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Meningococcal B vaccine-associated papillophlebitis and cilioretinal artery occlusion

Published:February 03, 2022DOI:https://doi.org/10.1016/j.jcjo.2022.01.015
      Vaccine-induced optic neuritis is a known subtype of optic neuritis that has been reported following the administration of various types of vaccines, both viral and bacterial. Ocular symptoms vary significantly and can result in permanent vision loss. There has been only one case of optic neuritis following meningococcal B vaccine (Bexsero; GlaxoSmithKline, London, UK) administration that was reported on the Vaccine Adverse Event Reporting System (2016, VAERS ID 686394), but the details of the event are not thoroughly explained. To our knowledge, this is the first case of retinal venous dilation, retinal hemorrhages, optic disc edema, and cilioretinal artery occlusion to occur after presumed meningococcal B vaccination to be described in the English-language ophthalmic literature.
      A 15-year-old previously healthy male presented with acute vision loss OS 2 weeks following administration of the meningococcal B vaccine. He stated that he saw a horizontal “dark red line” across his vision in the left eye on awakening that had changed to “gray” by the time of presentation. He denied any flashes of light, eye pain, headache, nausea, vomiting, or fever. Medical, surgical, social, and family histories were unremarkable. He was taking no medications.
      His best-corrected vision was 20/25 OD and counting fingers at 5 feet OS. No relative afferent pupillary defect was noted OU. No abnormalities were found on slit-lamp examination. Intraocular pressure measured 14 mm Hg OU. Funduscopic examination OD was within normal limits. There was 1+ optic disk swelling, rare blot hemorrhages, and superior macular retinal edema with arterial narrowing consistent with a cilioretinal artery occlusion OS (Fig. 1). Automated perimetry (24-2 Humphrey visual field) showed no abnormalities OD and cecocentral scotoma OS with a mean deviation of –12 dB (Fig. 2). Optical coherence tomography of the macula revealed intraretinal thickening consistent with macular edema OS.
      Fig 1
      Fig. 1Optical coherence tomography images as well as colour images of the fundus.
      Fig 2
      Fig. 2Results of Humphrey visual field at the patient's first visit.
      The patient was diagnosed with neuroretinitis and was treated with a 5-day course of intravenous methylprednisolone 1000 mg daily followed by an oral prednisone taper that consisted of 40 mg daily for 5 days and 20 mg daily for 5 days. Follow-up examination 11 days later revealed mild improvement in visual acuity (20/400 OS), but a mild relative afferent pupillary defect was noted OS. Four weeks after the onset of symptoms, the patient experienced improvement of vision to 20/30 OS. Funduscopic examination OS revealed resolving optic disc edema and mild tortuosity and dilatation of veins, but the retinal hemorrhage and cilioretinal artery distribution macular edema resolved. The 24-2 Humphrey visual field mean deviation was –8 dB. Extensive laboratory tests including C-reactive protein, rapid plasma reagin, rheumatoid factor, anti-neutrophil cytoplasmic antibody, anti-nuclear antibody, Bartonella henslae titer (IgM and IgG), and West Nile virus were negative. Chest x-ray was normal. Magnetic resonance imaging of the brain and orbit with and without contrast material was unremarkable. Follow-up at 1 year demonstrated 20/30 vision OS.
      The pathogenesis of vaccine-induced optic neuritis is unknown, primarily because an association between optic neuritis and vaccines has not been officially established.
      • Roszkiewicz J
      • Shoenfeld Y.
      Vaccines and optic neuritis: consequence or coincidence?.
      It is theorized that there is an autoimmune reaction to either the pathogen itself or to a vaccine adjuvant used to induce immune response.
      • Roszkiewicz J
      • Shoenfeld Y.
      Vaccines and optic neuritis: consequence or coincidence?.
      ,
      • Moradian S
      • Ahmadieh H.
      Early onset optic neuritis following measles-rubella vaccination.
      Specifically, it has been proposed that this reaction results in immune complex formation that attacks the retinal vasculature, leading to hyperpermeability and inflammation.
      • Moradian S
      • Ahmadieh H.
      Early onset optic neuritis following measles-rubella vaccination.
      In this case, macular edema and retinal hemorrhages without exudate supported a diagnosis of inflammatory disease of the retina and optic nerve (neuroretinitis).
      • Abel A
      • McClelland C
      • Lee MS.
      Critical review: typical and atypical optic neuritis.
      In the literature, neuroretinitis is often characterized by a triad of decreased visual acuity, optic disc edema, and macular star formation (which typically appears within 2–6 weeks).
      • Abdelhakim A
      • Rasool N.
      Neuroretinitis: a review.
      Several causation criteria have been proposed to try to establish a cause-and-effect relationship between triggering events and visual loss. These include a biologically plausible mechanism; a close temporal relationship between cause and effect coherent with the pharmacokinetics or pathogenesis of the purported cause; analogy from prior animal model or human cases; specificity of effect; exclusion of alternative etiologies including chance alone; dose–response gradient; and dechallenge/rechallenge. In our case, the presumed biologically plausible mechanism is believed to be autoimmune inflammatory disease triggered by the vaccine. The onset of symptoms and signs within 2 weeks is consistent with an immunologic mechanism that requires mobilization and activation of inflammatory lymphocytes. There is analogy for optic neuritis after vaccination with other vaccines. No other etiology for the optic neuritis and retinal findings was found despite a complete laboratory evaluation and neuroimaging. The most common alternative etiologies were not present, including multiple sclerosis or other antibody-mediated causes of optic neuritis. Although there was no rechallenge, a dechallenge led to improvement of symptoms and signs without recurrence. The presence of the optic disc edema in conjunction with the retinal findings is an atypical finding for idiopathic or demyelinating optic neuritis, retinal vein occlusion, or retinal artery occlusion. The presence of optic disc edema, retinal venous dilatation, retinal hemorrhages, and a concomitant cilioretinal artery occlusion suggests a partial retinal vein occlusion as the unifying mechanism in our case. The lower perfusion pressure of the cilioretinal artery in the setting of central retinal venous backpressure is the presumed mechanism for the ophthalmoscopic appearance in our case.
      • Hayreh SS
      • Fraterrigo L
      • Jonas J
      Central retinal vein occlusion associated with cilioretinal artery occlusion.
      Further study is necessary to determine whether our observation is confirmed by other cases in the future.

      Footnotes and Disclosure

      The authors have no proprietary or commercial interest in any materials discussed in this article.

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