Summary
Introduction
The P.A.D.U.A. technique is a method of addressing congenital urethral narrowing.
It involves passive dilation with a series of progressively larger indwelling catheters.
Utilization is limited by scant literature, particularly regarding technical details
and long-term durability. Tools for achieving safe and reliable urinary drainage are
critical in these patients, who require careful stewardship of their kidney and bladder
function.
Objective
To describe long-term urethral patency and urinary function following P.A.D.U.A.,
and to provide sufficient technical detail to reproduce the technique.
Study design
Patients with congenital urethral narrowing managed with P.A.D.U.A. were identified
and chart review was performed. Details of catheter exchange sequences were compiled
and described. The primary outcome was the attainment of adequate urethral caliber
by successful completion of P.A.D.U.A., and the secondary outcome was voiding per
urethra at most recent follow-up.
Results
P.A.D.U.A. achieved adequate urethral caliber in 9/11 (82%) of patients. This included
seven patients with Prune Belly Syndrome, one with isolated urethral atresia, and
one with a cloacal anomaly. P.A.D.U.A. failed to achieve urethral patency in one patient
with urethral duplication, who was unable to progress through the catheter sequence,
and one patient with Prune Belly Syndrome, who completed P.A.D.U.A. but developed
recurrent narrowing one week later. There were no delayed failures of urethral patency.
Patients who achieved patency underwent a median of seven catheter placements over
92 days. Median (range) initial and final catheter sizes were 3.5F (1.9–8F) and 14F
(8–16F). While 82% achieved patency, only 3/11 (27%) were voiding spontaneously per
native urethra at most recent follow-up.
Discussion
This series of patients undergoing P.A.D.U.A. for primary treatment of congenital
urethral narrowing is the largest to date and provides granular technical details.
It aligns with prior reports suggesting that P.A.D.U.A. achieves urethral patency
in most patients with Prune Belly Syndrome or isolated urethral atresia, but has limited
application in the hypoplastic duplicated urethra. Despite high rates of urethral
patency in this select population, many patients will not achieve and maintain spontaneous
voiding without catheterization. This is likely due to ongoing deterioration of bladder
function caused by the prenatal developmental insult, paralleling the phenomenon seen
in posterior urethral valves.
Conclusion
Graphical AbstractCurrent urinary status. Voiding status as of most recent follow-up for all patients in the cohort.
Keywords
Abbreviations:
PBS (Prune Belly Syndrome), P.A.D.U.A. (progressive dilation of the urethra anterior), PUV (posterior urethral valve), UTI (urinary tract infection), CIC (clean intermittent catheterization)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 accessOne-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:
Subscribe to Journal of Pediatric UrologyAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Current state of fetal intervention for lower urinary tract obstruction.Curr Urol Rep. 2018; 19: 12
- Campbell-Walsh-Wein urology.12th ed. 3. Elsevier, Philadelphia, PA2021: 3706 (illustrations (chiefly color) p)
- The radiologic and pathologic spectrum of the prune belly syndrome. The importance of urethral obstruction in prognosis.Radiol Clin North Am. 1977; 15: 83-92
- The prune belly syndrome. Report of 20 cases and description of a lethal variant.J Pediatr. 1973; 83: 786-793
- Urethral and genital malformations in prune belly syndrome.J Urol. 1982; 127: 94-96
- Urethral atresia and the prune belly syndrome. Report of 6 cases.Br J Urol. 1993; 72: 112-114
- Prune belly syndrome with urethral hypoplasia and vesico-cutaneous fistula: a case report and review of literature.Urol Ann. 2013; 5: 296-298
- Splenic torsion with prune belly syndrome.J Pediatr. 1981; 98: 91-92
- Prune belly syndrome--report of 47 cases.Arch Dis Child. 1982; 57: 856-859
- Urodynamics in the prune belly syndrome.Br J Urol. 1976; 48: 663-670
- (progressive augmentation by dilating the urethra anterior) procedure for the treatment of severe urethral hypoplasia.J Urol. 1988; 140: 1247-1249
- Urethral duplication.J Urol. 1986; 136: 909-912
- Urethral atresia and anhydramnios at 18 weeks of gestation can result in normal development.J Pediatr Urol. 2012; 8: e33-e35
- Management of congenital urethral strictures in infants. Case series.Urol J. 2019; 16: 67-71
- Fate of males with urethral "Y-duplication": 40-year long follow-up in 8 patients.J Pediatr Surg. 2017; 52: 1335-1339
- Y-type urethral duplication in the male.BJU Int. 2006; 97: 597-602
- Congenital H-type anourethral fistula with severe urethral hypoplasia: case report and review of the literature.J Pediatr Surg. 1998; 33: 1550-1553
- Role of a preoperative catheter regimen in achieving early primary endoscopic valve ablation in neonates with posterior urethral valves.J Urol. 2021; 205: 1792-1797
- The efficiency of bladder emptying in the prune belly syndrome.J Urol. 1992; 148: 600-603
- Clinical manifestations and management of prune-belly syndrome in a large contemporary pediatric population.Urology. 2015; 85: 211-215
- Structural study of the bladder in fetuses with prune belly syndrome.Neurourol Urodyn. 2018; 37: 148-152
- Fetal bladder histology in posterior urethral valves and the prune belly syndrome.J Urol. 1990; 144: 337-339
- A systematic review on renal and bladder dysfunction after endoscopic treatment of infravesical obstruction in boys.PLoS One. 2012; 7e44663
- Bladder dysfunction in boys with posterior urethral valves before and after puberty.J Urol. 1996; 155: 694-698
- Detrusor hypocontractility evolution in boys with posterior urethral valves detected by pressure flow analysis.J Urol. 2001; 165: 2248-2252
- The changing urodynamic pattern from infancy to adolescence in boys with posterior urethral valves.BJU Int. 2000; 85: 1104-1108
- Anterior urethral valves - a rare but challenging congenital pathology.J Pediatr Urol. 2020; 16: 585 e1-e7
- Renal replacement therapy and intermittent catheterization risk in posterior urethral valves.Pediatrics. 2019; 143
- Long-term outcome of prenatally detected posterior urethral valves: single center study of 65 cases managed by primary valve ablation.J Urol. 2008; 179 (discussion 12-3): 307-312
- Long-term outcomes in children treated by prenatal vesicoamniotic shunting for lower urinary tract obstruction.Obstet Gynecol. 2005; 106: 503-508
Article info
Publication history
Published online: November 01, 2022
Accepted:
October 29,
2022
Received in revised form:
October 27,
2022
Received:
June 6,
2022
Identification
Copyright
© 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
