Checkpoint inhibitor-related myasthenia-myocarditis-myositis overlap syndrome in the orbit.

We report a series of three patients who developed checkpoint inhibitor-related myocarditis with orbital myositis and/or myasthenia gravis overlap syndrome, with varying degrees of severity. In all cases, checkpoint inhibitor therapy was immediately discontinued upon diagnosis and corticosteroids were initiated. While two patients achieved substantial recovery, one patient passed away on hospital day three. These cases underscore the critical need for prompt recognition of adverse events associated with immune checkpoint inhibitors.

Episodic Upper Eyelid Edema in an African American Patient.

An African American patient in their 60s with a history of monoclonal gammopathy of unknown significance presents to the oculoplastic service for intermittent, bilateral upper eyelid swelling and pain for 2 years. Examination reveals hyperpigmented, spongy eyelid edema with redundant skin, and no lymphadenopathy is present. What would you do next?

Using optical coherence tomography to optimize Mohs micrographic surgery.

Mohs micrographic surgery (MMS) is considered the gold standard for treating high-risk cutaneous basal cell carcinoma (BCC), but is expensive, time-consuming, and can be unpredictable as to how many stages will be required or how large the final lesion and corresponding surgical defect will be. This study is meant to investigate whether optical coherence tomography (OCT), a highly researched modality in dermatology, can be used preoperatively to map out the borders of BCC, resulting in fewer stages of MMS or a smaller final defect. In this prospective study, 22 patients with BCC undergoing surgical excision were enrolled at a single institution. All patients had previously received a diagnostic biopsy providing confirmation of BCC and had been referred to our center for excision with MMS. Immediately prior to performing MMS, OCT was used to map the borders of the lesion. MMS then proceeded according to standard protocol. OCT images were compared to histopathology for agreement. Histopathologic analysis of 7 of 22 MMS specimens (32%) revealed a total absence of BCC, indicating resolution of BCC after previous diagnostic biopsy. This outcome was correctly predicted by OCT imaging in 6 of 7 cases (86%). Nine tumors (9/22, 41%) had true BCC and required a single MMS stage, which was successfully predicted by pre-operative OCT analysis in 7 of 9 cases (78%). The final six tumors (27%) had true BCC and required two MMS stages for complete excision; preoperative OCT successfully predicted the need for a second stage in five cases (5/6, 83.3%). Overall, OCT diagnosed BCC with 95.5% accuracy (Cohen's kappa, κ = 0.89 (p-value = < 0.01) in the center of the lesion. Following a diagnostic biopsy, OCT can be used to verify the existence or absence of residual basal cell carcinoma. When residual tumor is present that requires excision with MMS, OCT can be used to predict tumor borders, optimize surgery and minimize the need for additional surgical stages.

Transcutaneous retrobulbar amphotericin B for rhino-orbital-cerebral mucormycosis: a multi-center retrospective comparative study.

PURPOSE: To assess whether transcutaneous retrobulbar amphotericin B injections (TRAMB) reduce exenteration rate without increasing mortality in rhino-orbital-cerebral mucormycosis (ROCM). METHODS: In this retrospective case-control study, 46 patients (51 eyes) with biopsy-proven ROCM were evaluated at 9 tertiary care institutions from 1998 to 2021. Patients were stratified by radiographic evidence of local orbital versus extensive involvement at presentation. Extensive involvement was defined by MRI or CT evidence of abnormal or loss of contrast enhancement of the orbital apex with or without cavernous sinus, bilateral orbital, or intracranial extension. Cases (+TRAMB) received TRAMB as adjunctive therapy while controls (-TRAMB) did not. Patient survival, globe survival, and vision/motility loss were compared between +TRAMB and -TRAMB groups. A generalized linear mixed effects model including demographic and clinical covariates was used to evaluate the impact of TRAMB on orbital exenteration and disease-specific mortality. RESULTS: Among eyes with local orbital involvement, exenteration was significantly lower in the +TRAMB group (1/8) versus -TRAMB (8/14) (p = 0.04). No significant difference in mortality was observed between the ±TRAMB groups. Among eyes with extensive involvement, there was no significant difference in exenteration or mortality rates between the ±TRAMB groups. Across all eyes, the number of TRAMB injections correlated with a statistically significant decreased rate of exenteration (p = 0.048); there was no correlation with mortality. CONCLUSIONS: Patients with ROCM with local orbital involvement treated with adjunctive TRAMB demonstrated a lower exenteration rate and no increased risk of mortality. For extensive involvement, adjunctive TRAMB does not improve or worsen these outcomes.

Predictability of the Phenylephrine Test in Congenital Ptosis: Ten Years of Experience.

PURPOSE: To assess the predictability of phenylephrine testing for congenital ptosis and review outcomes of Müller's Muscle-conjunctival resection (MMCR) for congenital ptosis across ten years of follow-up. METHODS: In this retrospective case series, all patients who underwent MMCR for congenital ptosis at a single institution between 2010 and 2020 were identified. Exclusion criteria included patients who had not undergone preoperative testing with 2.5% phenylephrine in the superior fornix; patients who underwent revision surgery; and patients who had a broken suture in the early postoperative period. Demographics, margin-reflex distance 1 (MRD1) values pre- and postphenylephrine, millimeters of tissue resected intraoperatively, and final postoperative MRD1 were recorded. RESULTS: A total of 28 patients were included; 19 patients received MMCR and 9 patients received a combined MMCR plus tarsectomy. The amount of tissue resected ranged from 5 to 11 mm. There was no significant difference between median postphenylephrine MRD1 and median final postoperative MRD1 in either surgical group. Neither patient age nor levator function was significantly associated with a change in MRD1 in either group. The addition of a tarsectomy had no bearing on the final MRD1 value. CONCLUSIONS: MMCR is a viable option for patients with congenital ptosis and moderate levator function with a response to phenylephrine. In these patients, MRD1 after 2.5% phenylephrine testing correlates to the final postoperative MRD1 outcome within 0.5 mm.

A review of surgical management of progressive myogenic ptosis.

PURPOSE: Surgical correction of myogenic ptosis is a sophisticated endeavor, as the disease is progressive and the post-operative course is prone to significant complications. We sought to review the literature for repair techniques in different types of myogenic ptosis. METHODS: A PubMed/MEDLINE literature search of publications pertaining to surgical outcomes of progressive myogenic ptosis repair was performed. Studies included were original retrospective studies with a minimum of four patients. RESULTS: A total of 27 articles were identified and divided by etiology of myogenic ptosis; either chronic progressive external ophthalmoplegia (CPEO), oculopharyngeal muscular dystrophy (OPMD), myasthenia gravis (MG), or mixed. Surgical techniques predominantly involved levator advancement, levator resection, frontalis sling, blepharoplasty, and Fasanella-Servat. Success rates ranged from 60.5% to 100%. Significant postoperative complications included ptosis recurrence, under-correction, over-correction, keratopathy, lagophthalmos, sling exposure, and sling infection. CONCLUSION: Like surgical repair for other forms of ptosis, correction of progressive myogenic ptosis is guided by levator excursion. However, myogenic ptosis is especially challenging as it is characterized by worsening ptosis and the loss of protective corneal mechanisms. The goals of care with myogenic ptosis involves repairing ptosis just sufficiently to alleviate visual obstruction while avoiding adverse post-operative complications. This intentional under-correction subsequently increases susceptibility for ptosis recurrence. Myogenic ptosis repair therefore requires delicate balancing between function, sustained repair, and corneal protection.

Changes in Rat Scleral Collagen Structure Induced by UVA-Riboflavin Crosslinking at Various Tissue Depths in Whole Globe Versus Scleral Patch.

Purpose: To investigate structural changes in scleral collagen fibers at various tissue depths before and after photosensitized crosslinking (CXL) both isolated scleral patch versus whole globe using second-harmonic generation (SHG) imaging. Methods: Scleral tissues were harvested from Sprague-Dawley rats and separated into three groups: untreated sclera (control), full-thickness scleral patch for CXL (Free Scleral CXL group), and sclera in intact globe for CXL (Globe CXL group). The CXL groups were soaked in 0.1% riboflavin and irradiated with 365 nm ultraviolet-A light (power, 0.45 mW/cm2) for 30 minutes. SHG images were acquired every 5 µm between 10 and 60 µm from the outer scleral surface. Collagen fiber waviness was calculated as the ratio of the total length of a traced fiber and the length of a straight path between the fiber ends. Results: In the Free Scleral CXL group, collagen waviness was significantly increased compared to the control group at 35 to 50 µm (P < 0.05). In the Globe CXL group, collagen waviness was decreased compared to control at all depths with statistical significance (P < 0.05) achieved from 10 to 45 µm. Conclusions: Depending upon its initial state (i.e., free scleral patch versus mechanically loaded intact globe under pressure), collagen may experience different structural changes after CXL. In addition, the extent of the CXL effects may vary at different depths away from the surface. Translational Relevance: Understanding the CXL effects on collagen structure may be important in optimizing the scleral crosslinking protocol for future clinical applications such as preventing myopic progression.

Ocular surface disease in thyroid eye disease: A narrative review.

Ocular surface disease (OSD) in the setting of thyroid eye disease (TED) is traditionally thought of as a natural consequence of anatomical changes such as proptosis and corneal exposure. However, a growing body of research suggests that ocular surface inflammation and multi-factorial changes to the homeostasis of the ocular surface contribute substantially to the OSD seen in TED patients. In this paper we review the existing literature which highlights the work and existing theories underlying this new paradigm shift.

Collagen Structural Changes in Rat Tarsus After Crosslinking.

Purpose: Surgery is the standard treatment for floppy eyelid syndrome, but crosslinking (CXL) tarsus has recently been proposed as an alternative. To the best of our knowledge, this study is the first to use second-harmonic generation (SHG) microscopy to examine tarsal collagen ex vivo before and after photo-activated crosslinking. To quantify crosslinking, this study examined fluorescence recovery after photobleaching (FRAP), which indirectly measures tissue stiffness. Methods: Upper eyelid tarsal plates were dissected from 21 Sprague-Dawley rats (total of 42 tarsal plates). Six normal plates were sent for histopathology and SHG imaging; the remaining 36 were crosslinked with phosphate-buffered saline (PBS) alone or riboflavin in PBS (concentrations of 0.1%, 0.3%, and 0.5%). Tissues were irradiated with 365-nm ultraviolet A light (power, 0.45 mW/cm2) for 30 minutes and immediately underwent SHG microscopy. Stiffness was indirectly measured with FRAP using fluorescein isothiocyanate (FITC)-dextran. Results: SHG imaging of normal tarsus showed that the organization of collagen bundles is complex and varies greatly depending on location. After crosslinking with high-concentration riboflavin (0.5%), collagen fibers showed clear structural changes, becoming more densely packed and wavier compared to control. FRAP half-time to fluorescence recovery was significantly increased (P < 0.05), indirectly indicating increased tissue stiffness. No structural changes were observed after crosslinking with lower riboflavin concentrations of 0.1% and 0.3%. Conclusions: This is the first report of SHG microscopy used to image tarsus collagen before and after crosslinking. These results highlight collagen structural changes, with effects on tissue stiffness indirectly confirmed by FRAP. Translational Relevance: Collagen fibers in the tarsus may be a therapeutic target for crosslinking in order to treat symptomatic floppy eyelid syndrome.

Building Your Brand: Analysis of Successful Oculoplastic Surgeons on Social Media.

PURPOSE: To determine the impact of social media content from oculoplastic surgeon-held accounts on public engagement. METHODS: This study analyzed successful Instagram accounts (defined by >500 followers as of May 2019) held by American Society of Ophthalmic Plastic Reconstruction Surgery members. Each account's 20 most and 20 least successful posts (based on number of likes) were characterized by the post's subject, location, media type, and tag usage. Posts were then analyzed using a web-based application that calculates the engagement level of Instagram users with the selected post. RESULTS: A total of 4,812 posts were analyzed from 37 Instagram accounts. Of these American Society of Ophthalmic Plastic Reconstruction Surgery members, the majority were men (67.6%, n = 25) in private practice solely (70.3%, n = 26). Numerous post characteristics, like content and media type, impacted success in a statistically significant manner; for example, public engagement rose when featuring the posting doctor, especially when smiling and wearing a white coat. Meanwhile, posts featuring an office procedure had a negative effect. Formatting was also found to contribute to success. For example, photographs were preferred over videos, while multiple images were best presented sequentially as a carousel (with the viewer swiping through the series) rather than in a large collage. Use of tags (hashtags, accounts, and locations) were associated with increased engagement. CONCLUSIONS: Social media is an important communication and marketing tool, especially in esthetic fields like oculoplastics. Guidelines for success are presented in this study; content, location, subject, media type, and tags statistically significantly impact public engagement.The influence of social media is rapidly growing and can be strategically harnessed by oculoplastic surgeons to educate both patients and healthcare providers, collaborate with colleagues, and for referrals and marketing.Supplemental Digital Content is available in the text.

The influence of social media is rapidly growing and can be strategically harnessed by oculoplastic surgeons to educate both patients and healthcare providers, collaborate with colleagues, and for referrals and marketing. Supplemental Digital Content is available in the text.

Rare Optic Neuropathy in a Jamaican Couple Linked to Cassava Root Consumption.

We report central visual loss with selective papillomacular bundle damage in a Jamaican couple, husband and wife, with long-term cassava root consumption. The two cases presented independently with gradual central visual loss. Examination revealed bilateral temporal pallor of the optic nerve head and automated static perimetry demonstrated a central or caecocentral scotoma in each patient. Optical coherence tomography findings are described. The only mutual risk factor, reported independently, was long-term cassava consumption. Cessation of cassava intake and vitamin supplementation resulted in partial recovery of visual function. As thiocyanate levels in urine were not measured, the aetiology in these patients is not definitively confirmed, but chronic cassava consumption should be considered in similar cases as a rare cause of potentially reversible optic neuropathy.

Sinus pericranii in the upper eyelid: diagnosis and management guidelines.

Sinus pericranii (SP) is a rare malformation involving aberrant connections between the dural venous sinuses and extracranial veins. Classically considered congenital pathology, there are reported instances of post-traumatic SP in adults. Very rarely, the upper eyelid is involved and the resulting ptosis can obstruct the visual axis. In these cases, surgical removal may be warranted. Because of the high risk of massive intraoperative hemorrhage, careful preoperative identification and diagnostic workup is essential. The authors report a patient with new onset, asymmetric eyelid ptosis referred for evaluation, and subsequently diagnosed with SP. Through a multi-disciplinary assessment, deferring surgical intervention was determined to be appropriate management. The lesion was monitored clinically, and at 6 months follow-up, there were no noted adverse outcomes.