Benjamin J. Thomas

Familial Exudative Vitreoretinopathy: Spectral-Domain Optical Coherence Tomography of the Vitreoretinal Interface, Retina, and Choroid

PURPOSE The in vivo microstructural features of familial exudative vitreoretinopathy (FEVR) have not been well described. We present new anatomic features of FEVR with functional and genetic correlations. DESIGN Consecutive, retrospective, observational case series. PARTICIPANTS Patients with FEVR treated from 2009 to 2014. METHODS We identified 346 patients with FEVR. Those imaged with spectral-domain optical coherence tomography (SD OCT) with or without enhanced depth imaging (EDI) were included, and images were correlated with best-corrected visual acuity (BCVA), widefield angiography, fundus autofluorescence (AF), and wnt signaling pathway mutations. MAIN OUTCOME MEASURES Exploratory SD OCT findings and BCVA. RESULTS A total of 225 imaging sessions were acquired in 74 eyes from 41 patients. Mean age was 19.0 years. Sixty-seven eyes (91%) had interpretable images, of which 50 (75%) had anomalous microstructural findings; all eyes with FEVR severity of stage 2 or greater had abnormalities. A broad spectrum of features were identified: various forms of posterior hyaloidal organization, vitreomacular traction (VMT), vitreopapillary traction, vitreo-fold traction, vitreo-laser scar adhesion, diminished foveal contour, persistent fetal foveal architecture, cystoid macular edema (CME), intraretinal exudates and subretinal lipid aggregation, dry or edematous radial folds, and disruption of the ellipsoid zone. Mean foveal, central macular, and choroidal thicknesses were 305±145 μm, 337±160 μm, and 216±64 μm, respectively. In stages 1 to 2, greater foveal and central macular thicknesses (Rho=0.493, 0.544, respectively; both P<0.001) correlated with poorer BCVA, but not choroidal thickness (Rho=0.032; P=0.868). Posterior hyaloidal organization (P<0.001), VMT (P<0.001), CME (P<0.001), exudation (P<0.001), and disruption of the ellipsoid zone (P<0.001) were associated with poorer BCVA. Disruption of the ellipsoid zone (β=0.699; P<0.001) and posterior hyaloidal organization (β=0.289; P=0.011) retained significance in multivariate modeling (R2=0.627; P<0.001). Spectral-domain OCT detected all cases of angiographic edema and areas of outer retinal dysfunction that were hypoautofluorescent on AF. Microstructural-genetic associations were not identified. CONCLUSIONS Spectral-domain OCT imaging identified microstructural anomalies in the majority of patients with FEVR.

Hybrid 25- and 27-Gauge Vitrectomy for Complex Vitreoretinal Surgery.

BACKGROUND AND OBJECTIVE The authors report the technique of using the 27-gauge (G) vitreous cutter through 25-G valved cannulas to allow hybrid instrumentation of both gauges. PATIENTS AND METHODS Vitrectomy is initiated with standard placement of 25-G valved cannulas, followed by insertion of a 27-G vitreous cutter through the 25-G cannulas. RESULTS The hybrid procedure emphasizes the advantages of both platforms: The 25-G cutter is more efficient for core vitrectomy and is more rigid to facilitate peripheral vitrectomy; the 25-G platform enjoys a wider armamentarium of instrumentation options; and the smaller profile of the 27-G cutter can be maneuvered more easily into tight surgical planes to act analogous to vertical scissors, with the added benefits of aspiration and spatula-like features. The authors illustrate this technique in three cases: diabetic tractional retinal detachment with dense plaques, posterior stage 4B retinopathy of prematurity, and sutureless scleral fixation of an intraocular lens. CONCLUSIONS Hybrid use of the 25-G and 27-G platforms offers greater versatility for the management of complex vitreoretinal conditions.

Progressive Retinal Detachment In Infants With Retinopathy Of Prematurity Treated With Intravitreal Bevacizumab Or Ranibizumab

Purpose: Fibrovascular contraction and tractional retinal detachment (TRD) are recognized complications associated with the use of anti–vascular endothelial growth factor agents in vasoproliferative vitreoretinopathies. The authors characterize TRDs that developed after intravitreal bevacizumab or ranibizumab therapy for vascularly active retinopathy of prematurity. Methods: This is an international, multicenter, interventional, retrospective, case series. Thirty-five eyes from 23 infants were included. Inclusion required anti–vascular endothelial growth factor treatment of Type 1 retinopathy of prematurity with progression to TRD. Results: Mean gestational age was 26 ± 2 weeks, and mean birth weight was 873 ± 341 g. Mean postmenstrual age on the day of injection was 35 ± 2 weeks. Retinal detachment was noted a mean of 70 days (median, 34; range, 4–335) after injection. Eleven percent detached within 1 week, 23% within 2 weeks, and 49% within 4 weeks. The highest stage of retinopathy of prematurity noted was 4A in 29%, 4B in 37%, and 5 in 34% of eyes. Time to RD negatively correlated with postmenstrual age at the time of injection (Rho = −0.54; P < 0.01). Three TRD configurations were observed: 1) conventional peripheral elevated ridge or volcano-shaped Stage 5 detachment, 2) midperipheral detachment with tight circumferential vectors, and 3) very posterior detachment with prepapillary contraction. Full or partial reattachment was achieved with surgical intervention in 86% of eyes. Conclusion: Progressive atypical TRD may occur after anti–vascular endothelial growth factor injections for retinopathy of prematurity. The configuration of the detachment varies with the extent of primary retinal vascularization present at the time of treatment.

THE CUTTING EDGE OF RETINOPATHY OF PREMATURITY CARE: Expanding the Boundaries of Diagnosis and Treatment

Purpose: To discuss the latest advances and controversies in the diagnosis and care of infants with retinopathy of prematurity (ROP). Methods: Literature review. Results: Retinopathy of prematurity remains a major global issue. Industrialized nations now treat profoundly premature infants with posterior and aggressive disease, and middle-income nations are experiencing ROP epidemics. Remote digital imaging may address the decreasing ratio of ROP providers to premature infants, in addition to improving patient care. Widefield angiography, optical coherence tomography, and the Wnt signaling pathway have provided new insights into ROP pathogenesis. Anti–vascular endothelial growth factor treatment is increasing in popularity, but the dearth of information to guide dosing, unpredictable reactivation, persistent vascular abnormalities, the “crunch” phenomenon, and the presently unknown effects of systemic vascular endothelial growth factor suppression remain issues to continue investigating. Neurodevelopmental delay has been raised as a potential consequence, but the evidence currently is weak. Vitrectomy is the treatment of choice for Stages 4 and 5. Illumination techniques, ab interno incisions, plasmin-assisted vitrectomy, staged surgery in the interest of corneal clearing for advanced Stage 5, and immediate sequential bilateral vitreoretinal surgery, are useful techniques. Conclusion: We are making progress in ROP management. Our goal as clinicians is to continue expanding the boundaries of our abilities to keep this blinding disease in check globally.

RETINAL VASCULAR TORTUOSITY AND EXUDATIVE RETINOPATHY IN A FAMILY WITH DYSKERATOSIS CONGENITA MASQUERADING AS FAMILIAL EXUDATIVE VITREORETINOPATHY

Purpose: To report a novel presentation of dyskeratosis congenita masquerading as familial exudative vitreoretinopathy. Methods: Observational case series involving single family and literature review. Results: A brother and sister were diagnosed with familial exudative vitreoretinopathy at ages 4 and 2, respectively. Both patients were managed with laser photocoagulation. Eight years after the initial presentation, both siblings developed pancytopenia secondary to bone marrow failure. Laboratory work-up revealed severely shortened telomere length in both patients, and genetic testing revealed a missense mutation in the gene that encodes the reverse transcriptase component of telomerase, confirming the diagnosis of dyskeratosis congenita. The father of both children was a carrier of the same mutation, who exhibited marked retinal vascular tortuosity of the second-order vessels. Conclusion: Dyskeratosis congenita is a severe multisystem disorder, which should be considered in cases of pediatric exudative retinopathies with concurrent signs and/or symptoms of bone marrow failure.

MICROBIOLOGIC SPECTRUM AND VISUAL OUTCOMES OF ACUTE-ONSET ENDOPHTHALMITIS UNDERGOING THERAPEUTIC PARS PLANA VITRECTOMY.

Purpose: To report the clinical presentation, microbiologic spectrum, and visual outcomes associated with acute-onset infectious endophthalmitis undergoing therapeutic pars plana vitrectomy. Methods: Multicenter interventional retrospective noncomparative consecutive case series. Billing records were reviewed to identify all charts for patients undergoing pars plana vitrectomy within 14 days of diagnosis of acute-onset infectious endophthalmitis over a 4-year period at 5 large tertiary referral retina practices. Statistical analysis was performed to assess for factors associated with visual outcomes. Results: Seventy patients were identified. The most common clinical setting was postcataract surgery (n = 20). Only 3 patients (4.3%) presented with 20/400 or better visual acuity (VA). Although most of the patients initially underwent vitreous tap and intravitreal antibiotic injection (n = 47, 67.1%), all patients eventually underwent pars plana vitrectomy within 14 days of presentation with 68.5% (48/70) of patients undergoing pars plana vitrectomy within 48 hours of presentation. Positive intraocular cultures were obtained in 56 patients (80%). The most common identified organism was Streptococcus sp (n = 19). Visual acuity at last follow-up was 20/400 or better in 19 patients (27.1%). Three patients underwent evisceration or enucleation (4.3%). Last recorded postoperative VA (mean LogMAR 1.99 ± 0.94, Snellen VA equivalent finger count) improved from presenting VA (mean LogMAR 2.37 ± 0.38, Snellen VA hand motions) (P ⩽ 0.001). There was no statistically significant correlation between the underlying etiology or the timing of surgery with this VA outcome. Conclusion: Although less than one-third of patients achieved 20/400 or better VA, this VA often improved significantly from presenting VA.

WIDE-FIELD IMAGING OF NONEXUDATIVE AND EXUDATIVE CONGENITAL X-LINKED RETINOSCHISIS.

Purpose: To describe wide-field imaging features of patients with congenital X-linked retinoschisis. Methods: This is a retrospective nonconsecutive series of 36 eyes from 18 patients with congenital X-linked retinoschisis from 2008 to 2014. Wide-field color fundus photographs, optical coherence tomography images, and wide-field fluorescein angiography images were reviewed. Patients were classified to have either exudative or nonexudative retinoschisis based on the presence or absence of lipid exudates. Results: Eleven eyes exhibited exudative retinoschisis (30%), whereas the remaining were nonexudative. Exudative disease occurred more commonly in older patients (14.4 vs. 4.0 years; P < 0.001). The most frequent location of exudation was the macula. Subretinal hemorrhage was present in 4 eyes (11%). Macular findings included an atypical foveal avascular zone in 7 eyes (19%) and submacular fibrosis or retinal folds in 6 eyes (17%). Peripheral characteristics included fibrosis or folds (11%), bridging vessels (8%), and vascular sheathing (8%). Thirteen of the 22 eyes (59%) demonstrated leakage on fluorescein angiography. Conclusion: Exudation may be more common in congenital X-linked retinoschisis than previously recognized. The presence of exudates with concurrent angiographic leakage suggests that exudation may be due to chronic vascular permeability and not solely caused by intraschisis hemorrhage, which has been classically described.

A Comparison of Intraoperative Dexamethasone Intravitreal Implant and Triamcinolone Acetonide Used During Vitrectomy and Epiretinal Membrane Peeling: A Case Control Study.

BACKGROUND AND OBJECTIVE Intravitreal corticosteroids may accelerate the normalization of macular morphology after vitrectomy and epiretinal membrane (ERM) peeling. The authors compared the visual and anatomic outcomes of eyes undergoing this procedure with the intraoperative use of the dexamethasone intravitreal implant (DEX) (Ozurdex; Allergan, Irvine, CA) versus triamcinolone acetonide (IVTA) (Triesence; Alcon, Fort Worth, TX). PATIENTS AND METHODS Interventional, retrospective, consecutive, case-control study. RESULTS Fifty-five participants were included (DEX = 19; IVTA = 36). Best-corrected visual acuity (BCVA) improved at 1, 2, 3, and 6 months in both the DEX (P = .40, .45, .02, .08, respectively) and IVTA (P < .01, = .10, < .01, < .01) arms. Similarly, central macular thickness (CMT) improved at 1, 2, 3, and 6 months in both the DEX (P < .01, = .03, < .01, < .01) and IVTA (P < .01, < .01, < .01, < .01) arms. There were no statistical differences between DEX and IVTA regarding BCVA and CMT. CONCLUSION Both intraoperative DEX and IVTA, used at the completion of vitrectomy and at membrane peeling for epiretinal membrane, were effective in improving visual and anatomic outcomes, though there was a trend toward greater significance with IVTA.

Endoilluminator phototoxic maculopathy associated with combined ICG-assisted epiretinal membrane and internal limiting membrane peeling

Phototoxic maculopathy caused by endoillumination during macular surgery is uncommon. Previously identified risk factors have included intensity of the light source, proximity to the retinal surface, and length of exposure. In the era of indocyanine green (ICG)-assisted internal limiting membrane (ILM) peeling, the use of ICG, and the technique of ILM peeling may both contribute to subsequent phototoxic maculopathy. We present cases of routine chromovitrectomy who developed phototoxic maculopathy in the precise discrete distribution of the ILM rhexes, and discuss potential mechanisms and implications.

PARS PLANA VITRECTOMY FOR LATE VITREORETINAL SEQUELAE OF INFECTIOUS ENDOPHTHALMITIS: Surgical Management and Outcomes

Purpose: To report the visual acuity outcomes after pars plana vitrectomy for delayed vitreoretinal sequelae of infectious endophthalmitis. All eyes were initially treated with intravitreal antibiotics (Abx). Methods: Multicenter, retrospective, consecutive case series. Results: Forty-two eyes met the study criteria. The mean follow-up was 48 weeks (SD ± 61.8). Mean interval from Abx to pars plana vitrectomy was 13 weeks (SD ± 14.3, range 2–70). Indications for pars plana vitrectomy included vitreous opacities (VO) (n = 22), epiretinal membrane (n = 9), and retinal detachment (n = 11). LogMAR visual acuity improved from 1.87 (Snellen equivalent: 20/1,482) preoperatively to 1.35 (Snellen equivalent: 20/447) at final evaluation (P < 0.001). LogMAR visual acuity improved significantly for patients with vitreous opacities (P < 0.01) and retinal detachment (P = 0.02) but not for patients with epiretinal membranes (P = 0.08). Conclusion: Patients with infectious endophthalmitis can gain vision if they have a pars plana vitrectomy for delayed sequelae such as vitreous opacities or for retinal detachment.