Neuronale intestinale DysplasieEine kritische 10-Jahres-Analyse klinischer und bioptischer Diagnostik

Abstract

61 cases of neuronal intestinal dysplasia are compared in a follow-up study. Two clinically and bioptically different types of intestinal dysplasia can be distinguished. One type with involvement of the sympathetic nerves and the clinical signs of intestinal spasticity, ulcerative colitis with haemorrhagic stools. Histotopochemically, this disease pattern shows aplasia or hypoplasia of the sympathetic nerves with enhanced parasympathetic activity (elevated acetylcholinesterase activity in the lamina propria mucosae and orbicular musculature). One type with involvement of the plexus submucosus clinically accompanied by adynamia of the colon with megacolon formation. This type becomes manifest usually around the 6th month of life. Bioptically there are large groups of ganglion cells and Schwann's cells, but there is also acetylcholinesterase activity as in the other type. This 2nd form is seen more frequently. A third form is a combination of both diseases. The incidence rate of neuronal intestinal dysplasia is equal to that of Hirschsprung's disease.

Malformations of Intestine in Premature Infants under the Mask of Necrotizing Enterocolitis

“…В 1983 г. B. Fadda классифицировал интестинальную нейрональную дисплазию на типы А и В [17]. Тип А наблюдается в 5% случаев интестинальной нейрональ-ной дисплазии и характеризуется аплазией и гипо-плазией симпатической мезентериальной иннервации кишечника, и особенно иннервации кровеносных сосу-дов толстой кишки.…”

Intestinal Neuronal Dysplasia Type B: An Updated Review of a Problematic Diagnosis

“…Intestinal neuronal dysplasia type B was conceived as a neuropathologic etiology for dysmotility 1,2,4 and managed by partial colonic resection in some patients. 2 Soon after the initial description, IND B was observed proximal to the aganglionic segment in some patients with HSCR and has been regarded by some physicians as a marker of functionally abnormal bowel that should be resected along with the aganglionic segment and transition zone. 18 Unless otherwise specified, topics covered in this review are considered equally relevant to both IND and HSCR-associated IND B.…”

“…Patients, particularly infants, often have spontaneous resolution of their symptoms with conservative medical management. 2,14,32 A few studies have evaluated retrospectively the clinical outcomes of patients receiving a diagnosis of IND B. Although the diagnostic criteria used in each of these investigations vary, conclusions regarding management are generally relatively uniform.…”

“…This observation and associated histopathologic findings in rectal biopsy specimens became the diagnostic criteria for IND B, 1 of 2 types of intestinal neuronal dysplasia. 2 Little has been written about type A, perhaps because it is claimed to be rare 3 and diagnosis requires glyoxylic acid-induced catecholamine fluorescence studies of frozen sections. In contrast, a 2017 PubMed search identified more than 100 papers related to type B, the subject of this update.…”

Is the RET proto-oncogene involved in the pathogenesis of intestinal neuronal dysplasia type B?

“…Intestinal neuronal dysplasia (IND), a malformation of the enteric nervous system (ENS) (1), is subclassified into two clinically and histologically distinct subtypes (2). IND type A (OMIM 243180) is a very rare condition characterized by congenital aplasia or hypoplasia of the sympathetic innervation.…”