IS TAMULOSIN EFFECTIVE FOR LOWER URINARY TRACT DYSFUNCTION (LUTD) IN PRUNE BELLY SYNDROME (PBS)?
Shaheer Ali, BS, Daniel Wong, MD, Shane F. Batie, MD, Michelle K. Arevalo, MD, Adam Kern, MD, Linda A. Baker, MD.
University of Texas Southwestern Medical Center, Dallas, TX, USA.
BACKGROUND: Most male PBS patients void spontaneously with significant LUTD that may necessitate urological intervention to preserve renal function. Most have underactive bladder (UAB) of large capacity, infrequent voiding with diminished bladder urge/sensation, vesicoureteral reflux (VUR), and elevated post-void residuals (PVRs) despite a dilated bladder neck and posterior urethra. Since 2015, we assessed the efficacy and tolerance of tamsulosin in a non-randomized open-label pilot trial of male PBS cases with significant LUTD as a medical alternative to the initiation of clean intermittent catheterization (CIC) or vesicostomy.
METHODS: After ICD-10 Q79.4 search, a retrospective chart review was performed of PBS patients with LUTD despite standard urotherapy receiving tamsulosin (0.2mg bid or 0.4mg qd), evaluating demographic data, medical/surgical history, and voiding symptoms/parameters pre- and post-tamsulosin. International Children's Continence Society (ICCS) standard definitions were used, including bladder capacity(BC) {BC = (age[yrs]+1) X 30}. No pill counting was performed to assess medication compliance. Drug tolerance and treatment outcomes (judged by decreased PVRs, improved voiding patterns, no urinary retention, no UTIs, or parental/child subjective symptomatic improvement) were assessed initially and at ≥1 year.
RESULTS: 36 PBS patients were managed clinically from 2015-current. 13 patients had urinary drainage via vesicostomy (n=5), ureterostomy (n=2) or CIC (n=6). Of the remaining 23 that voided per urethra, 9 (mean age 5.0 yrs [range: 1.9 - 10.4]) had received abdominoplasty, had no VUR, and were included in this tamsulosin trial for significant UAB. Group A (GrA, n= 5) were toilet-trained (mean age 7.0 yrs [range: 2.4 - 10.4] and mean RUBACE score = 10) while Group B (GrB, n=4) were in diapers (mean age 2.5 years [range 1.9 - 3.2] and mean RUBACE score =12). GrA had implemented timed double/triple voiding, yet still has significantly elevated bladder capacity (mean 3.5X normal) and PVR (mean PVR= 32.5% BC) pre-tamsulosin. Duration of tamsulosin therapy was mean 3.7 and 0.9 years for GrA and GrB, respectively, and for the groups combined ranged from 0.2 - 7.2 yrs (mean: 2.49 yrs). There were no adverse events, UTIs, or changes to baseline VUR or hydroureteronephrosis while on therapy. PVR was reduced in all GrA patients with post-tamsulosin mean PVR= 13.1% BC. All (child or parents) reported increased frequency of urination, subjective improved voiding and less abdominal distention. 3 were on tamsulosin for <1 year, with 1 partial success, 1 self-discontinued and 1 failure requiring vesicostomy. 6 patients were on tamsulosin for ≥1 year [range 1.2-7.2yrs] with 100% having partial success and initial parental satisfaction. Of these 6, 3 (50%) remain on tamsulosin for mean 5.7 yrs, while 3 stopped at mean 1.5 yrs (1 for CIC and 2 self-discontinued).
CONCLUSIONS: As an adjunct to behavioral urotherapy (timed double/triple voiding) and as an intervention prior to urinary drainage, tamsulosin was 100% tolerated and 89% efficacious in 9 PBS patients with UAB/LUTD. PBS requires lifelong management and randomized studies with proper control groups are required to assess long-term success of tamsulosin in select PBS patients.
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