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Published on: June 23, 2015
Autosomal Dominant Polycystic Kidney Disease: Role of Imaging in Diagnosis and Management
Devang Odedra1, Sandra Sabongui1, Korosh Khalili1
1From the Department of Medical Imaging, Toronto General Hospital, University Health Network, 200 Elizabeth St, Toronto, ON, Canada M5G 2C4 (D.O., K.K., S.K.); Department of Medical Imaging, North York General Hospital, North York, Ontario, Canada (D.O.); Temerty Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada (S.S.); Department of Medical Imaging, The Ottawa Hospital, University of Ottawa, Ottawa, Ontario, Canada (N.S.); and Division of Nephrology, University Health Network, Toronto, Ontario, Canada (Y.P.).
Insights
Autosomal dominant polycystic kidney disease (ADPKD) diagnosis relies on imaging and cyst counts. Larger kidney volumes (MCIC 1C-E) indicate high risk for kidney failure, benefiting from specific treatments.
Area of Science:
- Radiology and Medical Imaging
- Nephrology
- Genetics
Background:
- Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic kidney disorder, characterized by progressive renal function decline.
- Diagnosis is typically established via imaging, mainly ultrasound, using age-dependent criteria and cyst counts.
- The Mayo Clinic Imaging Classification (MCIC) system categorizes ADPKD into typical (Class 1) and atypical (Class 2).
Purpose of the Study:
- To highlight the role of imaging in diagnosing ADPKD, assessing disease severity, and predicting renal function decline.
- To emphasize the importance of height-adjusted total kidney volume (TKV) in predicting ADPKD progression.
- To discuss the radiologist's role in diagnosis, complication assessment, and therapeutic interventions.
Main Methods:
- Utilizing imaging techniques, primarily ultrasound, to diagnose ADPKD based on cyst presence and age-dependent criteria.
- Applying the Mayo Clinic Imaging Classification (MCIC) system, including subdivisions based on height-adjusted total kidney volume (TKV).
- Assessing complex cysts using subtraction imaging when malignancy is suspected.
Main Results:
- Height-adjusted TKV is a key predictor of future renal function decline and renal failure in ADPKD.
- Patients with larger height-adjusted TKV (MCIC 1C-E) face a high risk of end-stage renal disease.
- These high-risk patients may benefit from vasopressin receptor antagonists to slow cyst growth and preserve renal function.
Conclusions:
- Radiologists play a crucial role in ADPKD diagnosis, severity assessment, and predicting outcomes.
- Imaging, particularly height-adjusted TKV, is vital for risk stratification and guiding treatment decisions.
- Interventional radiology offers therapeutic options for ADPKD complications like cyst drainage and embolization.
Abstract:
Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic kidney disorder with progressive renal function decline, and disease severity is determined based on the type of genetic mutation. The diagnosis is usually established at imaging, primarily at US, and is based on age-dependent criteria and the number of visible cysts. ADPKD is classified into class 1 (typical) and class 2 (atypical) according to the Mayo Clinic Imaging Classification (MCIC) system. Height-adjusted total kidney volume (TKV) has emerged as a predictor of future renal function decline and renal failure in ADPKD, and several methods can be used for estimation. MCIC class 1 ADPKD is further subdivided into five types based on height-adjusted TKV (A, B, C, D, and E). Patients with a larger height-adjusted TKV (ie, MCIC 1C-E) are at high risk for progression to end-stage renal disease and will potentially benefit from vasopressin receptor antagonists, which have been shown to reduce the rate of cyst growth and slow renal function decline. Other renal complications primarily relate to hemorrhage within cysts or cyst infections. Subtraction images are key for assessment of complex cysts when malignancy is suspected, as the presence of protein and blood can limit the assessment for an enhancing component. The radiologist has a central role in establishing a diagnosis, excluding mimics, identifying complications, assessing severity, and predicting future renal failure. Interventional radiologists play a therapeutic role in management of complications by cyst drainage, sclerotherapy, or embolization. © RSNA, 2022 Online supplemental material and the slide presentation from the RSNA Annual Meeting are available for this article.
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