1/15. liver transplantation in patients with situs inversus.Two patients with situs inversus and biliary atresia were treated with hepatic transplantation, one with an auxiliary liver and the other with an orthotopic graft which was placed using a piggy-back technique. Both transplants functioned well initially. The auxiliary liver was rejected after 1 1/2 months, and the patient died after an attempt at retransplantation many months later. The recipient of the orthotopic liver has perfect liver function 10 months postoperatively.- - - - - - - - - - ranking = 1keywords = situs inversus, inversus, situs (Clic here for more details about this article) |
2/15. Extrahepatic biliary atresia with laterality sequence anomalies.The patient was the first child of first cousin parents. He was born at term after an uneventful pregnancy with normal height, weight and head circumference. jaundice appeared at 15 days of age. Ventricular septal defects and valvular pulmonary stenosis were diagnosed. An hepatic workup revealed extrahepatic biliary atresia and abdominal situs inversus. Hepatic biopsy showed cirrhosis with intrahepatic cholestasis. Genetic factors are suggested in extrahepatic biliary atresia. Analysis of segregation patterns suggested the existence of two major groups, one with various combinations of anomalies within the laterality sequence and the other with one or two anomalies mostly involving the cardiac, gastrointestinal, and urinary systems. This patient belongs to the first group.- - - - - - - - - - ranking = 0.2keywords = situs inversus, inversus, situs (Clic here for more details about this article) |
3/15. liver transplantation from situs inversus to situs inversus.Congenital anatomic anomalies often present technical obstacles during liver transplantation. biliary atresia (BA) is the most common indication for liver transplantation in children, and up to 28% of children with situs inversus are complicated by BA. A boy aged 2 years 11 months with BA, situs inversus, and dextrocardia received a liver transplant from his father. The donor also had situs inversus and dextrocardia without other anomalies. Graft function was excellent postoperatively, and no significant complications were encountered. This is only the second report of the successful use of a living related donor graft for a patient with BA and situs inversus. This case was particularly rare because the donor also had situs inversus, which made the present procedure more feasible.- - - - - - - - - - ranking = 2.6keywords = situs inversus, inversus, situs (Clic here for more details about this article) |
4/15. Rapid progression of intrapulmonary arteriovenous shunting in polysplenia syndrome associated with biliary atresia.This report describes a patient with biliary atresia (BA) associated with polysplenia syndrome who showed a rapid progression of intrapulmonary arteriovenous shunting (IPS), resulting in a fatal outcome. Intrauterine ultrasonography at 36 weeks of gestation revealed fetal abnormalities, including situs inversus, absent retrohepatic inferior vena cava, and azygous connection. She was diagnosed postnatally as BA because of persistent acholic stool and neonatal jaundice. She underwent hepatic portoenterostomy at age 158 days. The gallbladder and the hepatic ducts were hypoplastic, and the common bile duct was absent. Magnetic resonance image and operative findings also identified polysplenia and an absent portal trunk. Liver histology showed cirrhotic changes and bile duct proliferation. Postoperatively, she achieved good bile secretion, with gradual decrease of total bilirubin. However, she had repeated febrile episodes, and computerized tomography at age 7 months showed multiple liver cysts. Thereafter, she presented with exertional dyspnea. Contrast-enhanced echocardiography showed IPS with a degree of 2/III at age 8 months and 3/III at 10 months. (99m)technetium-labeled macroaggregated albumin ((99m)Tc-MAA) scintigraphy revealed a shunt ratio of 25.5% at 9 months and 39.7% at 10 months. Percutaneous transhepatic drainage of the bile cysts was performed without success. Sludged bile was obtained. However, respiratory distress rapidly progressed, and she died at age 11 months. In the present patient, the association of polysplenia syndrome and absent portal vein with BA, as well as liver cirrhosis, seemed to be contributing factors to rapid progression of IPS in early life.- - - - - - - - - - ranking = 0.2keywords = situs inversus, inversus, situs (Clic here for more details about this article) |
5/15. biliary atresia associated with hypoplastic left heart syndrome: a case report and review of the literature.biliary atresia is a cholestatic disorder of infancy that is associated with other anatomic anomalies in approximately 20% of cases. These frequently are defects in situs determination and laterality, causing syndromes of heterotaxy and complex heart defects. The authors describe an infant with both biliary atresia and hypoplastic left heart syndrome (HLHS) in the absence of a laterality defect or evidence of any other morphologic defect. The presence of a hypoplastic left ventricle in a patient with biliary atresia has previously been reported only in the context of a heterotaxy syndrome. The coexistence of these 2 disorders raises etiologic considerations and represents a potential challenge for the surgical treatment of both conditions.- - - - - - - - - - ranking = 0.013419134820246keywords = situs (Clic here for more details about this article) |
6/15. Case of living donor liver transplantation in a patient with biliary atresia combined with situs inversus.Until recently, situs inversus was considered to be an absolute contraindication for liver transplantation. However, recent reports have suggested that situs inversus should not be considered a contraindication. This study presents a successful living donor liver transplantation performed in a 4-month-old male infant with biliary atresia and situs inversus. The surgical findings revealed abdominal situs inversus with polysplenia and an absent retrohepatic inferior vena cava and intestinal malrotation.- - - - - - - - - - ranking = 1.6keywords = situs inversus, inversus, situs (Clic here for more details about this article) |
7/15. Living-donor liver transplantation for situs inversus: 2 case reports.Two cases of living-donor liver transplantation performed in patients with situs inversus are reported. The authors discuss the operative management for a situs inversus recipient to undergo liver transplantation.- - - - - - - - - - ranking = 1.0529878394331keywords = situs inversus, inversus, situs (Clic here for more details about this article) |
8/15. biliary atresia with situs inversus: an experience shared.biliary atresia (BA) is a well-known entity and can present with multiple congenital anomalies. BA is one of the most common conditions in which pediatric liver transplant is performed. Identification of biliary atresia with situs inversus (SI) has not been documented in pakistan. We report two such cases. First was an eighty-day-old baby boy, icteric from day of birth. On further evaluation had dextrocardia, SI, gross hydronephrosis (HN) of left kidney and stasis at pelvi ureteric junction (PUJ). Liver biopsy showed biliary cirrhosis secondary to extra hepatic biliary atresia (EHBA). The second baby presented at two months of age. Ultrasound abdomen and hepatobiliary scintigraphy confirmed liver in left hypochondrium (SI) and findings suggestive of BA. echocardiography confirmed SI with mesocardia. In this paper we have described the association of BA with SI in two patients presenting at the pediatric gastroenterology, hepatology and nutrition unit.- - - - - - - - - - ranking = 1keywords = situs inversus, inversus, situs (Clic here for more details about this article) |
9/15. Management of a cadaveric orthotopic liver transplantation in a pediatric patient with complex congenital heart disease.Pediatric orthotopic liver transplantations (OLT) are commonly performed nowadays. Two primary reasons for OLT in children are complications from either extrahepatic biliary atresia (EHBA) or inborn errors of metabolism. However, congenital liver disease may be associated with significant other congenital abnormalities. We present a case of a successful OLT in a pediatric patient with a history of EHBA, situs inversus, and complex congenital heart disease. The cardiac anomalies include dextrocardia, absence of the atrial septum (single atrium), single atrioventricular valve (a-v canal), and an incomplete ventricular septum. Prior surgery include a Kasai procedure for EHBA, banding of the proximal main pulmonary artery, and Broviac catheter placement. We present the anesthesia concerns and management for this complicated case.- - - - - - - - - - ranking = 0.2keywords = situs inversus, inversus, situs (Clic here for more details about this article) |
10/15. Orthotopic transplantation of the liver in children with biliary atresia and polysplenia syndrome: report of two cases.biliary atresia is the most common indication for liver transplantation in infants and children, despite the advent of the Kasai operation. Coexisting anomalies, which have been noted in up to 27% of patients with biliary atresia, may form an association known as the "polysplenia syndrome," which includes (1) polysplenia, (2) midgut malrotation, (3) preduodenal portal vein, (4) absent prerenal inferior vena cava with azygos continuation, (5) situs inversus, (6) symmetric liver, (7) hepatic arterial anomalies, and (8) bilobed right lung with hyparterial bronchus. Two of 31 patients undergoing orthotopic liver transplantation for biliary atresia following failed portoenterostomy over the past 11 years manifested the polysplenia syndrome with absent prerenal inferior vena cava. The clinical course of these patients, constellation of anomalies, and technical adjustments required to perform liver transplantation are described. We do not believe that these complex congenital anomalies preclude liver transplantation.- - - - - - - - - - ranking = 0.2keywords = situs inversus, inversus, situs (Clic here for more details about this article) |
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