Cases reported "Biliary Atresia"

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11/43. A complication associated with the use of neonatal liver in orthotopic liver transplantation.

    BACKGROUND: Neonatal livers have been associated with a higher incidence of vascular complications but are nevertheless used because of donor shortage. AIMS: We report a complication of hepatic venous outflow obstruction because we retained the neonatal retrohepatic cava from the first transplant for retransplantation using a left lateral segment. methods: Technical and post-operative complications associated with a small "native" inferior cava and transplantation are described. RESULTS: Successful treatment with balloon angioplasty resulted in re-establishing the flow in the hepatic venous outflow tract. The child is well 3 years on with stable graft function. CONCLUSION: Percutaneous transluminal angioplasty is an effective non-surgical therapy for post liver transplant hepatic venous outflow obstruction. Replacement of the inferior venacava rather than retaining neonatal venacava may be a better option during retransplantation.
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12/43. Intrahepatic biliary cysts in biliary atresia in the era of liver transplantation.

    OBJECTIVES: The development of intrahepatic biliary cysts (IBC) after Kasai operation in patients with biliary atresia (BA) is recognized as an important problem; however, management strategy for IBC has not been clarified, particularly in the light of the increased use of liver transplantation. methods: Forty consecutive BA patients underwent hepatic portoenterostomy during 18 years from 1983 to 2000. We compared the clinical course and prognosis of the patients who developed IBC with those who did not. RESULTS: Seven of the 40 patients developed IBC. Three patients had type A (non-communicating cyst) and three patients had type C (multiple cystic dilation) IBC, and the remaining patients had type B (communicating cyst). Of the 7 patients, one patient underwent successful internal intestinal drainage, and one patient died of complications at the time of internal intestinal drainage. Three patients underwent liver transplantation due to either hepato-pulmonary syndrome (one case) or liver failure (two cases). One patient with IBC with liver failure was judged to require transplant, but was found to have pulmonary hypertension and was thus not a candidate. The remaining patient has survived without jaundice for 21 months postoperatively. Two of 21 patients with good initial bile drainage and without IBC underwent liver transplantation. The percentage of patients undergoing transplant was significantly higher in the group with IBC than in the group without IBC (P < 0.05). CONCLUSIONS: IBC was associated with worsening liver function. Previously, IBC was treated using internal/external drainage, or the patients were observed without treatment, with limited success. We now consider it reasonable to carry out liver transplantation in patients with long-standing IBC.
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13/43. 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.
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14/43. Congenital absence of the portal vein in liver transplantation for biliary atresia.

    BACKGROUND: Extrahepatic biliary atresia is the most common indication for liver transplantation in children. Coexistent congenital anomalies are common (25%), but the combination of polysplenia syndrome (10%) and absence of portal vein is rare. AIMS: We report a case of successful reduced size liver transplantation on a 13-month-old girl with extrahepatic biliary atresia, polysplenia syndrome and congenital absence of the portal vein. methods: Technical and post-operative problems related to absence of the portal vein and polysplenia are described and the literature reviewed. RESULTS: The donor portal vein was successfully anastomosed to the confluence of the superior mesenteric vein and splenic vein. The child is well with good graft function at a follow-up of 20 months. CONCLUSION: Absence of the portal vein and polysplenia syndrome is not a contraindication for liver transplantation although an increased post-operative morbidity may be expected.
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15/43. Use of a silastic silo for closure of the abdominal wall in a pediatric patient receiving a cadaveric split liver.

    Presented is the successful management of a difficult abdominal wall closure after pediatric liver transplantation. A 5-week-old boy with biliary atresia underwent urgent cadaveric split liver transplantation. The left lateral segment of an adult donor was utilized. Postoperatively, abdominal skin and fascia could not be closed. A SILASTIC (Dow Corning, Midland, MI) silo was applied, and complete closure was possible 6 days later.
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16/43. Simultaneous correction of biliary atresia and small bowel atresia in the neonate.

    The coexistence of biliary atresia and small bowel atresia has been described. However, attempts to correct the biliary atresia at the same time as small bowel atresia repair have not been detailed. In 1986, a 2,170-g female was delivered at 36 weeks' gestation. Postpartum, duodenal obstruction was suspected after oral feedings were not tolerated. At 37 hours after birth, laparotomy showed unsuspected biliary atresia in addition to intestinal malrotation, duodenal web, and type IIIb jejunal atresia with an apple-peel configuration of the upper jejunum and mesentery supplied by the superior mesenteric artery with normal small bowel length and normal ileal mesentery. A modified Roux-en-Y hepatic portoenterostomy was performed using the blind end of the apple-peel configuration of jejunum, with a side-to-side duodenojejunostomy 25 cm from the portoenterostomy. Microscopic examination of the fibrous extrahepatic biliary tissue showed a disarray of small bile ductules. The maximal ductal size at the portoenterostomy site was 600 microns. A liver biopsy specimen showed normal architecture with eosinophils infiltrating the portal triads and periductal areas. Postoperatively, she has maintained normal growth and development with no episodes of cholangitis. Liver biopsies at 1 year and 3 years were normal. At 4 years, total and conjugated bilirubin, serum proteins, liver enzymes, coagulation factors, and trace elements were normal. We conclude that simultaneous correction of biliary atresia and small bowel atresia can be accomplished in the neonate with excellent early results.
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17/43. Successful pregnancy outcome in a patient with congenital biliary atresia.

    Congenital biliary atresia is one of the most common congenital anomalies of the biliary tree. Without surgical correction, two-thirds of all cases of extrahepatic biliary atresia are fatal within 18 months. Historically, the preferred surgical procedure to correct this anomaly has been the Kasai procedure. Surgical treatment of congenital biliary atresia is associated with substantial perioperative morbidity and mortality, as well as long-term sequelae. Portal hypertension and hypersplenism can complicate the course of up to 50% of patients who survive for more than 2 years. Not surprisingly, few of these patients reach adulthood. We report the case of a woman with congenital biliary atresia surgically corrected with the Kasai operation who subsequently reached adulthood and became pregnant. Despite a prenatal course complicated by portal hypertension, bleeding esophageal varices, and hypersplenism, the woman delivered a healthy infant at 36.5 weeks' gestation.
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18/43. A novel approach to the management of late-onset liver failure in biliary atresia.

    PURPOSE: Late-onset hyperbilirubinemia in patients who have undergone a successful portoenterostomy (PE) for biliary atresia (BA) is usually considered evidence of ongoing severe liver failure. The authors recently have treated 2 patients who had acute hyperbilirubinemia years and months after a successful PE and had dilated intrahepatic cysts. A combined operative and percutaneous approach reestablished drainage and a reduction in their bilirubin levels. methods: Data from 2 cases of BA and late-onset hyperbilirubinemia from obstruction were reviewed and analyzed. RESULTS: Two patients (15-year-old boy and a 2.5-year-old girl) presented with increasing serum bilirubin after a PE for BA in infancy. Both had extensive preoperative workup, which showed intrahepatic biliary dilatation in one and a large bile lake in the other. They underwent attempted percutaneous transhepatic cholangiography and stenting, followed by an intraoperative transhepatic approach in which the dilated ducts were connected to the PE. A rapid and sustained reduction in the serum bilirubin level was noted in these patients. CONCLUSIONS: When patients with BA after a successful PE present with sudden onset of hyperbilirubinemia, imaging for biliary obstruction should be carried out. If biliary dilatation is found, then a combined radiologic and operative approach may help improve the bile flow and delay the need for liver transplant.
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ranking = 4
keywords = operative
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19/43. Sequential therapy in a case of extrahepatic biliary atresia associated with ventricular septal defect: liver transplantation followed by surgical closure of the septal defect.

    patients with extrahepatic biliary atresia (EHBA), the commonest indication for liver transplantation in pediatric cases, may have associated congenital anomalies, e.g. interventricular septal defect (IVSD). Even if there is a well established therapeutical algorithm for a ventricular septal defect as an isolated disease, the surgical approach raises special problems in a liver transplanted patient. We present the case of a little girl with EHBA and IVSD. The patient underwent a living related liver transplantation when she was 1 year old. Persistence of the symptomatic IVSD required open heart surgery, performed 9 months posttransplantation. The decision to perform the closure of the IVSD was difficult, yet it proved to be beneficial; the child had an uneventful postoperative course, with remission of the symptoms and a good late outcome. To our knowledge, this is the first case of a living-related liver transplantation patient undergoing open heart surgery for IVSD closure.
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20/43. Liver retransplantation in an infant requiring cavoportal hemi transposition.

    Historically inability to achieve portal inflow to the liver allograft operatively was felt to be a contraindication to orthotopic liver transplantation (OLTx). Cavoportal hemi transposition has been utilized more recently in adult OLTx recipients but rarely in pediatric recipients. Here we report the case of a 10-month-old male with biliary atresia, who required urgent retransplantation with an in situ split cadaver donor allograft for failure of his first liver allograft from portal vein thrombosis. At the time of retransplantation, cavoportal hemi transposition was required to effect portal vein inflow to the allograft because of extensive thrombosis of the recipients portomesenteric venous system.
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