Open angle glaucoma in a case of Type IV Ehler Danlos syndrome: A rarely reported association.
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Date
2014-08
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Abstract
A 26-year-old male presented to us with defective vision in the
left eye. He had best corrected visual acuity (BCVA) of hand movement (HM) in right eye and 6/9 in left eye. He had ptosis
with ectropion in both eyes and relative afferent pupillary
defect (RAPD) in right eye. Intraocular pressure (IOP) was
46 and 44 mmHg in right and left eye, respectively. Fundus
showed glaucomatous optic atrophy (GOA) in right eye and cup
disc ratio (CDR) of 0.75 with bipolar rim thinning in left eye.
Systemic examination showed hyperextensible skin and joints,
acrogeria, hypodontia, high arched palate, and varicose veins.
He gave history of easy bruising and tendency to fall and history
of intestinal rupture 5 years ago for which he had undergone surgery. He was diagnosed as a case of Type IV Ehler-Danlos
syndrome (EDS) with open angle glaucoma. He underwent
trabeculectomy in both eyes. This is a rare case that shows
glaucoma in a patient of EDS Type IV. Very few such cases have
been reported in literature.
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Keywords
Acrogeria, easy bruising, Ehler-Danlos syndrome, glaucoma, hyper extensible joints, trabeculectomy
Citation
Mitra Arijit, Ramakrishnan R, Kader Mohideen Abdul. Open angle glaucoma in a case of Type IV Ehler Danlos syndrome: A rarely reported association. Indian Journal of Ophthalmology. 2014 Aug ; 62 (8): 880-884.