Prune Belly Syndrome (PBS) (Eagle-Barrett Syndrome)
摘要
Eagle and Barret first described prune belly syndrome (PBS) in 1950 (Eagle Jr and Barrett 1950). This rare multisystem disease is characterized by the triad of abdominal muscle deficiency, bilateral intra-abdominal cryptorchidism, and urinary tract abnormalities, including megaureter, hydroureter, hydronephrosis, vesicoureteral reflux, and megacystis (Quilici et al. 2023). The name of this syndrome is derived from the crumpled appearance of the abdomen, similar to prune skin, which results from the absence of abdominal wall muscles and protrusion of the anterior abdominal wall (Achour et al. 2018). The symptoms can be identified during pregnancy or the newborn period (“RareDiseases. Prune belly syndrome (2023)”). The clinical manifestations of PBS can range from a stillbirth caused by severe renal and respiratory dysplasia to a child that looks almost normal (Achour et al. 2018). The varying severity and patterns of abdominal muscle weakness can also cause PBS patients to manifest unique characteristics (Arlen et al. 2019). PBS is a rare congenital disorder affecting primarily boys. It occurs in 3.8 out of every 100,000 live male births. Less than 5% of all PBS cases are girls (Arlen et al. 2019). Incidence is higher in infants born to younger mothers, and prevalence is higher among the black population (Puvabanditsin et al. 2022).