Severe congenital chorioretinitis caused by congenital lymphocytic choriomeningitis virus infection





Abstract


Purpose


To describe a case of congenital lymphocytic choriomeningitis virus (LCMV), a potentially severe and under-diagnosed etiology of congenital chorioretinitis.


Observations


A 5-month old boy presented with esotropia. Examination revealed light perception vision in the right eye and normal fixation and following behavior in the left eye, and a 50PD esotropia with full versions. The external, anterior segment, and pupil exams were normal. Fundus examination demonstrated slightly pale optic nerves, numerous geographic atrophic and hyperpigmented lesions along the vascular arcades in both eyes that extended into the fovea of the right eye. Head computed tomography (CT) imaging demonstrated bilateral cerebral volume loss with consequential ex vacuo dilation of the lateral ventricles and scattered intracranial calcifications. Serum IgG and IgM titers for toxoplasmosis, rubella, cytomegalovirus (CMV), herpes simplex virus (HSV), syphilis, and zika were all negative. Upon communication of negative TORCHS titers, the mother recalled a severe rat infestation of their home during the pregnancy. A LCMV antibody titer was then ordered and which resulted positive for IgG antibodies.


Conclusions and Importance


Congenital LCMV infection is an under-recognized cause of congenital chorioretinitis.



Introduction


Lymphocytic choriomeningitis virus (LCMV), an emerging fetal teratogen and member of the arenavirus family, is an often undiagnosed cause of acquired and congenital infection in humans. Acquired, postnatal humanhuman huinfecion with LCMV usually consists of a brief febrile illness with mild symptoms that tend to fully resolve within 3 weeks often with no long-term sequalae. In contrast, congenital infection with LCMV can cause severe and permanent damage to the fetus, mainly targeting the central nervous system and retina.



Case description


A 5-month-old boy presented with a 2-month history of esotropia of the right eye. On examination, he had light perception vision in the right eye and normal fix and follow vision in the left eye. His pupils were equal and reactive without afferent pupillary defect (APD). The motility exam revealed a 50PD esotropia with full versions. The anterior segment and external exams were normal. Intraocular pressure was normal by palpation. Fundus examination demonstrated smoderately pale optic nerves with the cup:disc ratio of 0.1 in both eyes, numerous geographic atrophic and hyperpigmented lesions consistent with scarring along the vascular arcades and maculae in both eyes. The pigmentary retinal lesions included the fovea of the right eye but spared the fovea in the left eye ( Figs. 1 and 2 ). The vitreous was clear without active inflammation. The cycloplegic refraction was −5.50D in the right eye and −1.25D in the left eye.




Fig. 1


Diffuse chorioretinal scarring in the macula, including the fovea, and periphery of the right eye (Retcam).



Fig. 2


Diffuse chorioretinal scarring in the macula, excluding the fovea, and periphery of the left eye (Retcam).


Serum IgG and IgM titers for toxoplasmosis, rubella, cytomegalovirus (CMV), herpes simplex virus (HSV), syphilis, and Zika were all negative. A head CT scan without contrast demonstrated broad regions of volume loss within the bilateral posterior cerebral hemispheres at the level of the parietal, occipital, and posterior temporal lobes with anomalous gyration sulcation ( Fig. 3 a,c). There was also an associated severe volume loss of the posterior supratentorial white matter with ex vacuo dilation of the lateral ventricles ( Fig. 3 a–b). There was no hydrocephalus. There were also two foci of dystrophic calcification at the lateral margin of the right caudate nucleus and another focus along the ependymal surface of the right lateral ventricle ( Fig. 3 d).


Jul 10, 2021 | Posted by in OPHTHALMOLOGY | Comments Off on Severe congenital chorioretinitis caused by congenital lymphocytic choriomeningitis virus infection
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