eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology
Hirschsprung Disease: Treatment & Medication
Updated: Nov 17, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
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
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Further Reading
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
Treatment & Medication: Hirschsprung Disease