Skip to main content

Congenital Megacolon (Hirschsprung Disease)

John K. Uffman, MD, MPH Reviewed 06/2018
 


BASICS

DESCRIPTION

  • A disease of the colon characterized by functional obstruction, fecal accumulation, and colonic dilation:

    • 74–80% of cases involve rectosigmoid colon, 12–22% involve long segment of th...

DIAGNOSIS

HISTORY

  • Onset early in infancy; newborn fails to pass meconium in 24 to 48 hours after birth (60–90% of infants with Hirschsprung disease).

  • Obstipation

  • May have diarrhea (usually intermittent)

  • C...

TREATMENT

GENERAL MEASURES

  • Treatment may be symptomatic or definitive.

  • Correct fluid and electrolyte imbalances.

  • Abdominal decompression with a nasogastric tube

  • Removal of fecal accumulation: retention en...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Postsurgical monitoring for fluid/electrolyte balance, protein-calorie nutrition, and growth

  • Long-term monitoring for continence and constipation

DIET

REFERENCES

1
Haricharan RN, Georgeson KE. Hirschsprung disease. Semin Pediatr Surg.  2008;17(4):266–275. {L-End} [View Abstract on OvidMedline]
2
Langer JC. Hirschsprung disease. Curr Opin Pediatr....

ADDITIONAL READING

  • De La Torre L, Langer JC. Transanal endorectal pull-through for Hirschsprung disease: technique, controversies, pearls, pitfalls, and an organized approach to the management of post...

SEE ALSO

Constipation 

CODES

ICD10

Q43.1 Hirschsprung's disease 

ICD9

751.3 Hirschsprung's disease and other congenital functional disorders of colon 

SNOMED

  • 204739008 Hirschsprung's disease (disorder)

  • 24291004 Congenital dilatat...

CLINICAL PEARLS

  • All infants with primary constipation must be evaluated for Hirschsprung disease.

  • Early diagnosis helps prevent toxic enterocolitis.

  • An unprepped barium enema followed by a suction recta...

Subscribe to Access Full Content

Sign Up for a 10-Day Free Trial

Sign up for a 10-day FREE Trial now and receive full access to all content.

×