Retinal vein and artery occlusion as the first manifestation of primary antiphospholipid syndrome in a pediatric patient

Abstract

Antiphospholipid syndrome is an acquired autoimmune disease characterized by hypercoagulability associated with recurrent venous and arterial thromboembolism in the presence of antiphospholipid antibodies. Herein, we report a case of rapid sequential retinal vein and artery occlusion as the first manifestation of a primary antiphospholipid syndrome triggered by an acute Mycoplasma infection in a previously healthy 11-year-old patient. On day 1, ophthalmoscopy revealed a central retinal vein occlusion. The patient developed temporal branch retinal artery occlusion the next day. On day 3, a central retinal artery occlusion was observed. Serum lupus anticoagulant, immunoglobulin (Ig) G anticardiolipin, IgG anti-β2-glycoprotein 1 antibody, and Mycoplasma pneumoniae IgM antibody levels were increased. Thus, retinal vascular occlusions can be the first manifestation of primary antiphospholipid syndrome. Although it may not improve visual prognosis, prompt diagnosis and treatment are essential to avoid further significant morbidity.

Ocular venous occlusion in pediatrics: Should thrombophilia investigation and anticoagulant treatment be initiated?

“…Overall, reported risk factors include: APLA, [15][16][17][18][19] homocysteinemia, platelet over-responsiveness, 9,20 vasculitis, acute infections, inflammatory states, and optic pathway tumors. 3,11,[21][22][23][24][25][26][27] In some reports, more than one genetic abnormality was found in patients with RVO, suggesting synergistic interactions that may increase the risk.…”

Pediatric Central Retinal Vein Occlusion Secondary to Concurrent Mechanisms of Optic Neuritis and Antiphospholipid Syndrome

“…14 Although rare cases of CRVO and CRVO/central retinal artery occlusion in children with antiphospholipid syndrome have been described in the literature, the temporal association of CRVO with acute optic neuritis in our patient points to its unique mechanism. [15][16][17] According to the Virchow triad, the 3 factors that predispose patients to venous thrombosis are venous stasis, hypercoagulability, and vessel wall injury or inflammation. 18 Our patient's optic neuritis-induced optic disc edema caused retinal venous congestion that was seen on his initial fundus photographs as dilated and tortuous retinal veins (Figure 1B), which in conjunction with his hypercoagulable state (undiagnosed antiphospholipid syndrome), and possibly perivenular inflammation associated with the demyelinating disorder, resulted in CRVO.…”