Hirschsprung's disease surgery: Difference between revisions

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{{Hirschsprung's disease}}
{{Hirschsprung's disease}}
{{CMG}}
{{CMG}} ; {{AE}} {{ADG}}


==Overview==
==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).<ref name="pmid28154902">{{cite journal |vauthors=Gosain A, Frykman PK, Cowles RA, Horton J, Levitt M, Rothstein DH, Langer JC, Goldstein AM |title=Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis |journal=Pediatr. Surg. Int. |volume=33 |issue=5 |pages=517–521 |year=2017 |pmid=28154902 |doi=10.1007/s00383-017-4065-8 |url=}}</ref><ref name="pmid28180937">{{cite journal |vauthors=Langer JC, Rollins MD, Levitt M, Gosain A, Torre L, Kapur RP, Cowles RA, Horton J, Rothstein DH, Goldstein AM |title=Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease |journal=Pediatr. Surg. Int. |volume=33 |issue=5 |pages=523–526 |year=2017 |pmid=28180937 |doi=10.1007/s00383-017-4066-7 |url=}}</ref>
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]].<ref name="pmid28154902">{{cite journal |vauthors=Gosain A, Frykman PK, Cowles RA, Horton J, Levitt M, Rothstein DH, Langer JC, Goldstein AM |title=Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis |journal=Pediatr. Surg. Int. |volume=33 |issue=5 |pages=517–521 |year=2017 |pmid=28154902 |doi=10.1007/s00383-017-4065-8 |url=}}</ref><ref name="pmid28180937">{{cite journal |vauthors=Langer JC, Rollins MD, Levitt M, Gosain A, Torre L, Kapur RP, Cowles RA, Horton J, Rothstein DH, Goldstein AM |title=Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease |journal=Pediatr. Surg. Int. |volume=33 |issue=5 |pages=523–526 |year=2017 |pmid=28180937 |doi=10.1007/s00383-017-4066-7 |url=}}</ref><ref name="pmid18162828">{{cite journal |vauthors=Dasgupta R, Langer JC |title=Evaluation and management of persistent problems after surgery for Hirschsprung disease in a child |journal=J. Pediatr. Gastroenterol. Nutr. |volume=46 |issue=1 |pages=13–9 |year=2008 |pmid=18162828 |doi=10.1097/01.mpg.0000304448.69305.28 |url=}}</ref>


==Surgery==
==Surgery==


Treatment of Hirschsprung's disease consists of surgical removal of the abnormal section of the colon. There are two steps involved to achieve this goal.
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.  
=== Swenson & Bill Procedure ===
*Orvar Swenson, first performed this surgery in 1948 (Swenson 839).
*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.
*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.
* Later, when the child’s [[weight]], [[age]], and other conditions become favorable, a pull-through procedure will be performed.<ref name="pmid18872852">{{cite journal |vauthors=SWENSON O, BILL AH |title=Resection of rectum and rectosigmoid with preservation of the sphincter for benign spastic lesions producing megacolon; an experimental study |journal=Surgery |volume=24 |issue=2 |pages=212–20 |year=1948 |pmid=18872852 |doi= |url=}}</ref>
*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).
=== Pull-through Procedure ===
** The Swenson procedure leaves a small portion of the diseased bowel.
*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.<ref name="pmid8080373">{{cite journal |vauthors=Sawin R, Hatch E, Schaller R, Tapper D |title=Limited surgery for lower-segment Hirschsprung's disease |journal=Arch Surg |volume=129 |issue=9 |pages=920–4; discussion 924–5 |year=1994 |pmid=8080373 |doi= |url=}}</ref>
** The Soave procedure leaves the outer wall of the colon unaltered.
*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.<ref name="pmid8080373">{{cite journal |vauthors=Sawin R, Hatch E, Schaller R, Tapper D |title=Limited surgery for lower-segment Hirschsprung's disease |journal=Arch Surg |volume=129 |issue=9 |pages=920–4; discussion 924–5 |year=1994 |pmid=8080373 |doi= |url=}}</ref>
** The Boley procedure is just a small modification of the Soave procedure.
*The Swenson, Soave, Duhamel, and Boley procedures vary slightly from each other, leading to equivalent results.
** 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).
** The Swenson procedure leaves a small portion of the diseased [[bowel]].<ref name="pmid18872852">{{cite journal |vauthors=SWENSON O, BILL AH |title=Resection of rectum and rectosigmoid with preservation of the sphincter for benign spastic lesions producing megacolon; an experimental study |journal=Surgery |volume=24 |issue=2 |pages=212–20 |year=1948 |pmid=18872852 |doi= |url=}}</ref>
*Sawin notes that Pull-through procedures requires a colostomy, but with recent advacnes in awareness among doctors and parents with early diagnosis, doctors can keep the colon clean and perform the pull-through procedure without a colostomy.  
** The Soave procedure leaves the outer wall of the colon unaltered.<ref name="pmid14131949">{{cite journal |vauthors=SOAVE F |title=HIRSCHSPRUNG'S DISEASE: A NEW SURGICAL TECHNIQUE |journal=Arch. Dis. Child. |volume=39 |issue= |pages=116–24 |year=1964 |pmid=14131949 |pmc=2019134 |doi= |url=}}</ref>
*In general, 85 percent of patients that have the pull-through surgery live normal lives afterwards. The other 15 percent have to take a laxative for the rest of their lives.
** The Boley procedure is just a small modification of the Soave procedure.<ref name="pmid512801">{{cite journal |vauthors=Kleinhaus S, Boley SJ, Sheran M, Sieber WK |title=Hirschsprung's disease -- a survey of the members of the Surgical Section of the American Academy of Pediatrics |journal=J. Pediatr. Surg. |volume=14 |issue=5 |pages=588–97 |year=1979 |pmid=512801 |doi= |url=}}</ref>
*In rare cases, if the mother has Hirschsprung’s and passes it on to her child, the child is said to have total Hirschsprung’s, where there are no ganglion cells at all in the colon (Sawin).
** 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.<ref name="pmid14217784">{{cite journal |vauthors=DUHAMEL B |title=RETRORECTAL AND TRANSANAL PULL-THROUGH PROCEDURE FOR THE TREATMENT OF HIRSCHSPRUNG'S DISEASE |journal=Dis. Colon Rectum |volume=7 |issue= |pages=455–8 |year=1964 |pmid=14217784 |doi= |url=}}</ref>
*In this case, the Martin pull-through operation is conducted. The Martin operation is the most invasive of the operations, for it requires a colostomy no matter how early you diagnose total Hirschsprung’s.
 
*Even rarer, notes Dr. Sawin, there are times where the disease begins in the small intestine, making treatment even harder.
=== Martin's Pull-through Procedure ===
*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]].  
*In rare cases, there are no [[ganglion cells]] in the [[colon]]. In this case, Martin's pull-through operation is conducted.  
*On very rare occasions, pull-through surgery is not the preferred method when it comes to treating Hirschsprung’s disease.
*Among all of the other operations for treating Hirschsprung's disease, this is the most invasive procedure.
*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.
*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.<ref name="pmid6834220">{{cite journal |vauthors=Davies MR, Cywes S |title=Inadequate pouch emptying following Martin's pull-through procedure for intestinal aganglionosis |journal=J. Pediatr. Surg. |volume=18 |issue=1 |pages=14–20 |year=1983 |pmid=6834220 |doi= |url=}}</ref>


==References==
==References==

Latest revision as of 19:21, 17 August 2017

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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. 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.[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 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.[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 of 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.
    • 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'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.[9]

References

  1. 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.
  2. 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.
  3. 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. 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. 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.
  6. SOAVE F (1964). "HIRSCHSPRUNG'S DISEASE: A NEW SURGICAL TECHNIQUE". Arch. Dis. Child. 39: 116–24. PMC 2019134. PMID 14131949.
  7. 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.
  8. DUHAMEL B (1964). "RETRORECTAL AND TRANSANAL PULL-THROUGH PROCEDURE FOR THE TREATMENT OF HIRSCHSPRUNG'S DISEASE". Dis. Colon Rectum. 7: 455–8. PMID 14217784.
  9. 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.