PURPOSE: Children born with persistent cloaca undergo complex pelvic reconstruction early in life. Long-term risks of bladder dysfunction and chronic kidney disease are well described. We report upper urinary tract outcomes and the risk of chronic kidney disease stage progression in this patient population.MATERIALS AND METHODS: We retrospectively studied a cohort of patients undergoing posterior sagittal anorecto-vagino-urethroplasty at a single institution from 2006 to 2013. Inclusion criteria consisted of complete urological care at our institution. Chronic kidney disease stage was calculated from cystatin C or nuclear medicine glomerular filtration rate.RESULTS: A total of 44 patients met inclusion criteria. Of the patients 12 had undergone vesicostomy or ureterostomy. A total of 19 patients had hydronephrosis, 19 had vesicoureteral reflux and 15 had a tethered spinal cord. Median length of the common channel was 3.5 cm. Median age at posterior sagittal anorecto-vagino-urethroplasty was 7.3 months. Median followup was 5.3 years. A total of 30 patients had neurogenic bladder, of whom 27 required clean intermittent catheterization and 3 had undergone vesicostomy. Of the patients 38 had stage I or II, 5 had stage III and 1 had stage IV chronic kidney disease. During followup no patient with initial stage I to III chronic kidney disease had stage progression. The patient with stage IV chronic kidney disease had a renal allograft placed at age 34 months before needing dialysis.CONCLUSIONS: Early outcomes in patients with stage I to III chronic kidney disease demonstrate that renal function can be maintained despite a high rate of lower urinary tract dysfunction. Aggressive bladder management may help prevent progressive renal injury in this population.
Cloaca Disease Progression