摘要
Heterotaxy syndrome is a rare complex syndrome characterized by cardiac and extra cardiac congenital malformations. The syndrome is divided into two main groups; right isomerism (Ivemark syndrome, asplenia) and left isomerism (polysplenia syndrome). Here we report a polysplenia syndrome with agenesis of head and uncinate process of the pancreas in a 52 year old woman who admitted to our clinic with complaints of weakness, lumbalgia, abdominal distention and constipation.