Recent advances in the treatment of hilar cholangiocarcinoma: portal vein embolization

The clinical application of portal vein embolization (PVE) has contributed to improving the postoperative outcome of hilar cholangiocarcinoma. The enlarged nonembolized lobe after PVE protects the patient from postoperative hepatic failure, due to the increased functional reserve, and shortens the hospital stay. Although numerous reports have shown beneficial effects of PVE on postoperative outcome after extended hepatectomy, no randomized controlled study has been performed so far. It is urgent to establish a “gold standard” of PVE, because the indications, approach to the portal vein, types of embolic materials, and methods used to evaluate the function of the future liver remnant are variable among institutions. The indications and procedures of PVE for hilar cholangiocarcinoma may be different from those for hepatocellular carcinoma or colorectal metastasis, because, in many patients with hilar cholangiocarcinoma, biliary cancer is associated with biliary obstruction and cholangitis. This review article summarizes the contribution of PVE to the outcome of postoperative management in patients with hilar cholangiocarcinoma needing extended hepatectomy. We also describe our PVE procedure, which has been established from our experience of more than 240 cases of biliary cancer. Furthermore, the drawbacks of PVE, which may reduce the pool of candidates for surgery, are also discussed.     

Utility of computed tomography in identifying an ectopic thyroid in infants and pre-school children

In purpose of identifying thyroid tissue in patients with congenital hypothyroidism during childhood, ultrasonography is usually used as a screening examination, and scintigraphy is performed secondarily. Though these methods are useful, it is not easy to identify the accurate location of thyroid tissue by these methods. We previously reported the utility of computed tomography (CT) in identifying thyroid tissue in four cases of congenital hypothyroidism. The purpose of this study is to investigate whether CT is useful in identifying thyroid tissue, compared to ultrasonography or scintigraphy. Nineteen cases (0 month to 18 years of age) that were suspected to have ectopic thyroid tissue or thyroid agenesis on ultrasonography were examined by CT. CT was useful in diagnosing ectopic thyroid tissue or thyroid agenesis in all the cases, whereas ultrasonography or scintigraphy was less accurate in this purpose in seven cases. Plain CT had a difficulty in identifying thyroid tissue in two cases, in which contrasted image was required. This study showed that CT, especially an enhanced CT, is useful in identifying thyroid tissue, when the gland is not identified in the normal position by ultrasonography.     

Present status and prospects of living-related liver transplantation

Living-related liver transplantation (LRLT) is a relatively new surgical modality that has developed, in part, to overcome the shortage of available cadaveric livers for transplantation and as a method to provide liver graft implants from living donors for patients end-stage with liver disease in areas where the use of cadaveric livers is not yet practiced or permitted. Since 1988 almost 500 LRLTs have been performed globally. The safety of donors who provide a portion of their liver for grafting is of utmost concern, and only one donor death from this procedure has been reported in the literature. Postoperative survival in recipients depends on their pretransplant physical status, but emergency patients in rapid need of a liver have a poorer survival than elective LRLT patients for whom survival is about 80%. Children and infants are the main recipients of LRLTs, but adult patients particularly in Japan, are increasing in number, and present indications for LRLT surgery include not only cholestatic end-stage liver diseases but also metabolic disorders affecting the liver and emergency LRLTs for fulminant hepatic failure. Many ethical problems relating to the concept of liver transplantation, donor liver source, recipient selection, and reimplantation have yet to be resolved. But we believe that LRLTs and cadaveric liver transplantations are saving lives and that the practice should be continued.     

Risk factors of thrombotic recurrence and major bleeding in patients with intermediate-risk for recurrence of venous thromboembolism

Journal of Thrombosis and Thrombolysis - Prolonged anticoagulation therapy is recommended for patients with intermediate-risk for recurrence of venous thromboembolism (VTE). The current study aimed...     

Mondini dysplasia with recurrent bacterial meningitis caused by three different pathogens

Mondini dysplasia is rare, but has an important association with recurrent bacterial meningitis. We herein describe the case of a 3‐year‐old girl with unilateral sensorineural hearing loss who presented with three independent episodes of bacterial meningitis within 8 months. Temporal bone computed tomography indicated the characteristic features of Mondini dysplasia in the right inner ear. This was treated by surgical closure of the inner ear defect via oval window and additional vaccination was administered. Appropriate vaccination might prevent the recurrent bacterial meningitis associated with Mondini dysplasia.     

Repeated piezoelectric lithotripsy for gallstones with and without ursodeoxycholic acid dissolution: A multicenter study

The use of bile acid dissolution therapy in extracorporeal shockwave lithotripsy of gallstones, remains controversial. Our study examined whether chemolitholysis after sufficient disintegration enhanced stone clearance within 6 months of the first lithotripsy. A total of 143 patients who developed one to three radiolucent stones measuring⪯30 mm in diameter were randomly separated into two treatment groups: 47% were given lithotripsy alone, and 53% lithotripsy plus ursodeoxycholic acid (UDCA). Repeated piezoelectric lithotripsy was given, with no limit on the total number of treatment sessions, to pulverize or disintegrate stones into fragments<3 mm. Stones were disintegrated in 97% of all patients, and the fragments were ⪯2 mm in 50% of these patients. According to an intention-to-treat analysis, 52% in the lithotripsy alone group and 58% in the UDCA group were free of stones 6 months after the first lithotripsy (P=0.61). Of the patients with fragments⪯2 mm, 71% in the former and 86% in the latter group were free of stones 6 months after the first lithotripsy, with no significant difference between the groups. Biliary pain occurred in 25% of all patients, including 3 with acute cholecystitis. We concluded that the sufficient disintegration of gallstones achieved with repeated lithotripsy enhanced the early clearance of fragments, regardless of whether chemolitholysis was employed.     

Prevalence of diabetes mellitus in individuals with airflow obstruction in a Japanese general population: The Yamagata-Takahata Study

Background

Diabetes has been reported as a comorbidity of chronic obstructive pulmonary disease (COPD) in Western countries, but it has not been demonstrated in epidemiological reports in Japan. The purpose of this study was to clarify whether the relationship between airflow obstruction and diabetes can be confirmed in a Japanese general population.

Myocardial bridging increases the risk of coronary spasm

BACKGROUND: Myocardial bridging (MB) has been associated with cardiac events. Whether coronary spasm is one factor contributing to those events is unknown. HYPOTHESIS: This study investigated whether the likelihood of coronary spasm is increased in patients with MB. METHODS: A spasm-provocation test was performed by infusing acetylcholine into the left coronary artery in 114 Japanese patients with chest pain. The test result was defined as positive when the diameter of the coronary artery was reduced by > or = 50% and ST-segment changes were documented. Myocardial bridging was defined as a > 15% reduction in coronary arterial diameter during systole after intracoronary injection of nitroglycerin. RESULTS: Myocardial bridging was identified in 41 patients (36%) and was located in the mid-segment of the left anterior descending coronary artery (LAD) in all patients. Patients with MB experienced coronary spasm more frequently than patients without MB (MB+: 73%; MB-: 40%, p = 0.0006). Furthermore, among patients with a positive spasm-provocation test, coronary spasm occurred more frequently in the mid-segment of the LAD in patients with MB than in those without MB (MB+: 73%; MB-: 45%, p = 0.0259). Multivariate regression analysis demonstrated that MB was a predictor of coronary spasm (odds ratio: 3.478, p = 0.0088). CONCLUSIONS: These results suggest that MB increases the risk of coronary spasm and that coronary spasm may be the proximate etiology of cardiac events associated with MB.     

Lymphstasis in a boy with Noonan syndrome: implication for the development of skeletal features

We report on a Japanese boy with Noonan syndrome who had short stature, bilateral cryptorchidism, poor pubertal development, mild mental retardation, complex cardiac lesions consisting of hypertrophic cardiomyopathy, mitral valve stenosis and insufficiency, subvalvular aortic stenosis, and single coronary artery, and various dysmorphic features including hypertelorism, epicanthic folds, low set malrotated ears, high arched palate, micrognathia, webbed neck, low posterior hairline, shield chest, pectus excavatum, cubitus valgus, borderline short metatarsals, lymphedema, redundant skin, and nail dysplasia. Because of marked lymphedema in the bilateral lower legs, lymphatic scintigraphy was carried out at 13.3 years of age, indicating extreme lymphstasis in the lower extremities, severe lymphstasis in the forearm, the elbow, and the axillary regions, moderate lymphstasis around the ascending aorta, and mild lymphstasis in the bilateral lungs. The results, in conjunction with those suggested in Turner syndrome, imply that lymphatic hypoplasia/dysplasia and resultant distended lymphatics and lymphedema are relevant to the development of not only soft tissue and visceral anomalies but also skeletal anomalies in Noonan syndrome.