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Pediatria pre prax 4/2014
Hirschsprung’s disease
Hirschsprung’s disease is a severe congenital pathology. The cause of HD is a defect in migration of neural cells from neural crest to intestinal wall during embryonal development. The result is aganglionosis of myenteric Auerbach‘s and submucosal Meissner’s plexus. Aganglionic intestine is in permanent spasm with failure of relaxation, missing peristalsis and dilation of proximal segment. Diagnosis is confirmed by irigography and biopsy. Therapy is only surgical: resection of aganglionic part, transposition of normal inervated colon and creation of anastomosis. The aim of surgery is to allow the child normal stooling.
Keywords: Hirschsprung’s disease, diagnosis, surgical therapy.
