Corneal Neurotization for Neurotrophic Keratopathy: A Multicentre Experience.

PURPOSE: Corneal neurotization is an emerging technique that offers potential for visual rehabilitation in neurotrophic keratopathy. This study reports on a multicenter experience and outcomes for both direct and indirect methods of corneal neurotization. METHODS: Retrospective case series. Sixteen patients with neurotrophic keratopathy who underwent corneal neurotization across 5 centers in Australia and Israel were identified for inclusion. Corneal neurotization was performed via direct neurotization from the ipsilateral or contralateral supraorbital/supratrochlear nerve or by the use of an interpositional sural nerve graft. Change in corneal sensitivity (measured in millimeters by the Cochet-Bonnet aesthesiometer), visual acuity, and corneal health. RESULTS: Over a mean follow-up period of 31.3 months (range: 3 months-8 years), mean corneal sensitivity improved from 3.6 mm (range: 0-25 mm) to 25.3 mm (range: 0-57 mm). Visual acuity improved on average from 20/380 to 20/260. Twelve of 16 patients (75.0%) improved in at least 2 out of the 3 main outcome measures. Nine patients (56.3%) showed an improvement in visual acuity; 13 (81.3%) showed an improvement in average corneal sensitivity; and 11 (68.8%) showed an improvement in corneal health. There were no intraoperative or postoperative complications. CONCLUSIONS: Corneal neurotization is an emerging surgical treatment option for the management of neurotrophic keratopathy. With appropriate case selection, outcomes are favorable and complication rates are low, for a condition that is otherwise challenging to manage. Patients with severe neurotrophic keratopathy should be considered for this surgical treatment option.

Cognitive Performance of Patients With Multiple Sclerosis and Optic Neuritis at Presentation.

BACKGROUND: Cognitive dysfunction is common among patients with multiple sclerosis (MS), but the effect of coexisting optic neuritis (ON) at the first presentation of multiple sclerosis on the course of cognitive decline is unknown. The purpose of this study was to assess whether ON at presentation has any effect on the progression of cognitive decline in MS. METHODS: Historical cohort study. We retrospectively compared the cognitive performance of patients with relapsing-remitting MS with and without ON at the time of MS diagnosis. Subjects were included if cognitive test results were available both at baseline and after at least 36 months from presentation and grouped based on the presence (MS-ON) or absence (MS-non-ON) of optic neuritis at presentation. RESULTS: One hundred seventy consecutive subjects with MS were found suitable, with a 1:2 male:female ratio and a mean age at diagnosis of 33.0 ± 10.9 years. Forty-six patients (27.1%) presented with ON. No significant differences were found in cognitive performance at onset between the 2 groups. Both groups had a similar follow-up duration. The prevalence of cognitive decline in the general score was significantly higher in the MS-ON group compared with the MS-non-ON group (6.5% vs 0%, respectively; P < 0.001), as well as in the attention (8.7% vs 1.6%; P = 0.046) and the executive function (17.4% vs 2.4%; P = 0.001) domains. CONCLUSIONS: Optic neuritis at presentation of MS is associated with a higher prevalence of cognitive decline over time. Potential benefit of early intervention to prevent cognitive decline may be warranted.

A Multidisciplinary Thyroid Eye Clinic: A One-stop Shop.

BACKGROUND: Little is known about the success of multidisciplinary thyroid eye disease (TED) clinic. OBJECTIVES: To present the characteristics, treatments, and outcomes of patients treated in a multidisciplinary TED clinic. METHODS: A medical record review of all patients who attended a TED clinic was performed. Data included demographics, medical history, laboratory tests, visual function tests, ocular examinations, clinical activity score (CAS), and assessment of quality-of-life (QOL). RESULTS: Clinic visits included 132 patients seen during 385 appointments at a TED clinic (mean 12 appointments per patient). Management of TED included medical treatments for 48 patients (36.3%) and surgical treatment for 56 (42.4%). There was a positive significant correlation between the CAS and thyroid-stimulating immunoglobulin (TSI) activity at the first visit and at the last follow-up visit (P < 0.01 and P < 0.02, respectively). However, no correlation was found between the CAS and the thyroid-stimulating hormone levels or between the free triiodothyronine (fT3) and fT4 levels at the first or last visit. There was a significant negative correlation between the CAS and color vision (-0.347, P < 0.01, Pearson correlation) at the first visit, but not between the CAS and visual acuity and visual field at either the first or last visit. Changes in the QOL and the CAS scores were significantly negatively correlated (-0.240, P < 0.01). CONCLUSIONS: Treatment and management decisions for TED should be based on multiple parameters including clinical examinations by ophthalmologists and endocrinologists, laboratory tests, and CAS and QOL scores.

New insight into the physiologic function of Müller's muscle.

PURPOSE: Müller's muscle is a sympathetically innervated smooth muscle which serves as an accessory upper eyelid retractor. Its physiologic function and purpose have not yet been clearly defined. We hypothesize that sympathetic innervation to Müller's muscle serves to adjust the upper eyelid's position to variations in pupil size in response to changes in light intensity. METHODS: This is a single center cross-sectional study. Healthy volunteers were asked to fixate on a distant non-accommodative target, and a video scan of the anterior segment was performed for each subject's right eye using the Heidelberg Spectralis® optical coherence tomography scanner in infrared mode. The video was taken both in photopic and scotopic conditions, recording the resultant transition of the pupil and eyelids. The pupil diameter (PD), upper eyelid margin-to-reflex distance (MRD1), lower eyelid margin-to-reflex distance (MRD2), and vertical palpebral fissure height (PFH) were measured. RESULTS: Thirty-three healthy volunteers (19 women, 57.6%) with a median age of 40 years (range 30-58) were included. The mean PD under photopic conditions increased significantly under scotopic conditions, from 3483 ± 521 µm to 6135 ± 703 µm, respectively (P < 0.0001). An increase in MRD1 was observed following transition from light to dark, with a mean change of 348 ± 311 µm (P < 0.0001). There was no significant change in MRD2. CONCLUSIONS: Upper eyelid retraction occurs after transition from photopic to scotopic conditions. This movement suggests the existence of an "eyelid-light reflex" involving Müller's muscle that adjusts the position of the eyelids as the pupil dilates under scotopic conditions.

Quality of life of patients with thyroid eye disease: 3-year follow-up in a multidisciplinary clinic in Israel.

BACKGROUND: Changes in the quality of life (QOL) of patients with thyroid eye disease (TED) were examined during a 3-year follow-up in a multidisciplinary eye clinic, and factors that may improve QOL were identified. METHODS: A retrospective review of medical records of all patients who attended the TED clinic at Sheba Medical Center, Israel, from May 2016 to May 2019 was performed. The retrieved data included demographics, comprehensive ophthalmic examination findings, clinical activity scores (CAS), laboratory test results, and QOL assessments by the Graves' Orbitopathy QOL (GO-QOL) questionnaire. RESULTS: One hundred thirty-two TED clinic patients were examined. Thirty patients (22.72%) received medical treatment consisting of steroids according to the European Group on Graves' Orbitopathy (EUGOGO) protocol, high-dose steroids, or immunosuppressive drugs. Twenty-eight patients (21.21%) underwent surgical rehabilitation (decompression, strabismus, or eyelid surgery). There was a significant increase in total QOL score after steroid treatment according to the EUGOGO protocol, after decompression surgery, and after strabismus surgery compared to pre-treatment total QOL (p=0.04, p=0.021, and p=0.042, respectively, matched pairs). In addition, there were significant positive correlations between the changes in the total QOL score and the change in thyroid-stimulating immunoglobulin (TSI) as well as the change in CAS among the patients who underwent medical and surgical interventions. CONCLUSIONS: QOL improved significantly after medical/surgical treatments. A change in the CAS and in the TSI may also correlate with change in QOL. Periodic evaluation of TED patients' QOL is recommended for enhanced and more comprehensive management.

Low-dose Bleomycin Injections for Orbital Lymphatic and Lymphatic-Venous Malformations: A Multicentric Case Series Study.

PURPOSE: Orbital lymphatic malformations (LM) are associated with ocular morbidity and facial disfigurement. Surgery is challenging and may not be effective. We describe the outcome of bleomycin injections for venous LM and lymphatic-venous malformation (LVM) malformations of the orbit in 5 tertiary referral centers between January 2010 and December 2018. METHODS: Multicenter retrospective case series, 5 oculoplastic referral centers: Sheba and Rabin Medical Centers, Israel; Mulago Hospital, Uganda; Sri Sankaradeva Nethralaya, India; and Clinique Ophtalmologique de Tunis, Tunisia. All patients diagnosed with orbital LM/LVM were assigned to successive (range 1-6) intralesional 5 international units bleomycin injections. They all underwent complete ophthalmic and orbital evaluations, orbital imaging, and ancillary testing as needed. Clinical photographs were assessed pre- and posttreatment along with objective assessments of clinical improvement. Additional injections were provided in cases of incomplete response. RESULTS: A total of 21 patients (17 women, mean ± standard deviation age 18 ± 13 years, range 2-48 years) underwent bleomycin injections. The mean injection dose was 12 ± 10 international units in 1-3 injections. There was a dramatic improvement in lesion size, appearance, proptosis, and ocular motility in 20/21 patients (95%) after a mean follow-up of 18 months. Visual acuity slightly improved after treatment (20/50-20/30; P = 0.076). No side effects were noted after bleomycin injections. CONCLUSIONS: Bleomycin injections for LM/LVM of the orbit are effective; local or systemic side effects were not seen in this series. To the best of our knowledge, this is the largest reported series of this treatment.

Kelly Punch Punctoplasty and Simple Punctal Dilatation Are Equally Effective for Punctal Stenosis: A Prospective Study.

PURPOSE: To examine the clinical, imaging, and histopathologic differences between Kelly punch punctoplasty and punctal dilatation, both with mini Monoka stent insertion. METHODS: This is a prospective, comparative clinical study. Patients with bilateral punctal stenosis and for whom surgery was advised participated in the study. The right eye underwent Kelly punch punctoplasty and the left eye simple punctal dilatation, both with mini Monoka stents were participated. Data were collected and analyzed before and 3 months following the procedure and included the degree of epiphora (Munk score) and punctal size as measured by anterior segment optical coherence tomography (AC- OCT). All specimens removed by the Kelly punch punctoplasty were sent for histopathologic evaluation, including Masson's trichome muscle staining. RESULTS: The study included 46 eyes of 23 patients (4 males and 19 females) whose mean age at presentation was 60.43 ± 14.81 years (range 19-84 years). There was a significant decrease in the Munk score after both punctoplasties compared with baseline values (P < 0.01, matched pairs). There were no significant differences between groups in the delta Munk score. There was a significant increase in punctal size after both procedures compared with baseline values as measured by AS-OCT. Horner-Duverney's muscle was visible in only 3 of the 23 (13.04%) specimens from patients who underwent the Kelly punch punctoplasty. CONCLUSIONS: The Kelly punch punctoplasty and the simple dilator punctoplasty, both with stents, are equally effective treatments for epiphora due to acquired punctal stenosis. Only a few specimens removed during the Kelly punch method show muscle fibers.

Changes in Refraction and Visual Acuity after Upper Eyelid Blepharoplasty versus Posterior Approach Ptosis Procedures.

PURPOSE: To evaluate surgically induced refractive changes (SIRC) and visual acuity (VA) changes after blepharoplasty combined with posterior approach ptosis surgery (Müller's muscle-conjunctival resection [MMCR]) versus upper eyelid blepharoplasty alone. METHODS: In this prospective, comparative, clinical study on patients undergoing MMCR and blepharoplasty, comprehensive ophthalmic examinations were performed preoperatively and 3 months postoperatively. SIRC were calculated with the 10-step Holladay method. RESULTS: Fifty-six patients participated in the study, 31 in the blepharoplasty group and 25 in the ptosis group. logMAR VA improved significantly after surgery in both groups (p < 0.001). In both groups, most patients showed significant changes in SIRC sphere and spherical equivalent of >0.5 D (blepharoplasty group: 61.29 and 67.74%; ptosis group: 72.72 and 72.72%, respectively). Patients undergoing combined blepharoplasty ptosis surgery showed the greatest SIRC cylinder. CONCLUSIONS: Upper eyelid blepharoplasty with or without MMCR is associated with significant SIRC 3 months postoperatively. This may affect decision-making for all patients, especially for those who intend to seek refractive correction in addition to the index upper eyelid surgery.

Change in Eyelid Carcinoma T Category With Use of the 8th Versus 7th Edition of the American Joint Committee on Cancer: Cancer Staging Manual.

PURPOSE: To evaluate the frequency and nature of changes in T category when eyelid carcinomas are staged using the criteria in the 8th edition instead of the 7th edition of the American Joint Committee on Cancer staging manual. METHODS: Following Institutional Review Board approval, a retrospective review was conducted for all consecutive patients with the diagnosis of eyelid carcinoma treated by the senior author from January 2012 through December 2016. After a review of the clinical and pathologic data, each patient's disease was staged using both the 7th-edition and 8th-edition American Joint Committee on Cancer criteria for eyelid carcinomas. Changes in T categories between the 2 staging systems were examined. RESULTS: The review initially identified 167 patients with the diagnosis of eyelid carcinoma. Four patients were excluded because of incomplete or unclear data. The remaining 163 patients included 78 men and 85 women aged 21 to 97 years (median, 68 years). Eighty-two patients had basal cell carcinoma; 35, squamous cell carcinoma; 32, sebaceous carcinoma; 6, mucinous eccrine carcinoma; 3, Merkel cell carcinoma; 3, adenocarcinomas; and 2, adnexal carcinoma. The most common T category according to the 7th-edition criteria was T2a; the most common T category according to the 8th-edition criteria was T1b. Of the 163 patients, 64 (39%) had a lower T category with the 8th-edition than with the 7th-edition criteria, 59 (36%) had a higher T category, and 40 (25%) had the same T category. CONCLUSIONS: Application of the 8th-edition American Joint Committee on Cancer criteria for eyelid carcinoma changed the T category in 75% of patients. In general, the new 8th-edition American Joint Committee on Cancer tumor, node, metastasis (TNM) designations allow for a more objective and consistent designation of the T category.

Impact of Food and Drug Administration Approval of Vismodegib on Prevalence of Orbital Exenteration as a Necessary Surgical Treatment for Locally Advanced Periocular Basal Cell Carcinoma.

PURPOSE: To test the hypothesis that the US Food and Drug Administration approval of vismodegib in early 2012 has reduced the prevalence of orbital exenteration for locally advanced periocular basal cell carcinoma (BCC). METHODS: Following institutional review board approval, the authors reviewed clinical and pathological data of patients with locally advanced periocular BCC (T4 per the eyelid carcinoma classification in the 8th edition of the AJCC Cancer Staging Manual) treated by the senior author during 2006-2018. Patients were grouped into those who were treated before February 2012 ("before vismodegib approval") and those who presented later ("after vismodegib approval"). RESULTS: Forty-two patients with locally advanced periocular BCC were treated during the study period, of whom 31 were men. The median age at presentation was 66 years (range, 43-90). Twenty-two patients had T4a and 20 had T4b tumors. Thirteen patients were treated before and 29 were treated after vismodegib approval. The 2 groups did not differ in age distribution (p = 0.164), sex distribution (p = 0.270), prevalence of recurrent tumor at presentation (p = 0.317), or duration of treatment with vismodegib (p = 0.605). Orbital exenteration was significantly more prevalent in patients treated before vismodegib approval than after (46% vs. 10%, p = 0.016), and vismodegib treatment was significantly more prevalent in patients treated after vismodegib approval than before (when vismodegib was given in clinical trials; 69% vs. 23%, p = 0.008). There was a trend toward more patients retaining their eyes at last follow-up in patients treated after vismodegib approval (83% vs. 54%, p = 0.066). CONCLUSIONS: The prevalence of orbital exenteration as a necessary surgical procedure in patients with a locally advanced periocular BCC has fallen since the Food and Drug Administration approval of vismodegib. Although vismodegib is not specifically approved for organ-sparing, it has changed the authors' practice and enabled eye preservation in patients with locally advanced periocular BCC, who would otherwise require an orbital exenteration.

Extraocular Muscle Enlargement and Thyroid Eye Disease-like Orbital Inflammation Associated with Immune Checkpoint Inhibitor Therapy in Cancer Patients.

PURPOSE: To describe thyroid eye disease (TED)-like orbital inflammatory syndrome in 3 cancer patients treated with immune checkpoint inhibitors. METHODS: All consecutive patients treated by the senior author who were receiving immune checkpoint inhibitors and developed TED-like orbital inflammation were included. RESULTS: Three cancer patients treated with immune checkpoint inhibitors developed orbital inflammation. The first patient was treated with a combination of a cytotoxic T-lymphocyte antigen-4 inhibitor and a programmed cell death protein 1 inhibitor and developed TED-like orbital inflammation with normal thyroid function and antibody levels. The second patient had a previous diagnosis of Graves disease without TED, and developed TED soon after initiating treatment with a programmed cell death protein 1 inhibitor. The third patient developed acute hyperthyroidism with symptomatic TED following treatment with an investigational cytotoxic T-lymphocyte antigen-4 inhibitor agent. All 3 patients were managed with either systemic steroids or observation, with resolution of their symptoms and without the need to halt immune checkpoint inhibitor treatment for their cancer. DISCUSSION AND CONCLUSIONS: TED-like orbital inflammation may occur as a side effect of immune checkpoint inhibitor therapy with anti-cytotoxic T-lymphocyte antigen-4 or anti-PD-1 inhibitors. To the best of their knowledge, this is the first reported case of TED as a result of programmed cell death protein 1 inhibitor monotherapy. All 3 patients were treated with systemic steroids and responded quickly while continuing treatment with immune checkpoint inhibitors for their cancer. With increasing use of this class of drugs, clinicians should be familiar with the clinical manifestations and treatments for this adverse reaction.

Prevalence of a Histologic Change of Ocular Adnexal Lymphoma in Patients With a History of Lymphoma.

PURPOSE: The authors examined the prevalence of a histologic change of ocular adnexal lymphoma (OAL) grade in patients with a history of lymphoma in nonocular sites. METHODS: In this retrospective study, the authors reviewed the clinical and pathological data of 209 patients with OAL treated by the senior author during 2000 to 2017. RESULTS: Of 209 patients with OAL, 65 (31%) had a history of lymphoma. In 54 of the 65 patients (83%), the original lymphoma and OAL were of the same histologic type. In 8 of the 65 patients (12.3%), the OAL was more indolent than the original lymphoma: 6 patients with a history of diffuse large B-cell lymphoma, one of mantle cell lymphoma, and one of grade 3 follicular lymphoma had biopsy-proven extranodal marginal-zone lymphoma in the orbital area. Two additional patients (3%) with a history of chronic lymphocytic leukemia developed OAL: diffuse large B-cell lymphoma in one patient and extranodal marginal-zone lymphoma in the other. One patient (1.5%) with a history of a low-grade follicular lymphoma relapsed as a different low-grade histology of extranodal marginal-zone lymphoma. Lower-grade OAL than the original lymphoma was more common than higher-grade OAL than the original lymphoma (p = 0.048). CONCLUSIONS: In this cohort of 209 patients with OAL, the authors found that nearly one third had a history of lymphoma, 17% of whom had a different histologic type of lymphoma in the orbit, more commonly a more indolent type. This underscores the importance of biopsy of OAL even in patients with a known history of lymphoma to determine the histologic subtype of orbital lymphoma and to help guide appropriate treatment.

Pathological Features and Clinical Course in Patients With Recurrent or Malignant Orbital Solitary Fibrous Tumor/Hemangiopericytoma.

PURPOSE: A risk assessment score for metastasis based on age, tumor size, and mitotic figures has been suggested for nonorbital solitary fibrous tumor (SFT)/hemangiopericytoma. The authors herein examine the clinicopathological features of recurrent and metastatic orbital SFT and evaluate the existing risk assessment score for orbital SFT. METHODS: The American Society of Ophthalmic Plastic and Reconstructive Surgery Oncology Database was queried for patients with recurrent or malignant orbital hemangiopericytoma/SFT. The medical records were reviewed for clinical and pathologic findings, treatments, and outcomes. RESULTS: Eight patients from 3 institutions were identified with recurrent orbital hemangiopericytoma/SFT. Median age at diagnosis was 59 years, and 4 patients were women. The mean size of tumor was 2.1 ± 1.1 cm. All patients were initially treated with surgery and experienced local recurrence after a median of 4 (range 0.5-10) years. Five patients were treated with orbital radiation. Two patients also developed distant metastases and eventually died of their disease. Median Ki-67 was 5% (range 1-65%) and 5 mitotic figures/10 high-power fields (range 2-30). The previously described risk stratification model for nonorbital SFT did not correlate with the propensity to develop metastases in this cohort; however, both patients with distant metastasis had > 4 mitotic figures /10 high-power fields. CONCLUSIONS: In this cohort of recurrent orbital hemangiopericytoma/SFT, median time to recurrence was 4 years underscoring the importance of careful continued follow-up. The current risk stratification models have limited use for orbital lesions, mostly due to the fact that orbital SFTs are smaller than even the smallest size criteria in this risk assessment model.

Rituximab Monotherapy Is Effective in Treating Orbital Necrobiotic Xanthogranuloma.

The authors report a case of a patient with bilateral orbital necrobiotic xanthogranuloma and no associated systemic paraproteinemia. Orbital biopsy showed strong expression of CD20-positive cells. The patient was treated with systemic rituximab monotherapy, with excellent clinical response and marked regression of the orbital lesions on imaging. At the time of writing, the patient has been treated with bimonthly rituximab maintenance therapy for 22 months and has stable clinical and imaging findings with sustained response to treatment and no reported side effects. To the authors' knowledge, this is the first reported case of orbital necrobiotic xanthogranuloma successfully treated with rituximab monotherapy. They hope that this well-documented case will encourage clinicians to consider rituximab monotherapy as a possible treatment option, albeit one entailing an off-label use of this drug, for patients with necrobiotic xanthogranuloma of the orbit.

Eye-Preserving Surgery Followed by Adjuvant Radiotherapy for Lacrimal Gland Carcinoma: Outcomes in 37 Patients.

PURPOSE: To describe the clinical outcomes of eye-preserving surgery followed by adjuvant radiotherapy in patients with lacrimal gland carcinoma. METHODS: Thirty-seven patients with lacrimal gland carcinoma who underwent eye-preserving surgery were studied. RESULTS: At last follow up, 32 patients were alive without disease, 3 patients were alive with disease with distant metastasis, 1 patient had died of disease, and 1 patient had died of other cause. The 5-year recurrence-free survival rate was worse in patients without than in patients with adjuvant radiotherapy (p = 0.001) and worse in patients with T3-T4 tumors than in patients with T1-T2 tumors (p = 0.027). At last follow up, 25 patients (68%) had visual acuity of 20/40 or better. CONCLUSIONS: In patients with lacrimal gland carcinoma, eye-preserving surgery and adjuvant radiotherapy is associated with reasonable local control rates and visual and ocular function. Postoperative adjuvant radiotherapy seems to enhance local control rates.

Desmoplastic Melanoma of the Periorbital Region.

Desmoplastic melanoma (DM) is a rare subtype of melanoma and an even smaller proportion of periocular melanomas. Here, the authors report 2 cases of DM in the periocular region. Staged according to the American Joint Committee on Cancer (AJCC) eighth edition classification, patient 1 presented with a stage IIIC (pT4apN1cM0) DM in the left lateral canthus with upper and lower eyelid and patient 2 presented with a stage IIIB (T4aN1bM0) DM in the left brow and supraorbital region with a parotid lymph node metastasis. In both patients, the lesions were amelanotic, with inflammatory appearance, and had been noted for several years before the correct diagnosis was made. In both patients, wide excision led to large surgical defects, and perineural invasion prompted adjuvant radiation therapy postoperatively. Patient 2 was treated with an immune checkpoint inhibitor for his parotid metastasis. Ophthalmologists should be aware of DM, its neurotrophic nature, and potential to metastasize with locally advanced lesions.

Clinical Presentation and Anatomical Location of Orbital Plasmacytomas.

PURPOSE: To evaluate the clinical and anatomical location of orbital plasmacytomas and assess local control following therapy. METHODS: The American Society of Ophthalmic Plastic and Reconstructive Surgery Oncology Database was queried to identify patients diagnosed with orbital plasmacytoma. These patients' records were reviewed for demographic characteristics, clinical and radiologic findings, treatments, and outcomes. RESULTS: Thirty patients from 4 institutions (24 from MD Anderson Cancer Center, 3 from SUNY Downstate Medical Center, 2 from University of California, Los Angeles, and 1 from Oregon Health and Science University) were identified. Eighteen patients (60%) were diagnosed with multiple myeloma (MM) before and 11 (37%) were diagnosed with MM immediately after orbital plasmacytoma. Based on imaging, 4 distinct anatomical patterns were identified: 1) bony plasmacytoma affecting the superotemporal orbit, epidural space, and temporal fossa (15 patients; 50%); 2) discrete orbital plasmacytoma (7 patients; 23%); 3) infiltrative plasmacytoma either originating from a sinus (4 patients; 13%); or 4) originating from the orbital floor and infiltrating facial soft tissue (4 patients; 13%). Of the 29 patients with available treatment data, 2 had radiation only, 3 had chemotherapy only, 6 had chemoradiation, and 18 had stem cell transplant following chemoradiation (n = 17) or only chemotherapy (n = 1). Following treatment, 10 patients achieved complete and 11 achieved partial responses. CONCLUSION: Orbital plasmacytomas were found exclusively in patients with MM diagnosed before or immediately after orbital plasmacytoma. Plasmacytomas can have 4 distinct anatomical patterns of origin. Following treatment, all patients had good to excellent local control of their orbital lesions.

Different Clinical Courses on Long-Term Follow-Up of Age-Related Macular Degeneration Patients Treated with Intravitreal Anti-Vascular Endothelial Growth Factor Injections.

PURPOSE: To assess the long-term outcome of neovascular age-related macular degeneration (AMD) treated with multiple intravitreal anti-vascular endothelial growth factor (VEGF) injections. METHODS: All patients treated with over 30 intravitreal anti-VEGF injections for neovascular AMD between 2007 and 2014 were retrospectively reviewed. RESULTS: A total of 67 eyes received 2,960 (mean 45 ± 9.1 per eye) anti-VEGF injections. Eyes with good final visual acuity (VA) had better initial VA (p = 0.020) and maintained it. Patients with moderate-to-poor final VA improved significantly after the first 3 monthly injections, and thereafter deteriorated consistently, mostly during the third (p = 0.019) and fourth (p = 0.006) years. Eyes with worse final VA had more intraretinal fluid (p = 0.05) and subretinal fibrosis (p = 0.04). CONCLUSION: Two distinct clinical courses were identified: good final VA was associated with initial and long-term stability of good VA; eyes with worse final VA had worse initial VA, progressive deterioration following the initial improvement, and more scarring and intraretinal fluid. This probably underscores the long-term benefits of early detection and treatment.

The impact of IgG-4-ROD on the diagnosis of orbital tumors: A retrospective analysis.

This study was to determine the prevalence of immunoglobulin G4 (IgG4)-related orbital disease (IgG4-ROD) among patients who have previously undergone biopsy and were diagnosed to have idiopathic orbital inflammatory disease (IOID) or orbital lymphoproliferative disease (OLD), namely, lymphoma and benign reactive lymphoid hyperplasia (BRLH). This is a retrospective cross-sectional study. The charts and slides of all patients who underwent biopsies and were histopathologically diagnosed to have either IOID or OLD were reviewed. Demographics, clinical features, initial histopathological diagnoses, treatment received, and final outcome were noted. Using the diagnostic criteria for diagnosis for IgG4 disease, those cases that would classify as "possible IgG4-related disease (IgG4-RD)" were reviewed, reclassified, and reassigned a diagnosis of IgG4-ROD. We reviewed 105 patients' clinical charts. Of these 105 patients, upon reviewing the histopathology, 18 (17.15%) patients were found to fit the diagnostic criteria for possible IgG4-ROD. Of these 18 patients who were now reassigned the diagnosis of IgG4-ROD, the most common previous histopathological diagnosis was found to be IOID, for eight patients (44%), then BRLH, which was noted in five patients (27.8%), followed by lymphoma, which was noted in two patients (11.1%). Previously diagnosed cases of IOID and OLD were found to fulfill the criteria for IgG4-ROD. Given the advent of recent diagnostic and histopathological techniques, all cases of suspected IOID and OLD should be screened for IgG4-ROD and all previously diagnosed cases must be closely followed up, given the systemic implication of IgG4-RD. Histopathological reassessment of previously diagnosed cases may be considered.

Comparison of lateral orbital decompression with and without rim repositioning in thyroid eye disease.

PURPOSE: Rim-off lateral wall decompression may be associated with functional and cosmetic deficit. Our objective, therefore, was to describe the functional and cosmetic results of deep lateral orbital decompression with and without anterior rim repositioning for thyroid eye disease. METHODS: In this retrospective comparative case series all consecutive thyroid eye disease patients who underwent deep lateral wall decompression at the Royal Victorian Eye and Ear Hospital between 1990-2007 and the Goldschleger Eye Institute, Sheba Medical Center between 2008-2011 were included. Patients were divided into two groups: the "rim-on" group in which the anterior lateral orbital rim was repositioned and the "rim-off" group in which it was left off. Main outcome measures were: proptosis reduction, postoperative oscillopsia and diplopia, presence of visible or palpable lateral orbit depression. RESULTS: One hundred and twelve patients who underwent 186 orbital decompressions were included in the final analysis. The average proptosis reduction for two- and three-wall decompressions ranged between 4.6-4.9 mm in the rim-on and 4.6-5.7 mm in the rim-off group respectively. The prevalence of postoperative oscillopsia was similar in both groups. The preoperative diplopia worsened in 17 patients (32.1 %) in the rim-on group and in seven patients (12.3 %) in the rim-off group (P = .02, chi-square test). None of the patients developed visible or palpable lateral orbit depression. CONCLUSIONS: Deep lateral orbital decompression without anterior rim repositioning may be an effective approach to enhance functional and cosmetic outcomes in thyroid eye disease patients without increasing the risk of lateral wall depression or postoperative oscillopsia.