Siddharth Kesarwani

Outcomes of a Protocol-Based Management for Zone 1 Retinopathy of Prematurity: The Indian Twin Cities ROP Screening Program Report Number 2

PURPOSE To report the outcomes of zone 1 aggressive posterior retinopathy of prematurity (ROP) treated with aggressive laser photocoagulation by a protocol-based approach over a 10-year period. DESIGN Prospective, interventional case series. METHODS SETTING Tertiary eye institute and neonatal intensive care units in the twin cities of Hyderabad and Secunderabad in South India. STUDY POPULATION Babies screened under the Indian Twin Cities Retinopathy of Prematurity Screening (ITCROPS) program between January 1, 1997 and March 31, 2007. INTERVENTION Multiple sessions of laser photocoagulation at any sign of plus disease with abnormal shunt vessels with or without neovascularization in zone 1. OUTCOME MEASURE Based on the anatomic status of the retina at the final follow-up, the outcome was classified into good (completely regressed with no vitreoretinal changes), fair (regression with vitreoretinal distortion), or poor (progression into partial or total retinal detachment involving macula). Descriptive statistics (mean and range) were used for gestational age at birth, birth weight, and post-conceptional age at first presentation and at the first intervention. Trends in incidence of zone 1 ROP over 1 decade were analyzed. Outcomes were expressed in terms of proportions and 95% confidence interval (CI). RESULTS Of the 3654 babies screened, 227 eyes of 115 babies were detected to have zone 1 aggressive disease, defined as zone 1 retinal vessels having abnormal closed-loop shunts, dilation and tortuosity, flat new vessels, or rapid progression. Laser was performed in 169 eyes. Disease regressed with good outcome in 142 eyes (84%) (95% CI, 77.6-89.2) and progressed to poor outcome in 13 eyes (7.69%) (95% CI, 4.1-12.8), while fair outcome was seen in 6 eyes (3.55%) (95% CI, 1.3-7.5). Thus 148 babies (87.5%) (95% CI, 81.6-92.14) had a favorable outcome. The outcomes in 8 treated eyes (4.7%) lost to follow-up were unknown. CONCLUSIONS Zone 1 aggressive ROP has a good outcome if the screening is done early; intervention is prompt and adequate, with frequent follow-up until complete regression is achieved.

Surgical technique for removing congenital fibrovascular pupillary membrane, with clinicopathological correlation

Congenital fibrovascular pupillary membrane, rare in newborns, poses challenges for the ophthalmic surgeon. Predicting its clinical course is difficult, as is removing the membrane surgically without inducing a cataract. We describe a 22-day-old girl who presented with a pupillary membrane causing progressive decrease in the size of the dilated pupil, necessitating removal at 4 months of age. Histopathology demonstrated tissue of embryonic muscle origin, suggesting that the membrane was more likely a remnant of fetal iris than of fetal vasculature. The smooth muscle or fibroblastic elements in the tissue explains progressive contracture of the membrane.

Optic disc doubling.

Many developmental defects can simulate the optic disc (optic disc pseudodoubling). True optic disc duplication with 2 independent retinal vasculatures is rare. Associated optic nerve duplication is rarer still and seen in lower vertebrates. We report a rare case of unilateral double optic discs with a single optic nerve in the left eye of a 41-year-old man, otherwise asymptomatic in the affected eye.

Macular folds: an unusual association in topiramate toxicity.

We report two uncommon cases of acute onset myopia with macular folds following use of topiramate. A 25‐year‐old woman, with no prior history of glasses, taking topiramate for recurrent headaches, presented with decreased vision. On examination, she was found to have a refractive error of ‐5.00 DS in both eyes and intraocular pressure of 10 mmHg and 6 mmHg in the right and left eyes, respectively. She had closed angles on gonioscopy, ciliary effusion on ultrasonic biomicroscopy with inner limiting membrane folds in the macula in both eyes. She was on anti‐glaucoma medication when she presented to us.

Bietti crystalline retinal dystrophy with subfoveal neurosensory detachment and congenital tortuosity of retinal vessels: case report

A 34-year-old man presented with reduction and distortion of vision in both the eyes. The best-corrected vision was 20/20 parts, N6 in either eye. The external and slit lamp examination of both the eyes was unremarkable. The fundus examination showed multiple intraretinal crystalline deposits at the posterior pole, extending up to midperiphery, tortuous retinal blood vessels with S-shaped deflections, and absent foveal reflex in both the eyes. There were no corneal crystals, and the color vision was defective in both the eyes. Fundus autofluorescence and fundus fluorescein angiogram (FFA) were suggestive of geographic areas of retinal pigment epithelium (RPE) and choriocapillary (CC) loss. OCT revealed subfoveal neurosensory detachment. Flash ERG and EOG were normal except for a slight decrease in amplitude and delay in latency of pattern ERG waveforms. The Humphrey’s visual field showed paracentral scotoma with reduction in the amplitude of waveforms from the corresponding area in the multifocal ERG in both the eyes. Systemic evaluation for crystalline retinopathy was unremarkable. He was diagnosed to be a case of Bietti crystalline retinopathy (local/regional variant). The subfoveal neurosensory detachment could represent early RPE dysfunction caused by these crystals and could account for the mild visual disturbance in both the eyes. Retinal vascular tortuosity and neurosensory detachment seen in this case is the first time to be reported in literature.

Post-traumatic cyclodialysis cleft with hypotonic maculopathy.

A 41-year-old man presented with complaints of decreased vision following blunt trauma to his left eye with a stone. At presentation to the clinic, visual acuity in the right eye was 6/6 and left eye was 6/24. The intraocular pressures were 14 mmHg and 6 mmHg in the right and left eye, respectively. Slitlamp examination of the right eye was within the normal limit. A detailed examination of the left eye revealed a faint corneal scar (Figure 1), vitreous in the anterior chamber and a pupillary sphincter tear causing the pupil to remain mid-dilated and fixed. The zonules supporting the lens in the left eye were torn and there was phacodonesis with a faint posterior subcapsular opacity. Ultrasonic biomicroscopy of the left eye showed a superior cyclodialysis cleft from the 9 o’clock to the 3 o’clock position (Figure 2A). The left eye showed extensive chorioretinal folds involving the macula. There was elevation of the optic disc with blurring of margins and hyperaemia. These findings were consistent with hypotonic maculopathy (Figure 3A). The fundus in the right eye was normal. The left eye of the patient was treated with oral corticosteroids starting with 1.0 mg/kg and tapered over two months. We also prescribed topical atropine 1% three times a day for three months. At six months post-injury the visual acuity in the left eye was 6/9 and intraocular pressure was 8 mmHg. There was resolution of the chorioretinal folds and optic disc oedema (Figure 3B). An ultrasonic biomicroscopy showed partial closure of the cyclodialysis cleft with increased intraocular pressure and marked improvement in vision (Figure 2B).

Bilateral response after unilateral subconjunctival bevacizumab injection in a child with Stevens-Johnson syndrome

Bevacizumab is widely used for several ocular conditions, including age-related macular degeneration, diabetic retinopathy, corneal neovascularization, neovascular glaucoma, and retinopathy of prematurity. We describe a 14-year-old patient with Stevens-Johnson syndrome in whom subconjunctival injection of bevacizumab in one eye caused bilateral regression of corneal neovascularization.

Acute idiopathic frosted branch angiitis in an 11-month-old infant treated with intravitreal triamcinolone acetonide

An 11-month-old boy presented with bilateral vision loss following upper respiratory tract infection. Extensive perivascular sheathing resembling frosted branch angiitis and severe macular edema was noted in both eyes. All tests for etiological diagnosis were negative. Treatment with systemic corticosteroids and antibiotics caused resolution of perivascular infiltrates. Macular edema resolved with improvement in visual acuity following consecutive bilateral intravitreal triamcinolone acetonide injections. To our knowledge, this is the youngest reported case of frosted branch angiitis.

Spectral domain optical coherence tomography finding in posterior microphthalmos

An eight‐year‐old boy presented with decreased vision in both eyes. At presentation, the visual acuity was 6/60 in both eyes with high plus spheres. Anterior segment examination was normal. Fundus examination and spectral domain optical coherence tomography were consistent with posterior microphthalmos and showed an elevated foveal contour and fold in the outer plexiform layer. External limiting membrane, photoreceptor and retinal pigment epithelium were not involved in the fold. To the best of our knowledge this is the first such case report with optical coherence tomography imaging of the retinal layer involved in a case of posterior microphthalmos.

Macular cherry-red spot helps diagnose rare storage disorder in an infant with repeated respiratory tract infections: case report.

Abstract A seven-month-old male child was brought in for an eye test for poor vision and nystagmus noticed from four months of age. The child had delayed milestones of development and multiple (six times) episodes of unexplained lower respiratory tract infection (from two months of age) treated by pediatricians at different centers without complete cure. Fundus examination showed bilateral cherry-red spots at the macula. There were diffusely distributed hyper-pigmented patches (Mongolian spots) on the back and extensor aspect of the extremities. The case was sent back to the pediatricians for a re-evaluation to rule out storage disorder. Lysosomal enzyme assay in the leucocytes showed a significantly reduced β-galactosidase level (15.6 nmol/hr/mg protein in contrast to a normal range of 79.6 to 480.0). This confirmed the patient to be a case of lysosomal storage disease, the GM1 gangliosidosis (type I).