Satoshi Obata1, Satoshi Ieiri2, Takashi Akiyama3, Naoto Urushihara4, Hisayoshi Kawahara5, Masayuki Kubota6, Miyuki Kono7, Yuji Nirasawa8, Shohei Honda9, Masaki Nio10, Tomoaki Taguchi11. 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. 11. 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. taguchi@pedsurg.med.kyushu.
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 procedures performed in five 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 catheter 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.
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 HDpatients 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 procedures performed in five 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 catheter 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.
Entities:
Keywords:
Hirschsprung’s disease; Mortality; Nationwide survey; Short bowel syndrome
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