Abstract
Findings in 23 children with neuronal intestinal dysplasia (NID) are presented. Twelve children had Hirschsprung's disease that masked the existence of proximal NID. In all cases the diagnosis was established by histological-histochemical examination. Amelioration of symptoms by conservative treatment alone was achieved in 4 children; in 13 cases colostomy was necessary. In 6 patients colon resection was performed: 2 suffered from both colitis and obstruction; the other 4 were treated for obstruction, multiple perforations, persistant adynamia of the left colon, and fistulation. In 9 patients the colostomy was closed. Four of 6 children who did not have sonographic confirmation of motility had complications due to colon dysmotility after closure of their normally functioning colostomy. Therapeutic measures are based exclusively on clinical and functional parameters. The clinical picture dictates the emergency measures (colostomy, colon resection, and colectomy). Normal colostomy function is not a sufficient criterion of normal colon motility. Functional sonography of the colon is included in the planning of further therapy. Indications, timing, and extent of colon resection can be reliably determined using the sonographic method.
Similar content being viewed by others
References
Aldridge RT, Campbell PE (1968) Ganglion cell distribution in the normal rectum and anal canal. J Pediatr Surg 3: 475–490
Alison F, Bocquet L, Schmelck P-H (1974) Hyperplasie des plexus myenteriques. Arch Franc Pediatr 31: 315–323
Bughaighis AG, Emery JL (1971) Functional obstruction of the intestine due to neurological immaturity. Prog in Pediatr Surg 3: 37–52
Fadda B, Pistor G, Meier-Ruge W, Hofmann-von Kapherr S, Müntefering H, Espinoza R (1987) Symptoms, diagnosis, and therapy of neuronal intestinal dysplasia masked by Hirschsprung's disease. Report of 24 cases. Pediatr Surg Int 2: 76–80
Fadda B, Maier WA, Meier-Ruge W, Schärli A, Daum R (1983) Neuronale intestinal Dysplasie. Z Kinderchir 38: 305–311
Garret JR, Howard ER (1980) Myenteric plexus of the hind-gut: developmental abnormalities in humans and experimental studies. Ciba Foundation Symposium (Elsevier Amsterdam), pp 326–354
Gulotta F, Straaten G (1977) Hirschsprung'sche Krankheit mit gleichzeitiger Aganglionose bei sog. neuronaler Colondysplasie (Dysganglionosis colica). Z Kinderchir 20: 42–49
Hofmann-v. Kap-herr S (1983) Neuronale Colondysplasie und M. Hirschsprung. Report held at the 16th Annual Meeting, BAPS-Congress, Fukuoka, Japan
Karnovsky MJ, Roots L (1964) A “direct-coloring” thiocholine method for cholinesterase. J Histochem Cytochem 12: 219–221
Klos I, Maier WA, Morger R, Schweizer P (1978) Die neuronale Colondysplasie. Z Kinderchir 23: 53–54
Lassmann G, Wurnig P (1973) Lokale Ganglienzellhyperplasie in der Submucosa am oralen Ende des aganglionären Segmentes bei M. Hirschsprung. Z Kinderchir 12: 236–243
Le Quesne G, Reilly BJ (1975) Functional immaturity of large bowel in the newborn infant. Radiol Clin North Am 13: 331–341
Meier-Ruge W (1971) Über ein Erkrankungsbild des Colons mit Hirschsprung-Symptomatik. Verh Dtsch Ges Pathol 55: 506–510
Meier-Ruge W (1985) Angeborene Dysganglionosen des Colon. Kinderarzt 2: 151–164
Munakata K, Morita K, Okabe J, Sueoka H (1985) Clinical and histologic studies of neuronal intestinal dysplasia. J Pediatr Surg 20: 231–235
Nachlas MM, Tsou KC, De Souza E (1957) Cytochemical demonstration of succinic dehydrogenase by the use of a neuw p-nitrophenyl substituted ditetrazole. J Histochem Cytochem 5: 420–436
Okamoto E, Ueda T (1967) Embryogenesis of intramural ganglia of the gut and its relation to Hirschsprung's disease. J Pediatr Surg 2: 437–443
Pistor G (1987) Functional colon sonography. Prog Pediatr Surg (in press)
Pistor G, Hofmann-v. Kap-herr S (1984) Funktionelle Colonsonographie bei neuronaler intestinaler Dysplasie —Bericht über 3 Fälle. Fortschr Med 102: 397–400
Puri P, Lake BD, Nixon HH, Mishalany H, Claireaux AE (1977) Neuronal colonic dysplasia: an unusual association of Hirschsprung's disease. J Pediatr Surg 12: 681–685
Sacher P, Briner U, Stauffer UG (1982) Zur klinischen Bedeutung der neuronalen intestinalen Dysplasie. Z Kinderchir 35: 96–97
Saul RA, Sturner RA, Burger PC (1982) Hyperplasia of the myenteric plexus. Am J Dis Child 136: 852–854
Schärli AF, Meier-Ruge W (1981) Localised and disseminated forms of neuronal intestinal dysplasia mimicking Hirschsprung's disease. J Pediatr Surg 16: 164–170
Author information
Authors and Affiliations
Additional information
Offprint requests to: G. Pistor
Rights and permissions
About this article
Cite this article
Pistor, G., Hofmann-von Kap-herr, S., Grüssner, R. et al. Neuronal intestinal dysplasia. Pediatr Surg Int 2, 352–358 (1987). https://doi.org/10.1007/BF00175648
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00175648