title: Isolated hypoganglionosis: results of a nationwide survey in Japan.
abstract: OBJECTIVE
  This study analyzes how isolated hypoganglionosis (IH) is diagnosed and treated in Japanese pediatric surgical departments.
  
  
  METHODS
  A second questionnaire was sent to 90 pediatric surgical departments that took part in an initial survey on variant Hirschsprung's disease.
  
  
  RESULTS
  Of 109 cases registered as having a certain diagnosis of IH, we targeted 90 patients and excluded the remaining 19. Symptom onset was neonatal ileus with a male:female ratio of 34:56. In most cases, results from radio-contrast enemas and suction rectal biopsies were normal. Anorectal manometry demonstrated that there was no relaxation in 73 % of the 37 patients examined. Furthermore, out of 55 patients who were examined intraoperatively, IH was diagnosed or suspected in 29 cases. Another 12 cases were eventually diagnosed with IH by other institutions using paraffin-embedded specimens. Stomas were initially created in 89 out of 90 cases and the mortality rate of the group that initially received jejunostomies was significantly lower than that of the ileostomy group (p < 0.05).
  
  
  CONCLUSIONS
  Preoperative diagnosis of IH is often difficult and intraoperative biopsies were only able to provide a correct diagnosis in 52 % of cases. More accurate diagnosis and the initial creation of jejunostomies rather than ileostomies may assist in improving patient management and outcomes.
authors: Department of Pediatric Surgery, Aichi Children's Health and Medical Center, 1-2 Osakada, Morioka-cho, Obu-city, Aichi, 474-8710, Japan, yoshio_watanabe@mx.achmc.pref.aichi.jp.
authors: Yoshio
authors: Y
authors: Watanabe
authors: Yutaka
authors: Y
authors: Kanamori
authors: Keiichi
authors: K
authors: Uchida
authors: Tomoaki
authors: T
authors: Taguchi
mesh_terms: D013505:Digestive System Surgical Procedures / Q000379:methods*; D016145:Endoscopy, Digestive System / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D006627:Hirschsprung Disease / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D007422:Intestines / Q000473:pathology*; D007564:Japan / Q000453:epidemiology; D008297:Male; D011159:Population Surveillance; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends
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