1. Joel Dean

2. A Brief Overview of

5. History Harald Hirschsprung, 1886 “Constipation in newborns due to dilation and hypertrophy of the colon” Orvar Swenson, 1948 First successful surgical treatment.

6. Epidemiology Incidence 1/4400 – 1/7000 live births >90% diagnosed in newborn period Racial distribution- similar to general population Male: Female Short segment; 4:1 Long segment; 1:1

9. Assessment for Hirschsprung disease in infants and children.

13. TestSensitivity (95% CI)Specificity (95% CI) Rectal suction biopsy93 percent100 percent (77 - 98 percent)(96 - 100 percent) Contrast enema76 percent97 percent (57 -89 percent)(91 - 99 percent) Anorectal manometry83 percent93 percent (63 - 93 percent)(85 - 97 percent) Comparison of sensitivity and specificity for rectal suction biopsy, contrast enema, and anorectal manometry in the diagnosis of Hirschsprung's disease in children Data from: De Lorijn, F, Reitsma, JB, Voskuijl, WP, et al. Diagnosis of Hirschsprung's disease: a prospective, comparative accuracy study of common tests. J Pediatr 2005; 146:787.

14. Swenson

15. Duhamel

16. Soave

18. Hirschsprung’s Associated Enterocolitis

19. Algorithm for the diagnosis and management of the child with obstructive symptoms after a pull- through From: Langer, JC. Persistent obstructive symptoms after surgery for Hirschsprung's Disease: Development of a diagnostic and therapeutic algorithm. J Pediatr Surg 2004; 39:1458.

20. QUESTIONS?