Hybrid Neurofibroma/Schwannoma of the Orbit.

A 68-year-old female presented for assessment of a space occupying lesion of her right orbit, demonstrated on MRI. An upper lid crease anterior orbitotomy was performed and the lesion excised completely. Postoperatively, she had reduced sensation in the distribution of the supraorbital nerve. Histopathologic examination of the excised lesion revealed a hybrid neurofibroma/schwannoma. This represents the fourth reported case of such a lesion arising within the orbit.

Lacrimal Gland Orbital Lobe Cysts Associated With Lymphoid Hyperplasia or Mucosa-Associated Lymphoid Tissue Lymphoma in Patients With Chronic Autoimmune Disease.

Large cysts in the orbital lobe of the lacrimal gland are rare and are associated with Sjögren syndrome and B-cell mucosa-associated lymphoid tissue lymphoma. The authors describe 4 new cases of large orbital lobe lacrimal gland cysts. The first 2 patients, both with Sjögren syndrome, had unilateral cysts associated with chronic inflammation. Mucosa-associated lymphoid tissue lymphoma was also identified in the cyst wall of the second case and could not be completely excluded in the first case. The third patient, with a history of rheumatoid arthritis, had bilateral cysts, again associated with mucosa-associated lymphoid tissue lymphoma. The fourth patient, with no history of systemic disease, had a unilateral cyst associated with reactive lymphoid hyperplasia. Finally, the authors report the long-term outcomes of 3 previously reported cases.

Pyoderma gangrenosum affecting the eye, orbit, and adnexa. A review.

While pyoderma gangrenosum (PG) most commonly affects the skin it can also involve other organs. It rarely affects the eye, orbit, or adnexa. Its early recognition and proper management with prompt initiation of immunosuppressive therapy are essential for the preservation of the eye and, in turn, vision, in these cases. Here, we review the limited literature already published regarding ocular involvement in PG.

Orbital abscess following posterior subtenon injection of triamcinolone acetonide.

Orbital cellulitis is a serious sight threatening and potentially life threatening condition which can be complicated by orbital abscess formation. Posterior subtenon (PST) injection of corticosteroid is commonly used in the treatment of posterior segment inflammation including post-operative macular oedema. We report a case of orbital abscess formation as a late complication of PST triamcinolone acetonide and discuss the presentation, diagnosis and management.

Challenges in the management of Neisseria gonorrhoeae keratitis.

We describe the presentation and subsequent management of a case of keratitis caused by Neisseria gonorrhoeae. A thirty-nine year old gentleman presented with a purulent ocular discharge. Corneal melt with corneal perforation occurred. Neisseria gonorrhoeae was cultured. Systemic and topical antibiotics were given. Deep lamellar keratoplasty was performed for corneal perforation. At three months post treatment no recurrence of infection was noted. The possibility of Neisseria gonorrhoeaea keratitis should always be considered in patients with a purulent ocular discharge even if the case history is not immediately suggestive of the same. Severe gonococcal keratitis may be unilateral. Deep lamellar keratoplasty can be considered as a therapeutic option in patients with severe gonococcal keratitis.

Lipofuscin accumulation and autophagy in glaucomatous human lamina cribrosa cells.

BACKGROUND: Disease associated alterations in the phenotype of lamina cribrosa (LC) cells are implicated in changes occurring at the optic nerve head (ONH) in glaucoma. Lipofuscin, the formation of which is driven by reactive oxygen species (ROS), is an intralysosomal, non-degradable, auto-fluorescent macromolecule which accumulates with age and can affect autophagy - the lysosomal degradation of a cell's constituents. We aimed to compare the content of lipofuscin-like material and markers of autophagy in LC cells from normal and glaucoma donor eyes. METHODS: The number and size of peri-nuclear lysosomes were examined by transmission electron microscopy (TEM). Cellular auto-fluorescence was quantified by flow cytometry. Cathepsin K mRNA levels were assessed by PCR. Autophagy protein 5 (Atg5) mRNA and protein levels were analysed by PCR and Western blot. Protein levels of subunits of the microtubule associated proteins (MAP) 1A and 1B, light chain 3 (LC3) I and II were analysed by Western blot. Immunohistochemical staining of LC3-II in ONH sections from normal and glaucomatous donor eyes was performed. RESULTS: A significant increase in the number of peri-nuclear lysosomes [4.1 × 10,000 per high power field (h.p.f.) ± 1.9 vs. 2.0 × 10,000 per h.p.f. ± 1.3, p = 0.002, n = 3] and whole cell auto-fluorescence (83.62 ± 45.1 v 41.01 ± 3.9, p = 0.02, n = 3) was found in glaucomatous LC cells relative to normal LC cells. Glaucomatous LC cells possessed significantly higher levels of Cathepsin K mRNA and Atg5 mRNA and protein. Enhanced levels of LC3-II were found in both LC cells and optic nerve head sections from glaucoma donors. CONCLUSIONS: Increased lipofuscin formation is characteristic of LC cells from donors with glaucoma. This finding confirms the importance of oxidative stress in glaucoma pathogenesis. Intracellular lipofuscin accumulation may have important effects on autophagy the modification of which could form the basis for future novel glaucoma treatments.

Porous orbital implant exposure: the influence of surgical technique.

OBJECTIVES: To examine orbital implant exposure rates following enucleation and evisceration. METHODS: A retrospective chart review of all patients who underwent an evisceration or enucleation in a single centre over a 5-year period was performed. The indication for surgery, the type and size of orbital implant used, details of the surgical technique adopted and the postoperative complications observed, in particular orbital implant exposure, were recorded. RESULTS: Over a 5-year period 24 patients had enucleation and 14 had evisceration; 22 of these procedures were performed by an ophthalmic surgeon with a special interest in orbit and oculoplastics while the remaining 16 procedures were performed by 5 ophthalmic surgeons with other sub-specialty interests. Orbital implant exposure occurred in 8 cases. One case had been performed by the first of these surgeons described. Implant exposure occurred in 4% of cases performed by this surgeon. The remaining 7 cases had been performed by the group of surgeons with sub-specialty interests outside of orbit and oculoplastics. Implant exposure occurred in 48% of cases performed by this group. CONCLUSIONS: Orbital implant exposure remains a significant cause of morbidity in patients undergoing enucleation and evisceration. Rates of orbital implant exposure were significantly lower when surgery was performed by an orbital surgeon. Differences in surgical technique are the most likely explanation.

Thyroid-like ophthalmopathy in a euthyroid patient receiving Ipilimumab.

A 68-year-old lady with metastatic malignant melanoma was treated with Ipilimumab. She presented to Eye Casualty unable to move her eyes. Physical examination confirmed ophthalmoplegia and identified proptosis bilaterally. Radiological imaging showed bilateral enlargement of all the extra-ocular muscles suggestive of thyroid eye disease. Laboratory investigations found this patient to be euthyroid. A diagnosis of thyroid-like orbitopathy secondary to Ipilimumab therapy was made. Thyroid function tests should be performed for all patients prior to their commencement of Ipilimumab. Thyroid-like eye disease may develop in patients treated with Ipilimumab even if they remain euthyroid.

Closantel toxicity.

Closantel is a broad-spectrum antihelminthic agent. It is a veterinary drug used only in animals-usually cattle, sheep and goats. A man in his 60s accidentally ingested approximately 1500 mg closantel. His visual acuity deteriorated. Optical coherence tomography (OCT) showed disruption of the outer retinal layers. Electroretinography identified abnormalities in macula and inner retinal function. He received methylprednisolone 1 g daily intravenously for 3 days. Improvements in both his visual acuity and OCT appearance followed. This case illustrates the profoundly destructive effect of this drug on humans even when consumed in low dose. We provide a concise summary of the small number of cases of closantel toxicity in humans, previously reported, for future reference as needed by others.

An nusual ocular manifestation of multiple myeloma.

A man in his 70s with a history of multiple myeloma presented with a 4-day history of right ptosis and a rapidly enlarging upper eyelid mass. On examination, a large, firm, smooth, pink lesion was found to descend from the conjunctiva of the superior fornix. The patient underwent excision biopsy. Histopathological analysis demonstrated monoclonal plasma cells expressing light-chain kappa, consistent with extramedullary plasmacytoma. Clinical ophthalmic manifestations of multiple myeloma are rare but also diverse. Ocular surface manifestations of multiple myeloma are extremely uncommon. Variable examination findings mean those involving the conjunctiva and may be particularly challenging to diagnose. Secondary ocular extramedullary plasmacytoma, despite its rarity, should be considered in patients with multiple myeloma.

Tectonic Descemet's stripping automated endothelial keratoplasty for corneal perforation.

We report the use of Descemet stripping automated endothelial keratoplasty (DSAEK) for the treatment of sterile corneal perforation after trauma. In an eye with corneal perforation and cataract due to trauma, cataract surgery and DSAEK were performed. Corneal integrity was promptly restored and the patient avoided tectonic anterior lamellar and penetrating keratoplasty. DSAEK may be performed for the management of corneal perforation.

Insidious ocular surface lesion in an 81-year-old woman.

Ocular surface melanoma (OSM) is rare. An 81-year-old Caucasian woman presented with a 4-month history of right eye pain and reduced vision. Histopathological examination of the excisional biopsy identified invasive amelanotic melanoma of the conjunctiva expressing Melan A and SOX10. X-ray of chest, CT of liver and MRI of the brain and orbit did not identify macroscopic metastases. She was given adjuvant topical mitomycin-C 0.04% for four cycles of 2 weeks. Her vision improved and the cornea was clear at 6 months.

Ptosis, ophthalmoplegia and corneal endothelial disease - ocular manifestations of mitochondrial disease.

PURPOSE: To describe two patients with bilateral ptosis, ophthalmoplegia, cataracts and corneal endothelial disease requiring corneal transplantation. OBSERVATIONS: Histopathological analysis of muscle biopsy samples from both patients identified features consistent with a mitochondrial cytopathy. A single multigenic mitochondrial deoxyribonucleic acid (DNA) deletion was detected in the first patient. Pathogenic mutations in the POLG gene which codes for mitochondrial DNA polymerase, tasked with replicating the mitochondrial genome were identified in the second patient. CONCLUSION: The collection of clinical features present in both cases described can be explained by a diagnosis of mitochondrial disease. IMPORTANCE: Corneal endothelial disease, in addition to ptosis, ophthalmoplegia, cataract, pigmentary retinopathy and optic atrophy should be recognised as a feature of mitochondrial disease.

Rhegmatogenous retinal detachments: primary reattachment rates and visual outcomes over a 4-year period.

BACKGROUND AND AIMS: To describe rhegmatogenous retinal detachment surgery in a Dublin tertiary referral centre over a 4-year period and to examine factors predictive of surgical and anatomical success. METHODS: A retrospective study was performed by reviewing the surgical log and the charts of patients who underwent a primary retinal detachment repair in a specialist centre over a 4-year period between 2012 and 2015. We excluded exudative and tractional cases. Multiple parameters were recorded including macular status, visual acuity, type of procedure, complications and visual and anatomical outcomes at 6 months post operation. Primary reattachment rate was calculated alongside change in visual acuity. Chi-square testing and analysis of variance were utilized to determine the effect which lens status, macular status, extent of breaks and type of procedure had on outcome and visual success. RESULTS: A total of 613 primary rhegmatogenous retinal detachment repairs were performed over the 4-year period. Our primary reattachment rate was calculated to be 88.58% (annual range 85.25-91.30%) with a perioperative complication rate of 2.94%. We noted a statistical significant improvement in VA with a median logMAR VA of 1.0 at presentation to 0.5 at 6 months post procedure. Macular status (chi-square test p = 0.15, X2 = 2.072) and lens status (chi-square test p = 0.2974, X2 = 1.086) had no statistical effect on the final anatomical outcome. However, eyes with giant retinal tears were more likely to redetach than those without (chi-square test p = 0.0069, X2 = 7.3). There was no statistical significant difference in the proportion of eyes achieving visual success by surgery category (one-way ANOVA analysis p = 0.501). CONCLUSIONS: This is the first study of its kind in Ireland and will help surgeons benchmark their results against international standards in the future. Accurate recording of logMAR acuity and intraoperative complications is imperative to assist with prospective studies.

National incidence of eyelid cancer in Ireland (2005-2015).

AIMS: We report on the incidence of cutaneous eyelid tumours in Ireland over the 11-year-period from 2005 to 2015, we identify associations between demographic factors and cutaneous eyelid tumour risk. METHODS: Skin cancers, including basal cell carcinoma (BCC), squamous cell carcinoma (SCC), melanoma, and other cancers, located on the eyelid or canthus according to ICD-10 coding, as registered by the National Cancer Registry of Ireland (NCRI), were captured from the period 2005 to 2015. Age standardised rates (ASR) were calculated according to the European Standard Population (2013). Longitudinal data analysis using linear regression, and associations with age and sex were evaluated with the statistics program R. RESULTS: There were 4824 patients diagnosed with eyelid BCC during the study period, the ASR in men and women was mean 15.87 and 13.49 per 100,00, respectively. The relative risk for eyelid BCC in men compared with women was 1.18, age was associated with incidence. There were 528 patients diagnosed with SCC; the ASR of eyelid SCC in men and women was 2.10 and 1.39 per 100,000, respectively, and increased in women annually (ß = 0.07, p = 0.0005). The relative risk for eyelid SCC in men compared with women was 1.51, and age was exponentially associated with SCC. Melanoma and other eyelid tumours were uncommon-50 and 55 cases, respectively. CONCLUSION: Incidence of both BCC and SCC increases with age and male sex. The incidence of eyelid SCC is increasing in women, and under age 50, eyelid BCC is more common in women than men. SYNOPSIS: We describe the recent incidence of eyelid cancers in Ireland, from National Cancer Registry Data. We find eyelid BCC, and also SCC, are associated with increased age. Rate of eyelid SCC is increasing in women.

Acquired lacrimal drainage apparatus obstruction in children.

BACKGROUND: Pediatric acquired lacrimal drainage apparatus (LDA) obstruction is much rarer than congenital LDA obstruction. Its etiology and treatment outcomes have not been well defined. Our aim was to examine the etiology and management of acquired LDA obstruction in children and report the results of its management. METHODS: We retrospectively reviewed the medical records of patients ≤16 years of age who presented with acquired epiphora to investigate the causes and describe the management of this condition. RESULTS: A total of 31 patients (16 males [52%]) were included. Mean age of patients was 10.9 years (range, 3-16). The main causes of acquired LDA obstruction were keratoconjunctivitis, herpes simplex blepharokeratoconjunctivitis, and trauma. Silicone tube intubation, endonasal or external dacryocystorhinostomy, and the insertion of lacrimal bypass tubes were the mainstays of management. CONCLUSIONS: It is important to suspect acquired LDA obstruction in children with acquired, persistent epiphora. Surgical management is similar to that in adults.

Choroidal Melanoma Metastatic to the Contralateral Medial Rectus After Orbital Exenteration

A 78-year-old Caucasian woman presented with pain in her right and only eye that was worse on abduction. Her history was significant for a choroidal melanoma affecting her left eye for which she underwent an orbital exenteration 12 years previously. Computed tomography and magnetic resonance imaging of the right orbit identified a mass lesion affecting the medial rectus, suspicious for metastatic melanoma. A histopathological diagnosis of metastatic melanoma was subsequently made following biopsy of the right medial rectus.

Health technology assessment: A primer for ophthalmology.

Rising healthcare costs and increasing demands for health care require techniques to choose between competing uses and even rationing of health care. Economic evaluations and health technology assessments are increasingly a means to assess the cost effectiveness of healthcare interventions so as to inform such resource allocation decisions. To date, the adoption of health technology assessments, as a way of assessing cost effectiveness, in ophthalmology has been slower, relative to their implementation in other specialities. Nevertheless, demands for eye services are increasing due to an ageing population. The prevalence of conditions such as glaucoma, cataract, diabetic eye disease and age-related macular degeneration increases with age, and it is predicted that global blindness will triple by 2050. So there is a challenge for ophthalmologists to ensure that they can contribute to, interpret, critically evaluate, and use findings from economic evaluations in their clinical practice. To aid this, this article serves as a primer on the use of health technology assessments to assess cost effectiveness using economic evaluation techniques for ophthalmologists. Healthcare systems face many challenges worldwide - changing demographics and evolution of new technologies are only going to intensify. With this in mind, ophthalmology needs to be ready and able to engage with health economists to prepare, interpret, critically evaluate and use findings of economic evaluations and health technology assessments.