Pyloroduodenal deformity due to liver malformation associated wtih omphalocele

While congenital malformed and malpositioned livers are rare, they are relatively common in patients with omphaloceles. A malpositioned, malformed liver causing pyloroduodenal deformity and apparent pyloric obstruction in an infant or child with an omphalocele has not been previously reported. Thee such cases are described in which symptoms of upper gastrointestinal obstruction developed shortly after primary closure of the omphaloceles. Recognition of this condition at the time of primary closure of an omphalocele may allow consideration of additional procedures to prevent postoperative pyloric obstruction.