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Antenatal diagnosis of choledochal cyst
J D Bancroft1, J C Bucuvalas, F C Ryckman
1Division of Gastroenterology and Nutrition, Children's Hospital Medical Center, Cincinnati, Ohio.
Insights
Antenatal diagnosis of choledochal cysts in infants via ultrasonography is feasible. Prompt surgical intervention leads to excellent long-term outcomes, even with advanced liver fibrosis.
Area of Science:
- Pediatric Surgery
- Fetal Medicine
- Gastroenterology
Background:
- Congenital choledochal cysts are rare bile duct malformations.
- Antenatal diagnosis is crucial for timely management.
- Associated fetal findings can include abdominal masses.
Purpose of the Study:
- To evaluate the feasibility of antenatal ultrasonography for choledochal cyst diagnosis.
- To review clinical presentation, management, and outcomes of infants with choledochal cysts.
Main Methods:
- Retrospective review of five infants with antenatally diagnosed choledochal cysts.
- Analysis of seven previously reported cases.
- Ultrasonography, intraoperative cholangiography, liver histology, and surgical outcomes were assessed.
Main Results:
- All diagnosed infants had cystic dilatation of the common bile duct (Type 1).
- Jaundice was common in neonates, but palpable masses were infrequent.
- Distal common bile duct obstruction and liver fibrosis were prevalent, yet surgical correction yielded significant improvement.
Conclusions:
- Ultrasonography is effective for antenatal detection of choledochal cysts.
- Neonates with suspected choledochal cysts require prompt surgical evaluation and management.
- Early surgical intervention ensures excellent long-term prognosis in infants.
Abstract:
We report five infants in whom antenatal diagnosis of choledochal cyst was established by ultrasonography, and we review the seven previously reported cases. All but one infant had cystic dilatation of the common bile duct (type 1 cysts), and all infants were diagnosed during the second or third trimester. Eight of 12 infants (67%) developed jaundice in the first few days of life, but only 25% had a palpable abdominal mass. Seven of nine infants (78%) demonstrated complete obstruction of the distal common bile duct on intraoperative cholangiography. Liver histology was available for six patients. Five of six had cirrhosis or fibrosis with bile duct proliferation. All of the infants with fibrosis or cirrhosis had distal common bile duct obstruction. Despite liver biopsy findings of extensive fibrosis plus ascites with failure to thrive in one of our patients, all five patients demonstrated clinical and biochemical improvement following surgical excision and porto- or choledochoenterostomy. All were free of symptoms by 6 months of age. Congenital choledochal cyst should be considered in the differential diagnosis of any sonolucent abdominal mass of the fetus. Neonates with distal common bile duct obstruction and fibrosis in association with presumed choledochal cyst should have prompt surgical exploration, intraoperative cholangiography, and close postoperative follow-up. The long-term outcome with prompt surgical correction is excellent.