Yoshio Watanabe1, Wataru Sumida2, Hidemi Takasu2, Kazuo Oshima2, Yutaka Kanamori3, Keiichi Uchida4, Tomoaki Taguchi5. 1. Department of Pediatric Surgery, Aichi Children's Health and Medical Center, 7-426 Morioka-cho, Obu, Aichi, 474-8710, Japan. yoshio_watanabe@mx.achmc.pref.aichi.jp. 2. Department of Pediatric Surgery, Aichi Children's Health and Medical Center, 7-426 Morioka-cho, Obu, Aichi, 474-8710, Japan. 3. Division of Surgery, Department of Surgical Subspecialties, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan. 4. Department of Gastrointestinal and Pediatric Surgery Graduate School of Medical Sciences, Mie University, 2-174 Edobashi, Tsu, Mie, 5148507, Japan. 5. Department of Pediatric Surgery Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, Japan.
Abstract
PURPOSE: Isolated hypoganglionosis (IH) is a rare disease, with few well-established therapeutic strategies. This study aims to verify our preliminary therapeutic strategies developed to date in a comparison with data obtained from a nationwide survey of congenital-type IH. METHODS: Of the 90 registered IH cases assessed in a survey of Japanese pediatric surgical departments, 40 patients who had initially undergone jejunostomy (JE) and 41 treated with ileostomy (IL) were analyzed. Thirteen patients with JE sites located less than 50 cm from the ligament of Treitz were defined as having undergone upper jejunostomy (UJE). Postsurgical plain abdominal X-ray findings and survival rates, estimated using the Kaplan-Meier method, were used to evaluate improvements following stoma creation. RESULTS: Improvements in bowel obstruction were observed in significantly more UJE patients (9/13) than non-UJE patients [20/63 (22 JE and 41 IL cases); p = 0.01]. Furthermore, the JE patients demonstrated a significantly higher survival rate than the IL patients (p = 0.01). Following the completion of the 10-year follow-up period, three JE patients died after undergoing massive bowel resection. CONCLUSIONS: To manage IH successfully, patients should undergo JE less than 50 cm from the ligament of Treitz during the neonatal period. Properly managing the distal intestines is important for achieving long-term survival.
PURPOSE: Isolated hypoganglionosis (IH) is a rare disease, with few well-established therapeutic strategies. This study aims to verify our preliminary therapeutic strategies developed to date in a comparison with data obtained from a nationwide survey of congenital-type IH. METHODS: Of the 90 registered IH cases assessed in a survey of Japanese pediatric surgical departments, 40 patients who had initially undergone jejunostomy (JE) and 41 treated with ileostomy (IL) were analyzed. Thirteen patients with JE sites located less than 50 cm from the ligament of Treitz were defined as having undergone upper jejunostomy (UJE). Postsurgical plain abdominal X-ray findings and survival rates, estimated using the Kaplan-Meier method, were used to evaluate improvements following stoma creation. RESULTS: Improvements in bowel obstruction were observed in significantly more UJE patients (9/13) than non-UJE patients [20/63 (22 JE and 41 IL cases); p = 0.01]. Furthermore, the JE patients demonstrated a significantly higher survival rate than the IL patients (p = 0.01). Following the completion of the 10-year follow-up period, three JE patients died after undergoing massive bowel resection. CONCLUSIONS: To manage IH successfully, patients should undergo JE less than 50 cm from the ligament of Treitz during the neonatal period. Properly managing the distal intestines is important for achieving long-term survival.
Authors: Hau D Le; Erica M Fallon; Vincent E de Meijer; Alpin D Malkan; Mark Puder; Kathleen M Gura Journal: Semin Pediatr Surg Date: 2010-02 Impact factor: 2.754