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Prune-Belly Syndrome

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Pediatric Surgery

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Abstract

Prune-belly syndrome has three main features: urinary tract dilation, cryptorchidism, and abdominal wall flaccidity, with potential associated pulmonary, cardiac, gastro-intestinal, and musculoskeletal abnormalities. It has three categories of presentation according to the severity of the malformations. Category 1 patients have the most severe nephro-urological abnormalities, with associated pulmonary hypoplasia, and rarely survive the neonatal period. Category III patients have scant uro-nephrological abnormalities and only require orchidopexy and potentially abdominoplasty. Up to 30% of patients with category II syndrome, despite initial normal renal function, develop chronic renal failure during childhood or adolescence due to urinary tract obstruction and infection. In order to prevent late renal insufficiency in this group, continuous urinary tract monitoring and antibiotic prophylaxis are necessary in these patients. Upper urinary tract reconstruction is indicated when there is concern of progressive functional or anatomical deterioration due to obstruction or high-grade vesicoureteral reflux (VUR), with or without recurrent upper tract infections. Abdominoplasty improves body posture, as well as bladder emptying, cough, and defecation in some patient series. Orchidopexy in one or two stages is required in all patients of categories II and III, being best performed early in life. Comprehensive surgery that includes urinary tract reconstruction, orchidopexy, and abdominoplasty is feasible and has good results in properly selected patients. With individualization of care and improvements in the surgical management, the overall outlook for survival and quality of life of these patients has improved considerably.

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Authors and Affiliations

  1. Pediatric Urology Unit, Division of Urology, Hospital das Clínicas, University of São Paulo, São Paulo, SP, Brazil

    Francisco T. Dénes

  2. Section of Pediatric Urology, Hasbro Children’s Hospital, Warren Alpert School of Medicine of Brown University, Providence, RI, USA

    Anthony A. Caldamone

Authors
  1. Francisco T. Dénes
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  2. Anthony A. Caldamone
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Correspondence to Francisco T. Dénes .

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Editors and Affiliations

  1. Newman Clinical Research Professor, University College Dublin, Dublin, Ireland

    Prem Puri

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Dénes, F.T., Caldamone, A.A. (2023). Prune-Belly Syndrome. In: Puri, P. (eds) Pediatric Surgery. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-43567-0_186

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  • DOI: https://doi.org/10.1007/978-3-662-43567-0_186

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  • Print ISBN: 978-3-662-43566-3

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