Abstract
Purpose
To report an unusual case of congenital bilateral abnormality due to coexisting ocular toxoplasmosis and retinopathy of prematurity.
Observations
A prematurely born baby with bilateral retinopathy of prematurity (ROP) (one eye treated) was found to have a hyperpigmented torpedo maculopathy lesion in one eye while the other eye presented with microphthalmia, congenital cataract and non-specific pigmentary retinopathy. Following negative TORCH screening, laboratory tests subsequently revealed increased plasma IgG and IgM for toxoplasmosis.
Conclusions
We present an unusual case of coexistence of congenital ocular toxoplasmosis with torpedo maculopathy and retinopathy of prematurity.
1
Introduction
Retinopathy of prematurity (ROP) is a vasoproliferative, multifactorial disorder of the retina that occurs principally in newborn preterm infants and is strongly related to low gestational age, low birth weight, supplemental oxygen therapy, fluctuations in oxygenation and poor postnatal growth. ,
Congenital Toxoplasma infection occurs via vertical transmission from the mother and may cause severe congenital malformation, fetal developmental retardation, and the infection of neural tissues. The best described clinical presentation of ocular toxoplasmosis is focal necrotizing chorioretinitis, ultimately resulting in characteristic atrophic scars. Torpedo maculopathy (TM) is a benign, congenital lesion of the retinal pigment epithelium (RPE) located usually temporal to the fovea with orientation towards the central macula. It is usually unilateral, asymptomatic and can affect the outer retina and the choroid. Association with other ocular conditions include numerous conditions including infections such as congenital Zika syndrome.
Herein we report an unusual case of congenital torpedo maculopathy in an eye with retinopathy of prematurity (ROP) and a non-specific pigmentary retinopathy in the fellow microphthalmic eye with congenital cataract in a premature baby with positive Toxoplasma serology.
2
Case report
An extremely premature baby boy, born at 26 weeks of gestation with birth weight of 800 g, was referred to the Medical Center for repair of intestinal perforation at 31 weeks of gestation. He already had intraventricular hemorrhage and necrotizing colitis diagnosed at the referring hospital. On admission, the baby had hypotension and features of septic shock. He underwent laparotomy for repair of stomach perforation as well as perforation along the greater curvature of stomach at the site of the feeding tube.
The first ophthalmic examination was done at 32 weeks, which revealed dense tunica vasculosa lentis, dilated iris vessels and dull view of fundus showing zone I vascularization, stage 1 with no Plus or pre-plus disease in the right eye (OD). The left eye (OS) had less pronounced tunica vasculosa lentis, normal iris vessels and same retinal findings in addition to perimacular lesion. Close surveillance was recommended.
Subsequent screening after 1 week showed very dull fundus views due to worsening of anterior segment findings in OD. There were central lenticular changes, iris vessels encroaching on lens and poor pupillary dilation due anterior synechiae with anterior segment inflammation Fig. 1 . Limited funduscopic view showed vascularization in zone 2 stage II with no Plus or pre-plus disease and non-specific peripheral pigmentary retinopathy OD. There were residual tunica vasculosa and stage 2, zone II with no Plus or pre-plus disease but peri-macular lesion in OS. The lesion did not show any activity such as bleeding or fluid exudation and resembled torpedo shape. Pupillary dilation could not be performed due to unstable systemic condition with respiratory distress syndrome of newborn and pulmonary interstitial emphysema. The baby was evaluated extensively including the TORCH profile, but none of the tests was positive. Prednisolone acetate 1% eyedrops was started in OD four times daily. Follow-up examination after 2 days showed further deterioration, pupillary occlusion, central cataract, shallow anterior chamber and corneal edema in OD. B-scan ultrasonography demonstrated microphthalmia (axial length was 14.25 mm and 16.54 mm in OD and OS, respectively), and flat retina. On subsequent examination within a week, OS showed pre-plus disease and straightening of vessels at the periphery, stage 3 in posterior Zone II. Avascular area in the periphery was fluffy and raised with “popcorns” forming. Darkening of macular scar was also seen in OS Fig. 2 . Subsequently, panretinal photocoagulation was performed in OS, while periorbital triamcinolone acetonide was administered in OD in an effort to decrease the ocular inflammation.
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
