Borg Helena, Holmdahl Gundela, Olsson Ingrid, Wiklund Lars-Martin, Sillén Ulla
Department of Pediatric Surgery, The Queen Silvia Children's Hospital, The Sahlgrenska Academy at University of Gothenburg, 416 85 Gothenburg, Sweden.
J Pediatr Surg. 2009 Sep;44(9):1778-85. doi: 10.1016/j.jpedsurg.2009.03.001.
Risk factors for the presence of neurogenic bladder dysfunction (NBD) in children born with high anorectal malformations (ARMs), were investigated, to identify the need for urodynamics in these patients.
The study included 37 patients with high ARMs (21 boys and 16 girls). Bladder function was evaluated with urodynamics both before and after anorectoplasty (posterior sagittal anorectoplasty [PSARP]). All patients were investigated with spinal radiograph. Spinal ultrasound was performed in the neonatal period, and magnetic resonance imaging was added in case of abnormal ultrasound or urodynamics and in case of cloacal malformation.
In ARM patients with rectourethral and vestibular fistulas and cloacas, NBD was identified in 9 children (25%). The bladder dysfunction was innate in all cases except in one girl with cloaca, indicating that the risk of iatrogenic denervation seems minimal using the PSARP technique. All children with innate NBD had a spinal cord malformation either as spinal cord regression or tethering with or without a lipoma. Concerning vertebral status, almost all children with NBD had partial sacral agenesis. Abnormal perineal appearance was highly correlated to NBD in boys, especially in those with a spinal cord regression malformation. Innate NBD was not found in any child with normal spinal cord.
From these results, we suggest that spinal ultrasound and perineal inspection are used as screening procedures for NBD in children with ARM. Urodynamic investigation is recommended only when spinal cord anomalies or other signs indicative of NBD are present. In case of spinal cord malformation, repeated urodynamics during follow-up is mandatory because of the risk for developing tethered cord syndrome.
研究患有高位肛门直肠畸形(ARM)的儿童出现神经源性膀胱功能障碍(NBD)的危险因素,以确定这些患者是否需要进行尿动力学检查。
该研究纳入了37例高位ARM患者(21例男孩和16例女孩)。在肛门直肠成形术(后矢状位肛门直肠成形术[PSARP])前后均通过尿动力学评估膀胱功能。所有患者均进行了脊柱X线检查。在新生儿期进行脊柱超声检查,若超声或尿动力学检查异常以及存在泄殖腔畸形,则加做磁共振成像。
在患有直肠尿道瘘、前庭瘘和泄殖腔畸形的ARM患者中,9名儿童(25%)被诊断为NBD。除1例患有泄殖腔畸形的女孩外,所有病例的膀胱功能障碍均为先天性,这表明使用PSARP技术导致医源性去神经支配的风险似乎极小。所有先天性NBD患儿均存在脊髓畸形,表现为脊髓发育不良或脊髓栓系伴或不伴脂肪瘤。关于脊柱状况,几乎所有NBD患儿都有部分骶骨发育不全。男孩异常的会阴外观与NBD高度相关,尤其是那些患有脊髓发育不良畸形的男孩。脊髓正常的儿童未发现先天性NBD。
根据这些结果,我们建议将脊柱超声和会阴检查作为ARM患儿NBD的筛查程序。仅当存在脊髓异常或其他提示NBD的体征时,才建议进行尿动力学检查。对于存在脊髓畸形的情况,由于有发生脊髓栓系综合征的风险,随访期间必须重复进行尿动力学检查。