Case reports on Low Vision Assessment & Management of Marfan Syndrome (MFS) Patients, in a Tertiary Hospital in Kano, Nigeria

Abstract

Marfan syndrome (MFS) is an inherited disorder that affects the heart, joints, skeleton, skin, and eyes. People living with MFS are  described as tall, long, slender built in appearance, with arachnodactyly, chest wall deformities, and scoliosis. A long, narrow face with  deep-set eyes, down-slanting palpebral fissures, flat cheekbones, and a small chin are the facial features often found in people with MFS.  The onset varies from infancy to all ages with most cases being diagnosed in the first two decades of life. In children, ocular findings are  microspherophakia, congenital/infantile glaucoma, high refractive error at a young age, uveitis, retinal detachments, and enophthalmos  secondary to the absence of retrobulbar fat. The basic management procedures include topical dilating agents for chronic dilation of the  eye to increase pupillary size for aphakic correction, thorough and careful refraction, the use of contact lenses and/or glasses, removal of the dislocated lens, cataract surgery and treatment of amblyopia. Low vision assessments were carried out on the patients. The approach  to the management of the two patients, whose cases are being reported, was different for each case and differed from  conventional low vision management. Spectacle corrections of OD -20.00DS (1.76LogMar 6/36 near: 0.80M@23cm) and OD +7.5.0DS (6/9  1M@12cm) were issued for Cases 1 & 2 respectively. 8X Telescope (6/9) and magnifiers (4X Handheld N5 and spectacle +16 DS 0.50m/ N5)  were recommended for cases 1 and 2 respectively. Low vision and rehabilitation were found to maximize visual functioning and are  reliable options for MFS.