Pediatric Hirschsprung Disease Treatment & Management

  • Author: Holly L Neville, MD; Chief Editor: Carmen Cuffari, MD   more...
 
Updated: Jul 13, 2010
 

Medical Care

  • If a child with Hirschsprung disease has symptoms and signs of a high-grade intestinal obstruction, initial therapy should include intravenous hydration, withholding of enteral intake, and intestinal and gastric decompression.
  • Decompression can be accomplished through placement of a nasogastric tube and either digital rectal examination or normal saline rectal irrigations 3-4 times daily.
  • Administer broad-spectrum antibiotics to patients with enterocolitis.
  • Immediately request surgical consultation for biopsy confirmation and treatment plan.
  • While awaiting surgical intervention in the event of a planned single-stage pull-through procedure, the baby should receive scheduled vaccinations.
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Surgical Care

  • The surgical options vary according to the patient's age, mental status, ability to perform activities of daily living, length of the aganglionic segment, degree of colonic dilation, and presence of enterocolitis.
  • Surgical options include colostomy at the level of normal bowel, rectal irrigations followed by rectal resection with a pull-through procedure once bowel caliber is restored to normal, and a staged procedure with placement of a diverting colostomy followed by a pull-through procedure. The single-stage pull-through procedure may be performed with laparoscopic, open, or transanal techniques. This procedure is generally performed after the newborn has had rectal irrigations at home and has passed the physiologic nadir.
  • The ability to perform a single-stage pull-through procedure largely depends on the availability, experience, and capabilities of the staff pathologist because aganglionic intestine must not be in the pull-through segment.
  • Recurrent postoperative enterocolitis may require treatment. Current therapeutic options include rectal dilations, application of topical nitric oxide, posterior myotomy/myectomy,[5] or injection of botulinum toxin.[6]
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Diet

A special diet is not required. However, preoperatively and in the early postoperative period, infants on a nonconstipated regimen, such as breast milk, are more easily managed.

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Activity

Postoperatively, patients may return to their normal physical activities.

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Contributor Information and Disclosures
Author

Holly L Neville, MD  Assistant Professor of Clinical Surgery, Division of Pediatric Surgery, University of Miami Miller School of Medicine

Holly L Neville, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, and Association of Women Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Hisham Nazer, MB, BCh, FRCP, DCh, DTM&H  Professor of Pediatrics, Consultant in Pediatric Gastroenterology, Hepatology and Clinical Nutrition, Bushnaq Medical Centre, University of Jordan

Hisham Nazer, MB, BCh, FRCP, DCh, DTM&H is a member of the following medical societies: Royal College of Paediatrics and Child Health, Royal College of Physicians, Royal College of Surgeons in Ireland, Royal College of Surgeons of Edinburgh, and Royal Society of Tropical Medicine and Hygiene

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Steven M Schwarz, MD, FAAP, FACN, AGAF  Professor of Pediatrics, Children's Hospital at Downstate, SUNY-Downstate Medical Center

Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research

Disclosure: Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor; Johnson & Johnson, Inc. Grant/research funds Independent contractor

Chief Editor

Carmen Cuffari, MD  Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

References

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  4. Emir H, Akman M, Sarimurat N, et al. Anorectal manometry during the neonatal period: its specificity in the diagnosis of Hirschsprung's disease. Eur J Pediatr Surg. Apr 1999;9(2):101-3. [Medline].

  5. Wildhaber BE, Pakarinen M, Rintala RJ, Coran AG, Teitelbaum DH. Posterior myotomy/myectomy for persistent stooling problems in Hirschsprung's disease. J Pediatr Surg. Jun 2004;39(6):920-6; discussion 920-6. [Medline].

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Abdominal radiograph demonstrating small bowel obstruction and megacolon in infant with Hirschsprung Disease.
Barium enema demonstrating transition zone. The transition zone shows the transition from dilated, normally innervated bowel to normal caliber, noninnervated bowel.
Intraoperative finding in total colonic aganglionosis. Note the decompressed bowel adjacent to the distended colon.
 
 
 
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