The first UK national blepharospasm patient and public involvement day; identifying priorities.

PURPOSE: We describe the planning and outcomes of the first 'Blepharospasm Day' in the UK. Blepharospasm is a distressing condition for patients and carers. Our 'patient and public involvement' event aimed to: cultivate a more informed patient group via active dialogue, help clinicians more effectively prioritise research and to facilitate peer-to-peer support for affected patients and public. DESIGN: A national one-day event was organised by the oculoplastics department at Moorfields Eye Hospital. The event was divided into informative lectures delivered by professionals and a patient panel, during which patients shared their experiences and expectations. METHODS: Data were collected from a variety of sources including: an interactive voting "LiveWall" poster, a pre-event questionnaire; "living with Blepharospasm", transcripts from patient panel discussions; and a feedback questionnaire. RESULTS: The event was well-received with 100% of respondents rating it good or excellent. Four research themes were identified: "aetiology", "alternative treatments", "faster, more accurate diagnosis", and "symptom control". Delegates' self-reported knowledge of blepharospasm increased significantly after the event. Limitations of the BdSI severity-assessment tool were noted with 22% of respondents failing to utilise it appropriately. CONCLUSION: Through our innovative "Blepharospasm Day", patient's priorities for research were identified, delegates understanding of blepharospasm increased and an independent blepharospasm patients-representatives' group was established; a first in the UK. Furthermore, short-fallings identified in the BdSI tool highlight the need for better severity-assessment tools. We demonstrate the benefits of the 'patient and public involvement' approach in the management of complex conditions such as blepharospasm. ABBREVIATIONS: PPI: Patient and public involvement; SLV-PSP: sight loss and vision sector - priority setting partnership; BRC: Biomedical Research Centre; NIHR: National Institute for Health Research; BsDI: Blepharospasm Disability Index.

Sensory Trick Frames: A New Device for Blepharospasm Patients.

OBJECTIVE: To determine whether the use of unique customized spectacles provided with modified side arms may be helpful in reducing benign essential blepharospasm (BEB) in patients describing periocular sensory tricks (ST). METHODS: A prospective descriptive study of patients with BEB with positive periocular or temporal region ST phenomenon response under the care of the Botox Clinic at Moorfields Eye Hospital, London, UK. Nine consecutive patients with BEB describing ST were recruited, and the disease frequency and severity were assessed with the Jankovic Rating Scale (JRS) and the Blepharospasm Disability Index (BSDI) before and after the use of the sensory trick frames (STF). RESULTS: A reduction in the score was noted in both severity (p = 0.0115) and frequency patterns (p = 0.0117) in the JRS in patients using the STF. A significant reduction of the BSDI score was also observed (p = 0.0314). CONCLUSION: All the patients selected and fitted with the STF had a reduction in spasms and related symptoms. This new device may be helpful in some selected BEB patients who previously responded positively to periocular pressure alleviating maneuvers.

The "Wedge Sign": An Imaging Sign for Aggressive Lacrimal Gland Disease.

PURPOSE: Lacrimal gland carcinoma can form a triangle of tissue back to the orbital apex, intraconal spread apparently being prevented by the intermuscular septum. The "wedge sign" frequency is assessed in lacrimal carcinoma, lacrimal lymphoma, or dacryoadenitis. DESIGN: Retrospective masked review of images from patients with biopsy-proven lacrimal gland pathology. METHODS: For each patient, the presence of a triangle of tissue between the lateral rectus and lateral orbital wall and the superior rectus and the orbital roof was assessed by masked review of computed tomography or magnetic resonance imaging. For the lateral compartment, the wedge was classified as "grade 1" if it just reached the sphenoidal trigone and "grade 2" if it was a complete triangle reaching the orbital apex. Comparison of proportions was made using the Fisher exact test, using an α risk of 0.05 as clinically significant. RESULTS: Imaging for 116 patients was reviewed: 39 with lacrimal gland carcinoma, 37 with lymphoma, and 40 with dacryoadenitis. The lateral wedge (grade 1 or 2) was most common in patients with carcinoma (16/39; 41%), was present in 11 of 37 patients (30%) with lymphoma, and was rarest in patients with dacryadenitis (6/40; 15%) (P = 0.033). The proportion in patients with carcinoma (41%) was similar to that in patients with lymphoma (30%) (P = 0.345); the proportion in patients with lymphoma (30%) was similar to that in patients with dacryoadenitis (15%) (P = 0.170), but carcinoma (41%) was significantly different from dacryoadenitis (15%) (P = 0.013). The proportions for malignant lesions (carcinoma and lymphoma; 27/76, 36%) and benign dacryoadenitis (15%) were significantly different (P = 0.029). Likewise, a superior wedge, of any extent, was present in 10 of 39 carcinomas (26%), 2 of 40 dacryoadenitis (5%), and 6 of 37 lymphomas (16%) (P = 0.033). CONCLUSIONS: The "wedge sign" is most common in lacrimal gland carcinoma, but can occur in patients with severe forms of dacryoadenitis or lymphoma and generally indicates life-threatening lacrimal gland pathology that requires urgent biopsy. It is significantly more common in lacrimal gland carcinoma compared with dacryoadenitis and in malignancy (lymphoma and carcinoma) compared with inflammation.

Orbital melanoma masquerading as a "Galloping haemangioma".

We describe a 32-year-old pregnant woman who was referred to our clinic after 6 weeks of observation elsewhere with a rapidly expanding orbital mass, proptosed globe and slowly decreasing of vision in her left eye. To our examination the patient presented with congested optic disc fine macular striae and some slight choroidal elevation without any retinal pigmentation. An MRI scan without contrast was performed, suggesting the signal charactheristics of an orbital mass consistent with a cellular lesion such as a cavernous hemangioma or a solitary fibrous tumour. Despite the benign-looking imaging, the fast-growing pattern of the lesion suggested a more sinister picture. A fine needle aspiration of the orbital mass was carried out, revealing the presence of an amelanotic melanoma. This case highlights the importance of considering the presence of a melanoma when there is a clear history of a rapid lesion progression.

Secondary orbital ball implants after enucleation and evisceration: surgical management, morbidity, and long-term outcome.

PURPOSE: To investigate effectiveness of a simplified surgical technique for secondary ball implantation in anophthalmic sockets and to compare long-term results of secondary ball implantation in patients previously enucleated or eviscerated. METHODS: The study is a case series analysis of the clinical charts of 110 consecutive patients who underwent secondary ball implantation after enucleation or evisceration, from January 1998 to December 2011, under the care of 1 surgeon. Patients undergoing primary evisceration and implant exchange were excluded. Primary surgery was due to trauma in 48.8% patients, endophthalmitis and phthisis bulbi in 25.6%, tumors in 22.1%, and orbital vascular malformations in 3.5%. This study adheres to the principles outlined in the Declaration of Helsinki. RESULTS: Of 110 identified cases, 24 were excluded for insufficient follow-up (less than 2 years); mean follow-up was 6.4 years. Group A patients (previously enucleated) received a polyglactin mesh-wrapped implant. Group B patients (previously eviscerated) kept their own sclera as a secondary anterior capping on the polyglactin mesh-wrapped implant. There were 2 implant exposures (4.9%; 2 of 41) in group A. Hard palate graft was used to repair the exposed implant successfully. No exposure was noted in group B. No statistically significant between-group difference in exposure rate was found. CONCLUSIONS: Stable secondary ball implantation can be achieved long term, and a reliable surgical technique is the most important factor in predicting implant stability. In patients who had secondary implants following evisceration, sclera and polyglactin mesh may act as duplicate barriers between anterior surface of implants and overlying tissues.