Pathology Associates Of Lexington, P.A.
Pathology Associates Of Lexington, P.A.
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Pathology Associates Of Lexington, P.A.
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        Catastrophic aortic dissection
      

Pathologists performing autopsies on sudden unexpected deaths will have the opportinuty to not only determine that the manner of death is natural but may also be able to discern the etiology. Most are said to involve a background of hypertension.

  • hypertension: positive history; otherwise unexplained LVH in absence of hypertensive history.
  • aortic athersclerosis: look for a crack in an athersclerotic plaque.
  • aortitis: be it rheumatoid, luetic, or other, the histology should tip off to an inflammatory pathogenesis.
  • connective tissue failure disorders: the prototype is Marfan syndrome, which may not have full genetic penetrance (FA10-64). The tip off may be one or more of the following.
    • history of sleep apnea.
    • history of aortic murmurs, especially regurgitation.
    • history of mitral valve prolapse.
    • physical exam: pectus excavatum or the opposite, pectus carinatum (pigeon chest).
    • physical exam: long face, limbs (wingspan greater than height), & fingers.
    • physical exam: kyphosis.
    • gross exam: aortic root dilation (annulo-aortic ectasia).
    • gross exam: floppy/redundant mitral valves.
    • gross exam: elongated mitral valve chordae.
    • micro exam: cystic medial necrosis revealed by elastic stain = image HERE.
    • other connective tissue disorders or syndromes (some with aortic dilation) and a write-up about molecular testing...at NMF with list HERE. And another syndrome HERE. And Google "Marfan mimmics".
  • traumatic
  • combination of the above: case FA10-64 may be such a case.

Family members of those who clearly fit a Marfan diagnosis as well as of those who have only scant suggestion of the diagnosis (only mild genetic phenotypic penetrance...forme fruste cases) might find guidance through the National Marfan Syndrome Foundation (has family history assessment info, clinical trial info around the world & contact sources, etc.) or possibly be directed through Best Doctors or other means to a genetic counselor who may be able to help clarify whether there seems to be any real risk that this autosomal dominant disorder runs in their family.

(posted 5/8/2010; latest addition 5/15/2010)

 

 

 

 

 

 
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