Cilioretinal artery occlusion and protein S deficiency in pregnancy

• Published in: Acta ophthalmologica (Oxford, England) England), 2014-09, Vol.92 (s253), p.0-0
• Main Authors: CARAMELLO, C, MATEO OROBIA, AJ, HERRERA, L, ZABADANI, K, PINILLA, I, PEREZ NAVARRO, I, ALMENARA, C, ESTEBAN, O, ASCASO, J, CASAS, P
• Format: Article
• Language: English
• Subjects: Macular degeneration; Ophthalmology; Proteins; Veins & arteries
• ISSN: 1755-375X; 1755-3768
• DOI: 10.1111/j.1755-3768.2014.F036.x

Purpose Protein S (PS) is a vitamin K‐dependent plasma protein that has antithrombotic effects. Congenital deficiency of PS is a relatively rare disorder characterized clinically by thrombosis in young people. Cilioretinal arteries are present in about 20% of individuals, supplying a variable territory of the inner retina and macula. To our knowledge, only three cases of branch retinal artery occlusion associated with PS deficiency have been reported. Methods We report the case of a 25 year‐old woman, 38 weeks pregnant, who developed cilioretinal artery branch occlusion in her left eye and who was found to have a PS deficiency. Her best corrected visual acuity (BCVA) was 20/20 in the right eye and 20/30 in the left one. Fundoscopic examination showed an area of retinal pallor located on the posterior pole. Visual field study demonstrated a paracentral scotoma. Spectral‐domain optical coherence tomography (SD‐OCT) showed retinal atrophy in the cilioretinal artery irrigation area. Retinal Nerve Fiber Layer thickness was decrease in the papillomacular bundle. Results Blood analysis demonstrated that PS values recovered after childbirth. The BCVA was 20/20 in both eyes and the funduscopic exploration returned to normal. Conclusion When cilioretinal occlusion occurs, the extent of visual loss depends on the precise area of macular supply. Protein S defiency is another factor that should be considered in young patients with unexplained retinal vascular occlusion, particularly if it occurs during pregnancy.