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Full Version: atrophy of the muscularis - saonew
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A 68-year-old, well-developed, well-nourished black male presents to
the emergency department complaining of shortness of breath. He denies
chest pain. He has no significant past medical history and takes no
medications. A chest x-ray shows clear lung fields, mild cardiomegaly
and a widened thoracic aorta with linear calcifications. An MRI of the
chest shows aortic dilatation in the thorax, extending proximally,
with atrophy of the muscularis and wrinkling of the intimal surface.
What is the most likely etiology of this condition?
A. Atherosclerosis
B. Hypertension
C. Marfan's syndrome
D. Syphilis infection
E. Takayasu's arteritis
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The correct answer is D. Although rare now because of advances in treatment, syphilitic aortitis and aneurysm are still seen, especially in underserved populations. This complication generally occurs 10 to 40 years after initial infection. The vasa vasorum of the aorta undergoes obliterative endarteritis, leading to atrophy of the muscularis and elastic tissues of the aorta and dilatation. Linear calcifications are often seen in the ascending aorta by x-ray. The intimal wrinkling or "tree barking" is also a common feature. Syphilitic aneurysm can be associated with respiratory distress, cough, congestive heart failure and rarely, rupture.
Atherosclerosis (choice A) is the most common cause of aortic aneurysms. These are most often located in the abdominal aorta, distal to the renal arteries. Intimal wrinkling and linear calcifications are not seen.
Hypertension (choice B) is usually responsible for dissecting aneurysms located within 10 cm of the aortic valve. Patients present with sudden chest pain, which is usually severe and tearing in nature. The chronic hypertension causes a cystic medial necrosis, allowing the separation of vessel layers.
Marfan's syndrome, an autosomal dominant connective tissue disorder (choice C) is also associated with dissecting aneurysms, usually of the ascending aorta. The patients are often very tall with arachnodactyly and ligamentous laxity. Their life-span is generally shortened. This patient's description and age are not consistent with this diagnosis.
Takayasu's arter itis (choice E) is a syndrome characterized by ocular disturbances and weak pulses in the arms. It occurs most frequently in young females. It is considered a giant cell arteritis, and does not cause aneurysms.
Good question Saonew, Shabash,keep it up.