title: Isolated intestinal neuronal dysplasia Type B (IND-B) in Japan: results from a nationwide survey.
abstract: OBJECTIVE
  Intestinal neuronal dysplasia Type B (IND-B) has been proposed to be an allied disorder of Hirschsprung's disease (ADHD). The original histological criteria included hyperganglionosis, giant ganglia, ectopic ganglion cells and an increased AChE activity in the lamina propria. The criteria for IND-B have been gradually revised. The present diagnostic criteria are [1] more than 20 % of the submucosal ganglia contain nine or more ganglion cells and [2] the patient is older than 1 year. To clarify the current status of IND-B in Japan, a nationwide retrospective cohort study was performed.
  
  
  METHODS
  Questionnaires were sent to 161 major institutes of pediatric surgery and gastroenterology in Japan.
  
  
  RESULTS
  A total of 355 cases of ADHD were collected, including 18 cases of IND-B (5 %). Based on original criteria, 13 out of 18 cases were diagnosed as IND-B. However, only four cases met the current criteria. Three of the four patients (75 %) required pull-through operation. All of the patients exhibited giant ganglia and ganglioneuromatosis-like hyperplasia of the myenteric plexus.
  
  
  CONCLUSIONS
  IND-B cases matching the current criteria are thought to be quite rare and they are associated with marked hyperplasia of the myenteric plexus. "True" IND-B is a rare and intractable disease.
authors: Department of Pediatric Surgery, Faculty of Medical Sciences, Kyushu University, Fukuoka, Japan, taguchi@pedsurg.med.kyushu-u.ac.jp.
authors: T
authors: T
authors: Taguchi
authors: H
authors: H
authors: Kobayashi
authors: Y
authors: Y
authors: Kanamori
authors: O
authors: O
authors: Segawa
authors: A
authors: A
authors: Yamataka
authors: M
authors: M
authors: Sugiyama
authors: T
authors: T
authors: Iwanaka
authors: N
authors: N
authors: Shimojima
authors: T
authors: T
authors: Kuroda
authors: A
authors: A
authors: Nakazawa
authors: Y
authors: Y
authors: Oda
authors: K
authors: K
authors: Miyoshi
authors: S
authors: S
authors: Ieiri
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D017615:Enteric Nervous System / Q000473:pathology*; D005260:Female; D006627:Hirschsprung Disease / Q000453:epidemiology / Q000473:pathology*; D006801:Humans; D015994:Incidence; D007413:Intestinal Mucosa / Q000294:innervation* / Q000473:pathology; D007564:Japan / Q000453:epidemiology; D008297:Male; D012189:Retrospective Studies; D013368:Submucous Plexus / Q000473:pathology*; D011795:Surveys and Questionnaires