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Prune Belly Syndrome

Prune belly syndrome (PBS), also called Eagle-Barrett or triad syndrome, is a rare congenital disorder (incidence about 1 in 35,000–50,000; the EAU 2026 paediatric guideline gives 1 in 30,000–50,000, with male predominance in over 95%) defined by the classic triad of abdominal-wall musculature deficiency, urinary-tract dilation, and bilateral cryptorchidism in males.[1][38] It is among the most complex conditions in transitional urology: survivors require lifelong multidisciplinary urological care that must be deliberately handed off from pediatric to adult providers.[2][3]

Adult survivors may have a large hypotonic bladder with variable emptying and upper-tract dilation.[5][13] In males, cryptorchidism and accessory-gland abnormalities may limit fertility; abdominal-wall weakness may affect voiding efficiency.[20][24]

Disease Overview and Spectrum​

PBS is a spectrum disorder, from mild (near-normal life expectancy) to lethal (neonatal pulmonary hypoplasia).[4] The urinary-tract abnormalities include large hypotonic bladders, dilated tortuous megaureters, vesicoureteral reflux (about 78%), and hydronephrosis.[5] A histologic study of three PBS fetuses versus seven controls found less smooth muscle, type-III collagen and innervation. The fetal histology study helps explain the phenotype but offers no functional outcome data.[6]

In a single-center cohort of 46 children, 39% had documented CKD and 17% had undergone renal transplantation.[5] Prognostic markers for renal failure include nadir serum creatinine >0.7 mg/dL, bilaterally abnormal kidneys on imaging, and clinical pyelonephritis.[7]

Renal Function and Transplantation​

Renal preservation is the primary lifelong goal. Pyelonephritis and obstruction are potentially modifiable contributors to later renal decline. This distinction arose from a small historical pathologic series; congenital dysplasia and reduced nephron reserve remain relevant throughout life.[8]

For those reaching ESRD, transplant outcomes are encouraging:

  • A single-center series (13 transplants in 9 patients) reported graft survival of 90.9%, 57.7% and 38.5% at 5, 10 and 15 years, versus 89.9%, 80% and 74% in 106 matched recipients with nonurologic causes. The overall comparison was not statistically significant (P = 0.134), but long-term survival was proportionally lower; eight of nine PBS recipients underwent urologic surgery before transplantation.[9]
  • Australian KRT registry data (37 patients) showed 1-, 5-, and 10-year first-graft survival of 91%, 71% and 51%, equivalent to or better than matched congenital-kidney-disease controls, and peritoneal dialysis was feasible despite the abdominal-wall deficiency.[10]
  • The ESPN/ERA-EDTA registry (88 boys) reported graft-loss risk comparable to other CAKUT groups and slightly higher mortality than in congenital obstructive uropathy (HR 1.96, 95% CI 1.03–3.74).[11]
  • In a retrospective single-center series of 22 children after a first PBS kidney transplant, 11 (50%) had at least one febrile UTI during follow-up; many required procedures to achieve adequate bladder emptying.[42]
  • In an eight-recipient series, abdominal-wall distensibility permitted adult-kidney placement in young children.[12] In a separate five-boy series, abdominal-wall deficiency caused mechanical graft dysfunction in one recipient, which resolved after Monfort reconstruction; the authors emphasized pretransplant assessment of the abdominal wall.[43]

Lower Urinary Tract Management​

Bladder dysfunction is the central and evolving challenge. Voiding is highly variable and changes over time: in a 34-patient urodynamic series, 44% voided spontaneously and 56% required clean intermittent catheterization (CIC).[13] Voiding or CIC needs may change over time, so reassess PVR, infections, upper-tract findings and renal function; repeat urodynamics when pressure or compliance findings could change management.[13][18] The series (follow-up 6 months–19 years) described three voiding patterns, none correlating with residual volumes.[13]

The flow patterns cannot determine who needs CIC. Serial PVR, infections, renal and upper-tract findings and, where necessary, pressure-flow assessment guide the decision. There is no validated universal PBS-specific PVR percentage mandating catheterization.[13] When ongoing drainage is required and feasible, intermittent catheterization generally avoids the burdens of a chronic indwelling catheter; AUA/SUFU support (Statement 35; Strong Recommendation, Grade C) comes from neurogenic, not PBS-specific, evidence.[14] Do not prescribe Valsalva or Credé from the flow-curve shape alone: adjacent EAU neuro-urology guidance cautions against these methods unless urodynamics demonstrate safe pressures.[36]

Surgical and pharmacologic options:

  • Monfort abdominoplasty was associated with improved emptying in a 12-patient series (paired UDS in eight). In the overall Monfort abdominoplasty series, PVR fell from 40.3% to 13% of capacity and UTIs from 5.7 to 1.2 per patient-year, with better continence and bladder sensation; among patients without concomitant urinary reconstruction, PVR fell from 40% to 14.3%. The Monfort series suggests possible functional benefit but provides no adult revision efficacy estimate.[20]
  • Reduction cystoplasty lowers bladder volume (about 52%) and can help in childhood, but long-term capacities re-expand (740–2,300 cc) and it does not reliably improve voiding dynamics.[21] It is most useful in the pediatric setting.[15][21]
  • Selective ureteral tailoring and reimplantation for severe VUR or upper-tract stasis, guided by longitudinal renal-function studies and not performed empirically.[16][17]
  • Persistently unsafe storage or emptying requires reassessment and specialist treatment. A pressure of 40 cm H₂O is a historical upper-tract risk marker, not a universal PBS threshold or automatic indication for augmentation. Treatment depends on compliance, detrusor activity, drainage and renal trajectory.[14][19]

Urodynamic surveillance​

PBS is not automatically neurogenic LUTD. Its adult surveillance is individualized using congenital anatomy, bladder function, infections, reflux and renal status. The AUA/SUFU adult NLUTD schedule can inform discussion, but its categories and intervals are not validated PBS-specific recommendations.[18] Follow renal function and upper tracts regularly, more closely when abnormal; obtain multichannel UDS when pressure or compliance information will change management or deterioration may be clinically silent. Neither congenital megacystis nor prior reconstruction alone establishes a neurogenic high-risk category, and AUA/SUFU does not mandate a universal two-year UDS interval for PBS.[14][18]

Practical point. Voiding status in PBS changes over time. A patient voiding spontaneously in adolescence may later need CIC, and the reverse also occurs. Reassess serially.[13]

Reproductive and Sexual Health​

This is a major transition-era concern. In a long-term series of 41 boys treated with one- or two-stage Fowler-Stephens orchiopexy, most reached spontaneous puberty with satisfactory sexual function.[22] However, retrograde ejaculation is common (7 of 9 in one cohort), and late orchiopexy historically produced sexually active but infertile men with azoospermia and elevated FSH.[23]

A 2024 scoping review found frequent azoospermia in the published semen reports, but documented four assisted-reproduction conceptions and two natural conceptions; its heterogeneous case literature does not provide a population fertility probability.[39] In 15 postpubertal patients (mean orchiopexy age 18 months), motile spermatozoa were found in 4/8 who submitted semen, with generally normal hormonal profiles, though prostate hypoplasia (66.6%) and unilateral seminal-vesicle absence (66.6%) were prevalent and likely contribute to infertility.[24] Intracytoplasmic sperm injection (ICSI) has enabled successful pregnancies, and the Australian registry reported 3 PBS men achieving parenthood at a median age of 35.[10][25] Early orchiopexy is advocated; current EAU guidance for undescended testes aims for 6–12 months and by 18 months at the latest, with technique tailored to anatomy and comorbidity.[22][37]

Malignancy Surveillance​

As survival into adulthood improves, malignancy risk becomes increasingly relevant:

  • Testicular malignancy. Cryptorchidism carries an increased risk of testicular cancer, and a retroperitoneal germ-cell tumor has been reported in PBS.[26][37] Counsel patients about malignancy risk (EAU weak recommendation) and testicular self-examination during and after puberty.[37]
  • Bladder cancer. Advanced cancer has been reported in a 38-year-old patient with a nonaugmented, nondefunctionalized bladder.[27] A 2026 systematic review found seven bladder carcinomas among 20 published PBS tumor cases, including an index metastatic case at age 33. Selected PBS case reports support prompt investigation of new hematuria; they do not support routine cystoscopic screening of asymptomatic patients.[40]

After lower urinary tract reconstruction incorporating bowel, lifelong follow-up addresses metabolic, stone, perforation and malignancy risks. The AUA/SUFU 2021 guideline specifies annual focused history, examination, basic metabolic panel and urinary-tract imaging for adults with NLUTD after bowel-incorporating lower urinary tract reconstruction (Statement 58; Expert Opinion); the interval for a patient with PBS should reflect the reconstruction and individual renal and bladder risk.[14]

Transition Process and Models of Care​

A retrospective congenital neurogenic-bladder transition program reported successful transfer in 76/79 patients deemed ready, among 157 enrolled[29]. Follow-up remained active in 55/76 transferred versus 63/171 adults without formal transition (72.4% vs 36.8%)[29]. These selected, nonrandomized groups support coordinated care.[29] The International Children's Continence Society supports early, developmentally appropriate transition preparation, building self-management, health literacy, and independence via a readiness-checklist approach.[30]

Elements:

  • Dedicated multidisciplinary clinics with transition coordinators; structured staged models (e.g., T1–T4) with milestone-based progression.[2][28][31]
  • Time alone with the adolescent during consultations to build independence and address confidential issues.[30]
  • Psychosocial-readiness assessment. Health literacy and family support are primary predictors of success; address self-esteem, body image (abdominal-wall appearance), and reproductive counseling.[2][32][33]

Barriers mirror the rest of transitional urology: limited patient education, scarce adult-urologist expertise in congenital disease, financial constraints, and geographic disparity.[2][3]

Quality of Life​

In an online survey of 32 children with PBS, scores were significantly below those of healthy controls across all PedsQL domains: physical (66.3 vs 84.4), emotional (68.4 vs 80.9), social (63.1 vs 87.4), and school (53.0 vs 78.6; p < 0.01 for each). Scores of 19 caregivers were lower than those of a healthy adult comparison group as well.[34] In a retrospective series of 25 children, clinically significant pulmonary and orthopedic problems affected 55% of survivors; chronic constipation was also common.[35] Body-image concerns and the psychosocial weight of infertility should be addressed through transition and into adult care.

Lifelong Surveillance​

Illustrative adult schedule adapted from adjacent NLUTD practice, not a validated PBS guideline. Adjust intervals to renal status, anatomy and changes; pediatric care may require more frequent assessment.

DomainCloser follow-up (abnormal renal or bladder findings)Stable follow-up
History / exam / symptoms, including constipation and other bowel symptoms[44]AnnualAnnual[18]
Renal function (eGFR and, with CKD, urine ACR; consider cystatin C when low muscle mass makes creatinine-based eGFR less accurate and GFR affects decisions)[41]At least annually; more often when CKD progression risk is higher and results would guide treatment[18][41]At least annually, including urine ACR with CKD[18][41]
Upper-tract imaging (US)AnnualIndividualize; every 1–2 years is the AUA/SUFU interval for stable moderate-risk NLUTD[18]
UrodynamicsWhen pressure/compliance information changes managementSymptom-triggered[14][18]
PVREach visitEach visit
UTI / hematuriaEach visitEach visit[27]
Metabolic panel (if bowel reconstruction)AnnualAnnual[14]
Fertility / sexual healthPeriodicallyPeriodically[24]

Key Principles​

  • Renal preservation is the lifelong goal; address infection, obstruction and bladder safety while recognizing persistent congenital renal vulnerability.[8]
  • Bladder emptying changes over time. Combine PVR, symptoms, renal imaging and indicated UDS; the 56% CIC estimate is from one historical 34-patient series.[13]
  • Monfort abdominoplasty was associated with lower PVR and fewer UTIs in a 12-patient series, in addition to its cosmetic effect.[20]
  • Unsafe pressure or compliance requires treatment, but no single pressure cut-off automatically mandates augmentation.[14][19]
  • Counsel on fertility early. Retrograde ejaculation and accessory-gland hypoplasia are common, but motile sperm (4/8 tested in one series) and ICSI-assisted parenthood are achievable; advocate early orchiopexy.[22][24][25]
  • Transplant outcomes are encouraging. Five-year graft survival was 91% in one single-center series and 71% in the Australian registry, and peritoneal dialysis was feasible despite the lax abdominal wall.[9][10]

See Also​

References​

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2. Akdağcık Z, Soytürk S, Sılay MS, et al. "Transitional Urology in Congenital and Neurological Conditions: A Global Review of Structured Care Models and Clinical Outcomes." World J Urol. 2025;43(1):628. doi:10.1007/s00345-025-06021-5

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11. Yalcinkaya F, Bonthuis M, Erdogan BD, et al. "Outcomes of Renal Replacement Therapy in Boys With Prune Belly Syndrome: Findings From the ESPN/ERA-EDTA Registry." Pediatr Nephrol. 2018;33(1):117-124. doi:10.1007/s00467-017-3770-9

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18. Ginsberg DA, Boone TB, Cameron AP, et al. "The AUA/SUFU Guideline on Adult Neurogenic Lower Urinary Tract Dysfunction: Diagnosis and Evaluation." J Urol. 2021;206(5):1097-1105. doi:10.1097/JU.0000000000002235

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24. Lopes RI, Tavares A, Dénes FT, Cocuzza M. "Gonadal Function and Reproductive System Anatomy in Postpubertal Prune-Belly Syndrome Patients." Urology. 2020;145:292-296. doi:10.1016/j.urology.2020.04.124

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42. Braz Ascar PC, Rombaldi MC, Genzani CP, et al. "RUBACE profile of pediatric patients with prune belly syndrome undergoing renal transplantation: Findings from a case series." J Pediatr Urol. 2026 Apr 16:105959. doi:10.1016/j.jpurol.2026.105959

43. Fusaro F, Zanon GF, Ferreli AM, et al. "Renal transplantation in prune-belly syndrome." Transpl Int. 2004;17(9):549-552. doi:10.1007/s00147-004-0759-7

44. Grimsby GM, Harrison SM, Granberg CF, Bernstein IH, Baker LA. "Impact and frequency of extra-genitourinary manifestations of prune belly syndrome." J Pediatr Urol. 2015;11(5):280.e1-280.e6. doi:10.1016/j.jpurol.2015.06.005