Department of Pediatric Surgery, Chang Gung Children's Medical Center, Chang Gung University College of Medicine, Taoyuan 333, Taiwan.
J Pediatr Surg. 2009 Dec;44(12):2406-9. doi: 10.1016/j.jpedsurg.2009.09.016.
BACKGROUND/PURPOSE: Congenital colonic atresia (CA) or stenosis is an infrequent cause of low intestinal obstruction in the neonate. Atresias can occur at any level, and the management of CA is determined by the atretic site and by the presence or absence of associated anomalies. We report our experience dealing with upper rectal atresia during a 5-year period.
Between January 2004 and December 2008, 3 female newborns with upper rectal atresia with or without associated anomalies were treated. Modes of clinical presentation, methods of diagnosis, associated anomalies, alternative management techniques, and clinical outcome were retrospectively analyzed.
All 3 patients had progressive abdominal distension, bilious vomiting, and failure to pass meconium. Contrast enema showed an atresia at the upper rectum in 2 patients. At laparotomy, case 1 was found to have type III atresia of the upper rectum. Resection of the dilated portion of the proximal colon with end sigmoid colostomy was accomplished in the neonatal period followed by a transanal mucosectomy with takedown of the colostomy and a pull-through procedure at age 3 months. Case 3 had multiple jejunoileal atresias and an upper rectal atresia. The initial management was multiple resections of atretic bowel and anastomoses and an end sigmoid colostomy. The secondary procedure was a takedown of the colostomy and transanal mucosectomy with a pull-through procedure. Case 2 had type I upper rectal atresia in association with imperforate anus complicated by colon perforation during performance of a distal colostogram leading to a complicated and protracted clinical course. All the patients are currently well with voluntary bowel movements, and one has occasional soiling with follow-up of 9 months to 3 years.
Colon atresia, especially at the level of the upper rectum, is uncommon. Whether to proceed with an ostomy or to individualize the operative procedure according to the location of the atresia is still controversial. Transanal mucosectomy was a useful technique at the time of the definitive pull-through for the treatment of upper rectal atresia. In cases of upper CA associated with imperforate anus, delay in diagnosis and potential complications may result if the diagnosis of upper rectal atresia is missed.
背景/目的：先天性结肠闭锁（CA）或狭窄是新生儿低位肠梗阻的一个罕见原因。闭锁可发生在任何部位，CA 的治疗取决于闭锁部位以及是否存在相关畸形。我们报告了我们在 5 年期间处理高位直肠闭锁的经验。
2004 年 1 月至 2008 年 12 月，我们治疗了 3 例伴有或不伴有相关畸形的高位直肠闭锁的女新生儿。回顾性分析了临床表现模式、诊断方法、相关畸形、替代治疗技术和临床结果。
所有 3 例患者均有进行性腹胀、胆汁性呕吐和胎粪排出失败。对比灌肠显示 2 例患者存在高位直肠闭锁。剖腹探查时，例 1 发现高位直肠 III 型闭锁。新生儿期行近端结肠扩张段切除及乙状结肠造口术，3 个月时行经肛门直肠黏膜切除术，行造口关闭和拖出术。例 3 有多处空肠回肠闭锁和高位直肠闭锁。初始治疗是多次切除闭锁肠段和吻合，并进行乙状结肠造口术。二次手术是关闭造口和经肛门直肠黏膜切除术，并进行拖出术。例 2 有高位直肠闭锁，同时伴有肛门闭锁伴直肠穿孔，在行远端结肠造影时导致复杂和迁延的临床病程。所有患者目前情况良好，能自主排便，1 例有偶尔的污染，随访时间为 9 个月至 3 年。
结肠闭锁，特别是高位直肠闭锁，并不常见。是否行造口术，还是根据闭锁部位个体化手术，仍存在争议。经肛门直肠黏膜切除术是高位直肠闭锁根治性拖出术的一种有用技术。对于高位 CA 伴肛门闭锁，如漏诊高位直肠闭锁，可能会导致诊断延迟和潜在并发症。
