Combined central retinal vein and cilioretinal artery occlusion in a 25-year-old woman

Rom J Ophthalmol. 2022 Apr-Jun;66(2):178-184. doi: 10.22336/rjo.2022.35.

Abstract

We report a case of a 25-year-old woman with sudden and painless diminution in vision and central scotoma in her left eye (LE). She was a smoker and had been taking combined oral contraceptive (COC) pills for 1 year. On admission, the best-corrected visual acuity (BCVA) was 1,5/50 in the LE. Posterior segment examination revealed optic disc edema with flame-shaped retinal hemorrhages, mildly tortuous and dilated retinal veins. Moreover, retinal edema in the peripapillary and perimacular region, foci of hemorrhages and Roth's spots in the posterior pole, as well as pale superior papillomacular bundle were observed. Fundus fluorescein angiography (FFA) confirmed the delayed flow of contrast through the cilioretinal artery in the LE. The clinical picture suggested left central retinal vein (CRVO) with cilioretinal artery occlusion (CLRAO). All laboratory and imaging tests were normal except for homozygous methylenetetrahydrofolate reductase (MTHFR) gene mutation (A1298C genotypes). However, serum homocysteine (Hcy) level was normal. Low molecular weight heparin (LMWH) treatment was administered. Retinal lesions, as well as BCVA improved, but central scotoma remained. Abbreviations: aPTT = activated partial thromboplastin time, BCVA = best-corrected visual acuity, CBC = complete blood count, CLRAO = cilioretinal artery occlusion, COC = combined oral contraceptive, CRA = central retinal artery, CRP = serum C-reactive protein, CRVO = central retinal vein occlusion, CT = computed tomography, CTA = computed tomography angiography, ECG = electrocardiography, ESR = erythrocyte sedimentation rate, FERG = flash electroretinogram, FFA = fundus fluorescein angiography, GCA = ganglion cell analysis, GCL = ganglion cell layer, Hcy = homocysteine, ICGA = indocyanine green angiography, INR = international normalized ratio, IOP = intraocular pressure, IPL = inner plexiform layer, LE = left eye, LMWH = low molecular weight heparin, mfERG = multifocal electroretinogram, MTHFR = methylenetetrahydrofolate reductase, OCT = optical coherence tomography, RE = right eye, VF = visual field.

Keywords: A1298C mutation; CLRAO; CRVO; MTHFR polymorphism; central retinal vein occlusion; cilioretinal artery occlusion; methylenetetrahydrofolate reductase gene polymorphism.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Ciliary Arteries
  • Contraceptives, Oral, Combined
  • Female
  • Heparin, Low-Molecular-Weight
  • Homocysteine
  • Humans
  • Methylenetetrahydrofolate Reductase (NADPH2)
  • Retinal Artery Occlusion* / diagnosis
  • Retinal Artery Occlusion* / pathology
  • Retinal Artery Occlusion* / therapy
  • Retinal Vein* / pathology
  • Scotoma

Substances

  • Contraceptives, Oral, Combined
  • Heparin, Low-Molecular-Weight
  • Homocysteine
  • Methylenetetrahydrofolate Reductase (NADPH2)