Hoehner J C, Ein S H, Shandling B, Kim P C
Division of General Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada.
J Pediatr Surg. 1998 Jul;33(7):961-5; discussion 965-6. doi: 10.1016/s0022-3468(98)90515-2.
BACKGROUND/PURPOSE: No surgical treatment for total colonic aganglionosis (TCA) clearly has been proven superior. To identify clinical criteria associated with long-term functional outcome, a 28-year retrospective institutional review of this entity from 1969 through 1996 inclusive was undertaken.
Total colonic aganglionosis (TCA), defined here as aganglionosis extending from the anus to at least the ileocecal valve but no further than 50 cm proximal to the ileocecal valve, was identified in 29 infants and children. Appropriate leveling ileostomy was performed in 28 of 29 patients, and definitive surgical reconstruction was performed in 26 of 29. Three groups were identified based on the definitive surgical repair performed: group 1, construction lacking or incorporating a short ganglionic-aganglionic common channel (modified Soave or modified Duhamel, n = 8); group II, construction of an extended common channel (Martin-Duhamel, Martin-Soave; n = 6); and group III, all others including an intermediate-length common channel (n = 13).
Functional outcome at extended follow-up (mean, 6.6 +/- 5.6 years; range, 0.7 to 23) was determined based on survival, long-term ostomy requirements, growth, major complications, continence, and enterocolitis and bowel movement frequency. Although long-term functional outcome was deemed satisfactory in six of seven patients in group I, function was satisfactory in none of six group II patients. Group III results were intermediate (satisfactory in 6 of 13).
Acceptable long-term outcome was most frequent in TCA patients whose definitive repair did not incorporate an extended ganglionic-aganglionic common channel. The use of extensive lengths of aganglionic bowel to maximize fluid absorption is frequently met with substantial morbidity.
背景/目的：目前尚无明确证据表明哪种针对全结肠无神经节症（TCA）的手术治疗方法更为优越。为了确定与长期功能预后相关的临床标准，我们对1969年至1996年（含）期间该病症进行了一项为期28年的机构回顾性研究。
共纳入29例婴儿和儿童，确诊为全结肠无神经节症（TCA），此处定义为无神经节症从肛门延伸至至少回盲瓣，但不超过回盲瓣近端50 cm。29例患者中有28例行适当的低位回肠造口术，29例中有26例行确定性手术重建。根据所进行的确定性手术修复方式将患者分为三组：第1组，构建方式缺乏或包含短的神经节-无神经节共同通道（改良Soave或改良Duhamel，n = 8）；第II组，构建延长的共同通道（Martin-Duhamel、Martin-Soave；n = 6）；第III组，包括中等长度共同通道在内的其他所有方式（n = 13）。
基于生存情况、长期造口需求、生长发育、主要并发症、控便能力、小肠结肠炎以及排便频率，确定了延长随访期（平均6.6±5.6年；范围0.7至23年）的功能预后。尽管第1组7例患者中有6例的长期功能预后被认为满意，但第II组6例患者中无1例功能满意。第III组结果居中（13例中有6例满意）。
在全结肠无神经节症患者中，确定性修复未采用延长的神经节-无神经节共同通道的患者，最常获得可接受的长期预后。使用长段无神经节肠管以最大化液体吸收，常常伴随着较高的发病率。
