Research Article|Articles in Press

Long Term Renal Outcome and Risk of Elevated Blood Pressure in Children Undergoing Complete Primary Repair of Bladder Exstrophy (CPRE)



      : Since the initiation of staged reconstruction for bladder exstrophy (BE), hypertension has been a known complication of the procedure. Hypertension is a well-established risk factor for chronic kidney disease (CKD) progression and associated with cardiovascular/cerebrovascular morbidity and mortality. Few studies exist evaluating the risk of developing hypertension among patients with bladder exstrophy who underwent CPRE. We hypothesized that long-term blood pressure levels may be elevated in males vs females, and may be correlated with presence of hydronephrosis, bladder neck reconstruction, or continence status.


      : We sought to revisit our long-term experience with CPRE and determine factors associated with incidence of elevated blood pressures.


      : We reviewed all BE patients undergoing CPRE at our institution from 1999 to 2019. Patients were considered eligible for inclusion if last renal ultrasound was obtained at least 5 years after repair. Upper tract outcomes based on imaging, history of pyelonephritis and renal function tests measured by serum creatinine and estimated glomerular filtration rate (eGFR, Schwartz formula) were reviewed. Systolic/diastolic blood pressures (SBP/DBP) from all encounters were captured. All blood pressure values were age adjusted by percentile.


      : A total of 36 patients were considered eligible for review. Median follow-up of this cohort was 10.01 (5.16-21.47) years. The mean creatinine for the patients available was 0.58mg/dL (SD = 0.20), at mean age of 8.90 years Neither SBP or DBP were significantly elevated in males vs females, but had lower odds of elevation >90th percentile for those with higher eGFR, lower renal length, and reimplantation. Pyelonephritis incidence was 38% (n=14) with first episode at mean age of 8.8 years, and mean of 3.7 episodes per patient.


      : At long term follow up, blood pressures following CPRE were not significantly elevated, despite the relatively frequent occurrence of CKD, and hydronephrosis. Male gender does appear to suggest higher risk for long-term deterioration in this regard. Higher eGFR, higher renal length, and presence of ureteral reimplantation were associated with lower likelihood of systolic/diastolic blood pressure elevation. Continence status and bladder neck reconstruction were not associated with likelihood of blood pressure elevation.


      : Blood pressure and upper-tract outcomes for patients undergoing CPRE at birth are positive for the majority of patients. To avoid complications from hypertension, patients should be closely evaluated as the risks associated with elevated blood pressure are significant. Ultimately, larger-scale prospective and multi-institutional studies are further needed to characterize risks of hypertension in this complex patient population.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Journal of Pediatric Urology
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


      1. Siffel, Csaba, Adolfo Correa, Emmanuelle Amar, Marian K. Bakker, Eva Bermejo‐Sánchez, Sebastiano Bianca, Eduardo E. Castilla et al. "Bladder exstrophy: an epidemiologic study from the International Clearinghouse for Birth Defects Surveillance and Research, and an overview of the literature." In American Journal of Medical Genetics Part C: Seminars in Medical Genetics, vol. 157, no. 4, pp. 321-332. Hoboken: Wiley Subscription Services, Inc., A Wiley Company, 2011.

        • Ellison Jonathan S.
        • Ahn Jennifer
        • Shnorhavorian Margarett
        • Grady Richard
        • Merguerian Paul A.
        Long-term fate of the upper tracts following complete primary repair of bladder exstrophy.
        Journal of Pediatric Urology13. 2017; 4: 394-e1
        • Husmann D.A.
        • McLorie G.A.
        • Churchill B.M.
        Hypertension following primary bladder closure for vesical exstrophy.
        Journal of pediatric surgery28. 1993; 2: 239-241
        • Rao MV
        • Qiu Y
        • Wang C
        • Bakris G
        Hypertension and CKD: Kidney Early Evaluation Program (KEEP) and National Health and Nutrition Examination Survey (NHANES), 1999–2004.
        Am J Kidney Dis. 2008; 51 (Gallibois, Claire M., Natasha A. Jawa, and Damien G. Noone. "Hypertension in pediatric patients with chronic kidney disease: management challenges." International journal of nephrology and renovascular disease10 (2017): 205.): S30-S37
        • Shnorhavorian Margarett
        • Grady Richard W.
        • Andersen Amy
        • Joyner Byron D.
        • Mitchell Michael E.
        Long-term followup of complete primary repair of exstrophy: the Seattle experience.
        The journal of urology180. 2008; 4S: 1615-1620
        • Grady Richard W.
        • Mitchell Michael E.
        Complete primary repair of exstrophy.
        The Journal of urology162. 1999; 4: 1415-1420
        • Mitchell Michael E.
        • Bagli Darius J.
        Complete penile disassembly for epispadias repair: the Mitchell technique.
        The Journal of urology155. 1996; 1: 300-304
        • Schwartz George J.
        • Munoz Alvaro
        • Schneider Michael F.
        • Mak Robert H.
        • Kaskel Frederick
        • Warady Bradley A.
        • Furth Susan L.
        New equations to estimate GFR in children with CKD.
        Journal of the American Society of Nephrology20. 2009; 3: 629-637
        • Nguyen Hiep T.
        • Benson Carol B.
        • Bromley Bryann
        • Campbell Jeffrey B.
        Jeanne Chow, Beverly Coleman, Christopher Cooper et al. "Multidisciplinary consensus on the classification of prenatal and postnatal urinary tract dilation (UTD classification system).
        Journal of pediatric urology10. 2014; 6: 982-998
        • Goonasekera CD
        • Dillon MJ
        Reflux nephropathy and hypertension.
        Journal of human hypertension. 1998 Aug; 12: 497-504
        • Wang MH
        Is There Hope for Renal Growth on Imaging Studies Following Ureteral Reimplant for Boys With Fetal Hydronephrosis and Urinary Reflux?.
        Urology Case Reports. 2015 Jul 1; 3: 114-116
        • Franklin Stanley S.
        • Thijs Lutgarde
        • Hansen Tine W.
        • O’brien Eoin
        • Staessen Jan A.
        White-coat hypertension: new insights from recent studies.
        Hypertension62. 2013; 6: 982-987