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Prenatal hepatic hilar cyst: choledochal cyst or cystic biliary atresia?
Maria Manousi, Maria Yvelise De Verney, Kanavaki Aikaterini, Siafakas Constantinos, Maria Klavdianou, Christos Salakos
IASO Children's Hospital, Athens, Greece
Introduction
Prenatal hepatic hilar cysts present a diagnostic challenge in differentiating choledochal cysts from cystic biliary atresia, due to their distinct surgical timing and outcomes.
Case Presentation
A female neonate born full-term at a district hospital was prenatally diagnosed at 32 weeks with a hepatic hilar cyst (18.5 × 17.8 mm). Postnatally, she was followed up in the outpatient neonatal service with a suspected diagnosis of a choledochal cyst. She breastfed well and gained weight until two weeks of age, when the parents noted stool discoloration and mild jaundice.
Laboratory tests showed elevated AST (154 U/L), ALT (95 U/L), γGT (682 U/L), total bilirubin (7.5 mg/dL), and direct bilirubin (6.3 mg/dL), with normal ALP. She was referred to our tertiary center for further evaluation.
Ultrasound on day 20 revealed a 22 × 17 mm hilar cyst with a thickened wall, continuous with a beaded gallbladder that was non-contractile. Increased periportal echogenicity was noted, without a triangular cord sign. MRCP showed an elongated gallbladder communicating with a 21 × 22 mm cystic lesion, suggestive of a type I choledochal cyst, without intrahepatic duct dilation. The intrapancreatic bile duct was not seen.
Due to inconclusive imaging and worsening liver tests, an intraoperative cholangiogram and liver biopsy were performed on day 30. Contrast injection outlined a cystic lesion with irregular, atretic intrahepatic ducts (cloudy pattern) and no opacification of the common bile duct or duodenum, consistent with cystic biliary atresia. A Kasai portoenterostomy was performed, and biopsy confirmed the diagnosis. Jaundice resolved postoperatively, with direct bilirubin falling to 0.2 mg/dL by four months.
Conclusion
Intraoperative cholangiography and liver biopsy are the gold standards for diagnosing biliary atresia and should be promptly performed when diagnosis is uncertain due to time-sensitive treatment outcomes.