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HERDIN Record #: NCR-PHC-16051216534847 Submitted: 13 May 2016 Modified: 13 May 2016

Cystic Medial Degeneration without Marfanoid Feature.

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Background: Cystic medial degeneration is the result of fragmentation of elastic fibers, and loss of smooth muscle cells or alteration in smooth muscle cell functions. In advance forms there is a dramatic loss of elastic fibers and smooth muscle cells with the accumulation of basophilic amorphous material giving the media a true cystic appearance. Many ascending aortic aneurysms are asymptomatic when diagnosed being incidentally noted on chest x-ray or other imaging study. Between 25% and 75% of patients, however, present with chest pain that results in the diagnosis of an aneurysm.

Objective: To report a case of Medial cystic degeneration without Marfanoid features. Setting: Philippine Heart Center Case summary: We report the case of an apparently healthy 32 year old male while undergoing medical examination for pre-employment purposes incidentally noted to have an abnormal auscultatory finding. Physical examination did not demonstrate the phenotypic manifestations of Marfan syndrome. An echocardiogram revealed aortic aneurysm which was confirmed by a CT scan and aortogram. After aneurysmectomy with Dacron graft implantation with aortic valve sparing technique. Histopathologic reports revealed cystic medial degeneration. His post-operative course was uncomplicated and was discharge after 10 days.

Summary: Cystic medial degeneration in younger age group is classically associated with Marfanoid syndrome. Young adults without Marfanoid syndrome and who are not at risk for atherosclerosis lowers this suspicion. This case illustrated the importance of cardiac auscultation when assessing an individual even with a low like hood for alteration in arterial structure. We consider this case to be of interest because of its rarity to cystic medial degeneration in a 32 year old male without Marfanoid feature.

Conclusion: Ascending aortic aneurysms with an associated cystic medial degeneration is a potentially lethal disease entity with an expansive differential diagnosis that requires a high index of suspicion on the part of the health care provider. Our case illustrated the importance of cardiac auscultation when assessing an individual, thus, this will avoid potentially fatal complications when left untreated or unrecognized. The urgent imaging to confirm the diagnosis of aneurysm and prompt initiation of surgical intervention is required.

Publication Type
Research Report
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Philippine Heart Center Medical Library CR.010.05 Fulltext Print Format

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