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Pathology definition - Hirschsprung disease
Hirschsprung disease
Hirschsprung disease usually involves the rectum. Hirschsprung disease is characterized by dilation of the colon which is proximal to the aganglionic segment ( megacolon).
Hirschsprung disease is caused by failure of the migration of the neural crest cell during the process of embryogenesis. There will be an absent of the ganglion cells of the Auerbach and Meissner plexuses.
Hirschsprung disease commonly associated with Down syndrome and more common in boys than girls. The patient may present with bloating of the abdomen , chronic constipation and failure in passing the meconium. In severe case, the patient may present with perforation of the colon and producing foul smelling, watery stool due to acute enterocolitis.
References
1.Martin, L W, and A M Torres. “Hirschsprung’s Disease.” The Surgical Clinics of North America 65, no. 5 (October 1985): 1171–1180.
2.Barr, L. C., J. Booth, M. I. Filipe, and J. O. Lawson. “Clinical Evaluation of the Histochemical Diagnosis of Hirschsprung’s Disease.” Gut 26, no. 4 (April 1, 1985): 393–399. doi:10.1136/gut.26.4.393.
Hirschsprung disease usually involves the rectum. Hirschsprung disease is characterized by dilation of the colon which is proximal to the aganglionic segment ( megacolon).
Hirschsprung disease is caused by failure of the migration of the neural crest cell during the process of embryogenesis. There will be an absent of the ganglion cells of the Auerbach and Meissner plexuses.
Hirschsprung disease commonly associated with Down syndrome and more common in boys than girls. The patient may present with bloating of the abdomen , chronic constipation and failure in passing the meconium. In severe case, the patient may present with perforation of the colon and producing foul smelling, watery stool due to acute enterocolitis.
References
1.Martin, L W, and A M Torres. “Hirschsprung’s Disease.” The Surgical Clinics of North America 65, no. 5 (October 1985): 1171–1180.
2.Barr, L. C., J. Booth, M. I. Filipe, and J. O. Lawson. “Clinical Evaluation of the Histochemical Diagnosis of Hirschsprung’s Disease.” Gut 26, no. 4 (April 1, 1985): 393–399. doi:10.1136/gut.26.4.393.
