May 2007
Volume 48, Issue 13
Free
ARVO Annual Meeting Abstract  |   May 2007
Peripheral Exudative Choroidal Vasculopathy Associated With Myotonic Dystrophy - A Clinico-Histopathologic Case Report
Author Affiliations & Notes
  • A. K. Junk
    Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida
    Wills Eye Institute, Jefferson College of Medicine, Philadelphia, Pennsylvania
  • R. P. Wilson
    Wills Eye Institute, Jefferson College of Medicine, Philadelphia, Pennsylvania
  • R. C. Eagle, Jr.
    Wills Eye Institute, Jefferson College of Medicine, Philadelphia, Pennsylvania
  • J. L. Federman
    Wills Eye Institute, Jefferson College of Medicine, Philadelphia, Pennsylvania
  • Footnotes
    Commercial Relationships A.K. Junk, None; R.P. Wilson, None; R.C. Eagle, None; J.L. Federman, None.
  • Footnotes
    Support Supported by NIH center grant P30 EY014801 and by an unrestricted grant to the University of Miami from Research to Prevent Blindness
Investigative Ophthalmology & Visual Science May 2007, Vol.48, 1459. doi:https://doi.org/
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      A. K. Junk, R. P. Wilson, R. C. Eagle, Jr., J. L. Federman; Peripheral Exudative Choroidal Vasculopathy Associated With Myotonic Dystrophy - A Clinico-Histopathologic Case Report. Invest. Ophthalmol. Vis. Sci. 2007;48(13):1459. doi: https://doi.org/.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose:: Myotonic Dystrophy is an adult-onset autosomal dominant disorder characterized by a variety of systemic manifestations including myotony, muscular dystrophy, cardiac conduction defects, cardiomyopathy, frontal balding, and insulin-resistance. Christmas tree cataract is the most common ocular manifestation. In contrast to other inherited muscle dystrophies, Myotonic Dystrophy has also been associated with abnormal iris vasculature resulting in spontaneous, recurrent hyphemas. Peripheral exudative choroidal vasculopathy has not been linked to Myotonic Dystrophy to date.

Methods:: A 52-year old male with Myotonic Dystrophy and atrial fibrillation on anticoagulation therapy presented after he had sustained a spontaneous vitreous hemorrhage and developed secondary uncontrolled intraocular pressures. Vitrectomy and lensectomy with silicon oil placement led to a brief recovery followed by recurrent hemorrhage and elevated intraocular pressures. Enucleation of the blind and painful eye revealed retinal detachment due to subretinal hemorrhage originating from a peripheral exudative hemorrhagic choroidal vascular malformation. The presence of peripheral exudative choroidal vasculopathy in the other eye was evident in fundus photography and fluorescein angiography.

Results:: Myotonic Dystrophy may be associated with vascular malformation of the iris and the choroid. In the presence of peripheral exudative choroidal vasculopathy anticoagulation therapy may increase the risk of subretinal hemorrhage and result in a protracted course and grave visual prognosis.

Conclusions:: Serial retinal exam and angiography may be necessary to diagnose and follow asymptomatic peripheral exudative choroidal vasculopathy. Its presence may increase the risk of ocular bleeding in patients on anticoagulation therapy. The benefits of anticoagulation therapy in these individuals should be carefully reassessed.

Keywords: choroid • genetics • pathology: human 
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