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Forme fruste choledochal cyst.

Y K Sarin1, M Sengar, A S Puri

  • 1Department of Pediatric Surgery, Maulana Azad Medical College and Associated Lok Nayak Hospital and Department of Gastroenterology, G.B. Pant Hospital, New Delhi 110 002, India.

Indian Pediatrics
|December 13, 2005
PubMed
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Forme fruste choledochal cyst (FFCC) is a rare condition presenting as pancreatitis. Surgical intervention involving pancreatico-biliary disconnection and reconstruction led to successful outcomes in two pediatric patients.

Area of Science:

  • Gastroenterology and Hepatobiliary Surgery
  • Pediatric Surgery
  • Medical Diagnostics

Background:

  • Pancreatico-biliary maljunction (PBMJ) is a congenital anomaly of the pancreatic and bile ducts.
  • Forme fruste choledochal cyst (FFCC) represents a less common variant of choledochal cysts, often presenting with subtle findings.
  • Early diagnosis and management are crucial for preventing complications associated with PBMJ and FFCC.

Observation:

  • Two pediatric patients presented with clinical features suggestive of pancreatitis.
  • Diagnostic imaging revealed minimal dilatation of the extrahepatic biliary duct (EHBD) in association with PBMJ.
  • A high index of clinical suspicion was necessary for identifying this rare condition.

Findings:

  • The patients were diagnosed with Forme fruste choledochal cyst (FFCC) secondary to PBMJ.

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  • Surgical management involved pancreatico-biliary disconnection.
  • Reconstruction of the biliary channel was achieved using an enteric conduit.

    • Implications:

    • This case series highlights the importance of recognizing FFCC in pediatric patients with pancreatitis and PBMJ.
    • Surgical disconnection and reconstruction offer a viable treatment option for FFCC.
    • Further research into the long-term outcomes of FFCC management is warranted.