Rodrigo Alexandre Trivilato, Gabriela Dadalt, Deborah Lima Assenço, Débora Fernandes Barbalho, Márcio Rodrigues Costa, Nadin Chater, Rodrigo Rosa Lima, Bernardo Monteiro Antunes Barreira, João Paulo de Bessa Teixeira, Fernando Cruvinel, José Luiz Figueiredo
{"title":"Challenges in the surgical treatment of a 8-year old child living with Prune Belly syndrome: case report","authors":"Rodrigo Alexandre Trivilato, Gabriela Dadalt, Deborah Lima Assenço, Débora Fernandes Barbalho, Márcio Rodrigues Costa, Nadin Chater, Rodrigo Rosa Lima, Bernardo Monteiro Antunes Barreira, João Paulo de Bessa Teixeira, Fernando Cruvinel, José Luiz Figueiredo","doi":"10.36311/jhgd.v33.13793","DOIUrl":null,"url":null,"abstract":"Prune Belly syndrome, also known as Eagle-Barret syndrome, is a rare disease, with a prevalence of 3.8 live births per 100,000 births. Its main characteristic is the hypoplasia of the abdominal muscles, giving rise to the name “prune belly syndrome”. The gold standard treatment is surgery, ideally with correction of cryptorchidism and phimosis between 6 to 18 months of life. Correction of urinary malformations and abdominoplasty should be performed up to 4 years of age. Little evidence exists in the literature about late treatment and its implications for prognosis. The context mentioned above led us to present an uncommon case of a six years old child in which a surgical approach was performed later than usual.","PeriodicalId":35218,"journal":{"name":"Journal of Human Growth and Development","volume":" ","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2023-03-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Human Growth and Development","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.36311/jhgd.v33.13793","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 0
Abstract
Prune Belly syndrome, also known as Eagle-Barret syndrome, is a rare disease, with a prevalence of 3.8 live births per 100,000 births. Its main characteristic is the hypoplasia of the abdominal muscles, giving rise to the name “prune belly syndrome”. The gold standard treatment is surgery, ideally with correction of cryptorchidism and phimosis between 6 to 18 months of life. Correction of urinary malformations and abdominoplasty should be performed up to 4 years of age. Little evidence exists in the literature about late treatment and its implications for prognosis. The context mentioned above led us to present an uncommon case of a six years old child in which a surgical approach was performed later than usual.