Kumar A, Agarwala S, Srinivas M, Bajpai M, Bhatnagar V, Gupta D K, Gupta A K, Mitra D K
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
Indian J Pediatr. 2005 Dec;72(12):1039-42. doi: 10.1007/BF02724407.
To evaluate the incidence, types and the effect on outcome of associated anomalies in neonates with anorectal malformations (ARM).
This retrospective study was carried out on all neonates with ARM admitted to the neonatal surgical intensive care unit (NSICU) from 1998 through 2003.
Of the 754 neonates admitted to the NSICU during the study period of 6 years, there were 124 (16.4%) neonates with anorectal malformations. Of these 110 were included in the study. 73 % were male and 27% female. 86% of these were high ARM (HARM) while only 14% were low ARM (LARM). Associated anomalies were seen in 68% of patients. The incidence was 72% for HARM and 50% for LARM. The major associated anomalies consisted of esophageal (13%), gastrointestinal (GIT) (11%), genitourinary (GUT) (32%), skeletal (26%), cardiac (33%) and miscellaneous 26%. The overall survival rate was 84% (82% for HARM and 94% for LARM). The survival among those with associated esophageal anomalies was 43%, GIT 67%, GUT 80%, cardiac 61%, skeletal 76% and miscellaneous 79% respectively. This difference in survival was significant only for those with esophageal (p=0.004) and cardiac anomalies (p=0.0026). The survival rates among those with one, two or more than two organ systems involved with associated anomalies were 88%, 82% and 58% respectively. This difference was significant only for more than two organ systems involvement (p=0.003).
Associated anomalies are common in neonates with ARM, the incidence being similar for HARM and LARM. The survival depends upon the number and severity of associated anomalies both in patients with LARM and HARM. Neonates with more number of organ systems involved have a poorer survival specially when associated with esophageal and cardiac anomalies. All neonates with ARM merit a meticulous search for associated anomalies so that the management can be tailored for each baby.
评估肛门直肠畸形（ARM）新生儿相关畸形的发生率、类型及其对预后的影响。
对1998年至2003年入住新生儿外科重症监护病房（NSICU）的所有ARM新生儿进行回顾性研究。
在为期6年的研究期间，入住NSICU的754例新生儿中，有124例（16.4%）患有肛门直肠畸形。其中110例纳入研究。男性占73%，女性占27%。这些患儿中86%为高位肛门直肠畸形（HARM），仅14%为低位肛门直肠畸形（LARM）。68%的患儿存在相关畸形。HARM的发生率为72%，LARM为50%。主要相关畸形包括食管（13%）、胃肠道（GIT，11%）、泌尿生殖系统（GUT，32%）、骨骼（26%）、心脏（33%）及其他（26%）。总体生存率为84%（HARM为82%，LARM为94%）。伴有食管畸形者的生存率为43%，GIT畸形者为67%，GUT畸形者为80%，心脏畸形者为61%，骨骼畸形者为76%，其他畸形者为79%。仅食管畸形（p = 0.004）和心脏畸形（p = 0.0026）患儿的生存差异具有统计学意义。伴有1个、2个或2个以上器官系统相关畸形患儿的生存率分别为88%、82%和58%。仅2个以上器官系统受累时差异具有统计学意义（p = 0.003）。
ARM新生儿中相关畸形常见，HARM和LARM的发生率相似。LARM和HARM患儿的生存取决于相关畸形的数量和严重程度。涉及器官系统较多的新生儿生存率较低，尤其是伴有食管和心脏畸形时。所有ARM新生儿均需仔细检查是否存在相关畸形，以便为每个患儿制定个性化治疗方案。
