Impact of age at diagnosis on clinical features in children with anomalous arrangement of the pancreaticobiliary duct

Abstract

Aims: In patients with an anomalous arrangement of the pancreaticobiliary duct (AAPBD), clinical presentations may differ between infants and older children. The optimal timing of surgery remains controversial, particularly in early infancy. The aim of this study was to evaluate the clinicopathological features and clinical outcomes using comparative methods between infants and older cases. MATERIALS AD METHODS: From 1983 to 2007, a total of 85 consecutive children with AAPBD were treated at our institute. They included 46 with the cystic type, 33 with the fusiform type, and 6 with the non-dilatation type. These patients were divided into 2 age groups: "infant" (n=9), <12 months old; and "older", >1 year old (n=76). A retrospective study was performed.

Results: Mean age was 5.2 months (range, 8 days-11 months) in the infant group and 5.2 years (range, 1.2-17.3 years) in the older group. Jaundice was significantly more frequent in the infant group ( P<0.05), whereas abdominal pain was more common in the older group ( P<0.001). Bleeding tendencies such as cranial hemorrhage or bloody stools were noted in only 3 infants. In terms of liver histology, liver cirrhosis was observed in 2 infants, one of whom was a 3-month-old girl with severe jaundice resulting in living-donor liver transplantation, despite bile drainage. A single postoperative death occurred due to an adenocarcinoma arising in a choledochal cyst in a 12-year-old girl.

Conclusions: Problems characteristic of infantile AAPBD were a severe bleeding tendency and irreversible liver cirrhosis, which could develop as young as 3 months old. The surgical recommendation for infantile AAPBD is thus early surgery before the age of 3 months to prevent liver failure.