eMedicine Specialties > Gastroenterology > Colon

Hirschsprung Disease: Treatment & Medication

Author: Steven L Lee, MD, Chief, Pediatric Surgery, Department of Surgery, Kaiser-Permanente, Los Angeles Medical Center
Coauthor(s): Shant Shekherdimian, MD, Consulting Surgeon, Department of Surgery, Kaiser Foundation Hospital; Jeffrey J DuBois, MD,, Chief of Children's Surgical Services, Division of Pediatric Surgery, Kaiser Permanente, Women and Children's Center, Roseville Medical Center
Contributor Information and Disclosures

Updated: Dec 28, 2009

Treatment

Medical Care

The general goals of medical care are 3-fold: (1) to treat the complications of unrecognized or untreated Hirschsprung disease, (2) to institute temporary measures until definitive reconstructive surgery can take place, and (3) to manage bowel function after reconstructive surgery.

  • Management of complications of recognized aganglionosis is directed toward reestablishing normal fluid and electrolyte balance, preventing bowel overdistension (with possible perforation), and managing complications, such as sepsis. Thus, intravenous hydration, nasogastric decompression, and, as indicated, administration of intravenous antibiotics remain the cornerstones of initial medical management.
  • Because cardiac malformation (2-5%) and trisomy 21 (5-15%) are associated with congenital aganglionosis, cardiac evaluation and genetic testing may be warranted.
  • Colonic lavage, consisting of mechanical irrigation with a large-bore rectal tube and large volumes of irrigant, may be required.
  • Balanced salt solutions may help prevent electrolyte imbalances.
  • Nasogastric decompression, intravenous fluids, antibiotics, and colonic lavage may also need to be used in postoperative patients who develop enterocolitis as a complication. Sodium cromoglycate, a mast cell stabilizer, has been reported to be of benefit in these patients as well.25
  • Routine colonic irrigation and prophylactic antibiotic therapy have been proposed as a means of decreasing the risk of enterocolitis.26,27
  • Injecting the nonrelaxing internal sphincter mechanism with botulinum toxin (BOTOX®) has been shown to induce more normal patterns of bowel movements in postoperative patients with enterocolitis.

Surgical Care

Surgical management of Hirschsprung disease begins with the initial diagnosis, which often requires a full-thickness rectal biopsy. Traditionally, treatment also includes creating a diverting colostomy at the time of diagnosis, and, once the child grows and weighs more than 10 kg, the definitive repair is performed.

This standard of treatment was developed in the 1950s after reports of relatively high leak and stricture rates with the single stage procedure were initially described by Swenson. However, with the advent of safer anesthesia and more advanced hemodynamic monitoring, a primary pull-through procedure without a diverting colostomy is increasingly being performed. Contraindications to a one-stage procedure include massively dilated proximal bowel, severe enterocolitis, perforation, malnutrition, and inability to accurately determine the transition zone by frozen section.

For neonates who are first treated with a diverting colostomy, the transition zone is identified and the colostomy is placed proximal to this area. The presence of ganglion cells at the colostomy site must be unequivocally confirmed by a frozen-section biopsy. Either a loop or end stoma is appropriate, usually based on the surgeon's preference.

A number of definitive procedures have been used, all of which have demonstrated excellent results in experienced hands. The 3 most commonly performed repairs are the Swenson, Duhamel, and Soave procedures. Regardless of the pull-through procedure chosen, cleaning the colon before definitive repair is necessary.

  • Swenson procedure
    • The Swenson procedure was the original pull-through procedure used to treat Hirschsprung disease.
    • The aganglionic segment is resected down to the sigmoid colon and the remaining rectum, and an oblique anastomosis is performed between the normal colon and the low rectum.
  • Duhamel procedure
    • The Duhamel procedure was first described in 1956 as a modification to the Swenson procedure.
    • Key points are that a retrorectal approach is used and a significant portion of aganglionic rectum is retained.
    • The aganglionic bowel is resected down to the rectum, and the rectum is oversewn. The proximal bowel is then brought through the retrorectal space (between the rectum and sacrum), and an end-to-side anastomosis is performed on the remaining rectum.
  • Soave (endorectal) procedure
    • The Soave procedure was introduced in the 1960s and consists of removing the mucosa and submucosa of the rectum and pulling the ganglionic bowel through the aganglionic muscular cuff of the rectum.
    • The original operation did not include a formal anastomosis, relying on scar tissue formation between the pull-through segment and the surrounding aganglionic bowel. The procedure has since been modified by Boley to include a primary anastomosis at the anus.
  • Anorectal myomectomy
    • For children (and occasionally adults) with ultrashort-segment Hirschsprung disease, removing a strip of posterior midline rectal wall is an alternative surgical option.
    • The procedure removes a 1-cm wide strip of extramucosal rectal wall beginning immediately proximal to the dentate line and extending to the normal ganglionic rectum proximally.
    • The mucosa and submucosa are preserved and closed.
  • Procedures for long-segment Hirschsprung disease
    • Patients with total colonic involvement require modified procedures to bypass the aganglionic colon yet preserve the absorptive surface area and allow for proper growth and nutritional support.
    • Most procedures include a side-to-side anastomosis of the ganglionic/propulsive small bowel to a short segment of the aganglionic/absorptive colon.
    • Whether a short right colonic patch or a small bowel-to-rectal wall Duhamel anastomosis is created is perhaps less important than maintaining a short patch length (<10 cm).
    • Long-segment anastomoses, such as the Martin procedure, are no longer advocated.
  • A laparoscopic approach to the surgical treatment of Hirschsprung disease was first described in 1999 by Georgeson.28 The transition zone is first identified laparoscopically, followed by mobilization of the rectum below the peritoneal reflection. A transanal mucosal dissection is performed, followed by prolapsing of the rectum through the anus and anastomosis. Functional outcomes appear to be equivalent to open techniques based on short-term results.28,29,30
  • Transanal pull-through in which no intra-abdominal dissection is performed has also been described.14,31 The entire procedure is performed from below in a manner similar to perineal rectosigmoidectomy. The transition zone is identified and anastomosis is performed. Similar to the laparoscopic approach, outcomes have been similar to open single stage approaches with the benefits of minimal analgesia and shortened hospital stays.14,32,33
  • However, a retrospective study among 41 patients, comparing the transanal pull-through approach to the transabdominal approach, demonstrated a significantly higher rate of incontinence with the transanal approach.34 Whether or not these findings will be reproducible in larger, prospective trials remains to be determined.
  • The possibility of stem cell transplantation into the aganglionic gut and the reactivation of dormant stem cells in the gut to regenerate the enteric nervous system are being actively investigated.35 Experiments have demonstrated that neural crest stem cells (NCSC) are present, even in the adult gut, and are capable of proliferation and differentiation. In addition, researchers have been able to inject neural crest stem cells and later identify them in the native rectum. Whether or not injected stem cells or reactivated native progenitor cells will have the capability to recreate a functional enteric nervous system remains to be elucidated.

Consultations

  • Pediatric surgeons
  • Pediatric gastroenterologists
  • Geneticists (if trisomy 21 is present)

Diet

  • The patient should have nothing by mouth before the operation.
  • Institute tube feeding or formula/breast milk once bowel function resumes.
  • High-fiber diets and diets containing fresh fruits and vegetables may optimize postoperative bowel function in certain patients.

Activity

Limit physical activity for about 6 weeks to allow the wound to heal properly (applies more to older children).

Medication

The goals of pharmacotherapy are to eradicate infection, to reduce morbidity, and to prevent complications.

Antibiotics

Empiric antimicrobial therapy must be comprehensive and cover all likely pathogens in the context of this clinical setting. Antibiotic selection should be guided by blood culture sensitivity whenever feasible.


Ampicillin (Marcillin, Omnipen, Principen)

Bactericidal activity against susceptible organisms. Alternative to amoxicillin when unable to take medication orally.

Adult

1-2 g IV q6h

Pediatric

25 mg/kg IV q6h

Probenecid and disulfiram elevate levels; allopurinol decreases effects and has additive effects on ampicillin rash; may decrease effects of oral contraceptives

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Adjust dose in renal failure; evaluate rash and differentiate from hypersensitivity reaction


Gentamicin (Garamycin, Jenamicin)

Aminoglycoside antibiotic for gram-negative coverage. Used in combination with both an agent against gram-positive organisms and one that covers anaerobes. Not the DOC. Consider if penicillins or other less-toxic drugs are contraindicated, when clinically indicated, and in mixed infections caused by susceptible staphylococci and gram-negative organisms.
Dosing regimens are numerous; adjust dose based on CrCl and changes in volume of distribution. May be administered IV/IM.

Adult

5-7 mg/kg/d IV

Pediatric

2.5 mg/kg IV q8h; check peak and trough levels after third dose

Coadministration with other aminoglycosides, cephalosporins, penicillins, and amphotericin B may increase nephrotoxicity; aminoglycosides enhance effects of neuromuscular blocking agents; thus, prolonged respiratory depression may occur; coadministration with loop diuretics may increase auditory toxicity of aminoglycosides; possible irreversible hearing loss of varying degrees may occur (monitor regularly)

Documented hypersensitivity; non–dialysis-dependent renal insufficiency

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Narrow therapeutic index (not intended for long-term therapy); caution in renal failure (not on dialysis), myasthenia gravis, hypocalcemia, and conditions that depress neuromuscular transmission; adjust dose in renal impairment


Metronidazole (Flagyl)

Imidazole ring-based antibiotic active against various anaerobic bacteria and protozoa. Used in combination with other antimicrobial agents (except for Clostridium difficile enterocolitis).

Adult

500 mg PO/IV q6-8h

Pediatric

7.5 mg/kg IV q6h

May increase toxicity of anticoagulants, lithium, and phenytoin; cimetidine may increase toxicity; disulfiramlike reaction may occur with orally ingested ethanol

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Adjust dose in hepatic disease; monitor for seizures and development of peripheral neuropathy

Toxins

Induce more normal patterns of bowel movements in postoperative patients with enterocolitis.


Botulinum toxin type A (BOTOX®)

Binds to receptor sites on motor nerve terminals and inhibits release of acetylcholine, which in turn inhibits transmission of impulses in neuromuscular tissue.

Adult

1.25-2.5 U IM; may repeat q3-4mo

Pediatric

>12 years: Administer as in adults
<12 years: 0.25-1 U IM; may repeat q3-4mo

Aminoglycosides or drugs that interfere with neuromuscular transmission may potentiate effects

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Do not exceed recommended dose and frequency of administration; presence of antibodies to botulinum toxin type A may reduce effects of therapy

More on Hirschsprung Disease

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

Related eMedicine Topics

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 Clinical Guidelines

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Keywords

Hirschsprung disease, Hirschsprung's disease, megacolon, colonic aganglionosis, ultrashort-segment Hirschsprung disease, congenital aganglionosis, aganglionic megacolon, acquired megacolon, congenital dilation of the colon, congenital megacolon, aganglionic megacolon, meconium, anorectal reflex, chronic constipation, enterocolitis, rectosigmoid colon aganglionosis, total colonic aganglionosis,
 
trisomy 21, Down syndrome, bowel obstruction, bilious vomiting, abdominal distention, poor feeding, failure to thrive, myenteric plexus, Auerbach plexus, submucosal plexus, Meissner plexus, colonic lavage, full-thickness rectal biopsy, simple suction rectal biopsy, anorectal manometry, diverting colostomy, Swenson procedure, Duhamel procedure, Soave procedure, anorectal myomectomy

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Contributor Information and Disclosures

Author

Steven L Lee, MD, Chief, Pediatric Surgery, Department of Surgery, Kaiser-Permanente, Los Angeles Medical Center
Steven L Lee, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Association for Academic Surgery, Society of American Gastrointestinal and Endoscopic Surgeons, and Society of Laparoendoscopic Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Shant Shekherdimian, MD, Consulting Surgeon, Department of Surgery, Kaiser Foundation Hospital
Disclosure: Nothing to disclose.

Jeffrey J DuBois, MD,, Chief of Children's Surgical Services, Division of Pediatric Surgery, Kaiser Permanente, Women and Children's Center, Roseville Medical Center
Jeffrey J DuBois, MD, is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, and California Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Vivek V Gumaste, MD, Associate Professor of Medicine, Mt Sinai School of Medicine; Adjunct Clinical Assistant, Mt Sinai Hospital; Director, Division of Gastroenterology, City Hospital Center
Vivek V Gumaste, MD is a member of the following medical societies: American College of Gastroenterology and American Gastroenterological Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Oscar S Brann, MD, FACP, Associate Clinical Professor, Department of Medicine, University of California at San Diego; Consulting Staff, Mecklenburg Medical Group
Oscar S Brann, MD, FACP is a member of the following medical societies: American Gastroenterological Association
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law Medicine and Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

 
 
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