Faculty Bibliography

PURPOSE/OBJECTIVE:To determine whether testosterone supplementation is associated with retinal artery occlusion (RAO) or retinal vein occlusion (RVO). METHODS:Retrospective matched cohort study using data from a large national U.S. insurance database. The testosterone cohort consisted of all male patients who filled a prescription for testosterone from 2000 to 2013. Five controls were matched on age (±3 years), sex, race, and similar time in plan (±3 months) for every exposed patient. Exclusion occurred for <2 years in the plan, <1 eye care visit, medications known to affect androgen levels, and systemic diseases associated with occlusions or increased testosterone. Cox proportional hazard regression assessed the hazard of a new diagnosis of RAO or RVO while controlling for age, race, diabetes mellitus, and hypertension. RESULTS:A total of 35,784 incident testosterone users were compared with 178,860 matched controls. Ninety-three (0.3%) RAOs and 50 (0.1%) RVOs were found in the testosterone cohort and contrasted with 316 (0.2%) RAOs and 232 (0.1%) RVOs in the control group. After multivariate analysis, testosterone supplementation significantly increased the hazard of RAO (hazard ratio: 1.43, 95% confidence interval: 1.12-1.81, P = 0.004), but not of RVO (hazard ratio: 1.03, 95% confidence interval: 0.74-1.42, P = 0.86). CONCLUSION/CONCLUSIONS:Although the incidence of RAO and RVO is low in users of testosterone, supplementation therapy is associated with an increased hazard of RAO, but apparently not of RVO.

An 8-year-old girl with a history of type 1 diabetes was referred for an evaluation of pigment changes in the right macula. Curvilinear hypopigmented streaks were noted in the fovea, with corresponding disruption of the photoreceptor layer on spectral domain optical coherence tomography. On further questioning, a history of laser pointer play 2 years prior was elicited, with acknowledgement of direct laser pointing in the child's eye. The family was advised to remove any commercial laser devices from the home, and observation of the pathology was recommended.

BACKGROUND AND OBJECTIVE/OBJECTIVE:Anti-vascular endothelial growth factor (VEGF) therapy is increasing in popularity for treatment of retinopathy of prematurity (ROP). Despite many technical benefits, issues remain prompting further investigation. PATIENTS AND METHODS/METHODS:Retrospective case report and literature review. RESULTS:A 42-week-old postmenstrual age female with gestational age of 28 weeks and birth weight of 990 g presented with prominent progression of peripapillary purely tractional atypical stage 4A ROP in both eyes following intravitreal bevacizumab therapy in the right eye only. CONCLUSION/CONCLUSIONS:The authors present the first reported case, to their knowledge, of a "crunch" phenomenon tractional retinal detachment from fellow eye administration of bevacizumab. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:e102-e104.].

The authors report three cases of persistent fetal vasculature (PFV) presenting with an atypical peripheral fibrovascular stalk. A 5-month-old with congenital exotropia with a vitreous hemorrhage in the right eye underwent vitrectomy, and a mid-peripheral vitreal vascular stalk was visualized within the area of hemorrhage. The second case is of an 8-year-old with two fetal vascular remnants in the right eye: (1) a stalk superotemporally extending from the pars plana to the mid-peripheral retina that was associated with a retinal break and (2) a focal lens opacity. The third case is a 12-year-old with a history of familial exudative vitreoretinopathy and peripapillary choroidal neovascular membrane found to have a peripheral vitreal stalk in the right eye. These cases demonstrate a rare form of PFV with primarily peripheral remnants. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:e83-e88.].

Purpose : In many resource-challenged communities, there is an unmet need for coste ffective, portable, reliable, and easy-to-use, retinal photography to aid in the screening and diagnosis of posterior segment eye diseases to help prevent blindness. Smartphonebased fundus photography offers an elegant solution. While this technology is rapidly evolving in adults, it has lagged behind in the pediatric patient population. This study presents a feasibility analysis of a novel smartphone-based retinal imaging device tailored to pediatric retinal care. Methods : Our existing RetinaScope device, utilizing the smartphone's camera, Bluetooth connectivity, and processing power to perform 100-degree montaging, was modifed for use in a pediatric setting. This included a child-friendly 3D printed housing of animals, attention grabbing targets, enhanced image stitching and video-recording capability to capture continuous clips of the fundus exam. Fundus photographs were acquired in a pediatric outpatient clinic, inpatient bedside, emergency room, and during exam under anesthesia. The ease of image acquisition and comfort of the patient were assessed via a validated 5-point survey. Time for image acquisition per eye was also recorded. A subset of the images was graded by two independent, masked, expert graders and the accuracy of the grader to clinician diagnosis was compared. The study was approved by the University of Michigan Institutional Review Board Committee and has ClinicalTrials.gov Identifier NCT03076697. Results : Fundus photographs were acquired in 62 eyes of 42 patients. Mean age of the cohort was 6.7 years (range 6 weeks to 18 years). Average time to acquire a standard 5 field photograph of each eye was 2.3 min +/- 1.1 min. Patients rated their overall experience of image acquisition favorably, average Likert score 4.6 +/- 0.8. The diagnoses included: Coats disease, retinoblastoma, retinopathy of prematurity, commotio retinae, coloboma, ocular albinism, and optic nerve hypoplasia, among others. A sub-set of images was graded by two masked vitreoretinal specialists. There was 93% agreement between imagebased diagnosis and the treating clinician's diagnosis. Conclusions : We report a novel smartphone-based device for wide-field fundus photography in pediatric patients. Our device is able to rapidly acquire diagnostic quality images of the retina without causing undue distress to the patients

Purpose/UNASSIGNED:To report 2 cases of chronic macular detachment associated with peripheral retinoschisis in which surgical repair resulted in significant visual recovery. Observations/UNASSIGNED:A 44-year-old man and 60-year-old woman were evaluated for chronic macular detachment, with a duration of 5 years and 6 months, respectively. In each case, optical coherence tomography was used to establish a diagnosis of full-thickness macular detachment resulting from peripheral retinoschisis and to confirm or identify the pathogenic outer layer breaks. After surgical repair with pars plana vitrectomy, endolaser photocoagulation of outer layer breaks, and gas tamponade, both patients had significant improvement in vision. The best-corrected visual acuity improved from 20/50- to 20/20 at one year post-operatively in the first patient and from 20/1250 to 20/200 at 8 months post-operatively in the second. Conclusion and importance/UNASSIGNED:In cases of chronic schisis-detachment involving the macula, surgical intervention can sometimes result in unexpected levels of visual recovery.

PURPOSE/OBJECTIVE:To compare functional and anatomic outcomes of treatment with intravitreal aflibercept versus bevacizumab and/or ranibizumab in patients with recurrent macular edema (ME) secondary to central retinal vein occlusion (CRVO). METHODS:Retrospective, comparative case series of patients with recurrent ME in the setting of CRVO. Patients with recurrent ME received treatment with aflibercept (Group 1, G1) or bevacizumab and/or ranibizumab (Group 2, G2). Primary outcome measures were best-corrected visual acuity (BCVA) and central foveal thickness (CFT). RESULTS:Of the 20 eyes (20 patients) with recurrent ME included in the study, 9 received aflibercept (G1) and 11 received bevacizumab and/or ranibizumab (G2). Median BCVA at recurrence of ME and at most recent follow-up was 20/60 (G1) and 20/80 (G2) and 20/40 (G1) and 20/50 (G2, P > 0.05 for all comparisons), respectively. Median CFT at recurrence of ME and at most recent follow-up was 492 μm (G1) and 448 μm (G2) and 291 μm (G1) and 295 μm (G2, P > 0.05 for all comparisons), respectively. Complete resolution of ME for at least 4 months was found in 78% (G1) and 55% (G2) of patients with a median injection free interval of 11 (G1) and 13 (G2) months (P > 0.05). CONCLUSIONS:In patients with recurrent ME secondary to CRVO, there was improvement in BCVA and CFT in all groups, although patients treated with aflibercept showed a trend toward better anatomical outcomes decreased need for recurrent injections.

We present an updated clinical review of the pathophysiology, progression, and current treatments in pediatric patients with congenital X-linked retinoschisis (CXLRS). CXLRS is an X-linked inherited retinal degeneration characterized by splitting of the superficial layers of the retina. Most recent classification divides CXLRS into 4 distinct clinical phenotypes: type 1, foveal; type 2, foveolamellar; type 3, complex; and type 4, foveoperipheral. The majority of retinoschisis cavities remain stable throughout life and may spontaneously collapse. However, a select number of patients progress to macula-involving peripheral retinoschisis, rhegmatogenous, and combined tractional-rhegmatogenous detachments that require further intervention. Although several advances have been made over the past several decades, medical therapy remains limited to case series‒based carbonic anhydrase therapy and prophylactic laser retinopexy. Recent advances in genetic-based clinical trials with the retinoschisis gene are promising. Vitreoretinal surgical approaches remain complex, case-based, and require careful planning depending on the configuration and location of the retinoschisis cavity.

PURPOSE: To develop an anatomical classification scheme for combined hamartoma of the retina and retinal pigment epithelium (RPE) and specify recommendations for follow-up interval. METHODS: Retrospective review of patients with combined hamartoma of the retina and RPE examined during a 7-year period (2008-2015). The clinical presentation, fundus examination, and optical coherence tomography were analyzed. RESULTS: Lesions were classified based on location, fundus features, and optical coherence tomography findings. Lesion location: macular/peripapillary-Zone 1; mid-periphery-Zone 2; and far periphery-Zone 3. Associated fundus findings: no retinal traction-Stage 1; retinal traction and/or retinoschisis-Stage 2; and retinal detachment-Stage 3. Optical coherence tomography findings: epiretinal component only-A; partial retinal involvement-B; and complete retinal and RPE involvement-C. Complete ophthalmologic evaluation is recommended at least every 6 months for patients younger than 12 years, with more frequent follow-up in patients with: lesions in the macula/peripapillary (Zone 1) or with retinal traction, retinoschisis, or retinal detachment (Stage 2 and 3). Surgical intervention is recommended in patients with vision loss secondary to macular traction or retinal detachment. CONCLUSION: A new clinical classification system is proposed for evaluating and managing patients with combined hamartoma of the retina and RPE. The zone and stage of combined hamartoma of the retina and RPE lesion will assist in determining follow-up interval and surgical intervention. Application of a uniform classification scheme will facilitate assessment and comparison of findings across different studies.

PURPOSE: To report an unusual case of ciliochoroidal effusion and presumed acute macular neuroretinopathy associated with cervical traction therapy for the treatment of cervical spinal stenosis. METHODS: Case report. RESULTS: A 75-year-old man reported sudden onset of a wedge-shaped paracentral scotoma in the right eye. Fundus examination showed a ciliochoroidal effusion in the right eye. Optical coherence tomography revealed intraretinal fluid in both eyes without leakage on fluorescein angiography. B-scan ultrasonography and anterior segment ultrasound biomicroscopy of the right eye showed peripheral ciliochoroidal effusion. He had recently started intensive cervical traction therapy for the treatment of cervical spinal stenosis. There was spontaneous resolution of the choroidal effusion and intraretinal fluid after stopping cervical traction treatments. Optical coherence tomography imaging after resolution of the intraretinal fluid revealed thinning of the outer nuclear layer and attenuation of the ellipsoid and interdigitation zones corresponding to a persistent paracentral scotoma, consistent with acute macular neuroretinopathy. CONCLUSION: This is the first report of adverse ocular effects of cervical traction. We postulate that venous and arterial compromise during cervical traction therapy resulted in both ciliochoroidal effusion and a watershed infarct in the outer retina.