Syndromic paucity of
bile ducts or "
Alagille syndrome" is characterized by peculiar
facies, chronic
cholestasis, posterior embryotoxon,
butterfly-like
vertebral arch defects and peripheral
pulmonary artery hypoplasia or
stenosis. We present a two-year-old
female child with the 'partial' or 'incomplete'
Alagille syndrome. The
child had three of the five major features of the
syndrome. A brief
review of
literature of the
syndrome is presented.