title: Current clinical features in diagnosis and treatment for immaturity of ganglia in Japan: analysis from 10-year nationwide survey.
abstract: BACKGROUND
  To identify the current clinical features in diagnosis and treatment for immaturity of ganglia (IG) in Japan, we retrospectively analyzed data for patients with IG from the nationwide surveys in Japan. This survey was performed by Japanese Study Group of allied disorders of Hirschsprung's disease (ADHD).
  
  
  METHODS
  In primary research, data on totally 355 cases of ADHD were collected for 10 years (2001-2010). Fifteen patients were IG. All IG patients were confirmed by pathological examination. In secondary research, detail questionnaires were sent and collected.
  
  
  RESULTS
  Male/female ratio was 9/6 and mean birth weight was 2474 g. All cases (100 %) were onset in neonatal period. Primary symptoms were abdominal distention (86.7 %), vomiting (53.3 %), and late egestion of meconium (26.7 %). An abnormal distention of intestine was recognized in 86.7 % on X-ray, and microcolon was recognized in 58.3 % on contrast enema. Caliber change was recognized in 58.3 % on laparotomy. An enterostomy was made in 13 patients (86.7 %), and an ileostomy was made in 69.2 %. Pathological diagnosis was performed in 100 %. Enterostomy was closed in 100 %.
  
  
  CONCLUSIONS
  Totally, 15 definitive cases of IG in 10 years were collected and analyzed. All cases were onset in the neonatal period and almost all underwent enterostomy, but no mortalities occurred.
authors: Department of Pediatric Surgery, Reproductive and Development Medicine, Faculty of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka, 812-8582, Fukuoka, Japan.|Japanese Study Group for Allied disorders of Hirschsprung's Disease, Fukuoka, Japan.
authors: Satoshi
authors: S
authors: Ieiri
authors: Department of Pediatric Surgery, Reproductive and Development Medicine, Faculty of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka, 812-8582, Fukuoka, Japan.|Department of Surgical Pathology, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
authors: Kina
authors: K
authors: Miyoshi
authors: Department of Pediatric Surgery, Reproductive and Development Medicine, Faculty of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka, 812-8582, Fukuoka, Japan.
authors: Kouji
authors: K
authors: Nagata
authors: Department of Pediatric Surgery, Reproductive and Development Medicine, Faculty of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka, 812-8582, Fukuoka, Japan.
authors: Junko
authors: J
authors: Miyata
authors: Department of Surgical Pathology, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
authors: Kennichi
authors: K
authors: Kohashi
authors: Department of Surgical Pathology, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
authors: Yoshinao
authors: Y
authors: Oda
authors: Department of Pediatric Surgery, Reproductive and Development Medicine, Faculty of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka, 812-8582, Fukuoka, Japan. taguchi@pedsurg.med.kyushu-u.ac.jp.|Japanese Study Group for Allied disorders of Hirschsprung's Disease, Fukuoka, Japan. taguchi@pedsurg.med.kyushu-u.ac.jp.
authors: Tomoaki
authors: T
authors: Taguchi
mesh_terms: D015331:Cohort Studies; D004766:Enterostomy; D005260:Female; D005724:Ganglia / Q000473:pathology* / Q000601:surgery*; D006306:Health Surveys / Q000706:statistics & numerical data*; D006627:Hirschsprung Disease / Q000453:epidemiology* / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D007564:Japan / Q000453:epidemiology; D008297:Male; D012189:Retrospective Studies
keywords: Allied disorders of Hirschsprung’s disease; Enterostomy; Immaturity of ganglia; Pathological diagnosis