Cystic Diseases of the Kidney Follow-up

Updated: Mar 28, 2017
  • Author: Thomas Patrick Frye, DO; Chief Editor: Bradley Fields Schwartz, DO, FACS  more...
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Follow-up

Further Outpatient Care

Patients with multicystic dysplastic kidney should be observed with periodic sonography to monitor for neoplastic changes.

Patients with autosomal dominant polycystic kidney disease (ADPKD) should be screened for intracranial saccular aneurysms with magnetic resonance angiography (MRA) or another imaging modality.

In patients with medullary sponge kidney (MSK), regular follow-up care with sonography and urinalysis is recommended to monitor for calculi or infection.

In patients with simple, intermediate, and suspicious renal cysts, if the CT identification of a simple cyst is equivocal, observe the cyst with repeat scans. For Bosniak category IIF lesions, perform contrast-enhanced renal CT scan studies in 6 months and annually thereafter for at least 5 years.

Some experts suggest that patients with Bosniak category II lesions require no follow-up. However, the data are confusing because reported larger cases series did not separate category IIF from category II lesions. Thus, these series report malignancy rates of up to 14% for category II lesions. Given these rates, some physicians also choose to follow up with patients with category II lesions (treating them as IIF lesions).

Patients with tuberous sclerosis (TS) should be screened with periodic CT scan or sonography to monitor for carcinoma development. A noncystic mass that lacks the fat of a typical angiomyolipoma or an enlarging cyst may suggest carcinoma.

In patients with von Hippel-Lindau syndrome (VHLS), perform renal sonography annually to monitor cyst or other mass development. Biochemical pheochromocytoma screening starting at age 5 with annual lifelong screening. [25]  In patients with multiple cysts, perform CT scan or MRI every 1-3 years to monitor for RCC.

Acquired cystic renal disease

The value of screening patients with chronic kidney disease for the development of acquired renal cystic disease (ARCD) is debated. Some advocate screening because of the associated risk of RCC. Decision analyses have demonstrated that screening is valuable only in patients with a life expectancy of more than 25 years. Thus, in the United States, screening is limited to patients who have been on dialysis for more than 5 years, have extended expected survival, and are showing signs of ARCD. [17]

Ultrasonography or CT scan should be used for initial screening and repeated every 1-2 years thereafter. In patients with known ARCD, contrast-enhanced CT scan can be performed annually to screen for carcinoma. This screening may be most valuable in younger patients and in patients with large cysts.

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Prognosis

Bilateral multicystic dysplastic kidney (MCDK) is incompatible with life. More typically, the disease is unilateral or segmental and is discovered on prenatal sonogram.

In autosomal dominant polycystic kidney disease (ADPKD), renal insufficiency typically develops in people older than 30 years, and 50% of patients progress to end-stage renal failure by the age of 60 years. One third of patients die secondary to renal failure, one third die due to complications from hypertensive nephropathy, and 6-10% die secondary to subarachnoid hemorrhage.

Neonates presenting with autosomal recessive polycystic kidney disease (ARPKD) often die within 6 weeks secondary to pulmonary disease and renal failure. If patients survive this first period, they have an 80% chance of living to 15 years. For patients presenting in infancy, approximately one third progress to severe renal insufficiency at age 5 years and nearly 100% progress by age 20 years.

In juvenile nephronophthisis (JNPHP) and medullary cystic kidney disease (MCKD), patients typically progress to renal failure within 5-10 years of presentation. In von Hippel-Lindau syndrome, as many as 40% of patients develop renal cell carcinoma, which is the leading cause of death.

Acquired cystic renal disease is progressive while the patient remains on dialysis. The disease often regresses after transplantation, but associated tumors may become more aggressive because of the patient's immunosuppression. [17]

Medullary sponge kidney carries an excellent prognosis and is typically nonprogressive.

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Patient Education

Genetic counseling is important in all of the heritable cystic renal diseases. Individuals with autosomal dominantly inherited diseases (eg, ADPKD) need to be counseled that their offspring have a 50% chance of developing the disease. Those with autosomal recessively inherited diseases (eg, ARPKD) should be counseled that all of their offspring will carry the disease. Parents of children with an autosomal recessively inherited disease should be counseled that subsequent children have a 25% chance of having the disease and a 50% chance of being an unaffected carrier of the gene.

For patient education information, see Blood in the Urine.

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