In Marfan’s syndrome, Aoic aneurysm occurs most commonly in –
Correct Answer: Ascending aoa
Description: Ans. is 'a' i.e., Ascending Aoao In marfan syndrome, aoic aneurism typically begins at the base of aoa and involves ascending aoa. Clinical manifestations of marfan syndrome Skeletal abnormalitieso Patient is unusually tallo Fingers and hands are long and slender and have spider like appearance (arachnodactyly).o Because the lower segment of the body largely contributes the tall stature, theUpper segment______________ (usually 2SD below mean for age, race and sex)lower segmentSevere chest deformities, pectus excavatum, pectus craniatum.Scoliosis accompanied by kyphosiso High arched palate and high pedal arches or pes planuso Joint ligaments in hands and feet are lax.o Head is commonly dolichocephalicCardiovascular changeso Mitral valve prolapse can give rise to mitral regurgitationo Dilatation of ascending aoa owing to cystic medial necrosis (loss of medial dilatation of aoic valve ring and the root of the aoa giving rise to severe aoic Occular changeso Bilateral subluxation or dislocation of the lens referred to as ectopia lens (typically o Ocular globe is frequently elongated.Associated changesSpontaneous pneumothoraxo Inguinal and incisional hernias.Note - Some patients may develop hypermobility of joints like Ehlers - Danlos syndrom
Category:
Pathology
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