Vol.:(0123456789) 1 3 Pediatric Surgery International https://doi.org/10.1007/s00383-019-04462-9 ORIGINAL ARTICLE Nationwide survey of outcome in patients with extensive aganglionosis in Japan Satoshi Obata 1  · Satoshi Ieiri 2  · Takashi Akiyama 3  · Naoto Urushihara 4  · Hisayoshi Kawahara 5  · Masayuki Kubota 6  · Miyuki Kono 7  · Yuji Nirasawa 8  · Shohei Honda 9  · Masaki Nio 10  · Tomoaki Taguchi 1 Accepted: 8 February 2019 © Springer-Verlag GmbH Germany, part of Springer Nature 2019 Abstract Purpose Hirschsprung’s disease-related short bowel syndrome (HDSBS) is characterized by aganglionosis that extends orally to 75 cm from Treitz’s band. The condition is reported be associated with a high mortality rate of 50–80%. This retrospective study aimed to survey the current trends in HDSBS treatment in Japan. Methods Patient data were extracted from the results of a nationwide survey we conducted, resulting in the retrospective collection of the data of 1087 HD patients from 2008 to 2012 in Japan. Results A total of 11 (0.9%) cases of HDSBS were noted. All patients underwent jejunostomy as neonates. Radical proce- dures performed in fve patients (A-colon patch method in four, Duhamel’s procedure in one). Ziegler’s myotomy-myectomy and serial transverse enteroplasty (STEP) were performed in each patient as palliative procedures. No radical operations were performed in 4 of the 11 cases. The mortality rate was 36.4%. Four patients died, 1 patient who underwent STEP and 3 patients who received no radical procedures. The causes of death were sepsis due to enterocolitis or central intravenous cath- eter infection, and hepatic failure. All patients who underwent radical procedures survived and showed satisfactory outcomes. Conclusion HDSBS still showed a high mortality rate, although surgical approaches such as the A-colon patch method resulted in satisfactory outcomes. Keywords Hirschsprung’s disease · Nationwide survey · Short bowel syndrome · Mortality The Japanese Study Group for Hirshsprung’s disease: Satoshi Obata, Satoshi Ieiri, Takashi Akiyama, Naoto Urushihara, Hisayoshi Kawahara, Masayuki Kubota, Miyuki Kono, Yuji Nirasawa, Shohei Honda, Masaki Nio, Tomoaki Taguchi. * Tomoaki Taguchi taguchi@pedsurg.med.kyushu 1 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 2 Department of Pediatric Surgery, Field of Developmental Medicine, Graduate School of Medical and Dental Sciences, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima 890- 8520, Japan 3 Department of Pediatric Surgery, Hiroshima City Hiroshima Citizen Hospital, 7-33, Moto-machi, Naka-ku, Hiroshima 730- 8518, Japan 4 Department of Pediatric Surgery, Shizuoka Children’s Hospital, 860, Urushiyama, Aoi-ku, Shizuoka 420-0953, Japan 5 Department of Pediatric Surgery, Hamamatsu University Hospital, 1-20-1, Handayama, Higashi-ku, Hamamatsu, Shizuoka 431-3192, Japan 6 Department of Pediatric Surgery, Niigata University Graduate School of Medicine and Dental Sciences, 1-757, Asahimachi- Dori, Chuo-ku, Niigata 951-8510, Japan 7 Department of Pediatric Surgery, Kanazawa Medical University, 1-1, Daigaku, Uchinada-machi, Kahoku-gun, Ishikawa 920-0293, Japan 8 Department of Pediatric Surgery, Kyorin University School of Medicine, 6-20-2, Shinkawa, Mitaka, Tokyo 181-0004, Japan 9 Department of Gastroenterological Surgery I, Hokkaido University Graduate School of Medicine, 5, Kita-14jo-nishi, Kita-ku, Sapporo, Hokkaido 060-8648, Japan 10 Department of Pediatric Surgery, Tohoku University School of Medicine, 1-1, Seiryo-machi, Aoba-ku, Sendai, Miyagi 980- 0872, Japan