Combined Central Retinal Artery and Vein Occlusion Associated with Factor V Leiden Mutation and Treated with Hyperbaric Oxygen

Abstract

Background: Combined central retinal artery occlusion (CRAO) and central retinal vein occlusion (CRVO) is an uncommon retinal vascular disease which causes sudden visual acuity loss and is associated with poor prognosis and the development of severe complications. We report a very rare case of combined CRAO and CRVO in a patient with factor V Leiden (FVL) mutation (only 3 cases published). To our knowledge, this is the first case of combined CRAO and CRVO treated with hyperbaric oxygen therapy (HBOT). Case and Results: A 49-year-old woman presented with complaints of sudden loss of vision in her left eye (LE), with best corrected visual acuity (BCVA) of 1/20. A complete ophthalmic evaluation with fundus angiography showed combined CRAO and CRVO. The patient was urgently treated with HBOT (she completed a total of 9 sessions in 7 days), with marked visual acuity and angiographic improvement (BCVA of 10/10). Forty-five days later, she developed a new LE CRVO, and BCVA decreased to 5/10 and later to <1/20 because of significant macular edema. A detailed investigation showed an abnormal resistance to activated protein C, and a genetic study showed homozygosity for FVL mutation. The patient was submitted to 3 monthly injections of 1.25 mg bevacizumab. After 10 months, the patient is in a stable condition with BCVA of 6/10. Conclusions: Combined CRAO and CRVO in young adults should be investigated thoroughly for embolic sources, thrombophilic disorders and local ocular conditions. This is the first case of this severe disease that was treated with HBOT, and the visual result was very good.

Oxygen therapy in patients with retinal artery occlusion: A meta-analysis

“…More than 9 hours of treatment are effective for restoring a patient’s vision. A case presentation revealed that a CRAO patient who was treated with hyperbaric oxygen therapy for a total of 13.5 hours gained marked visual acuity [ 40 ]. The time between onset and starting oxygen therapy is critical in RAO.…”

Efficacy of early Hyperbaric Oxygen Therapy in Central Retinal Artery Occlusion

“…As far as we know our case was the most successfully treated patient in term of improvement in visual function. Lemos et al [10], reported a patient with initial VA of 20/400 had a marked improvement in vision after HBOT and increased to 20/32. Although the inal VA of their patient was higher than that of our patient the initial VA was also much higher than ours which was an indirect evidence of better initial retinal function.…”

Central retinal artery and vein occlusion as a complication of persistent hyaloid artery – a case report

“…Consequently, the formation of this compartment could have generated a spatial compromise, giving rise to secondary CRVO [ 7 ]. Combined CRAO and CRVO in patients with FVL mutation, even though very rare, is described in the literature [ 18 ]. However, genetic analysis excluded this cause in our patient.…”

“…We speculated that in our patient, PHA twisting led to torsion of the residual primordial common bulb, branching off to HA and CRA. In this case, we considered that CRAO would have occurred first, opposed to the inflammatory and thrombotic aetiology of combined CRVO and CRAO [ 18 , 19 ]. The consequential CRVO advanced by venous stasis due to decrease in arterial inflow.…”