PURPOSE: Forme fruste choledochal cyst (FFCC) is a choledochal cyst that has minimal or no dilatation of the extrahepatic bile duct (EHBD) and is associated with pancreaticobiliary malunion (PBMU). The authors reviewed the long-term outcome of their patients with FFCC. METHODS: Inpatient and outpatient records of 281 patients with choledochal cyst were reviewed. In this study, minimal dilatation of the EHBD was defined as its maximum diameter being less than 10 mm. RESULTS: There were 17 cases of FFCC identified. In all 17 patients, PBMU was present. The mean age at EHBD excision was 2.9 years. Fourteen patients had hepatico-jejunostomy, and three had hepatico-duodenostomy. The histology of the excised EHBD showed mucosal ulceration/sloughing (in 35.3% patients), fibrosis (52.9%), and inflammatory cell infiltration (41.2%). Over a mean postoperative follow-up period of 9.8 years, there have been no episodes of cholangitis or anastomotic stricture formation. CONCLUSIONS: The treatment of choice for FFCC in children is EHBD excision and hepatico-jejunostomy. There is little surgical morbidity if performed carefully.
PURPOSE: Forme fruste choledochal cyst (FFCC) is a choledochal cyst that has minimal or no dilatation of the extrahepatic bile duct (EHBD) and is associated with pancreaticobiliary malunion (PBMU). The authors reviewed the long-term outcome of their patients with FFCC. METHODS: Inpatient and outpatient records of 281 patients with choledochal cyst were reviewed. In this study, minimal dilatation of the EHBD was defined as its maximum diameter being less than 10 mm. RESULTS: There were 17 cases of FFCC identified. In all 17 patients, PBMU was present. The mean age at EHBD excision was 2.9 years. Fourteen patients had hepatico-jejunostomy, and three had hepatico-duodenostomy. The histology of the excised EHBD showed mucosal ulceration/sloughing (in 35.3% patients), fibrosis (52.9%), and inflammatory cell infiltration (41.2%). Over a mean postoperative follow-up period of 9.8 years, there have been no episodes of cholangitis or anastomotic stricture formation. CONCLUSIONS: The treatment of choice for FFCC in children is EHBD excision and hepatico-jejunostomy. There is little surgical morbidity if performed carefully.
Authors: Andrew J Murphy; Jason R Axt; Seth J Crapp; Colin A Martin; Gabriella L Crane; Harold N Lovvorn Journal: Pediatr Surg Int Date: 2012-04-21 Impact factor: 1.827