A Case of the Blues-Colored Pencil Orbitopathy in an 18-Month-Old Boy.

Orbital penetrating injuries from pencils are rare. This report describes a case of penetrating orbital injury in a young child with a retained blue-colored pencil core foreign body, which led to rapid onset of orbital abscess requiring surgical drainage. Intraoperatively, orbital tissues were stained a bright-blue color. Histopathological study of specimen also highlighted bright blue aggregates of foreign material infiltrating the orbital tissues. Cultures grew Streptococcus mitis and Exophiala jeanselmei , which are rare causes of orbital abscess. Unique properties of retained colored pencil core as compared to graphite pencil core are herein discussed. Due to differences in composition, colored pencil core foreign bodies may require more timely surgical intervention compared to noncolored graphite pencil core.

CHORIORETINITIS SCLOPETARIA AND ORBITAL EMPHYSEMA CAUSED BY A HIGH-VELOCITY LIQUID MISSILE.

PURPOSE: To highlight a case of chorioretinitis sclopetaria, with concomitant macular hole formation and orbital emphysema, caused by a commercial-grade pressure washer. METHODS: This is a retrospective case report. RESULTS: A 19-year-old man presented to the emergency department with a left eye injury, incurred after being sprayed with a commercial-grade pressure washer. He endorsed ipsilateral blurred vision, pain, and linear floaters. Left eye visual acuity was 20/40. Dilated fundus examination showed inferior vitreous hemorrhage, retinal whitening, and preretinal, intraretinal, and subretinal hemorrhages, consistent with chorioretinitis sclopetaria. Optical coherence tomography revealed a full-thickness macular hole. Computed tomography scan of the orbits showed subcutaneous and postseptal orbital emphysema. Two months after injury, vitreous and retinal hemorrhages and macular hole resolved. Five months after injury, visual acuity improved to 20/20. CONCLUSION: Chorioretinitis sclopetaria is defined as a full-thickness chorioretinal disruption resulting from a high-velocity projectile passing adjacent to or into the orbit without penetrating the globe. Chorioretinal deformation and ocular comorbidities are influenced by the velocity of the missile and its spatial relationship to the orbit. Although this pattern of injury is typically associated with indirect trauma to the globe by a BB or a bullet, this is the first report of chorioretinitis sclopetaria precipitated by a high-velocity liquid missile.

Outcomes of Patients With Thyroid Eye Disease Partially Treated With Teprotumumab.

PURPOSE: In response to the coronavirus (COVID-19) pandemic, teprotumumab production was temporarily halted with resources diverted toward vaccine production. Many patients who initiated treatment with teprotumumab for thyroid eye disease were forced to deviate from the standard protocol. This study investigates the response of teprotumumab when patients receive fewer than the standard 8-dose regimen. METHODS: This observational cross-sectional cohort study included patients from 15 institutions with active or minimal to no clinical activity thyroid eye disease treated with the standard teprotumumab infusion protocol. Patients were included if they had completed at least 1 teprotumumab infusion and had not yet completed all 8 planned infusions. Data were collected before teprotumumab initiation, within 3 weeks of last dose before interruption, and at the visit before teprotumumab reinitiation. The primary outcome measure was reduction in proptosis more than 2 mm. Secondary outcome measures included change in clinical activity score (CAS), extraocular motility restriction, margin reflex distance-1 (MRD1), and reported adverse events. RESULTS: The study included 74 patients. Mean age was 57.8 years, and 77% were female. There were 62 active and 12 minimal to no clinical activity patients. Patients completed an average of 4.2 teprotumumab infusions before interruption. A significant mean reduction in proptosis (-2.9 mm in active and -2.8 mm in minimal to no clinical activity patients, P < 0.01) was noted and maintained during interruption. For active patients, a 3.4-point reduction in CAS ( P < 0.01) and reduction in ocular motility restriction ( P < 0.01) were maintained during interruption. CONCLUSIONS: Patients partially treated with teprotumumab achieve significant reduction in proptosis, CAS, and extraocular muscle restriction and maintain these improvements through the period of interruption.

Delayed-Onset Inflammatory Optic Nerve Sheath Mass and Perineuritis Following Cessation of Ipilimumab and Nivolumab Therapy.

A 65-year-old male presented with headaches and painless episodes of unilateral vision loss. He had a history of renal cell carcinoma, in remission following surgery and immunotherapy with ipilimumab and nivolumab, discontinued 2 years and 3 months before presentation, respectively. MRI revealed an optic nerve sheath mass and perineuritis. After 1 month of corticosteroid therapy, there was a robust clinical and radiographic response, which relapsed dramatically following cessation. An optic nerve sheath biopsy showed chronic mild inflammation, and extensive work-up for alternative etiologies of orbital inflammation was negative. Following a prolonged taper of corticosteroids, he demonstrated complete response. In the setting of ocular immune privilege, ophthalmic immune-related adverse events (irAE) are rare, although multifarious. While on-treatment irAE are well-characterized, posttreatment irAE have become increasingly recognized across multiple organ systems. We report a case of a delayed-onset inflammatory optic nerve sheath mass and perineuritis after cessation of immunotherapy.

TATTOO-ASSOCIATED CASES OF POSTERIOR SEGMENT UVEITIS WITH VOGT-KOYANAGI-HARADA DISEASE-LIKE FEATURES.

PURPOSE: To present two patients with treatment-naïve posterior segment uveitis with Vogt-Koyanagi-Harada-like features associated with tattoo-related inflammatory skin changes. METHODS: Retrospective report of two cases. RESULTS: Using clinical history and multimodal imaging, a diagnosis of posterior segment uveitis with Vogt-Koyanagi-Harada-like features was made in association with tattoo skin changes in both patients. CONCLUSION: Physicians should be aware that tattoo-associated posterior segment uveitis with Vogt-Koyanagi-Harada-like features can occur.

Intravitreous treatment of severe ocular von Hippel-Lindau disease using a combination of the VEGF inhibitor, ranibizumab and PDGF inhibitor, E10030: Results from a phase 1/2 clinical trial.

BACKGROUND: Treatment options for severe ocular von Hippel-Lindau (VHL) disease are limited. This trial evaluated preliminary safety and potential efficacy of combination intravitreous injection with ranibizumab, a vascular endothelial growth factor (VEGF) inhibitor, and E10030, a PDGF inhibitor, for eyes with VHL disease-associated retinal hemangioblastoma (RH) not amenable or responsive to thermal laser photocoagulation. METHODS: This was a prospective, single-arm, open-label phase 1/2 study, comprised of three adults with VHL-associated RH and vision loss. Intravitreous injections of ranibizumab (0.5 mg) and E10030 (1.5 mg) were given unilaterally every 4 weeks in the study eye through 16 weeks, then every 8 weeks through 48 weeks. Supplementary standard care therapies were allowed without restriction after 40 weeks. The primary outcome was the ocular and systemic adverse effect profile at 52 weeks. Secondary outcomes included changes in best-corrected visual acuity (BCVA), RH size, exudation, epiretinal proliferation and retinal traction, and need for ablative treatment of RH or ocular surgery at week 52. RESULTS: Three participants each received nine injections prior to week 52 and were followed for 104 weeks. One participant manifested mild episodic ocular hypertension in the study eye. Change in BCVA in the study eye at week 52 for the three participants was -5, -12 and +2 letters. No reduction in RH size was measured at 52 weeks. Variable mild improvements in exudation in two participants at week 16 were not sustained through week 52. CONCLUSIONS: Combination intravitreous injection with ranibizumab and E10030 demonstrated a reasonable preliminary safety profile, but limited treatment effect.

Development of Herpes Zoster Ophthalmicus in an Immunocompetent Pediatric Patient Following Facial Trauma.

Herpes zoster ophthalmicus (HZO) is a neuro-oculo-dermic infection caused by reactivation of latent varicella zoster virus in the dorsal root ganglia of the ophthalmic division of the trigeminal nerve. Although a rare diagnosis in an otherwise healthy, vaccinated pediatric patient, this entity may occur with increasing frequency among those with preceding trauma, particularly in the month prior to presentation. Herein, we highlight a case of HZO in a vaccinated, immunocompetent adolescent in the setting of recent facial trauma.

Progression of Geographic Atrophy with Subsequent Exudative Neovascular Disease in Age-Related Macular Degeneration: AREDS2 Report 24.

PURPOSE: To examine whether the rate of geographic atrophy (GA) enlargement is influenced by subsequent exudative neovascular age-related macular degeneration (nAMD) and hence, to explore indirectly whether nonexudative nAMD may slow GA enlargement. DESIGN: Post hoc analysis of a controlled clinical trial cohort. PARTICIPANTS: Age-Related Eye Disease Study 2 participants 50 to 85 years of age. METHODS: Baseline and annual stereoscopic color fundus photographs were evaluated for (1) GA presence and area and (2) exudative nAMD presence. Two cohorts were constructed: eyes with GA at study baseline (prevalent cohort) and eyes in which GA developed during follow-up (incident cohort). Mixed-model regression of the square root of GA area was performed according to the presence or absence of subsequent exudative nAMD. MAIN OUTCOME MEASURES: Change over time in square root of GA area. RESULTS: Of the 757 eyes in the incident GA cohort, over a mean follow-up of 2.3 years (standard deviation [SD], 1.2 years), 73 eyes (9.6%) demonstrated subsequent exudative nAMD. Geographic atrophy enlargement in these eyes was significantly slower (0.20 mm/year; 95% confidence interval [CI], 0.12-0.28 mm/year) compared with the other 684 eyes in which subsequent exudative nAMD did not develop (0.29 mm/year; 95% CI, 0.27-0.30 mm/year; P = 0.037). Of the 456 eyes in the prevalent GA cohort, over a mean follow-up of 4.1 years (SD, 1.4 years), 63 eyes (13.8%) demonstrated subsequent exudative nAMD. Geographic atrophy enlargement in these eyes was similar (0.31 mm/year; 95% CI, 0.24-0.37 mm/year) compared with the other 393 eyes in which subsequent exudative nAMD did not develop (0.28 mm/year; 95% CI, 0.26-0.29 mm/year; P = 0.37). CONCLUSIONS: In eyes with recent GA, GA enlargement before the development of exudative nAMD seems slowed. This association was not observed in eyes with more long-standing GA, which have larger lesion sizes. Hence, perilesional nonexudative choroidal neovascular tissue (presumably present before the development of clinically apparent exudation) may slow enlargement of smaller GA lesions through improved perfusion. This hypothesis warrants further evaluation in prospective studies.

Concurrent vascular flow defects at the deep capillary plexus and choriocapillaris layers in acute macular neuroretinopathy on multimodal imaging: A case series.

PURPOSE: To describe multimodal imaging findings with focus on retinal and choroidal vascular features in acute macular neuroretinopathy (AMN). OBSERVATIONS: Three eyes from 3 patients (1 man, 2 women) with average age of 31 were included in this retrospective case series at a single institution. Each case showed petaloid hyporeflective areas on infrared images (IR) with variable levels of outer retinal defects on spectral domain optical coherence tomography (OCT). En face OCT angiography (OCT-A) images showed quantifiable reduction in vessel density at levels of the deep capillary plexus (DCP) and choriocapillaris (CC) layers. In 2 of the cases with near-infrared autofluorescence imaging (NIRAF), there were subtle areas of hypoautofluorescence corresponding in location to the lesions seen on IR. In one case, fluorescein angiography (FA) showed a small area of retinal vascular leakage in the area of the IR lesion, and in other 2 cases, there were paracentral areas of hypofluorescence in the area of the IR lesions. En face structural OCT image at the retinal pigment epithelium (RPE) level in each case showed no evidence of projection artifact from the retina. CONCLUSIONS AND IMPORTANCE: The pathogenesis of AMN is suspected to involve a vasogenic insult. However, the precise localization of the vascular insult has been controversial and unclear. Our findings demonstrate that concurrent vascular flow defects in both DCP and CC could be possible in AMN and suggest that an inflammatory and vascular etiology in concert could underlie the pathogenesis of AMN.

Deep Learning Automated Detection of Reticular Pseudodrusen from Fundus Autofluorescence Images or Color Fundus Photographs in AREDS2.

PURPOSE: To develop deep learning models for detecting reticular pseudodrusen (RPD) using fundus autofluorescence (FAF) images or, alternatively, color fundus photographs (CFP) in the context of age-related macular degeneration (AMD). DESIGN: Application of deep learning models to the Age-Related Eye Disease Study 2 (AREDS2) dataset. PARTICIPANTS: FAF and CFP images (n = 11 535) from 2450 AREDS2 participants. Gold standard labels from reading center grading of the FAF images were transferred to the corresponding CFP images. METHODS: A deep learning model was trained to detect RPD in eyes with intermediate to late AMD using FAF images (FAF model). Using label transfer from FAF to CFP images, a deep learning model was trained to detect RPD from CFP (CFP model). Performance was compared with 4 ophthalmologists using a random subset from the full test set. MAIN OUTCOME MEASURES: Area under the receiver operating characteristic curve (AUC), κ value, accuracy, and F1 score. RESULTS: The FAF model had an AUC of 0.939 (95% confidence interval [CI], 0.927-0.950), a κ value of 0.718 (95% CI, 0.685-0.751), and accuracy of 0.899 (95% CI, 0.887-0.911). The CFP model showed equivalent values of 0.832 (95% CI, 0.812-0.851), 0.470 (95% CI, 0.426-0.511), and 0.809 (95% CI, 0.793-0.825), respectively. The FAF model demonstrated superior performance to 4 ophthalmologists, showing a higher κ value of 0.789 (95% CI, 0.675-0.875) versus a range of 0.367 to 0.756 and higher accuracy of 0.937 (95% CI, 0.907-0.963) versus a range of 0.696 to 0.933. The CFP model demonstrated substantially superior performance to 4 ophthalmologists, showing a higher κ value of 0.471 (95% CI, 0.330-0.606) versus a range of 0.105 to 0.180 and higher accuracy of 0.844 (95% CI, 0.798-0.886) versus a range of 0.717 to 0.814. CONCLUSIONS: Deep learning-enabled automated detection of RPD presence from FAF images achieved a high level of accuracy, equal or superior to that of ophthalmologists. Automated RPD detection using CFP achieved a lower accuracy that still surpassed that of ophthalmologists. Deep learning models can assist, and even augment, the detection of this clinically important AMD-associated lesion.

Two cases of severe Purtscher-like retinopathy demonstrating recurrence and progression to neovascularization and vitreous hemorrhage.

PURPOSE: To report the clinical course of two cases with Purtscher-like retinopathy (PLR), associated with peritoneal dialysis (PD), demonstrating disease recurrence and progression to neovascularization and vitreous hemorrhage. OBSERVATIONS: Case 1 (45-year old woman) experienced acute bilateral vision loss. Medical history included hypertension, end-stage renal failure (ESRF), PD, and obstructive sleep apnea. Visual acuity (VA) was 20/100 OD, 20/80 OS. Fundus findings were pathognomonic for PLR and included white streaks within arterioles. Nine months later, repeat imaging demonstrated disease recurrence and progression, including increased ischemia and new retinal neovascularization. The patient was managed with pan-retinal photocoagulation, sleep apnea treatment, and oral corticosteroids. Four months later, VA remained stable without additional progression.Case 2 (74-year old woman) experienced acute bilateral vision loss. Medical history included hypertension, ESRF, and PD, complicated by peritonitis. VA was 20/25 OD, 20/32 OS. Fundus findings were pathognomonic for PLR and included white streaks within arterioles. Three months later, further acute vision loss occurred, coinciding with recurrent peritonitis. Repeat imaging revealed disease recurrence and progression, including severely increased retinal ischemia. The PD catether was removed and the patient converted to hemodialysis. Bilateral vitreous hemorrhage later complicated the course. CONCLUSIONS AND IMPORTANCE: PLR can occur in association with PD, particularly in acute peritonitis. Contrary to classical descriptions, PLR may take a chronic and progressive course, with increasing ischemia and progression to neovascularization or vitreous hemorrhage. Increased surveillance for complications is recommended and treatment of neovascularization may be required.

Periorbital swelling and episcleritis may be a sign of cutaneous lupus erythematosus.

It is important that physicians recognize persistent periorbital edema and conjunctival injection as possible disease manifestations of CLE, especially prior to cutaneous involvement. This may lead to more rapid diagnosis and treatment.

The Aesthetic Oculoplastic Surgery Video Teleconference Consult.

BACKGROUND: Skype video telemedicine consults are gaining popularity to evaluate patients from distant locations. No study has analyzed the utility of this means of patient evaluation in a cosmetic oculoplastic patient population. OBJECTIVES: The authors sought to provide an evidence-based analysis of the utility of Skype video consults in a cosmetic oculoplastic surgery patient population with regards to patient demographics, reasons for consult, and procedural conversion rate. METHODS: A 1-year retrospective chart review (May 2016 to May 2017) of patients who underwent aesthetic oculoplastic Skype consults from 2 authors' practices was performed. The authors analyzed patient demographics, referral source, chief complaint, location of residence, length of consult, and conversion to face-to-face consultation and intervention. RESULTS: Seventy-nine patients (60 women and 19 men) underwent Skype evaluations. Mean age was 49 years. Sixty-four consults (81%) lasted 15 minutes or less. Referral sources included the internet (67%), another physician (19%), self-referral (7.5%), referred by former patients (4%), and social media sites (2.5%). Consultations were obtained for revision (49%), or first-time (30%) eyelid/eyebrow surgery, cosmetic ptosis surgery (6%), laser skin procedures (5%), cosmetic orbital decompression (5%), and lower eyelid fat prolapse (5%). Twenty patients (25%) followed-up with in-person consultation. Sixteen of these patients (80%) had surgical (56%) or nonsurgical (44%) interventions. CONCLUSIONS: Skype consults are an efficient, in-office modality to increase patient flow through the office, expand patient base, and generate income. In this report, 25% of Skype contacts followed-up with formal in-person consultations, of which 80% had surgical or nonsurgical interventions.

Anterior chamber fibrinoid syndrome after cataract extraction in a patient on ibrutinib for B-cell chronic lymphocytic leukemia: a case report and review of the literature.

BACKGROUND: Ibrutinib is a tyrosine kinase inhibitor commonly used in patients with chronic lymphocytic leukemia. Based on the published literature, it has a very sound ophthalmologic safety profile. In the following, we describe a case of anterior chamber fibrinoid syndrome in a patient on ibrutinib for B-cell chronic lymphocytic leukemia after uncomplicated cataract extraction. CASE PRESENTATION: A 75-year-old white man with B-cell chronic lymphocytic leukemia on ibrutinib therapy and without significant past ocular history presented 1 day after uncomplicated phacoemulsification with in-the-bag intraocular lens implantation with multiple, discrete, pigmented cords in the anterior chamber. His vision was 20/100 and intraocular pressure was 43 mmHg. There was no hypopyon, hyphema, or cellular reaction. The dilated fundus examination was unremarkable. He was diagnosed as having fibrinoid syndrome and started on topical prednisolone, brimonidine, timolol-dorzolamide, and orally administered acetazolamide. Within 2 weeks, the fibrin cords disappeared completely, vision improved to 20/30, and the intraocular pressure normalized off all medications. CONCLUSIONS: The precise etiology of fibrinoid syndrome remains unclear. This is the first case of fibrinoid syndrome in a patient on ibrutinib, which is known to cross the blood-brain barrier and induce intraocular changes. It is important to differentiate this syndrome from toxic anterior segment syndrome and endophthalmitis, and to initiate appropriate treatment. The fibrin bands tend to be exquisitely sensitive to topical steroids and to resolve within a few weeks without sequelae.