Ieiri Satoshi, Suita Sachiyo, Nakatsuji Takanori, Akiyoshi Junko, Taguchi Tomoaki
Department of Pediatric Surgery, Reproductive and Developmental Medicine, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan.
J Pediatr Surg. 2008 Dec;43(12):2226-30. doi: 10.1016/j.jpedsurg.2008.08.049.
We conducted a nationwide survey in Japan, to identify recent trends in the diagnosis and treatment of patients who had total colonic aganglionosis with or without small bowel involvement (TCSA). These data were compared with 2 similar studies conducted within the last 30 years.
Patient data were collected in 3 phases; phase I included 135 patients between 1978 and 1982; phase II, 107 patients between 1988 and 1992; and phase III, 101 patients between 1998 and 2002.
The incidence of TCSA was 1:59,059, 1:58,084, and 1:58,375, and the male-female ratios were 1.5:1, 1.5:1, and 2.2:1 in each phase, respectively. Patients with associated anomalies increased from 15.2% (phase I) to 22.8% (phase III). The incidence of preoperative enterocolitis decreased over time. For treatment, Duhamel's procedure and ascending colon patch methods have increased over time, whereas Martin's procedure has decreased. The mortality rate dropped from 40.9% to 15.8%; however, a high mortality rate persists in those cases with small bowel involvement (35.5%).
A marked decrease in the overall mortality rate was observed during the study period. However, further efforts are still required especially in cases involving aganglionosis extending orally to 75 cm from Treitz's band.
我们在日本开展了一项全国性调查，以确定全结肠无神经节症（伴或不伴小肠受累，即TCSA）患者诊断和治疗的近期趋势。这些数据与过去30年内开展的两项类似研究进行了比较。
分三个阶段收集患者数据；第一阶段纳入了1978年至1982年间的135例患者；第二阶段，1988年至1992年间的107例患者；第三阶段，1998年至2002年间的101例患者。
TCSA的发病率分别为1:59,059、1:58,084和1:58,375，各阶段的男女比例分别为1.5:1、1.5:1和2.2:1。合并异常的患者从15.2%（第一阶段）增至22.8%（第三阶段）。术前小肠结肠炎的发病率随时间下降。在治疗方面，杜哈梅尔手术和升结肠补片法随时间增加，而马丁手术则减少。死亡率从40.9%降至15.8%；然而，小肠受累的病例死亡率仍然很高（35.5%）。
在研究期间观察到总体死亡率显著下降。然而，仍需进一步努力，特别是在无神经节症累及从屈氏韧带向口侧延伸75 cm的病例中。
