Orbital cavernous venous malformation shrinkage during fractionated stereotactic radiotherapy contributing to the development of radiation retinopathy.

BACKGROUND: Posterior movement of ocular tissue secondary to orbital cavernous venous malformation shrinkage from fractionated stereotactic radiotherapy can allow healthy structures to move into the radiation field during treatment. This may carry an increased risk of radiation-induced retinopathy. METHODS: We present a case of a young female whose radiotherapy treatment for an orbital cavernous venous malformation resulted in a 3 mm reduction in proptosis and subsequent retinopathy. RESULTS: The severity of the patient's radiation-induced radiotherapy exceeded expectations. The venous malformation shrinkage during treatment and ensuing posterior movement of the globe suggested an increased involvement of ocular tissue in the radiation field, prompting consideration of interval neuroimaging and tumour mapping. CONCLUSIONS: We describe and suggest a protocol of onboard neuroimaging during the radiation therapy course to better target tumour volumes and minimise collateral tissue damage. To our knowledge, this has not been previously described in the ophthalmic literature.

Presumed Lacrimal Gland Pleomorphic Adenoma With Extensive Ossification and Necrosis.

Calcification within pleomorphic adenomas of the lacrimal gland is well recognized but uncommon, being seen more readily in lacrimal gland carcinomas. Bony formation, ossification, in pleomorphic adenomas of the lacrimal glands is even rarer. Together with extensive sclerosis, or "coagulative necrosis," ossification and necrosis should alert the clinician to the risk of malignant transformation. However, both can mimic carcinomatous change, leading to misinterpretation of malignancy in an otherwise benign lacrimal gland neoplasm. We present 2 case reports of patients with clinically presumed pleomorphic adenomas of the lacrimal gland whose histopathology demonstrated lacrimal gland ossification and necrosis without features of malignancy or invasive disease.

Magnetic Resonance Imaging of Idiopathic Orbital Myositis.

PURPOSE: To characterize the qualitative and quantitative features of idiopathic orbital myositis (IOM) on MRI. METHODS: This was a multicenter retrospective study of patients with active IOM with MRI. Patients with incomplete clinical records, poor-quality or interval scans without active myositis, and specific orbital myositis (i.e., orbital myositis secondary to an identified condition) were excluded. An enlargement ratio was calculated by dividing the diameters of the affected extraocular muscle (EOM) by the contralateral unaffected EOM. RESULTS: Twenty-four patients (mean age: 44.4 ± 17.8 years-old, male: 11) between 2011 and 2022 were included. One case (4.2%) was pediatric (17 years old), and 6 cases presented with recurrence. Active IOM was characterized by fusiform EOM enlargement, high T2 signal, and contrast enhancement. Average maximal EOM diameters ranged from 4.6 to 7.7 mm (enlargement ratio: 1.4-2.2). Eighteen (75%) patients had single EOM involvement, most commonly the medial rectus. Other ipsilateral structures affected included focal orbital fat (16/24, 66.7%) and lacrimal gland (8/24, 33.3%). Contralateral changes in the EOM and/or lacrimal gland were observed in 7 patients (29.2%). Patients presenting with recurrence were likely to develop ongoing recurrent episodes (p = 0.003). CONCLUSIONS: Various radiological patterns of involvement described including EOM enlargement, contrast enhancement, abnormal signal, and involvement of other orbital structures are indicative of active IOM. IOM remains a heterogeneous spectrum of acute and chronic clinico-radiological presentations. Inflammation may involve other ipsilateral or contralateral orbital structures or may be bilateral despite presenting clinically as unilateral disease. Quantitative measurements may have utility in differentiating IOM from other causes of orbital myositis.

Ocular manifestations of obstructive sleep apnea: a systematic review and meta-analysis.

BACKGROUND: The association of obstructive sleep apnea (OSA) with development of eye diseases is unclear. This current systematic review and meta-analysis attempts to summarize and analyze associations between OSA and ocular disorders in the literature. METHODS: PubMed, EMBASE, Google Scholar, Web Of Science, and Scopus databases were searched from 1901 to July 2022 in accordance with the Preferred Reporting in Systematic Review & Meta-Analysis (PRISMA). Our primary outcome assessed the association between OSA and the odds of developing floppy eyelid syndrome (FES), glaucoma, non-arteritic anterior ischemic optic neuropathy (NAION), retinal vein occlusion (RVO), keratoconus (KC), idiopathic intracranial hypertension (IIH), age-related macular degeneration (AMD), and central serous chorioretinopathy (CSR) through odds ratio calculated at the 95% confidence interval. RESULTS: Forty-nine studies were included for systematic review and meta-analysis. The pooled OR estimate was highest for NAION [3.98 (95% CI 2.38, 6.66)], followed by FES [3.68 (95% CI 2.18, 6.20)], RVO [2.71(95% CI 1.83, 4.00)], CSR [2.28 (95% CI 0.65, 7.97)], KC [1.87 (95% CI 1.16, 2.99)], glaucoma [1.49 (95% CI 1.16, 1.91)], IIH [1.29 (95% CI 0.33, 5.01)], and AMD [0.92 [95% CI 0.24, 3.58] All observed associations were significant (p < 0.001) aside from IIH and AMD. CONCLUSION: OSA is significantly associated with NAION, FES, RVO, CSR, KC, and glaucoma. Clinicians should be informed of these associations so early recognition, diagnosis, and treatment of eye disorders can be addressed in at-risk groups, and early referral to ophthalmic services is made to prevent vision disturbances. Similarly, ophthalmologists seeing patients with any of these conditions should consider screening and referring patients for assessment of possible OSA.

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.

Histopathological Characteristics of Lacrimal Gland Prolapse.

PURPOSE: Lacrimal gland prolapse is an acquired clinical condition that can present as an upper eyelid mass. Patients may undergo lacrimal gland biopsy when there is diagnostic uncertainty. We aim to describe the histopathological features of this patient group. METHODS: Retrospective case series involving 11 patients. RESULTS: The mean age at presentation was 52.3 ± 16.2 years (range: 31-77 years) with 8 patients (72.3%) being female. The most common presenting symptom was a palpable mass (9; 81.8%) followed by dermatochalasis (4; 36.4%). Three cases (27.3%) were bilateral. Common imaging findings include lacrimal gland enlargement and visualization of the prolapse. All biopsies demonstrated features of mild chronic inflammation with preserved glandular structures. Ten patients (90.9%) underwent surgical intervention involving lacrimal gland pexy and 1 patient (9.1%) was elected for observation only. One patient required repeat surgery after 4 years due to recurrence of symptoms. At the last follow-up, all patients had stable disease or complete resolution of symptoms. CONCLUSION: We present a case series of patients diagnosed with lacrimal gland prolapse who underwent a biopsy during their workup. All biopsies demonstrated features of mild chronic inflammation (dacryoadenitis). All patients had stable disease or complete resolution of symptoms. This case series suggests that chronic inflammation is a common finding in patients with lacrimal gland prolapse but bears minimal clinical consequence.

Prevalence of Obstructive Sleep Apnea With Floppy Eyelid Syndrome: A Systematic Review and Meta-analysis.

PURPOSE: This study investigated the prevalence of obstructive sleep apnea (OSA) in floppy eyelid syndrome (FES) patients and evaluated the severity of OSA with FES prevalence. METHODS: Cochrane CENTRAL, Medline, Science Direct, Google Scholar, and PubMed databases were searched for studies on FES patients and its association with OSA syndrome, of any design, published from January 1, 1997, to January 1, 2022. A random-effects model that weighted the studies was used when there was heterogeneity between studies ( p < 0.10) and if I 2 values were more than 50%. All p values were 2-tailed and considered statistically significant if <0.05. RESULTS: A total of 12 studies comprising 511 patients were included in this meta-analysis. Of these, 368 were male (77.6%) and the average age was 55.10 years. The overall prevalence of OSA in FES patients was 57.1% (95% CI: 46.5-74.8%), M:F ratio was 48:1 (98% male), and 69.1% of patients received their OSA diagnosis at the time of the study. Of those with FES, tear film abnormalities were the most common ocular comorbidity (78.9%) followed by keratoconus (20.6%), glaucoma (9.8%), and lower eyelid ectropion (4.6%). Obesity was the most common systemic morbidity (43.7%) followed by hypertension (34.0%) and diabetes mellitus (17.9%). CONCLUSION: This meta-analysis demonstrates OSA is a common comorbidity in the FES population. Ophthalmologists are often the first to evaluate patients with FES, and considering this coincidence, routine screens for sleep apnea symptoms in at-risk FES patients should be undertaken. Large case-control studies are required to better elucidate the exact prevalence of OSA and other morbidities in patients with FES, and to better understand the etiology of FES.

IgG4-related ophthalmic disease in association with adult-onset asthma and periocular xanthogranuloma: a case report.

A 54-year-old male presented with a three-year history of bilateral upper eyelid and peri-orbital swelling and adult-onset asthma. Histopathology of a left orbital biopsy showed lymphoid follicles with foamy macrophages and Touton giant cells. Clinical, histological and radiological features were consistent with adult-onset asthma and periocular xanthogranuloma. Treatment with rituximab led to a complete clinical and radiological remission. Nine years later, his condition relapsed with a biopsy of the left orbit and lacrimal gland demonstrating features of IgG4-related disease and adult-onset asthma and periocular xanthogranuloma. Immunohistochemistry showed increased numbers of IgG4+ plasma cells (290 per high power field) and an elevated IgG4+/IgG+ plasma cell ratio of 480%. Involvement by both disorders in the orbit and ocular adnexa of a single patient has not previously been reported in the literature, to the best of our knowledge, and suggests a possible aetiologic or pathophysiologic association.

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.

Idiopathic Orbital Myositis Revisited.

PURPOSE OF REVIEW: To review current knowledge regarding idiopathic orbital myositis. RECENT FINDINGS: Recent publications have focused on possible causes of orbital myositis and the process to reach a diagnosis of idiopathic orbital myositis. With inflamed and enlarged extraocular muscles, features to distinguish between competing diagnostic possibilities are based on imaging in the context of history and clinical signs. Idiopathic orbital myositis is characterized by the clinical triad of acute onset of orbital pain exacerbated on eye movement, double vision, and redness or swelling of the eyelids or conjunctiva, along with the radiological finding of homogeneous, fusiform enlargement of one or more extraocular muscles. In atypical or inconclusive clinico-radiological findings for a diagnosis of idiopathic orbital myositis, or where the clinical behavior changes or fails to respond to corticosteroid treatment, a systemic and oncologic work-up and muscle biopsy are warranted to exclude specific local or systemic disease as cause of the inflamed and enlarged muscle. As our understanding of idiopathic orbital myositis evolves, the diagnostic focus is shifting toward earlier identification of underlying local or systemic disease through systemic work-up and muscle biopsy.

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.