Ocular features and management challenges of Marfan's Syndrome in Benin City, Nigeria.

Aims And Objectives: The objective of this study is to describe the ocular features of Marfan's Syndrome in Benin City, Nigeria and highlight problems associated with their management

Materials And Methods: A study of all consecutive patients with a diagnosis of Marfan's syndrome at the eye clinic of the University of Benin Teaching Hospital, Benin City, Nigeria was done between June 1st 2009 and December 31st 2010.

Results: There were 13 patients (8 males and 5 females). The age range was 10 to 50 years with a mean age of 23+10.1 years. The duration of symptoms was 1 year to 22 years with a mean of 7.9 years. The ocular features were ectopia lentis in 92.3% of the patients, cataract in 69.2%, glaucoma and myopia in 30.8% each. Others were strabismus (7.7%), unilateral corneal opacities from the use of traditional eye medication (15.4%), phthisis bulbi and retinal detachment in 7.7% each. Three (23%) patients were bilaterally blind while 7(53.8%) had unilateral blindness. The best corrected visual acuity in 4 out of 5 patients who had cataract extraction at 8 weeks post op. was 6/60 to 6/12. There was no improvement in 1 patient.

Conclusion: Patients with Marfan's syndrome in Benin City, Nigeria have visual impairment and blindness due to delay in presentation and the use of traditional eye medications. Health education and early presentation to hospital is recommended. This will help in reducing avoidable visual impairment and blindness and thus improve survival and quality of life in these patients.