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Congenital biliary tract dilatation in infancy and childhood--74 cases experience
1Department of Pediatrics, Chang Gung Memorial Hospital, Taipei, Taiwan, R.O.C.
Insights
Congenital biliary tract dilatation (CBTD) affects infants and children, with Type Ia being most common. Surgical cyst excision is effective, though reoperation and complications like sepsis can occur.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Medical Imaging
Background:
- Congenital biliary tract dilatation (CBTD) is a rare condition affecting the bile ducts.
- This study reviews a twelve-year experience with CBTD, focusing on diagnosis, classification, and management.
Purpose of the Study:
- To analyze the clinical characteristics, diagnostic methods, and surgical outcomes of congenital biliary tract dilatation.
- To evaluate the effectiveness of cyst excision and biliary tract reconstruction in managing CBTD.
Main Methods:
- Retrospective review of 74 cases of CBTD diagnosed between 1981 and 1992.
- Preoperative diagnosis was confirmed using sonography.
- Cases were classified according to Todani's system, and associated conditions were noted.
Main Results:
- Abdominal pain, jaundice, and vomiting were common symptoms.
- Type Ia was the most frequent classification (40/74), followed by Type IV-A (25/74).
- Cholelithiasis and perforation were common associated conditions; sonography achieved 100% diagnostic accuracy.
Conclusions:
- Congenital biliary tract dilatation requires surgical intervention, with cyst excision and reconstruction being the standard treatment.
- While generally effective, complications such as sepsis and cholangitis can lead to mortality, necessitating careful postoperative management.
Abstract:
This report presented a twelve-year experience from 1981 to 1992. Seventy-four cases of congenital biliary tract dilatation were at diagnosed an age of 6 days to 16 years. Twenty-two cases were infants. There were 54 females and 20 males. The ratio of female to male was 2.7:1. The classic triad of abdominal pain, jaundice and a palpable mass was seen in eleven cases (14.9%). Most children suffered from abdominal pain (50/74), vomiting (45/74), anorexia (42/74) and jaundice (34/74). Prolonged jaundice was the main symptom in infancy (15/22). A long common pancreatico-biliary channel was seen in six cases (6/47); the bile amylase level was elevated in five cases (5/20), one patient had a complex union with obstructive jaundice. All these cases were diagnosed by preoperative sonography accurately (100%). According to the Todani's classification, type Ia was the most common (40/74), followed by type IV-A (25/74) and type Ic (8/74). Cholelithiasis (13/74), perforation (9/74), and atresia/stenosis of distal choledochus (8/74) were the most common associated conditions. Cyst excision with biliary tract reconstruction was performed in all cases. Reoperation was needed in ten cases. Two cases died postoperatively due to sepsis and cholangitis induced hepatic failure.