Marfan syndrome

Question 1

skeletal features of Marfan

Answer 1

arachnodyactlyly lower upper to lower body segment ratio, increased arm to height ratio pectus deformity, scoliosis and kyphosis joint hypermorbility

Question 2

ocular features of marfan

Answer 2

ectopia lentis- lens dislocation

Question 3

cardiovascular features of marfan

Answer 3

aortic dilation, can have regurgitation and dissection MVP

Question 4

pulmonary manifestations of marfan

Answer 4

spontaneous pneumothorax from apical blebs

Question 5

skin manifestations of Marfan’s

Answer 5

recurrent or incisional hernia skin striae

see hypermobility

Question 6

major cause of death in Marfan’s pts?

Answer 6

aortic dissection in 30-45% of pts

Question 7

when to screen for aortic dissection in a Marfan pt?

Answer 7

TTE at diagnosis and at regular intervals with close monitoring of the aortic root and ascending aorta Operative treatment is needed in aortic root diameter >50 mm to reduce risk for rupture or dissection.

Question 8

Comparing Ehler Danlos and Marfan’s

Question 9

what is arachnodactyly?

Answer 9

Spider like hands

seen in Marfan’s syndrome