any recent contact with sick people or any occupational exposure. On physical examination, our patient was afebrile with normal vital signs. Enlarged, non-tender, freely mobile bilateral cervical and occipital lymph nodes were palpable and measured up to 4cm. His right inguinal lymph nodes were similarly enlarged. The left palatine tonsil was slightly erythematous and enlarged. A monospot test was negative for Epstein-Barr virus infection. Given these findings, the primary care physician prescribed a course of antibiotics for a possible infectious etiology consisting of a three-day course of azithromycin followed by amoxicillin-clavulanate one week later due to persistent symptoms. Initial investigative tests showed normal blood counts and serum electrolytes. An HIV antibody enzyme-linked immunosorbent assay (ELISA) test was also negative.\nOur patient returned to the clinic for re-evaluation. With the exception of the enlarged lymph nodes, he remained otherwise clinically asymptomatic. On physical examination, the lymph nodes appeared unchanged, and there were no newly involved nodal chains. Upon more thorough investigation, our patient indicated that approximately once month ago he ate raw kibbe, a Middle Eastern dish that consists of spiced uncooked beef or lamb with grains. Additional laboratory studies were ordered and are listed in Table . Our patient was diagnosed with acute toxoplasmosis and counseled regarding dietary habits and risk factors. No specific treatment was administered, and close follow-up was planned to ensure resolution of the lymphadenopathy. |
A 53-year-old Egyptian female with past medical history of hypertension with nephropathy leading to end stage renal disease, one month after the start of hemodialysis, presented with fever and shortness of breath at an Egyptian hospital. Blood cultures grew Staphylococcus aureus and the sepsis was treated aggressively with piperacillin with tazobactam and Imipenem. She was transported from Egypt for further treatment in the United States.\nOn presentation at the referral center the patient was found to be in septic shock.\nHer blood pressure on admission was 73/41 mm of mercury, with a heart rate of 120 beats per minute and a respiratory rate of 22 breaths per minute. Her white cell count on admission was 9,400 cells/μL; the differential count included 97.9% neutrophils with 2% lymphocytes. C-reactive protein on admission was 4.7 mg/dL. She was hypotensive and tachycardic. She had vesiculobullous lesions on the nose (), forearms, and the feet (). Some of the lesions desquamated to leave ulcers. She had a previously placed left sided internal jugular central line for hemodialysis. This central line was immediately removed on presentation and a fresh dialysis catheter was placed. Blood cultures grew Candida albicans on day 1 and continued to grow in all blood culture bottles consistently, during her stay. Appropriate investigations for infective endocarditis were performed.\nA transthoracic echocardiogram revealed bulky leaflets of the mitral valve with a highly mobile vegetation about 2.3 cm long attached to the anterior leaflet (). This vegetation was prolapsing into the left atrium and was causing moderate mitral regurgitation. Computed tomogram (CT) scan of the chest, abdomen, and pelvis was also performed. It showed bilateral pleural effusions in the chest, with triangular opacities in the lungs suggestive of infarcts (). There was mild splenomegaly with triangular hypodensities consistent with splenic infarcts (). A CT scan of the abdomen and pelvis was found to appropriately visualize the renal system; there were atrophic kidneys bilaterally, with no evidence of stones. The bladder was collapsed on the scan.\nDespite initiating parenteral antifungal therapy, the patient deteriorated over the course of 5 days. Her disease progressed to cause multiple organ failure and she was placed on palliative care due to grave prognosis and to honor the family's wishes. She died due to a cardiac arrest. |
A 36-year-old patient, gravida 11 para 6, with an ultrasound diagnosis of “missed miscarriage”, presented to our hospital with lower abdominal pain. She was 18+ gestational weeks by the last menstrual period (LMP) and 15+ gestational weeks by the ultrasound. Her previous obstetric history was significant for five lower segment cesarean sections (LSCS), four miscarriages, and one uterine rupture.\nIn her fifth pregnancy, she had a fetal demise at ~24 weeks of gestation, which was medically terminated. The patient was induced once; however, details of the medical induction were not available to review. The pregnancy termination was complicated by uterine rupture, which was managed by laparotomy, hysterotomy, and uterine rupture repair. Two of the five LSCS were done electively following the uterine rupture repair. The patient was healthy otherwise.\nOn physical examination, the patient was vitally stable, and the abdomen was soft with no tenderness on palpation. Pelvic transabdominal ultrasound was requested to rule out scar rupture. The myometrium–bladder interface was preserved, and scar thickness was 5 mm, which was normal for a previous cesarean section., All laboratory findings were within the normal ranges.\nConservative, medical, and surgical management options and the associated risks and benefits were discussed with the patient. She opted for medical management. Accordingly, a low-dose misoprostol of 100 µg was inserted vaginally in the posterior fornix, and 6 hours later, a repeat dose was given. Two hours after the second dose, the patient started to have pain and contractions. She was vitally stable. Her vaginal examination revealed an opened cervical os, and tissues were felt in the vagina; hence, the patient was shifted to labor and delivery suite for monitoring. After receiving adequate analgesia, a pelvic transabdominal ultrasound was done for scar thickness, which showed a thinning out of the myometrium at a scar site measuring 2 mm. Two hours later, the fetus was aborted that weighed 60 g, and 20 units of oxytocin infusion was started. One hour later, the placenta was delivered completely. The estimated blood loss was 180 mL. The patient was vitally stable all through with no extra bleeding.\nThe following day, the patient was stable with minimal vaginal bleeding and the repeated complete blood count (CBC) was normal; a prophylactic antibiotic amoxicillin–clavulanate 625 mg every 8 hours orally for 7 days was started. On Day 2 post miscarriage, the patient was discharged home with a follow-up appointment. |
A 74-year-old male with super obesity (BMI of 59.9) presented to an outside facility with right upper quadrant pain that began two days prior. He had the following comorbidities: type II diabetes mellitus, coronary artery disease, congestive heart failure with ejection fraction of 40%, atrial fibrillation on long-term dual anticoagulation, sick sinus syndrome requiring implanted pacemaker, asthma, pulmonary hypertension, wheelchair dependence, and a 20 pack-year smoking history. Initial evaluation revealed leukocytosis and computed tomography scan findings of a distended gallbladder with inflammatory changes concerning for acute cholecystitis. Intravenous antibiotics were started, and the patient was transferred to our facility for further surgical evaluation and intervention.\nA Hepatobiliary Iminodiacetic Acid (HIDA) scan was performed which revealed acute acalculous cholecystitis (). A percutaneous cholecystostomy tube (PCT) was placed by interventional radiology. The patient was discharged to home with planned interval cholangiograms and PCT checks. Subsequently, the patient had complications with his PCT including misplacement, occlusion, and pain. Three months after discharge, a cholangiogram demonstrated persistent cystic duct obstruction. The patient expressed interest in PCT removal and a cholecystectomy.\nPreoperative evaluation by pulmonology revealed undiagnosed obstructive sleep apnea (OSA) and determined that the patient had an intermediate increased risk of perioperative complications. Evaluation by cardiology found that the patient had a moderate increased risk for cardiac complications with a laparoscopic cholecystectomy and a moderate to high risk with a laparotomy. After six weeks of appropriate therapy for OSA, the patient underwent a robotic assisted laparoscopic cholecystectomy.\nFour robotic ports and two laparoscopic ports were placed (). Dissection began with limited workspace due to patient body habitus and was further restricted by significant scarring surrounding the gallbladder. This workspace challenge was ameliorated after suspending the falciform ligament with a transfascial suture. Dissection exposed the cystic duct and cystic artery. Near infrared fluorescence cholangiography (NIRF-C) with injection of indocyanine green dye was performed to confirm the location of the cystic artery (). The critical view of safety was obtained. The cystic artery and duct were clipped and divided, and the anterior wall of the gallbladder was removed along with the remaining gallstones. Due to significant inflammation, the posterior wall of the gallbladder was unable to be removed safely from the liver bed. The posterior gallbladder wall was cauterized to reduce the chance of bile leak, and a drain was placed in the gallbladder fossa. The patient did well postoperatively, was discharged home postoperative day one, and recovered without complications. Pathology revealed severe acute on chronic cholecystitis with cholelithiasis. |
A 71-year-old man with progressive angina was found to have severe stenosis in the circumflex artery and complex CTO of the left anterior descending artery (, Supplementary ). Coronary artery bypass surgery was offered to the patient but declined. He underwent uncomplicated stenting of the circumflex artery, followed by staged CTO recanalization of the LAD. A guidewire was passed relatively easily across the occlusion into the true lumen of the distal LAD beyond the bifurcation. Antegrade wiring of the diagonal artery was difficult. An epicardial collateral from the distal right coronary artery was used to access the diagonal branch retrograde, and this wire was steered into the antegrade guide catheter and externalized (). Both the LAD and diagonal were dilated with 3.0 mm noncompliant balloons. The diagonal was stented into the proximal LAD, and the distal LAD was rewired. The distal LAD was dilated through the stent struts to allow passage of stents into the distal LAD (Culotte technique). The distal LAD was stented using four everolimus drug-eluting stents. The stents were postdilated with 3.0 mm noncompliant balloons in the diagonal and distal LAD and 4.0 mm noncompliant balloon in the proximal LAD. Within the distal-most stent in the distal LAD, the balloon had a persistent waist until an inflation pressure of 12 ATM. At that point, the balloon suddenly expanded. Angiography revealed contrast filling of the right ventricle (RV), with the appearance of one or two focal jets of contrast extravasation at the location of the rigid lesion in the distal LAD, consistent with iatrogenic LAD-RV fistula (, Supplementary ). Of note, the flow beyond the stents in the distal LAD was not seen, likely due to shunt flow and “coronary steal.” The patient remained hemodynamically stable and was asymptomatic, and it was elected to manage the fistula conservatively. Serial echocardiograms revealed only trace pericardial effusion. He was discharged in stable condition.\nApproximately three weeks after the CTO procedure, the patient was presented to the emergency department with chest pain and dyspnea. He described several episodes of “tearing” sensation in the chest. Blood pressure and heart rate were 123/69 mmHg and 64 beats per minute, respectively. A 12-lead ECG revealed inferior T wave inversion. Troponin I was elevated to 0.06 ng/mL. Urgent coronary angiography was performed, which revealed patent stents in the proximal LAD and diagonal branch. LAD-RV fistula appeared relatively unchanged compared to during the CTO procedure (). The right coronary artery was normal and provided a very faint collateral to the apical LAD. Left ventriculography in the left anterior oblique projection revealed no evidence of a ventricular septal defect. The patient developed profound hypotension of unclear etiology during angiography. A right heart catheterization revealed normal filling pressures, normal cardiac output and ratio of pulmonic to systemic flow (Qp:Qs) 1.7. A transthoracic echocardiogram revealed preserved ejection fraction and normal left ventricular wall motion with turbulent flow signals at the LV and RV apex throughout the cardiac cycle (Supplementary ). There was no pericardial effusion. The patient was transferred to the cardiac intensive care unit in stable condition.\nHeart team evaluation was undertaken, and it was decided to perform coil embolization of the distal LAD. A standard 6 French left coronary guide catheter was used to advance a ProGreat (Terumo Medical Corp., Somerset, New Jersey) guidewire and 2.8 French catheter into the distal LAD. This was used to deploy two Ruby (Penumbra, Inc., Alameda, California) coils at the distal end of the stent.\nThere was still persistent shunt flow, so two Tornado (Cook Medical, Bloomington, Indiana) coils were subsequently deployed. Angiogram of the LAD confirmed complete cessation of flow into the distal LAD and absence of shunt flow (, Supplementary ). Hemodynamics postprocedure demonstrated a 20 mmHg increase in systolic blood pressure and normalization of Qp:Qs. A follow-up echocardiogram revealed the obliteration of apical shunt flow, normal left ventricular ejection fraction, and no left ventricular wall motion abnormalities. Creatine kinase eight-hour postprocedure was normal. The patient was discharged home in stable condition. At follow-up, one month later, the patient remained asymptomatic. |
This is a case of a 30 year-old gentleman with a history of Crohn's disease. He was on a regimen of infliximab, infused every 8 weeks and oral methotrexate daily. He had no other significant medical history. He was in his usual state of health until he developed a sore throat and fevers on day 0. His symptoms began while he was traveling in Europe. During his trip, he took a 10-day course of amoxicillin and felt some improvement.\nAfter returning to the US he felt well but on day 14 he developed fevers and sore throat again. He was evaluated at an urgent care clinic and sent home with a diagnosis of a viral syndrome and instructed to treat this with non-steroidal anti-inflammatory agents. He felt some improvement initially but presented again on the day of admission with concern that he may not be well enough for his infusion of infliximab. He was found to have a temperature of 38.3 C and on day 30 was sent to the emergency department for further evaluation.\nIn the emergency department, he reported a non-productive cough, fevers, and sore throat. He had elevated liver enzymes: aspartate aminotransferase (AST) 340, alanine aminotransferase (ALT) 540, alkaline phosphatase 145. Additional testing was sent including a Monospot test, cytomegalovirus (CMV) and Epstein's Barr virus (EBV) serum viral levels, respiratory viral panel by PCR, adenovirus serum viral level, HIV antibody/antigen as well as HIV viral level, viral hepatitis serologic panel, human herpesvirus type 6 (HHV6) serum viral level, Varicella zoster virus (VZV) serum viral level, and syphilis IgG. He was admitted to the inpatient medicine service for fevers and hepatitis of unknown origin. His social history was remarkable for frequent trips to the Midwest for work. He did not pursue outdoors activity while traveling. He did not smoke, drink alcohol or use illicit drugs.\nOn day 31 he developed daily fevers to 40 C and subsequently progressed to hypoxemic respiratory failure that required high-flow supplemental oxygen and transfer to the intensive care unit. He had a chest CT showing ground-glass opacities at the lung bases and a left upper lobe nodular opacity (, ). His CT also demonstrated tonsillar enlargement and splenomegaly. He had a laryngoscopy performed that revealed an exudative pharyngitis. He was noted to have atypical lymphocytosis that peaked to 12,000 cells/uL on day 33. He was started on broad-spectrum antibiotics for presumed hospital-acquired pneumonia; including coverage of atypical organisms. He underwent a bronchoscopy on day 33. Bronchoalveolar lavage (BAL) fluid was negative for Legionella culture, AFB stain and culture, pneumocystis stain, and bacterial culture. Mycoplasma, zygomycetes and Mycobacterium tuberculosis were not detected by PCR. A respiratory viral panel by PCR from the lavage fluid was positive for Bocavirus and one of 2 samples for aspergillus galactomannan was moderately elevated but later determined to be negative on repeat testing.\nOn day 33 his AST peaked at 351 and his ALT at 662. His viral studies sent earlier returned negative. He had further serologic testing for atypical organisms including Blastomyces, Coccidioides, Q fever, Bordetella pertussis, as well as urine Legionella antigen that all returned negative. He was started on methylprednisolone and his fevers improved. His antibiotics were discontinued when his bacterial cultures were negative at 48 h. On day 37 the fungal PCR from BAL returned positive for Histoplasma capsulatum and cultures from that fluid later grew the organism. Urine Histoplasma antigen testing, sent earlier during his hospitalization, ultimately returned with a titer of 2.16 ng/mL (normal high 0.1 ng/mL). His Coccidioides antibody was also positive, but this was felt to be due to cross-reactivity from his Histoplasma infection.\nOn day 37 he was started on liposomal amphotericin B and 6 h later he had his highest fever to 42 C along with hypotension, tachycardia and worsening hypoxemia despite having received a dose of methylprednisolone earlier that day. He was felt to have a paradoxical worsening with treatment and exacerbation of immune reconstitution inflammatory state rather than an infusion reaction due to amphotericin given this time course . His infliximab was restarted the following day with subsequent resolution of his fever, hemodynamic instability and improvement in his respiratory status within hours of this infusion. He continued amphotericin B for one week before transitioning to oral itraconazole. His ALT/AST improved significantly. He was discharged on oral itraconazole with continued clinical improvement and recovery as an outpatient. |
The patient was an 81-year-old Caucasian woman with a history of persistent right-sided flank pain with no associated symptoms including dysuria, hematuria, frequency, or urinary retention. She had no history of prior abdominal surgery, trauma, or congenital defects. Given the unclear etiology, we obtained cross-sectional imaging which demonstrated a right-sided Bochdalek diaphragmatic hernia incarcerating her proximal ureteral segment (Fig. ). Initially, she was managed conservatively with surveillance monitoring since her pain was tolerable and her renal function was preserved. Although her kidney function remained unchanged, follow-up imaging at the next visit surprisingly demonstrated an interval increase in hydroureteronephrosis (Fig. ). Additionally, the entrapped ureteral portion had progressed with now obvious hydronephrosis of the renal pelvis. A Tc-99m MAG-3 nuclear medicine renal scan with furosemide confirmed our suspicion – she had moderate obstruction of the right kidney. This study also demonstrated an asymmetric split function with 64% left and 36% right in the setting of a stable baseline creatinine (0.7mg/dL).\nIn attempt to reduce the entrapped ureter and unobstruct the right kidney, a double-pigtail ureteral stent was endoscopically placed. We were able to reduce the herniation with the use of a super stiff wire and a standard soft stent was placed in the usual retrograde fashion. During her stent exchanges, it became apparent that the herniated ureteral segment was enlarging as the curl of the double-J stent retracted into the distal ureter requiring ureteroscopy to visualize and engage. Longer stents were used to ensure that the distal curl remained in the bladder lumen despite recurrent herniation. Stiffer stents were also used however these maneuvers eventually were no longer able to straighten and reduce the ureter, leaving it entrapped in the thoracic cavity. After several stent exchanges were completed, the patient became symptomatic with ureteral stent discomfort including flank pain, urinary frequency and urgency. Due to concern that her diaphragmatic defect was enlarging, we discussed definitive surgical options with her; nephrectomy versus resection and anastomoses of her right ureter with diaphragmatic hernia repair. She ultimately opted to preserve her right kidney and proceed with open-resection reduction and repair.\nWe approached her open surgical repair through a standard supra-11 incision carried down through her flank muscles where we entered the retroperitoneal space. First, we freed her affected kidney from the surrounding tissue and then identified the incarcerated ureteral segment using our previously placed stent. The redundant ureter was mobilized out of the diaphragmatic defect and returned to the retroperitoneal abdominal cavity. We closed her diaphragmatic defect using interrupted 0 silk sutures, and then excised the redundant proximal ureteral segment where we spatulated and reapproximated the proximal and distal ends. Last, we replaced the patient’s ureteral stent and completed the anastomosis over a 7-French x 26 cm double-J ureteral stent. Due to the dissection of the renal hilum and perinephric tissue, the kidney appeared to have more mobility. To help maintain proper drainage and reduce tension on the anastomosis, we performed a nephropexy, securing the posterior aspect of the kidney to the flank muscles with a 0 silk. From there, we performed the standard two-layered incision closure.\nThe patient had an uncomplicated post-operative course and was discharged home after regaining bowel function and returning to her baseline physical activity on post-operative day three. There were no complications during surgery or recovery. Two months later, we removed her ureteral stent and obtained follow-up imaging which demonstrated repair of the diaphragmatic defect without hernia recurrence (Fig. ). We also repeated the Tc-99m MAG-3 nuclear medicine renal scan, which now demonstrated normal clearance of the previously obstructed right kidney. Post operative creatinine was 0.7 mg/dL with no obstruction detected. Split cortical function was 54% left and 46% right; an improvement the patient’s preoperative findings. |
A 44-year-old woman without any specific medical history presented to the emergency room of our hospital after a snake bite, (Agkistrodon ussuriensis, Chinese viper) near the right lateral malleolus, in a parking lot three months before being referred to the pain clinic. At that time, the patient complained of severe edema up to the right femoral region, acute pain, abdominal pain, muscle pain, double vision, dizziness, and nausea. Agkistrodon halys antivenin (6,000 IU) was intravenously injected, followed by the administration of antitetanus immunoglobulin, an antihistamine, a steroid, and antibiotics. At the time of the injury, the Visual Analogue Scale (VAS) pain intensity score was 100/100 (the patient felt as if her foot was exploding). The score gradually reduced up to 4 days after the injury as the swelling decreased.\nWhen the patient was referred to our pain clinic during the 3rd month after the injury (), she had static allodynia on the 2nd, 3rd, and 4th digits of the right foot along with repetitive pain that felt like needle stabs. The patient also exhibited hyperalgesia in the entire foot as well as constant tingling sensation the upper lateral malleolus. The pain worsened during walking; she experienced stiffness and edema with a VAS intensity of approximately 50/100 on the lateral sole and the top of the foot in addition to pain in the foot at the slightest exertion, such as walking up or down the stairs. The patient felt a sickly cold sensation in the heel that was alleviated by using a hot or cold pack. An electrophysiologic study performed at the end of 1st month after the injury indicated findings suggestive of damage to the right superficial peroneal nerve or around peripheral nerve; however, no abnormal features were observed in a 3-phase bone scan or on thermographic examination.\nOn the first day of the visit, an intravenous regional block was performed with 40 cc of 0.5% mepivacaine and 30 mg of ketorolac. In addition, 300 mg/day of gabapentin was administered in three divided dose. The constant tingling sensation and pain the upper lateral malleolus completely disappeared but she sporadically felt tingling pain near the bite area. The pain during walking decreased to a VAS intensity of 20/100, but the cold sensation in the heel persisted. On the 3rd day, when performing a secondary intravenous regional block with 40 cc 0.5% mepivacaine and 30 mg ketorolac, the patient developed urticaria in the entire calf with severe pain on the bite site and in the stiff and painful regions during walking. Therefore, dexamethasone 5 mg was injected through the same intravenous route as the mepivacaine injection. The severe pain diminished immediately after the injection.\nThe next day, the patient had minimal pain, when relaxed, and experienced pain with a VAS intensity of 15/100 on the lateral dorsal part of the foot while wearing shoes and walking. However, the sickly cold sensation was still present in the heel. On the 5th day, a lumbar sympathetic ganglion block was performed on the 2nd and 3rd vertebrae. The cold sensation in the heel reduced for 2-3 days after the block but returned at the same level as before; therefore, we decided to perform a sympathectomy using alcohol. The preoperative temperature of both heels was almost the same (left heel, 31.1℃; right heel, 31.0℃). 2 ml of contrast medium and 2 ml of 4% lidocaine were injected in the sympathetic ganglia at the L2 and L3 level. After a 10-min wait to confirm the absence of motor weakness and other abnormalities, 3 ml of 99% alcohol was injected on each side. The foot temperature at 15 min after the injection indicated a difference of 3.3℃ between the left (32.1℃) and right (35.4℃) side, and the patient felt a burning sensation on her foot.\nThe cold sensation in the heel completely disappeared 2 days after the sympathectomy. The following day, the patient was discharged with a VAS score of 10/100 and similar levels of pain while walking and relaxing. The dose of gabapentin was increased to 600 mg tid. The decrease in the pain was maintained during the 2-month follow-up period. A reduced dose of gabapentin (300 mg tid) was administered for the next 2 months. When the pain disappeared, administration of the drug (gabapentin 300 mg tid) as well as follow-up were discontinued. |
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%.\nIn 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. |
A 39-year-old male with a history of a sustained low platelet count detected during health checkups presented to our hospital. US performed at an outpatient clinic had suggested the presence of chronic liver disease with splenomegaly and collateral formation of the portal vein. Contrast–enhanced computed tomography (CT) revealed severe stenosis of the IVC; based on this finding, the patient was diagnosed with BCS. We obtained HV waveforms using pulsed-wave Doppler devices as previously reported.[ The Doppler gate was placed on the right HV 1 to 3 cm distal to the inferior vena cava by an intercostal approach and a flattened monophasic pattern was detected that likely reflected blocked transmission of cardiac pulsation from the right atrium to the HV due to IVC stenosis (Fig. A). In addition, TE (FibroScan) yielded an LS value of 17.8 kPa, suggesting congestion and/or severe fibrosis of the liver.\nX-ray venography showed complete obstruction of the IVC, and the pressures below and above the obstruction were 17 and 8 mm Hg, respectively (pressure gradient, 9 mm Hg; Fig. B). The IVC obstruction was treated by intravascular needle puncture followed by balloon dilation. This treatment decreased the pressure gradient between below and above the obstructed site to 2 mm Hg, indicating successful removal of the IVC obstruction.\nOn the day after PTA, the waveform of the patient's RHV, assessed by Doppler US, had changed to a biphasic pattern (Fig. A). His LS value decreased rapidly to 8.7 kPa, suggesting that the high LS value before PTA was due to congestion of the liver, rather than to liver fibrosis. One year after PTA, the patient's HV waveform had further improved to a triphasic pattern (Fig. A), and his LS value was 9.4 kPa. Three years after PTA, IVC restenosis had not occurred, the patient's HV waveform remained triphasic, and his LS value had decreased slightly to 7.2 kPa. |
A 36-year-old married man was admitted for his maladaptive gambling behavior, suicide attempt, and depressive mood. He had taken over his father's business and had worked successfully as the president of a ready-mixed concrete cooperation until he fell into gambling. This happened when he lost more than 13 billion won (about 1.4 million US$) because of a downward trend in the stock market. He struggled and made every effort to recover his loss. When he went to a casino while vacationing with a friend, he gambled forty million won that he had, and he succeeded twice. Since that time, he began to visit the casino after finishing business early in the morning. The frequency of his visits increased rapidly, and he eventually visited this casino almost daily by the end of the year 2000. He played mostly 'baccarat' and rarely played slot machines or any other type of game. His family members and employees did not know about his repeated and maladaptive gambling behavior because he concealed it from them. As his gambling continued, he lost money more frequently. However, he could not control his desire to recover all of his losses at once. As time passed, his financial losses continued to increase, and he lost not only part of his father's property, but also fell into debt to his friends. He attempted suicide by hanging himself in his house in the autumn of 2001, at which point his family became aware of his maladaptive gambling behavior. His gambling behavior continued for more two years, and his suicidal thoughts, attempts, and depressive mood caused his mother and brother to take him to the Depressive Disorder Clinic, Department of Psychiatry, Chonbuk National University Hospital, Jeonju, Korea. He was voluntarily admitted to the psychiatric ward at Chonbuk National University Hospital in February 2002.\nThere were no psychiatric disorders including pathological gambling, substance abuse, or depression among his family and relatives. In the first interview after admission, he appeared physically healthy, but somewhat tense and confused by his abrupt admission. He felt guilty about his gambling behavior, especially toward his parents, In an unreasonable belief, however, he insisted that he could regain his gambling losses by gambling again. He had not been addicted to alcohol or other substances and demonstrated no other impulsive behaviors (e.g. sexual, aggressive). However, he was a heavy smoker at two packs a day.\nImmediately after admission, his laboratory results were found to be in the normal range; complete blood count (CBC), liver function test (LFT), urine analysis, thyroid function test (TFT), venereal disease research laboratory (VDRL) test, electrocariogram (EKG), chest X-ray, and other routine laboratory tests. His blood pressure was 130/80 mmHg; and respiration and other physical findings were in the normal range.\nWithout any medication, he was examined for mood, thought, behavior, and craving for gambling one week after admission. He had a score of 10 in the Korean South Oaks Gambling Screening (KSOGS), and a score of 18 in the Gambling Symptom Assessment Scales (GSAS). According to the DSM-IV-TR, he met the criteria for pathological gambling. He did not meet the DSM-IV-TR criteria for major depressive disorder, but he met the criteria for dysthymic disorder.\nBaseline imaging was obtained by fMRI on the seventh day after admission (). On the eighth day, fluvoxamine (50 mg/day) was prescribed for him with no concomitant mediations. In the three weeks after admission, the dose of fluvoxamine was raised to 200 mg/day and maintained during a follow-up period; there were no significant adverse effects of the medication. Beck's Depression Inventory (BDI), State-Trait Anxiety Inventory (STAI), and GSAS had been administered before medication, and they were reexamined on the seventh day after medication. The second fMRI scan was also obtained on the seventh day after medication ().\nThree weeks after discharge (six weeks after beginning of the fluvoxamine prescription), BDI, STAI, and GSAS were again administered, and the same assessments were also carried out at six and nine months after beginning of the medication (). Additionally, fMRI scans took place at six weeks and six months after medication ( and ).\nThe patient did not undergo individual or group cognitive behavioral therapy, nor did he participate in Gamblers Anonymous during or after his admission to the hospital. He had not gone to gamble during the follow-up period. He also had reported that his desire to gamble had decreased, and he was disabused of his unreasonable belief that he could regain his losses by gambling. He presently maintained his job and social relationships stably after treatment.\nfMRI was performed on a 1.5T magnetic resonance scanner (Magnetom VISION, Siemens, Erlangen, Germany). For functional imaging, echo-planar imaging (EPI) was used (matrix = 64×64). Five rest conditions were alternated with five activation conditions, and each period consisted of 120 images at 22-second intervals (repetition time). These 1,200 images were collected and analyzed according to the time. During the activation period, the stimuli consisted of card pictures showing a full house, four-of-a-kind, and a royal straight flush. During the resting period, the patient was shown landscape pictures. Analysis of the images from the activation period was performed using SPM 99 statistical parametric mapping software to establish reference vectors corresponding to activation and resting periods. Among regions in which changes in signal intensity were cross-correlated with the reference vector, only regions meeting a threshold of p = .0001 and an extent threshold of 30 voxels (size 2×2×2 mm) were considered as significant signals. The anatomical locations of the activated foci were assigned using the Talairach atlas and were then presented as the appropriate Brodmann's areas. |
A 66-year-old male presented with a history of sudden ocular pain and redness in OD for 10 days. The patient had a history of a hypermature cataract in the right eye 7 years previously. He denied recent trauma in OD. No inflammatory antecedents were reported. Best corrected visual acuity in OD was hand motion close to the face, while OS was 0.8. Slit lamp examination of OD showed conjunctival congestion, moderate corneal edema and a deep anterior chamber with lens particles at the inferior angle (). There was a chamber reaction with flare (2+) and cells (2+). Numerous large white flecks were observed circulating in the aqueous. Lens cortex material was noted to protrude through the dehiscence site of the anterior capsule. The pupil was mid-dilated and adhered to the disrupted anterior lens capsule. The lens showed the presence of a hypermature cataract.\nThe intraocular pressure (IOP) measured with Goldmann applanation tonometer was 55 mmHg in OD and 17 mmHg in OS.\nGonioscopy with Goldmann 2-mirror goniolens demonstrated the presence of wide-open angles and lens particles that were thickly attached to the inferior trabecular meshwork (). There was no sign of angle recession, and no corneal or scleral perforation was noted in OD. Following dilation of the pupils, slit lamp examination of the anterior segment showed fragmented lens particles protruding through the ruptured anterior capsule margin. Evaluation of the anterior capsule of the lens showed that there was a pre-existing anterior capsular dehiscence, extending along the pupillary margin. This was associated with an intracapsular distension, and protrusion of the anterior sub-capsular lens particles through the dehiscence into the anterior chamber. Phacodonesis was not observed. The fundus was invisible due to the hypermature cataract. The examination of the left eye was within normal limits. Ultrasound B-scan of the right eye was within normal limits.\nA diagnosis of right eye lens-particle glaucoma was made. Medical therapy for the elevated IOP included 250 mL of 20% mannitol (1 g/kg body weight) intravenously over 45 minutes in the acute phase, followed by Acetazolamide 250 mg tablet four times daily, Dorzolamide 2%/timolol 0.5% eye drops twice daily and brimonidine 0.15% eye drops twice daily for maintenance of IOP control. Cataract surgery was recommended for the treatment of lens-particle glaucoma. An anterior chamber paracentesis was performed, and a 0.2-mL sample of the white material in the aqueous was obtained.\nThe patient underwent an extracapsular cataract extraction. A posterior lens capsule rupture which might have been produced during surgery was confirmed intraoperatively. Anterior vitrectomy was performed with an automated vitrector (Alcon. Accurus® 400VS) for the vitreous prolapse and for the removal of the sticky cortical matter in the anterior vitreous and anterior chamber angle. Histological examination of the anterior chamber aspirate revealed lens cortical fibers, numerous hemosiderin laden macrophages and neutrophils (). No bacterial analysis, florescence stain or culture with the aqueous humor sample was done.\nPostoperatively, the patient was treated with topical moxifloxacin 0.5%, prednisolone 1% eye drops in a tapering dose regimen and atropine 1% eye drops twice daily for 2 weeks. At the 1-month postoperative follow-up, the right eye IOP was 16 mmHg without any intraocular antihypertensive medication. |
In this article we report a case of a 60 year old female patient with a ten year history of Rheumatoid arthritis treated with prednisolone 10mg/ daily, methotrexate 10mg /week, etanercept 25mg/weekly [for more than a year] and also risedronate sodium 35mg /weekly for prevention of osteoporosis. The main complaint of the patient was an ulcer with a 3 weeks reported duration, which caused moderate constant pain. She had done a full blood count and biochemical panel which showed no abnormal findings. She initially visited for this reason a general dentist who prescribed fluconazole and as the lesion did not resolve, she consulted an oral surgeon who suggested evaluation by an oral medicine specialist. The clinical examination revealed an ulcer with irregular margins covered with necrotic slough, located on the gingiva of the upper left quadrant [#25,#26], where the patient had an old bridge, (Fig. ) and extended from the buccal to palatal surface. She had no palpable neck lymph nodes. According to the medical history and clinical findings the differential diagnosis, was : osteonecrosis, ulceration due to methotrexate, lymphoproliferative disorder of the oral soft tissue due to immune suppression. An immediate soft tissue biopsy [from the palatal surface] and orthopantomography were performed. The radiographic findings showed normal bone underneath the ulcer. The histological examination revealed lymphocytic infiltrate in hematoxylin eosin stain (Fig. ), and a panel of immunohistochemistry markers for lymphoma revealed positivity for the CD30 [cluster of differentiation (Fig. ) and Epstein–Barr virus encoded early RNA [EBER]. These findings were considered suggestive for B-cell origin NHL or lymphomatoid granulomatosis because of the presence of areas with angiocentric local allocation. Further molecular tests were scheduled [polymerase chain reaction [PCR]] and revealed monoclonal B-cell population among the lymphocytic infiltrate so, the final diagnosis was B-cell origin NHL.\nThe patient was referred to a tertiary oncology and hematology clinic [Laiko Hospital, Athens Greece] and was investigated with PET-scan [Positron emission tomography] and CT [Computed tomography], which showed that the only site affected was the oral mucosa. She was also evaluated by a Rheumatologist. Both the Hematologist and Rheumatologist in charge considered that her NHL was possibly related to the use of etanercept and secondary to methotrexate so they stopped her of the agents. The patient was treated with a combination of the anti-CD20 monoclonal antibody rituximab [Rituximab 1000mg / 6 months] and classic CHOP [Cytoxan, Hydroxyrubicin [Adriamycin], Oncovin [Vincristine], Prednisone],chemotherapy regimen. Three years after the initial diagnosis she is free of disease (Fig. ), and her rheumatoid arthritis is controlled only with low dose prednisolone. |
A 45-year-old man was brought to the emergency room because of a motor-bike accident. His major problem was a head injury, but at the same time his left ankle was swollen with a very tensed skin over its lateral side and the foot in slight equinus and supination (Fig. ). Neurological and vascular examination showed no pathological signs. The radiographic examination showed a total anterolateral dislocation of the left talus in all projections (Fig. ) without any accompanying fracture.\nIn both cases a closed reduction was performed under general anesthesia. With the knee flexed to relax the posterior leg muscles, traction was applied to the foot and counter-traction to the tibia. The talus was then pushed to a posterior and medial direction. Reduction occurred relatively easily (Figs. ). The reduction appeared stable with manual manipulation and was confirmed radiologically in the operating room (Figs.). After reduction, pulses could be palpated clearly in case I.\nIn patient I, a below knee back slab and then full plaster cast was applied for a total of 6 weeks. Then partial weightbearing with the use of a walker for 1 week, 1 week with 2 crutches, 1 week with one crutch and finally full weight bearing and motion was allowed at a summation of 9 weeks. The patient was followed up for three years at which time no signs of avascular necrosis or osteoarthritis were detected clinically or radiologically. She had no symptoms, more than 90% range of motion in the ankle and subtalar joints and no degenerative changes on radiographs, an excellent result according to the criteria of Kenwright and Taylor []. Patient II was lost from follow-up. He was a traveler and as soon as his general condition allowed it, he was transferred back to his home country. It was reported to us that one year later he was walking with no problems. |
An 18-month-old male patient with Pompe disease was referred to our clinic due to swelling of his gingiva while he was being treated by enzyme replacement therapy in the intensive care unit of the university hospital. During the anamnesis, it was learned that the patient had a birth weight of 3.270 g from a normal term pregnancy and cesarean section, and his parents were second-degree relatives. The other child of the family had died at 16 months due to Pompe disease, and the patient had a 16-year-old healthy brother.\nThe medical history of the patient revealed severe muscle hypotonia, respiratory problems, gastric reflux, and a mild cardiomyopathy. His respiration was through a ventilator and he was fed by gastrostomy tube; he was conscious and cooperative but could not speak.\nAs a result of genetic assessment of the peripheral blood sample at the genetic department of another university, it was learned that a homozygous c.1408_1410 del mutation had been detected.\nHis first dental examination was performed by us when he was hospitalized at the intensive care unit. His mouth was found to be open due to macroglossia and respiratory failure, and a nonfluctuant, normal gingiva colored swelling at the right anterior region of the maxilla was detected. No color change, focus of bleeding, cystic formation, or infection was encountered at the region. Tooth eruption was noted for 61, 71, and 81 numbered teeth (). His anamnesis, obtained from his mother, revealed that gingival swelling was also present when 61 numbered tooth erupted. His parents were recommended to perform finger massage to the region of 51 numbered tooth. At the examination six months later, 51, 52, 62, and 74 numbered teeth had erupted (Figures and ), and there was fusion between 51 and 52 numbered teeth, and an enamel fracture at the mesial edge of 61 numbered tooth, which his mother was unaware of it, was thought to be secondary to intubation, and 84 numbered tooth was seen to be erupted and it was learned that a swelling at the site of this tooth, similar to the previous one, was present. His parents were recommended to finger massage to this area as well, and no antibiotics or anti-inflammatory drugs were prescribed during this process. The swelling was found to have decreased at the follow-up, one week later.\nSymptoms of the patient disappeared overall; on the other hand, parents of the patient could not bring the patient to us after leaving the intensive care unit. Therefore, medical doctors of the patient were informed about the necessity of our follow-ups when the patient was brought to the hospital once again. The follow-up process will provide clarifying the uncertain oral signs. |
A 45 year old Sri Lankan man presented to us with a 2 day history of fever, head ache, arthralgia, myalgia especially affecting the lower limbs and lower abdominal pain. In addition he had vomiting and passing of dark colored urine for one day. He said that there was an apparent reduction of volume of urine he passed. The day before he had taken treatment from a general practitioner and has been on oral amoxicillin, paracetamol (acetaminophen) and vitamin B complex tablets since then. As his symptoms got aggravated he was admitted to our hospital.\nHe is used to standing bare footed at the mouth of a large urban drain (polluted with animal excreta) discharging into a stream after the rains and catching fish because he noticed the fish of the stream concentrating there to eat matter flushed along the drain. The last time he did this was 6 days before admission. That indicates his exposure to leptospirosis. His past medical history and the family history were unremarkable except that there was a history of an allergic reaction to an unidentified agent 2 decades ago that needed hospitalization.\nUpon examination there was a mild pallor and icterus but no conjunctival suffusion, no fever. His pulse rate was 88/min, blood pressure was 120/80 mm mercury, his lungs were clear to auscultation and his liver was felt 1 cm below the right costal margin. The results of his central nervous system examination were normal. There was neutrophilic leucocytosis. Leptospirosis was diagnosed and intravenous ceftriaxone and supportive therapy was initiated.\nUpon the second assessment the patient was very pale and a sample of urine from him indicated hemoglobinuria as shown in Fig. .\nFurther details were asked and he revealed that he had eaten cooked leaves of Kuppameniya (Acalypha indica) 1 day before the onset of fever, but denied any diagnosed hematological disease affecting his brother or relatives from the maternal side of his family. He did not know about any illness during his neonatal period.\nHence we started a workup for the possibility of hemolytic anemia as well. Table summarizes his laboratory investigation results.\nHe denied ingestion of other drugs or food that may induce hemolysis. A hemolytic crisis triggered by Acalypha indica in a G6PD deficient person was diagnosed, antibiotic was stopped. Blood transfusions were given (700 ml packed cells during the day 3, 350 ml each on day 4 and day 6). A consultant hematologist’s opinion obtained. After his symptoms subsided on the fifth day after admission, he was sent home on ferrous sulfate 400 mg and ascorbic acid 100 mg thrice daily, folic acid 1 mg daily supplements with a list of drugs to be avoided. Reticular cell count was repeated 6 weeks after discharge. It has returned to normal (0.5%). Patient was again referred to the hematologist, a positive Brewer’s test confirmed the diagnosis. Brewer’s test [] is the only test available at Sri Lankan state hospitals like ours for confirmation of G6PD deficiency []. Later a G6PD qualitative reflex quantitative estimation was performed and level was 2.0 U/g Hb (reference range 4.60–13.5) and that further confirmed the diagnosis. |
A 4-month-old female infant was referred to the genetic clinic for aniridia and an enlarged anterior fontanel during November 2019. She was the first child of non-consanguineous parents, both of whom are now 33 years old. The mother underwent surgical tumor removal due to mucinous cystadenoma of the right ovary at the age of 27. Simultaneously, she suffered from polycystic ovarian syndrome (PCOS) and subclinical hypothyroidism. Because of the PCOS, the infant was conceived through in vitro fertilization and embryo transfer (IVF-ET). Euthyrox was given orally before pregnancy, and thyroid function was well-controlled. A genetic karyotype analysis of both parents was done prior to in vitro fertilization (IVF), and results were normal.\nThe mother of the patient had a routine prenatal examination during pregnancy. The ultrasound examination at 12 weeks of gestation revealed no major structural abnormalities in the fetus, and the thickness of nuchal translucency (NT) was normal. Noninvasive prenatal testing (NIPT) at 14 + 5 weeks showed no abnormalities. There was a mild reduction in the amount of amniotic fluid (AF) observed at 28 weeks of gestation, but no other abnormal findings were identified at this time. Percutaneous umbilical blood sampling was recommended to the mother to exclude possible genetic disorders, but she declined. The amount of AF was monitored regularly during mid-late gestation (weeks 24 through 39), and results are shown in Fig. . The amount of AF was reduced than normal between 28 and 30 weeks and at 38 weeks of gestation. The baby was born at 40 weeks of gestation. Her birth weight was 2820 g, height was 49 cm, head circumference (HC) was 31.5 cm, and Apgar score was 10; all parameters were in normal range.\nAfter delivery, however, due to “poor response”, the infant was transferred to the neonatal intensive care unit. After a routine blood test showed that the white blood cells (WBC) increased to 33.7 × 109/L and granulocytes were at 72.8%, antibiotic treatment was given. Physical examination showed that the anterior fontanelle was 2.5 × 2.5 cm, full, and accessible to the bone seam, and the posterior fontanelle was at 2x2cm and not closed; thyroid function appeared to be normal; ultrasound scan showed normal liver, gallbladder, pancreas, spleen and kidney; normal MRI brain scan and diffusion-weighted brain imaging was reported; echocardiography showed that the atrial septal defect (central type) was 2 mm; and the newborn hearing screening was in the normal range. After 12 days of antibiotic treatment, the WBC decreased to 9.1 × 109/L, and the granulocytes decreased to 34.6%. The infant was then discharged from the hospital.\nSigns of bilateral aniridia and ptosis were noticed by physicians at Xi’an Angel Women’s & Children’s Hospital when the infant was 6 weeks old. Developmental delays were observed at 3 months old with manifestations of great motor retardation. At 4 months old, she was diagnosed with aniridia, ptosis, macular dysplasia, nystagmus, low set ears, and an enlarged anterior fontanel. Genetic examination was recommended. On November 6, 2019, SNP array analysis was performed in our hospital, and arr [hg19]11p15.1p11.2(18742043–44,991,839) xl (26.25 Mb) was detected. The molecular diagnosis confirmed that the patient had both WAGR and Potocki-Schaffer syndromes.\nThe patient is now 6 months old with normal body length, weight, and head circumference. After intensive pediatric physical therapy, she can raise her head but cannot sit. Her intellectual ability is equivalent to that of a 3-month-old. Physical examination revealed an enlarged anterior fontanel, aniridia, ptosis, macular dysplasia, nystagmus, low set ears, rough face, micrognathia, and atrial septal defect. The growth stage-based development and phenotype assessments are shown in Table . |
A 45-year-old Caucasian woman with RP, previously diagnosed in 2005, presented to our clinic following a referral for suspected Usher syndrome given her history of concomitant high frequency hearing loss. Apart from a mild loss in visual acuity that had been relatively stable for several years, our patient did not have any other significant ocular symptoms, including, most notably, an absence of pain, redness and photophobia. Her medical history was significant for hypertension, for which she was taking a diuretic medication. She was also taking vitamin A supplements. She was not taking any ocular medications and she denied a history of ocular trauma or pathology aside from RP.\nHer family history included a 49-year-old sister who was blind with RP and had hearing loss since childhood. Another sister, 47 years old, had a slowly progressive form of tunnel vision with RP (her hearing status was presumed normal). Her two other siblings, a brother and a sister, did not have any vision or hearing impairments.\nOn examination, our patient had a visual acuity of 20/30+2 in her right eye and 20/30 in her left eye. There was no improvement with pinhole correction. Her intraocular pressures were within normal limits in both eyes. With slit lamp examination, her external examination was normal and her sclera and conjunctiva were not injected. Several small, round KPs were observed on the inferior corneal endothelium in her right eye; however, the anterior chamber was quiet and deep. In the inferior aspect of her left corneal endothelium there were numerous large, greasy-white mutton-fat KPs (Figure A). There were 2+ cells based on Standardization of Uveitis Nomenclature classification [] but no flare in the anterior chamber of her left eye. Her irides were round and flat with no evidence of posterior synechiae or heterochromia. Both eyes had posterior subcapsular cataracts (Figure B) and there were occasional pigmented anterior vitreous cells in her left eye. A retinal examination revealed pale optic nerves with arteriolar attenuation and mid-peripheral bone spicule pigment distribution. There were no signs of pars planitis or retinitis with scleral depression. In addition to her known RP, bilateral uveitis with granulomatous uveitis in her left eye was diagnosed based on examination findings.\nA macular optical coherence tomography scan demonstrated a preserved macula with no cystoid macular edema. Her previous records indicated an extinguished response with vestigial flicker on electroretinography, suggestive of advanced RP, and moderate bilateral sensorineural hearing loss with severe loss at higher frequencies from an audiology assessment. Our patient had no obvious speech impediments and she did not have cochlear implants. Prior visual field testing had revealed bilateral ring scotomas with central sparing.\nA thorough uveitis work-up demonstrated that her angiotensin-converting enzyme, calcium, and liver enzyme levels were within normal limits. She had a slightly elevated erythrocyte sedimentation rate at 25 mm/h (normal range: 0 to 20 mm/h). Tests for human leukocyte antigen B27 and syphilis were negative. A chest X-ray showed no definite hilar or mediastinal lymphadenopathy and no signs of tuberculosis. Findings from an X-ray of her sacroiliac joints were normal.\nBased on these results, our patient was diagnosed with type 2 Usher syndrome with an associated granulomatous uveitis of undetermined etiology. Given the absence of symptoms and apparent chronicity of presentation, treatment was not indicated at the time of her presentation. She was encouraged to attend follow-up appointments to monitor the progression of her disease and any possible complications of the uveitis. Formal genetic testing was discussed, but access to clinical testing is generally problematic and research testing has yet to be initiated. |
A 58-year old male was brought to our emergency department after a fall from a ladder. He sustained a bicondylar left tibial plateau fracture with an ipsilateral left diaphyseal tibia fracture. His BMI was 30.5, he had a 25-year history of smoking, and he took omeprazole for gastro-esophageal reflux disease (GERD). Exam demonstrated extensive soft tissue swelling to his left lower extremity. A temporary external fixator spanning both fractures was applied to allow soft tissue rest. The patient remained on the orthopaedic ward in stable condition and was brought back to the operating room for definitive ORIF ten days after initial treatment. Definitive management consisted of stabilizing the fractures with a 13-hole lateral locking plate (Synthes, West Chester, PA, Figure ). DVT prophylaxis was performed by administration of subcutaneous heparin, 5000 units twice daily.\nFour days after ORIF, the patient developed extensive swelling and erythema to the left lower extremity as well as frank scrotal edema. He was tachycardic and febrile. Compression gray scale ultrasound as well as Doppler imaging revealed an extensive occlusive DVT from the common femoral to the popliteal vein.\nSystemic anticoagulation with heparin and warfarin was immediately started and a CT venogram was obtained, demonstrating an extension of the DVT into the common iliac vein. A stump of the left common iliac vein at the crossover from the right common iliac artery, diagnostic with MTS, was found. With still increasing soft tissue swelling and fear of a possible pulmonary embolism, a decision for an immediate endovascular intervention was made.\nBoth right internal jugular and right iliac vein were cannulated and a seven French inferior vena cave (IVC) sheath was positioned in the common iliac vein using a guide wire. Contrast venography was performed at this point and demonstrated the extensive DVT (Figure ). A Bard-Ecclipse filter device (Bard Peripheral Vascular, Tempe, AZ) was deployed at the L1/L2-level. A glidewire and a glide catheter were then used to traverse the thrombus in the left common iliac vein. The glidewire was replaced by an Amplatz wire (Cook, Bloomington IN) over which an Angiojet (Possis, Minneapolis, MN) was advanced and mechanical thrombolysis was performed.\nThe patient was closely monitored after the venous intervention, and formal anticoagulation was continued for two months. At this time, repeat ultrasound studies demonstrated extensive residual thrombus with extensive collateral formation. Catheter-directed thrombolysis with recombinant tissue plasminogen activator (rTPA) was performed, and stents were placed to maintain patency of the common iliac vein. Following 10 mm angioplasty of the stent segment, improved venous outflow was demonstrated by completion venogram (Figure ).\nThree months following thrombolysis and iliac stent placement, the patient was noted to have significant improvement in his lower extremity swelling. Following healing of his fracture, he resumed full weight-bearing on his left lower extremity. |
A 69-year-old man was referred for surgical assessment after the findings of an abdominal wall and left atrial (LA) mass. He had mild obstructive lung disease and a 30-pack-year smoking history. He sought medical attention after experiencing abdominal discomfort and had noted a 30 lb weight loss over the last few months, apparently related to dieting. There were no other constitutional symptoms. His symptoms persisted despite a course of antibiotics, for presumed diverticulitis. Physical examination was unremarkable. Laboratory investigations revealed a hemoglobin of 131 g/L (normal: 140–180), with all other values within normal limits. Emergency computed tomographic (CT) scan revealed a 5 × 4 cm heterogenous mass in the left anterior abdominal wall and an intracardiac mass. CT head, chest, and abdomen did not show any other masses.\nTwo biopsies of the abdominal wall mass were examined, and most of the tissue was necrotic. The conclusion was that a definitive diagnosis was not possible (in spite of the immunohistochemistry) and that the definitive diagnosis was best accomplished following cardiac and abdominal mass resection.\nTransesophageal echocardiogram (TEE) revealed a 4.4 × 3.5 cm mass attached to the posterolateral wall of the left atrium, above the mitral annulus. Mean and peak gradients across the mitral inflow were 4 and 12 mmHg, respectively (). Cardiac CT and MRI corroborated the TEE findings ().\nCoronary angiography showed a 90% right coronary artery (RCA) lesion. The patient underwent extensive resection of the LA mass and repair of the left atrium, left ventricle, left ventricular outflow tract, as well as mitral valve replacement and saphenous vein graft to RCA. The postoperative course was complicated by acute renal failure requiring dialysis and reintubation for volume overload. Ten days postoperatively, he became acutely hypotensive, developed multiorgan failure, and succumbed to his illness.\nMacroscopic examination of the cardiac specimen revealed an atrial mass weighing 30.4 grams with a side to side dimension of 4.3 cm, length of up to 4.5 cm, and a thickness of up to 2.9 cm from the atrial endocardium. The mass involved the atrial wall posteriorly and the entire posterior mitral leaflet, except for its free margin (). The most protuberant part of the mass was considerably softer in consistency and deeper yellow in color with small darker areas. The cut surface of the mass had a soft to firm grey white appearance. Histology showed sheets of densely packed spindle cells replacing and infiltrating the atrial wall and the mitral leaflet, as well as the epicardial adipose tissue. The tumour cells had large, pleomorphic nuclei with prominent nucleoli (). Frequent abnormal mitotic figures were identified (). In some areas, the tumour showed malignant cartilaginous and osteoid differentiation (). Extensive tumour necrosis was seen, and the free surface had a fairly thick rim of thrombus. Some areas showed tumour extension to the epicardial fat and to the resection margin. The tumour involved the mitral leaflet tissue. Immunohistochemistry had positive staining for vimentin, mild focal positivity to S100 (), and negative staining for smooth muscle actin, CD117, CD34, and O13 (CD99). Staining with MDM2 and platelet-derived growth factor (PDGF) was not performed. The left sided cardiac mass was malignant. It showed no evidence of metastasis to the liver, right side of the heart or lungs, and the heart showed no evidence of patent foramen ovale. The morphology of the cardiac tumour compared to the abdominal, and the immunostaining profile, all supported the diagnosis of primary cardiac intimal spindle cell sarcoma. |
The patient is a 69-year-old woman. She was infected with pneumonia at the age of three and had a high fever for a long period. After the fever abated, she became aware of hearing loss. Because she lived in a mountain village, she rarely visited medical facilities. Since then, she had never worn a hearing aid, had gone to a school for the deaf from elementary school to high school, and communicated with people in sign language. Her husband also used sign language, so she had not used oral communication from elementary school to the present. In 2011, family stress triggered the onset of tinnitus. Because around the same time, she began suffering from sleep onset disorder (it took about one hour until sleep onset), nocturnal awakening, and palpitations, she went to a nearby psychosomatic medicine clinic. She received medication at the clinic but her tinnitus did not improve, so she was referred to our department in 2014.\nVarious questionnaires were given at her consultation. The result of THI (Tinnitus Handicap Inventory) [], which is used to evaluate the severity of tinnitus, was a high of 94, which indicates the most severe form of tinnitus. Also, an SDS (Self-rating Depression Scale) [] score, which is used to measure depression tendency, was as high as 61, showing that she had a tendency toward depression. The STAI (State-Trait Anxiety Inventory) [], which is used to measure anxiety tendency, returned a State Anxiety (STAIs) score of 69 and a Trait Anxiety (STAIt) score of 67, indicating that she had an extremely high anxiety tendency. As one of the personal features of her tinnitus, she had no concept of the “loudness of tinnitus” because she had been deaf since childhood.\nIn imaging tests, there were no abnormal findings except for a slight enlargement of the inner ear canals observed by temporal bone CT scan. Head MRI showed no abnormal findings within the skull or in the internal auditory canals.\nFor treatment, we started oral administration of a serotonin reuptake inhibitor (SSRI) (paroxetine hydrochloride, Paxil®, 12.5 mg, started as one tablet a day, increased to three tablets a day) and a sleep-inducing agent (suvorexant, Belsomra®, 15 mg, one tablet a day). One and a half months later, the sleep onset disorder and nocturnal awakening improved, but early morning arousal persisted. Then, a benzodiazepine anxiolytic was added (etizolam, Depas®, 0.5 mg, one tablet a day). At four months after the initial visit, THI was 84, SDS 43, STAIs 50, and STAIt 48, which showed that her psychological condition had improved, although the tinnitus distress level did not change.\nAt this time, there was no improvement in perceived palpitations, and “pulsatile tinnitus” that seemed to synchronize with the heartbeat became the chief complaint concerning tinnitus, which led us to suspect that she had autonomic disorders. Six months after the initial visit, she started to receive psychotherapy (autogenic training). After the start of the treatment, we treated the patient with psychotherapy once a month, which continued until the 7th therapy session was completed. At the end of psychotherapy, our test results showed THI at 60, SDS 45, STAIs 32, and STAIt 43, showing a further improvement trend. The THI score was still high at 60, but the subjective tinnitus distress became “not so annoying,” and the “echoing tinnitus” that was the cause of the patient’s discomfort at the time of the initial visit disappeared. Only the pulsatile tinnitus, which seemed to be related to palpitations, remained.\nOne year and seven months after the initial visit, palpitations and pulsatile tinnitus, as well as anxiety and insomnia, were aggravated due to work stress. She restarted psychotherapy. At the same time, SSRIs were replaced by noradrenergic and specific serotonergic antidepressants (NaSSAs) (mirtazapine, Reflex®, 15 mg, started as one tablet a day, increased to two tablets a day). As a result, improvement of the palpitations and insomnia gradually occurred, and two years and one month after the initial visit, our test results showed THI at 40, SDS 47, STAIs 40, and STAIt 46.\nBy three years after the initial visit, the symptoms had stabilized and the anxiolytic drug was discontinued, but oral administration of the NaSSA and sleep induction drugs continued. The subjective tinnitus and palpitations at the time of sleep almost disappeared, and the sleep onset disorder and nocturnal awakening rarely occurred.\nNow 4.5 years have passed and she is taking only a low dose NaSSA (mirtazapine, Reflex®, 15 mg, 0.5 Tablets a day). The latest test results were THI 0, SDS 43, STAIt 47, and STAIs 50, indicating that the tinnitus distress had disappeared completely. |
A 45-yr-old man presented to our department with the complaints of urinary and fecal incontinence and muscle weakness in the lower limbs. He had been admitted to another hospital two months ago, with occipital headaches, nausea and vomiting. He had developed urinary and fecal incontinence and muscle weakness in the lower limbs during the subsequent days. Craniocervical magnetic resonance imaging (MRI) study had been performed and ischemia or myelitis in the upper cervical cord and lower brainstem was considered. The patient was treated with conservative medications with no clinical benefit. Subsequently, he was referred to our hospital two months later. On admission to our department, he was able to walk with a cane. Upper extremity muscle strength was completely normal, but for the lower extremities it was graded for musculus iliopsoas and musculus quadriceps femoris 3/5 on the left side and 4/5 on the right side. Craniocervical MRI at that time revealed a diffuse pontomedullary lesion, which was hyperintense on T2-weighted images most likely corresponding to edema. Prominent flow voids of perimedullary blood vessels were identified as well (). Cerebral and spinal angiography was scheduled because of the presumable vascular malformation. Angiography showed a DAVF fed by the occipital artery of the left external carotid artery. Interestingly, the venous drainage was toward the perimedullary veins (). The fistula site was catheterized by a commercially available microcatheter and the DAVF was occluded by Histoacryl mixed with iodized oil at a concentration of 25%. Control angiography showed disappearance of the fistula ().\nUnfortunately, on clinical follow-up, there was no clinical improvement after treatment. On urodynamic examination, overactive detrusor was detected. The patient was given anticholinergic drug with clean intermittent catheterization and needed to use bilateral cane for walking 3 months after discharge from the hospital. The neurologic examination showed spastic paraparesis and bilateral clonus of the ankles. The patient was hospitalized again for rehabilitation. MRI at that time showed disappearance of the oedema in the brainstem but pathologic signal still persisted in the midportion of the cervical spinal cord (). Cerebral angiography showed a small recurrence of the DAVF (). Superselective angiography showed the neuromeningeal branch of the ascending pharyngeal artery as the feeder of the recurrent fistula (). This fistula was embolized with glue at the 25% concentration. Control angiography showed occlusion of the fistula (). There was no improvement in the clinical condition. Knee-ankle foot orthoses was prescribed for the right lower extremity. However, 6 months later he came back on a wheelchair. He had no caregiver support in the home and had not been doing his exercises which led to an increase in his spasticity. He had also developed depression which made him reluctant to keep up his self mobility. He was readmitted to the hospital and after treatment with antidepressives and physical therapy for one month, he was able to walk with his orthoses. However, he was unwilling to use orthoses and found it more practical for himself to move around in a wheelchair. |
A 45-year-old Hispanic male who underwent cadaveric renal transplant in the right lower quadrant 17 years earlier, presented to the hospital with a three-month history of generalized abdominal pain with localization to the right side for two weeks. He was on chronic immunosuppression with tacrolimus, azathioprine, sirolimus and prednisone. The pain was more pronounced in the right upper quadrant, and the ultrasound imaging of the abdomen was suggestive of cholecystitis. Laboratory studies did not reveal any abnormalities. He could not confirm if he had had any problems or surgeries on his gall bladder. Hence, he underwent laparoscopic exploration of the gall bladder fossa. During surgery, adhesions of the omentum were found in the gall bladder fossa in the absence of the gall bladder, and an inflamed appendix was found elevated due to the transplanted kidney in the right lower quadrant. Laparoscopic appendectomy was performed and the tissue underwent pathological examination. He was discharged after an uneventful post-operative course.\nPathology of his appendix by immunostaining revealed anaplastic cells strongly positive for CD3, CD56 together with strong focal EBV-encoded RNA (EBER) staining (Figures , , and ). The malignant cells were negative for CD20, CD30, CD45, CD5, Alk-1 and TCK. The tissue was found to be positive for the T-cell receptor (TCR) by gamma gene rearrangement studies by PCR analysis (Figure ). Immunoglobulin heavy chain rearrangement (IgH) by PCR analysis did not detect a clonal B-cell population, thereby confirming T-cell lymphoma. A bone marrow examination revealed no involvement with negative flow cytometry and showed normal male karyotype (46, XY). A staging positron emission tomography (PET) scan showed increased radiotracer uptake in the right cervical and left groin lymph nodes along with the 3.3 cm liver mass. Non-specific uptake in the stomach was also observed.\nThe patient was re-admitted to the hospital 10 days later, with increasing abdominal pain, symptoms of gastric outlet obstruction, weight loss, headaches and fever. A lumbar puncture was negative for infection or lymphoma. Cranial imaging with a computed tomography (CT) scan was also negative. An esophagogastroduodenoscopy (EGD) was performed revealing multiple ulcerated nodular masses in the stomach and duodenum (Figures and ). A stomach biopsy gave similar results as the appendix with large anaplastic cells with irregular nuclei. Immunostaining of the gastric specimen confirmed T-cell lymphoma as well as positive EBER staining.\nInitial treatment management involved reducing the dose of the patient's immunosuppressive agents and starting chemotherapy. Administration of azathioprine, prednisone and tacrolimus was stopped and low dose sirolimus at 1 mg was given daily. The first cycle of chemotherapy (PEGS) included cisplatin 25 mg/m2, etoposide 40 mg/m2 and solumedrol 250 mg administered on days one, two and three, and gemcitabine (Gemzar) 1000 mg/m2 on day one (ongoing Phase II trial SWOG 0350). Our patient had a positive and rapid clinical response to this regimen. Thus, the chemotherapy was changed to a standard CHOP regimen (cyclophosphamide, doxorubicin [Adriamycin], vincristine, prednisone). His gastric outlet obstruction was supported with total parenteral nutrition (TPN) for a few weeks, after which the patient was able to eat well. A repeat PET scan after the second cycle of CHOP showed a significant response.\nThe main complications during the therapy were pancytopenia, febrile neutropenia and pneumonia. These were managed successfully. He recovered well and is presently receiving treatment as an outpatient. His allo-grafted kidney is also fully functional. Restaging is planned after a total of six cycles of CHOP with a PET scan and EGD. |
Subsets and Splits
No community queries yet
The top public SQL queries from the community will appear here once available.