Abstract：
BACKGROUND: Prune belly syndrome (PBS) is characterized by the triad of abdominal flaccidity, bilateral undescended testicles and genitourinary tract anomalies. A variable spectrum of abdominal wall laxity is observed in PBS. We present the first case of a novel technique using a minimally invasive abdominoplasty to specifically address patients with localized abdominal wall weakness in PBS.
METHODS: A two-years-old child with PBS presented with recurrent febrile urinary tract infections. Ultrasonography demonstrated a dysplastic right kidney associated with significant ipsilateral ureterohydronephrosis. Voiding urethrocystogram did not show vesicoureteral reflux and DMSA scan depicted a non-functioning right kidney. During laparoscopic right nephroureterectomy and first stage Fowler-Stephens bilateral orchiopexies, a significant right-sided lateral abdominal wall bulging was observed. A minimally invasive laparoscopic abdominoplasty was performed with a one-way running suture using an unabsorbable 2.0 prolene approximating the edges of the musculofascial defect. While undergoing the second-stage Fowler-Stephens orchiopexy, no bulging was observed.
CONCLUSIONS: A minimally invasive abdominoplasty to improve abdominal wall lateral bulging in PBS was feasible and presented good cosmetic result. We anticipate that this technique can be applied for children with PBS with primary lateral abdominal wall bulging, employing one or more suture lines depending on the fascial defect size.
METHODS: A two-years-old child with PBS presented with recurrent febrile urinary tract infections. Ultrasonography demonstrated a dysplastic right kidney associated with significant ipsilateral ureterohydronephrosis. Voiding urethrocystogram did not show vesicoureteral reflux and DMSA scan depicted a non-functioning right kidney. During laparoscopic right nephroureterectomy and first stage Fowler-Stephens bilateral orchiopexies, a significant right-sided lateral abdominal wall bulging was observed. A minimally invasive laparoscopic abdominoplasty was performed with a one-way running suture using an unabsorbable 2.0 prolene approximating the edges of the musculofascial defect. While undergoing the second-stage Fowler-Stephens orchiopexy, no bulging was observed.
CONCLUSIONS: A minimally invasive abdominoplasty to improve abdominal wall lateral bulging in PBS was feasible and presented good cosmetic result. We anticipate that this technique can be applied for children with PBS with primary lateral abdominal wall bulging, employing one or more suture lines depending on the fascial defect size.
摘要：
背景：修剪腹部综合征（PBS）的特征是腹部松弛的三联征，双侧睾丸未降和泌尿生殖道异常。在PBS中观察到腹壁松弛的可变光谱。我们介绍了第一例使用微创腹部成形术的新技术，以专门针对PBS中局部腹壁无力的患者。
方法：一名患有PBS的2岁儿童反复出现发热性尿路感染。超声检查显示右肾发育不良与同侧输尿管肾积水有关。尿路膀胱造影未显示膀胱输尿管反流，DMSA扫描显示右肾无功能。在腹腔镜右肾输尿管切除术和一期Fowler-Stephens双侧睾丸固定术中，观察到明显的右侧外侧腹壁鼓胀。使用不可吸收的2.0prolene近似肌肉筋膜缺损的边缘，通过单向运行缝合线进行了微创腹腔镜腹部成形术。在接受第二阶段福勒-斯蒂芬斯睾丸手术时，没有观察到鼓起。
结论：在PBS中进行微创腹部成形术以改善腹壁外侧膨出是可行的，并且具有良好的美容效果。我们预计这种技术可以应用于PBS的儿童原发性侧腹壁鼓胀，根据筋膜缺损的大小采用一条或多条缝合线。
方法：一名患有PBS的2岁儿童反复出现发热性尿路感染。超声检查显示右肾发育不良与同侧输尿管肾积水有关。尿路膀胱造影未显示膀胱输尿管反流，DMSA扫描显示右肾无功能。在腹腔镜右肾输尿管切除术和一期Fowler-Stephens双侧睾丸固定术中，观察到明显的右侧外侧腹壁鼓胀。使用不可吸收的2.0prolene近似肌肉筋膜缺损的边缘，通过单向运行缝合线进行了微创腹腔镜腹部成形术。在接受第二阶段福勒-斯蒂芬斯睾丸手术时，没有观察到鼓起。
结论：在PBS中进行微创腹部成形术以改善腹壁外侧膨出是可行的，并且具有良好的美容效果。我们预计这种技术可以应用于PBS的儿童原发性侧腹壁鼓胀，根据筋膜缺损的大小采用一条或多条缝合线。
