Nievelstein R A J, Vos A, Valk J, Vermeij-Keers Chr
Department of Radiology, University Medical Centre Utrecht, Wilhelmina Children's Hospital, Utrecht, The Netherlands.
J Pediatr Surg. 2002 Aug;37(8):1138-45. doi: 10.1053/jpsu.2002.34459.
The aim of this study was to evaluate the spectrum of developmental anomalies in the caudal region of patients with anorectal malformations (ARM) and relate them to the new insights in the embryogenesis of this region.
Forty-nine patients with ARM were investigated with magnetic resonance imaging (MRI); 24 preoperatively (preop) and 25 postoperatively (postop). Of this group, 17 patients had a high (or intermediate) ARM, 28 a low ARM, and 4 a cloacal malformation (CM). The evaluation of the imaging studies included the level and type of ARM (preop); the developmental state of the sphincter muscle complex (SMC); and the associated anomalies of spinal cord, spine, and urogenital system. The anomalies detected were ordered and related to recent embryologic observations in the caudal region.
With MRI, the level of ARM was depicted effectively in 23 of 24 patients (96%) investigated preop, including 5 of 9 clinically proven fistulae in those with high ARM and CM (56%). Maldevelopment of the SMC was observed in 14 of 49 patients (29%). Associated anomalies of spinal cord and spine were found in 51% of patients, more frequently in those with high ARM, low ARM with fistulae, and cloacal malformations. Associated anomalies of the urogenital system were found in 37% of patients.
As illustrated by the current study, MRI has become indispensable for the visualization of the nature of congenital ARM and associated anomalies. Moreover, MRI aids in understanding the morphology and pathogenesis of these complex congenital malformations. Based on the MRI and recent embryologic observations, a new and simplified classification of ARM is introduced, including a more appropriate nomenclature.
本研究旨在评估肛门直肠畸形(ARM)患者尾侧区域发育异常的范围,并将其与该区域胚胎发生的新见解相关联。
对49例ARM患者进行了磁共振成像(MRI)检查;24例术前检查,25例术后检查。该组患者中,17例为高位(或中间位)ARM,28例为低位ARM,4例为泄殖腔畸形(CM)。影像学研究评估包括ARM的水平和类型(术前);括约肌复合体(SMC)的发育状态;以及脊髓、脊柱和泌尿生殖系统的相关异常。将检测到的异常进行整理,并与尾侧区域最近的胚胎学观察结果相关联。
通过MRI,在24例术前检查的患者中有23例(96%)有效地显示了ARM的水平,包括9例高位ARM和CM患者中临床上证实的5例瘘管(56%)。49例患者中有14例(29%)观察到SMC发育不良。51%的患者发现有脊髓和脊柱相关异常,在高位ARM、伴有瘘管的低位ARM和泄殖腔畸形患者中更为常见。37%的患者发现有泌尿生殖系统相关异常。
如本研究所示,MRI对于先天性ARM及其相关异常的性质可视化已变得不可或缺。此外,MRI有助于理解这些复杂先天性畸形的形态和发病机制。基于MRI和最近的胚胎学观察结果,引入了一种新的、简化版的ARM分类,包括更合适的命名法。