Altered Distribution of Interstitial Cells of Cajal in Normoganglionic and Transitional Zone of Hirschsprung Disease and Their Clinical Significance

Radmila Jankovic, Sanja Sindjic-Antunovic, Marija Lukac, Dragana Vujovic, Jovan Jevtic, Milica Skender-Gazibara


Objective − The aim of this study was to evaluate interstitial cells of Cajal (ICC) in Hirschsprung disease (HD), especially in children who had postsurgical problems.

Material and Methods − The study included tissue samples of normoganglionic (NZ) and transitional zone (TZ) of 33 HD pediatric patients. Samples of bowel from 10 autopsy controls who did not have dysmotility were also analyzed. Hematoxylin-eosin (H&E) and immunohistochemical (MAP-2 and c-Kit (CD117)) staining were performed. Myenteric ganglia were analyzed at 5 microscopic fields H&E and MAP-2 stained sections (magnification 400×). The diameters of the submucosal nerves were measured at 3 microscopic H&E fields (magnification 400×). The ICC were analyzed on c-Kit immunostained slides at 3 microscopic fields per each bowel layer (magnification 200×): deep submucosa, circular and longitudinal muscular layer and zone of myenteric plexus.

Results − The myenteric ganglion cell count was significantly lower in TZ of all children with HD, while there were no significant differences in NZ between the study group and age-matched controls. The ICC network was affected in all cases of HD. The total ICC count was significantly lower in NZ than in control (P<0.001). A critically lower ICC count (<50% of minimal ICC count in the control group) in the NZ was found in patients with postoperative problems (constipation and enterocolitis).

Conclusion − Decreased count of the ICC in the NZ could be the cause of intestinal motility difficulties in the postoperative period in children with HD.


Hirschsprung Disease; Interstitial Cells of Cajal; Enteric Nervous System

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