Microcornea and Thickened Lens in Angle Closure following Nonsurgical Treatment of Retinopathy of Prematurity.

PURPOSE: To characterize the clinical features in young patients with angle closure and to determine the characteristics associated with acquired anterior segment abnormality following retinopathy of prematurity (ROP) treatment. METHODS: We performed two retrospective case-control series. In the first series, we identified consecutive young angle closure patients without prior surgeries, with and without a history of ROP treatment; in the second series we identified consecutive patients who underwent ROP treatment, without and without anterior segment changes. RESULTS: In the first series, 25 eyes of 14 consecutive angle closure patients were included: 19 eyes (11 patients, 78.6%) had a history of treated ROP, while 6 eyes (3 patients) belonged to full-term patients. The treated ROP eyes had significantly shallower anterior chambers (1.77 ± 0.17 mm vs 2.72 ± 0.18 mm, P < 0.0001) and thicker lenses (5.20 ± 0.54 mm vs 3.98 ± 0.20 mm, P = 0.0002) compared to the full-term controls. In the second series, 79 eyes of 40 patients were included, with median gestational age of 24.6 weeks. Acquired iridocorneal adhesion was noted in the eight eyes (10.1%) at a mean age of 4.7 years and was associated with prior zone 1 and plus disease (P = 0.0013), a history of initial intravitreal bevacizumab treatment (IVB, P = 0.0477) and a history of requiring additional IVB after initial treatment (P = 0.0337). CONCLUSIONS: Many young angle closure patients may have a history of treated ROP and may present with the triad of increased lens thickness, microcornea, and angle closure.

Subretinal Injection of Voretigene Neparvovec-rzyl in a Patient With RPE65-Associated Leber's Congenital Amaurosis.

Leber's congenital amaurosis (LCA) is a rare inherited retinal degeneration (IRD) that causes severe vision loss, nyctalopia, and nystagmus within the first few years of life. RPE65 gene mutations cause approximately 6% of LCA cases and have become the target for therapy since voretigene neparvovec-rzyl became the first U.S. Food and Drug Administration-approved gene therapy product for IRDs in 2017. The surgery involves pars plana vitrectomy with subretinal injection of a viral vector that carries a functional copy of the RPE65 gene. Intraoperative optical coherence tomography is a useful adjunctive tool to confirm the injection has reached the subretinal space.

Vitreoretinal lymphoma followed by systemic diffuse large B cell lymphoma.

Vitreoretinal lymphoma as the presenting diagnosis in association with a systemic lymphoma without central nervous system involvement is exceedingly rare, and the classification of this condition is not well-established. Here, we describe a patient with intermittent blurry vision in the left eye for 2 years in the setting of a recent incidental diagnosis of diffuse large B cell lymphoma from an axillary lymph node biopsy. The diagnosis of panuveitis with an extensive exudative retinal detachment was made. The patient was treated with pars plana vitrectomy as well as systemic chemotherapy, intrathecal methotrexate, intravitreal methotrexate, and intravitreal rituximab with good post-operative outcomes.

Differentiating Veins From Arteries on Optical Coherence Tomography Angiography by Identifying Deep Capillary Plexus Vortices.

PURPOSE: To introduce a simple method for differentiating retinal veins from arteries on optical coherence tomography angiography (OCTA). DESIGN: Cross-sectional pilot study. METHODS: Four default en face slabs including color depth encoded, grayscale full-thickness retina, superficial plexus, and deep capillary plexus (DCP) from nine 3×3-mm and nine 6×6-mm OCTA scans were exported and aligned. Nine ophthalmologists with minimum OCTA experience from 2 eye institutions were instructed to classify labeled vessels as arteries or veins in 3 stages. Classification was performed based on graders' own assessment at stage 1. Graders were taught that a capillary-free zone was an anatomic feature of arteries at stage 2 and were trained to identify veins originating from vortices within the DCP at stage 3. Grading accuracy was analyzed and correlated with grading time and graders' years in practice. RESULTS: Overall grading accuracy in stages 1, 2, and 3 was (50.4% ± 17.0%), (75.4% ± 6.0%), and (94.7% ± 2.6%), respectively. Grading accuracy for 3×3-mm scans in stages 1, 2, and 3 was (49.9% ± 16.3%), (79.2% ± 9.6%), and (96.9% ± 3.1%), respectively. Accuracy for 6×6-mm scans in stages 1, 2, and 3 was (51.4% ± 20.8%), (72.3% ± 7.9%), and (93.2% ± 3.3%), respectively. Grading performance improved significantly at each stage (all P < .001). No significant correlation was found between accuracy and time spent grading or between accuracy and years in practice (r = -0.164 to 0.617, all P ≥ .077). CONCLUSIONS: We describe a simple method for accurately distinguishing retinal arteries from veins on OCTA, which incorporates the use of vortices in the DCP to identify venous origin.

An Eight-Year-Old Girl Presents With Two Weeks of Vision Loss and Nightly Headaches.

Intraocular medulloepithelioma is a rare congenital tumor that arises from the nonpigmented epithelium of the ciliary body. It is the second most common primary intraocular neoplasm during the first decade of life. It may present with an iris mass or cyst (56%), glaucoma (48%), cataract (26%), leukocoria (18%), decrease in vision (41%), or pain (30%). Here, the authors present a case of a medulloepithelioma investigated with multimodal imaging, including the first characterization with intraoperative optic coherence tomography. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:314-317.].

Circumferential retinal hemorrhages after ophthalmic examination with scleral depression in an infant with anti-VEGF treated retinopathy of prematurity.

We report a case of retinal hemorrhages in a baby with retinopathy of prematurity (ROP) following examination with indirect ophthalmoscopy and scleral depression. There have been rare reports of examination-induced retinal hemorrhages during ROP screening, although those hemorrhages were diffusely scattered in the posterior pole. In this report the hemorrhages were found on the surface of the neovascular ridge. Changes in intraocular pressure caused by scleral depression may result in rupture of the fragile and immature retinal vessels, which have poor autoregulation in these premature babies. Ophthalmologists performing ROP screening examinations should be aware of the possibility of causing retinal hemorrhages with scleral depression, although the hemorrhages will resolve spontaneously.

A Novel Pathogenic Variant in NDP Gene With Incomplete Penetrance Manifests as X-Linked Familial Exudative Vitreoretinopathy.

Familial exudative vitreoretinopathy (FEVR) is a rare hereditary ocular disorder characterized by incomplete or abnormal development of peripheral retinal vasculature. The genes responsible for this disorder are associated with the wingless-related integration site (Wnt) signaling pathway, a critical pathway for the development of normal retinal vasculature. A pathogenic variant in any one of these genes may disrupt retinal vasculogenesis. Furthermore, the type and number of pathogenic variants may influence the severity of disease and clinical course. Here, the authors identify a novel pathogenic variant in the NDP gene, not previously described in the literature. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:120-124.].

Multi-drug resistant Mycobacterium chelonae scleral buckle infection.

PURPOSE: To describe a case of Multi-drug resistant Mycobacterium chelonae scleral buckle infection. OBSERVATIONS: A 56 year-old male with history of retinal detachment repair with scleral buckle 20 years prior presented with 8 months of intermittent pain and redness in the left eye. The patient was diagnosed with scleral buckle infection, the buckle was removed, and cultures revealed multi-drug resistant Mycobacterium chelonae. The postoperative course included orbital cellulitis treated with systemic linezolid, clarithromycin, and imipenem. All systemic antibiotics were discontinued on post-operative day 25, visual acuity improved to 20/25, the retina remained attached, and no recurrence occurred over 3 years of follow-up. CONCLUSIONS AND IMPORTANCE: NTM infections are typically chronic and often require lengthy treatment. SB infection is rare, but often associated with biofilm and antibiotic resistance. In spite of removing the SB, anchoring sutures, sheath surrounding the buckle and associated biofilm, a prolonged course of systemic antibiotics may be necessary in some patients.

Management of inadvertent needle penetration resulting in subretinal triamcinolone acetonide and retinal detachment.

PURPOSE: To report management of inadvertent needle penetration during subtenons triamcinolone acetonide administration resulting in retinal detachment. OBSERVATIONS: A 71-year-old female with history of diabetes, hypothyroidism, and mild myopia underwent subtenons triamcinolone acetonide (TA) injection in the right eye for nodular scleritis. There was unexpected patient movement concurrent with the injection resulting in needle penetration, subretinal and intravitreal injection of TA, superotemporal retinal break, and macula-involving retinal detachment. The patient underwent partial subretinal TA removal, successful retinal detachment repair, and recovered 20/25 visual acuity. CONCLUSIONS AND IMPORTANCE: In spite of prominent subretinal TA and retinal detachment, successful repair of retinal detachment and recovery of good visual acuity is possible.

Retinal vasproliferative tumor in a case of X-linked retinoschisis detachment.

PURPOSE: To describe the first published case of X-linked retinoschisis (XLRS) detachment with retinal vasoproliferative tumor (RVPT) and provide a literature review of the subject. OBSERVATIONS: The authors describe a case of a 17 year old male with X-linked retinoschisis who presented with a retinal detachment and a retinal vasoproliferative tumor. The patient was treated with pars plana vitrectomy, endolaser, subtenon's kenalog and anti-VEGF (vascular endothelial growth factor) intravitreal injections. He regained 20/60 vision with a flat macula and had significant resolution of the associated vasoproliferative leakage seen on fluorescein angiography. CONCLUSIONS AND IMPORTANCE: This case adds XLRS to the conditions associated with RVPT and gives support for treatment with laser photocoagulation and anti-VEGF therapy with bevacizumab to control the exudative process.

Clinical features, antimicrobial susceptibilities, and treatment outcomes of patients with culture positive endophthalmitis after penetrating keratoplasty.

PURPOSE: To report the clinical features organisms and treatment outcomes in patients with endophthalmitis after penetrating keratoplasty (PK). METHODS: Retrospective noncomparative case series. RESULTS: Eleven eyes of 11 patients with culture positive endophthalmitis after PK were included. The time to diagnosis of endophthalmitis from last PK was less than 1 week in 3/11 (27%), between 1 and 4 weeks in 3/11 (27%), and greater than one month in 5/11 (46%) (range 2-924 days). The distribution of isolates included gram positive (GP) 9/11 (82%), gram negative (GN) 1/11 (9%), and fungal 1/11 (9%) species, respectively. Of GP bacteria tested, 9/9 (100%) were sensitive to Vancomycin. Of fungal isolates tested, none (0/1) were sensitive to Amphoteracin, Fluconazole, and/or Voriconazole. Among patients with rim culture data available, 1/7 (14%) donor rims were culture positive for Candida glabrata and 6/7 (86%) were culture negative. Patients were treated with primary tap and inject in 10/11 (91%) and primary vitrectomy in 1/11 (9%). VA of ≥5/200 was present in 2/11 (18%) at time of endophthalmitis diagnosis, and was recorded in 6/11 (55%) at last follow-up. CONCLUSIONS AND IMPORTANCE: Patients with endophthalmitis after PK presented at variable time points after surgery. Gram positive organisms were the most common isolate. VA outcomes after treatment were generally poor.

Management of Retinopathy of Prematurity--Use of Anti-VEGF Therapy.

Retinopathy of prematurity (ROP) is a leading cause of childhood blindness worldwide. Although laser photocoagulation re-mains the gold standard for treating threshold and prethreshold disease (type 1 ROP), the off-label use of anti‒vascular endothelial growth factor (anti-VEGF) therapy to treat ROP is increasing. Benefits include acute regression of ROP, growth of retinal vasculature beyond the demarcation line, lesser degree of myopia and peripheral visual field loss, and avoidance of sedation and intubation required for laser. However, controversies regarding anti-VEGF in this vulnerable population persist including choice of anti-VEGF agent, dosing, systemic absorption, safety, and late recurrence. This review updates recent evidence regarding the use of anti-VEGF therapy in the management of ROP.

Clinical Features, Antibiotic Susceptibilities, and Treatment Outcomes of Endophthalmitis Caused by Staphylococcus epidermidis.

PURPOSE: To report the clinical features, antibiotic susceptibilities, and visual acuity outcomes with endophthalmitis caused by methicillin-sensitive Staphylococcus epidermidis and methicillin-resistant S. epidermidis. DESIGN: Retrospective case series. PARTICIPANTS: All patients seeking treatment at a tertiary referral center between 2006 and 2016 with endophthalmitis caused by S. epidermidis. METHODS: All records were reviewed for patients with a clinical diagnosis of endophthalmitis and positive vitreous culture results for S. epidermidis. Data were compared with the prior series at the same institution. MAIN OUTCOME MEASURES: Clinical settings, antibiotic susceptibilities, and visual acuity. RESULTS: Among 96 eyes of 96 patients, the most common postprocedural clinical settings were cataract surgery (47/96 [49%]), intravitreal injection (21/96 [22%]), trauma (8/96 [8%]), glaucoma surgery (7/96 [7%]), and penetrating keratoplasty (5/96 [5%]). The initial treatment included intravitreal vancomycin and ceftazidime in 89 of 96 eyes (93%) and intravitreal vancomycin and amikacin in 7 of 96 eyes (7%). A vitreous tap and injection with antibiotics was performed as the initial treatment in 83 of 96 eyes (86%) and pars plana vitrectomy was performed in 13 of 96 eyes (14%). All isolates were sensitive to vancomycin in both decades. In the most recent series, visual acuity at last follow-up was 5/200 or better in 68 of 96 eyes (71%) compared with 71 of 86 eyes (83%) in the prior study. In the current study, susceptibility to methicillin and moxifloxacin was present in 45 of 96 eyes (47%) and 29 of 85 eyes (34%), respectively, compared with 34 of 86 eyes (40%) and 27 of 39 eyes (69%) in the prior study. Final visual acuity was not significantly different between those eyes that were methicillin or fluoroquinolone sensitive and those that were resistant. CONCLUSIONS: In the current and prior series, all S. epidermidis isolates were sensitive to vancomycin. Visual acuity outcomes were not dependent on methicillin or fluoroquinolone sensitivity.

Antiphosphospholipid syndrome presenting with amaurosis fugax and cotton wool spots.

PURPOSE: To describe the importance of considering vaso-occlusive disease on the differential diagnosis of a patient presenting with amaurosis fugax (AF) and unilateral cotton wool spots (CWS). OBSERVATIONS: A 69-year-old female with history of obesity, hyperlipidemia and recent orthopedic surgery, presented with 3 days of worsening monocular AF and CWS in the right eye. She was diagnosed with antiphosphospholipid syndrome based on positive serologic testing for antiphosphatidylserine IgM, anticardiolipin IgM. The patient was treated with lipid lowering medication, long-term aspirin, and has followed a weightloss and physical therapy program under medical supervision. The CWS resolved and AF symptoms have not recurred. CONCLUSIONS AND IMPORTANCE: Antiphospholipid syndrome can be considered in the differential diagnosis of patients presenting with AF, assymetric CWS, and/or rapid progression of symptoms.

Comparison of Regional vs. General Anesthesia for Surgical Repair of Open-Globe Injuries at a University Referral Center.

PURPOSE: This study compares the clinical features and physician selection of either Regional Anesthesia (peribulbar or retrobulbar block) with Monitored Anesthesia Care (RA-MAC) or General Anesthesia (GA) for open globe injury repair. DESIGN: A non-randomized, comparative, retrospective case series at a University Referral Center. PARTICIPANTS: All adult repairable open globe injuries receiving primary repair between January 1st, 2004 and December 31st, 2014 (11 years). Exclusion criteria were patients less than 18 years of age and those treated with primary enucleation. METHODS: Data was gathered via retrospective chart review. MAIN OUTCOME MEASURES: Data collected from each patient was age, gender, injury type, location, length of wound, presenting visual acuity, classification of anesthesia used, duration of the procedure performed, months of clinical follow-up, and final visual acuity. RESULTS: During the 11 years study period, 448 patients were identified who had open globe injuries with documented information on zone of injury. Globe injury repair was performed using RA-MAC in 351/448 (78%) patients and general anesthesia in 97/448 (22%) patients. Zone 1, 2 and 3 injuries were recorded in 241, 135, and 72 patients respectively. The rates in specific zones, of RA-MAC versus GA were as follows: Zone 1 - 213/241 (88%) vs 28/241 (12%), Zone 2 - 104/135 (77%) vs 31/135 (23%) and Zone 3 - 34/72 (47%) vs 38/72 (53%). Open globe injuries repaired under RA-MAC had significantly shorter wound length (p<0.001), more anterior wound location (p<0.001) and shorter operative times (p<0.001). RA-MAC cases also had a better presenting and final visual acuity (p<0.001). Neither class of anesthesia conferred a greater visual acuity improvement (p=0.06). The use of GA did not cause any delay in the time elapsed from injury until surgical repair (p=0.74). CONCLUSIONS: RA-MAC is a reasonable alternative to GA for the repair of open globe injuries in selected adult patients. RA-MAC was selected more often for Zone 1 and Zone 2 injuries. For eyes with Zone 3 injuries, there are equal selection ratio for RA-MAC and GA.

Adenoid cystic carcinoma of the lacrimal gland in a 14-year-old male.

Adenoid cystic carcinoma (ACC) of the lacrimal gland is an aggressive, malignant epithelial neoplasm. This tumor is rarely seen in adults and even less commonly seen in children and adolescents; thus, there have been no large studies to date describing the optimal treatment of this malignancy in the pediatric population. Here, we report a case of lacrimal gland ACC in a 14-year-old male treated with neoadjuvant intra-arterial chemotherapy followed by globe-sparing tumor resection and chemoradiation. At 2-year follow-up, he remains disease free without evidence of tumor recurrence.

Avascular Retinal Findings in a Child With Achondroplasia.

The authors present clinical and angiographic findings in a 12-year-old girl with achondroplasia who presented with bilateral retinal peripheral nonperfusion and unilateral rhegmatogenous retinal detachment, which has not been previously described in achondroplasia. This report contributes incremental knowledge regarding aberrant retinal vascular phenomena observed in pediatric disease states and implicates the possible role of mutations in the FGFR3 gene in peripheral vascular abnormalities. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:272-274.].

Assessing the Effectiveness of Arnica montana and Rhododendron tomentosum (Ledum palustre) in the Reduction of Ecchymosis and Edema After Oculofacial Surgery: Preliminary Results.

PURPOSE: The purpose of this study was to provide preliminary data on the potential effectiveness of the combination of topical Arnica montana and Rhododendron tomentosum (Ledum palustre) in reducing postoperative ecchymosis and edema in a select population of healthy volunteers after oculofacial surgery. METHODS: This retrospective review examined the postoperative course of healthy volunteers using topical Arnica and Ledum after undergoing common oculofacial procedures, including blepharoplasty, browpexy, and rhinoplasty, in the hands of 4 surgeons at tertiary referral centers from July 1, 2012 to December 31, 2012 using medical records review. Each patient included had used topical hydrogel pads (OcuMend, Cearna Inc., Chicago, IL) containing Arnica 50 M (10) 50% and Ledum 50 M (10). The pads were applied bilaterally after surgery through postoperative day 6. At each postoperative visit, the patients were evaluated by their respective surgeons and assigned a subjective physician-patient rating score comparing each patient's observed healing compared with expected healing if not using Arnica/Ledum. Photographs of patients undergoing equivalent procedures, but not using Arnica/Ledum were used as controls for comparison. Physician-patient rating scores were categorical: markedly accelerated healing defined as approximately 7 days ahead of expected, accelerated healing, defined as <7 days ahead of expected, and no appreciable difference from expected. The proportion of patients with each physician-patient rating score was calculated for postoperative days 1 to 2, 3 to 5, 6 to 8, and overall. Difference of proportions was calculated with 95% confidence intervals using Newcombe unpaired difference comparison of proportions. Photographs documenting the clinical progression of selected patients are provided. RESULTS: A total of 27 patients (16 females, 11 males) were included in the study. Age range was 18 to 70 years. The majority of patients were white (52.9%), and underwent blepharoplasty (78.9%). The median duration of follow-up was 7 days, range 1 to 14 days. The proportions of patients with markedly accelerated healing were 38.5% (5 of 13), 85.7% (6 of 7), 60.0% (12 of 20), and 51.9% (14 of 27) at POD 1 to 2, 3 to 5, 6 to 8, and overall, respectively. The proportions of patients with accelerated healing at the same time points were 15.4% (2 of 13), 14.3% (1 of 7), 30.0% (6 of 20), and 37.0% (10 of 27), respectively. The proportions of patients with no appreciable difference at the same time points were 46.2% (6 of 13), 0% (0 of 7), 10.0% (2 of 20), and 11.1% (3 of 27) of patients, respectively. The proportion of patients using Arnica/Ledum with markedly accelerated healing was significantly more than the proportion of those demonstrating no appreciable difference from expected at POD 3 to 5 (85.7% vs. 0%, p = 0.05), POD 6 to 8 (60.0% vs. 10.0%), and overall (51.9% vs. 11.1%, p = 0.05). No adverse effects were reported. CONCLUSIONS: The preliminary results from this study demonstrate that the combination of topical Arnica montana and Rhododendron tomentosum (Ledum palustre) may be effective in reducing postoperative ecchymosis and edema after oculofacial surgery.

Epidemiology of Herpes Zoster Ophthalmicus: Recurrence and Chronicity.

PURPOSE: A hospital-based epidemiology study to describe herpes zoster ophthalmicus (HZO) prevalence and risk factors for recurrent and chronic disease. DESIGN: Retrospective, hospital-based cohort study. PARTICIPANTS: All patients evaluated in the Broward and Miami Veterans Administration Healthcare System (MIAVHS) during the study period. METHODS: Retrospective medical record review of patients seen in the MIAVHS from January 1, 2010, through December 31, 2014, with a HZO clinical diagnosis. Assessment of the patient's clinical course was defined by the following: an acute episode of HZO was defined as quiescence of disease within 90 days of initial presentation, HZO recurrence was defined as any recurrent eye disease or rash 90 days or more after quiescence of disease was noted off therapy, and chronic HZO was defined as active disease persisting more than 90 days from initial presentation. MAIN OUTCOME MEASURES: Main outcome measures included the frequency of HZO with and without eye involvement, HZO recurrence rates, and risk factors for recurrent or chronic HZO. RESULTS: Ninety patients with HZO were included in the study. The mean age at incident episode of HZO was 68±13.8 years (range, 27-95 years). Most patients were white (73%), immune competent (79%), and did not receive zoster vaccination at any point during the follow-up (82%). Patients were followed for a mean of 3.9±5.9 years (range, 0-33 years). The period prevalence of HZ in any dermatome was 1.1%, the frequency of HZ involving V1 (HZO) was 0.07%, and the frequency of HZO with eye involvement was 0.05%. The overall 1-, 3-, and 5-year recurrence rates for either recurrent eye disease or rash were 8%, 17%, and 25%, respectively. Ocular hypertension (hazard ratio [HR], 4.6; 95% confidence interval [CI], 1.3-16.5; odds ratio [OR], 6.7; 95% CI, 1.5-31.2) and uveitis (HR, 5.7; 95% CI, 1.7-19.0; OR, 6.7; 95% CI, 1.5-31.2) increased the risk of recurrent and chronic disease. CONCLUSIONS: This study supports newer data indicating that a significant proportion of patients experience recurrent and chronic HZO. Further study is needed to guide preventative and therapeutic approaches to recurrent and chronic HZO.