Hirschsprung's disease surgery
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ; Associate Editor(s)-in-Chief: Aditya Ganti M.B.B.S. [2]
Overview
The mainstay of treatment for Hirschsprung's disease is surgery. The method of applied surgery is pull-through surgery. A portion of the colon with nerve cells is pulled through and sutured over the part without nerve cells.[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.
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, connected to a bag outside of the body. This allows bowel contents to be discharged into the bag.
- Later, when the child’s weight, age, and other conditions became favorable, a pull-through procedure is performed.[4]
Pull-through Procedure
- 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 for treating Hirschsprung’s disease in younger patients.[5]
- 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.[5]
- The Swenson, Soave, Duhamel, and Boley procedures vary slightly from each other, leading to equivalent results. They are include:
- The Swenson procedure leaves a small portion of the diseased bowel.[4]
- The Soave procedure leaves the outer wall of the colon unaltered.[6]
- The Boley procedure is just a small modification of the Soave procedure. [7]
- 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.[8]
Martin pull-through Procedure
- 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 form of the operations.
- When a child has total Hirschsprung's disease or small intestine Hirschsprung's disease, an ileostomy or jejunostomy is performed along with the Martin operation; then the child is treated for short bowel syndrome.
- If the affected portion of the lower intestine is restricted to the lower portion of 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.
- ↑ 4.0 4.1 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.
- ↑ 5.0 5.1 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.