Forme Fruste Choledochal Cysts in Children: Clinical Presentation and Treatment Outcomes-A Retrospective Multicenter...

Area of Science:

• Pediatric Surgery
• Gastroenterology
• Hepatobiliary Surgery

Background:

• Forme fruste choledochal cyst (FFCC) presents with minimal extrahepatic bile duct dilatation, often missed on ultrasound.
• FFCC shares risks of inflammation and malignancy with classic choledochal cysts.
• Anomalous pancreaticobiliary junction (APBJ) is frequently associated with FFCC.

Purpose of the Study:

• To present the management experience of FFCC in pediatric patients from two tertiary centers.
• To evaluate the clinical success and long-term outcomes of surgical treatment for FFCC.
• To highlight the importance of considering FFCC in pediatric patients with unexplained biliary symptoms.

Main Methods:

• Retrospective analysis of pediatric patients treated for FFCC between 2008 and 2023.
• Evaluation of clinical success defined by symptom resolution and absence of major complications.
• Surgical procedures included Roux-en-Y hepatico-jejunostomy or hepatico-duodenostomy with biliary reconstruction.

Main Results:

• Fourteen children underwent surgical treatment for FFCC, with a mean age of 5.3 years.
• Roux-en-Y hepatico-jejunostomy was performed in 13 patients; one had hepatico-duodenostomy.
• No cases of cholangitis or anastomotic stricture were observed during a mean follow-up of 6.2 years; 14.3% had minor wound infections.

Conclusions:

• FFCC poses diagnostic challenges due to subtle imaging and non-specific symptoms.
• Surgical excision and biliary reconstruction, typically Roux-en-Y hepatico-jejunostomy, offer safe and effective treatment for FFCC.
• Active consideration of FFCC is vital in pediatric patients with unexplained pancreatitis or biliary issues, irrespective of ductal dilatation, to prevent severe complications.