Unusual association of omphalocele and wandering spleen

A wandering spleen is a rare anomaly and its association with omphalocele has been reported in the literature only once. We present a female infant that has a wandering spleen associated with omphalocele. The patient was admitted with the diagnosis of omphalocele. Primary repair was performed and the patient was discharged on the 6th postoperative day. Nine months later, the girl was referred to our emergency unit with complaints about abdominal distention and restlessness. After 2 days of clinical observation, an immobile, firm abdominal mass was palpated. Ultrasonographic evaluation revealed that the mass was the spleen and it was found exactly under the left side of the umbilicus, instead of its original location. During the operation, the spleen was beneath the left side of the umbilicus, with its upper surface completely adhered to the peritoneum, and it was rotated 180 degrees clockwise. Then the spleen was rotated back and placed in its normal anatomic location. At 1 month postoperatively, a radionuclide scan was performed and splenic perfusion was evaluated to be normal. In conclusion, herniations and eventrations of organs through defects on the abdominal wall may either result in restriction of normal rotations of the stomach and the spleen or in inefficient fusion after the rotation has been completed.