COEXISTENCE OF VATER ASSOCIATION AND RECURRENT UROLITHIASIS - A CASE-REPORT

VATER association is diagnosed by the combined presence of at least th ree of the following features: vertebral anomalies, anal atresia, trac heo-esophageal fistula and/or esophageal atresia, radial ray anomalies , and renal anomalies (53%). Urolithiasis has not been reported in thi s syndrome. A 4-month old girl presented because of irritability, and the presence of stones in the diapers. Physical examination revealed a nal atresia for which colostomy was performed in the newborn period. T he diagnosis of VATER association was established by the additional fi ndings of hemivertebrae, sacral dysgenesis, and horseshoe kidney which was partly non-functional. Urinary pH was repeatedly below 6. An excr eted stone consisted of pure uric acid. Metabolic investigations detec ted no specific pathology in purine metabolism, Urolithiasis did not r ecur after reconstructive anal and anorecto-vaginoplasty, implying tha t it was a consequence of colostomy and/or of the underlying renal ano maly, We suggest that after colostomy patients with VATER association should be followed for possible urate stones, e.g. by regular screenin g of urinary pH.