Exomphalos

This code represents an omphalocele, a congenital abdominal wall defect where abdominal organs protrude into the base of the umbilical cord, covered by a sac of peritoneum and amnion. It results from a failure of the abdominal wall to close properly during fetal development.

Use this code for documented cases of omphalocele, typically identified prenatally via ultrasound or at birth. Documentation should clearly state the presence of an omphalocele, distinguishing it from other abdominal wall defects.

• Differentiate carefully between omphalocele and gastroschisis; they are distinct conditions.
• Remember the Excludes1 note: do not code this with umbilical hernia (K42.-).
• Look for associated congenital anomalies, as omphalocele often occurs with other genetic syndromes.