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[Pathological study of Willis's circle in autopsy cases of polycystic kidney disease]
1Division of Neurosurgery, Tohoku University School of Medicine, Sendai, Japan.
Insights
Polycystic kidney disease (PKD) is linked to vascular abnormalities, particularly intracranial aneurysms. Pathological findings reveal defects in the internal elastic lamina of the Circle of Willis, increasing aneurysm risk in PKD patients.
Area of Science:
- Pathology
- Nephrology
- Neurology
Background:
- Polycystic kidney disease (PKD) is a genetic disorder characterized by numerous cysts in the kidneys.
- Patients with PKD have a higher incidence of cerebrovascular abnormalities, including intracranial aneurysms.
- The underlying vascular pathology in PKD patients with intracranial aneurysms requires further investigation.
Observation:
- Autopsy examination of two patients with polycystic kidney disease and intracranial aneurysms.
- Detailed pathological study of the vascular system, focusing on the Circle of Willis.
- Histological analysis revealed abrupt interruption and partial disappearance of the internal elastic lamina in the Circle of Willis.
Findings:
- Consistent abnormalities in the internal elastic lamina of the Circle of Willis were observed in both cases.
- These vascular defects were frequently found in the walls of the Circle of Willis.
- The identified histological abnormalities suggest an inherent vascular system abnormality in polycystic kidney disease.
Implications:
- The findings suggest a congenital predisposition to vascular defects in patients with polycystic kidney disease.
- Abnormalities in the internal elastic lamina may contribute to the increased frequency of intracranial aneurysms in PKD.
- This study highlights the importance of considering cerebrovascular screening in individuals with polycystic kidney disease.
Abstract:
Two autopsy cases of polycystic kidney disease with intracranial aneurysms were reported. We made a pathological study of their vascular system with special reference to Willis's circle in order to prove the inherent abnormality of vascular system in patients with polycystic kidney disease. A 57-year-old man was revealed to have polycystic kidney disease with left middle cerebral artery aneurysm and aortic stenosis. The other 58-year-old man was revealed to have polycystic kidney disease with cerebral aneurysms both in anterior communicating artery and basilar artery, aortic dissecting aneurysm, cerebral infarction and myocardial infarction. Histologically, both abrupt interruption and partial disappearance of internal elastic lamina are found frequently in the wall of Willis's circle. This abnormality of internal elastic lamina may be attributable to congenital factors and rise the frequency of occurrence of intracranial aneurysms in polycystic kidney disease.