Isolated intestinal neuronal dysplasia Type B (IND-B) in Japan: Results from a nationwide survey

T. Taguchi, H. Kobayashi, Y. Kanamori, O. Segawa, A. Yamataka, M. Sugiyama, T. Iwanaka, N. Shimojima, Tatsuo Kuroda, A. Nakazawa, Y. Oda, K. Miyoshi, S. Ieiri

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

Purpose: 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.

Original languageEnglish
Pages (from-to)815-822
Number of pages8
JournalPediatric Surgery International
Volume30
Issue number8
DOIs
Publication statusPublished - 2014

Fingerprint

Japan
Ganglia
Myenteric Plexus
Hirschsprung Disease
Hyperplasia
Gastroenterology
Rare Diseases
Neuronal intestinal pseudoobstruction
Surveys and Questionnaires
Mucous Membrane
Cohort Studies
Retrospective Studies
Pediatrics

Keywords

  • Allied disorders of Hirschsprung's disease
  • Ganglioneuromatosis
  • Giant ganglia
  • Intestinal neuronal dysplasia
  • Variant Hirschsprung's disease

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery

Cite this

Taguchi, T., Kobayashi, H., Kanamori, Y., Segawa, O., Yamataka, A., Sugiyama, M., ... Ieiri, S. (2014). Isolated intestinal neuronal dysplasia Type B (IND-B) in Japan: Results from a nationwide survey. Pediatric Surgery International, 30(8), 815-822. https://doi.org/10.1007/s00383-014-3542-6

Isolated intestinal neuronal dysplasia Type B (IND-B) in Japan : Results from a nationwide survey. / Taguchi, T.; Kobayashi, H.; Kanamori, Y.; Segawa, O.; Yamataka, A.; Sugiyama, M.; Iwanaka, T.; Shimojima, N.; Kuroda, Tatsuo; Nakazawa, A.; Oda, Y.; Miyoshi, K.; Ieiri, S.

In: Pediatric Surgery International, Vol. 30, No. 8, 2014, p. 815-822.

Research output: Contribution to journalArticle

Taguchi, T, Kobayashi, H, Kanamori, Y, Segawa, O, Yamataka, A, Sugiyama, M, Iwanaka, T, Shimojima, N, Kuroda, T, Nakazawa, A, Oda, Y, Miyoshi, K & Ieiri, S 2014, 'Isolated intestinal neuronal dysplasia Type B (IND-B) in Japan: Results from a nationwide survey', Pediatric Surgery International, vol. 30, no. 8, pp. 815-822. https://doi.org/10.1007/s00383-014-3542-6
Taguchi, T. ; Kobayashi, H. ; Kanamori, Y. ; Segawa, O. ; Yamataka, A. ; Sugiyama, M. ; Iwanaka, T. ; Shimojima, N. ; Kuroda, Tatsuo ; Nakazawa, A. ; Oda, Y. ; Miyoshi, K. ; Ieiri, S. / Isolated intestinal neuronal dysplasia Type B (IND-B) in Japan : Results from a nationwide survey. In: Pediatric Surgery International. 2014 ; Vol. 30, No. 8. pp. 815-822.
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AU - Taguchi, T.

AU - Kobayashi, H.

AU - Kanamori, Y.

AU - Segawa, O.

AU - Yamataka, A.

AU - Sugiyama, M.

AU - Iwanaka, T.

AU - Shimojima, N.

AU - Kuroda, Tatsuo

AU - Nakazawa, A.

AU - Oda, Y.

AU - Miyoshi, K.

AU - Ieiri, S.

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AB - Purpose: 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.

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