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

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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.

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Acknowledgments

This study was supported by a grant from The Ministry of Health, Labor Sciences Research Grants for Research on intractable disease. The authors thank The Japanese Society of Pediatric Surgeons, The Japanese Society of Pediatric Nutrition, Gastroenterology and Nutrition and The Japanese Study Group of Pediatric Constipation. The authors thank Dr. Bryan Quinn for reading the manuscript, and also thank Ms. Masutomi and the Yamazaki Department of Pediatric Surgery, Kyushu University, for their help in processing the data.

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Authors and Affiliations

  1. Department of Pediatric Surgery, Faculty of Medical Sciences, Kyushu University, Fukuoka, Japan

    T. Taguchi, K. Miyoshi & S. Ieiri

  2. Department of Pediatric Surgery, Juntendo University School of Medicine, Tokyo, Japan

    H. Kobayashi & A. Yamataka

  3. Division of Surgery, Department of Surgical Subspecialties, National Center for Child Health and Development, Tokyo, Japan

    Y. Kanamori

  4. Division of Pediatric Surgery, Department of Surgery II, Tokyo Women’s Medical University, Tokyo, Japan

    O. Segawa

  5. Department of Pathology, National Center for Child Health and Development, Tokyo, Japan

    A. Nakazawa

  6. Department of Anatomic Pathology, Kyushu University, Fukuoka, Japan

    Y. Oda

  7. Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan

    N. Shimojima & T. Kuroda

  8. Department of Pediatric Surgery, Tokyo University, Tokyo, Japan

    M. Sugiyama & T. Iwanaka

Authors
  1. T. Taguchi
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  2. H. Kobayashi
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  3. Y. Kanamori
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  4. O. Segawa
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  5. A. Yamataka
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  6. M. Sugiyama
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  7. T. Iwanaka
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  8. N. Shimojima
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  9. T. Kuroda
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  10. A. Nakazawa
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  11. Y. Oda
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  12. K. Miyoshi
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  13. S. Ieiri
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Corresponding author

Correspondence to T. Taguchi.

Additional information

All authors belongs to the Japanese Study Group of Allied disorders of Hirschsprung’s Disease.

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Cite this article

Taguchi, T., Kobayashi, H., Kanamori, Y. et al. Isolated intestinal neuronal dysplasia Type B (IND-B) in Japan: results from a nationwide survey. Pediatr Surg Int 30, 815–822 (2014). https://doi.org/10.1007/s00383-014-3542-6

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  • DOI: https://doi.org/10.1007/s00383-014-3542-6

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