Urological anomaly in an infant with Soto's syndrome: report of a case

OBJECTIVE: To discuss the diagnosis and the etiology of a bilateral urologic anomaly in a patient with Soto's Syndrome (cerebral gigantism). To alert the pediatric physicians and urologists about the coexistence of these two malformations. RESULTS: A three month old boy, born of a 35 week uncomplicated first pregnancy by cesarian, with phenotypic, radiologic and metacarpophalangeal profile of Soto's Syndrome was admitted to the Hospital because of hyperpnea, tachycardia and 39o C recurrent fever. The culture of urine disclosed 500.000 col/ml. Cistouretrographic study showed bilateral megaloureter and hydronephrosis. Vesico-ureteral bilateral reflux was diagnosed and antibiotic therapy was administered. Patient's follow up was excellent and was discharged taking preventive nitrofurantoin. CONCLUSIONS: As in other cases of urinary tract infection, pediatric physicians must consider urologic anomalies in children with cerebral gigantism who present the clinical presentation of Soto's Syndrome. Autonomic Nervous System development failure in ureteral muscles could be an alternative etiology.