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Extrahepatic biliary atresia associated with cyanotic congenital heart disease. Three case reports and a review
Insights
This study reviews three infants with extrahepatic biliary atresia and cyanotic congenital heart disease. The combined conditions caused atypical skin discoloration, delaying diagnosis and impacting outcomes.
Area of Science:
- Pediatric Surgery
- Neonatology
- Cardiology
Background:
- Extrahepatic biliary atresia and cyanotic congenital heart disease are serious conditions in infants.
- Simultaneous occurrence of these diseases is rare but presents diagnostic challenges.
Observation:
- Three infants presented with both conditions, exhibiting unusual green-hued skin discoloration.
- This atypical presentation masked the early detection of either biliary atresia or heart disease.
Findings:
- Surgical correction of cardiac lesions was possible in all infants.
- Two infants succumbed to hepatic disease despite interventions; one achieved satisfactory bile drainage post-portoenterostomy.
Implications:
- Clinicians should consider this rare concurrence when evaluating infants with unusual skin discoloration.
- Early recognition and management of both conditions are crucial for improved infant outcomes.
Abstract:
Three patients with extrahepatic biliary atresia associated with cyanotic congenital heart disease are presented and their clinical courses reviewed. In these patients, the coincidence of cyanosis and jaundice caused an atypical green-appearing cutaneous discoloration which masked the early clinical detection of biliary atresia in one infant and cyanotic heart disease in the other two. In all infants, either partial or complete correction of the congenital cardiac lesion was possible. Two infants died from hepatic disease despite attempted surgical revision and one has satisfactory bile drainage one month following portoenterostomy. Awareness of the rare concurrence of cyanotic congenital heart disease and extrahepatic biliary atresia is helpful to the clinician when evaluating infants with atypical cutaneous discoloration. Early oversight of either underlying disease process can be avoided, and early evaluation and management of both entities can be possible.