Discussion :
This child has arteriohepatic dysplasia {hepatic – bile duct paucity` arterial – pulmonary stenosis} which is also known as syndromic bile duct paucity or Alagille syndrome. It consists of a paucity of interlobular bile ducts with chronic cholestasis. Patients with this syndrome typically have the following major features: peculiar facies, chronic cholestasis, butterfly-like vertebral arch defects, and peripheral pulmonary artery hypoplasia or stenosis` either isolated or associated with complex cardiovascular abnormalities.
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