Sacrococcygeal teratoma of newborn infants; a report of two cases]

Two cases of sacrococcygeal teratoma in female infants are reported. Case 1. A newborn baby with a hemispheric mass on her hip underwent surgery 3 days after birth and the lesion proved to be an immature teratoma. The serum AFP level was very high but became normal one month after the operation. The child also had agenesis of the corpus callosum and arachnoid cysts in right middle fossa. She died after developing shunt infection. Case 2. A newborn baby with a mature teratoma was operated on the day following birth. The tumor was subtotally removed, and there has been no recurrence after 6 months. Sacrococcygeal teratoma in female infants is often associated with agenesis of the corpus callosum and arachnoid cysts. They tend to develop almost at the same time as the teratoma. It is often difficult to determine whether the infant should be treated by chemotherapy or not if the teratoma is immature, especially when it has been totally removed, the serum AFP is normal, and the tumor is pathologically of a low grade.     

Gene therapy using an adenovirus vector for apoptosis-related genes is a highly effective therapeutic modality for killing glioma cells

Preclinical studies in animal models and human clinical trials have evaluated the safety and efficacy of adenoviral vectors for cancer gene therapy. These studies have indicated that gene delivery via adenoviral vectors, including p53 gene therapy, represents a promising therapeutic modality for many types of human cancers. This review focuses on novel strategies to induce apoptosis in glioma cells by transduction with adenoviral vectors carrying a variety of apoptosis-related genes, including Fas ligand, Fas, FADD, caspase-8, p53, p33ING1, p73alpha, Bax, Apaf-1, caspase-9, IkappaBdN, caspase-3, Bcl-2, and Bcl-X(L). We conclude that adenoviral vector-mediated delivery of apoptosis-related genes other than p53 is a potentially useful gene therapy approach toward the treatment of human brain tumors.     

Hemangioma located just above the left main coronary artery, in a subject who had cardiac arrest due to ventricular fibrillation, led to a diagnosis of Brugada syndrome

We report the case of a 38-year-old Asian man with a pericardial hemangioma on the left main coronary artery. The patient presented initially at our hospital after cardiopulmonary resuscitation following an episode of ventricular fibrillation (VF). Because of spontaneous coved-type ST segment elevation on the higher intercostal space V1 to V2 in a 12-lead electrocardiogram, documented VF in the absence of structural heart disease, and a family history of sudden death, he was diagnosed with Brugada syndrome. Transesophageal echocardiography showed a smooth-surfaced mass with well-demarcated borders, directly above the left main coronary artery. Computed tomography confirmed the presence of the mass, which showed no enhancement at early phase, but did demonstrate homogenous enhancement at delay phase by contrast material. There were no findings from either the nuclear medicine or the tumor marker investigations which indicated that the mass located just above the main coronary arteries was malignant. Therefore, taken together, these findings suggested that the tumor might be a pericardial hemangioma. The relationship between the location of the hemangioma just above the left main coronary artery and the occurrence of VF was not clear, i.e. whether the presence of the hemangioma caused the stimulation of the left main coronary artery and as a result, led to the spasm of the left main coronary artery and the occurrence of VF. Furthermore, as the tumor did not extend into any of the adjacent structures, such as the coronary arteries or the right ventricular outflow tract, surgical resection was not performed; instead, the patient received a dual chamber implantable cardioverter-defibrillator.     

Risk factors for major morbidity after liver resection for hepatocellular carcinoma

Background:

Bile leakage, and organ and/or space surgical‐site infection (SSI) are common causes of major morbidity after partial hepatectomy for hepatocellular carcinoma (HCC). The purpose of this study was to analyse risk factors for major morbidity and to explore strategies for its reduction after partial hepatectomy for HCC.

Methods:

Risk factors for bile leakage and organ/space SSI were analysed in patients who underwent partial hepatectomy for HCC between 2001 and 2010. The causes, management and outcomes of intractable bile leakage requiring endoscopic therapy or percutaneous transhepatic biliary drainage were analysed. In addition, causative bacteria, outcomes and characteristics of organ/space SSI were investigated. Risk factors were identified using multivariable analysis.

Results:

Some 359 patients were included in the analysis. The prevalence of bile leakage and organ/space SSI was 12·8 and 8·6 per cent respectively. Repeat hepatectomy and an operating time of at least 300 min were identified as independent risk factors for bile leakage. The main causes of intractable bile leakage were latent strictures of the biliary system caused by previous treatments for HCC and intraoperative injury of the hepatic duct during repeat hepatectomy. Independent risk factors for organ/space SSI were repeat hepatectomy and bile leakage. Methicillin‐resistant Staphylococcus aureus was detected more frequently in organ/space SSI after repeat hepatectomy than after initial partial hepatectomy.

Conclusion:

Repeat hepatectomy and prolonged surgery were identified as risk factors for bile leakage after liver resection for HCC. Bile leakage and repeat hepatectomy increased the risk of organ/space SSI. Copyright © 2012 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd.     

New Surgical Approach to Large Splenorenal Shunt in Living Donor Liver Transplantation: Diversion of SMV and SPV Blood Flow

Introduction

The management of a large splenorenal shunt is important because it affects recipient outcome, particularly in living donor liver transplantation.

Methods

To manage large splenorenal shunts in living donor liver transplantation, we diverted superior mesenteric vein and splenic portal vein blood flow by ligation at the root of the splenic portal vein.

Result

This procedure was applied for five patients in whom superior mesenteric vein blood flow had been completely stolen by a splenorenal shunt preoperatively. Postoperative course was excellent in all cases.

Conclusion

This technique completely prevents morbidity related to large splenorenal shunts after living donor liver transplantation.     

Coronary ostium occlusion by coronary cusp displacement in Williams syndrome

Williams syndrome is a contiguous gene deletion syndrome resulting from a heterozygous deletion on chromosome 7q11.23, and is characterized by distinctive facial features and supravalvular aortic stenosis (SVAS). This syndrome rarely presents unpredictable cardiac death, and yet, as illustrated in the present case, it is still not possible to predict it, even on close monitoring. We herein describe the case of a 6‐year‐old Japanese girl with Williams syndrome, who had sudden cardiac collapse due to cardiac infarction after pharyngitis. Cardiac failure followed a critical course that did not respond to catecholamine support or heart rest with extracardiac mechanical support. Although marked coronary stenosis was not present, the left coronary cusp abnormally adhered to the aortic wall, which may synergistically cause coronary ostium occlusion with SVAS. Altered hemodynamic state, even that caused by the common cold, may lead to critical myocardial events in Williams syndrome with SVAS.     

CHA2DS2-VASc score is a useful-predictor of not prognosis but coronary-arteriosclerosis in chronic atrial-fibrillation compared with CHADS2 score: A two-center study of 320-slice CT,part 2

Background

The aim of this study is to predict the risk of coronary-arteriosclerosis and prognosis in subjects with chronic-atrial-fibrillation (CAF) using the CHADS2 and CHA2DS2-VASc scores by 320-slice-CT and invasive-coronary-angiography (ICA) in a two-center-study.

Methods

53 CAF subjects who underwent 320-slice-CT and ICA within 6-months (43 male; 69 ± 9 years; CHADS2 score 2.2 ± 1.3; CHA2DS2-VASc score 3.5 ± 1.6) in the two-institutes were analyzed. CT and ICA data were transferred to the analysis-center and were analyzed by cardiologists.

Results

Agatston-calcium-score and frequencies of the presence of various-kinds of plaques and > 50% and > 75% coronary artery stenosis were significantly higher in the subjects with CHA2DS2-VASc score ≥ 3 compared with those with score < 3. However there were no-significant differences in the Agatston-calcium-score and frequencies of the presence of various-kinds of plaques and > 50% and > 75% coronary artery stenosis evaluated by 320-slice CT between the subjects with CHADS2 score ≥ 2 and < 2. Frequency of > 50% coronary artery stenosis by ICA was significantly higher in the subjects with CHA2DS2-VASc score ≥ 3 compared with those with score < 3. However, there were no-significant differences in the frequencies of > 50% and > 75% coronary artery stenosis by ICA between the subjects with CHADS2 score ≥ 2 and < 2. During a mean of 15.9 months, composite rate of cardiac death and heart failure did not differ between subjects with CHADS2 score ≥ 2 and score < 2 and between subjects with CHA2DS2-VASc score ≥ 3 and score < 3.

Conclusions

The CHA2DS2-VASc score is a useful predictor of not prognosis but coronary-arteriosclerosis in subjects with CAF compared with CHADS2 score in this two-center-study.