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The management of prenatally diagnosed choledochal cysts
T C Mackenzie1, L J Howell, A W Flake
1Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
Insights
Prenatally diagnosed biliary cysts require early surgical intervention for optimal outcomes. Prompt exploration is crucial to differentiate between biliary atresia and choledochal cysts, ensuring timely treatment for congenital biliary anomalies.
Area of Science:
- Pediatric Surgery
- Fetal Medicine
- Gastroenterology
Background:
- Congenital biliary tree abnormalities are rare but increasingly diagnosed prenatally.
- Improved imaging techniques enhance antenatal detection of biliary anomalies.
Purpose of the Study:
- To evaluate the prognosis of prenatally diagnosed biliary cystic lesions.
- To assess the outcomes of early surgical intervention for these conditions.
Main Methods:
- Retrospective review of 3 pediatric cases with antenatally detected biliary cystic lesions.
- Surgical exploration and treatment within two weeks of birth.
Main Results:
- Two patients had biliary atresia with cystic lesions, treated with Kasai procedures, showing good outcomes.
- One patient had a type I choledochal cyst and malrotation, treated with cystectomy and Ladd's procedure, with a good outcome.
- All infants were born at term with elevated bilirubin and confirmed cystic masses postnatally.
Conclusions:
- Prenatally diagnosed biliary cysts represent a distinct disease spectrum requiring early management.
- Distinguishing biliary atresia from choledochal cysts prenatally is challenging, necessitating early surgical exploration.
- Early surgical intervention, in the absence of severe anomalies, leads to excellent outcomes for prenatally diagnosed biliary cysts.
Background:
Although congenital biliary tree abnormalities are uncommon in the United States, more are being diagnosed antenatally with improved imaging techniques.
Methods:
To determine the prognosis of prenatally diagnosed biliary disease, the authors reviewed the treatment of 3 children who had biliary cystic lesions found during routine prenatal ultrasonography.
Results:
All 3 children were born at term. They had elevated bilirubin levels, and postnatal ultrasound scans confirmed the presence of a biliary cystic mass. They underwent exploration within 2 weeks of life. At operation, 2 children were found to have biliary atresia with a cystic biliary lesion. They underwent Kasai procedures and are doing well at 5 and 9 months of age. The third child was found to have a type I choledochal cyst and malrotation at exploration. He underwent cystectomy with Roux-en-Y hepaticojejunostomy and a Ladd's procedure and is doing well at 3 years of age.
Conclusions:
Prenatally diagnosed biliary cysts represent a different spectrum of disease than those diagnosed later in life. The sequelae of biliary disease start before birth, and early operation may be necessary to achieve a good outcome. Because it is impossible to distinguish between choledochal cysts and biliary atresia on antenatal ultrasound scan or magnetic resonance imaging, children with presumed choledochal cysts should undergo early exploration to rule out potential biliary atresia. Excellent outcome is possible with early operation in the absence of severe associated anomalies.