Squamous carcinoma ex pleomorphic adenoma of the lacrimal gland.

We present a very rare case of squamous cell carcinoma (SCC) ex pleomorphic adenoma of the lacrimal gland. Our patient presented with a 12 month history of painful proptosis of his left eye associated with severe headache. Imaging showed a left lacrimal gland lesion with extensive orbital disease extending into lateral and superior rectus muscles, cavernous sinus and the greater wing of the sphenoid. A lacrimal gland biopsy showed a combination of small bland glandular structures and sclerotic, elastin-containing stroma showing that the SCC had arisen on a background of a probable pleomorphic adenoma. Treatment with cisplatin and 5-Fluorouracil proved efficacious with a significant reduction of orbital and post-orbital disease on interval scanning.

Metastatic testicular choriocarcinoma presenting as eyelid swelling and ptosis.

A 34-year-old man presented with an 8-day history of swelling and ptosis affecting the right upper eyelid. An MRI scan showed right superior rectus enlargement. Histology of an incisional biopsy of the muscle demonstrated metastatic choriocarcinoma to the orbit, positive for pan-cytokeratins, beta-HCG and GATA3. Possible primary sites included testis. An ultrasound of the testes identified bilateral testicular masses, highly suspicious for primary testicular malignancy. A CT scan of the chest, abdomen and pelvis identified disseminated metastatic disease conferring a poor prognostic germ cell tumour. The overall interpretation was of disseminated testicular choriocarcinoma and the patient is currently undergoing intensive chemotherapy.

A case and literature review of orbital microcystic adnexal carcinoma.

Microcystic adnexal carcinoma (MAC) is a very rare and locally aggressive sweat gland tumour. MAC has been well reported presenting as a periocular cutaneous lesion, rarely with subsequent direct orbital invasion and only once as a primary orbital lesion. Local recurrence is frequent after primary surgical excision and the role of adjuvant radiotherapy is ill-defined. We describe a case of orbital MAC treated successfully with radiotherapy after incomplete margin clearance post exenteration surgery and review the associated literature.

A new cause of lacrimal gland calcification: Retained metallic foreign bodies.

A 49-year-old male presented with a 4 month history of dysaesthesia in the left periorbital region. A CT scan showed a lacrimal gland mass with areas of dense calcification. Biopsies of the left lacrimal gland revealed a silver material with associated chronic granulomatous inflammation and secondary calcification. The histological specimen was sent for X-ray microanalysis. This confirmed the silver material to be aluminium. The metal fragments were subsequently discovered to be from a car aerial, which caused an orbital impaling injury 20 years prior to presentation. This is the first reported case of lacrimal gland calcification secondary to chronic metal foreign body exposure. The case is presented alongside literature reviews of lacrimal gland calcification and associated radiological findings.

The "leicester lasso" lateral canthoplasty.

PURPOSE: To describe an innovative technique of lateral canthal tendon (LCT) anchoring to the lateral orbital rim on its inner aspect using a "lasso" technique, in order to provide the ideal vector. METHODS: A retrospective case review of seven patients (n = 7), performed from 2009 to 2013 at our institution by the senior author (RGS). RESULTS: Excellent results in all cases with optimal restoration of form and function. CONCLUSIONS: The 'Leicester Lasso' technique is a safer technique of securing the LCT to the orbital rim.

Inverting sutures for tarsal ectropion (the leicester modified suture technique).

PURPOSE: To describe a simple technique to correct tarsal lower eyelid ectropion with or without marked eyelid laxity (The Leicester Modified Suture technique). METHODS: A retrospective interventional case series of patients undergoing correction of tarsal ectropion with inverting sutures. The study adhered to the principles of the Declaration of Helsinki. RESULTS: Twenty patients (25 eyelids) met the inclusion criteria. The success of the procedure was assessed by improvement of symptoms, eyelid position, and the need for reoperation. The mean postoperative follow up was 3.6 months (range 2-15 months). Eighteen patients (90%) had a successful outcome, while in 2 patients (10%) the outcome was satisfactory, as there was mild residual eversion of the eyelids. None of the patients required reoperation. CONCLUSIONS: Tarsal lower eyelid ectropion can be surgically challenging to correct via the transconjunctival plication or subciliary reattachment of retractors. This method is simple to use, resulting in an excellent outcome with no tissue dissection. This technique is easy to learn and implement for oculoplastic surgeons at all levels of experience.

Lacrimal gland biopsies-results from a tertiary centre in the UK.

PURPOSE: To report the histopathological results of lacrimal gland biopsies over a 21-year period in a tertiary referral centre in the United Kingdom. To the best of our knowledge, this represents the largest series to be published in the United Kingdom. METHODS: A retrospective observational review was carried out for patients who underwent lacrimal gland biopsies in a tertiary referral centre at the University Hospitals of Leicester, United Kingdom between the years of 2000 and 2021. RESULTS: Lacrimal gland biopsies were performed on 248 patients during the specified 21-year period. They comprised 157 (63.3%) females and 91 (36.7%) males. The mean age at presentation was 50.8 years (range 15-94 years). The majority of patients were Caucasian (69.4%, n = 172) followed by Asians (25.0%, n = 62), African/Afro-Caribbean (4.8%, n = 12) and other ethnicities (0.8%, n = 2). The most common histopathological diagnosis was chronic inflammation dacryoadenitis (69.0%, n = 171) followed by lymphomas (15.3%, n = 38). CONCLUSION: Our study shows that chronic inflammation accounts for the majority of histopathological diagnosis followed by lymphoproliferative disorders.

Feasibility and clinical utility of handheld fundus cameras for retinal imaging.

BACKGROUND/OBJECTIVES: Handheld fundus cameras are portable and cheaper alternatives to table-top counterparts. To date there have been no studies comparing feasibility and clinical utility of handheld fundus cameras to table-top devices. We compare the feasibility and clinical utility of four handheld fundus cameras/retinal imaging devices (Remidio NMFOP, Volk Pictor Plus, Volk iNview, oDocs visoScope) to a table-top camera (Zeiss VisucamNM/FA). SUBJECTS/METHODS: Healthy participants (n = 10, mean age ± SD = 21.0 ± 0.9 years) underwent fundus photography with five devices to assess success/failure rates of image acquisition. Participants with optic disc abnormalities (n = 8, mean age ± SD = 26.8 ± 15.9) and macular abnormalities (n = 10, mean age ± SD = 71.6 ± 15.4) underwent imaging with the top three scoring fundus cameras. Images were randomised and subsequently validated by ophthalmologists masked to the diagnoses and devices used. RESULTS: Image acquisition success rates (100%) were achieved in non-mydriatic and mydriatic settings for Zeiss, Remidio and Pictor, compared with lower success rates for iNview and oDocs. Image quality and gradeability were significantly higher for Zeiss, Remidio and Pictor (p < 0.0001) compared to iNview and oDocs. For cup:disc ratio estimates, similar levels of bias were seen for Zeiss (-0.09 ± SD:0.15), Remidio (-0.07 ± SD:0.14) and Pictor (-0.05 ± SD:0.16). Diagnostic sensitivities were highest for Zeiss (84.9%; 95% CI, 78.2-91.5%) followed by Pictor (78.1%; 95% CI, 66.6-89.5%) and Remidio (77.5%; 95% CI, 65.9-89.0%). CONCLUSIONS: Remidio and Pictor achieve comparable results to the Zeiss table-top camera. Both devices achieved similar scores in feasibility, image quality, image gradeability and diagnostic sensitivity. This suggests that these devices potentially offer a more cost-effective alternative in certain clinical scenarios.

A prospective, multicentre study of malignant and premalignant lesions at the base of periocular cutaneous horns.

PURPOSE: The primary objective of the study was to determine the incidence of the various pathological conditions present at the base of the periocular cutaneous horns. The secondary objective was to study the presentation of these cutaneous horns with a view to finding any clinical indicators for premalignant, malignant and benign lesions at the base. METHODS: Prospective multicentre study of patients presenting with cutaneous horns. Informed consent followed by excision biopsy of the base lesion was performed in all the cases included for the study. The biopsy specimens were examined histologically and results analysed. RESULTS: Twenty-six patients presented with cutaneous horns in the periocular region over a period of 2 years, of these two patients presented with more than one cutaneous horn. 23 patients underwent excision biopsy. There were a total of 25 specimens. Of the base lesions 2/25 (8%) were malignant, 7/25 (28%) were pre-malignant and the remaining 16/25 (64%) were benign. Analysis of the findings on clinical presentation revealed no clinical indicators to correlate with the final histology. CONCLUSION: The incidence of malignant and pre-malignant lesions present at the base of periocular cutaneous horns was 36% with 8% of them being malignant. It is important to perform an excision biopsy with histological diagnosis of the base of the cutaneous horn as there are no definite clinical features that point towards a potential for malignancy.

Orbital metastasis from neuroendocrine tumour: Case report and literature review.

Orbital metastases from neuroendocrine tumour are rare entities. An incidental finding of orbital metastasis originating from neuroendocrine tumour is presented in the context of a comprehensive review of all case reports and series published to date. Demographics, clinical features, diagnostic work-up, treatment and prognostic data from the published literature are discussed. Ninety-five patients with orbital metastases from neuroendocrine tumours have been reported so far. Average age at presentation is 63.8 years (range 25-86), with no significant gender predominance. Gastrointestinal tract is the most common site of primary neuroendocrine tumours (62-85%). Typical presentation includes proptosis (80-85%) and diplopia (27-62%) in patients with known primary neuroendocrine tumour elsewhere. Histological subtype and presence of metastases seem to be important prognostic factors. Incidental finding of neuroendocrine tumour mestastases in asymptomatic patients with unknown primary disease is uncommon. In such cases, orbital biopsy and structural and functional imaging are essential to establish a diagnosis and stage the disease.

Prevalence, indications and demographic characteristics of Botulinum neurotoxin use in a tertiary oculoplastic centre.

OBJECTIVES: Over the past 40 years, Botulinum Neurotoxin (BoNT) treatment has been used in many presentations to the hospital eye service. There is little published on its practice in an ophthalmology setting. We aim to report on the prevalence of BoNT use, indications for treatment, age, gender, socioeconomic and ethnic variations observed, and dosages used. METHODS: We performed a retrospective cross sectional observation study on the use of botulinum neurotoxin treatment in the oculoplastic department of a busy tertiary centre in the University Hospitals of Leicester, United Kingdom. RESULTS: The prevalence of BoNT in the service was 13.7 per 100,000 people. Of the 145 cases identified, the commonest indications for treatment were hemifacial spasm (62% of cases) and blepharospasm (29% of cases). Proportionally, twice as many females than males received BoNT (χ2 = 17.3, p < 0.0001). For those >30 years of age, the prevalence increased with age with those >90 years having a seven times higher prevalence than the mean. Overall, no significant differences were found in ethnicity and median index of multiple deprivation rank between those with treatment and the general population although specific district council variations were noted. CONCLUSIONS: These findings would assist commissioners in providing adequate resources to meet demand based on the demographics of their local population. Further qualitative and quantitative research is required to enhance our understanding of some of these trends.

Acute Leukemia Presenting in the Pediatric Orbit.

We present a case series to evaluate the clinical features of acute leukemia presenting with primary orbital manifestations. We undertook a retrospective case review of primary orbital presentations of acute myeloid leukemia (AML) and acute lymphocytic leukemia (ALL) over a 10-year period at two hospital sites (Hereford County Hospital and Leicester Royal Infirmary). Our case series included four patients - two with AML and two with ALL. Patients were young (mean age of four years and five months) at presentation, all with unilateral disease, and presented with orbital signs. Although there was some confusion with the diagnosis at the time of referral, a suspicion of malignancy was made rapidly once ophthalmic review was initiated. All four cases were diagnosed with the assistance of peripheral blood film and bone marrow biopsy, without the need for orbital biopsy. All four cases had resolution of the orbital mass and remain disease-free.

Periorbital Masson's tumor: a case series.

Intravascular papillary endothelial hyperplasia (IPEH) or Masson's tumor is an unusual variety of benign vascular tumor. Involvement of periorbital area is uncommon. We report 2 cases of periorbital IPEH and review relevant literature.

Chronic Macular Oedema as a Late MIRAgel-Related Complication.

BACKGROUND: MIRAgel® (MIRA, Waltham, MA, USA) is a hydrogel scleral buckle introduced in 1979 to treat rhegmatogenous retinal detachments. Its use was discontinued because late complications that require surgical removal were reported. METHODS: Case report. RESULTS: We report a case of left eye MIRAgel® buckle surgery 28 years ago presenting with a tender palpable erythematous swelling at the lower lid, with marked conjunctival chemosis and progressive ophthalmoplegia. Imaging revealed a large, well-defined, horseshoe-shaped lesion in the extraconal space of the left orbit with globe distortion, with histological confirmation of an expanded hydrogel buckle. He recovered well following removal of the explant but developed chronic macular oedema a year later, which persisted despite sub-Tenon's triamcinolone injections. Repeat imaging demonstrated remaining hydrogel explant. Macular oedema settled well upon successful surgical removal with no recurrence to date. CONCLUSION: Our case is the first to describe macular oedema as a late MIRAgel-related complication, with complete removal of the explant being the definitive treatment. Macular oedema indicates postoperative inflammation secondary to the remaining explant fragments. Given the friability of hydrolysed MIRAgel®, we recommend ophthalmologists to warn patients regarding the possibility of further inflammation in the globe or the orbit in case of incomplete removal.

The role of intravenous methylprednisolone immunosuppression in the management of active thyroid eye disease.

PURPOSE: To analyze the efficacy of intravenous methylprednisolone (IVMP) immunosuppression in the management of active thyroid eye disease (TED). METHODS: This is a consecutive case series of 36 patients treated with 1 g of IVMP over 3 days, between January 2000 and January 2008. The indications for this treatment were moderate to severe active TED with or without optic nerve compression. RESULTS: There were 32 hyperthyroid, 2 hypothyroid, and 2 euthyroid patients in this series. Nine patients presented with optic nerve compromise, 3 had severe active TED and 24 had moderately active TED. Twenty-seven patients received one course of IVMP and nine patients received two courses. All patients had tapering oral prednisolone over 1 month following the IVMP. The mean improvement in proptosis was 1.31 mm (range = 0-3 mm). Optic nerve function improved in seven of nine patients (77.8%). Extra ocular muscle movement improved in 18 patients (50%). Overall benefit from IVMP was seen at 1/12 in 27 patients (75%) and at 3/12 in 30 patients (83%). After IVMP one patient underwent radiotherapy and three patients had nonsteroidal immunosuppression. CONCLUSIONS: IVMP immunosuppression is effective in 83% of patients. The effect lasts from 3 months to 8 years, with a mean of 15 months in 30.5%. It remains a useful modality of treatment especially when the optic nerve or cornea is threatened because of moderate to severe active TED. We encountered no severe side effects of pulsed IVMP and 3 g over 3 days is a safe dose.

Yellow lid sign in Wegener's granulomatosis.

Orbital manifestations in Wegener's granulomatosis develop in over 50% of patients. Ischemic vasculitis and optic nerve compression result in visual impairment. This case report highlights the importance of the yellow lid sign in the diagnosis of orbital granulomatosis as well as provides an explanation for the reversal of this phenomenon.

Fronto-orbital mucocele and orbital involvement in occult obstructive frontal sinus disease.

AIMS: To describe the clinical features of orbital involvement arising from occult obstructive frontal sinus disease and to highlight key features to aid diagnosis. METHODS: A retrospective review of the case notes of 15 patients who presented to three regional oculoplastics units over a 7-year period were reviewed. Patient demographics, clinical characteristics, relevant medical history, microbiology results, imaging, management and outcomes were assessed. RESULTS: The mean age of our patients was 55 years. Pain was the presenting feature in 8 patients while 14 presented with upper lid swelling and redness. This swelling was medial in 60% and diffuse in 40% of patients. Proptosis was noted in 11 patients. The correct diagnosis was suspected in only 53% of patients at the time of first presentation to an ophthalmologist. The mean time from onset of symptoms to the time of correct diagnosis was 53 weeks (median 3 weeks). On direct questioning, eight patients reported a previous history of intermittent upper lid swelling and eight patients had a past history of sinusitis or frontal sinus trauma. CONCLUSIONS: We present a large series of patients with occult sinus disease presenting with orbital involvement. As patients with this potentially life-threatening condition are often misdiagnosed, we hope this paper draws the ophthalmologist's attention to key pearls in the history and examination to make a prompt diagnosis.