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Biliary atresia with choledochal cyst: implications for classification
Aleixo M Muise1, Dan Turner, Eytan Wine
1Division of Gastroenterology, Hepatology & Nutrition, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Biliary atresia (BA) associated with choledochal cyst (CC) represents a distinct subtype. Early antenatal diagnosis is crucial, as type 3 BA with CC shows poorer outcomes compared to type 1.
Area of Science:
- Pediatric Surgery
- Hepatology
- Developmental Biology
Background:
- Biliary atresia (BA) and choledochal cysts (CC) are congenital biliary tract malformations.
- Current classification systems for BA may not fully encompass all presentations, particularly those diagnosed antenatally.
- Investigating the association between BA and CC is essential for refining diagnostic and prognostic models.
Observation:
- A case of antenatal diagnosis of CC associated with BA is presented.
- A literature review identified 88 cases of BA associated with CC.
- Antenatal diagnosis of BA with CC was associated with a low incidence of congenital anomalies.
Findings:
- BA associated with CC predominantly presents as type 1 BA (common bile duct atresia).
- Type 3 BA (porta hepatis atresia) in this cohort was linked to significantly poorer and earlier adverse outcomes compared to type 1.
- Patients with type 3 BA were over five times more likely to experience poor outcomes.
Implications:
- BA associated with CC may represent a unique subtype requiring tailored management.
- Antenatal diagnosis supports classifying these cases within an embryonic BA spectrum.
- Further research into genetic factors may refine BA classification and predict outcomes.
Background & Aims:
To illustrate the limitations of the embryonic and perinatal classification system of biliary atresia (BA), we present a child with an antenatal diagnosis of choledochal cyst (CC) associated with BA and review the published literature.
Methods:
Medline and Pubmed were searched for "BA and CC," "cystic biliary atresia," "BA and cysts," and "biliary cystic malformations."
Results:
A 7-week-old with an antenatal diagnosis of CC was found to have BA associated with CC. The literature search identified 88 cases of BA with CC. Sixty-seven cases had type 1 BA (atresia of the common bile duct), 2 had type 2 BA (atresia of the common hepatic duct), and 19 had type 3 BA (atresia of the porta hepatis). Of the 27 cases of antenatal diagnosis of BA with CC only 1 had associated congenital anomalies. Outcome analysis showed poor outcomes were significantly more common (P = .009) and occurred earlier (P = .0249) in patients with type 3 BA. Children with type 3 BA were 5.4 times more likely to develop poor outcomes compared with type 1 (hazard ratio, 5.4; 95% confidence interval, 1.03-27.8).
Conclusions:
BA associated with CC forms a distinct subtype of BA, characterized by a preponderance of type 1 BA, a relatively good clinical outcome after surgery, and an absence of associated congenital anomalies. Antenatal diagnosis of many affected infants supports their inclusion within the embryonic BA group and suggests that a broader interpretation of the embryonic phenotype and further classification of BA based on genetic susceptibility may be required.
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