Marfan Syndrome
OVERVIEW
- connective tissue disorder
- autosomal dominant
CLINICAL FEATURES
- GENERAL: tall, long thin fingers
- AIRWAY: cervical spine/ligamentous abnormality, high arch palate, crowded teeth
- RESP: emphysema, spontaneous pneumothorax, pectus excavatum, tracheomalacia, OSA, kyphoscoliosis
- CVS: dilated ascending aorta, dissecting aneurysms, AR, MVP, MR, coronary thrombosis
- CNS: cataracts, retinal detachment, lens dislocation
- MSK: easy joint dislocation, arachnodactyly,
MANAGEMENT
ICU
- valve replacement
- aortic root replacement (deep hypothermic arrest)
- scoliosis repair
Anaesthesia
- may be a difficult intubation
- pressure cares
- lung protective ventilation
- chest infection cares
Critical Care
Compendium
Chris is an Intensivist and ECMO specialist at The Alfred ICU, where he is Deputy Director (Education). He is a Clinical Adjunct Associate Professor at Monash University, the Lead for the Clinician Educator Incubator programme, and a CICM First Part Examiner.
He is an internationally recognised Clinician Educator with a passion for helping clinicians learn and for improving the clinical performance of individuals and collectives. He was one of the founders of the FOAM movement (Free Open-Access Medical education) has been recognised for his contributions to education with awards from ANZICS, ANZAHPE, and ACEM.
His one great achievement is being the father of three amazing children.
On Bluesky, he is @precordialthump.bsky.social and on the site that Elon has screwed up, he is @precordialthump.
| INTENSIVE | RAGE | Resuscitology | SMACC