Bilobed flap reconstruction after en-bloc removal of solitary fibrous tumor of the lacrimal sac.

Purpose: To report a rare case of a solitary fibrous tumor (SFT) of the lacrimal sac and discuss considerations for management of similar cases. Observations: We present the case of a 41-year-old woman who presented with a primary lacrimal sac SFT for which she underwent en-bloc surgical resection. We discuss management options for SFTs and our surgical approach for this case: bilobed flap reconstruction of the medial canthus and inferior orbit. Conclusions: We present an uncommon presentation of a rare tumor and a successful one-stage reconstruction with a bilobed flap.

Implants and spacers for paralytic ectropion: Literature review and assessment of a thin-profile porous polyethylene implant.

Purpose: There is no ideal treatment paradigm for paralytic ectropion. This study evaluated lower eyelid spacers and the efficacy of a novel lower eyelid thin profile, bio-integratable, porous polyethylene. Methods: A retrospective review of 15 consecutive patients who underwent thin-profile porous polyethylene implantation and canthoplasty for paralytic ectropion was carried out. A comprehensive literature review of spacers for paralytic ectropion and retraction using the Pubmed database with search terms "[implant or graft or spacer] and [paralytic ectropion or paralytic retraction]," "graft and paralysis and ectropion," "implant and paralysis and ectropion," "graft and paralysis and retraction," and "implant and paralysis and retraction" was carried out. Results: The mean patient age was 69 years (range: 50-88). Lagophthalmos improved from a mean of 5.7 mm (SD = 3.3, range 3-14 mm) to 1.4 mm (SD = 1.1, range 0-3.5 mm), P < 0.0001. MRD 2 improved from a mean of 6.7 mm (SD = 2.3, range 2-12 mm) to 4.2 mm (SD = 0.9, range 3-6 mm), P = 0.0005. No patients needed additional lower eyelid surgery. There were no implant exposures at a mean follow-up of 7.6 months (SD = 7.9, range 0.7-21.6 months). Detailed literature review revealed that hard palate and ear cartilage are the most reported spacers, each with unique disadvantages. Conclusion: The thin-profile porous polyethylene implant is a useful addition to the management of symptomatic paralytic ectropion. Meaningful comparison of lower eyelid spacers is difficult because of variations in surgical technique, spacer size, and poorly reported outcome data. No spacer proves superior.

Perioperative Considerations for Antithrombotic Therapy in Oculofacial Surgery: A Review of Current Evidence and Practice Guidelines.

PURPOSE: Recent survey studies have demonstrated wide variability in practice patterns regarding the management of antithrombotic medications in oculofacial plastic surgery. Current evidence and consensus guidelines are reviewed to guide perioperative management of antithrombotic medications. METHODS: Comprehensive literature review of PubMed database on perioperative use of antithrombotic medication. RESULTS/CONCLUSIONS: Perioperative antithrombotic management is largely guided by retrospective studies, consensus recommendations, and trials in other surgical fields due to the limited number of studies in oculoplastic surgery. This review summarizes evidence-based recommendations from related medical specialties and provides context for surgeons to tailor antithrombotic medication management based on patient's individual risk. The decision to continue or cease antithrombotic medications prior to surgery requires a careful understanding of risk: risk of intraoperative or postoperative bleeding versus risk of a perioperative thromboembolic event. Cessation and resumption of antithrombotic medications after surgery should always be individualized based on the patient's thrombotic risk, surgical and postoperative risk of bleeding, and the particular drugs involved, in conjunction with the prescribing doctors. In general, we recommend that high thromboembolic risk patients undergoing high bleeding risk procedures (orbital or lacrimal surgery) may stop antiplatelet agents, direct oral anticoagulants, and warfarin including bridging warfarin with low-molecular weight heparin. Low-risk patients, regardless of type of procedure performed, may stop all agents. Decision on perioperative management of antithrombotic medications should be made in conjunction with patient's internist, cardiologist, hematologist, or other involved physicians which may limit the role of guidelines depending on patient risk and should be used on a case-by-case basis. Further studies are needed to provide oculofacial-specific evidence-based guidelines.

Aspiration as a Novel Technique to Address Facial and Periocular Myiasis.

Suction aspiration, which has not yet been described in the treatment for myiasis in the periorbital and facial regions, was used to achieve rapid resolution of maggot burden in a 78-year-old patient who presented with a large ulcerated squamous cell carcinoma of the face. This technique also facilitates submission of parasite samples for further analysis. Suction aspiration had no complications, such as significant residual ruptured maggots in the wound or eye injury. Suction aspiration is a safe and efficient technique to reduce maggot burden that has advantages over classic myiasis treatments, especially near the eyes and airway.

Graded transantral orbital decompression outcomes in stable thyroid eye disease: a series of 47 orbits.

Purpose: To provide outcome data for transantral orbital decompression for functional and aesthetic rehabilitation for exophthalmos in stable thyroid eye disease (TED).Methods: Retrospective, non-comparative chart review of consecutive TED patients who underwent primary-graded transantral orbital decompression for exophthalmos by a single surgeon (MAB) between 2005 and 2018. Additional inclusion criteria included follow-up >90 days and no prior orbital surgery. Outcome data collection included millimeters of proptosis reduction, postoperative complications, and patient satisfaction.Results: A total 30 patients (47 orbits) were included. Average reduction in proptosis: 2.8 mm (range 0-9 mm, median 2.5 mm). Post-operatively, new onset diplopia occurred in four patients, V2 numbness in 15 patients (17 orbits), and sinusitis in 2 patients (2 orbits). Phone surveys reached 9/30 patients (14 orbits): cosmetic improvement was reported in 14/14 orbits, relief of orbital ache reported in 10/10 orbits with pre-operative ache, relief of dry eye in 8/13 orbits with preoperative dry eye, and overall satisfaction score: 8.9/10 (range 4-10, median 10).Conclusions: Graded transantral orbital decompression is a viable option for stable TED patients with orbital ache, exophthalmos, and exposure keratopathy, resulting in a high degree of functional and cosmetic satisfaction in our cohort.

Screening Criteria for Detecting Severe Ocular Injuries in the Setting of Orbital Fractures.

PURPOSE: Define incidence of severe ocular trauma in orbital fracture patients and determine if ocular signs and symptoms are useful predictors of severe ocular injuries. METHODS: Retrospective chart review was performed on all patients with orbital fractures between April 1, 2013, and December 31, 2014. Patients were included if they had radiographic evidence of acute fracture of at least one orbital wall and were evaluated by the Ophthalmology service. Demographics, concurrent injury data, and symptoms and signs of ocular trauma were collected. Concurrent ocular injuries were grouped by severity. Predictive signs or symptoms for severe ocular trauma were identified by stepwise logistic regression analysis. The threshold point for predictive signs and symptoms was detected by a receiver operating characteristic (ROC). RESULTS: Five-hundred-twelve patients were included. The most common mechanisms of injury were assault (39%), fall (25%), and motor vehicle accident (21%). The incidence of any concurrent ocular trauma was 75% (383/512), with 14% (70/512) being severe. Four signs and symptoms were predictors of severity: blurred vision (P < 0.0001), pain with eye movements (P < 0.0001), visual acuity worse than 20/40 in the ipsilateral eye (P < 0.001), and restricted motility (P < 0.001). The presence of 2 or more of these signs or symptoms was predictive of severe ocular trauma with high sensitivity (91%) and specificity (86%). CONCLUSIONS: In cooperative patients with acute orbital wall fractures, the presence of 2 or more signs or symptoms is predictive of severe ocular trauma and necessitates the need for urgent ophthalmic consultation.Severe ocular injury associated with orbital wall fracture is more likely in patients with 2 or more ophthalmic signs or symptoms.

Evisceration in patients with Sturge-Weber syndrome.

The management of blind, painful eyes in Sturge-Weber syndrome patients poses unique challenges to the oculoplastic surgeon. Intraocular and orbital vascular malformations and calcification may theoretically lead to unexpected hemorrhage and difficulty placing an implant in a calcified scleral shell. We present two cases of patients with Sturge-Weber syndrome with blind, painful eyes who underwent evisceration with silicone implant and discuss the relevant current literature. Both of our patients had uncomplicated surgeries and post-operative courses. Our literature review reveals that both evisceration and enucleation are viable surgical options for globe removal in Sturge-Weber syndrome, yet careful preoperative planning must be undertaken to minimize risk.

Evaluation and Management of Chemotherapy-Induced Epiphora, Punctal and Canalicular Stenosis, and Nasolacrimal Duct Obstruction.

PURPOSE: To describe the frequency, mechanisms, and treatment of epiphora caused by chemotherapeutic agents. METHODS: Review of relevant articles published in PubMed. RESULTS: The chemotherapeutic drugs best documented to cause epiphora are 5-fluorouracil and docetaxel; with both of these drugs, the main mechanism underlying epiphora is canalicular stenosis. Drugs less commonly reported to cause epiphora include S-1, capecitabine, imatinib, topical mitomycin C, and radioactive iodine for treatment of papillary thyroid carcinoma. While all the above-mentioned drugs can be associated with epiphora, some drugs and administration schedules cause only punctal and canalicular inflammation, whereas others cause significant canalicular stenosis. For example, weekly administration of docetaxel is far more likely to cause canalicular stenosis than every-3-weeks administration. The literature suggests that, in patients who receive weekly docetaxel, silicone stenting at the first sign of recurrent or progressive canalicular stenosis can prevent severe irreversible canalicular stenosis and avoid the need for a conjunctivodacryocystorhinostomy. S-1 and radioactive iodine have been reported to cause nasolacrimal duct obstruction. Early recognition of punctal and canalicular stenosis or nasolacrimal duct blockage and early intervention with topical steroids and canalicular stenting in patients at risk for permanent canalicular scarring are important to avoid the need for more invasive and complicated procedures. CONCLUSION: A variety of chemotherapeutic agents have been reported to cause epiphora, and some of these drugs have also been documented to cause obstructions of the lacrimal drainage system. Early recognition and management of epiphora is important and leads to better outcomes.

Sentinel lymph node biopsy for ocular adnexal melanoma.

BACKGROUND: We sought to update our prior report of findings on sentinel lymph node biopsy (SLNB) and predictors of a positive SLN in patients with conjunctival or eyelid melanoma. METHODS: We reviewed the records of all patients with ocular adnexal melanoma who underwent SLNB at one institution during 2000-2015. We determined rates of positive and false-negative findings on SLNB, primary tumour features correlated with positive findings and rate of nodal recurrence (false-negative event) after negative findings. RESULTS: The study included 51 patients, 31 with conjunctival and 20 with eyelid melanoma. These patients include 30 patients who underwent SLNB during 2000-2008, described in our previous report, and 21 additional patients who underwent SLNB during 2008-2015. There were 30 women and 21 men with median age at SLNB of 62 years (range, 24-83). The nodal basins most commonly sampled were intraparotid (27 patients) and level II (14 patients). Ten patients had positive SLNB findings. Compared to tumours with negative findings, tumours with positive findings had greater median thickness (3.5 mm versus 2.2 mm, p = 0.04), greater median number of mitotic figures (6 versus 2, p = 0.03) and greater incidence of ulceration (80% versus 26%, p = 0.003). Perineural and vascular invasion were not significantly associated with positive findings. There were three false-negative events. Three patients (6%) had temporary marginal mandibular weakness which resolved spontaneously. CONCLUSION: SLNB in patients with ocular adnexal melanoma is safe and identifies nodal micrometastasis in approximately 20% of cases. Histologic features associated with a positive SLN included greater tumour thickness, greater number of mitotic figures and ulceration.

Prevalence and Severity of Ocular Surface Neoplasia in African Nations and Need for Early Interventions.

Ocular surface squamous neoplasia (OSSN) is a common ocular surface tumor with an increased incidence in African countries (3.4 and 3.0 cases/year/100,000). Despite its potential for vision loss and death, OSSN remains largely neglected by both eye and HIV care programs in Africa. The purpose of this review is to identify the barriers to timely diagnosis and early interventions for OSSN in Africa. PubMed searches were conducted targeting previous use of topical chemotherapy (interferon alpha 2b, Mitomycin-C, 5-Fluorouracil) and Human papillomavirus (HPV) vaccination in Africa. We found that OSSN is a significant vision and life-threatening health problem in Africa leading to significant loss of vision, as well as facial disfigurement and social stigma. We did not find any reports on the use of topical interferon, Mitomycin-C or HPV vaccination for OSSN in Africa. One report on the use of topical 5-FU for OSSN in Africa was found. Common barriers to early detection and management of OSSN in Africa include lack of sufficient laboratory infrastructure, lack of trained healthcare personnel, lack of compliance with follow-up visits, cost of topical chemotherapies, and cultural preferences for traditional medicines. In conclusion, OSSN is a significant vision and life-threatening health problem in Africa. There is not much literature on prevention or treatment options for early stages of OSSN in Africa. The use of topical chemotherapy as early interventions and judicious use of smart phone Apps to help with remote diagnosis of early OSSN should be further explored.

Erdheim-Chester disease with orbital involvement: Case report and ophthalmic literature review.

Erdheim-Chester disease (ECD) is a rare xanthogranulomatous disease in which orbital involvement can have devastating outcomes. Through a case report and review of the ophthalmic literature, we explore orbital findings, disease progression, and treatment options. Cases of orbital involvement in Erdheim-Chester disease were identified in the ophthalmic literature with a PubMed query and review of cited references. A total of 14 publications reporting 19 separate cases that included ophthalmic examination data were identified. Patient ages ranged from 26-77 years with a mean age of 50 years. Seventy-four percent (14/19) were men. Vision progression to no light perception was found in 32% (6/19) of the patients. Reviewed cases reported a variety of medical and surgical treatment approaches, however, only 53% reported cases (10/19) demonstrated disease improvement or stabilization. Erdheim-Chester disease with orbital involvement is a devastating disease with a poor prognosis. Awareness of this entity by the ophthalmologist is important as orbital signs and symptoms may manifest early, and orbital biopsy is often crucial to the definitive diagnosis.

Epithelial, non-melanocytic and melanocytic proliferations of the ocular surface.

Ocular surface tumors are commonly encountered by ophthalmologists and ophthalmic pathologists. These tumors have varied clinical manifestations. In this article, we discuss the most commonly encountered non-melanocytic and melanocytic ocular surface tumors, with emphasis on their common clinical features, morphologic patterns, and prognostic factors.

Minimally invasive eyelid care in dermatology: medical, laser, and cosmetic therapies.

There is a high demand for facial aesthetic enhancement, and the periorbital region is one of the first areas to show signs of aging. Many nonsurgical therapies exist to tackle these problems. These therapies are becoming more efficacious while reducing postprocedural recovery time. The goal is to restore the natural youthful appearance rather than to create an effect that differs from the patient's appearance earlier in life. Eyelid care, and cosmetic dermatology in general, intends to restore balance and symmetry. We discuss the initial consultation, relevant anatomy for the dermatologist, and medical, laser and cosmetic therapies.

Treatment Challenges with Benign Bone Tumors of the Orbit.

Benign mesenchymal tumors of the craniofacial complex present unique challenges for orbital surgeons because of their potential for orbital compartment syndrome, ocular morbidity, and facial disfigurement and because definitive surgical management may be associated with significant morbidity. While the precise classification of such lesions depends on radiologic as well as histologic evaluations and remains controversial, benign tumors involving the bony walls of the orbit share features of bony expansion, facial deformity, and the potential to cause significant orbital and ophthalmic morbidity. We herein present 2 cases of benign mesenchymal tumors with bony involvement in the orbitofacial region (1 juvenile ossifying fibroma and 1 central giant cell granuloma) and review the current management of similar benign fibro-osseous and reactive bone lesions of the orbit. These rare entities presented share common orbital and ophthalmic manifestations and remain without any effective definitive treatment options.

Correlation of American Joint Committee on Cancer T category for eyelid carcinoma with outcomes in patients with periocular Merkel cell carcinoma.

PURPOSE: Merkel cell carcinoma is a rare but potentially deadly cancer of the eyelid. To date, no studies have reported on clinical parameters at initial presentation of the eyelid tumor that may correlate with disease-free survival (DFS). The purpose of this study was to determine whether the American Joint Committee on Cancer (AJCC) T category for eyelid carcinoma correlates with metastasis and DFS in patients with Merkel cell carcinoma of the periocular region. METHODS: This is a retrospective cohort study from a tertiary referral cancer center. All consecutive patients treated for eyelid or periocular Merkel cell carcinoma between November 1, 1998, and November 1, 2012, were reviewed. The main outcome measures included AJCC T category for eyelid carcinoma and Merkel cell carcinoma at presentation, nodal metastasis at presentation, metastasis during follow up, and DFS. RESULTS: The study included 18 patients, 7 men and 11 women, with median age of 68.5 years. The AJCC T categories for both eyelid carcinoma and Merkel cell carcinoma were significantly correlated with DFS. Using the T categories for eyelid carcinoma, when TX and T2a were grouped into a single category and T2b and T3a into another category, the estimated DFS rate at 3 years was 100% for patients with TX or T2a lesions and 57% for patients with T2b or T3a lesions (p=0.0298). Four patients (22%) had lymph node metastasis at presentation. Presence of lymph node metastasis at presentation was the strongest single predictor of shorter DFS and an increased risk of metastasis during follow up (p=0.0222). CONCLUSIONS: The AJCC eyelid carcinoma T at presentation correlates with DFS in patients with Merkel cell carcinoma of the periocular region. The DFS rate at 3 years was significantly worse for patients with T2b or more extensive tumors by eyelid carcinoma T category. Presence of lymph node metastasis at presentation was predictive of shorter DFS and an increased risk of metastasis during follow up.

Clinical value of magnetic resonance imaging and other baseline testing for conjunctival mucosa-associated lymphoid tissue lymphoma.

Abstract The objective of this study was to assess the value of magnetic resonance imaging (MRI) of the orbit for conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma. The yield of other staging tests at baseline were also evaluated. Twenty-one consecutive patients treated for conjunctival MALT lymphoma were retrospectively studied. Lymphoma was staged according to both the Ann Arbor system and the seventh edition of the AJCC [American Joint Committee on Cancer] cancer staging manual. Findings on MRI of the orbit, whole-body positron emission tomography/computed tomography (PET/CT), CT of the chest/abdomen/pelvis, bone marrow (BM) biopsy and gastrointestinal (GI) endoscopy were recorded. Seventeen patients had orbital MRI. Fourteen of 17 patients (82%) with obvious conjunctival MALT lymphoma on clinical examination had a negative MRI scan. Only three patients had subtle conjunctival enhancement on orbital MRI. Ann Arbor stage at presentation was as follows: stage IE (15 patients), stage IIE (two patients) and stage IV (four patients). Eighteen of 21 patients had total-body PET/CT; four patients (22%) had hypermetabolic activity evident on PET scan. All 21 patients had bilateral BM biopsies. Fifteen of 21 patients (71%) had GI endoscopy. None of the patients had a positive BM biopsy or findings on GI endoscopy. Our data suggest that orbital MRI has a very low yield for identification of conjunctival MALT lymphoma. Clinical examination is critical in diagnosing and assessing treatment response in conjunctival MALT lymphoma. The yield for GI endoscopy and BM biopsy may also be low in staging of conjunctival MALT lymphoma.

Targeted therapy for orbital and periocular basal cell carcinoma and squamous cell carcinoma.

PURPOSE: To review the literature on targeted therapy for orbital and periocular basal cell carcinoma (BCC) and cutaneous squamous cell carcinoma (SCC) and provide examples of patients recently treated with such therapy. METHODS: The authors reviewed the literature on clinical results of targeted therapy and the molecular basis for targeted therapy in orbital and periocular BCC and cutaneous SCC. The authors also present representative cases from their practice. RESULTS: Mutation in the patched 1 gene (PTCH1) has been implicated in BCC, and overexpression of epidermal growth factor receptor (EGFR) has been shown in SCC. Vismodegib, an inhibitor of smoothened, which is activated upon binding of hedgehog to Ptc, has been shown to significantly decrease BCC tumor size or even produce complete resolution, especially in cases of basal cell nevus syndrome. Similarly, EGFR inhibitors have been shown to significantly decrease SCC tumor size in cases of locally advanced and metastatic disease. The authors describe successful outcomes after vismodegib treatment in a patient with basal cell nevus syndrome with numerous bulky lesions of the eyelid and periocular region and erlotinib (EGFR inhibitor) treatment in a patient with SCC who was deemed not to be a good surgical candidate because of advanced SCC of the orbit with metastasis to the regional lymph nodes, advanced age, and multiple medical comorbidities. CONCLUSIONS: Targeted therapy using hedgehog pathway and EGFR inhibitors shows significant promise in treatment of orbital and periocular BCC and cutaneous SCC, respectively. Such targeted therapy may be appropriate for patients who are not good candidates for surgery.

Sentinel lymph node biopsy for eyelid and conjunctival tumors: what have we learned in the past decade?

PURPOSE: To describe current indications, methods, and outcomes of sentinel lymph node (SLN) biopsy for eyelid and conjunctival tumors. METHODS: Review of experience to date and relevant articles published in PubMed in English. RESULTS: The use of SLN biopsy for conjunctival and eyelid tumors has evolved greatly in the past decade, and positive SLNs have been reported for conjunctival and eyelid melanoma, eyelid Merkel cell carcinoma, eyelid sebaceous carcinoma, and eyelid squamous cell carcinoma. Current indications for SLN biopsy of eyelid and conjunctival malignancies are the presence of conjunctival melanomas ≥2 mm in histologic thickness and/or histologic ulceration; cutaneous eyelid melanomas ≥1 mm thick, those with >1 mitotic figures per high-power field, and/or those with histologic ulceration; sebaceous carcinomas ≥10 mm in width; and Merkel cell carcinomas of any size. The frequency of false-negative biopsy results seems to be decreasing as more experience is gained with the technical nuances of the procedure and with the complex lymphatic drainage of the head and neck region. Given the emerging data published on feasibility and reported cases of microscopically positive SLNs identified in patients with otherwise normal examination of the regional lymph nodes and normal imaging studies, it seems appropriate to continue to further evaluate SLN biopsy for selected ocular tumors in future prospective studies. CONCLUSIONS: SLN biopsy is feasible for eyelid and conjunctival tumors, and continued use of the procedure is recommended. Future multi-institutional trials are needed to expand on currently available data, fine-tune patient selection criteria, and elucidate the relationships between SLN status and patient survival and tumor recurrence.