Congenital anomaly marked by enlargement of renal tubules, which can cause urinary stasis.
Abnormalities occur in the medullary pyramids of the kidney.
Autosomal dominant process.
Associated with dilatation of the collecting tubules in 1 or more renal pyramids in or both kidneys.
Patients can develop renal tubular acidosis but rarely have end stage renal disease.
Associated with several developmental anomalies suggesting disruption of the ureteral bud metamorphic blastema interface.
Patients can develop renal tubular acidosis but rarely have end stage renal disease.
Usually associated with normal renal function.
Patients may present with hematuria , infection or renal calculi.
Radiographically the cysts are located in the medulla.
Found in 0.5% of excretory autography exams.
Most cases are asymptomatic and is noted coincidentally on imaging studies.
Can come to clinical attention if complicated by infections, stone formation or hematuria.
While cases may be noted in siblings and families, most cases are sporadic.
Can occur in all age groups with no clear sexual predilection.
Estimated incidence of 1 per 5-10,000 population.
Morbidity higher in women.
Most patients live a normal life expectancy with only a few patients progressing to renal failure.