We present a case of juvenile type dermatomyositis and severe retinopathy. A 10-year-old girl presented with progressive weakness of proximal muscles, generalized rash, including heliotrope-type eyelid erythema, and bilateral visual disturbance. Laboratory data showed a markedly elevated serum creatine kinase and electromyography revealed a myogenic pathology. Funduscopic examination showed numerous cotton wool spots and macular edema. She developed massive rhabdomyolysis, generalized skin lesions, systemic edema, renal failure, and respiratory failure. After she received steroid pulse therapy, plasma exchange, and high-dose immunoglobulin, her general condition and visual symptoms improved. She remained well when we followed up her condition 5 years after the discharge.

Drug refractory atrial flutter (AF) with secundum atrial septal defect (ASD) and pulmonary valvular stenosis was treated by surgical correction and intraoperative radiofrequency (RF) current ablation. Supraventricular arrhythmia, especially AF, is frequently found in aged patients with ASD. Perioperative managements for this arrhythmia were difficult because of drug refractoriness. We performed this ablation combined with intracardiac corrections, and sinus rhythm has been maintained without any drugs for 18 months. This case indicated that RF current ablation during open-heart surgery is useful and safe method of treatment of AF.

A 64-year-old man with congenital factor V deficiency and hereditary spherocytosis was attending our hospital for type II diabetes and stage 4 diabetic nephropathy. Coronary angiography performed to assess chest pain revealed severe triple-vessel disease, including total occlusion of the right coronary artery. The patient required surgical coronary revascularization. In the preoperative examination, the activated partial thromboplastin time (APTT) and prothrombin time-international normalized ratio (PT-INR) were high (89.5 s and 1.95) and factor V activity was low (6% ; normal range, 70-135%). Hemodialysis was performed on the day of the operation, and 6 units of fresh frozen plasma (FFP) were administered, which reduced immediately the preoperative PT-INR to 1.33. We performed off-pump coronary artery bypass grafting (OPCAB) and perioperatively administered 6 units of FFP with 4 units of red blood cells (RBC) transfusion. The postoperative course of the patient was uneventful, and he was discharged on postoperative day 22. Here we report the case of a patient with a very rare disease of congenital factor V deficiency and hereditary spherocytosis complicated with stage 4 diabetic nephropathy who required OPCAB.

We report an unusual case of a 71 year-old man who developed chronic consumption coagulopathy caused by an abdominal aortic aneurysm. He was diagnosed as having the dissecting aortic aneurysm (DeBakey type IIIa) and the abdominal aortic aneurysm in 1989, and had been attending to our hospital as an outpatient since then. He developed macrohematuria in March 1990. The laboratory data showed the decrease in platelet, fibrinogen, plasminogen and α₂ plasmin inhibitor and the increase in FDP. The bleeding tendency was controlled by the administration of gabexate mesilate and heparin, but the laboratory data revealed that consumption coagulopathy continued. The abdominal aortic aneurysm was successfully replaced with a prosthetic vascular graft in June 1992. Postoperative hematological findings revealed the improvement, and he discharged 32nd day and doing well after operation.

A 5-year-old boy with tricuspid atresia who underwent the Björk procedure died due to right atrial thrombus and left pulmonary embolus 37 days after operation. It is suggested that thromboembolism may be a frequent complication after the Björk procedure due to the turbulent blood flow at the right atrio-ventricular anastomosis and also due to congestive blood flow. Anticoagulation therapy seems to be essential for postoperative management.

A 6-year-old boy was admitted with infective endocarditis and aortic regurgitation. Clinical signs of infection were severe. The leukocyte count was 13, 100/μl and the C-reactive protein (CRP) was elevated to 17.2mg/dl. Blood culture was positive for Staphylococcus aureus. Echocardiography showed a vegetation 3mm in diameter on the aortic valve, and a perforation of the right coronary cusp with moderate aortic regurgitation. With antibiotic therapy, clinical signs and laboratory data of infection improved at an early stage. We decided to operate after his complete recovery from infection. Laboratory data normalized completely in 6 weeks, but echocardiography demonstrated aneurysmal change of the right coronary sinus and severe aortic regurgitation. The Ross operation was performed on the 44th day. At operation, it was noted that the non-coronary cusp was destroyed completely leaving only strings of fibrous tissue. A perforation of 3mm in diameter was also found on the right coronary cusp. There was a mural aneurysm near the right coronary orifice without abscess formation in the surrounding structure. A pulmonary autograft was transplanted to the aortic root after resection of the destroyed aortic cusps, aortic root and the mural aneurysm. The right ventricular outflow tract was reconstructed using an autologous pericardium as a posterior wall and the Monocusp ventricular outflow patch (MVOP) #22 as an anterior transannular patch. The postoperative course was uneventful. Postoperative echocardiography revealed no aortic regurgitation.

Objective : With clinical application in mind, we developed a method to measure the movement of a selected joint three-dimensionally as a conic domain (joint sinus cone). The method was applied to shoulder joint motion during daily living activities exercises in a group of hemiparetic poststroke patients. The results were compared to the exercise range of physically unimpaired persons. Methods : The subjects dressed in jackets and performed certain other tasks in a sitting position and the range of motion of the shoulder joint in three dimensions was measured using a 6-dimensional electromagnetic tracking system. Measurement results were analyzed with a plane display using a Lambert Azimuthal equal area. Results : This method was able to determine findings difficult to see with the naked eye, such as the narrow range of motion in a paralyzed shoulder joint and poor functionality also in the contralateral shoulder. Conclusion : The dressing measurement was achieved using magnetic measurement equipment. By displaying the results through a map projection it was possible to appreciate the whole range of the motion. Because this method facilitates the expression of complex joint exercise ranges, it will be useful in the analysis of daily behavior restrictions due to impairment.

We report a case of syphilitic aortitis (SA) associated with severe right coronary ostial stenosis, aortic regurgitation (AR), and annuloaortic ectasia (AAE). A 48-year-old man presented to a regional hospital with easy fatigability and nocturnal dyspnea. Echocardiography revealed Seller's grade 3 AR. A computed tomography scan showed AAE, dilatation of the ascending aorta, and calcification of both coronary ostia. Coronary angiography demonstrated that the left coronary artery was intact ; however, the right coronary artery was obscure. Active syphilis was detected on routine blood tests on admission. Therefore, the patient was started on a course of ampicillin/sulbactam (ABPC/SBT). Subsequently, he underwent the Bentall procedure and coronary artery bypass grafting with the right internal thoracic artery. The intraoperative findings showed degeneration of the aorta and severe right coronary ostial stenosis. The pathological findings of the aortic wall and aortic valve were consistent with SA. The postoperative course was uneventful. The patient continued receiving ABPC/SBT for 3 weeks postoperatively, and was then switched to oral amoxicillin.

The cases of neonates and infants who underwent successful delayed sternal closure (DSC) using silastic rubber after open heart surgery were reviewed. The indication for DSC was cardiac dilatation with tamponade-like behavior upon attempted sternal closure in all. In 7 of 10 cases, DSC were possible within 4 days after operation. There were statistical decrease in heart rate (HR), left atrial pressure (LAP), cardiothoracic ratio (CTR) and inspiratory oxygen concentration of the respirator at the time of DSC compared to those in the early postoperative period. None of the patients had mediastinitis or other severe infection in the postoperative course. One patient each died of progressive pulmonary venous obstruction and of non-cardiac disease late postoperatively and 8 patients are long-term survivors. It is concluded that DSC is recommended whenever there is any hemodynamic deterioration due to attempted sternal closure following open heart surgery in infancy as it could be safely performed within 3 to 4 days after operation without any complication.

Purpose: To investigate the influence of multiple recurrences and repeated surgeries of Hunner lesions on bladder capacity under general anesthesia in patients with interstitial cystitis (IC). Methods: We retrospectively reviewed the clinical records of Hunner-type IC (HIC) patients who underwent transurethral fulguration or resection of Hunner lesions combined with hydrodistension by a single surgeon between 2011 and 2020. Recurrence was defined as reappearance of uncontrolled urinary symptoms in association with new Hunner lesions identified by cystoscopy. Recurrent Hunner lesions were then treated by transurethral surgeries. The recurrence-free rate, potential predictive factors of recurrence, and changes in bladder capacity under anesthesia were examined at each surgical procedure. Results: A total of 92 surgeries were performed in 47 HIC patients, 23 (49%) of whom required multiple procedures (range, 1–5 times). The mean recurrence-free time after the first surgery was 21.7 months. The recurrence-free rate was 53% at 24 months, and decreased to 32% at 48 months. There were no significant differences in age, sex, bladder capacity under anesthesia at the first surgery, duration from symptom onset to the first surgery, O’Leary-Sant questionnaire including symptom and problem indexes, visual analogue scale pain score, and the number of comorbidities between the cases with or without recurrence. Bladder capacity under anesthesia was gradually decreased as the number of surgeries was increased, and bladder capacity at the fourth procedure was significantly decreased to 80% of the capacity at the first surgery. Conclusions These results suggest that multiple recurrences and repeated surgeries of Hunner lesions result in a reduction of bladder capacity under anesthesia in HIC patients although no predictive factors for recurrence of Hunner lesions were detected.