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Ectopia Lentis in a Child with Marfans Syndrome-Anaesthetic Challenges, a Case Report


Affiliations
1 Yenepoya Medical College, Mangaluru, India

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.

Keywords

Marfans Syndrome, Difficult Airway.
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  • Esturo K. Motoyama, Peter J. Davis: Smiths Anaesthesia for infants and children ed 5th, St. Luis 1990, The CV Mosby Co. Appendix E 897
  • Murdoch JL, Walker BA, Halpern BL, Kuzma JW, Mckusick VA. life expectancy and causes of death in the Marfan Syndrome. New Engl J Med 1972; 286: 804.

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  • Ectopia Lentis in a Child with Marfans Syndrome-Anaesthetic Challenges, a Case Report

Abstract Views: 100  | 

Authors

Venkatesh Kode
Yenepoya Medical College, Mangaluru, India
Meghana Mukund
Yenepoya Medical College, Mangaluru, India

Abstract


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.

Keywords


Marfans Syndrome, Difficult Airway.

References





DOI: https://doi.org/10.18311/isacon-Karnataka%2F2017%2FEP119