Cheeklift With and Without Posterior Lamellar Spacer Grafts for Treatment of Lower Eyelid Retraction.

BACKGROUND: To compare outcomes of lower eyelid retraction repair using a subperiosteal midface lifting technique with and without posterior lamellar grafts. METHODS: Charts of patients undergoing a sub-periosteal midface lift for treatment of lower eyelid retraction using 4 techniques for posterior lamellar reconstruction were reviewed. Thirty patients were included in each of the groups: midface with hard palate graft (HPG), midface lift with acellular cadaveric graft (ADG), midface lift with retractor disinsertion (RD) and midface lift alone (NG). Measurements of distance from pupil center to lower lid margin (MRD2) and from lateral limbus to lower lid margin (MRD2limbus) were taken from pre- and postoperative photographs and compared. Secondary outcomes included rates of reoperation, major and minor complications, resolution of symptoms and keratopathy. RESULTS: One hundred twenty operations were assessed (n = 30 for each surgical group). The average follow-up time was 20 weeks. The median MRD2 elevation was 0.95 mm (NG), 0.85 mm (HPG), 1.59 mm (ADG) and 1.02 mm (RD). The median MRD2limbus elevation was 1.06 mm (NG), 0.92 mm (HPG), 1.45 mm (ADG) and 1.12 mm (RD). There were no significant differences in MRD2 or MRD2limbus between the 4 groups (p = 0.06 and 0.29, respectively). Reoperation rates were highest with in the hard palate graft group (33%) compared to other techniques (p = 0.0006). CONCLUSIONS: Similar degrees of lower eyelid elevation were achieved with all the midface lifting techniques, and complication rates did not significantly differ between techniques. However, the higher reoperation rates with the use of spacer grafts suggest that a no-graft technique may be preferable. LEVEL OF EVIDENCE III: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Marginal corneal infiltrates as an ocular manifestation of acute generalized exanthematous pustulosis.

Purpose: To report a case of keratoconjunctivitis with marginal corneal infiltrates in a patient with acute generalized exanthematous pustulosis (AGEP) secondary to trimethoprim-sulfamethoxazole. Observations: A 63-year-old female presented with a diffuse pustular skin rash and bilateral keratoconjunctivitis with marginal corneal infiltrates. Skin biopsy led to the diagnosis of AGEP secondary to trimethoprim-sulfamethoxazole use. Treatment of the ocular findings with topical corticosteroids and lubrication led to near-full resolution after two weeks. Conclusions and Importance: To the best of our knowledge, this is the first reported association between AGEP and keratoconjunctivitis with marginal corneal infiltrates. A hypersensitivity reaction to a foreign antigen is implicated in the pathogenesis of both AGEP and sterile marginal infiltrates, and we suggest that the patient's underlying hypersensitivity process associated with AGEP accounted for the ocular findings.

Fungal Endophthalmitis Secondary to Aspergillus terreus Exacerbated by Intravitreal Dexamethasone in a Patient With Sarcoidosis.

Purpose: To report a case of Aspergillus terreus endophthalmitis associated with systemic immunosuppression and an intraocular steroid implant in a patient with sarcoidosis. Methods: A case report was evaluated and a literature review performed. Results: A patient with a history of pulmonary and ocular sarcoidosis presented with new-onset uveitis and was treated for presumed sarcoid flare with oral prednisone and an intravitreal dexamethasone implant before developing worsening vision. She was ultimately diagnosed with A terreus endophthalmitis. Despite both systemic and local antifungals, the visual acuity at the most recent follow-up was no light perception without pain or active inflammation. No definitive source of the fungal disease had been identified. Conclusions: Endophthalmitis resulting from A terreus is associated with poor outcomes. Given the ability of fungal endophthalmitis to mimic other causes of uveitis, one must maintain a high suspicion in patients with any degree of immunosuppression.

Coats Plus Syndrome in a Premature Infant, With a Focus on Management.

Purpose: A premature infant was diagnosed with Coats plus syndrome based on a genetic evaluation showing biallelic heterozygous pathogenic CTC1 variants. Methods: A case study was performed, including findings and interventions. Results: A premature infant born 30 weeks gestational age weighing 817 g was evaluated for retinopathy of prematurity at 35 weeks corrected gestational age. An initial dilated fundus examination showed an exudative retinal detachment (RD) in the right eye and avascularity post-equatorially in the left eye with telangiectasias and aneurysmal dilations. Genetic evaluation showed biallelic heterozygous pathogenic CTC1 variants, diagnostic of Coats plus syndrome. Sequential examination under anesthesia with fluorescein showed progressive ischemia despite confluent photocoagulation. Conclusions: CTC1 gene variants manifest as Coats plus syndrome, which has a clinical appearance consistent with retinovascular ischemia, capillary remodeling, aneurysmal dilation, and exudative RD. Systemic and local corticosteroids in conjunction with peripheral laser ablation decreased vascular exudation and avoided intraocular intervention.

Five-Year Visual Acuity Outcomes of Fovea-Involving Rhegmatogenous Retinal Detachments Following Pars Plana Vitrectomy (PPV) or Combined PPV/Scleral Buckle.

BACKGROUND AND OBJECTIVE: Macular involvement is considered a poor prognostic factor for visual recovery after rhegmatogenous retinal detachment (RRD) repair. Few prior studies report outcomes longer than 2 years after repair with pars plana vitrectomy (PPV) or combined PPV/scleral buckle (SB). The purpose of this study is to evaluate long-term (at least 5 years) visual outcomes following fovea-involving RRD repair with PPV or PPV/SB. PATIENTS AND METHODS: This retrospective case series evaluated eyes that underwent fovea-involving RRD repair with PPV or PPV/SB. Eyes with single surgery anatomic success and 5 years or more of follow-up were included. Eyes with other ophthalmic pathology affecting central vision were excluded. Paired analysis compared changes in best visual acuity (BVA) between two timepoints. RESULTS: Fifty-one eyes were included. Median (interquartile range, IQR) time to final follow-up was 7 (IQR: 3) years. Median pre-operatively BVA was 19.95 (IQR: 41.25) letters and improved to 80.15 (IQR:15.05) letters and 80.16 (IQR: 8.80) letters at the 1-year and final follow-up, respectively (P < 0.001). Thirteen of 45 eyes (28.89%) had an improvement in BVA of at least 10 letters from the 1-year to the final follow-up. Average BVA was 20/40 or better in 35/45 eyes (75.56%) at 1-year and 45/51 eyes (88.24%) at final follow-up. No eye had an average BVA of 20/200 or worse at final follow-up. CONCLUSIONS: Patients with fovea-involving RRD successfully repaired with PPV or PPV/SB have favorable long-term visual acuity outcomes. BVA may continue to significantly improve even beyond 1 year after surgery. The majority of patients have a BVA of 20/40 or better 5 years after surgery. [Ophthalmic Surg Lasers Imaging Retina 2022;53:674-680.].

SHORT-TERM OUTCOMES AFTER INTERIM TREATMENT WITH BROLUCIZUMAB: A Retrospective Case Series of a Single Center Experience.

PURPOSE: To examine outcomes of eyes with neovascular age-related macular degeneration that were switched to brolucizumab because of an unsatisfactory response to bevacizumab, ranibizumab, and/or aflibercept and then switched back because of the presence or risk of intraocular inflammation. METHODS: Retrospective case series of 51 eyes. Visual acuity and retinal anatomy on optical coherence tomography were recorded at the first brolucizumab injection (T1), the final brolucizumab injection (T2), and 6 months following the final brolucizumab injection (T3). RESULTS: At T2, 41 eyes (41/51%, 80%) had decreased subretinal fluid (31 eyes), intraretinal fluid (12 eyes), or pigment epithelial detachment height (12 eyes). At T3, decreased subretinal fluid was sustained in 17 eyes (17/31%, 55%), decreased intraretinal fluid was sustained in eight eyes (8/12%, 67%), and decreased pigment epithelial detachment height was sustained in eight eyes (8/12%, 67%). Mean logarithm of the minimum angle of resolution visual acuity at T1, T2, and T3 was 0.396 (∼20/50), 0.441 (∼20/55), and 0.468 (∼20/59), respectively. During the brolucizumab treatment period, 11 eyes (11/51%, 22%) developed intraocular inflammation, including one case of retinal vasculitis. CONCLUSION: Interim treatment with brolucizumab resulted in anatomical improvements in 41 eyes (41/51%, 80%) that were maintained in 22 of these eyes (22/41%, 54%) for at least 6 months after switching back to the original anti-vascular endothelial growth factor therapeutic. There were no corresponding significant changes in visual acuity.

Effect of Premature Tube Extrusion for Simple Congenital Nasolacrimal Duct Obstruction: A Comparison of Monocanalicular and Bicanalicular Intubation.

ABSTRACT: The objective of this article is to compare the incidence of premature dislocation of silicone tubes and the effect on treatment success between monocanalicular (MCI) and bicanalicular (BCI) intubation in pediatric patients with simple congenital nasolacrimal duct obstruction. Retrospective comparative case series of 108 eyes of 78 pediatric patients with simple congenital nasolacrimal duct obstruction who underwent probing with either BCI (n = 38 eyes) or MCI (n = 70 eyes) from 2017 to 2020. Premature tube extrusion was defined as any tube removed prior to the 3 month postoperative appointment. Success was defined as resolution of tearing 3 months post tube removal. Ages ranged from 10 months to 5.35 years (mean, 1.95 years; Standard deviation (SD), 0.91). Premature tube extrusion occurred in 15 eyes with BCI and 29 eyes with MCI. Success rates were not significantly different regardless of intubation type between the planned tube removal (90.6%) and the premature tube extrusion cohorts (84.1%), P = 0.89. There was no significant difference in treatment success between the planned tube removal (92.7% MCI, 87% BCI) and the premature tube extrusion cohorts (86.2% MCI, 80% BCI). Complications included 2 infections (1 MCI, 1 BCI) and 2 cases of tube related keratopathy (1 MCI, 1 BCI) that all resolved with tube removal. There were 2 BCI patients that presented to the emergency department for premature tube extrusion. Silicone intubation regardless of stent type is an effective treatment for simple congenital nasolacrimal duct obstruction. There was no significant difference in treatment success between tubes that extrude prematurely, and tubes removed at term based on type of intubation.

Rickettsial neuroretinitis: A report of 2 cases.

PURPOSE: The authors present two cases of neuroretinitis caused by Rickettsia rickettsii infection. OBSERVATIONS: Case 1 is a 24-year-old male who presented with 2 months of vision loss. Case 2 is a 38-year-old female who presented with 4 weeks of eye pain and vision loss. Examination of both patients revealed neuroretinitis characterized by optic disc swelling with macular exudates, and subsequent serological analysis was positive for Rickettsia rickettsii. Both patients responded favorably to treatment with oral doxycycline and prednisone. CONCLUSIONS AND IMPORTANCE: Given the potential for neuroretinitis to cause permanent vision loss, the presence of acute vision loss, optic disc edema, and macular exudates should prompt an evaluation for Rickettsial disease in endemic areas, even in the absence of systemic symptoms or known history of a tick bite.

Symptomatic Liver Metastasis Prompting Diagnosis of Uveal Melanoma.

We report the case of a 66-year-old male with symptomatic liver metastasis as the presenting manifestation of primary uveal melanoma. Upon initial presentation to an emergency department with 2 months of abdominal pain, back pain, and unintended weight loss, a computed tomography scan demonstrated diffuse liver and bone lesions, prompting referral to a medical oncologist. He was eventually examined by an ophthalmologist, who found a lesion suspicious for uveal melanoma. This was ultimately confirmed to be the primary site of his malignant metastatic melanoma, which caused his death within 2 months of presentation.

A case of giant cell arteritis presenting with nodular posterior scleritis mimicking a choroidal mass.

PURPOSE: Herein we present a case of giant cell arteritis presenting with nodular posterior scleritis and exudative retinal detachment mimicking a choroidal mass. OBSERVATIONS: A 67-year-old man presented with sudden onset left-sided periorbital pain, blurry vision, and a choroidal lesion in the posterior pole. Despite treatment with high-dose oral prednisone for suspected nodular posterior scleritis mimicking a choroidal mass, the vision in his left eye did not recover, and he developed optic nerve pallor on exam. Further evaluation revealed an ESR of >140 mm/hr (Upper limit of normal = 20 mm/hr), a CRP of 113 mg/L (Upper limit of normal = 9 mg/L), and a temporal artery biopsy consistent with GCA. The patient was started on methotrexate and the oral steroids were slowly tapered. CONCLUSIONS: Given the potential for GCA to present with scleritis and the potential for nodular posterior scleritis to mimic a choroidal mass, presence of a painful choroidal lesion with optic nerve swelling should prompt an evaluation for GCA to prevent permanent and bilateral vision loss.

Iodine-125 Episcleral Plaque Brachytherapy for AJCC T4 Posterior Uveal Melanoma: Clinical Outcomes in 158 Patients.

BACKGROUND/AIMS: The aim of this study is to report the burden of ocular morbidity following iodine-125 episcleral plaque brachytherapy (EPBT) in the treatment of American Joint Committee on Cancer (AJCC) T4-staged posterior uveal melanoma (PUM). METHODS: Clinical records of patients with T4-staged PUM treated with 125I EPBT were analyzed for incidence of treatment failure and radiation-induced complications. RESULTS: Cumulative incidence of local treatment failure was 9% (95% CI 5-15%) at 5 years and was associated with decreased tumor height (HR = 0.78; p = 0.01). Cumulative incidence of enucleation at 5 years was 21% and was correlated with worsening baseline visual acuity (HR = 1.42; p = 0.05). Increasing patient age was associated with higher rates of vitreous hemorrhage (HR = 1.03; p = 0.02) and cataract surgery (HR = 1.05; p < 0.001). Increased tumor height was associated with higher rates of neovascular glaucoma (HR = 1.16; p = 0.03) and vitreous hemorrhage (HR = 1.23; p < 0.001). CONCLUSION: 125I EPBT is an effective treatment for T4-staged PUM and achieves high rates of local control. Treatment failure appears to be more common among minimally elevated tumors. Other causes of ocular morbidity were associated with increasing tumor height, patient age, and baseline visual acuity.

Primary Isolated Lacrimal Gland Amyloidosis: A Case Report and Review of the Literature.

We report a case of a 42-year-old woman who presented with a one-year history of a painless right orbital mass and right upper lid ptosis. Magnetic resonance imaging (MRI) revealed a superotemporal right orbital mass involving the lacrimal gland, and subsequent tissue biopsy and histopathologic evaluation was consistent with amyloidosis. An otherwise negative workup by hematology/oncology confirmed a diagnosis of primary isolated lacrimal gland amyloidosis. To the best of our knowledge, this is the first documented case of isolated lacrimal gland amyloidosis without immunoglobulin (Ig) light chain restriction on in situ hybridization testing with a report of MRI findings. In addition, this is the second reported case of the disease without Ig light chain restriction on immunohistochemistry staining, and it is the third case with reported MRI results.

Genetic Risk Factors for Radiation Vasculopathy.

Purpose: The purpose of this study was to perform a genome-wide scan for polymorphisms associated with risk of vision loss from radiation complications in patients treated with proton beam irradiation for choroidal melanoma. Methods: We identified a cohort of 126 patients at high risk of radiation complications due to tumor location within 2 disc diameters of the optic nerve and/or fovea who provided a blood sample to the Massachusetts Eye and Ear Uveal Melanoma Repository. Controls (n = 76) were defined as patients with visual acuity 20/40 or better 3 years after treatment. Cases (n = 50) were selected as patients with visual acuity 20/200 or worse due to radiation damage 3 years after treatment. Genotyping of these samples was performed using the Omni 2.5 chip (Illumina, Inc.). Results: Hypertension (odds ratio [OR] = 3.749, P = 0.0009), visual acuity at diagnosis of choroidal melanoma (OR = 1.031, P = 0.002), tumor distance to fovea (OR = 0.341, P = 6.52E-05), tumor distance to optic disc (OR = 0.481, P = 5.41E-05), and height of tumor (OR = 1.704, P = 0.0069) were associated with poor vision (20/200 or worse). Individual single nucleotide polymorphism (SNP) analysis was performed controlling for the risk factors identified using stepwise regression and the first principal component. Although this analysis determined that there were 74,529 nominally significant SNPs (P < 0.05), there were no SNPs that reached genome-wide significance (P < 5E-08). The SNP reaching the highest significance level (P < 1E-04) was rs11678387, located on chromosome 2, intergenic between EPB41L5/RALB (P = 4.43E-05). Conclusions: Visual loss from radiation vasculopathy after treatment for choroidal melanoma is not only related to tumor location but may be influenced by hypertension and possibly genetic factors.

Purtscher's retinopathy as the presenting manifestation of immune thrombocytopenic purpura.

PURPOSE: To report the case of a 52-year-old man with Purtscher's retinopathy as the presenting manifestation of immune thrombocytopenic purpura (ITP). OBSERVATIONS: Treatment with corticosteroids led to the resolution of hematologic findings within 1 week, and normal visual acuity was achieved after 2 months with no additional treatment. CONCLUSIONS AND IMPORTANCE: This is the first reported association between Purtscher's retinopathy and ITP. Complement activation has been implicated in the pathogenesis of both ITP and Purtscher's retinopathy, and we suggest that the patient's systemic process accounted for the retinal findings.

Ranibizumab for the Prevention of Radiation Complications in Patients Treated With Proton Beam Irradiation for Choroidal Melanoma.

PURPOSE: To investigate the safety and potential efficacy of ranibizumab for prevention of radiation complications in patients treated with proton irradiation for choroidal melanoma. METHODS: Forty patients with tumors located within 2 disc diameters of the optic nerve and/or macula were enrolled in this open-label study. Participants received ranibizumab 0.5 mg or 1.0 mg at tumor localization and every 2 months thereafter for the study duration of 24 months. The incidence of adverse events, visual acuity, and other measures of ocular morbidity related to radiation complications were assessed. Historical controls with similar follow-up meeting the eligibility criteria for tumor size, location, and baseline visual acuity were assembled for comparison. RESULTS: Fifteen patients with large tumors and 25 patients with small/medium tumors were enrolled. Thirty-two patients completed the month 24 visit. No serious ocular or systemic adverse events related to ranibizumab were observed. At 24 months, the proportion of patients with visual acuity ≥ 20/200 was 30/31 (97%) in the study group versus 92/205 (45%) in historical controls (P < .001). The proportion of patients with visual acuity ≥20/40 was 24/31 (77%) in the study group versus 46/205 (22%) in controls at 24 months (P<.001). Clinical evidence of radiation maculopathy at month 24 was seen in 8/24 (33%) patients with small/medium tumors versus 42/62 (68%) of controls (P = .004). Three patients with large tumors developed metastases. CONCLUSIONS: In this small pilot study, prophylactic ranibizumab appears generally safe in patients treated with proton irradiation for choroidal melanoma. High rates of visual acuity retention were observed through 2 years.

Leukostasis Retinopathy: A New Clinical Manifestation of Chronic Myeloid Leukemia With Severe Hyperleukocytosis.

The authors report a new clinical manifestation of chronic myeloid leukemia. A 41-year-old man presented with significant visual loss, leading to a diagnosis of chronic myeloid leukemia. His white blood count exceeded that of any previously reported case of the disease with documented retinal findings (562,000/mm(3)), and clinical evaluation revealed the blockage of temporal retinal vessels by white blood cells. Hematologic findings resolved within 1 month of chemotherapy with dasatinib, and further treatment with intravitreal anti-VEGF agents resulted in the complete resolution of fundus findings. The authors propose that leukostasis retinopathy be recognized as a clinical manifestation of this life-threatening disease.