Acute periocular haemorrhage associated with severe proptosis as a presenting sign of orbital tumours.

Periocular haemorrhage without a preceding history of trauma, and in the absence of vascular malformations is rare. While acute proptosis is well described in the setting of orbital pathology, accompanying periocular haemorrhage has been rarely reported. We describe three cases with these concomitant presenting signs in orbital malignancies - metastatic small cell bladder carcinoma, haemangiopericytoma (solitary fibrous tumour) and myeloma. Clinical photographs and radiology are presented with review of the current literature. All cases had an associated rapid onset of severe proptosis and co-existing periocular bruising on the same side. The presence of ecchymosis of the eyelids in addition to proptosis without a history of trauma warrants thorough investigation to rule out underlying potential sight and life threatening illness.

The use of the paramedian forehead flap alone or in combination with other techniques in the reconstruction of periocular defects and orbital exenterations.

PURPOSE: The paramedian forehead flap (PMFF) is a reconstructive option for large eyelid defects and orbital exenterations. We report a series of cases where PMFF reconstruction was carried out at various institutions in Australia. METHODS: This study was a multi-centre, retrospective, non-comparative case series investigating the clinical outcomes of the PMFF for reconstructing periocular defects and orbital exenterations. RESULTS: This case series describes twenty-seven patients (Female = 15, Male = 12), operated between 1991 to 2019, with a median age of 81 years (range: 45-93 years). Defect locations involved combinations of the medial canthus (16/27, 59.3%), upper eyelids (7/27, 25.9%), lower eyelid (4/27, 14.8%), both upper and lower eyelids (5/27, 18.5%), and orbital (7/27, 25.9%). There were no cases of flap necrosis. Minor post-operative complications were observed in ten patients with the most common being lagophthalmos. Median duration of follow-up was 17months (Range: 2months- 23years). CONCLUSIONS: The PMFF is a versatile reconstructive tool for a range of periocular defects and orbital exenterations with minor post-operative complications.

Corneal Complications Secondary to Involutional Entropion at Presentation.

PURPOSE: To determine the frequency and significance of corneal complications at presentation of involutional entropion in an Australian population. Patient demographics and duration of symptoms were collated to assess how these factors related to presentations. METHODS: Case records were retrospectively interrogated at an Oculoplastic Ophthalmology practice over a 15-year period to identify patients with lower eyelid involutional entropion. All patients had ophthalmic examination by an ophthalmologist. Patients meeting inclusion criteria had data collected including detailed assessment of corneal examination findings. RESULTS: The final cohort included 203 patients of which 50.7% were male. The mean age was 75.1 years. Cornea findings at presentation included superficial punctate keratopathy (69%), no change (25.6%), corneal ulcer (4%), and other (1%). One percent of patients had vision loss attributable to complications of entropion. Range of duration of symptoms was 2 weeks to 6 years with a mean of 11.5 months. CONCLUSIONS: At presentation, involutional entropion has mild corneal findings with 95.1% of patients demonstrating superficial punctate keratopathy or normal corneal epithelium. Vision loss is a rare complication and was observed in 1% of patients in this study, all with preexisting corneal pathology.

Bilateral Enophthalmos as an Unusual Presenting Feature of Non-Hodgkin's Orbital Lymphoma.

Lymphoma is the commonest orbital malignancy. The typical presentation is proptosis or swelling, which warrants imaging and confirmation by tissue biopsy. Enophthalmos is a much rarer clinical sign and if bilateral and symmetrical can often present late. We describe a patient who presented with bilateral enophthalmos and symptomatic, secondary entropion due to bilateral non-Hodgkin's lymphoma in which orbital fat was replaced by a monoclonal proliferation of small B cells. Low-dose orbital radiotherapy and entropion surgery relieved the patient's symptoms.

Lacrimal gland pleomorphic adenoma with extensive necrosis.

Lacrimal gland pleomorphic adenomas (LGPA) are benign mixed tumors. Diagnosis is based on clinical and radiological findings which usually prompts complete excision of the lesion to minimise recurrence and a cumulative risk of malignant transformation. Necrosis in pleomorphic adenoma has been rarely reported in salivary gland PA, either spontaneously or due to iatrogenic interventions. Necrosis is suggestive of a malignant process and makes interpretation of histology specimens difficult. A 23 year old woman, while awaiting biopsy for a mass in the left lacrimal gland, which had been symptomatic for only several months, presented with acute pain and swelling of the left lateral lid. An incisional biopsy showed an inflamed lacrimal gland with focal necrosis and atypia of adjacent cytology and gland architecture. Subsequent excisional biopsy confirmed an LGPA with some inflammation but no necrosis. Necrosis may occur as an atypical presentation in LGPA.

Cutaneous pleomorphic adenoma of the periocular region - a case series.

Mixed tumour of the skin is a rare entity also known as chondroid syringoma and pleomorphic adenoma. These usually present as slow-growing skin nodules with a smooth surface, clear boundaries, and no ulceration. Case series exist describing pleomorphic adenomas in the periocular region including the lids and orbit, separate to the more familiar lacrimal gland pleomorphic adenoma. These may arise from accessory or ectopic lacrimal gland tissue but in the eyelids are more likely to arise from sweat glands in the skin. Histopathological analysis of these lesions is important to identify complete excision, minimising recurrences and in identifying rare but potential malignant transformation. We describe the clinical features and outcomes in three cases of pleomorphic adenoma with two at the medial canthus (including one recurrence) and one in the brow region.

Current practice trends for lacrimal gland neurotoxin in the management of epiphora-a BOPSS survey.

PURPOSE: Injection of botulinum neurotoxin A (BoNTA) to the lacrimal gland (LG) offers a simple and effective treatment in the management of epiphora. However, there is little data on current practice trends or uptake as an alternative to surgery. This study assesses current practice trends of such treatment amongst BOPSS (British Oculoplastic Surgery Society) members. METHODS: All consultant BOPSS members were invited to participate in a web-based survey which consisted of 5 questions, with a reminder invitation to participate. The role, dose, potential side effects, use as an alternative to surgical intervention, and impact on service delivery were assessed. RESULTS: Fifty-one BOPSS consultants (43% uptake) completed the survey. Ninety percent of respondents were regularly using LG BoNTA in their management of epiphora. The main indicators for considering BoNTA use were medical comorbidities and elderly patients. The mean first treatment dose of Botox® was 3.6 units (SD 1.5). Diplopia and ptosis complications were always discussed in the consent for treatment in addition to dry eye. Twenty-five percent of surgeons reported doing less conjunctivo-dacryocystorhinostomies (cDCR) due to the availability of LG BoNTA. No respondents felt that the requirement for repeated BoNTA treatments was impacting on their service delivery. CONCLUSION: Uptake of LG BoNTA in the management of epiphora is at a similar rate to all other available treatments. As a result, respondents are performing less surgical procedures, particularly cDCR in patients at higher surgical morbidity.

In vitro study examining the effectiveness of antiseptic prophylaxis for antibiotic-resistant bacterial endophthalmitis.

PURPOSE: To assess the effectiveness of current antiseptic agents on multidrug-resistant (MDR) isolates in an in vitro setting. SETTING: Department of Microbiology, The National Maternity Hospital, Dublin, Ireland. DESIGN: Organisms were selected based on current review of endophthalmitis literature: methicillin-resistant Staphylococcus aureus (MRSA), methicillin-resistant Staphylococcus epidermidis (MRSE), Pseudomonas aeruginosa, carbapenem-resistant and extended-spectrum ß-lactamase Klebsiella pneumoniae, and vancomycin-resistant (VRE) Enterococcus faecalis. METHODS: Samples were exposed to povidone-iodine (PVI) 5% and chlorhexidine (CHX) 0.05% for 0.5 minutes, 1 minute, 3 minutes, and 5 minutes. After inactivation, organisms were incubated under standard conditions and growth assessed after 16 hours. RESULTS: MRSA and MRSE responded to 3-minute PVI exposure. CHX eradicated MRSA growth after 5 minutes but failed to completely suppress MRSE. Pseudomonas and Klebsiella required 3-minute CHX exposure and 5-minute PVI exposure for complete clearance. Eradication of enterococci (VRE positive and negative) was not achieved at 10 minutes of CHX or PVI exposure. Comparison of PVI vs CHX showed a benefit for PVI in MRSA (P < .01) and MRSE (P < .001) eradication at 3 minutes. CONCLUSIONS: Current recommendations of 3 minutes of antisepsis prior to intraocular surgery are not sufficient for complete eradication of MDR organisms from the ocular surface. A bespoke approach is suggested to patients at risk for carriage of MDR organisms to minimize the risk of endophthalmitis.

5-year longitudinal study of clinical and patient-reported outcomes in acute anterior uveitis.

INTRODUCTION: Acute anterior uveitis (AAU) is a frequently encountered form of uveitis, most commonly an immune-mediated condition associated with the HLA-B27 gene with or without spondyloarthritis, or idiopathic in nature. This study's aim was to measure clinical and patient-reported outcomes 5 years after the first episode of immune-mediated AAU. METHODS: This is a longitudinal observational study. Ninety-six patients who underwent evaluation at the time of presentation with their first episode of AAU were invited to return for evaluation 5 years later. Standardised ocular history, clinical examination and quality of life (QOL) assessment with the Short Form 36 (SF-36) and the Vision Core Measure 1 (VCM 1) questionnaire were completed and analysed. RESULTS: Fifty-four patients (56%) returned for subsequent assessment. Physical function was the only sub scale domain of the SF-36 that had significantly deteriorated over the 5 years since the first episode of AAU (45.95 vs. 49.37, p = 0.003). Only 7.4% (n = 4) of patients expressed "more than a little concern" regarding their vision, reflected by a VCM1 score of 2.0 or more. At 5 years, the mean best corrected visual acuity (BCVA) of eyes affected by AAU was LogMAR 0.02 and only 3% (n = 2) of eyes had a BCVA of less than Logmar of 0.3. Five affected eyes (8%) had developed cataract and no patients had developed glaucoma by the 5 year review. CONCLUSIONS: This study demonstrates that immune-mediated AAU has an excellent 5 year prognosis with minimal impact on patients' health and vision-related quality of life.

Paediatric cataract in the uveitis setting.

BACKGROUND/AIMS: Cataract formation is common in uveitis and is visually more threatening in the paediatric cohort due to the risk of amblyopia. In addition, paediatric uveitis can often be difficult to manage. We report our experience with IOL placement in cataract surgery in the setting of paediatric uveitis. METHODS: This non-comparative, retrospective interventional case series examined our cases of paediatric cataract occurring in patients with uveitis from 2003 to 2016. Parameters examined included visual acuity (VA), underlying diagnosis, immunosuppression status, intra-operative complications and requirement for further surgery. RESULTS: In total, 10 eyes of seven patients were identified. The mean age at diagnosis of uveitis was 7.7 years (range 5.2-14 years) with onset of cataract at a mean of 29.3 months later (range 0-66 months). Three cases were bilateral and four cases were unilateral. Final visual outcomes were excellent with 80% showing improvement in VA achieving greater than 6/9.5 (p < 0.05). These patients had significant co-morbidities with concurrent glaucoma, band keratopathy and cystoid macular oedema. Uveitis was quiet for a minimum of 6 months in all cases prior to surgery with augmentation of immunosuppression pre-operatively as well as intra-operative local or intra-venous steroids. Tight post-operative care was necessary as 80% developed further flare-up of uveitis requiring increased immunosuppression and surgical interventions to manage their uveitis. CONCLUSION: Paediatric uveitis patients who develop cataract can have good visual outcomes with IOL insertion at the time of surgery when there is aggressive control of uveitis in the pre, peri and post-operative period.

A Case Series of Intramuscular Orbital Choristomatous Cysts.

Intramuscular choristomatous cystic lesions within the orbit are rare but may require removal due to their volume effects. The authors describe 2 such cases of intramuscular cysts in adult patients. Both patients presented with symptoms due to local mass effects with swelling, proptosis and diplopia. Imaging confirmed cystic intramuscular masses. Surgical removal involved intraoperative decompression of the cyst to aid dissection with excision of some muscle fibers. Histology confirmed a keratinized stratified squamous lined dermoid cyst within lateral rectus for case 1 and stratified squamous non-keratinized conjunctival epithelium (simple primary conjunctival cyst) within inferior oblique for case 2.

Novel gene targets for miRNA146a and miRNA155 in anterior uveitis.

BACKGROUND/AIMS: Anterior uveitis (AU) is the most common form of intraocular inflammation. MicroRNAs (miRNA) are small, non-coding RNAs functioning as post-transcriptional repressors of gene expression. Knowledge of miRNAs can implicate specific genes and pathogenic signalling pathways in disease. This study examines miRNA expression, function and target genes in AU pathogenesis. METHODS: AU and healthy control (HC) peripheral blood mononuclear cells (PBMC) were initially screened for expression of five miRNAs by real-time PCR. Regulation of the aberrantly expressed miRNAs by TLR1/2, TLR3, TLR4, IL1ß and TNFα was quantified by real-time PCR and paired cytokine outputs measured by ELISA. Functional effects of miRNA overexpression using transfected THP1 cells examined IL6, IL8, IL10 and IL1ß cytokine outputs by ELISA. Target genes were identified using TargetScan online computational algorithm and relevant targets verified by cloning of the 3'UTR and luciferase reporter gene assays. RESULTS: Increased expression of miRNA146a (p<0.01), miRNA155 (p<0.05) and miRNA125a5p (p<0.01) was demonstrated in AU PBMC compared with HC. miRNA155 was increased following TLR1/2 (p<0.05) and TLR4 (p<0.05) stimulation and miRNA146a increased in response to IL1ß (p<0.05). In a proinflammatory environment, miRNA155 overexpression in THP1 cells yielded increased cytokine output whereas miRNA146a overexpression showed decreased cytokine output. CD80, PRKCE and VASN were confirmed as novel targets for miRNA146a and SMAD2, TYRP1 and FBXO22 for miRNA155. CONCLUSION: This study identifies overexpression of proinflammatory miRNA155, regulatory miRNA146a and miRNA125a-5p in AU. CD80, PRKCE and VASN are novel miRNA146a targets and SMAD2, TYRP1 and FBXO22 are novel targets for miRNA155.

Serial Spectral Domain Ocular Coherence Tomography Measurement of Outer Nuclear Layer Thickness in Rhegmatogenous Retinal Detachment Repair.

INTRODUCTION: The outer nuclear layer (ONL) contains mostly photoreceptor bodies and reduced post-operative ONL thickness (ONLT) is associated with poor visual outcome in rhegmatogenous retinal detachment (RRD). METHODS: Spectral domain ocular coherence tomography was recorded before and 6 weeks following RRD repair in 27 consecutive patients. RESULTS: The repair consisted of combined 20-guage pars plana vitrectomy (PPV) and encirclement in 66.7% of cases, 22.2% underwent PPV alone and 11.1% scleral buckle alone. The mean (± standard deviation) ONLT was reduced in detached retinae (94.1 ± 31.1 microns) compared to the unaffected eye (119.5 ± 26.8 microns). Postoperatively the ONLT increased to 103.3 (± 32.8) microns. ONLT at presentation in the affected eye is significantly and positively correlated with the final ONLT in the affected eye (Pearson's correlation: r = 0.80, p = 0.029). CONCLUSION: The finding that ONLT is reduced in detached retinae carries implications for visual prognosis.