Hirschsprung's disease surgery: Difference between revisions

Jump to navigation Jump to search
No edit summary
 
(5 intermediate revisions by 2 users not shown)
Line 4: Line 4:


==Overview==
==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]].<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>
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. Orvar Swenson, first performed this surgery in 1948. There are two steps involved in this procedure.
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 ===
=== 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.
*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 became favorable, a pull-through procedure is 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>  
* 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>  


=== Pull-through Procedure ===
=== 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.<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 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>
*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>
*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 Swenson, Soave, Duhamel, and Boley procedures vary slightly from each other, leading to equivalent results. They are include:
*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.<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>
** 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>
** 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>
** 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>
** 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>
** 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>
** 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>
** 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>


=== Martin pull-through Procedure ===
=== Martin's 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.
*In rare cases, there are no [[ganglion cells]] in the [[colon]]. In this case, Martin's pull-through operation is conducted.  
*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]].  
*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 [[rectum]], other surgical procedures, such as the posterior rectal myectomy, can be performed.<ref name="pmid6730717">{{cite journal |vauthors=Hickey M, Guiney EJ |title=The Duhamel/Martin operation in the treatment of Hirschsprung's disease |journal=Z Kinderchir |volume=39 |issue=2 |pages=110–1 |year=1984 |pmid=6730717 |doi=10.1055/s-2008-1044187 |url=}}</ref>
*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

Hirschsprung's disease Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Hirschsprung's Disease from other Diseases

Epidemiology and Demographics

Risk factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory findings

Electrocardiogram

X-ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary prevention

Secondary prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Hirschsprung's disease surgery On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Hirschsprung's disease surgery

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Hirschsprung's disease surgery

CDC on Hirschsprung's disease surgery

Hirschsprung's disease surgery in the news

Blogs on Hirschsprung's disease surgery

Directions to Hospitals Treating Hirschsprung's disease

Risk calculators and risk factors for Hirschsprung's disease surgery

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.