title: Early jejunostomy creation in cases of isolated hypoganglionosis: verification of our own experience based on a national survey.
abstract: OBJECTIVE
  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.affiliation: 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.
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Watanabe
authors.affiliation: Department of Pediatric Surgery, Aichi Children's Health and Medical Center, 7-426 Morioka-cho, Obu, Aichi, 474-8710, Japan.
authors.forename: Wataru
authors.initials: W
authors.lastname: Sumida
authors.affiliation: Department of Pediatric Surgery, Aichi Children's Health and Medical Center, 7-426 Morioka-cho, Obu, Aichi, 474-8710, Japan.
authors.forename: Hidemi
authors.initials: H
authors.lastname: Takasu
authors.affiliation: Department of Pediatric Surgery, Aichi Children's Health and Medical Center, 7-426 Morioka-cho, Obu, Aichi, 474-8710, Japan.
authors.forename: Kazuo
authors.initials: K
authors.lastname: Oshima
authors.affiliation: Division of Surgery, Department of Surgical Subspecialties, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Kanamori
authors.affiliation: Department of Gastrointestinal and Pediatric Surgery Graduate School of Medical Sciences, Mie University, 2-174 Edobashi, Tsu, Mie, 5148507, Japan.
authors.forename: Keiichi
authors.initials: K
authors.lastname: Uchida
authors.affiliation: Department of Pediatric Surgery Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, Japan.
authors.forename: Tomoaki
authors.initials: T
authors.lastname: Taguchi
mesh_terms: D000367:Age Factors; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006627:Hirschsprung Disease / Q000401:mortality / Q000601:surgery*; D006801:Humans; D007081:Ileostomy; D007223:Infant; D007231:Infant, Newborn; D007422:Intestines / Q000294:innervation*; D007564:Japan / Q000453:epidemiology; D007582:Jejunostomy; D053208:Kaplan-Meier Estimate; D008297:Male; D059351:Organ Sparing Treatments; D010149:Postoperative Pain / Q000000981:diagnostic imaging; D011859:Radiography; D015996:Survival Rate; D013997:Time Factors
keywords: Isolated hypoganglionosis; Japan; Survey; Survival rate; Upper jejunostomy