Tonni Gabriele, Ida Vito, Alessandro Ventura, Bonasoni Maria Paola
Department of Obstetrics & Gynceology, Guastalla General Hospital, AUSL Reggio Emilia, Italy.
Fetal Pediatr Pathol. 2013 Feb;31(1):13-24. doi: 10.3109/15513815.2012.659411. Epub 2012 Apr 16.
Prune-belly syndrome (PBS) is a rare congenital syndrome characterized by deficient abdominal muscles, urinary tract malformation, and in males, cryptorchidism and has an estimated incidence of 1 in 35,000 to 1 in 50,000 live births. The syndrome might be due to severe bladder outlet obstruction or to abdominal muscle deficiency secondary to a migrational defect of the lateral mesoblast between weeks 6 and 7 of pregnancy. The current review of the medical record reports a special focus on epidemiology, genetic factors, early prenatal diagnosis clusters, treatment, and prognosis of PBS.
梅干腹综合征(PBS)是一种罕见的先天性综合征,其特征为腹肌发育不全、泌尿系统畸形,男性患者还伴有隐睾症,估计发病率为每35000至50000例活产中有1例。该综合征可能是由于严重的膀胱出口梗阻,或因妊娠第6至7周侧中胚层迁移缺陷继发的腹肌缺乏所致。本次病历综述特别关注梅干腹综合征的流行病学、遗传因素、早期产前诊断聚类、治疗及预后。