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Paediatrics: Anorectal malformations

Paediatrics: Anorectal malformations
The incidence of anorectal malformation is 1/5000 live births.

Anorectal malformations

 

The incidence of anorectal malformation is 1/5000 live births. Anorectal malformations comprise part of the VACTERL association. The abnormality should be identified at birth. The baby presents with:

   failure to pass meconium;

 

   abdominal distension;

 

   bile-stained vomiting.

 

Anatomy

 

The precise anatomy varies but the malformation can be subdivided into high and low/intermediate anomalies in males and females.

 

Low/intermediate anomalies

 

   Rectum is present and passes through a normal sphincter complex.

 

   In boys (Fig. 23.17) there is a tiny fistulous track to the surface of the perineum, often anteriorly on to the scrotum. If meconium is visible on the perineum a local ‘cut-back’ procedure can be performed to open the fistula back to the rectum in anticipation of normal continence.

 

   In girls (Fig. 23.18) the rectum usually opens into the back of the introitus as a rectovestibular fistula. This abnormality is termed intermediate because, although normal continence is to be expected, reconstruction involves division of a common wall between rectum and vagina. For this reason treatment involves a 3-stage procedure with defunctioning colostomy, anorectal reconstruction, and then closure of the stoma.

 

   Vesicoureteric reflux is very common.

 

High anomalies

 

   These anomalies are rare in girls but common in boys (Fig. 23.17).

 

   The sphincter complex is poorly developed and the prospects for continence are mediocre.

In boys the rectum makes a fistulous connection with the urethra. Treatment involves a defunctioning colostomy within the first 48hr of birth, reconstruction at a few months of age (most commonly involving a posterior sagittal anorectoplasty performed through a midline perineal incision), and then closure of the colostomy.

 

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