eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology

Hirschsprung Disease: Treatment & Medication

Author: Holly L Neville, MD, Assistant Professor of Clinical Surgery, Division of Pediatric Surgery, University of Miami Miller School of Medicine
Contributor Information and Disclosures

Updated: Nov 17, 2008

Treatment

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.

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

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.

Activity

  • Postoperatively, patients may return to their normal physical activities.

Medication

Drug therapy currently is not a component of the standard of care for this disease itself; however, some medications may be used to treat complications of Hirschsprung disease. See Treatment. Medications may include antibiotics for the treatment of enterocolitis or the use of botulinum toxin injection at the anal sphincter for the treatment of recurrent enterocolitis due to anal hypertonicity.

More on Hirschsprung Disease

Overview: Hirschsprung Disease
Differential Diagnoses & Workup: Hirschsprung Disease
Treatment & Medication: Hirschsprung Disease
Follow-up: Hirschsprung Disease
Multimedia: Hirschsprung Disease
References
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References

  1. Meza-Valencia BE, de Lorimier AJ, Person DA. Hirschsprung disease in the U.S. associated Pacific Islands: more common than expected. Hawaii Med J. Apr 2005;64(4):96-8, 100-1. [Medline].

  2. Machens A, Hauptmann S, Dralle H. Modification of multiple endocrine neoplasia 2A phenotype by cell membrane proximity of RET mutations in exon 10. Endocr Relat Cancer. Oct 20 2008;[Medline].

  3. Edery P, Lyonnet S, Mulligan LM, et al. Mutations of the RET proto-oncogene in Hirschsprung's disease. Nature. Jan 27 1994;367(6461):378-80. [Medline].

  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].

  6. Minkes RK, Langer JC. A prospective study of botulinum toxin for internal anal sphincter hypertonicity in children with Hirschsprung's disease. J Pediatr Surg. Dec 2000;35(12):1733-6. [Medline].

  7. Belknap WM. Hirschsprung's Disease. Curr Treat Options Gastroenterol. Jun 2003;6(3):247-256. [Medline].

  8. Fujimoto T, Hata J, Yokoyama S, Mitomi T. A study of the extracellular matrix protein as the migration pathway of neural crest cells in the gut: analysis in human embryos with special reference to the pathogenesis of Hirschsprung's disease. J Pediatr Surg. Jun 1989;24(6):550-6. [Medline].

  9. Hackam DJ, Filler RM, Pearl RH. Enterocolitis after the surgical treatment of Hirschsprung's disease: risk factors and financial impact. J Pediatr Surg. Jun 1998;33(6):830-3. [Medline].

  10. Ikeda K, Goto S. Diagnosis and treatment of Hirschsprung's disease in Japan. An analysis of 1628 patients. Ann Surg. Apr 1984;199(4):400-5. [Medline].

  11. Kaplan P, de Chaderevian JP. Piebaldism-Waardenburg syndrome: histopathologic evidence for a neural crest syndrome. Am J Med Genet. Nov 1988;31(3):679-88. [Medline].

  12. Langer JC. Persistent obstructive symptoms after surgery for Hirschsprung's disease: development of a diagnostic and therapeutic algorithm. J Pediatr Surg. Oct 2004;39(10):1458-62. [Medline].

  13. Polley TZ, Coran, AG. Hirschsprung's disease in the newborn. Pediatric Surg. 1986;1:80-3.

  14. Puffenberger EG, Kauffman ER, Bolk S, et al. Identity-by-descent and association mapping of a recessive gene for Hirschsprung disease on human chromosome 13q22. Hum Mol Genet. Aug 1994;3(8):1217-25. [Medline].

  15. Reding R, de Ville de Goyet J, Gosseye S, et al. Hirschsprung's disease: a 20-year experience. J Pediatr Surg. Aug 1997;32(8):1221-5. [Medline].

  16. Roed-Petersen K, Erichsen G. The Danish pediatrician Harald Hirschsprung. Surg Gynecol Obstet. Feb 1988;166(2):181-5. [Medline].

  17. Swenson O, Sherman JO, Fisher JH. Diagnosis of congenital megacolon: An analysis of 501 patients. J Pediatr Surg. 1973;8:587-594. [Medline].

  18. Tiryaki T, Demirbag S, Atayurt H, Cetinkursun S. Topical nitric oxide treatment after pull through operations for Hirschsprung disease. J Pediatr Gastroenterol Nutr. Mar 2005;40(3):390-2. [Medline].

  19. Wartiovaara K, Salo M, Sariola H. Hirschsprung's disease genes and the development of the enteric nervous system. Ann Med. Feb 1998;30(1):66-74. [Medline].

  20. Yanchar NL, Soucy P. Long-term outcome after Hirschsprung's disease: patients' perspectives. J Pediatr Surg. Jul 1999;34(7):1152-60. [Medline].

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Further Reading

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Keywords

Hirschsprung disease, Hirschsprung's disease, Hirschsprung enterocolitis, Hirschsprung's enterocolitis, congenital aganglionosis, congenital megacolon, megacolon congenitum, Hirschsprung's disease, enterocolitis, abdominal distention, outflow incontinence, transmural intestinal necrosis, intestinal perforation, neonatal meconium plug syndrome, multiple endocrine neoplasia, MEN, Waardenburg syndrome, congenital deafness, malrotation, gastric diverticulum, intestinal atresia

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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.

Medical Editor

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.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

CME Editor

Steven M Schwarz, MD, FAAP, FACN, AGAF, Professor of Pediatrics, State University of New York, Downstate Medical Center College of Medicine; Distinguished Lecturer, New York Medical College, School of Public Health
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: TAP Pharmaceuticals Honoraria Speaking and teaching; Curemark, LLC Consulting fee Board membership

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.

 
 
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