Top

Published in:

01-08-2014 | Original Article

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

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

Published in: Pediatric Surgery International | Issue 8/2014

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.

Hirschsprung’s Disease and Allied Disorders (2008) 3rd ed. Holschneider A and Puri P (ed). Springer

Ravitch MM (1958) Pseudo Hirschsprung’s disease. Ann Surg 147:781–795PubMedCentralPubMed

Bentley JFR, Nixon HH, Ehrenpreis TH, Spencer B (1966) Seminar on pseudo-Hirschsprung’s disease and related disorders. Arch Dis Child 41:143–154CrossRef

Puri P (1997) Variant Hirschsprung’s disease. J Pediatr Surg 32:149–157PubMedCrossRef

Okamoto E and Toyosaka A (1996) Pseudo-Hirschsprung’s disease. Research on the pathophysiology, diagnosis and treatment (in Japanese) Nagai-Shoten

Maldonaldo JE, Greegg JA, Green PA, Brown AL (1970) Chronic idiopathic intestinal pseudo-obstruction. Am J Med 49:203–212CrossRef

Meier-Ruge W (1971) Casuistic of colon disorder with symptoms of Hirschsprung’s disease. Verh Dtsch Ges Pathol 55:506–510PubMed

Fadda B, Maier WA, Meier-Ruge W et al (1983) Neuronal intestinal dysplasia. Critical 10-years’ analysis of clinical and biopsy diagnosis. Z Kinderchir 38:305–311PubMed

Puri P, Rolle U (2004) Variant Hirschsprung’s disease. Semin Pediatr Surg 13:293–299PubMedCrossRef

10.

Puri P, Lake BD, Nixon HH et al (1977) Neuronal colonic dysplasia: an unusual association of Hirschsprung’s disease. J Pediatr Surg 12:681–685PubMedCrossRef

11.

Meier-Ruge WA, Ammann K, Bruder E et al (2004) Updated results on intestinal neuronal dysplasia (IND B). Eur J Pediatr Surg 14:384–391PubMedCrossRef

12.

Friedmacher F, Puri P (2013) Classification and diagnostic criteria of variants of Hirschsprung’s disease. Pediatr Surg Int 29:855–872PubMedCrossRef

13.

Kanamori Y, Hasizume K, Sugiyama M et al (2005) Type B intestinal neuronal dysplasia. Pediatr Int 47:338–340PubMedCrossRef

14.

Goldblum JR, Folpe AL, Weiss SW. Benign tumors of peripheral nerves. In: Enzinger and Weiss’s Soft Tissue Tumors, 6th ed. Philadelphia, PA, Elsevier Saunders; 2014:786–789

15.

Lake BD (1995) Intestinal neuronal dysplasia. Why does it only occur in parts of Europe? Virchows Arch 426:537–539PubMedCrossRef

16.

Csury L, Pena A (1995) Intestinal neuronal dysplasia. Myth or reality? Literature review. Pediatr Surg Int 10:441–446CrossRef

17.

Martucciello G, Pini Prato A, Puri P, Holschneider AM, Meier-Ruge W et al (2005) Controversies concerning diagnostic guidelines for anomalies of the enteric nervous system: a report from the fourth International Symposium on Hirschsprung’s disease and related neurocristopathies. J Pediatr Surg 40:1527–1531PubMedCrossRef

18.

Coerdt W, Mivhel JS, Rippin G et al (2004) Quantitative morphometric analysis of the submucous plexus in aged-related control groups. Virchows Arch 444:239–246PubMedCrossRef

19.

Meier-Ruge WA, Bronnimann PB, Gambazzi F et al (1995) Histopathological criteria for intestinal neuronal dysplasia of the submucosal plexus (type B). Virchows Arch 426:549–556PubMedCrossRef

20.

Kobayashi H, Hirakawa H, Puri P (1995) What are the diagnostic criteria for intestinal neuronal dysplasia. Pediatr Surg Int 10:459–464CrossRef

21.

Meier-Ruge W, Gambazzi F, Kaufeler RE et al (1994) The neuropathological diagnosis of neuronal intestinal dysplasia (NID B). Eur J Pediatr Surg 4:267–273PubMedCrossRef

22.

MacMahon RA, Moore CCM, Cussen LJ (1981) Hirschsprung-like syndromes in patients with normal ganglion cells on suction rectal biopsy. J Pediatr Surg 16:835–839PubMedCrossRef

23.

Feinstat T, Tesluk H, Schuffler MD et al (1984) Megacolon and neurofibromatosis: a neuronal intestinal dysplasia. Gastroenterology 86:1573–1579PubMed

24.

Wu JF, Chen HL, Peng SS et al (2003) Neurofibromatosis type 1 and intestinal dysplasia type B in a child: report of one case. Acta Paediatr Taiwan 44:232–234PubMed

25.

Cohen MS, Phay JE, Albinson C et al (2002) Gastrointestinal manifestations of multiple endocrine neoplasia type 2. Ann Surg 235:648–654PubMedCentralPubMedCrossRef

26.

King SK, Southwell BR, Hutson JM (2006) An association of multiple endocrine neoplasia 2B, a RET mutation; constipation; and low substance P –nerve fiber density in colonic circular muscle. J Pediatr Surg 41:437–442PubMedCrossRef

Title: Isolated intestinal neuronal dysplasia Type B (IND-B) in Japan: results from a nationwide survey
Authors: T. Taguchi
H. Kobayashi
Y. Kanamori
O. Segawa
A. Yamataka
M. Sugiyama
T. Iwanaka
N. Shimojima
T. Kuroda
A. Nakazawa
Y. Oda
K. Miyoshi
S. Ieiri
Publication date: 01-08-2014
Publisher: Springer Berlin Heidelberg
Published in: Pediatric Surgery International / Issue 8/2014
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI: https://doi.org/10.1007/s00383-014-3542-6

Keynote webinar | Spotlight on medication adherence

Springer Medicine

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

Abstract

Purpose

Methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusions

Please log in to get access to this content

Other articles of this Issue 8/2014

The influence of anorectal malformations on fertility: a systematic review

Botulinum toxin use in paediatric colorectal surgery

Congenital anomalies of the kidney and urinary tract (CAKUT) associated with Hirschsprung’s disease: a systematic review

Prevalence of Hirschsprung’s disease in premature infants: a systematic review

Spinal ultrasound in patients with anorectal malformations: is this the end of an era?

Rectopexy for paediatric rectal prolapse: good outcomes but not without postoperative problems