Histopathological criteria for intestinal neuronal dysplasia of the submucosal plexus (type B)

Abstract

The aim of this study was to review critically the diagnostic features of intestinal neuronal dysplasia type B (IND B). Over a period of 5 years colonic mucosal biopsies of 773 children with symptoms of chronic constipation were examined. Four biopsies taken 2-10 cm above the pectinate line were cut in serial sections and histochemical lactate dehydrogenase, succinate dehydrogenase, (SDH) and acetylcholinesterase (AChE) reactions performed. Presence of giant ganglia of the submucosal plexus, being characterized by more than seven nerve cells, established the diagnosis of IND B. Giant ganglia were found to be age-independent changes, while hyperplasia of the submucosal plexus, increase of AChE activity in nerve fibres of the lamina propria and low SDH activity in nerve cells proved to be age-dependent findings which disappear during the maturation of the enteric nervous system. Using these criteria IND B was diagnosed in 209 children. In 64 of these patients a combination of IND B and aganglionosis (Hirschsprung's disease) was found. IND B seems to be related to premature expression of laminin A during embryogenesis, resulting in premature nerve cell differentiation in the myenteric and submucosal plexus, which in turn blocks neuroblast colonization of the rectum. IND B, hypoganglionosis and aganglionosis, which are often combined, may therefore be considered to be different manifestations of the same developmental abnormality.

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

“…3,[5][6][7][8] Modified criteria evolved gradually, primarily because of a series of papers coauthored by Meier-Ruge in the mid-1990s that clarified in detail how enzyme histochemistry should be performed and ganglion cells counted. [7][8][9][10][11][12] During this period, Meier-Ruge and colleagues reported that abnormal AChE-positive innervation is an unreliable diagnostic feature after age 9 months, emphasized that submucosal ganglion cell hyperplasia was the key diagnostic feature of IND B, and commented that:…”

“…These giant ganglia comprise only 3-5% of all ganglia seen in a given case and are usually not observed in the distal rectum (within 6-7 cm of the pectinate line). 9 Because of the latter observation, Meier-Ruge insists that diagnostic biopsies be obtained 8 to 10 cm proximal to the dentate line, 13 which is much farther from the anus than the typical biopsy used to rule out HSCR.…”

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

“…Наиболее характерными морфологическими его признаками являются гигантские нервные ганглии в подслизистом слое более чем с 8 нервными клетками (нормальный ганглий содержит от 2 до 4 нервных кле-ток). При этой патологии отмечено нарушение моторно-эвакуаторной функции кишки в различных вариантах: от легких запоров до клинической картины, имитиру-ющей болезнь Гиршпрунга [16,18]. Для постановки диагноза, который определяется как «количественный», нужно по крайней мере на 25-30 участках толстой киш-ки обнаружить от 4 до 7 гигантских ганглиев.…”

“…В отли-чие от болезни Гиршпрунга, исследование довольно большого числа пациентов с интестинальной нейромы-шечной дисплазией типа В показало, что гиперплазия подслизистого сплетения и увеличение ацетилхолинэ-стеразной активности в нервных волокнах собственной пластинки слизистой оболочки зависит от возраста и может исчезнуть в процессе созревания нервной системы кишечника [1]. Провести дифференциацию между этой болезнью и незрелостью вегетативной нервной системы на первом году жизни крайне сложно [1,18,19]. ЗАКЛЮЧЕНИЕ У вышеописанных врожденных нарушений, как энте-ральной нервной системы, так и мышечной ткани стенки кишки, манифестирующих энтероколитом или перфо-рацией, гистологическая интерпретация порой бывает достаточно спорной и затруднительной.…”

Constipation as the Presenting Symptom in De Novo Multiple Endocrine Neoplasia Type 2B

“…27 Intestinal neuronal dysplasia type B (IND B) has several features resembling ganglioneuromatosis, most importantly submucosal ganglia with more than six nerve cells. 28 No mention is made about the myenteric plexus. Whether IND B represents an entity or a secondary phenomenon has been much debated, 29 but is beyond the scope of this article.…”