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Biliary obstruction in infants with cystic fibrosis requiring Kasai portoenterostomy
S K Greenholz1, B Krishnadasan, C Marr
1Division of Pediatric Surgery, University of California, Davis Medical Center, USA.
Insights
Cystic fibrosis can cause severe neonatal cholestasis due to bile duct issues. Surgical reconstruction of atrophic bile ducts in infants with cystic fibrosis can lead to improved liver function and jaundice resolution.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Medical Genetics
Background:
- Cystic fibrosis (CF) is linked to inspissated bile syndrome, causing cholestasis and bile duct plugging.
- Neonatal CF can present with profound intrahepatic cholestasis, potentially leading to bile duct hypoplasia or atresia.
Observation:
- Three infants with CF presented with persistent jaundice and absent biliary excretion despite conservative treatment.
- Ultrasound revealed contracted gallbladders and non-dilated bile ducts; liver biopsies showed portal fibrosis and bile duct paucity or plugging.
- Two infants had undergone intestinal resection for meconium ileus complications.
Findings:
- Surgical exploration revealed findings consistent with biliary atresia in all three infants.
- Two infants underwent Kasai portoenterostomy after failed cholangiograms, with subsequent resolution of jaundice and normalized liver function.
- One infant with hypoplastic ducts had transient bile flow but progressive liver failure, despite Kasai portoenterostomy.
Implications:
- Prolonged jaundice unresponsive to choleretics, with specific ultrasound and biopsy findings, suggests this CF manifestation in infants.
- Surgical exploration is warranted for suspected biliary atresia in CF infants.
- Bile duct reconstruction, such as Kasai portoenterostomy, can be effective in managing biliary complications in cystic fibrosis.
Abstract:
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.