Do children with bladder exstrophy void?
Sahar Eftekharzadeh, MD, MPH1, Ted Lee, MD2, Elizabeth B. Roth, MD3, John Weaver, MD1, Jay Shah, BA1, Alyssia Venna, MBS2, John V. Kryger, MD3, Travis W. Groth, MD3, Aseem R. Shukla, MD, M.H.C.I1, Richard S. Lee, MD2, Joseph G. Borer, MD2, Michael E. Mitchell, MD3, Douglas A. Canning, MD1, Dana A. Weiss, MD1.
1The Children's Hospital of Philadelphia, Philadelphia, PA, USA, 2Boston Children's Hospital, Boston, MA, USA, 3Children's Wisconsin, Milwaukee, WI, USA.
BACKGROUND: Achieving urinary continence while preserving kidney function are the main goals for every child with bladder exstrophy (BE). Nevertheless, continence status is variable after complete primary repair of bladder exstrophy (CPRE). There are sparse data documenting volitional voiding with bladder contractility after CPRE. We evaluated voiding patterns and bladder function in children undergoing CPRE using urodynamic studies (UDS). We hypothesized that the majority of children with BE would display bladder contractility sufficient for volitional voiding after CPRE.
METHODS: Within a prospective longitudinal multi-institutional database of children with BE treated at three centers, UDS studies for those with classic BE, who underwent primary CPRE between 2012 and 2020, were reviewed. Abstracted UDS data included: presence of detrusor contractions, bladder capacity, maximum detrusor voiding pressure, presence of sustained abdominal pressure, leak point pressure, and post-void residual (PVR). Additionally, intraoperative measurements of bladder dome to bladder neck length and right to left width were assessed.
RESULTS: Out of the entire database, 26 children with BE met inclusion criteria. Median age was 3.5 (IQR: 3.2-4.7) years at most recent evaluation with median follow-up of 3 (IQR: 3-5) years. In total, 38 UDS were assessed, as 7 (26.9%) children had 2 or more UDS. The most recent UDS was used for analysis if more than one study was available. Four (15.4%) children were on clean intermittent catheterization (1 via an appendicovesicostomy). Seven (26.9%) were on antimuscarinic medication at the time of UDS. Sixteen of the 26 (61.5%) voided via a sustained detrusor contraction with a peak voiding pressure of 39.5 cmH20, and a median PVR of less than 50% of their end fill volume. Two of the 4 children on CIC had documented detrusor contractions on UDS and use CIC only occasionally to empty completely. Intraoperative bladder width and bladder dome to bladder neck length did not correlate with the presence of voiding via a detrusor contraction (p=0.64).
CONCLUSIONS: This study demonstrates that the majority of children with BE develop the ability to produce sustained detrusor contractions sufficient to void per urethra with alow residual volume. Our findings suggest that children with BE have the potential for long term volitational voiding. 
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