Sharma Shilpa, Gupta Devendra K
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, 110029, India.
Pediatr Surg Int. 2017 Aug;33(8):829-836. doi: 10.1007/s00383-017-4106-3. Epub 2017 Jun 10.
Rectal atresia (RA) and rectal stenosis (RS) are rare anomalies with varied treatment options. A thorough literature review was done on reported cases/series of RA and RS. Based on evidence from cases managed over last 15 years, new insights into embryology were hypothesized. A comprehensive review was compiled with updated knowledge on diagnosis and management. RA is classified into five types I: II: III: IV: V as RS: RA with septal defect: RA with a fibrous cord between two atretic ends: RA with a gap: Multiple RA and/or RS. Current definitive surgical repair of these anomalies preserves the anal canal, dentate line, and sphincter complex. Most neonates with RA undergo sigmoid colostomy except few with RS who can rarely decompress adequately. Membranous RS and septal RA may seldom respond to dilatation or be amenable to transanal repair. Posterior sagittal anorectoplasty with an end-to-end/side repair is recommended for RA and most intramural RS. RS may be associated with a presacral mass and colonic/rectal motility disorders. The expected postoperative outcome is good if the normally developed anal sphincter complex is retained undamaged. Early recognition of the type of anomaly is necessary for appropriate management.
直肠闭锁(RA)和直肠狭窄(RS)是罕见的异常情况,治疗方案多样。我们对已报道的RA和RS病例/系列进行了全面的文献综述。基于过去15年所治疗病例的证据,对胚胎学提出了新的见解。结合诊断和治疗的最新知识进行了全面综述。RA分为五种类型:I型、II型、III型、IV型、V型,RS包括:伴有隔膜缺损的RA、两个闭锁端之间有纤维索带的RA、有间隙的RA、多发RA和/或RS。目前对这些异常情况的确定性手术修复保留了肛管、齿状线和括约肌复合体。大多数患有RA的新生儿需行乙状结肠造口术,少数患有RS的新生儿很少能充分减压。膜性RS和隔膜性RA很少对扩张治疗有反应或适合经肛门修复。对于RA和大多数壁内RS,建议采用后矢状位肛门直肠成形术并进行端端/侧方修复。RS可能与骶前肿块和结肠/直肠动力障碍有关。如果正常发育的肛门括约肌复合体未受损,术后预期效果良好。早期识别异常类型对于恰当的治疗很有必要。