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Laparoscopic Choledochal Cyst Excision and Roux-en-Y Choledochojejunostomy in Adults
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Congenital choledochal cyst in an infant with cystic fibrosis

Parviz Tabatabaie1, Gholam-Hossein Fallahi, Fatemeh Farahmand

  • 1Center of Excellence for Pediatrics, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.

Annals of Hepatology
|June 9, 2009
PubMed

Insights

Congenital choledochal cysts are rare biliary duct malformations. This case highlights a misdiagnosed infant with cystic fibrosis presenting with respiratory distress and an abdominal mass, ultimately diagnosed as a choledochal cyst.

Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Medical Diagnostics

Background:

  • Congenital choledochal cysts are rare congenital malformations of the biliary ductal system.
  • They are the most common malformation of the extrahepatic biliary ducts.
  • Early diagnosis is crucial but often challenging due to infrequent occurrence.

Observation:

  • A 4-month-old infant presented with respiratory distress and decreased consciousness.
  • Physical examination revealed a palpable mass in the right upper quadrant of the abdomen.
  • Sonography identified a cystic structure consistent with a choledochal cyst.

Findings:

  • The infant was diagnosed with cystic fibrosis.
  • The choledochal cyst diagnosis was confirmed through further evaluation.
  • The co-occurrence of cystic choledochal malformation and cystic fibrosis in an infant is noteworthy.

Implications:

  • This case underscores the importance of considering choledochal cysts in infants presenting with respiratory and abdominal symptoms.
  • It highlights the potential for misdiagnosis due to the rarity of choledochal cysts and the complexity of co-occurring conditions like cystic fibrosis.
  • Increased awareness and diagnostic vigilance are necessary for timely management of biliary duct malformations in pediatric patients.

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