Hirschsprung's disease surgery
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
The mainstay of treatment is surgery. The method of surgery employed is pull-through surgery. A portion of the colon that does have nerve cells is pulled through and sewn over the part that lacks nerve cells (National Digestive Diseases Information Clearinghouse).[1][2][3]
Surgery
Treatment of Hirschsprung's disease consists of surgical removal of the abnormal section of the colon. Orvar Swenson, first performed this surgery in 1948. There are two steps involved in this procedure.
- The first stage is colostomy. When a colostomy is performed, the large intestine is cut and an opening is made through the abdomen. This allows bowel contents to be discharged into a bag.
- Later, when the child’s weight, age, and condition is right, a pull-through procedure is performed.
- The pull-through procedure repairs the colon by connecting the functioning portion of the bowel to the anus. The pull through procedure is the typical method for treating Hirschsprung’s in younger patients.
- Swenson devised the original procedure, but the pull-through surgery has been modified many times. Sawin, an expert in pull-through surgery, notes that, although there are about five different pull-through procedures, they are all more or less equally effective in treating the disorder.
- The Swenson, Soave, Duhamel, and Boley procedures all vary slightly from each other with equivalent results (Sawin).
- The Swenson procedure leaves a small portion of the diseased bowel.
- The Soave procedure leaves the outer wall of the colon unaltered.
- The Boley procedure is just a small modification of the Soave procedure.
- The Duhamel procedure uses a surgical stapler to connect the good and bad bowel. The front of the bowel will end up with no cells, but the back will be healthy (Sawin).
- In rare cases, where there are no ganglion cells at all in the colon the Martin pull-through operation is conducted. The Martin operation is the most invasive of the operations.
- When a child has total Hirschsprung's or small intestine Hirschsprung's, an ileostomy or jejunostomy is conducted along with the Martin operation, and the child is then treated for short bowel syndrome.
- If the affected portion of the lower intestine is restricted to the lower portion of the rectum, other surgical procedures, such as the posterior rectal myectomy, can be performed.
References
- ↑ Gosain A, Frykman PK, Cowles RA, Horton J, Levitt M, Rothstein DH, Langer JC, Goldstein AM (2017). "Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis". Pediatr. Surg. Int. 33 (5): 517–521. doi:10.1007/s00383-017-4065-8. PMID 28154902.
- ↑ Langer JC, Rollins MD, Levitt M, Gosain A, Torre L, Kapur RP, Cowles RA, Horton J, Rothstein DH, Goldstein AM (2017). "Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease". Pediatr. Surg. Int. 33 (5): 523–526. doi:10.1007/s00383-017-4066-7. PMID 28180937.
- ↑ Dasgupta R, Langer JC (2008). "Evaluation and management of persistent problems after surgery for Hirschsprung disease in a child". J. Pediatr. Gastroenterol. Nutr. 46 (1): 13–9. doi:10.1097/01.mpg.0000304448.69305.28. PMID 18162828.