Ocular Mucous Membrane Pemphigoid: The Effect of Risk Factors at Presentation on Treatment Outcomes.

PURPOSE: Analyze the influence of risk factors at presentation in the long-term immunosuppressive therapy (IMT) outcomes of ocular mucous membrane pemphigoid (OMMP). DESIGN: Retrospective multicenter study. PARTICIPANTS: Patients with OMMP seen at the Duke Eye Center, Tecnologico de Monterrey, and Hospital Clinic of Barcelona from 1990 to 2022. METHODS: Data at presentation on demographics, direct immunofluorescence, ocular findings, sites of extraocular manifestations (EOMs), and previous treatments in patients with a clinical or laboratory diagnosis of OMMP, were analyzed with multivariable analysis and Kaplan-Meier plots to identify factors associated with adverse outcomes. MAIN OUTCOME MEASURES: (1) Inflammatory control (no conjunctival inflammation in both eyes at 3 months on IMT); (2) relapse (new-onset inflammation after absolute control in either eye); (3) progression (≥ 1 cicatrizing stage progression in either eye); and (4) vision loss (≥ 2 Snellen lines). RESULTS: A total of 117 patients (234 eyes), 61% (71/117) of whom were women, with a mean age of 66.6 (SD: 12.4) years (range: 37-97 years) and median follow-up of 34 months (interquartile range: 16-66 months; range: 3-265 months), were enrolled. Inflammatory control was achieved in 57% of patients (67/117), with high-risk EOM (HR-EOM), including esophageal, nasopharyngeal, and/or genital involvement (adjusted odds ratio [aOR]: 12.51; 95% confidence interval [CI]: 2.61-59.99; P = 0.002) and corneal scarring (aOR: 3.06; 95% CI, 1.15-8.14; P = 0.025), as significant risk factors for persistent inflammation. Disease relapse, progression, and vision loss occurred in 20% of patients (23/117), 12% of patients (14/117), and 27% of patients (32/117), respectively. Baseline corneal scarring was a risk factor for relapse (adjusted hazard ratio: 4.14; 95% CI: 1.61-10.62; P = 0.003), progression (aOR: 11.46; 95% CI: 1.78-73.75; P = 0.010), and vision loss (aOR: 3.51; 95% CI: 1.35-9.10; P = 0.010). HR-EOM was associated with stage progression (aOR, 34.57; 95% CI, 6.57-181.89; P<0.001) and vision loss (aOR, 8.42; 95% CI, 2.50-28.42; P = 0.001). No significant differences were found between IMT regimes and relapse (P = 0.169). CONCLUSIONS: Ocular mucous membrane pemphigoid presenting with HR-EOMs and corneal scarring has an increased risk of stage progression and vision loss. Corneal scarring and severe inflammation at baseline were associated with an increased risk of relapse. A disease progression staging system incorporating both the HR-EOMs and corneal involvement is required to predict the visual outcome of OMMP better. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Isotretinoin and the eye: A review for the dermatologist.

Due to the prevalence of acne vulgaris, isotretinoin is one of the most prescribed drugs among physicians and dermatologists. Although exhibiting an adequate safety profile, adverse events secondary to isotretinoin use are common. Before prescribing isotretinoin, physicians usually inquire about pregnancy and perform serologic tests including cholesterol, triglycerides, and liver enzymes. Ocular manifestations are commonly neglected. Despite being generally mild, ocular manifestations related to either topical or systemic isotretinoin may cause important ocular morbidity. The ocular surface is the most affected site within the eye; however, retinal and optic nerve disease also have been documented. Evaporative dry eye disease, which may range from mild to severe, is the most common adverse ocular effect associated with isotretinoin use. The aim of this review is to present an up-to-date overview for the dermatologist about the prevention, diagnosis, and treatment of the ocular side effects of isotretinoin, and when to refer to the eye specialist.

Leukemic infiltration of the ciliary body in a child with T-cell acute lymphoblastic leukemia.

A 14-year-old boy presented after 2 months of vision loss, redness, and pain in the right eye, initially treated as anterior uveitis with topical corticosteroids. He had a 1-year history of T-cell acute lymphoblastic leukemia, which had been in remission for 6 months. On examination, visual acuity in the right eye was light perception, with 4+ anterior chamber cells, pupillary membrane, and an intumescent cataract. Ultrasound biomicroscopy (UBM) revealed a ciliary body mass and capsular bag rupture. After consultation with his oncologist, he received 10 radiotherapy sessions. At 1 month, UBM showed resolution of the mass. After 1 year of remission, the patient underwent pars plana vitrectomy, pupillary membranectomy, and placement of a scleral-fixated intraocular lens. Thirty months after surgery, visual acuity was 20/25. Leukemic infiltration of the ciliary body is a rare manifestation of the disease that is often misdiagnosed as uveitis.

Clinical Course and Outcomes of Autoimmune Versus Non-Autoimmune Surgically Induced Scleral Necrosis: A Multicentric Comparative Study.

BACKGROUND: To analyze the clinical course and outcomes of autoimmune vs. non-autoimmune surgically induced scleral necrosis (SISN). METHODS: Multicentric, retrospective, comparative cohort study. Eighty-two eyes of 70 patients with SISN were classified according to pathogenic mechanism into autoimmune vs. non-autoimmune. Main outcome measures included necrosis onset, type of surgery, associated systemic disease, visual acuity, and treatment were analysed in patients followed for ≥ 6 months. RESULTS: Forty-six (65.7%) patients were women, and the median age was 66 (range: 24-90) years. Most patients (82.9%) had unilateral disease. The median time between surgery and SISN onset was 58 (1-480) months. Thirty-one (37.8%) eyes were classified as autoimmune, and 51 (62.2%) as non-autoimmune SISN. Autoimmune SISN was associated with a shorter time between the surgical procedure and SISN onset than non-autoimmune cases (median of 26 vs. 60 months, p = 0.024). Also, autoimmune SISN was associated with cataract extraction (93.5% vs. 25.5%, p < 0.001), severe scleral inflammation (58.1% vs. 17.6%, p < 0.001), and higher incidence of ocular complications (67.7% vs. 33.3%, p = 0.002) than non-autoimmune cases. Remission was achieved with medical management alone in 44 (86.3%) eyes from the non-autoimmune and in 27 (87.1%) from the autoimmune group (p = 0.916). Surgical management was required in 11 (13.4%) eyes, including two requiring enucleations due to scleral perforation and phthisis bulbi. CONCLUSIONS: Eyes with autoimmune SISN had a higher rate of cataract surgery, severe scleral inflammation, and ocular complications. Early SISN diagnosis and appropriate management, based on clinical features and pathogenic mechanisms, are critical to avoid sight-threatening complications.

Surgically induced scleral necrosis associated with concomitant tuberculosis infection: a diagnostic challenge.

Objective: Surgically induced scleral necrosis (SISN) is a potentially blinding sequela that may occur after any ocular procedure. SISN in the context of active tuberculosis is seldom seen. We report a case of a patient with asymptomatic tuberculosis who developed SISN after pterygium surgery. Methods: A 76-year-old Mexican-mestizo woman from Veracruz, Mexico, was referred to our clinic because of severe disabling pain and scleral thinning in her right eye. Results: Tubercular-related SISN was finally diagnosed and managed successfully with antitubercular therapy, topical and systemic corticosteroids. Conclusion: Tuberculosis must be considered as a differential diagnosis of high-risk patients in the context of refractory SISN in endemic countries.

Retinal Ischemia Following Focal Cerebral Arteriopathy in a Young Girl.

PURPOSE: We present a case of an ischemic retinopathy with severe vision loss secondary to a childhood stroke. METHODS: Case report. RESULTS: An otherwise healthy 9-year-old girl presented with a 1-day history of impaired gait and speech. After performing Computed Tomography and Magnetic Resonance Imaging, thrombosis of the left Internal Carotid Artery was observed and a diagnosis of ischemic stroke was established. Serological autoimmune, coagulation, and viral panels were unrewarding. Cardiac, inflammatory and coagulation disorders were ruled out. The cause was attributed to a Focal Cerebral Arteriopathy, a common cause of childhood stroke. The patient was treated with mechanical thrombectomy followed by anticoagulation. The day after, the patient referred vision loss of 20/100 in the left eye. Fundus evaluation of the left eye depicted diffuse intraretinal hemorrhages as well as cotton-wool spots and there was retinal whitening at the posterior pole. Six weeks after, visual acuity dropped to counting fingers. CONCLUSIONS: A macular optical coherence tomography revealed diffuse atrophic changes in the inner retinal layers at the macula, and the Angio-OCT showed an enlarged Foveal Avascular Zone. We propose ischemia-reperfusion as the primary explanation of this unusual event.

The evolution of the modified osteo-odonto-keratoprosthesis, its reliability, and long-term visual rehabilitation prognosis: An analytical review.

An exhaustive search of the world's literature was performed to analyze all case reports and series on the modified osteo-odonto-keratoprosthesis (MOOKP) published up to January 2022. The demographic profile, the primary indication for surgery, surgical technique variations, postoperative medical management, long-term functional and anatomical outcomes, and intra- and postoperative complications were analyzed and compared. Additionally, some of the authors' (GI, VP, and GA) unpublished MOOKP cases were studied. An extensive literature search yielded 37 case series and case reports. Overall, 958 patients were analyzed. The most common indication for surgery was autoimmune disease (39.1%), closely followed by chemical injury (38.8%). The most common intraoperative complications (21.67%) included maxillofacial, vitreous hemorrhage/vitritis, and mucosal. The most common postoperative complications (78.4%) were lamina and oral mucosa-associated, secondary glaucoma, and choroid/retinal detachment. Follow-up periods ranged from one to 364 months (median: 36.7 months). Altogether, 78% of patients achieved a visual acuity of 20/400 or better at the end of the follow-up period, and 91.2% improved at least temporarily after MOOKP surgery. Mean anatomic success at the end-of-follow-up for all patients was 88.25% (range, 50-100%). The long-term anatomic and functional success of the MOOKP makes it a reliable option for visual rehabilitation in patients with bilateral corneal blindness and end-stage ocular surface disease. This review aims to describe the evolution of the MOOKP procedure, analyzing all published case series for its long-term reliability, visual and anatomical outcomes, complications, and future directions.

The impact of central foveal thickness and integrity of the outer retinal layers in the visual outcome of uveitic macular edema.

BACKGROUND: To analyze the relationship between the central foveal thickness (CFT) and the integrity of the ellipsoid portion of inner segments (EPIS) and interdigitating zone (IZ) retinal layers in the visual outcome of uveitic macular edema (UME). METHODS: Prospective, observational, and cross-sectional study of eyes with UME. Spectral-domain optical coherence tomography (SD-OCT) macular morphological pattern, CFT, and integrity of the outer retinal layers were analyzed. We arranged the data by EPIS or IZ integrity and contrasted it with student t-test (quantitative variables) and Fisher exact test or χ² distribution (categorical variables) to evaluate visual impairment and retinal measures. Receiver operator curve (ROC) estimation and logistic regression (probit) assessed if the sample´s variance could be associated with IZ or EPIS integrity. RESULTS: We included 145 SD-OCT macular scans from 45 patients at different stages of UME. Cystoid macular edema (CME) increased the risk of severe (P ≤ 0.0162) and moderate visual loss (P ≤ 0.0032). The highest CFT values occurred in patients with moderate (478.11 ± 167.62 µm) and severe (449.4 ± 224.86 µm) visual loss. Of all morphological patterns of macular edema, only CME showed a statistically significant relationship with severe visual impairment (44.92%, p = 0.0035, OR 4.29 [1.62-11.4]). Likewise, an increased probability of severe visual loss correlated negatively with both, IZ (37.93%, P ≤ 0.001, OR 10.02) and EPIS (38.98%, P ≤ 0.001, OR 13.1) disruption. A CFT > 337 µm showed a higher probability of IZ (AUROC = 0.7341, SEN 77.59%, ESP 65.52) and EPIS (AUROC = 0.7489, SEN 76.37%, ESP 65.12%) loss of integrity. Moreover, when BCVA reached 0.44 LogMAR (≤ 20/50 Snellen eq.), it was more likely to have IZ (AUROC = 0.8706, ESP 88.51%, SEN 77.59%) and EPIS (AUROC = 0.8898, ESP 88.3%, SEN 76.27) disruption. CONCLUSIONS: Significantly increased CFT has a higher probability for EPIS and IZ disruption, which significantly increases the risk for irreversible visual loss in eyes with UME. Evaluating these layers' integrity by optical coherence tomography helps predict the visual outcome and make the right therapeutic decisions. Trial registration The study was registered on April 13, 2020, at the Instituto Tecnologico y de Estudios Superiores de Monterrey Research Committee (License No. COFEPRIS 20 CI 19 039 002), project registration No. P000338-CAVICaREMU-CI-CR002, and the Ethics Committee (License No. CONBIOETICA 19 CEI 011-2016-10-17), project registration No. P000338-CAVICaREMU-CEIC-CR002.

Update on indications, complications, and outcomes of scleral contact lenses.

Background: The role of scleral contact lenses (SCLs) has increasingly expanded since the first lens was fitted more than a century ago. While it was initially prescribed for the management of severely compromised corneas, the indications for modern SCL use have expanded to include less severe diseases. In this review, we aimed to provide an up-to-date overview of the current indications, complications, and outcomes for the various types of SCLs. Methods: In this narrative review, we thoroughly searched the PubMed/MEDLINE database for literature published from January 1980 to November 2021. Only relevant up-to-date English references were included. Furthermore, the figures in this manuscript were derived from our unit's patient documentation. Results: Currently, SCLs can successfully be used to manage ocular surface diseases, visually rehabilitate irregular corneas, and correct irregular refractive errors. Although newer materials have yielded the same visual outcomes with fewer complications, these consequences still occur in approximately one-third of contact lens wearers, including difficulties in insertion and/or removal, discomfort or pain, and developing either halos, blurriness, or haze. Even though most of these complications are minor and can be easily treated, a good practice is essential to avoid sight-threatening complications such as microbial keratitis. Conclusions: SCLs are indispensable in ophthalmic clinics. The development of better-quality SCLs has increased the number of indications and improved the achievable visual rehabilitation. The future of developing improvements in SCL design, materials, and fit, and the expansion of their indication range is promising.