Cholangitis in a child due to biliary tract anomalies

Area of Science:

• Pediatric Gastroenterology and Hepatology
• Medical Imaging
• Infectious Diseases

Background:

• Congenital hepatic fibrosis (CHF) is a rare genetic disorder affecting the liver.
• Cholangitis and steatorrhea are serious complications that can impede growth and development in affected children.
• Radiographic evaluation is crucial for diagnosing biliary tree abnormalities in pediatric liver diseases.

Observation:

• A 29-month-old child presented with persistent cholangitis and steatorrhea secondary to congenital hepatic fibrosis.
• Radiographic imaging, specifically percutaneous transhepatic cholangiography, revealed communicating intrahepatic cavernous ectasias.
• This imaging procedure was confirmed as safe for children of this age group.

Findings:

• The child experienced significant steatorrhea and growth failure, atypical for common cholangitis presentations.
• Successful treatment with trimethoprim-sulfamethoxazole led to the resolution of both steatorrhea and growth failure.
• Bacteriologic diagnosis was achieved through direct liver tissue culture and duodenal intubation.

Implications:

• Percutaneous transhepatic cholangiography is a valuable and safe diagnostic tool for evaluating biliary anomalies in pediatric CHF.
• Trimethoprim-sulfamethoxazole can be an effective treatment for specific bacterial infections contributing to cholangitis and associated complications in CHF.
• Early diagnosis and targeted antimicrobial therapy can significantly improve outcomes, including growth and symptom resolution, in children with congenital hepatic fibrosis.