Ji Weiwei, Xue Huiling, Gu Ying, Wan Yanmin, Lu Liangsheng, Fan Yong
Department of Pediatric Urology, Children's Hospital of Fudan University, Shanghai, China.
Nursing Department, Children's Hospital of Fudan University, Shanghai, China.
Front Pediatr. 2025 Sep 4;13:1624556. doi: 10.3389/fped.2025.1624556. eCollection 2025.
Prune belly syndrome (PBS), a rare congenital disorder characterized by the absence of abdominal wall musculature and abnormalities in the genitourinary tract, is primarily linked to the urethral obstruction during fetal development. Given the overall rarity of the PBS and its multisystem involvement, there is currently no consensus on the optimal management strategy for PBS patients. This case report elaborated the comprehensive therapeutic outcomes of a patient with PBS, who had previously undergone several surgeries, including vesicoscopic cross-trigonal ureteral reimplantation, and first-stage Fowler-Stephens orchiopexy (FSO) for right cryptorchidism. At the age of seven, the patient underwent one-stage FSO combined with microvascular anastomosis for the left intra-abdominal testis. With appropriate perioperative management, the left testis was successfully survived. Herein, we presented an illustrative case to serve as a treatment reference for PBS patients.
梅干腹综合征(PBS)是一种罕见的先天性疾病,其特征为腹壁肌肉组织缺失和泌尿生殖道异常,主要与胎儿发育期间的尿道梗阻有关。鉴于PBS总体罕见且累及多系统,目前对于PBS患者的最佳管理策略尚无共识。本病例报告阐述了一名PBS患者的综合治疗结果,该患者此前接受了多次手术,包括膀胱镜下经三角区输尿管再植术以及针对右侧隐睾的一期福勒-斯蒂芬斯睾丸固定术(FSO)。七岁时,该患者接受了一期FSO联合微血管吻合术治疗左侧腹腔内睾丸。通过适当的围手术期管理,左侧睾丸成功存活。在此,我们展示一个典型病例,为PBS患者提供治疗参考。