Table 4.
Characteristics of the symptomatic infants with ocular involvement at birth and during follow-up.
| Patient | Ocular findings at birth | Neuroimaging findings (MRI) | Other signs at birth | Hearing function | Antiviral therapy | Maternal infection | Last ophthalmological evaluation (age) | Neurological outcome |
|---|---|---|---|---|---|---|---|---|
| 1 | R: chorioretinitis in the paramacular area, inferior temporal sector, about ½ of the papillary diameter | Periventricular calcifications | no | SNHL (R&L 60 dB) at birth; normal at last evaluation | GCV 6 weeks | MPI in 2nd trimester | R: chorioretinal scar; normal vision (11.3 years) | normal |
| 2 | L: small chorioretinal scar | Ex-vacuo dilatation of the trigons and temporal horns of the lateral ventricles; thinning of the corpus callosum; subcortical and temporo-mesial atrophy | IUGR, petechiae, thrombocytopenia, hepatomegaly | SNHL (R 70 dB; L 40 dB) at birth; normal at last evaluation | GCV 6 weeks | ND | L: small chorioretinal scar; normal vision (8.6 years) | Speech delay; mild psychomotor delay |
| 3 | R: small focus of paramacular chorioretinal scar | Temporo-polar gliotic lesion; thinning of the corpus callosum | no | normal | GCV 6 weeks | NPI in 1st trimester | R: small chorioretinal scar; normal vision (8.2 years) | normal |
| 4 | R: small chorioretinal scar between the disc and the macula | Perivascular gliosis, ventricular asymmetry and dilatation of the temporal horns of the lateral ventricles | petechiae, thrombocytopenia, hepatosplenomegaly | normal | GCV 6 weeks | NPI in 1st trimester | R: small chorioretinal scar; abnormal VEPs; normal vision (9.9 years) | psychomotor delay; seizures |
| 5 | L: chorioretinitis below the inferior temporal branch | Mild ventriculomegaly | no | normal | VGC 6 months | NPI in the 3rd trimester | L: small chorioretinal scar; normal vision (7.1 years) | normal |
| 6 | R: chorioretinitis in paramacular region | Micro polygyria; mild ventriculomegaly, thinning of the corpus callosum | no | SNHL (no response R) at birth; (R no response; L 40 dB) at last evaluation | VGC 6 months | NPI in the 1st trimester | R: chorioretinal scar; abnormal VEPs; low vision (4.9 years) | Severe psychomotor delay; seizures |
| 7 | L: chorioretinal scar in the posterior pole | Periventricular WM abnormalities | no | normal | VGC 6 months | MPI in the 2nd trimester | L: chorioretinal scar; normal vision (9.5 years) | normal |
| 8 | R: multiple lesions involving the lower sector. L: large area of chorioretinitis interesting the posterior pole temporally to the macula | Several periventricular calcifications; WM abnormalities; micro polygyria and agyria; mild ventriculomegaly; hippocampal dysplasia | Microcephaly, seizures, cholestatic hepatitis | SNHL (R 50 dB; L 60 dB) at birth; normal at last evaluation | GCV 6 weeks | NPI in the 1st trimester | R: chorioretinal scars; abnormal VEPs; low vision (2.2 years) | Severe psychomotor delay; seizures |
| 9 | R&L: multiple foci of chorioretinitis | Multiple small periventricular cysts; mild ventriculomegaly | IUGR, microcephaly, severe SNHL | SNHL (R&L no response) prothesis | VGC 6 months | ND | R&L: chorioretinal scars; abnormal VEPs; low vision (2.9 years) | Severe psychomotor delay |
| 10 | R&L: multiple foci of chorioretinitis; pale optic disc | Micro polygyria and pachygyria; several subependymal calcifications; mild ventriculomegaly; corpus callosum hypoplasia; cisterna magna | IUGR, microcephaly, petechiae, thrombocytopenia; seizures | SNHL (R no response) prothesis | GCV 6 weeks | NPI in the 2nd trimester | R&L: chorioretinal scars; abnormal VEPs; low vision (2.1 years) | Severe psychomotor delay; spastic paresis; seizures |
| 11 | R: widespread chorioretinal involvement in the inferior temporal and nasal sectors and macular lesion; L: chorioretinitis in the lower sector | Several periventricular calcifications; WM abnormalities | IUGR, prematurity, microcephaly, hepatosplenomegaly, petechiae, seizures, SNHL | SNHL (R&L no response) prothesis | GCV 6 weeks | ND | R: chorioretinal scars; microphthalmia, exotropia; abnormal VEPs; normal vision (8.6 years) | Severe psychomotor delay; spastic paresis |
| 12 | R: chorioretinal scar in peripapillary area | Multiple small periventricular cysts; WM abnormalities | no | normal | VGC 6 months | MPI in the 2nd trimester | R: chorioretinal scar; normal vision (1.7 years) | normal |
| 13 | R: small area of chorioretinitis in paramacular region | Diffused WM abnormalities; mild ventriculomegaly | IUGR, prematurity, microcephaly, seizures | SNHL (L no response at birth and at last evaluation) | VGC 6 months | NPI in the 1st trimester | R: chorioretinal scar; exotropia; abnormal VEPs (1.1 years) | Spastic hemiparesis R; psychomotor delay |
| 14 | L: area of chorioretinitis in paramacular region | Tetra ventricular hydrocephalus; several calcifications; hypoplasia of the cerebellar worm; mega cisterna magna; diffused WM abnormalities | IUGR, prematurity, petechiae, tetra ventricular hydrocephalus | Normal at birth; SNHL (R&L 40 dB) at last evaluation | GCV 6 weeks | MPI in the 1st trimester | L: chorioretinal scar; abnormal VEPs; low vision (4.8 years) | Severe psychomotor delay; spastic paresis; seizures |
| 15 | R: area of chorioretinitis in paramacular region | WM abnormalities | Prematurity | normal | GCV 6 weeks | ND | R: small chorioretinal scar; normal vision (7.1 years) | normal |
| 16 | R: not clear evidence of small chorioretinal scar | Asymmetric signal by the optic nerves (related to chorioretinal damage) | IUGR | normal | no | ND | R: small chorioretinal scar; normal VEPs (2.9 years) | normal |
MRI, magnetic resonance imaging; GCV, ganciclovir; IUGR intrauterine growth restriction; L, left; R, right; SNHL, sensorineural hearing loss; VGCV, valganciclovir; WM, white matter; MPI, primary infection; NPI, non-primary infection; ND, not definable; VEPs, visual evoked potential.