SEGMENTAL ABSENCE OF INTESTINAL MUSCULATURE: CASE REPORT OF A RARE CAUSE OF NEONATAL INTESTINAL OBSTRUCTION

Segmental absence of intestinal musculature is a distinct histopathological entity with rare occurrence. The condition typically presents in neonates, with the primary manifestation being intestinal obstruction or perforation. Here we present the case of a neonate who initially presented with a high lesion of anal atresia. In the first few days following colostomy, the infant exhibited signs of intestinal obstruction. An exploratory laparotomy revealed ileal stenosis. A resected ileum specimen showed a narrowed segment of the ileum. Histopathological examination showed focal hypoplasia to total absence of muscularis propria with intact mucosal, submucosal, and serosal layers. Histochemical and immunohistochemical studies confirmed the absence and hypoplasia of muscularis propria, presence of ganglion cells, and absence of Cajal cells in the pathological segment. In cases of neonatal intestinal obstruction due to segmental absence of intestinal musculature, the possibility of defects of intestinal musculature should be investigated, particularly in conjunction with other congenital diseases.