Intralesional or intraorbital rituximab injection for the management of biopsy-proven idiopathic orbital inflammation involving the lacrimal gland.

OBJECTIVE: To evaluate the efficacy of intralesional rituximab injection for the management of idiopathic orbital inflammation (IOI) involving the lacrimal gland, which is the most common subtype. METHOD: Eighteen consecutive patients with biopsy-proven IOI involving the lacrimal gland were included. Rituximab (50 mg/5 mL) was injected intralesionally at monthly intervals. RESULTS: Clinically, all patients presented with upper eyelid swelling and ptosis. Most patients (56%) had periocular pain and a palpable superotemporal mass. Biopsies showed chronic inflammation without fibrosis in 14 patients (78%) and chronic inflammation and fibrosis in 4 patients (22%). Intralesional rituximab was injected once in 1 patient (6%) because of complete response after the first injection, twice in 11 patients (61%), and 3 times in 6 patients (33%) because of partial response after 2 injections. After a mean follow-up of 33 months (median, 33 months; range, 11-59 months), 16 patients (89%) showed a clinical response, including 14 patients (78%) a complete response (i.e., disappearance of all lesions) and 2 patients (11%) with a partial response (i.e., ≤30% decrease in lesion diameter). Two patients (11%) did not respond after 3 injections and were placed on systemic corticosteroid and methotrexate therapies. Two patients (11%) with a complete response developed subsequent recurrence 12 and 49 months after their last injections. Both were treated with 2 additional rituximab injections, 1 month apart, and showed complete response when examined 27 and 11 months after treatment, respectively. CONCLUSION: Intralesional rituximab injection may be an effective treatment for IOI involving the lacrimal gland, achieving a 78% complete response rate in this series. Local treatment with rituximab has the potential to avoid the ocular and systemic side effects of corticosteroid and systemic immunosuppressive treatment.

Integrative Exome and Transcriptome Analysis of Conjunctival Melanoma and Its Potential Application for Personalized Therapy.

Importance: Greater understanding of molecular features of conjunctival melanoma (CM) may improve its clinical management. Objective: To evaluate molecular features of CM and application of this information into clinical care. Design, Setting, and Participants: In a prospective case series of CM with integrative exome and transcriptome analysis, 8 patients at an academic ocular oncology setting were evaluated. The study was conducted from November 2015 to March 2018. Interventions/Exposures: Integrative exome and transcriptome analysis of CMs and clinical management of a patient's care by using this information. Main Outcomes and Measures: Molecular characterization of CM and its potential clinical application. Results: In the 8 patients (4 men) included in analysis, 4 subgroups of CM were observed, including the BRAF V600E mutation in 1 tumor, NRAS Q61R mutation in 3 tumors, NF1 mutations (Q1188X, R440X, or M1215K+ S15fs) in 3 tumors, and triple-wild type (triple-WT) in 1 tumor. The triple-WT case had CCND1 amplification and mutation in the CIC gene (Q1508X). Five tumors, including the triple-WT, also harbored mutations in MAPK genes. In addition to the genes linked to mitogen-activated protein kinase and phosphoinositol 3-kinase pathways, those involved in cell cycle and/or survival, ubiquitin-mediated protein degradation, and chromatin remodeling/epigenetic regulation (ATRX being the most frequently mutated: noted in 5 tumors) may play an important role. Other frequently mutated genes included PREX2 (n = 3), APOB (n = 4), and RYR1/2 (n = 4), although their relevance remains to be determined. The mutation burden ranged from 1.1 to 15.6 mutations per megabase (Mut/Mb) and was 3.3 Mut/Mb or less in 3 tumors and more than 10 Mut/Mb in 2 tumors. A patient with a large tumor and BRAF V600E mutation was treated with combined systemic BRAF (dabrafenib) and MEK (trametinib) inhibitors. After 3 months of therapy, her CM responded substantially and the residual tumor was removed by local surgical excision. Conclusions and Relevance: The NRAS Q61R and NF1 mutations were more common than the BRAF V600E mutation in this series. Although small tumors (where incisional biopsy is not indicated) are treated with surgical excision regardless of mutational profile, in large tumors carrying the BRAF V600E mutation, neoadjuvant therapy with combined systemic BRAF and MEK inhibitors followed by local excision may be used as an alternative to exenteration. Integrative omics analysis of CM may be informative and guide clinical management and treatment in selected cases.

Systemic anti-CD20 (rituximab) as primary treatment for symptomatic primary uveal lymphoma.

PURPOSE: Uveal lymphomas are indolent, frequently choroid-involving neoplasms that are mainly CD20-positive B-cell extranodal marginal zone lymphoma. Irreversible visual loss may occur from retinal detachment and/or glaucoma among untreated symptomatic patients, or from radiation-induced changes secondary to external beam radiotherapy. To avoid radiation-induced complications, we used systemic rituximab monotherapy as primary treatment, and present two cases to show its long-term effectiveness for symptomatic primary uveal lymphoma. OBSERVATIONS: Two elderly men who presented with painless blurred vision were clinically diagnosed with symptomatic primary uveal lymphoma, which were biopsy-confirmed to be marginal zone lymphoma. Both patients with symptomatic, primary marginal zone uveal lymphoma that appeared as multiple yellow, nummular choroidal infiltrates, had complete ocular remission after three and one cycles of systemic rituximab monotherapy (375mg/m2 infused intravenously once weekly for four consecutive weeks), with disappearance of the lesions and improvement of visual acuity. Both patients tolerated systemic monotherapy well without any adverse systemic or ocular effects. There was no local ocular recurrence at 29 and 39 months after the last treatment. CONCLUSIONS: and Importance: Systemic rituximab monotherapy induced complete ocular remission and improved visual acuity, without adverse effects, and without local ocular recurrence of uveal lymphoma 29-39 months following the last treatment. To our knowledge, this is the first manuscript to show long-term effectiveness of systemic rituximab monotherapy as the primary treatment for symptomatic primary uveal lymphoma. Long-term follow-up of this indolent neoplasm is still imperative to monitor its ocular and systemic course.

Removal of Tenon fortified by conjunctival-limbal autograft in treatment of pterygium.

PURPOSE: To describe a simple and effective technique for the prevention of pterygium recurrence. METHODS: This is a retrospective consecutive single-center case series with recurrence rate as the main outcome measure. Dissection of the conjunctiva, the Tenon's layer, and the sclera was continued up to the insertion of medial rectus. The exposed Tenon's layer was excised for 2 to 3 mm. The entire free edges of the conjunctiva was tacked firmly to the sclera. The conjunctival-limbal graft was sutured firmly to the sclera, recipient limbus, and to the conjunctiva incorporating the reciprocal edges. RESULTS: We report findings in a total of 36 patients, with 20 (%55.5) males and 16 (%44.4) females. Median age of the patients was 44.5 years (range 16-80). The mean follow-up period was 23.1 ± 6.5 months. The mean size of pterygium in mm length was 3.8 ± 0.7. Recurrence did not occur in any patient. Diplopia was not reported by any patients. CONCLUSIONS: Conjunctival-limbal autograft combining with selective exicison of Tenon's layer but without damage to any essential tissue such as the semilunar fold or Tenon's capsule pulley is a relatively simple and effective procedure.

Hemorrhagic retinal infarction due to inadvertent overdose of cefuroxime in cases of complicated cataract surgery: retrospective case series.

PURPOSE: To present 4 patients that developed hemorrhagic retinal infarction attributable to inadvertent overdose of cefuroxime after cataract surgery. DESIGN: Case series. SUBJECTS AND METHODS: Surgery in 4 patients was complicated-the posterior capsule was absent or torn-and anterior vitrectomy was performed. Cefuroxime was inadvertently injected at a dose higher than recommended in all 4 cases. RESULTS: Case 1 had hemorrhage in the central and inferior retinal regions, as well as optic atrophy. Case 2 had hemorrhage in the peripapillary and macular regions, as well as optic atrophy. Case 3 had hemorrhage in the peripapillary and inferior retinal regions, as well as macular pucker attributable to fibrovascular formation in the central retinal region. Case 4 had hemorrhage in the peripapillary, macular, and inferior retinal regions. The cefuroxime doses administered to the presented patients were much higher than reported in other cases and resulted in a higher concentration in the vitreous. Consequently, the severity of toxicity was much higher than in other reported cases of cefuroxime-induced toxicity. CONCLUSION: In cases of intracameral cefuroxime overdose, hemorrhagic retinal infarction can develop after cataract surgery.

Sensory exotropia due to keratoconus and review of the literature.

This case report describes a 17-year-old boy with sensory strabismus due to keratoconus and an ipsilateral nodular lesion of the bulbar conjunctiva. The aligned eye was the right eye and keratoconus in this eye was of late onset. Vision in the left eye was poor and keratoconus was advanced in this eye. Due to the longstanding nature of the keratoconus and its occurrence in a developmentally sensitive period, sensory exotropia had developed in the left eye. There was a nodular lesion of the bulbar conjunctiva in the ipsilateral eye. If keratoconus occurs before the age of 7 years and the prolonged visual loss is not corrected, sensory strabismus can develop, as in this patient.

Sensory exotropia associated with keratoconus and review of literature: strabismus and keratoconus.

PURPOSE: To present a case of sensory strabismus due to keratoconus with an ipsilateral nodular lesion of the bulbar con-junctiva. CASE PRESENTATION: The patient is a 17 year-old-boy. The fixing eye was the right eye and keratoconus in this eye was late onset. Vision in the left eye was poor and keratoconus was advanced in this eye. Due to longstanding of keratoconus and occurrence in the sensitive period, sensory exotropia had developed in the left eye. A nodular lesion of the bulbar conjunctiva was also present in the ipsilateral eye. CONCLUSION: Sensory exotropia is not unusual but to be due to longstanding keratoconus is not a usual strabismus. If keratoconus develops before 7 years of age, sensory strabismus can develop. We reported and reviewed literature that keratoconus seldom begins in the sensitive period where abnormal visual experience affects visual development and may induce sensory exotropia.

Evaluation of ultrasonic biomicroscopy results in anterior eye segment before and after cataract surgery.

BACKGROUND: The aim of this study was to assess the value of ultrasonic biomicroscopy in reporting decreases in intraocular pressure resulting from changes in anterior chamber depth and angle after phacoemulsification and intracapsular lens implantation in patients with cataract. METHODS: This prospective interventional case series included 50 eyes of 50 consecutive subjects operated at the same center. Patients with eye disease affecting visual acuity, a history of eye surgery, corneal surface irregularities, a pupil diameter < 5 mm after preoperative dilation, aged younger than 35 years, posterior capsule perforation, iris dialysis during surgery, intensive postoperative corneal edema, and inability to attend adequate follow-up were excluded. Intraocular pressure, anterior chamber depth and angle, and corneal thickness were measured before and one month after surgery. RESULTS: The mean preoperative intraocular pressure was 14 mmHg and postoperatively was 11 mmHg. Mean anterior chamber depth preoperatively was 2.8 mm and increased to 3.7 mm postoperatively. The mean anterior chamber angle was measured as 27° preoperatively and as 42° postoperatively. CONCLUSION: After phacoemulsification and intracapsular lens implantation, ultrasonic biomicroscopy showed that the iris diaphragm had shifted backwards, widening the angle of the anterior chamber and decreasing intraocular pressure.

Unilateral tilted disc and ipsilateral keratoconus in the same eye.

The objective of this case was to report unilateral tilted disc in a boy with ipsilateral keratoconus. The tilted disc syndrome is a non-hereditary bilateral condition. This configuration is accompanied by situs inversus of the retinal vessels, congenital inferonasal conus, thinning of the inferonasal retinal pigment epithelium and choroid, and myopic astigmatism. Unilateral tilted disc syndrome is a rare condition. Keratoconus is a disorder characterised by progressive corneal steepening. The author present a case of unilateral tilted disc in a boy with ipsilateral keratoconus. Pterygium is a common disorder and tilted disc syndrome is a bilateral condition. But unilateral tilted disc in a boy with ipsilateral keratoconus is the first report in literature. Due to these clinical presentation, this report is an exception in literature and reported an unknown clinical coincident.

Intravitreal bevacizumab combined with panretinal photocoagulation in the treatment of open angle neovascular glaucoma.

PURPOSE: To evaluate the clinical efficacy of intravitreal bevacizumab (IVB) combined with panretinal photocoagulation in patients with open angle neovascular glaucoma (NVG). METHODS: Nine patients (9 eyes) with NVG participated in this study. Patients received IVB (1.25 mg) as the initial treatment for NVG and were followed up for at least 4 months. IVB was offered as the first treatment of choice to patients with NVG. Panretinal photocoagulation was performed as soon as feasible after the second week and completed in all patients the fourth week after IVB. The main outcome measures are resolution of INV, inhibition of peripheral anterior synechia (PAS), and controllability of intraocular pressure (IOP). RESULTS: The mean follow-up period was 5.6+/-1.4 months (range, 4-9 months). The mean IOP before treatment was 35.1+/-9.7 mmHg (range, 24-56) under medication before IVB treatment. After IVB and after combined treatment, the mean IOP was reduced to 22.8+/-8.1 mmHg (range, 9-33) and 13.0+/-4.0 mmHg (range, 7-20), respectively. The mean referral INV was 3.6+/-0.4 grade (range, 3-4) and reduced to 1.6+/-0.4 (range 1-2) grade after IVB and 0.6+/-0.8 (range 0-2) grade after combined therapy. By IVB, combined panretinal photocoagulation recurrence of INV was not observed. CONCLUSIONS: In NVG, IVB treatment can reduce iris and angle neovascularization and inhibits further PAS formation temporarily. Panretinal photocoagulation inhibits neovascularization constantly. Therefore, management of open angle NVG is more feasible with bevacizumab combined with panretinal photocoagulation.

Apert's syndrome: ophthalmic importance and clinical findings.

Apert's syndrome is a rare form of craniosynostosis that exhibits with many ocular manifestations. We present two cases of Apert's syndrome. Our first case is a 10-year-old girl admitted with exotropia, V pattern and proptosis on examination. Investigations revealed coronal craniosynostosis, cleft palate, vaginal atresia and syndactyly of the hands and feet. The second case is a 5-year-old boy presented with hypertelorism, exotropia, dissociated vertical deviation and proptosis. Investigations revealed coronal craniosynostosis, bifid uvula and syndactyly of the hands and feet.