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  • • 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

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Epidemiology

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  • • 1/5000 births

    • 4:1 male:female ratio

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Symptoms and Signs

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  • • 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)

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Imaging Findings

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  • 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

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  • • Causes of low intestinal obstruction in neonates include:

    • -Rectal or colonic atresia

      -Meconium plug syndrome

      -Meconium ileus

      -Hypermagnesemia

      -Hypocalcemia

      -Hypokalemia

      -Hypothyroidism

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  • • History and physical exam

    • Abdominal x-ray

    • Contrast enema

    • Suction rectal biopsy (85-90% sensitive)

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Surgery

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  • • 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

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Complications

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  • • Anastomotic leak or stricture

    • Abscess

    • Obstruction

    • Enterocolitis (10-30%)

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Prognosis

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  • • 80-90% maintaining good bowel function

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References

Albanese CT et al: Perineal one-stage pull-through for Hirschsprung’s disease. J Pediatr Surg 1999;34:377.  [PubMed: 10211635]
Coran AG, Teitelbaum DH: Recent advances in the management of Hirschsprung’s disease. Am J Surg 2000;180:382.  [PubMed: 11137692]
Georgeson KE et al: Primary laparoscopic-assisted endorectal colon pull-through for Hirschsprung’s 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 Hirschsprung’s disease in the first year of life. J Pediatr Surg 1996;31:33.  [PubMed: 8632283]

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