Urologic Aspect in the Management of VATER Anomalies / 대한비뇨기과학회지

ABSTRACT

Ten cases of VATER syndrome were reviewed to determine the type of anomalies, genitourinary anomalies, complications of treatment and prognosis. None of the four cases that were born in our hospital and six cases that were referred to us were diagnosed prenatally. Eight patients were born at full term with normal birth weight, two were premature and one showed pre-eclampsia at IUP 34 weeks.The male to female ratio was 6 to 3 with one patient having ambiguous genitalia. Tracheo-esophageal fistula was found in five patients. Three of these five patients died after the parents refused treatment, and one died the next day after birth even with treatment. Imperforate anus was observed in seven patients. Unilateral renal agenesis, crossed renal ectopia and dysplastic kidney were found in four, three (one pelvic kidney, one crossed renal ectopia with fusion, one crossed renal ectopia) and one patients, respectively. There was one hypospadias. Six of the eight patients with vertebrae anomaly survived; four of the survivors had vesicoureteral reflux (VUR) and neurogenic bladder. Two of the four patients with VUR received ureteroneocystostomy then clean intermittent catheterization (CIC), sympatholytic and antibiotics. The other two patients with neurogenic bladder received clean intermittent catheterization (CIC) and vesicostomy. Complications of recurrent urinary tract infection (UTI) and acute epididymitis occurred when descending loop colostomy was performed due to imperforate anus and urethro-rectal fistula. Separated colostomy instead of descending loop colostomy is recommended for recto-urinary fistula since infections of the upper urinary tract in such cases required ureteroneocystostomy before the correction of imperforate anus and continued antibiotic administration was needed because of the possibility of delayed wound healing and recurrent UTI. A strong will by the patient`s parents, protection of renal function through early detection of genit-ourinary anomalies and other appropriate treatments are required for the high success rate in the surgical correction of VATER syndrome.