1-Year-Old Male Patient with Anorectal Malformation and Recto-urinary Fistula

• 1-year male patient presents for evaluation.
• Born with anorectal malformation suspected to be recto-urinary fistula.
• Colostomy was placed on day of life 2.
• Anorectoplasty was performed at 7 mo.
• Referred to colorectal clinic because of recurrent history of orchiepididymitis and febrile urinary tract infections with pseudomonas.
• On physical exam, anus was noted to be patent but located outside of the muscle complex.
• Sacral index was 0.74 on anterior posterior view and 0.58 on lateral view X-rays of the sacrum.
• Renal ultrasound showed grade II right hydronephrosis.
• Right ureter dilation at the UV junction was noted.
• Cystogram was performed with 5-fr catheter but this was difficult to pass at the level of the bulbar urethra.
• Grade IV right vesicoureteral reflux was noted.
• Patient unable to void after the catheter was removed.
• Catheter was reinserted and left in place.
• Renal scintigraphy was performed showing GFR of 68 ml/min.
• Right kidney was hypoperfused and hypofunctional, with only had a GFR of 12 ml/min.
• Spinal MRI did not show tethered cord.
• Reoperation for posterior sagittal anorectoplasty to place the rectum into the muscle complex and a vesicostomy was performed.
• After the repair, patient received dilations to goal Hegar size of 15.
• Cystogram will be performed through the vesicostomy.
• If patient has persistent vesicouretreal reflux, a ureteral reimplantation may be required.

Takeaways

• When deciding if a reoperation should be performed to move the anus to the muscle complex, the likelihood of bowel control should be considered by evaluating sacral index, anatomy of urinary tract and evaluation for tethered cord.
• A minor mislocation of the anus will not likely have impact on bowel control so reoperation may not be indicated.
• For patients presenting with acute, erythematous scrotum with history of anorectal malformation, orchiepididymitis should be suspected. For extreme recurrent cases of orchiepididymitis, a contrast study of the vas deferens should be performed. This involves passing a needle into the vas deferens and injecting contrast to evaluate if the vas deferens is connect to the ureter or to the bladder and receiving reflux of contaminated urine. In that circumstance, a vasectomy could be performed.