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Ectopia Lentis in a Child with Marfans Syndrome-Anaesthetic Challenges, a Case Report
Marfan’s syndrome is an autosomal dominant disorder of connective tissue.It involves mutation to gene that makes fibrillin. Cardio vascular manifestations include aortic root dilation and aortic dissection,mitral valve proplapse.Musculoskeletal and ocular (ectopia lentis)manifestations are seen.People with Marfan tend to be tall and thin with long arms,legs, fingers and toes and flexible joints. Ectopia lentis is a condition in which the lens is displaced because of weakened or broken zonules. In the absence of trauma, ectopia lentis should evoke suspicion for hereditary systemic disease such as Marfan’s syndrome.
Marfans Syndrome, Difficult Airway.
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