Abstract | PURPOSE: METHODS: An 11-year-old boy was referred for evaluation of strabismus present since birth. On examination his uncorrected visual acuity was 6/6 in each eye, with small angle left eye esotropia in the primary position, ipsilateral face turn, abduction deficit, lid fissure narrowing in adduction and widening in abduction; plus typical features of left-sided type I Duane syndrome were present. The left fundus demonstrated localized, well-compensated, markedly convoluted, dilated and tortuous retinal vessels and venous congenital retinal macrovessel, which traverse the macular region. The right fundus was unremarkable. In order to elucidate if this retinal vascular malformation is associated with similar lesions in the orbit, paranasal sinuses and/or brain, magnetic resonance imaging (MRI) was performed, followed by digital subtraction angiography of the brain vessels. RESULTS: CONCLUSIONS: Intracranial AVM might have affected the normal vascular development during the second month of gestation, causing involutional changes of the sixth nerve and resulted in Duane's syndrome by "steal phenomenon." Retinal AVM may point to concomitant intracranial AVMs, thus warranting neurological assessment. Patients with AVM of the retina should be examined early with brain and orbital neuroimaging to rule out cerebral AVMs.
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Authors | Dragana Vucic, Tanja Kalezic, Aleksandar Kostic, Milenko Stojkovic, Dijana Risimic, Branislav Stankovic |
Journal | Ophthalmic genetics
(Ophthalmic Genet)
2013 Mar-Jun
Vol. 34
Issue 1-2
Pg. 61-4
ISSN: 1744-5094 [Electronic] England |
PMID | 22697299
(Publication Type: Case Reports, Journal Article)
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Topics |
- Angiography, Digital Subtraction
- Arteriovenous Fistula
(complications, diagnosis)
- Arteriovenous Malformations
(diagnosis)
- Child
- Duane Retraction Syndrome
(complications, diagnosis)
- Humans
- Intracranial Arteriovenous Malformations
(diagnosis)
- Magnetic Resonance Imaging
- Male
- Neurocutaneous Syndromes
(complications, diagnosis)
- Retinal Artery
(abnormalities)
- Retinal Vein
(abnormalities)
- Visual Acuity
(physiology)
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