Renal malposition in patients with omphalocele

We describe a boy with a history of omphalocele who presented with gross hematuria. Subsequent evaluation revealed a cephalad right kidney malposition and the hematuria was of lower tract origin. To investigate the frequency of this radiographic finding the medical records of 15 patients with omphalocele who presented between 1979 and 1985 were reviewed. Studies of the urinary tract were performed after omphalocele closure. Of 7 cases (46 per cent) with abnormal cephalad renal displacement the kidney was on the right side only in 3 and it was bilateral in 4. The omphalocele contents consisted of gastrointestinal tract only in 9 patients, and liver and gastrointestinal tract in 6. All 6 patients with omphaloceles that included the liver had cephalad renal displacement. One patient with small bowel alone in the omphalocele had right kidney displacement. Clinicians should be aware of this variation to avoid confusion and further unnecessary evaluation.