Neural intestinal dysplasia

Classification according to ICD-10
K59.2	Neurogenic bowel disorder, not elsewhere classified
ICD-10 online (WHO version 2019)

The Neural intestinal dysplasia (NID) is a very rare congenital disorder of the nerve supply of the intestine with the clinical picture of chronic intestinal pseudo-obstruction (CIPO) . The submucosal plexus and the myenteric plexus are disturbed .

The first description comes from 1976 by the British doctors MS Tanner, B. Smith and JK Lloyd.

As early as 1971, the Swiss pathologist WA Meier-Ruge presented a case description at the 55th annual conference of the German Society for Pathology.

distribution

The frequency of IND B is given as between 25% and 65% of patients with Hirschsprung's disease, 6% of children with Hirschsprung's disease and 2.9% of children biopsied because of chronic constipation.

There is no gender preference.

The combination of NID with Hirschsprung's disease is also known as HAIND or HANID.

There is an association with neurofibromatosis type 1 and MEN2b as well as with hypoganglionosis of the myenteric plexus or aganglionosis of the rectum .

Classification

The following forms can be distinguished:

• NID type A , hypoplasia or aplasia of the sympathetic nerve supply to the intestine
• NID type B , parasympathetic nervous system disorder

The (NID) can be viewed as a special form of Hirschsprung's disease , including forms with a normal number, but isolated ganglion cell disorder , defective synapses or postganglionic disorders. According to F. Sitzmann the following classification results:

• Type A 11%
• Type B 64%
• AB mixed type
• NID aganglionosis mixed type

Clinical manifestations

Clinical criteria are:

• Often manifested in the infant with adynamia of the large intestine , chronic constipation , ileus and megacolon
• sometimes only occurring in adulthood, depending on the extent of the changes
• often combined with aganglionosis
• in type A early ulcerative colitis with diarrhea , blood in the stool, intestinal cramps

diagnosis

Diagnosis requires sampling and enzyme histochemical examination.

therapy

Conservative treatment or chronic constipation is usually unsuccessful, so surgical measures become necessary.

literature

• V. Barone, D. Weber, Y. Luo, V. Brancolini, M. Devoto, G. Romeo: Exclusion of linkage between RET and neuronal intestinal dysplasia type B. In: American journal of medical genetics. Volume 62, Number 2, March 1996, pp. 195-198, doi : 10.1002 / (SICI) 1096-8628 (19960315) 62: 2 <195 :: AID-AJMG15> 3.0.CO; 2-J , PMID 8882403 .
• W. Meier-Ruge, F. Gambazzi, RE kaufeler, P. Schmid, CP Schmidt: The neuropathological diagnosis of neuronal intestinal dysplasia (NID B). In: European journal of pediatric surgery: official journal of the Austrian Association of Pediatric Surgery .. [et al] = Journal for Pediatric Surgery. Volume 4, Number 5, October 1994, pp. 267-273, doi: 10.1055 / s-2008-1066116 , PMID 7857882 .

Individual evidence

1. ^ DE Schofield, EJ Yunis: Intestinal neuronal dysplasia. In: Journal of Pediatric Gastroenterology and Nutrition . Volume 12, Number 2, February 1991, pp. 182-189, doi: 10.1097 / 00005176-199102000-00008 , PMID 2051270 .
2. ↑ Pseudo-obstruction, chronic intestinal. In: Orphanet (Rare Disease Database).
3. ↑ MS Tanner, B. Smith, JK Lloyd: Functional intestinal obstruction due to deficiency of argyrophil neurones in the myenteric plexus. Familial syndrome presenting with short small bowel, malrotation, and pyloric hypertrophy. In: Archives of Disease in Childhood. Volume 51, number 11, November 1976, pp. 837-841, doi: 10.1136 / adc.51.11.837 , PMID 1008589 , PMC 1546064 (free full text).
4. ^ W. Meier-Ruge: About a disease picture of the colon with Hirschsprung symptoms. In: Negotiations of the German Society for Pathology. Vol. 55, 1971, pp. 506-510, PMID 4130757 .
5. ↑ ^{a b} P. P. Schmittenbecher, P. Sacher, D. Cholewa, A. Haberlik, G. Menardi, J. Moczulski, E. Rumlova, W. Schuppert, B. Ure: Hirschsprung's disease and intestinal neuronal dysplasia - an association frequent with implications for the postoperative course. In: Pediatric surgery international. Volume 15, Number 8, 1999, pp. 553-558, doi: 10.1007 / s003830050669 , PMID 10631732 .
6. ↑ E. Bruder, WA Meier-Ruge: Intestinal neuronal dysplasia type B - how do we understand it today? In: The Pathologist. Volume 28, Number 2, March 2007, pp. 137-142, doi: 10.1007 / s00292-007-0894-x , PMID 17279410 .
7. ^ WA Meier-Ruge, K. Ammann, E. Bruder, AM Holschneider, AF Schärli, PP Schmittenbecher, F. Stoss: Updated results on intestinal neuronal dysplasia (IND B). In: European journal of pediatric surgery: official journal of the Austrian Association of Pediatric Surgery .. [et al] = Journal for Pediatric Surgery. Volume 14, Number 6, December 2004, pp. 384-391, doi: 10.1055 / s-2004-821120 , PMID 15630639 (review).
8. ↑ B. Hanimann, D. Inderbitzin, J. Briner, P. Sacher: Clinical Relevance of Hirschsprung-Associated Neuronal Intestinal Dysplasia (HANID). In: European Journal of Pediatric Surgery. 2, 1992, p. 147, doi: 10.1055 / s-2008-1063425 .
9. ^ Pathology Outlines
10. ↑ B. Fadda, WA Maier, W. Meier-Ruge, A. Schärli, R. Daum: [Neuronal intestinal dysplasia. Critical 10-years' analysis of clinical and biopsy diagnosis]. In: Journal of Children's Surgery: Organ of the German, Swiss and Austrian Society for Children's Surgery = Surgery in infancy and childhood. Volume 38, Number 5, October 1983, pp. 305-311, doi: 10.1055 / s-2008-1059994 , PMID 6649903 .
11. ↑ ^{a b} Visceral Neuropathy, Familial, Autosomal Recessive.  In: Online Mendelian Inheritance in Man . (English)
12. ^ Neuronal Intestinal Dysplasia, Type B.  In: Online Mendelian Inheritance in Man . (English)
13. ↑ FC Sitzmann: Dual series pediatrics . 2nd Edition. Thieme, Stuttgart 2002, ISBN 3-13-125332-0 .
14. ↑ W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. 2nd edition, vol. II, p. 594, Springer 1996, ISBN 3-540-60224-0 .
15. ↑ ^{a b} F. Stoss, K. Ammann, H. Blaßnig et al .: Intestinal neuronal dysplasia - a disease of surgical relevance. In: Coloproctology (1999) Vol. 21, No. 6, pp. 263-268. Print ISSN 0174-2442, online ISSN 1615-6730
16. ↑ K. Beuster: From the Clinic for Pediatric Surgery at the University of Lübeck, Director: L. Wünsch Intestinal dysganglionoses - intestinal neuronal dysplasia type B (IND B) and hypoganglionosis with / without associated Hirschsprung's disease - and the possibility of subsequent maturation in childhood. Inaugural dissertation, Lübeck 2010 2.1.2.2

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