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A pull-through procedure is the definitive operation for Hirschsprung disease, involving the removal of the abnormal segment of bowel that has no nerves, pulling through the normal bowel and connecting it to the anus. Several types of pull-through procedures exist including the Soave, Swenson and Duhamel.
With Hirschsprung's disease, the segment lacking neurons (aganglionic) becomes constricted, causing the normal, proximal section of bowel to become distended with feces. This narrowing of the distal colon and the failure of relaxation in the aganglionic segment are thought to be caused by the lack of neurons containing nitric oxide synthase.
Video explanation. Tanner Marshall, MS, Rishi Desai, MD Hirschsprung disease is also known as congenital aganglionic megacolon, so Hirschsprung’s is a disease that’s present since birth, in which a ganglion, or cluster of nerves is missing, which ultimately leads to a blocked colon, causing it to enlarge.
Orvar Swenson (7 February 1909 – 13 April 2012) was a Swedish-born American pediatric surgeon.He discovered the cause of Hirschsprung's disease and in 1948, with Alexander Bill, performed the first pull-through operation in a child with megacolon, which then became a treatment for the disease.
Also called Hirschsprung's disease, it is a congenital disorder of the colon in which nerve cells of the myenteric plexus in its walls, also known as ganglion cells, are absent. It is a rare disorder (1:5000), with prevalence among males being four times that of females. Hirschsprung's disease develops in the fetus during the early stages of ...
Abdominal pain, abdominal bloating, vomiting, constipation, bloody stool [1] [2] Complications: Ischemic bowel [1] Usual onset: Rapid or more gradual [2] Risk factors: Intestinal malrotation, enlarged colon, Hirschsprung disease, pregnancy, abdominal adhesions, chronic constipation [1] [3] Diagnostic method: Medical imaging (plain X-rays, GI ...
The distal large bowel from the point of neuronal arrest to the anus is continuously aganglionic. It is a rare disorder (1:5000), with prevalence among males being four times that of females. [10] Achalasia is a motor disorder of the esophagus characterized by decrease in ganglion cell density in the myenteric plexus. The cause of the lesion is ...
Also known as Shah–Waardenburg syndrome, or Waardenburg–Shah syndrome, type 4 has most of the same features as type 2 (i.e. no telecanthus, or apparent wider eye gap), but with the addition of Hirschsprung's disease, which is a congenital lack of nerves in the intestines leading to bowel dysfunction.