Actualización en el manejo del síndrome de Prune Belly

Abstract

Introduction: Prune Belly syndrome is a rare congenital disorder that is characterized by a triad that includes abdominal muscle deficiency, cryptorchidism and urinary tract abnormalities. The incidence of Prune Belly syndrome in the world is around 1 per 30,000 live births, in Latin America it is reported that its incidence is 3.8 cases per 100,000 male live births. On physical examination, the characteristic wrinkled appearance of the abdominal wall is the most notable physical finding. The management of patients with Prune Belly Syndrome requires a large multidisciplinary team approach to help these children thrive, gain weight, and be prepared for urological surgery if necessary. Objective: To prepare an easy-to-understand scientific article with updated and real information on the treatment of Prune Belly syndrome. Materials and Methods: A bibliographic review was carried out, using articles updated in the last 5 years, published in journals with an impact factor greater than 1 to ensure the quality of the information. The main databases used were: PubMed, Scopus, Medline, ProQuest, Dovepress, Clinical Practice Guidelines, ReciMundo (world scientific journal of research and knowledge). Results: A bibliographic review article was prepared with updated and highly interesting information in relation to the proposed topic, in addition, once all the information was reviewed, it was possible to reach the conclusion about the best treatment proposed by experts. Conclusion: Surgical treatment of children with Prune Belly syndrome can be divided into three steps: urinary tract reconstruction, abdominal wall reconstruction, and orchidopexy. Current recommendations indicate performing the Montfort technique in the pediatric age, taking advantage of the moment for urinary tract reconstruction if required, and for orchidopexy in a single surgical step, it is recommended to perform a second surgery with placement of a polypropylene mesh. through abdominoplasty with the Grazer technique, with the aim of providing an aesthetic improvement and support of the intra-abdominal organs.