eMedicine Specialties > Radiology > Pediatrics

Hirschsprung Disease: Follow-up

Author: Ciro Yoshida, Jr, MD, Staff Physician, Department of Diagnostic Imaging, Federal University of São Paulo (UNIFESP)
Coauthor(s): Salomao Faintuch, MD, Clinical Fellow, Department of Vascular and Interventional Radiology, Beth Israel Deaconess Medical Center; Henrique M Lederman, MD, PhD, Consulting Staff, Department of Radiology, LeBonheur Children's Medical Center and St Jude Children's Research Hospital; Professor of Radiology and Pediatric Radiology, Chief, Division of Diagnostic Imaging in Pediatrics, Federal University of Sao Paulo, Brazil
Contributor Information and Disclosures

Updated: Sep 17, 2008

Intervention

The treatment for Hirschsprung disease (HD) is surgical and is based on the removal or bypass of the poorly functioning, aganglionic bowel, with anastomosis of normally innervated intestine to the distal rectum. This can be performed by means of a preliminary colostomy followed by a definitive pull-through procedure or an immediate definitive procedure.5 Examples of the latter include the Swenson procedure, the Soave pull-through procedure, and the Duhamel procedure. Treatment innovations are the transanal endorectal pull-through and the laparoscopic approach to the 3 techniques.6,14

In general, the treatment plan varies according to the extent of aganglionosis and the age of the patient. A 1-stage procedure is possible when diagnosis is made early, before colonic dilatation, in short-segment disease. Otherwise, a primary colostomy is required. For long-segment disease and total colonic aganglionosis, temporary enterostomy is often the first step in management before definitive surgery. In most cases, treatment restores nearly normal motility and enables most affected individuals to have normal bowel function.

Common complications include HAEC after the Swenson operation, diarrhea and incontinence after the Soave endorectal pull-through procedure, and constipation after the Duhamel procedure. Other early complications include fistula or stenosis of the anastomosis.

 


More on Hirschsprung Disease

Overview: Hirschsprung Disease
Imaging: Hirschsprung Disease
Follow-up: Hirschsprung Disease
Multimedia: Hirschsprung Disease
References
Further Reading
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References

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Further Reading

Evaluation and treatment of constipation in infants and children: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.  1999 Nov (revised 2006 Sep).  13 pages.  NGC:005245
 
ASGE guideline: guideline on the use of endoscopy in the management of constipation. American Society for Gastrointestinal Endoscopy.  2005 Aug.  3 pages.  NGC:004485

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Keywords

Hirschsprung disease, congenital megacolon, aganglionic megacolon, aganglionosis, HD, Hirschsprung's disease, transition zone, Swenson procedure, Soave pull-through procedure, Duhamel procedure, Hirschsprung-associated enterocolitis, Hirschsprung's-associated enterocolitis, HAEC, neurocristopathy 

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Contributor Information and Disclosures

Author

Ciro Yoshida, Jr, MD, Staff Physician, Department of Diagnostic Imaging, Federal University of São Paulo (UNIFESP)
Disclosure: Nothing to disclose.

Coauthor(s)

Salomao Faintuch, MD, Clinical Fellow, Department of Vascular and Interventional Radiology, Beth Israel Deaconess Medical Center
Salomao Faintuch, MD is a member of the following medical societies: American Roentgen Ray Society, Radiological Society of North America, and Society of Cardiovascular and Interventional Radiology
Disclosure: Nothing to disclose.

Henrique M Lederman, MD, PhD, Consulting Staff, Department of Radiology, LeBonheur Children's Medical Center and St Jude Children's Research Hospital; Professor of Radiology and Pediatric Radiology, Chief, Division of Diagnostic Imaging in Pediatrics, Federal University of Sao Paulo, Brazil
Henrique M Lederman, MD, PhD is a member of the following medical societies: Society for Pediatric Radiology
Disclosure: Nothing to disclose.

Medical Editor

Robert J Starshak, MD, Medical Director, Assistant Clinical Professor, Department of Radiology, Medical College of Wisconsin, Falls Medical Group
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

David A Stringer, BSc, MBBS, FRCR, FRCPC, Professor, National University of Singapore; Head, Diagnostic Imaging, KK Women's and Children's Hospital, Singapore
David A Stringer, BSc, MBBS, FRCR, FRCPC is a member of the following medical societies: British Columbia Medical Association, Canadian Association of Radiologists, European Society of Paediatric Radiology, Ontario Medical Association, Radiological Society of North America, Royal College of Physicians and Surgeons of Canada, Royal College of Radiologists, and Society for Pediatric Radiology
Disclosure: Sirius d'innovation None Board membership

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Eugene C Lin, MD, Consulting Radiologist, Virginia Mason Medical Center; Clinical Assistant Professor of Radiology, University of Washington School of Medicine
Eugene C Lin, MD is a member of the following medical societies: American College of Nuclear Medicine, American College of Radiology, Radiological Society of North America, and Society of Nuclear Medicine
Disclosure: Nothing to disclose.

 
 
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