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prevent chronic renal failure in patient with an anorectal malformation

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[size=20px]A newborn infant boy has imperforate anus. He is twelve hours old. He has hypospadias and a flat rocker bottom. Meconium was noted in the urine suggesting a urinary tract fistula or high lesion.

 

Which of the following will best help to prevent chronic renal failure in this patient with an anorectal malformation?

 

A early division of the rectourinary fistula

 

B avoiding urinary tract procedures during colostomy

 

C complete work up of the genitourinary tract with long term follow-up

 

D empiric use of prophylactic antibiotics

 

E posterior sagittal anorectoplasty[/size]

c

correct

 

It is commonly recognized that genitourinary (GU) anomalies are frequently associated with anorectal malformations (ARM). The overall incidence may be anywhere from 25 to 60%, but is higher in association with high ARMs. Moreover, the severity of these genitourinary anomalies is also more notable with high anorectal malformations. A recent review by Giuliani reinforces these observations made by McLorie. Genitourinary anomalies such as dysplasia and aplasia associated with high ARMs also result in a higher likelihood of end stage renal disease (ESRD) and death (6.4%) when compared to those GU anomalies associated with low ARM (1.1%).

 

Conditions leading to ESRD disease include obstructive uropathy, vesicoureteric reflux, neurovesical dysfunction and recurrent urinary tract infections. A posterior sagittal anorectoplasty repair itself has no bearing on renal dysfunction. Giuliani recommend that renal and bladder evaluation with ultrasound and possibly cystogram should be performed prior to or at the time of colostomy to determine if there is any need for concomitant urologic procedures at the time of colostomy when a high ARM is suspected or determined. At the very least, awareness of these GU anomalies can inform appropriate renal management as the patient progresses toward definitive anorectal repair.

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