Document Detail

Biliary obstruction in infants with cystic fibrosis requiring Kasai portoenterostomy.
MedLine Citation:
PMID:  9044117     Owner:  NLM     Status:  MEDLINE    
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.
S K Greenholz; B Krishnadasan; C Marr; R Cannon
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of pediatric surgery     Volume:  32     ISSN:  0022-3468     ISO Abbreviation:  J. Pediatr. Surg.     Publication Date:  1997 Feb 
Date Detail:
Created Date:  1997-05-02     Completed Date:  1997-05-02     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0052631     Medline TA:  J Pediatr Surg     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  175-9; discussion 179-80     Citation Subset:  IM    
Division of Pediatric Surgery, University of California, Davis Medical Center, USA.
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MeSH Terms
Biliary Atresia / complications,  surgery*
Cystic Fibrosis / complications*
Fatal Outcome
Gallbladder / pathology
Hepatic Duct, Common / pathology
Intestinal Obstruction / complications
Jaundice / etiology
Portoenterostomy, Hepatic*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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