Marfan syndrome

 

Issues:

Airway

·         High arched palate

·         Atlanto-axial instability

·         TMJ laxity


-> Beware direct laryngoscopy

-> Beware positioning

Breathing

·         Scoliosis

·         Pectus excavatum/carinatum

·         Restrictive lung disease

·         Bullous lung disease, spontaneous pneumothorax

Circulation

·         Aortic arch aneurysm, dissection, rupture

·         Coarctation (narrow after left subclavian artery)

·         Valve disease (AR, MR, MVP)

·         Myocardial infarction (medial necrosis of coronary arteries)

·         Conduction disease, arrhythmias

·         Pulmonary hypertension, cor pulmonale

-> Tests: 12 lead ECG and TTE

-> Monitoring: 5 lead ECG and arterial line

-> Goals: beware ↑SBP and ↑HR (= ↑aortic wall tension)

Neuro

·         Dural ectasia

o    ↑Risk failed epidural

o    ↑Risk dural puncture


-> Not contraindicated
-> Consider CT/MRI if elective

External

·         Ligamentous laxity

o    ↑Risk subluxation

o    ↑Risk nerve injury

-> Beware positioning

 

Obstetrics:

The pregnancy

·         Multi-disciplinary team

·         Tertiary centre

·         Regular echo

The birth

·         Caesarean (especially if aorta >4.5cm)

·         Spinal/epidural

·         Arterial line

·         Anti-hypertensive drawn up

·         Generous analgesia

·         Beware ergotamine

The epidural

·         Epidural is not contra-indicated

·         Discuss with patient beforehand

·         Consider CT/MRI

 

https://www.anesthesiaconsiderations.com/marfans-syndrome

 

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