Patients with anorectal malformations not infrequently have associated urologic problems. Each patient needs a complete urologic evaluation, and any identified issues such as a single kidney, hydronephrosis, vesicoureteral reflux or neurogenic bladder need to be followed closely. If this does not occur, some children with anorectal malformations can develop permanent renal injury. Some patients had poor kidneys to begin with but many were initially told that they had one or two good kidneys. Unfortunately, their kidneys deteriorated over time, often silently without obvious symptoms.
There are many potential reasons for kidneys to be injured. The most common is bladder dysfunction. Other causes include infection, reflux and obstruction. Kidneys that are injured from birth or that have become injured over time may support renal function quite well until the child begins to grow. As the child grows, the metabolism of the body may overwhelm the kidneys’ ability to keep up with demand. Symptoms of renal failure or abnormal findings on simple screening blood testing may not become obvious until this time. Unfortunately, it is often too late to reverse the process and preserve good renal function when caught at this late date.
Fortunately, much of this renal injury can be prevented if the process is caught and treated early. Every patient with an anorectal malformation should know the status of their kidneys. Patients at highest risk include those with high anorectal malformations (rectobladderneck and rectoprostatic fistula and cloacas) patients who have tethered cord and patients who have urinary infection or voiding abnormalities such as incontinence or retention. Such patients should have a complete evaluation to exclude neurogenic bladder. These patients should be followed at regular intervals by ultrasound at a minimum. Other testing may be required based on the outcome of the initial complete evaluation, symptoms, and follow-up ultrasound appearance as well as often urodynamics and bloodwork (renal panel and Cystatin C). The testing required is available almost anywhere and can be accessed through most pediatricians and pediatric urologists.