Upper urinary tract manifestations of the VACTERL association

Purpose: In patients suspected to have the vertebral, anal, tracheoesophage al, renal, radial, cardiac and limb abnormalities (VACTERL) association we studied the frequency of upper urinary tract anomalies, prevalence of these features, predictability of upper tract pathology and proper screening eva luation. Materials and Methods: From 1991 to 1998 we identified 55 patients with the VACTERL association. Upper urinary tract assessment, including initial ren al ultrasound and voiding cystourethrography, and followup data were availa ble for 29 boys and 15 girls. Patients were considered to have the VACTERL association when 3 or more organ systems were involved. Results: Average followup was 5.4 years. Upper urinary tract involvement wa s noted in 41 of the 44 patients (93.2%) and vesicoureteral reflux in 17 (2 5 renal units). Of the kidneys 21 were hydronephrotic without reflux or obs truction, 10 were solitary, 3 were multicystic dysplastic, 8 were obstructe d and only 17 were normal. A total of 27 patients (61%) underwent at least 1 genitourinary procedure, primarily ureteroneocystotomy. All children were alive at the last followup. Conclusions: The VACTERL association involves multiple serious anomalies. H owever, these infants generally have a good outcome. Since the upper urinar y system is the most common organ system involved, patients should receive prophylactic antibiotics until an initial urological assessment with renal ultrasound and voiding cystourethrography is performed. It is essential for physicians to know that most children with the VACTERL association have ur ological involvement that requires treatment and long-term management.