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Intracranial cysts in autosomal dominant polycystic kidney disease
W I Schievink1, J Huston, V E Torres
1Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, USA.
Insights
Autosomal dominant polycystic kidney disease (ADPKD) is linked to a higher incidence of intracranial arachnoid cysts. These cysts are typically asymptomatic and do not require treatment in ADPKD patients.
Area of Science:
- Nephrology
- Neurology
- Radiology
Background:
- Autosomal dominant polycystic kidney disease (ADPKD) is a systemic genetic disorder.
- ADPKD is known for its association with intracranial aneurysms.
- The occurrence of other intracranial pathologies in ADPKD patients warrants further investigation.
Purpose of the Study:
- To investigate the prevalence of intracranial arachnoid cysts in patients with ADPKD.
- To compare the incidence of these cysts in ADPKD patients versus a control group.
- To assess the clinical significance and management of intracranial arachnoid cysts in ADPKD.
Main Methods:
- Retrospective review of 247 ADPKD patients who underwent neuroimaging (MRI or CT).
- Comparison with a control group matched for age, sex, and imaging modality.
- Analysis of co-occurring conditions, including polycystic liver disease.
Main Results:
- Intracranial arachnoid cysts were found in 8.1% of ADPKD patients, significantly higher than 0.8% in controls (p < 0.0001).
- Polycystic liver disease was more common in ADPKD patients with arachnoid cysts (85.0% vs 52.4%, p < 0.004).
- No intracranial cysts were symptomatic, and none required surgical intervention.
Conclusions:
- Intracranial arachnoid cysts are a frequent incidental finding in ADPKD patients.
- This finding supports the systemic nature of ADPKD.
- Asymptomatic intracranial arachnoid cysts in ADPKD patients generally do not require treatment.
Abstract:
Autosomal dominant polycystic kidney disease (ADPKD) is a systemic disorder well known for its association with intracranial aneurysms. A series of patients with ADPKD who were screened for the presence of an intracranial aneurysm were reviewed and found to include an unexpectedly high number with intracranial arachnoid cysts. Among 247 patients with ADPKD who underwent magnetic resonance imaging (180 cases) or high-resolution contrast-enhanced computerized tomography (67 cases), there were 151 women and 96 men with a mean age of 44 years. Intracranial arachnoid cysts were found in 20 patients (8.1%) with ADPKD compared to two (0.8%) in a control group without ADPKD matched for age, sex, and method of imaging (p < 0.0001). Multiple intracranial arachnoid cysts were found in two patients. Polycystic liver disease was present in 17 (85.0%) of the 20 patients with intracranial arachnoid cysts compared to 119 (52.4%) of the 227 patients without (p < 0.004). Pineal cysts were found in two patients (0.8%) and choroid plexus cysts were found in three patients (1.2%) but this was not different from the control population. None of the intracranial cysts was symptomatic and none was treated surgically. Intracranial arachnoid cysts are a relatively frequent incidental finding in patients with ADPKD, providing further support for the systemic nature of this disease. In the authors' experience with approximately 1500 patients with ADPKD, no complication has been encountered from an intracranial arachnoid cyst, suggesting that asymptomatic intracranial arachnoid cysts in patients with ADPKD require no treatment.