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William R. DeFoor, Jr., MD, MPH, Andrea Bischoff, MD, Pramod Reddy, MD, Brian Vanderbrink, MD, Eugene Minevich, MD, Curtis Sheldon, MD, Marion Schulte, RN, Alberto Pena, MD.
Cincinnati Children's Hospital, Cincinnati, OH, USA.

Background: Children born with a persistent cloaca undergo complex surgical reconstruction early in life. Long-term risks of voiding dysfunction and renal deterioration have been well described. In our institution, a collaborative approach amongst all providers has long been our practice to coordinate the anorectal and genito-urinary reconstruction. In addition, our protocol includes an early aggressive management approach to the lower urinary tract. The purpose of this study is to report upper urinary tract outcomes in this patient population.
Methods: A retrospective cohort study was performed of all patients undergoing posterior sagittal ano-recto-vagino-urethroplasty (PSARVUP) for persistent cloaca at a single institution over the past ten years. Exclusion criteria included patients who did not receive post-operative urologic care at our institution. Patient demographics, surgical details, management of lower urinary tract dysfunction, renal anomalies, urinary tract reconstruction, and chronic kidney disease (CKD) stage were abstracted from the medical record. Kaplan-Meier curves of CKD stage progression were analyzed.
Results: A total of 56 patients with persistent cloaca were identified from 2005 to 2014, and 48 met inclusion criteria. Six infants were deceased from other major anomalies and 2 had not undergone repair. 41 patients were referred from outside institutions with a pre-existing colostomy, 15 of which also had a vesicostomy or ureterostomy performed prior to referral. On presentation, 27 patients had hydroureteronephrosis, 22 had vesicoureteral reflux, and 17 had a tethered spinal cord. The mean age at time of PSARVUP was 11 months (range 1 to 48). The range of the common channel was 1 to 7 cm. The median follow-up after surgery was 5.6 years. A total of 32 patients (64%) were diagnosed with neurogenic bladder and started on clean intermittent catheterization. 7 patients underwent a subsequent Mitrofanoff neo-urethra to facilitate CIC. At presentation, 5 patients had CKD stage III and one had CKD IV. The remaining 42 patients were CKD I or II. Three patients were treated for hypertension. The patient with CKD IV had a renal allograft placed at 34 months of age prior to the need for dialysis. In the follow-up period, no patients with initial CKD stage I or II had stage progression.
Conclusions: In this contemporary series of children with persistent cloaca undergoing PSARUVP, early outcomes in patients with chronic kidney disease stage I and II appear to show that global renal function is maintained despite a high rate of lower urinary tract dysfunction. A collaborative surgical approach along with aggressive bladder management may help prevent progressive renal injury in this patient population.

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