Marfan Syndrome

Marfan syndrome: • AD, ↑ length of long bone A→ TMJ laxity → dislocation with intubation B→ scoliosis, kyphoscoliosis, Pectus excavatum, restrictive lung defect spontaneous Pneumothorax→ careful with PPV C→ cystic media necrosis, aortic dissection, AR, MVP, MR, arrhythmia, aneurysm → Avoid sudden ↑ in contractility, β -blockers and volatiles are good, need endocarditis prophylaxis Other→ lens dislocation, retinal detachment Position→ careful Neuraxial→ potential difficult, may need a bigger dose and volume of LA Lab→ Echo, CT-chest and angio→ all for dissection, valve, tamponade, CXR→ pneumo, ABG, CBC, lytes, BUN, creat, PFT