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S. Q. Longmuir, R. Longmuir, K. Matthews, R. Olson, M. Abramoff; Retinal Arterial but not Venous Tortuosity correlates with Facioscapulohumeral Muscular Dystrophy (FSHD) Severity. Invest. Ophthalmol. Vis. Sci. 2009;50(13):5419.
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To determine if retinal Arterial but not Venous Tortuosity correlates with Facioscapulohumeral Muscular Dystrophy (FSHD) Severity.
Retrospective chart review was performed from July to October 2008 at the Pediatric Ophthalmology Department at the University of Iowa Hospitals and Clinics. The patient population was skewed toward early onset sporadic cases. A pediatric ophthalmologist (RO), retina specialist (MA), and a neuro-ophthalmologist (RL) were masked to the severity of the patient’s disease and rated arterial and venous tortuosity on a 1 to 4 scale. This data was compared to the patient’s disease severity rated by the pediatric neurologist (KM).
Eight patients average age 16.5 years (range, 4-36) were studied. Correlations between arterial tortuosity index and FSHD severity for the three graders were 0.83, 0.58, and 0.78 respectively, and between venous tortuosity were -0.12, 0.00, and 0.00 respectively. Very little venous tortuosity was noted by all three graders.
Previous reports have shown retinal vascular abnormalities did not correlate to disease severity; however few early onset patients were studied. Our results suggest that retinal arterial, but not venous tortuosity increases with disease severity. One potential explanation for this difference is that the arterial vessel wall connective tissue or smooth muscle is affected differentially by FSHD.
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