antiphospholipid syndrome ( aps ) is an autoimmune disease characterized by the presence of antiphospholipid antibodies , venous and/or arterial thrombosis , and repetitive fetal loss [ 1 , 2 ] .
the laboratory criteria to diagnose aps are high serum levels of lupus anticoagulant as well as anticardiolipin and -2 glycoprotein - i antibodies .
aps can occur with other systemic diseases such as systemic lupus erythematosus or as a single entity .
deep vein thrombosis represents the most frequent aps complication , as it occurs in 2955% of patients ; however , acute or indolent chronic ischemic presentations can involve any organ including the lung , skin , brain , liver , kidneys , adrenal glands , heart , and eyes .
ocular involvement can occur in 888% of patients [ 5 , 6 , 7 ] and it can be the first sign at presentation .
it is therefore important to make a quick diagnosis in order to consider anticoagulant treatment to prevent further thrombosis , as the risk of this complication is up to 29% in untreated aps patients .
visual symptoms at presentation vary from a reduction of visual acuity and amaurosis fugax to transient scotoma and visual field defect , all of which can be unilateral or bilateral .
it is important to underline that , when amaurosis fugax involves both eyes simultaneously , it is often a manifestation of central nervous system ischemia .
ocular discomfort , conjunctival hyperemia , and pain have often been reported in the literature as common symptoms of aps patients .
a 38-year - old female patient presented with reduced vision in the right eye , which had occurred the same day .
the patient had a negative ocular and medical history and no previous miscarriage as well as no relevant family history for ophthalmic pathologies .
visual acuity was finger count in the right eye ( 1-meter distance ) and 20/20 in the left eye .
fundoscopic examination revealed a subhyaloid hemorrhage , engorged and tortuous retinal veins , intraretinal hemorrhages , and cotton wool spots ( fig .
1 ) . fundus fluorescein angiography demonstrated a delayed inferior hemiretinal venous filling ( fig .
3 ) . as choroidal ischemia has been previously reported with aps , indocyanine green angiography was performed , which did not show any significant modification of choroidal perfusion .
prothrombotic conditions secondary to other factors such as sepsis , homocystinemia , and genetic defects of coagulation factors ( thrombin mutations , factor v leiden , antithrombin deficiency , etc . ) were ruled out .
laboratory investigations showed that the erythrocyte sedimentation rate , igg anticardiolipin , and -2 glycoprotein - i antibody were above normal limits .
thromboplastin inhibition test was strongly positive for lupus anticoagulant , while antinuclear antibody and antinative dna antibodies were negative .
serum total complement ( c3 , c4 ) , proteins s and c , and antithrombin iii were within normal limits .
other studies were normal or negative , including complete blood count , platelet count , renal , liver , and thyroid functions , hemoglobin a1c , ferritin , transferrin , and ldl cholesterol .
the patient met the criteria for aps ; however , according to the american college of rheumatology classification criteria , a concomitant diagnosis of systemic lupus erythematosus could not be established .
she started treatment with hydroxychloroquine 200 mg / daily and ticlopidine 250 mg / daily . considering the central location of the hemorrhage , we deferred any invasive treatment and opted for watchful waiting .
the subhyaloid hemorrhage resolved spontaneously and the patient recovered a visual acuity of 20/20 in her right eye 3 months after the initial episode ( fig .
this is the first reported case of simultaneous subhyaloid hemorrhage and branch retinal vein subocclusion as presentations of aps .
a prompt diagnosis of this condition is fundamental to consider a systemic treatment to avoid any further thrombosis .
this is often difficult , as ocular manifestations of aps can be variable . assessing the patient 's thrombotic risk by investigating the presence of high blood pressure and hypercholesterolemia ,
the use of tobacco and oral anticontraceptive agents as well as the history of previous of thrombosis , fetal loss , and acute ischemic events is mandatory in the process of deciding whether to start systemic treatment .
 we report an atypical presentation of antiphospholipid syndrome ( aps ) with concomitant subhyaloid hemorrhage , engorged and tortuous retinal veins , intraretinal hemorrhages , and cotton wool spots in a 38-year - old female . 
 medical treatment was preferred to any invasive treatment . 
 the subhyaloid hemorrhage resolved spontaneously and the patient recovered a visual acuity of 20/20 in her right eye 3 months after the initial episode . 
 a prompt diagnosis of this condition is fundamental to consider a systemic treatment to avoid any further thrombosis . 