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  • High imperforate anus: Above the striated muscle complex or levator ani

    Low imperforate anus: Rectal pouch descending into striated muscle complex and therefore have more favorable prognosis following reconstruction

    • Male patients most commonly have low imperforate anus with a perineal fistula or high anorectal agenesis with a rectoprostatic urethral fistula

    • Female patients most commonly have low imperforate anus and fistula from the blind-ending rectal pouch to perineal body or vaginal vestibule

    • 70% associated with other abnormalities such as the VACTERL abnormalities

    • -Vertebral abnormalities

      -Anal atresia

      -Cardiac abnormalities

      -Tracheoesophageal fistula and/or esophageal atresia

      -Renal agenesis and dysplasia

      -Limb (defects)

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Epidemiology

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  • • 1/2500-1/5000 births

    • High imperforate anus 2-fold more common in males

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Symptoms and Signs

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  • • Distended abdomen

    • Bilious emesis

    • Irritability

    • Failure to pass meconium

    • Fecaluria (if rectovesicular or rectourethral fistula)

    High imperforate anus: Absence of dimple or absence of gluteal fold

    Low imperforate anus:

    • -Presence of anal membrane, dimple, or fold (usually)

      -External fistula to perineum or vestibule

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Imaging Findings

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  • Plain lateral pelvic film: Shows gas filled rectal stump and relative location

    Pelvic US: Shows relation of rectal stump to striated muscle complex

    CT or MRI: Delineates the relation between the rectal stump and the striated muscle complex

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  • • Differentiating between low and high imperforate anus

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  • • History and physical exam

    • Abdominal pelvic x-ray

    • Pelvic US

    • Voiding cystourethrogram

    • Echocardiogram

    • Spinal MRI or US

    • Renal US

    • Upper GI

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Surgery

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  • Low imperforate anus: Cutback anoplasty (circumferential mobilization of anterior fistula and transposition to center of external anal sphincter)

    High imperforate anus: Diverting colostomy until at least 12 months of age followed by (most commonly) posterior sagittal anorectoplasty and closure of rectourinary fistula

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Complications

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  • • Leak or stricture, 5-10%

    • 30-80% have minimal problems with continence, remainder with significant problems with continence

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Prognosis

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  • • 15-20% mortality secondary to other abnormalities

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References

Albanese CT et al: One-stage repair of high imperforate anus in the newborn male. J Pediatr Surg 1999;34:834.  [PubMed: 10359190]
Georgeson KE, Inge TH, Albanese CT: Laparoscopically assisted anorectal pull-through for high imperforate anus—a new technique. J Pediatr Surg 2000;35:927.  [PubMed: 10873037]
Hendren WH: Management of cloacal malformations. Semin Pediatr Surg 1997;6:217.  [PubMed: 9368273]
Pena A, Hong A: Advances in the management of anorectal malformations. Am J Surg 2000;180:370.  [PubMed: 11137690]

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