Tushar M. Ranchod

Clinical Presentation of Familial Exudative Vitreoretinopathy

OBJECTIVE To describe the clinical characteristics, staging and presentation of patients with familial exudative vitreoretinopathy (FEVR) in our clinical practice over the last 25 years. DESIGN Case series, retrospective review. PARTICIPANTS We included 273 eyes of 145 patients. METHODS Data collected from charts included gender, gestational age at birth, birthweight, age at presentation, referring diagnosis, family history, prior ocular surgery, and clinical presentation in each eye. Eyes with invasive posterior segment procedures before initial presentation were excluded. MAIN OUTCOME MEASURES Demographics on presentation and clinical staging. RESULTS Patients were slightly male predominant (57%) with a mean birthweight of 2.80 kg (range, 740 g-4.76 kg), mean gestational age of 37.8 weeks (range, 25-42), and mean age at presentation of almost 6 years (range, <1 month-49 years). A positive family history of FEVR was obtained in 18% of patients. A positive family history for ocular disease consistent with but not diagnosed as FEVR was obtained in an additional 19%. Stage 1 FEVR was identified in 45 eyes, stage 2 in 33 eyes, stage 3 in 42 eyes, stage 4 in 89 eyes, and stage 5 in 44 eyes. Radial retinal folds were seen in 77 eyes, 64 of which were temporal or inferotemporal in location. CONCLUSIONS The FEVR patient population is remarkable for the wide range of age at presentation, gestational age, and birthweight. Although a positive family history on presentation may support the diagnosis of FEVR, a negative family history is of little help. The majority of retinal folds extended radially in the temporal quadrants, but radial folds were seen in almost all quadrants. Fellow eyes demonstrated a wide variation in symmetry. The presentation of FEVR may mimic the presentation of other pediatric and adult vitreoretinal disorders, and careful examination is often crucial in making the diagnosis of FEVR. FINANCIAL DISCLOSURE(S) The authors have no proprietary or commercial interest in any of the materials discussed in this article.

LuceDex: a prospective study comparing ranibizumab plus dexamethasone combination therapy versus ranibizumab monotherapy for neovascular age-related macular degeneration.

Background: The LuceDex prospective randomized pilot trial compared the combination of intravitreal ranibizumab and dexamethasone with ranibizumab monotherapy for treatment of neovascular age-related macular degeneration. Methods: Thirty-seven eyes of 37 patients were randomized 1:1 between combination therapy with intravitreal ranibizumab and dexamethasone (Group 1) and intravitreal ranibizumab monotherapy (Group 2). All study eyes received 4 monthly treatments followed by monthly treatment on indication. Results: In the LuceDex study, eyes gained an average of 11.1 and 5.9 Early Treatment of Diabetic Retinopathy Study letters in Groups 1 and 2, respectively, at Month 12. No more than zero Early Treatment of Diabetic Retinopathy Study letters were lost in 88% of Group 1 eyes and 70% of Group 2 eyes. The average number of treatments per study eye by Month 12 was 7.1 in Group 1 and 6.6 in Group 2. Choroidal neovascular membrane size decreased in Group 1 significantly compared with Group 2 (P < 0.05). Conclusion: The LuceDex pilot study suggested a possible benefit of adding intravitreal dexamethasone to treatment of neovascular age-related macular degeneration with intravitreal ranibizumab. A larger study is needed to further identify and define possible benefits of this combination therapy.

Effect of aspirin therapy on photodynamic therapy with verteporfin for choroidal neovascularization.

Background: Photodynamic therapy (PDT) with verteporfin is used for treatment of choroidal neovascularization in several conditions. Platelet aggregation is one of the mechanisms by which PDT is thought to work. This study sought to examine the hypothesis that systemic use of aspirin, an inhibitor of platelet aggregation, affects the efficacy of PDT. Methods: A retrospective review was conducted of data for patients treated with PDT at one institution between 2001 and 2004. End points included total number of PDT treatments, mean time between PDT treatments, change in visual acuity from baseline to 3 months after last PDT treatment, and concurrent or subsequent treatments other than PDT. Results: A total of 244 eyes of 222 patients met inclusion criteria, of which 102 eyes from 92 patients were included in the aspirin taking group. Aspirin takers received an average of 3.11 PDT treatments compared with 2.39 PDT treatments for nonaspirin takers (P = 0.001). Decrease in logMAR visual acuity was greater for aspirin takers (P = 0.0003), and a loss of ≥3 lines was seen in 58% of aspirin takers compared with 35% of nonaspirin takers (P = 0.0003). These differences remained statistically significant after controlling for patient age, lesion type, and lesion size. Conclusions: Patients taking aspirin required more PDT treatments and had worse visual outcomes than patients not taking aspirin, possibly due to aspirin’s ability to inhibit platelet aggregation and thereby diminish the efficacy of PDT.

Hyphema after intravitreal injection of ranibizumab or bevacizumab.

BACKGROUND Exudative age-related macular degeneration is commonly treated with intravitreal injection of medications containing antibodies against vascular endothelial growth factors, including ranibizumab (Lucentis, Genentech, South San Francisco, CA) and bevacizumab (Avastin, Genentech). To the best of our knowledge, hyphema has not previously been reported as a complication of intravitreal injection of ranibizumab or bevacizumab. METHODS Retrospective case series. RESULTS Three patients developed hyphema after intravitreal injection of anti-vascular endothelial growth factor medications: one after ranibizumab and two after bevacizumab. Two patients were pseudophakic and taking warfarin in combination with low-dose aspirin, and one was phakic and not taking any coagulation-altering medications. A total of 26,184 intravitreal injections of ranibizumab or bevacizumab were given during the study period, for an estimated incidence of hyphema after intravitreal injection of approximately 1 in 10,000. CONCLUSION The authors describe hyphema, either gross or microscopic, as a complication of intravitreal injection of anti-vascular endothelial growth factor medications. In all three patients, best-corrected vision returned to baseline after resolution of the hyphema and subsequent injections occurred without complication.

Peripapillary Choroidal Neovascularization In Congenital Retinoschisis

PURPOSE To report a case of peripapillary choroidal neovascularization (CNV) associated with congenital retinoschisis. METHODS Case report. RESULTS A 5-year-old white boy presented with regressed peripapillary CNV in the right eye along with peripheral retinoschisis in the same eye. The left eye presented with macular, lamellar, and peripheral retinoschisis changes. Optical coherence tomography and fluorescein angiography confirmed the presence of regressed peripapillary CNV in the right eye and also revealed retinal nonperfusion within the peripheral retinoschisis cavity in the right eye. CONCLUSION The authors report a case of peripapillary CNV associated with congenital retinoschisis. The authors hypothesize that peripheral retinal nonperfusion in the right eye may have contributed to the development of CNV in that eye.

Tips and tricks in pediatric vitreoretinal surgery.

Pediatric vitreoretinopathies pose unique challenges to the retinal surgeon because of the distinct anatomic and physiological differences from adult retinal diseases. The pars plana, through which the vitreoretinal instrumentation may be safely introduced, is not fully formed until approximately the age of 8 or 9 months, thereby necessitating entry through the pars plicata when a posterior approach is desired in the newborn eye. The vitreous gel, which is well formed and firm in normal newborn eyes, may be optically empty or abnormally syneretic in various pediatric diseases. The vitreoretinal adhesion is stronger in children than in adults, making the surgical induction of posterior vitreous detachment relatively difficult. The biochemistry of the newborn eye is in flux, with rising and falling levels of vascular endothelial growth factor (VEGF), insulin-like growth factor-1, tumor growth factor-b, and other cytokines, which may affect the progression or stabilization of pediatric vitreoretinopathies. To operate safely, the pediatric vitreoretinal surgeon must understand the characteristics that define diseases, such as retinopathy of prematurity (ROP), familial exudative vitreoretinopathy (FEVR), persistent fetal vasculature syndrome (PFVS), congenital x-linked retinoschisis (CXLRS), and Coats disease. We discuss below several common pitfalls and misunderstandings in the surgical approach to pediatric vitreoretinopathies.

Evaluation of Actual vs Expected Photodynamic Therapy Spot Size

PURPOSE To determine the accuracy of the photodynamic therapy (PDT) laser spot size on the retina as generated by 2 Food and Drug Administration (FDA)-approved lasers. DESIGN Prospective observational case series. METHODS Fundus photographs were taken of 1 eye of each of 10 subjects with the WinStation 4000 fundus photography system (OIS; Ophthalmic Imaging Systems, Sacramento, California, USA); disc size was calculated using OIS software. Slit-lamp photographs were taken of the PDT laser spot focused on the retina adjacent to the optic disc, using various spot sizes in combination with 3 different contact lenses and 2 different lasers. Spot size at the retina was determined by measuring the ratio of disc diameter to spot diameter in Adobe Photoshop (San Jose, California, USA) and applying this ratio to the OIS disc measurements. RESULTS Spot size at the retina averaged 87% of expected spot size for the Coherent Opal laser (Coherent Inc, Santa Clara, California, USA) and 104% of expected spot size for the Zeiss Visulas laser (Carl Zeiss Meditec Inc, Dublin, California, USA)(P = .002). Multivariate analysis demonstrated that percentage of expected spot size decreased with larger spot diameter (P = .01 for Coherent laser; P = .02 for Zeiss laser). CONCLUSIONS PDT spot size at the retina appears to be consistently smaller than expected for the Coherent laser while the spot size was consistently within 10% of expected size for the Zeiss laser. The deviation from expected size increased with larger spot size using the Coherent laser.

Retinal neovascularization after internal carotid artery occlusion.

PURPOSE To report a case of retinal neovascularization after surgical ligation of the internal carotid artery for treatment of direct carotid-cavernous fistula. METHODS Case report. RESULT A 44-year-old Asian woman presented with unilateral retinal neovascularization and vitreous hemorrhage. Surgical ligation of the ipsilateral internal carotid artery had been performed 14 years earlier as a treatment for carotid-cavernous fistula after failed attempts at fistula embolization. Vitrectomy was performed for removal of nonclearing vitreous hemorrhage, with intraoperative peripheral retinal ablation and cryotherapy of the lesion. CONCLUSION We report a case of retinal neovascularization associated with total occlusion of the ipsilateral internal carotid artery 14 years earlier.

Central retinal artery occlusion associated with hypertriglyceridemia.

PURPOSE To report a case of central retinal artery occlusion associated with hypertriglyceridemia. METHODS A 46-year-old man presented with decreased vision and was found to have a central retinal artery occlusion. Laboratory testing demonstrated markedly elevated trig-lycerides with no other abnormalities. Tests for cardiac and carotid embolic sources and hypercoagulability were negative. RESULTS Dietary management and medication compliance resulted in dramatically decreased serum triglyceride levels. Vision remained poor during follow-up. CONCLUSION Central retinal artery occlusion requires a medical evaluation for embolic and thrombotic sources. A fasting lipid panel is inexpensive to obtain and should be considered in the workup for central retinal artery occlusion.