Hirschsprung’s disease has been usually classified by the extent of the aganglionic segment. However, there are no unified classification criteria in the world yet. Generally, it is classified as “short-segment aganglionosis” when the aganglionic segment extends to the sigmoid colon; especially, the aganglionic segment extends only to the rectum under peritoneal reflection, which is termed as “rectal aganglionosis” (Fig. 8.1a), and extends to the sigmoid colon, which is termed as “rectosigmoid aganglionosis” (Fig. 8.1b); “long-segment aganglionosis” when the aganglionic segment extends beyond the sigmoid colon to the splenic flexure or transverse colon or ascending colon (Fig. 8.1c); and “total colon aganglionosis” when the aganglionic segment extends beyond the cecum to the distal ileum (Fig. 8.1d), and “extensive aganglionosis” when the aganglionic segment extends to the jejunum (Fig. 8.1e). Almost 80% of the patients with typical Hirschsprung’s disease have aganglionic segment limited to the rectum and the sigmoid colon [1–4]. Previous studies presented that rectal aganglionosis accounts for 5.7–14.6%, rectosigmoid aganglionosis accounts for 64.9–67.0%, long-segment aganglionosis accounts for 12.0–26.0%, and total colon aganglionosis accounts for 2.8–12.0% (Table 8.1) [1, 5–10]. Almost all studies were institutional experiences in North America or Europe; however, only Singh et al. presented the nationwide survey of Australia from 1997 to 2000 . Table 8.2 shows the nationwide survey of Japan for four series from 1978 [11–13]. Total patients’ number has been gradually decreased followed by the decrease of birth. Recent study presented that the percentage of rectal aganglionosis, rectosigmoid aganglionosis, long-segment aganglionosis, total colon aganglionosis, and extensive aganglionosis presented 11.1%, 63.1%, 14.9%, 7.9%, and 3.1%, respectively .
Short-segment aganglionosis Long-segment aganglionosis Total colon aganglionosis Extensive aganglionosis
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