1/3. biliary atresia associated with meconium peritonitis caused by perforation of small bowel atresia.BACKGROUND/PURPOSE: This report describes our experiences with 5 cases of biliary atresia associated with meconium peritonitis caused by perforation of small bowel atresia. methods: A review of medical records was undertaken in an effort to recognize cases of biliary atresia associated with meconium peritonitis. RESULTS: Five patients of 171 with biliary atresia (2.9%) were detected to have meconium peritonitis caused by perforation of small bowel atresia. The biliary atresia was not suspected during the initial operation for meconium peritonitis. Total parenteral nutrition (TPN) made it difficult to make an early differential diagnosis of biliary atresia because of the presence of TPN-associated cholestatic jaundice, and the Roux-en-Y limb used for hepatic portoenterostomy could not be made long enough to prevent cholangitis caused by preexisting short bowel. The main complications were severe, intractable cholangitis, short bowel syndrome with malnutrition; TPN-associated liver injury; and wound problems. Two patients died of ascending cholangitis, 1 patient of liver failure that was exacerbated by TPN-associated liver injury, and 1 patient is awaiting a liver transplant. Only 1 patient is in good health, being anicteric and showing normal growth and development. CONCLUSIONS: biliary atresia is evidently closely associated with meconium peritonitis caused by perforation of small bowel atresia. The management of these patients is more difficult than that of patients with the usual form of biliary atresia, because of the necessity for a long period of TPN and the combined short bowel syndrome. The ideal management of these conditions has yet to be determined.- - - - - - - - - - ranking = 1keywords = meconium (Clic here for more details about this article) |
2/3. biliary atresia associated with meconium peritonitis.biliary atresia, malrotation, meconium peritonitis and transient hypothyroidism are occasionally seen in neonatal infants. biliary atresia associated with malrotation has been reported in some patients with polysplenia syndrome, but biliary atresia associated with meconium peritonitis has only been described by a few investigators. Here we present a case of meconium peritonitis due to malrotation with volvulus, followed by biliary atresia and transient hypothyroidism during early infancy.- - - - - - - - - - ranking = 0.77777777777778keywords = meconium (Clic here for more details about this article) |
3/3. Biliary obstruction in infants with cystic fibrosis requiring Kasai portoenterostomy.cystic fibrosis is associated with an inspissated bile syndrome producing cholestasis secondary to plugging of macroscopically normal bile ducts. In extreme neonatal forms, with early profound intrahepatic cholestasis, the process can be associated with a marked decrease in ductal diameter, varying from hypoplasia to atresia. From 1990 to 1995 three infants were identified with cystic fibrosis, persistent jaundice, and complete absence of biliary excretion despite expectant and conservative treatment including choleretics and surgical biliary irrigation. Abdominal ultrasounds showed contracted gallbladders and no evidence of dilated ducts. Liver biopsy results in two infants showed portal fibrosis, paucity of bile ducts, and minimal inflammation. The third infant had moderate inflammation, bile duct replication, and plugging. Two infants had undergone intestinal resection followed by hyperalimenation for complications of meconium ileus in the newborn period. Surgical exploration was undertaken at 7 to 12 weeks of age. Gross findings were typical of biliary atresia with microgallbladders and nonpatency of the cystic duct. Cholangiograms failed to document ductal patency in two patients who were then treated with a Kasai portoenterostomy. The third infant had patent hypoplastic ducts and underwent only biliary irrigation. Although bile flow was transiently achieved, jaundice recurred, and at reexploration at 16 weeks of age a Kasai poroenterostomy was performed. Histological review of the biliary specimens showed microscopically patent ducts in two patients and proximal patency and distal atresia in the third. All the ducts had mural fibrosis with cystic changes. Bile drainage was achieved in each instance, although in one patient with hypoplastic ducts scant output of highly concentrated bile proved insufficient to arrest progressive liver failure. The subsequent two patients responded with resolution of hyperbilirubinemia and normalization of liver function. They remain free of biliary complications at 30 and 40 months postoperatively. This manifestation of cystic fibrosis in infants is suggested by prolonged jaundice unresponsive to choleretics, nondilated bile ducts and gallbladder on ultrasound, absent biliary excretion on nuclear scan, and characteristic liver biopsy. Exploration is warranted, and discovery of atrophic bile ducts may be best managed with reconstruction.- - - - - - - - - - ranking = 0.11111111111111keywords = meconium (Clic here for more details about this article) |