Cajal cells and post-surgical dismotility in Hirschsprung disease

Abstract

Hirschsprung disease (HD) is characterised by the absence of ganglion cells in myenteric and submucosal plexus in the distal colon in its classical form. As a consequence intestinal motility dysfunction occurs, the clinical picture resembles structural obstruction. Diagnosis of aganglionosis is performed by rectal biopsy and treatment is surgical by removing the aganglionosis portion. Intersticial Cajal cells modulate the motor function of smooth muscle cells by the synaptic connection with the enteric nervous system. The identification of the numeric decrease of these cells in the ganglionic intestinal portion in the HD allowed elaborating the hypothesis that numeric reduction of Cajal cells may cause symptoms of post-surgical intestinal dysmotility.The present study tryed to verify this hypothesis, by identifying an association between post-surgical complications and number of Cajal cells in the ganglionic segment.Medical records and histological samples of patients in post-surgical HD status and followed at Unicamp Clinics Hospital, operated from 2001 to 2014, were re-examined. Immunohistochemistry for Cajal cells were performed in order to count cells number and were compared to historical values found in healthy adults.Although most of the cases present a number of low-ICC, the count of these ganglion cells segment is not associated with the occurrence of postoperative symptoms. The findings of this survey are similar to other studies with similar methodology.