Radical repair of cor triatriatum with partially unroofed coronary sinus and persistent left superior vena cava 35 years after operation for tetralogy of Fallot

We report a rare case of simultaneous repair for Cor triatriatum (CT), partially unroofed coronary sinus (CS) with persistent left superior vena cava (PLSVC), moderately severe tricuspid regurgitation, and atrial flutter 35 years after radical operation for tetralogy of Fallot (TOF). A 40-year-old woman underwent patch closure of atrial septal defect as to drain blood from left SVC to right atrium (RA), excision of diaphragm in left atrium (LA), tricuspid annuloplasty, and cryoablation. Postoperative course was uneventful with successful anatomical correction.     

Predictors of Survival in Patients With Bone Metastasis of Lung Cancer

The prognosis of patients with bone metastasis from lung cancer has not been well documented. We assessed the survival rates after bone metastasis and prognostic factors in 118 patients with bone metastases from lung cancer. The cumulative survival rates after bone metastasis from lung cancer were 59.9% at 6 months, 31.6% at 1 year, and 11.3% at 2 years. The mean survival was 9.7 months (median, 7.2 months; range, 0.1-74.5 months). A favorable prognosis was more likely in women and patients with adenocarcinoma, solitary bone metastasis, no metastases to the appendicular bone, no pathologic fractures, performance status 1 or less, use of systemic chemotherapy, and use of an epithelial growth factor receptor inhibitor. Analyses of single and multiple variables indicated better prognoses for patients with adenocarcinoma, no evidence of appendicular bone metastases, and treatment with an epithelial growth factor receptor inhibitor. The mean survival period was longer in a small group treated with an epithelial growth factor receptor inhibitor than in the larger untreated group. The data preliminarily suggest treatment with an epithelial growth factor receptor inhibitor may improve survival after bone metastasis.     

Liver transplantation for multiple liver metastases from solid pseudopapillary tumor of the pancreas

Solid pseudopapillary tumor (SPT) of the pancreas usually shows a benign clinical course. However, sometimes, distant metastasis may occur. Even in such case, the prognosis is good only if metastatic lesions are resected completely. We report the case of a 14-year-old girl with SPT of the pancreas and unresectable synchronous liver metastasis who underwent successful living donor liver transplantation. For 2 years, she has been disease free. This is the first report on transplantation to relieve liver metastasis of SPT.     

Successful treatment of renovascular hypertension due to fibromuscular dysplasia by intravascular ultrasound-guided atherectomy

A 22-year-old man presented with renovascular hypertension, based on a stenosis of the distal portion of the right renal artery with a "string of beads"-like appearance. An intravascular ultrasound image at the renal artery lesion revealed irregularity of the vascular wall. Directional atherectomy was performed and histopathology of atherectomised tissues showed medial fibroplasia, a common type of fibromuscular dysplasia. After atherectomy his hypertension was markedly improved. We report here a case of renovascular hypertension due to fibromuscular dysplasia, successfully diagnosed and treated with IVUS-guided renal atherectomy.     

Seasonal changes in symptom score and uroflowmetry in patients with lower urinary tract symptoms

OBJECTIVE: To determine whether subjective or objective seasonal changes occur in patients with lower urinary tract symptoms (LUTS) suggestive of benign prostatic hyperplasia (BPH). MATERIAL AND METHODS: A total of 31 patients with LUTS were observed for >5 years. Their International Prostate Symptom Score (IPSS), quality of life (QOL) score, maximum flow rate (Qmax), voided volume (VV) a nd post-void residual (PVR) we re measured every month. RESULTS: Total IPSS, QOL, VV or PVR did not show any seasonal changes between the three seasons: summer (hot season: June to September); winter (cold season: December to March); and spring and fall (comfortable season: April, May, October and November). Furthermore, when the IPSS was examined by dividing it into storage symptoms (frequency, urgency and nocturia) and voiding symptoms (incomplete emptying, intermittency, weak stream and straining), no significant seasonal changes were detected. Only Qmax showed a significant seasonal change, being higher in both the cold season (median 10.4 ml/s) and the comfortable season (median 10.1 ml/s) than in the hot season (median 9.4 ml/s). CONCLUSIONS: It has generally been thought that LUTS worsen in winter. Our results indicate that the IPSS and the QOL score remain nearly constant if they are determined regularly over an extended period of time. Qmax may however be influenced by seasonal changes in temperature.     

Liver transplantation without isoniazid prophylaxis for recipients with a history of tuberculosis

Abstract:  Tuberculosis remains one of the most serious infections after organ transplantation. Isoniazid prophylaxis for liver transplant recipients with a history of tuberculosis is generally recommended. However, its benefit is controversial because of potential hepatotoxicity of isoniazid. It is crucial to determine appropriate post-transplant managements for the recipients with a history of tuberculosis. The purpose of this study was to investigate the necessity of isoniazid prophylaxis for liver transplant recipients who had a history of tuberculosis. The medical records of 1116 liver transplant recipients were studied, of whom seven had a history of tuberculosis (0.63%). One who underwent living-donor liver transplantation for fulminant hepatic failure was excluded from evaluation because of early death, caused by bacterial sepsis two months after transplantation, although reactivation of tuberculosis was not observed. The median observation period after transplantation was 25.5 months (range 12–82). Reactivation of tuberculosis did not occur in any of these six patients. In conclusion, we could not find rationale for isoniazid prophylaxis in liver transplant recipients with past diagnosis of tuberculosis, when the disease is considered to be inactive. Tuberculosis should be considered as cause of post-transplant infections, and careful post-transplant observations are essential for an early diagnosis.     

Case of unexpected epiglottic cyst found during endotracheal intubation by an emergency medical technician trainee

A 66-year-old man was scheduled for endoscopic right uretherectomy under general anesthesia. On the pre-anesthesia examination, the patient had no respiratory symptoms. An informed consent for endotracheal intubation by a trainee for emergency medical technician was obtained on the examination. General anesthesia was induced with propofol, ketamine, and fentanyl. The trainee ventilated the patient easily with facemask and succinylcholine was administered. On the attempt of endotracheal intubation by the trainee with video-laryngoscope (X-LITE VIDE), a huge epiglottic cyst was observed. Tracheal intubation was done safely by the responsible anesthesiologist. Laryngofiberscopic finding showed no respiratory sequlae such as bleeding from the cyst. Although the patient had been informed about the cyst before the surgery, he did not disclose it as he did not have any symptom from the cyst. This case suggest that unpredictable airway problem could occur in any anesthetic practice. The video-laryngoscope was useful in such a case to prevent fatal complication developing on endotracheal intubation by a trainee, and also it is a good tool to show the trainee how severe the cyst related-airway trouble would be.     

Intradural extramedullary ganglioneuroma associated with multiple hamartoma syndrome

A 51-year-old woman presented with a rare completely intradural and extramedullary spinal ganglioneuroma associated with multiple hamartoma syndrome and manifesting as complaints of neck pain and dizziness persisting for 8 months. Magnetic resonance imaging of the spinal cord revealed an intradural extramedullary lesion at the C1 level. She underwent right suboccipital craniectomy and C1-2 hemilaminectomy to remove the tumor. Histological examination confirmed ganglioneuroma. She also suffered from multiple facial trichilemmomas, thyroid goiter, multiple polyposis of the gastrointestinal tract, and pulmonary hamartoma indicating multiple hamartoma syndrome. These benign neoplasms were treated conservatively.     

Tacrolimus as prophylaxis for acute graft-versus-host disease in reduced intensity cord blood transplantation for adult patients with advanced hematologic diseases

BACKGROUND: Myeloablative cord blood transplantation (CBT) for adult patients offers a 90% chance of engraftment with a 50% rate of transplant-related mortality, mostly attributable to infection. We have demonstrated the feasibility of reduced-intensity CBT (RI-CBT) for adult patients, in which cyclosporine was used for acute graft-versus-host disease (GVHD) prophylaxis. Transplantation-related mortality (TRM) was 27% within 100 days. Therefore our objective was to evaluate the feasibility of RI-CBT with tacrolimus as GVHD prophylaxis for adult patients with hematologic malignancies. METHODS: Thirty-four patients with a median age of 56.5 years (range; 22-68) with hematologic diseases underwent RI-CBT at Toranomon Hospital between November 2003 and September 2004. Preparative regimen comprised fludarabine 25 mg/m2 on days -7 to -3, melphalan 80 mg/m2 on day -2, and 4 Gy total body irradiation on day -1. GVHD prophylaxis was continuous intravenous infusion of tacrolimus 0.03 mg/kg, starting on day -1. RESULTS: Thirty-one patients achieved neutrophil engraftment at a median of day 20. Median infused total cell dose was 2.4 x 10E7/kg (range; 1.6-4.8). Thirty-two patients achieved complete donor chimerism at day 60. Grade II-IV acute GVHD occurred in 45% of patients, with a median onset of day 26. Primary disease recurred in five patients, and TRM within 100 days was 12%. Estimated 1-year overall survival was 70%. CONCLUSION: This study demonstrated the possible improvement in transplant-related mortality by tacrolimus as GVHD prophylaxis in adult RI-CBT recipients.     

Ectopic cystic thymoma associated with Raynaud's phenomenon.

Mediastinal cystic tumors are well-marginated round lesions that comprise 12% to 18% of all mediastinal masses. These lesions include a variety of diseases with overlapping radiologic appearances and variable prognoses. Pathological examinations are almost always required for differential diagnosis. We encountered a case of anterior mediastinal tumor discovered in the process of investigation of Raynaud's phenomenon. Taking into account the tumor location, a pericardial cyst was initially suspected. However, the tumor was surgically resected and histopathological examinations demonstrated thymus-like tissue in the cyst walls. Raynaud's phenomenon greatly improved after surgery. These findings suggested that cystic thymoma originated from ectopic thymic tissue and is accompanied by paraneoplastic syndrome.