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Forensic Medicine · Thanatology: sudden death in Marfan syndrome
Sample itemA tall 24-year-old man with long limbs and arachnodactyly collapses with tearing chest pain and dies. Autopsy shows haemopericardium from an intimal tear in the ascending aorta. Which histological change underlies the lesion?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
D. Cystic medial degeneration of the aortic wall
Marfan syndrome weakens the aortic media through cystic medial degeneration with loss of elastic fibres. This predisposes to dissection and rupture into the pericardium causing tamponade. Look for a young tall person with sudden chest pain.
Option by option
A. Atheromatous plaque rupture
Atheroma is typical of older hypertensive patients and is not the lesion in a young man with a connective tissue disorder.
B. Giant cell infiltration of the media
Giant cell arteritis affects older people and does not cause dissection of the ascending aorta here.
C. Obliterative endarteritis of the vasa vasorum from syphilis
Syphilitic aortitis causes aneurysm in the tertiary stage and does not fit this patient.
D. Cystic medial degeneration of the aortic wall (correct)
Correct. Cystic medial degeneration weakens the media and allows dissection in Marfan syndrome.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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