Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + • Distal colon aganglionosis resulting in dysfunctional myenteric plexus• Associated cardiac defects in 2-5%• Trisomy 21 in 5-15%• Occasionally preceded by neonatal appendicitis or meconium plug syndrome• 50% of cases diagnosed in neonates, almost 50% of cases diagnosed in children younger than 2, and rarely in older children or adults +++ Epidemiology + • 1/5000 births• 4:1 male:female ratio +++ Symptoms and Signs + • Failure to pass meconium in first 24-48 hours of life• Feeding intolerance• Abdominal distention• Bilious emesis• Chronic constipation• Fever (if entercolitis complicating)• Rectal spasm• Abdominal pain (if complicated by enterocolitis)• Diarrhea, occasionally bloody (if enterocolitis present) +++ Imaging Findings + • Abdominal x-ray-Shows intestinal obstruction (occasionally distal transition point visualized)-Pneumatosis intestinalis if enterocolitis present• Contrast enema shows transition zone distally (but may not be present in neonates)• Anorectal manometry shows failure of relaxation to balloon distention + • Causes of low intestinal obstruction in neonates include:-Rectal or colonic atresia-Meconium plug syndrome-Meconium ileus-Hypermagnesemia-Hypocalcemia-Hypokalemia-Hypothyroidism + • History and physical exam• Abdominal x-ray• Contrast enema• Suction rectal biopsy (85-90% sensitive) +++ Surgery + • Diverting colostomy for neonates with enterocolitis and Hirschsprung disease (colostomy must contain ganglia)• Duhamel, Soave, or Swenson procedure at age 9-12 months or once proximal dilation has subsided after diverting colostomy +++ Complications + • Anastomotic leak or stricture• Abscess• Obstruction• Enterocolitis (10-30%) +++ Prognosis + • 80-90% maintaining good bowel function +++ References ++Albanese CT et al: Perineal one-stage pull-through for Hirschsprungâs disease. J Pediatr Surg 1999;34:377. [PubMed: 10211635] ++Coran AG, Teitelbaum DH: Recent advances in the management of Hirschsprungâs disease. Am J Surg 2000;180:382. [PubMed: 11137692] ++Georgeson KE et al: Primary laparoscopic-assisted endorectal colon pull-through for Hirschsprungâs disease: a new gold standard. Ann Surg 1999;229:678. [PubMed: 10235526] ++Langer JC et al: One-stage versus two-stage Soave pull-through for Hirschsprungâs disease in the first year of life. J Pediatr Surg 1996;31:33. [PubMed: 8632283] Your MyAccess profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth