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Published on: June 23, 2015
Co-occurrence of aortic dissection and polycystic kidney disease: a case report
Ganesh Raj1,2, Joseph Westaby3, Mary N Sheppard3
1CRY Cardiovascular Pathology Unit, Cardiovascular Clinical Academic Group, Molecular and Clinical Sciences Research Institute, St. George's, University of London, SW17 0RE, London, United Kingdom. ganesh5387@gmail.com.
Background:
Aortic dissection is a rare and often fatal condition which arises when there is aortic wall weakening. Multiple risk factors could lead to this condition, including hypertension, atherosclerosis, connective tissue diseases such as Marfan's, Ehlers-Danlos and Loeys Dietz syndromes, as well as smoking, cocaine usage, bicuspid aortic valve, pregnancy and trauma. Polycystic kidney disease (PKD) is a rare condition characterized by the development of multiple renal cysts, which is caused by genetic abnormalities leading to autosomal dominant or autosomal recessive inheritance. However, the presentation of both pathologies simultaneously is rare and has been infrequently reported.
Case Presentation:
We report a death attributed to the co-occurrence of both conditions, involving a 19-year-old Caucasian male with no known past medical history, who collapsed during sexual intercourse. Post-mortem examination revealed hemopericardium with an aortic dissection, which was associated with PKD. The heart and the great vessels were sent to the author's place of practice for a detailed cardiovascular pathology examination.
Conclusion:
This case is a reminder to all forensic and clinical pathologists that an extremely uncommon manifestation can occur; therefore, a meticulous approach should always be adopted before corroborating a cause of death, especially in young individuals. The relationship between both conditions in this case remains uncertain; however, PKD may represent an associated finding and possibly a predisposing factor rather than a direct causal mechanism. The co-occurrence of both pathologies raises the likelihood of an underlying predisposition to vascular pathology and may have implications for the screening and clinical evaluation of surviving blood relatives.
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