Multiple Cranial Neuropathies as the Initial Presentation of Primary Ductal Adenocarcinoma of the Lacrimal Gland.

Primary ductal adenocarcinoma of the lacrimal gland is a rare, aggressive malignancy that clinically and histologically resembles salivary duct carcinoma. Similar to other malignant epithelial lacrimal gland tumors, ductal adenocarcinoma typically presents with unilateral proptosis, pain, upper eyelid swelling, palpable mass, diplopia, ptosis, and blurred or decreased vision. Rarely, primary malignant epithelial lacrimal gland tumors may first present with multiple cranial neuropathies due to occult spread to the cavernous sinus, as in this case. With such a vast differential diagnosis, a practical yet systematic approach to multiple cranial neuropathies, as guided by clinical history, exam, and neuroimaging, allows for a more targeted diagnostic evaluation, especially when multiple diagnostic tests and interventions return unrevealing. A repeat biopsy or complete excision of the lacrimal gland may be necessary to yield the correct diagnosis.

Outcomes of adjunctive therapies post hedgehog inhibitors in the management of locally advanced basal cell carcinoma: A systematic review and pooled analysis.

Management of patients with locally advanced basal cell carcinoma (laBCC) with traditional strategies has yielded suboptimal outcomes. Targeted treatments including hedgehog inhibitor therapy (HHIT) present limitations when utilized as monotherapy. Herein, we report evidence-based outcomes from available literature on multimodality treatments adjuvant to HHIT in laBCC management. Utilizing a systematic search strategy in PubMed, we identified studies published from inception to April 15, 2020, screened for definitive inclusion/exclusion criteria, and performed individual study quality assessment and pooled analysis to assess impact of adjunctive treatment-based responses post-HHIT on clinical response and recurrence outcomes. Twenty-nine studies (n = 103) were included. Primary findings include a complete response (CR) rate of 90.5%, the median follow-up of 12 months post-HHIT completion. The recurrence rate was 10.8% with 12-month median time to recurrence. Mohs micrographic surgery (MMS) had 100% CR post-HHIT, while no difference was observed between surgery and radiation therapy (RT). MMS and surgery had comparable 2-year recurrence free rates (RFR) at 87% and 86% respectively, while RT had the lower 2-year RFR at 67%. Male gender portended a more advanced stage at diagnosis and worse outcomes. In a subset analysis, periorbital laBCCs with orbital involvement had a CR rate of 81.8% versus 100% in those without orbital involvement, with similar rates of recurrence. Limited available quantitative data and possible publication bias were limitations. Pooled analysis of observational data supports use of adjunctive therapies post-HHIT to improve treatment response in patients with laBCC. Longer-term follow-up is needed to study recurrence rates after combination therapy.

Masses of the Lacrimal Gland: Evaluation and Treatment.

Lacrimal gland lesions account for approximately 9 to 10% of all biopsied orbital masses. Potential causes include nongranulomatous and granulomatous inflammation, autoimmune disease, lymphoproliferative disorders, benign epithelial proliferation, malignant neoplasia, and metastatic disease. Inflammatory lesions and lymphoproliferative disorders are the most common and may be unilateral or bilateral; they may also be localized to the orbit or associated with systemic disease. Both benign and malignant epithelial lacrimal gland masses tend to be unilateral and involve the orbital lobe, but a more rapid onset of symptoms and periorbital pain strongly suggest malignant disease. On orbital imaging, both inflammatory and lymphoproliferative lesions conform to the globe and surrounding structures, without changes in adjacent bone, whereas epithelial lacrimal gland masses often show scalloping of the lacrimal gland fossa. Malignant epithelial lacrimal gland tumors can also have radiographic evidence of bony invasion and destruction. Masses of the lacrimal gland may be due to a broad range of pathologies, and a good working knowledge of common clinical characteristics and radiographic imaging findings is essential for diagnosis and treatment. All patients with inflammatory, lymphoproliferative, and epithelial neoplastic lesions involving the lacrimal gland require long-term surveillance for disease recurrence and progression.

Prostaglandin-Associated Periorbitopathy in Children and Young Adults with Glaucoma.

OBJECTIVE: To evaluate for relative palpebral and orbital changes after long-term unilateral exposure to prostaglandin analogues (PGAs) in patients with childhood glaucoma. DESIGN: Prospective cross-sectional cohort study. PARTICIPANTS: A total of 29 patients with history of childhood glaucoma, who were treated unilaterally with PGAs for at least 12 months. METHODS: Based on 4 standardized clinical photographs (en face with eyes open, right and left side views with eyes open, and en face with eyes closed), 3 masked expert graders each independently selected the eye they perceived to have received unilateral PGA treatment by physical appearance alone and graded the following features relative to the other eye: (1) ocular (e.g., conjunctival hyperemia, iris heterochromia, and buphthalmos), (2) palpebral (e.g., eyelash trichomegaly, eyelash hypertrichosis, eyelid erythema, eyelid edema, eyelid hyperpigmentation, high upper eyelid crease, upper eyelid ptosis, upper and/or lower eyelid retraction, and eyelid skin atrophy with presence of telangiectasias), and (3) periorbital (e.g., superior sulcus hollowing, proptosis, enophthalmos, hypoglobus, and hyperglobus). An interrater reliability analysis was performed using the Fleiss kappa (κ) statistic to determine consistency among raters. MAIN OUTCOME MEASURES: Frequencies of each feature of prostaglandin-associated periorbitopathy (PAP); group consensus; interrater reliability of selected PGA-treatment laterality. RESULTS: Median unilateral PGA exposure time was 31.7 months (interquartile range: 18.8-44.3 months). Eyelash trichomegaly and hypertrichosis (n = 22, 76%), high upper eyelid crease (n = 20, 69%), upper eyelid ptosis (n = 14, 52%), and superior sulcus hollowing (n = 15, 52%) were the most frequently observed features of PAP in PGA-treated eyes compared with untreated fellow eyes. Most of these changes were mild, but 20% to 30% of patients exhibited moderate eyelash and/or eyelid changes. One patient had severe PAP after long-term unilateral PGA exposure. Group consensus with correctly selected laterality was achieved in all patients. The inter-rater reliability was excellent (κ = 0.815, P < 0.001, 95% confidence interval [0.605, 1.000]). CONCLUSIONS: Mild-to-moderate changes in the ocular adnexa can develop in children and young adults with long-term PGA exposure. Patients and their families should be educated on the possibility of PAP, especially when initiating monocular PGA therapy.

CO2 Laser Lash Tilt Technique for the Treatment of Mild to Moderate Lash Ptosis and Augmentation of Upper Eyelid Blepharoplasty Results.

PURPOSE: To assess the safety, efficacy, and patient satisfaction of a newly described technique for the treatment of mild to moderate lash ptosis performed as augmentation to upper eyelid blepharoplasty. METHODS: Patient medical records for 27 consecutive cases of upper eyelid blepharoplasty between January 2016 and June 2017 and 19 consecutive cases of upper eyelid blepharoplasty with the laser lash tilt procedure between July 2016 and January 2017 performed by the senior author were retrospectively reviewed. Lash position in preoperative and postoperative photographs was graded in a randomized masked fashion on a 4-point scale by 5 oculoplastic surgeons. RESULTS: There was a significant improvement in mean lash position scores for the control group (0.52 ± 0.34, p < 0.001) with mean lash score improving from 1.14 ± 0.46 before surgery to 0.62 ± 0.46 after blepharoplasty alone. There was also significant improvement in mean lash position scores for the laser lash group (0.98 ± 0.52, p < 0.01) with mean lash score improving from 1.66 ± 0.62 before surgery to 0.68 ± 0.50 after blepharoplasty with laser lash treatment. The improvement in lash position seen in the laser lash group was significantly greater than the improvement seen in the control group (p = 0.001). There were no complications and all patients were satisfied with their results. CONCLUSIONS: Compared with upper eyelid blepharoplasty alone, the addition of the CO2 laser lash tilt technique provides a significant improvement in the upward tilt of the eyelashes as they emerge from the eyelid margin.

Clinical Implications of Direct Immunofluorescence Findings in Patients With Ocular Mucous Membrane Pemphigoid.

PURPOSE: To examine the clinical implications of positive or negative direct immunofluorescence biopsies (DIF) in patients with clinically typical ocular mucous membrane pemphigoid (MMP). DESIGN: Retrospective cohort study. METHODS: The study population was patients with clinically typical ocular MMP disease with documented DIF results who were followed for at least 1 year at the Duke University multidisciplinary ocular MMP clinic. Data were collected by chart review and included patient demographics, clinical examination findings, and history of autoimmune disease and/or malignancy, as well as topical, systemic, and surgical treatments received. Main outcome measures included MMP Disease Area Index, Foster stages, proportion legally blind, duration of follow-up, and use of systemic immunosuppression and ocular procedures in treatment. RESULTS: In multivariable analysis restricted to 55 patients, patients with negative and positive biopsies were similar in the outcome measures; however, positive-biopsy patients were more likely to be treated with systemic immunosuppression and were followed for longer at our clinic. Patients with isolated ocular disease were also more likely to have negative biopsies compared to those who also had extraocular disease. Patients who had conjunctival biopsies were more likely to have a negative direct immunofluorescence result than patients with biopsies from other sites. CONCLUSIONS: We encourage clinicians and patients to consider treatment with systemic immunosuppression even in the absence of diagnosis confirmation by DIF. Furthermore, this study supports current standard of care to pursue a nonocular biopsy of normal-appearing, perilesional skin or oral mucosa when possible.

Completeness of facial self-application of sunscreen in cosmetic surgery patients.

Previous studies have demonstrated incomplete sunscreen self-application to various facial regions in cosmetic surgery patients, but there is no scientific research on the completeness of facial and periocular sunscreen self-application using ultraviolet (UV) photography for assessment. This prospective, cross-sectional, qualitative study aimed to assess completeness of facial application of sunscreen in oculofacial surgery patients at the Duke Eye Center (Durham, North Carolina). Forty-four participants were enrolled, and completeness of facial sunscreen self-application was assessed using UV photography.

Distinct, bilateral epithelioid hemangioma of the orbit.

UNLABELLED: ABSTRACT Introduction: Epithelioid hemangioma (EH) is a benign abnormal proliferation of endothelial cells usually arising in the head and neck region and presenting clinically as raised cutaneous lesions. EH rarely involves the orbit and has not been reported to occur bilaterally. CASE: A healthy 38 year old female developed sequential proptosis of each orbit over the course of several months. The patient underwent excisional biopsy confirming the diagnosis of EH in each orbit. She was treated with a course of high dose steroids and has been stable at 13 months following the most recent excision. CONCLUSION: This is the first reported case of bilateral EH.

A case of bilateral silent sinus syndrome presenting with chronic ocular surface disease.

The silent sinus syndrome is characterized by atelectasis of the maxillary sinus in the setting of subclinical maxillary sinusitis and obstruction of the osteomeatal unit. The resultant expansion of orbital volume causes enophthalmos and hypoglobus. A review of the literature reveals only unilateral cases. The authors present a case of bilateral silent sinus syndrome presenting with chronic ocular surface disease. A 56-year-old man was referred for tarsorrhaphy to treat a chronic recurrent corneal ulcer. Exam revealed an area of corneal thinning in the left eye. The patient appeared cachectic and there was enophthalmos, hypoglobus, and lagophthalmos on both sides, left worse than right. Due to the patient's appearance and a history of sinonasal malignancy, recurrent malignancy was suspected. A systemic workup was unrevealing, and radiographic studies showed the features of silent sinus syndrome bilaterally. The patient underwent sinus surgery and placement of a left orbital floor implant.

Orbital surgery: state of the art.

Much has been written about the repair of orbital fractures, yet some debate still exists among surgeons with regard to indications for and timing of fracture repair and various surgical techniques. Controversies regarding the surgical maneuvers include the incision, surgical approach, and methods of wound closure. More detailed imaging modalities have allowed clinicians to understand the injuries more completely and plan for and execute more ideal reconstructions. Recent advances in orbital implant materials and the role of endoscopy in orbital fracture repair add to this debate about which techniques would be best for particular injuries. This article discusses these issues and provides the most current literature review regarding the management of various orbital fractures.