title: Clinical manifestations and management of prune-belly syndrome in a large contemporary pediatric population.
abstract: OBJECTIVE
  To review the clinical manifestations and operative management of a large contemporary pediatric cohort of patients with prune-belly syndrome (PBS).
  
  
  METHODS
  PBS patients aged <21 years followed up in our pediatric urology clinic were identified by the International Classification of Diseases, Ninth Revision code (756.71). Demographics, concomitant diagnoses, surgical history, imaging studies, and renal or bladder function were evaluated.
  
  
  RESULTS
  Data were available for 46 pediatric patients (44 boys and 2 girls). Mean age was 7.6 ± 4.7 years (range, 0.9-20 years). Average length of clinical follow-up was 6.8 ± 5 years. Forty-five children (97.8%) had hydroureteronephrosis, and 36 of them (78.3%) had vesicoureteral reflux. Five patients (10.9%) had significant pulmonary insufficiency, and 2 patients (4.3%) were oxygen dependent. Eighteen children (39.1%) had other congenital malformations, including cardiac in 4 patients (8.7%) and musculoskeletal anomalies in 10 patients (21.7%). Orchidopexy was the most common surgery, with all boys aged ≥3 years having undergone the procedure. Twenty-two patients (47.8%) had a history of ureteral surgery, 22 (47.8%) had bladder surgery, 11 (23.9%) had renal surgery, and 6 (13%) had urethral procedures. Nineteen patients (41.3%) underwent abdominoplasty. Eighteen children (39.1%) had documented chronic kidney disease, and 8 children (17.4%) underwent renal transplantation. Average age at transplantation was 5.1 ± 2.9 years. The mean nadir creatinine level for patients with end-stage renal disease was 1.4 mg/dL compared with 0.4 mg/dL for those not requiring transplantation (P <.001).
  
  
  CONCLUSIONS
  Children with PBS have significant comorbidities and require frequent operative intervention, with disease heterogeneity necessitating an individualized management approach. Early end-stage renal disease is prevalent, with approximately 15% of children requiring kidney transplantation.
authors.affiliation: Department of Pediatric Urology, Children's Healthcare of Atlanta and Emory University School of Medicine, Atlanta, GA.
authors.forename: Natan E
authors.initials: NE
authors.lastname: Seidel
authors.affiliation: Department of Pediatric Urology, Children's Healthcare of Atlanta and Emory University School of Medicine, Atlanta, GA.
authors.forename: Angela M
authors.initials: AM
authors.lastname: Arlen
authors.affiliation: Department of Pediatric Urology, Children's Healthcare of Atlanta and Emory University School of Medicine, Atlanta, GA.
authors.forename: Edwin A
authors.initials: EA
authors.lastname: Smith
authors.affiliation: Department of Pediatric Urology, Children's Healthcare of Atlanta and Emory University School of Medicine, Atlanta, GA. Electronic address: akirschmd@gmail.com.
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Kirsch
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D011535:Prune Belly Syndrome / Q000150:complications / Q000175:diagnosis* / Q000601:surgery*; D012189:Retrospective Studies; D014570:Urologic Diseases / Q000209:etiology / Q000601:surgery; D055815:Young Adult