Retinal arterial but not venous tortuosity correlates with facioscapulohumeral muscular dystrophy severity

Susannah Q Longmuir; Katherine D Mathews; Reid A Longmuir; Vinayak Joshi; Richard J Olson; Michael D Abràmoff

doi:10.1016/j.jaapos.2010.03.006

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Retinal arterial but not venous tortuosity correlates with facioscapulohumeral muscular dystrophy severity

Journal article

Peer reviewed

Retinal arterial but not venous tortuosity correlates with facioscapulohumeral muscular dystrophy severity

Susannah Q Longmuir, Katherine D Mathews, Reid A Longmuir, Vinayak Joshi, Richard J Olson and Michael D Abràmoff

Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus, Vol.14(3), pp.240-243

06/2010

DOI: 10.1016/j.jaapos.2010.03.006

PMID: 20603058

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Abstract

Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal-dominant disease beginning with facial and shoulder girdle weakness with variable progression. Exudative retinal detachment, retinal vessel irregularities on fluorescein angiography, and retinal vessel tortuosity have been found in association with FSHD. In this retrospective study, muscle affectedness severity was rated as mild, moderate, or severe by a neurologist masked to the retinal images. Three ophthalmologists masked to disease severity graded the degree of arterial and venous tortuosity on a scale of 1 to 4. An automated method estimated an index of tortuosity for arteries and veins from color fundus photographs. Spearman rank correlation coefficients were used to describe the relationship between retinal vessel tortuosity and disease severity. Seven patients with an average age of 13 years (range, 7-36 years) were selected. Correlation between the subjective tortuosity for arteries, and the severity of FSHD was 0.78 (p = 0.039). The correlation coefficient for venous tortuosity was -0.06 and was not significant (p = 0.882). The correlation coefficient between the average algorithmic computer-generated tortuosity indices for arteries and FSHD severity was high (0.85, p = 0.016), but for veins it was low and not significant (0.19, p = 0.662). The authors of previous reports have shown retinal vascular abnormalities did not correlate to FSHD disease severity. Our results suggest a correlation between the tortuosity of arteries and the severity of disease in FSHD patients. These results suggest the tortuosity of arteries can serve as a biomarker of severity of disease in these FSHD patients, either as determined by human experts or by an automated method.

Retinal Vein - pathology

Severity of Illness Index

Young Adult

Humans

Retinal Artery - pathology

Adolescent

Biomarkers

Muscular Dystrophy, Facioscapulohumeral - pathology

Adult

Retrospective Studies

Child

Fluorescein Angiography

Details

Title: Subtitle: Retinal arterial but not venous tortuosity correlates with facioscapulohumeral muscular dystrophy severity
Creators: Susannah Q Longmuir - Department of Ophthalmology, University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA. Susannah-longmuir@uiowa.edu
Katherine D Mathews
Reid A Longmuir
Vinayak Joshi
Richard J Olson
Michael D Abràmoff
Resource Type: Journal article
Publication Details: Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus, Vol.14(3), pp.240-243
DOI: 10.1016/j.jaapos.2010.03.006
PMID: 20603058
NLM abbreviation: J AAPOS
ISSN: 1091-8531
eISSN: 1528-3933
Publisher: United States
Language: English
Date published: 06/2010
Academic Unit: Roy J. Carver Department of Biomedical Engineering; Neurology; Electrical and Computer Engineering; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Neurology (Pediatrics); Ophthalmology and Visual Sciences
Record Identifier: 9983806290902771

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