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Cystic biliary atresia or atretic choledochal cyst: A continuum in infantile obstructive cholangiopathy
Santosh Kumar Mahalik1, Suvradeep Mitra2, Susama Patra2
1Pediatric Surgery, All India Institute of Medical Sciences, Bhubaneswar, India.
Insights
Biliary atresia and choledochal cysts, both infantile obstructive cholangiopathies, may represent different manifestations of the same underlying pathology. This case highlights overlapping features, suggesting a potential continuum.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Developmental Biology
Background:
- Infantile obstructive cholangiopathy encompasses conditions like biliary atresia (BA) and choledochal cysts (CC).
- Distinguishing between BA and CC can be challenging due to overlapping clinical and imaging features.
Abstract:
Introduction: Both cystic biliary atresia and choledochal cyst present as infantile obstructive cholangiopathy. Methods: We detail an infant with congenital biliary dilatation and obstructive cholangiopathy where clinicoradiological features (antenatally imaged subhepatic cyst, early onset jaundice, intrahepatic dilated biliary radicals) suggested a choledochal cyst but operative findings (lack of a distal communication of cyst with duodenum) and histomorphological features (cicatricial collagen and myofibroblastic hyperplasia in the cyst wall; ductal plate malformation, ductular cholestasis and strong expression of CD56 in the liver) were those associated with biliary atresia. Conclusion: The observations support the contention that BA and CC may be interim entities in a continuum of manifestation of the same pathology.
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