Acute Bilateral Photoreceptor Degeneration in an Infant After Vaccination Against Measles and Rubella

Key Points

Question

What was the cause of acute vision loss after a measles and rubella vaccination in an infant boy?

Findings

A 13-month-old Japanese boy developed bilateral acute vision loss with exudative retinal detachment 24 days after a measles and rubella vaccination. The retina reattached spontaneously in few days; however, the optical coherence tomography images showed a thin outer nuclear layer and the electroretinograms were nonrecordable at 18 days after the onset.

Meaning

Vision loss after a measles and rubella vaccination is an extremely rare event and may have been coincidental or related to the vaccination.

Abstract

Importance

Ocular inflammation is occasionally observed after vaccinations, and most of them resolve without permanent visual disturbances. However, there are some rare cases of severe ocular complications following vaccinations.

Objective

To report the findings in an infant boy who developed an acute loss of vision bilaterally after Haemophilus influenzae type b, Pneumococcal conjugate vaccination, and measles and rubella vaccination. His vision did not recover.

Design, Setting, and Participant

A retrospective review of the medical records of a 13-month-old Japanese boy.

Main Outcomes and Measures

Fundus and fluorescein angiographic findings, ultrasonographic and optical coherence tomographic images, and electroretinographic findings.

Results

A healthy 13-month-old boy had an acute loss of vision in both eyes 31 days after Haemophilus influenzae type b and Pneumococcal conjugate vaccinations and 24 days after a measles and rubella vaccination. He also developed a common cold 10 days before the vision loss. Ultrasonography showed an exudative retinal detachment 1 day after the onset of the visual reduction; however, his fundi appeared normal 4 days later. His eyes did not pursue objects, and pupillary light reflexes were not present. No signs of anterior uveitis were noted. He was treated with corticosteroids, but his vision did not improve. The retinal vessels gradually attenuated, and diffuse small white punctate lesions appeared in the deep retina. Optical coherence tomography showed a thinner outer nuclear layer and an absent ellipsoid zone. The electroretinograms were nonrecordable. These findings suggested a severe impairment of the photoreceptors, especially their outer segments. Western blot analysis of the patient’s sera detected an antibody against recoverin, a calcium-binding protein of photoreceptors.

Conclusions and Relevance

We hypothesize that an infection induced severe chorioretinitis with an exudative retinal detachment, which then produced an autoantibody against recoverin. The autoantibody then altered the function of the photoreceptors very rapidly. The initial infection may have been caused by the measles and rubella vaccination. However, because to our knowledge this has not been reported previously, the visual loss after the vaccinations may have been an extremely rare event that was coincidental or may have been related to the vaccination.

This brief report report the findings in an infant boy who developed an acute loss of vision bilaterally after Haemophilus influenzae type b, Pneumococcal conjugate vaccination, and measles and rubella vaccination.

Introduction

A degeneration of the photoreceptors has been reported in patients with inherited retinal dystrophies and paraneoplastic or nonparaneoplastic autoimmune retinopathy. These retinal dystrophies are usually seen in adults except for Leber congenital amaurosis. We report our findings in a 1-year-old boy who developed an acute loss of vision bilaterally 31 days after Haemophilus influenzae type b (Hib) and pneumococcal conjugate vaccination, and 24 days after measles and rubella (MR) vaccination.

Report of a Case

The patient was 13-month-old Japanese boy who was a full-term infant. He was healthy with normal physical and neurological development. He was vaccinated against Hib and pneumococcal conjugate vaccination on March 1, 2016, and against MR on March 8, 2016. He developed a general fever and upper respiratory tract infection from March 19, 2016, to March 23, 2016, although no vesicular lesions or eruption were noted. Around noon on the April 1, 2016, his father noticed that his eyes did not pursue objects, although he had looked at everything that morning. He was brought to a hospital on the next day, and ultrasonographic images of the eyes revealed an exudative retinal detachment in both eyes (Figure 1). He had no pupillary light reflex in both eyes. No signs of anterior uveitis, such as ciliary injection or posterior synechia, were noted. It was difficult to detect inflammatory cells in the anterior chamber because the examination was performed without general anesthesia. The magnetic resonance images of the central nervous system, including the optic nerve, were within the normal limits. Four days after the onset of his visual loss, his fundi appeared to be normal, although fluorescein fundus angiography showed dye leakage from the peripheral retinal vessels (Figure 2). He was treated with 2 consecutive courses of corticosteroid pulse therapy with methylprednisolone; however, no improvement was detected. Three weeks after the onset, his retinal vessels were attenuated, and numerous small white punctate lesions appeared diffusely in the deep layers of the retina (Figure 2). Optical coherence tomography showed that the outer nuclear layer was thin and the ellipsoid zone was absent. A few inflammatory cells were seen in the vitreous cavity in the optical coherence tomography images (Figure 2). Full-field electroretinograms were nonrecordable (Figure 2).

Figure 1. Ultrasonographic Images of Eyes of a 1-Year-Old Boy Who Developed an Acute Reduction of Vision in Both Eyes 24 Days After Vaccination Against Measles and Rubella.

Exudative retinal detachment can be seen 1 day after the onset of reduced vision in both eyes.

Figure 2. Fundi, Fluorescein Angiographic, Optical Coherence Tomographic Images, and Electroretinograms.

Numerous small white punctate lesions can be seen in the fundi (arrowheads). Optical coherence tomographic images show thinner outer nuclear layer and absent ellipsoid zone. Electroretinograms are nonrecordable.

Antibodies against the herpes simplex virus, varicella-zoster virus, cytomegalovirus, and Epstein-Barr virus were not detected in the serum but those against measles and rubella were present. The aqueous humor were not examined for antibodies. Western blot analysis of the patient’s sera detected an antibody against recoverin, a calcium-binding protein that is primarily detected in the photoreceptors.

He was treated with tacrolimus beginning 3 weeks after the onset because steroid pulse therapy was not effective. After then, his pupillary reflex became slightly positive. However, his eyes still did not pursue objects.

Four months after the onset, the punctate lesions in his fundi became indistinct, and the retinal pigment epithelium was slightly atrophic with some depigmentation (Figure 2). The optic nerve head was normal, and optical coherence tomography images showed no regeneration of the ellipsoid zone and normal thickness of the nerve fiber layer. His eyes still did not pursue objects, although his peripheral vision seemed to remain because he could avoid large objects when walking alone. General examination did not detect any neoplasms in other organs at 9 months after the onset.

Discussion

Our optical coherence tomography investigations of a 13-month-old boy with an acute loss of vision bilaterally showed that the outer nuclear layer was thin and the ellipsoid zone was not present. The full-field electroretinograms were nonrecordable. These findings indicated a severe impairment of the photoreceptors, especially their outer segments. Ultrasonography demonstrated an exudative retinal detachment at the onset, suggesting the presence of severe chorioretinitis, although edema, exudates, and hemorrhages were not seen in the retina ophthalmoscopically.

Diffuse photoreceptor impairments are seen in infants with Leber congenital amaurosis or paraneoplastic and nonparaneoplastic autoimmune retinopathy. Leber congenital amaurosis is not likely the cause of vision loss in our patient because a loss of visual acuity with exudative retinal detachment is rarely seen in Leber congenital amaurosis. Unfortunately, genetic examinations were not performed on the patient.

The incidence of uveitis after general vaccinations is reported to be 8 to 13 in 100 000 persons/y. Uveitis has been reported after vaccinations against diphtheria-tetanus-pertussis, influenza, bacille Calmette-Guérin, hepatitis B, varicella-zoster, human papilloma virus, and measles-mumps-rubella. These studies reported that the ocular complications included anterior uveitis, posterior uveitis, and panuveitis. Posterior and panuveitis have been reported after vaccinations against bacille Calmette-Guérin, hepatitis B, papilloma virus, and measles-mumps-rubella. These cases of uveitis resembled acute posterior multifocal placoid pigment epitheliopathy, Vogt-Koyanagi-Harada disease, or severe vitritis with multiple cream-colored lesions deep in the retina. The onset of the ocular inflammation ranged from 1 day to 4 to 6 weeks after the vaccination. The vision of most patients was reduced but normalized with or without steroid therapy.

Therefore, this infant boy had the most severe, bilateral visual impairment after a vaccination ever reported to our knowledge. In addition, his vision did not recover in spite of the prompt administration of corticosteroids. Tacrolimus seemed to have some effect; however, the improvement may have been the natural course of the disease process.

The presence of an autoantibody against recoverin in the boy’s sera suggests that an autoimmune mechanism was the cause of the photoreceptor inactivation. Probably the severe chorioretinitis with exudative retinal detachment first affected photoreceptors. The degeneration of the photoreceptors led to the production of autoantibody against recoverin and a subsequent rapid destruction of the remaining photoreceptors.

The vaccinations that he received several weeks before the onset of the visual loss may be related to the initial chorioretinitis with exudative retinal detachment. Although both Hib and pneumococcal conjugate and MR vaccinations are possible causes for the chorioretinitis, MR vaccination was more likely the cause of the chorioretinitis because both of the measles and rubella viruses can cause retinitis. Haemophilus influenzae type b and pneumococcal conjugate vaccination are less likely because he had the same vaccinations 3 times before the last vaccination. In Japan, the first vaccination against MR is scheduled between 1 and 2 years of age, whereas the first, second, and third vaccinations against Hib and pneumococcal conjugate vaccinations are scheduled before the age of 1 year.

Conclusions

We report our findings in a 13-month-old boy who developed acute and severe photoreceptor impairment 3 weeks after a MR vaccination. His vision did not recover despite prompt steroid treatment. Because an association between MR vaccination and vision loss has not been reported, the association may be an extremely rare event or may have been coincidental.