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Semin Pediatr Surg. 2004 Nov;13(4):293-9.

Variant Hirschsprung's disease.

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  • 1Children's Research Centre, Our Lady's Hospital for Sick Children, Crumlin, Dublin 12, Ireland.


Conditions that clinically resemble HD despite the presence of ganglion cells on suction rectal biopsy results, can be diagnosed by providing an adequate biopsy and employing a variety of histological techniques. Intestinal neuronal dysplasia is a distinct clinical entity that can be clearly proven histologically. Patients with IND not only have abnormalities of submucosal and myenteric plexuses but also defective innervation of the muscle. Internal sphincter achalasia, which is histologically characterized by nitrergic nerve depletion, can be diagnosed on anorectal manometry and successfully treated by internal sphincter myectomy. The outcome of smooth muscle disorders is generally fatal. The need for surgical intervention should be weighed carefully and individualized because most explorations have not been helpful and are probably not necessary.

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