George J. Manayath

Macular cysts in retinal dystrophy

Purpose of review To describe the entity of macular cysts in retinal dystrophy, differentiate it from cystoid macular edema (CME), and review the role of carbonic anhydrase inhibitors in management. Recent findings Macular cysts in retinal dystrophy are seen in retinopathies caused by mutations in the NR2E3 gene, juvenile X-linked retinoschisis (XLRS), and some other retinal dystrophies. These must be distinguished from CME. Optical coherence tomography can clearly demonstrate intraretinal cysts which may not be clinically detectable. Intravenous fluorescein angiography (IVFA) does not show macular hyperfluorescence (i.e. leakage). Molecular genetic testing aids in the diagnosis and elucidation of pathophysiology. Carbonic anhydrase inhibitors may promote resolution of the cysts resulting in visual improvement. Summary Non-CME macular cysts in retinal dystrophies can be differentiated from CME by a combination of clinical examination, IVFA, and molecular genetic testing to identify causative phenotype. Carbonic anhydrase inhibitors may be effective in promoting resolution.

Idiopathic pediatric retinal artery occlusion

We report a case of branch retinal artery occlusion (BRAO) in a healthy young girl. An eight-year-old girl presented with sudden loss of vision in her left eye. She had a pale retina with macular edema consistent with extensive BRAO. A thorough workup was performed to determine any etiologic factor. All test results were within normal limits. Her visual acuity improved from finger counting to 20/40 over two weeks, on immediate treatment with intravenous steroids (methyl prednisolone). This case suggests that BRAO can occur in healthy children without any detectable systemic or ocular disorders and a dramatic improvement may be achieved with prompt treatment with intravenous steroids.

Is myopia a protective factor against central serous chorioretinopathy

AIM To evaluate if any association exists between central serous chorioretinopathy (CSCR) and the refractive status of the eye. METHODS This retrospective, institutional, case control study included 499 patients, wherein 262 patients diagnosed as acute CSCR, were compared with an age and gender matched control group of 237 patients. All patients were evaluated with a detailed systemic and ocular history, objective and subjective refractions for both eyes and complete ocular examination by a retina specialist, at all visits. Optical coherence tomography confirmed the diagnosis of CSCR. RESULTS The mean age was found to be 40±7y in the study group (Group 1) compared to 38±10y in the control group (Group 2). Most common refractive status in the study group, was emmetropia seen in 191 patients (72.9%), followed by hypermetropia seen in 47 patients (17.9%) and astigmatism seen in 21 patients (8.0%). Only 3 subjects (1.1%) had myopia, which was less than or equal to 1.0 D, compared to 70 subjects (29.5%) in the control group, suggesting a statistically significant lower incidence of CSCR among the myopic patients (P< 0.0001). With respect to the systemic factors, 26 (9.9%) patients were using systemic steroids in the study group (Group 1) compared to none in the control group (Group 2) suggesting a statistically significant association of CSCR with systemic steroid use (P<0.05). No other significant systemic risk factors were noted. CONCLUSION Though CSCR is a multifactorial disease, myopia serves as a protective factor for CSCR. Thus, myopic eyes are less likely to develop CSCR. Since both retinal pigment epithelium (RPE) and choriocapillaris are postulated in the pathogenesis of CSCR, chorio-retinal thinning and atrophy seen in myopic eyes are less likely to cause CSCR.

Atypical retinocytoma with diffuse vitreous seeds: An insight

Retinocytoma is a rare benign form of retinoblastoma. It is usually found on routine examinations and also while screening families of patients with retinoblastoma. Distinctive features are a translucent retinal mass with calcification, retinal pigment epithelial disturbance, chorioretinal atrophy and absence of growth. We report a case with all the above features along with diffuse vitreous seeds and optical coherence tomographic documentation of intralesional cavitary lesions.

Low Fluence Photodynamic Therapy Versus Graded Subthreshold Transpupillary Thermotherapy for Chronic Central Serous Chorioretinopathy: Results From a Prospective Study

BACKGROUND AND OBJECTIVE The authors compare low fluence photodynamic therapy (PDT) against graded subthreshold transpupillary thermotherapy (TTT) for chronic central serous chorioretinopathy (CSC). PATIENTS AND METHODS Forty-two eyes of 42 patients with chronic CSC were included in this prospective, nonrandomized, interventional trial. All patients were offered PDT as an initial intervention. If they declined PDT, TTT was offered. RESULTS Twenty patients underwent PDT and 22 underwent TTT. The difference in best-corrected visual acuity between the TTT and PDT groups at baseline and at 6 months after treatment was not significant (P = .728). The mean foveal thickness decreased significantly in both groups (P = .001). However, patients in the TTT group required more treatments (P = .013) and longer time for resolution of the CSC (P = .013). CONCLUSIONS Both PDT and TTT may be equally effective in the treatment of chronic CSC. Graded subthreshold TTT may be a cost-effective and safe alternative for PDT. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:334-338.].

Progressive keratoconus, retinal detachment, and intracorneal silicone oil with obsessive-compulsive eye rubbing

An 18-year-old boy with an obsessive-compulsive disorder of eye rubbing presented with forme fruste keratoconus (KC) and posterior subcapsular cataracts. After evaluation, he underwent phacoemulsification in his left eye with intraocular lens implantation. The aggressive eye rubbing, however, aggravated the rapid progression to established KC, and further acute corneal hydrops within 3 months. Within the next 3 months, the eye rubbing precipitated rhegmatogenous retinal detachments (RDs) in both eyes. Furthermore, after undergoing a combined cataract and RD surgery with belt buckling and silicone oil endotamponade in his right eye, the repeated eye rubbing caused extrusion of the implanted silicone oil into the subconjunctival space and within the corneal stroma. This is the first report to our knowledge describing this unique complication associated with eye rubbing. It also highlights the need for increased vigilance and care that needs to be directed toward patients predisposed to such complications.

Polypoidal choroidal vasculopathy: Pearls in diagnosis and management

Polypoidal choroidal vasculopathy (PCV) is increasingly recognized as an important cause of exudative maculopathy in Asians as against Wet age-related macular degeneration in Caucasians. A panel of retinal experts methodically evaluated pertinent updated literature on PCV with thorough PubMed/MEDLINE search. Based on this, the panel agreed upon and proposed the current consensus recommendations in the diagnosis (clinical and imaging), management and follow-up schedule of PCV. Diagnosis of PCV should be based on the gold standard indocyanine green angiography which demonstrates early nodular hyperfluorescence signifying the polyp with additional features such as abnormal vascular network (AVN). Optical coherence tomography is an excellent adjuvant for diagnosing PCV, monitoring disease activity, and decision-making regarding the treatment. Current treatment modalities for PCV include photodynamic therapy, anti-vascular endothelial growth factor agents, and thermal laser. Choice of specific treatment modality and prognosis depends on multiple factors such as the location and size of PCV lesion, presence or absence of polyp with residual AVN, amount of submacular hemorrhage, presence or absence of leakage on fundus fluorescein angiography, visual acuity, and so on. Current recommendations would be invaluable for the treating physician in diagnosing PCV and in formulating the best possible individualized treatment strategy for optimal outcomes in PCV management.

Iatrogenic subretinal injection of Ozurdex® implant and its effect on macular edema

PURPOSE: The purpose of this study was to report a rare case of subretinal lodgement of Ozurdex® implant (Allergan Inc., Irvine, CA, USA) and its effect on macular edema in a case of central retinal vein occlusion (RVO). METHODS: A rare complication of subretinal lodgement of Ozurdex® implant without retinal perforation was encountered in a case of RVO with intractable macular edema. As associated retinal perforation was not noted, no intervention was done. The patient was regularly followed up at 1 month, and the effect on macular edema and intraocular pressure was analyzed. RESULTS: The corticosteroid pellets got disintegrated and totally absorbed with a subtle chorioretinal scar by the 3rd follow-up month without any intervention. Even though subretinal, it was capable of reducing macular edema by 181 microns at 1 month postinjection, and its effect started wearing off by 2 months. DISCUSSION: Subretinal lodgement of Ozurdex® implant is rare and preventable, yet a potential complication of intravitreal implants which is now in vogue. We speculate a too acute angle of injection or incomplete insertion of the drug delivery system applicator (DDS) away from the limbus or perhaps less refined previous DDS applicator to be a cause for subretinal delivery of the implant. The early disintegration of implant occurred due to breach in structural integrity that caused loss of controlled drug release and rapid absorption. It reduced macular edema up to 2 months without elevating intraocular pressure. CONCLUSION: A more widespread application of any technology always portends a more significant risk for complications, and an ophthalmologist should be aware of this potential risk. Though subretinal, corticosteroid implant was capable of reducing macular edema by 181 microns by 1 month and its effect wore off by 2 months.

Spontaneous anatomical and functional recovery of bilateral electric shock maculopathy

A 12-year-old boy presented with best-corrected visual acuity (BCVA) of 6/9 in both eyes following an episode of electric shock. Optical coherence tomography (OCT) showed disruption of the ellipsoid zone as well as retinal pigment epithelium (RPE) layer. Fundus autofluorescence (FAF) showed increased central hypoautofluorescence in both eyes. At 3-month follow-up, BCVA improved to 6/6 with OCT showing spontaneous resolution of maculopathy in both eyes with reorganized RPE layer and ellipsoid zone. To the best of our knowledge, this is the first case of bilateral electric shock maculopathy (ESM) with spontaneous anatomical as well as functional recovery. Ophthalmologists must be aware of various forms of ESM. OCT and FAF must be done in patients presenting with defective vision and history of electric shock for the diagnostic as well as prognostic evaluation.

Low-fluence photodynamic therapy for early onset choroidal neovascular membrane following laser in situ keratomileusis.

Figure 2a: Fundus photograph and fl uorescein angiography of the left eye following treatment with low-fl uence photodynamic therapy showing regressed subfoveal choroidal neovascular membrane with late staining Figure 2b: Optical coherence tomography (horizontal and vertical) of left eye macula following treatment with low-fl uence photodynamic therapy showing regressed subfoveal choroidal neovascular membrane Low-fluence photodynamic therapy for early onset choroidal neovascular membrane following laser in situ keratomileusis