Hirschsprung's disease surgery
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The mainstay of treatment for Hirschsprung's disease is surgery. The method of applied surgery is pull-through surgery. In this procedure, a portion of the colon with nerve cells is pulled through and sutured over the portion of the colon without nerve cells.
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.
Swenson & Bill Procedure
- The first stage is colostomy. When a colostomy is performed, the large intestine is excised and an opening is made through the abdomen and connected to a bag outside of the body. This allows the bowel's contents to be discharged into the bag.
- Later, when the child’s weight, age, and other conditions become favorable, a pull-through procedure will be performed.
- The pull-through procedure repairs the colon by connecting the functioning portion of the bowel to the anus, bypassing the dysfunctioning portion. The pull-through procedure is the typical method of treating Hirschsprung’s disease 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 vary slightly from each other, leading to equivalent results.
- 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 normal and diseased bowel. The front of the bowel will end up with no cells, but the back will be healthy.
Martin's Pull-through Procedure
- In rare cases, there are no ganglion cells in the colon. In this case, Martin's pull-through operation is conducted.
- Among all of the other operations for treating Hirschsprung's disease, this is the most invasive procedure.
- When a child has Hirschsprung's disease involving the total colon or small intestine, an ileostomy or jejunostomy is performed along with Martin's operation; then the child is treated for short bowel syndrome.
- When the affected portion is restricted to the lower portion of rectum, other surgical procedures such as a posterior rectal myectomy can be considered.
- 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.
- SWENSON O, BILL AH (1948). "Resection of rectum and rectosigmoid with preservation of the sphincter for benign spastic lesions producing megacolon; an experimental study". Surgery. 24 (2): 212–20. PMID 18872852.
- Sawin R, Hatch E, Schaller R, Tapper D (1994). "Limited surgery for lower-segment Hirschsprung's disease". Arch Surg. 129 (9): 920–4, discussion 924–5. PMID 8080373.
- SOAVE F (1964). "HIRSCHSPRUNG'S DISEASE: A NEW SURGICAL TECHNIQUE". Arch. Dis. Child. 39: 116–24. PMC 2019134. PMID 14131949.
- Kleinhaus S, Boley SJ, Sheran M, Sieber WK (1979). "Hirschsprung's disease -- a survey of the members of the Surgical Section of the American Academy of Pediatrics". J. Pediatr. Surg. 14 (5): 588–97. PMID 512801.
- DUHAMEL B (1964). "RETRORECTAL AND TRANSANAL PULL-THROUGH PROCEDURE FOR THE TREATMENT OF HIRSCHSPRUNG'S DISEASE". Dis. Colon Rectum. 7: 455–8. PMID 14217784.
- Davies MR, Cywes S (1983). "Inadequate pouch emptying following Martin's pull-through procedure for intestinal aganglionosis". J. Pediatr. Surg. 18 (1): 14–20. PMID 6834220.