Melanoma-associated retinopathy during pembrolizumab treatment probably controlled by intravitreal injections of dexamethasone.

PURPOSE: Melanoma-associated retinopathy (MAR) is a rare paraneoplastic syndrome due to antibodies targeting bipolar retinal cells. Its evolution, particularly in patients treated with immune checkpoint inhibitors (ICI), is currently poorly understood. In the few cases published, patients' visual function got worse when these molecules were prescribed. Here, we present a case of a patient with severe MAR treated with an ICI for melanoma progression. METHODS: A 68-year-old woman with a history of melanoma of the palpebral conjunctiva presented with sudden and gradually worsening visual disturbances. Simultaneously, a metastatic evolution of the melanoma was diagnosed and surgically treated exclusively. Visual acuity assessment, static automated perimetry and ERG results lead to the diagnosis of MAR. Since systemic corticosteroid therapy did not improve her symptoms, repeated intraocular corticosteroid injections were performed with a positive outcome. Later on, metastatic progression of the patient's melanoma led to the introduction of pembrolizumab, an ICI targeting PD-1. Immunotherapy has changed the prognosis of patient affected by metastatic melanoma, but these molecules may induce various immune-related adverse effects. In our case, intraocular corticosteroid injections were still performed simultaneously. Visual acuity assessment, static automated perimetry and ERG were performed during the course of this treatment. RESULTS: Full-field ERGs results suggested the possibility that the ophthalmologic treatment might restore the patient's retinal function despite the continued immunotherapy. CONCLUSION: We report the first case of MAR with a positive outcome after 1 year of ICI, possibly thanks to intravitreal corticosteroid therapy.

Evisceration with autogenous scleral graft and bioceramic implantation within the modified scleral shell: 133 cases over 17 years.

INTRODUCTION: To present long-term follow-up data on evisceration performed with autogenous scleral grafting and ceramic implantation in a modified scleral shell. METHODS: This was a retrospective analysis of all consecutive eviscerations performed in the Department of Ophthalmology, Montpellier University Hospital, France, between February 1998 and October 2015. For all patients, the technique used was a conventional anterior evisceration after total keratectomy, disinsertion of the medial rectus muscle, sectioning of the optic nerve and excision of sclera centered on the papilla. The scleral graft was then sutured just behind the sutured keratectomy, and the bioceramic implant was inserted by posterior way in the scleral shell. Demographic characteristics, implant size and type, cosmetic results from pictures of all patients and complications were recorded. This study was performed with Ethics Review Committee Approval, and in compliance with the Declaration of Helsinki. RESULTS: In total, 133 patients (36.6% women) were identified during the study period. The mean (SD) implant size was 17.32 (1.84) mm. The median follow-up after evisceration was 57.43 (24.7, 68.3) months. Two cases of implant exposure (1.5%) were recorded. For 24 patients (17.9%), additional surgeries were performed for ptosis (2.2%), conjunctival cyst (1.5%), or post-evisceration socket syndrome (6.7%). Cosmetics results were excellent for 50.1% of cases, good for 33.3% and fair for 16.6%; using a grading scale based on the superior sulcus deformity. CONCLUSION: Evisceration with autogenous scleral grafting and ceramic implantation can result in a high volume of restoration, good cosmetic results, and low risk of exposure of the implant.

Exophthalmos, Diplopia, and Bilateral Eyelid Edema: Symptoms of Ocular Mastocytosis.

PURPOSE: Mastocytosis is characterized by clonal mast cell proliferation with accumulation within various organs and uncontrolled activation with excessive mast cell mediator release. Ocular manifestations have rarely been published. We describe a 63-year-old man with bilateral exophthalmos that led to the diagnosis of systemic mastocytosis. CASE REPORT: A patient presented with bilateral eyelid edema with exophthalmos associated with binocular diplopia. Ophthalmologic examination showed bilateral axial, symmetrical, and painless exophthalmos with eyelid edema, and limitation in elevation of the right eye. Visual acuity was normal. Orbital magnetic resonance imaging showed increased volume of both the superior and medial recti muscles and right inferior oblique muscle, and histopathological examination of orbital fat and muscle biopsies revealed an infiltration by mast cells. Serum tryptase was elevated. The patient also complained of a long history of pruritis and diffuse skin erythema that could be elicited with just mild pressure (Darier's sign). A bone marrow biopsy confirmed the infiltration of abnormal mast cells with a D816V mutation in the KIT gene. Treatment with cladribine was initiated and resulted in resolution of both ocular and systemic signs and symptoms that persisted without relapse 18 months after discontinuation. Ocular mastocytosis is a rare condition, which was previously reported to involve the conjunctiva, cornea, uvea, eyelid, orbit, and choroid. Cases of ocular mastocytosis can be classified into two main groups: mast cells tumors (mastocytomas) and ocular manifestations associated with systemic mastocytosis. Histological examination of ocular samples is rarely performed, and there are no standard criteria for the diagnosis of ocular mastocytosis. Our case emphasizes cladribine could represent an alternative treatment. CONCLUSIONS: Our case is the first published case of exophthalmos and eyelid edema associated with systemic mastocytosis confirmed by pathologic examination of periocular biopsies that was treated effectively with cladribine.

Clinical and biometric determinants of actual lens position after cataract surgery.

PURPOSE: To evaluate the preoperative clinical and biometric determinants associated with the actual lens position after cataract surgery. SETTING: Department of Ophthalmology, University Hospital of Montpellier, France. DESIGN: Prospective longitudinal cohort study. METHODS: The data collected included clinical factors (age, sex, history of vitrectomy) and biometry factors (axial length [AL], anterior chamber depth [ACD], lens thickness, white-to-white [WTW] distance) that might affect actual lens position. Each patient had optical low-coherence reflectometry biometry (Lenstar) preoperatively and 1 month postoperatively. The actual lens position was measured as the postoperative position of the center of the intraocular lens (IOL). Patients were stratified into 3 groups by type of IOL: Acrysof SN60WF or SN6AT (Group 1), Tecnis ZCB00 or ZCT (Group 2), and Asphina 409 MV (Group 3). RESULTS: The study comprised 168 eyes (mean age 73.3 years ± 9.8 [SD]). The mean actual lens position was 4.88 ± 0.29 mm, 5.01 ± 0.29 mm, and 5.05 ± 0.32 mm in Group 1 (n = 67 eyes), Group 2 (n = 52 eyes), and Group 3 (n = 49 eyes), respectively. In the overall population, AL, ACD, anterior segment depth, and WTW distance were correlated with actual lens position (r = 0.48, P < .0001; r = 0.64, P < .001; r = 0.58, P < .0001; r = 0.39, P < .001, respectively). CONCLUSIONS: The AL, ACD, anterior segment depth, and WTW distance correlated with actual lens position after cataract surgery. The integration of these data in IOL formulas could help improve refractive outcomes after the surgery.

Ipsilateral uveitis and optic neuritis in multiple sclerosis.

Background. Uveitis is 20 times more frequent in multiple sclerosis (MS) patients than in the general population. Methods. A retrospective study of local multiple sclerosis (n = 700) and uveitis cohorts (n = 450) described the ophthalmological and neurological characteristics of patients with multiple sclerosis and uveitis. Results. Uveitis and multiple sclerosis were associated in seven patients. The time intervals between diagnoses of MS and uveitis ranged from 6 months to 15 years. Analysis of the patients' characteristics revealed that multiple sclerosis was associated with an older age of onset than usually expected, that is, 39 years. Uveitis was bilateral in three cases and mainly posterior (5/10). Five patients presented with acute optic neuritis (two in one eye and three in both eyes). All eyes presenting with acute optic neuritis were also affected by uveitis (P = 0.02), though not simultaneously. Conclusion. The ipsilateral association between optic neuritis and uveitis in this series of patients with multiple sclerosis may suggest a reciprocal potentiation between optic neuritis and uveitis in multiple sclerosis.

Uveitis and common variable immunodeficiency: data from the DEF-I study and literature review.

PURPOSE: To examine the relation between uveitis and common variable immunodeficiency (CVID). METHODS: Retrospective analysis of patients included in the French DEFI cohort of adults with CVID and of patients identified by reviewing the literature. RESULTS: Four patients were identified in the DEFI study (frequency of uveitis: 1.6%). The course of uveitis was not changed in the patients who started intravenous immunoglobulins replacement therapy after CVID diagnosis. Ten cases of CVID-associated uveitis were listed in the literature. Overall, among the 14 patients, uveitis was always chronic, usually bilateral (n = 11) and granulomatous (n = 9). Seven patients presented with a "sarcoid-likeâ syndrome. Nine patients had granulomatous uveitis suggestive of ocular sarcoidosis. Five patients were treated with local corticosteroids, and 9 required systemic treatment (corticosteroids alone n = 5 and/or immunosuppressive agents n = 4). CONCLUSIONS: CVID can be associated with uveitis. Since uveitis can reveal the immunodeficiency, CVID diagnosis should be considered at the time of uveitis diagnosis.