Hirschsprung's disease medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aditya Ganti M.B.B.S. [2]
Overview
Medical therapy only plays a supportive role in the management of Hirschsprung's disease. Medical therapy indications include preventing complications of Hirschsprung's disease, preventing infections during reconstructive surgery, and managing postoperative bowel function. Intravenous fluid resuscitation and maintenance, nasogastric decompression, and administration of intravenous antibiotics (as indicated) remain the cornerstones of initial medical management.[1][2]
Medical Therapy
Supportive therapy
- All patients should be kept nothing by mouth (NPO).
- Patients should receive intravenous fluids.
- A large tube (20-24 F) is introduced through the rectum, and small amounts of saline solution (10-20 mL) are passed through the lumen of the tube in order to clear it.
- The liquid rectal and colonic content is expected to drain through the lumen of the tube. The tube is then rotated in different directions and moved back and forth.
- The operator continues to instill small amounts of saline solution, allowing the evacuation of gas and liquid stool through the tube.
- Metronidazole is usually given. In older children, oral antibiotics can be given when they begin to improve and when they are no longer NPO.
Empiric antimicrobial therapy
- Preferred regimen (1): Ampicillin (Marcillin, Omnipen, Principen) 1-2 g IV or IM q4-6h, or 50-250 mg/kg/day divided q4-6hr, not to exceed 12 g/day.
- Preferred regimen (2): Gentamicin (Garamycin, Jenamicin) 3-5 mg/kg/day IV or IM divided q8h.
- Preferred regimen (3): Metronidazole (children) 30 mg/kg/day PO or IV divided q6h, maximum 4000 mg/day.
References
- ↑ Langer JC, Rollins MD, Levitt M, Gosain A, Torre L, Kapur RP, Cowles RA, Horton J, Rothstein DH, Goldstein AM (2017). "Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease". Pediatr. Surg. Int. 33 (5): 523–526. doi:10.1007/s00383-017-4066-7. PMID 28180937.
- ↑ Burkardt DD, Graham JM, Short SS, Frykman PK (2014). "Advances in Hirschsprung disease genetics and treatment strategies: an update for the primary care pediatrician". Clin Pediatr (Phila). 53 (1): 71–81. doi:10.1177/0009922813500846. PMID 24002048.