Taguchi Tomoaki, Obata Satoshi, Ieiri Satoshi
The Japanese Study Group for Hirschsprung's Disease, 3-1-1, Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
Department of Pediatric Surgery, Reproductive and Developmental Medicine, Faculty of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, Fukuoka, 812-8582, Japan.
Pediatr Surg Int. 2017 Apr;33(4):497-504. doi: 10.1007/s00383-016-4054-3. Epub 2017 Jan 5.
The diagnosis and surgical treatments of Hirschsprung's disease (HD) have undergone various changes in the last few decades because of establishment of laparoscopic procedures. A retrospective nationwide survey for 4 decades was performed to study the changing profile of HD in Japan.
The patient data were collected in 4 phases: Group 1, between 1978 and 1982; Group 2, between 1988 and 1992; Group 3, between 1998 and 2002; and Group 4, between 2008 and 2012.
The incidence and the male/female ratio remained almost the same over time (1/4, 895 in newborns and 2.9:1 in Group 4). The patients with a family history increased to 7.1% in Group 4, in comparison to 2.8-6.0% in other groups. Regarding the extent of aganglionosis, sigmoid colon increased to 63.1% in Group 4, compared to 51.9% in Group 3. Manometry was performed less frequently in Group 4 (45.8%) than in Group 3 (66.1%). Transanal endorectal pull-through (TAEPT) was the most popular operation in Group 4 (49.6%). In addition, laparoscopy-assisted operations increased to 46.9% in Group 4, in comparison to 29.7% in Group 3. The incidence of preoperative enterocolitis and the mortality rate in Group 4 were 17.2% and 2.4%, respectively, and were markedly decreased in comparison to Group 1 (29.2% and 6.5%, respectively). The mortality rate decreased over time to 2.4% in Group 4. Over the last decade, there has been remarkable improvement in the mortality rate associated with the small intestine (aganglionosis extending orally to more than 30 cm of the terminal ileum). The rates were 25.5% in Group 4, 53.6% in Group 1, 33.3% in Group 2, and 35.5% in Group 3. In addition, the mortality rates of the remaining aganglionosis subgroups also improved.
Primary operations without laparotomy, including TAEPT and laparoscopy-assisted operations, have become the first choice for the definitive surgical treatment of HD in Japan. The mortality rate has decreased over time. However, the mortality rate of small intestinal aganglionosis is still relatively high. The development of new treatment strategy for small intestinal aganglionosis is called for.
由于腹腔镜手术的建立，过去几十年来先天性巨结肠症（HD）的诊断和外科治疗发生了各种变化。进行了一项为期40年的全国性回顾性调查，以研究日本HD的变化情况。
患者数据分4个阶段收集：第1组，1978年至1982年；第2组，1988年至1992年；第3组，1998年至2002年；第4组，2008年至2012年。
发病率和男女比例随时间几乎保持不变（新生儿中为1/4895，第4组中为2.9:1）。有家族史的患者在第4组中增至7.1%，而其他组为2.8%-6.0%。关于无神经节细胞症的范围，乙状结肠在第4组中增至63.1%，而第3组中为51.9%。第4组中测压的实施频率（45.8%）低于第3组（66.1%）。经肛门直肠拖出术（TAEPT）是第4组中最常用的手术（49.6%）。此外，腹腔镜辅助手术在第4组中增至46.9%，而第3组中为29.7%。第4组中术前小肠结肠炎的发生率和死亡率分别为17.2%和2.4%，与第1组相比（分别为29.2%和6.5%）明显降低。死亡率随时间下降至第4组中的2.4%。在过去十年中，与小肠相关的死亡率（无神经节细胞症向口腔延伸至回肠末端超过30 cm）有显著改善。第4组中的发生率为25.5%，第1组中为53.6%，第2组中为33.3%，第3组中为35.5%。此外，其余无神经节细胞症亚组的死亡率也有所改善。
包括TAEPT和腹腔镜辅助手术在内的非剖腹初次手术已成为日本HD确定性外科治疗的首选。死亡率随时间下降。然而，小肠无神经节细胞症的死亡率仍然相对较高。需要开发针对小肠无神经节细胞症的新治疗策略。
