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.

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.

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.

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.

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.

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.

Clinical and Radiological Features of Intramuscular Orbital Amyloidosis: A Case Series and Literature Review.

PURPOSE: Orbital amyloidosis of the extraocular muscles (EOMs) is a rare condition, and its clinicoradiological features are not well elucidated. This study describes the characteristic clinical signs, MRI features, and potential treatment options. METHODS: Retrospective multicenter case series and literature review of EOM amyloidosis. RESULTS: Five cases were identified for inclusion. Common clinical findings were diplopia, ophthalmoplegia, and proptosis. Systemic amyloidosis was more likely to present with multiple muscle involvement, but no particular pattern was observed with localized disease. On MRI, amyloid deposition was characterized as a heterogeneous intramuscular mass with T2 hypointensity and post contrast enhancement. Management is dependent on the extent of disease and functional impairment; options include surgical debulking and radiation therapy. CONCLUSION: EOM amyloidosis is uncommon. The combination of clinical and radiologic findings described in this study should lead to its clinical suspicion.

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.

Hughes flap in the management of lower lid retraction.

PURPOSE: We present a retrospective case series on the use of Hughes flap in managing acquired cicatricial lower lid retraction. METHODS: This was a multicentre, retrospective case series. Data was collected from medical records across different sites within Australia (Adelaide, Melbourne, and Sydney) and New Zealand (Hamilton). RESULTS: Fourteen patients were identified. The aetiology of cicatricial lower lid retraction included previous lid lesion excision and reconstruction, eyelid trauma, orbital fracture repair, orbital radiotherapy, and lateral canthal dystopia from previous lid surgeries. 4/14 (29%) cases had undergone other surgery to correct the retraction prior to the Hughes flap. Pre-operative lagophthalmos due to lower lid retraction was noted in 11/14 (79%) cases with a median 2 mm (range: 1-5 mm). Exposure keratopathy was present in 7/14 (50%) cases. There were no peri-operative complications during Hughes flap reconstruction. One patient had post-operative upper eyelid retraction that did not require any further intervention. One patient had persistent lagophthalmos and exposure keratopathy that is being managed conservatively. One patient had wound dehiscence and further lid retraction following flap division, which required further surgery. Median length of follow-up was 15 months (range: 0.5-84 months). At final review, improvement or resolution of symptoms was seen in 13/14 (93%) cases. CONCLUSIONS: A Hughes flap is an effective surgical technique for the management of cicatricial lower lid retraction.

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.

Histopathologic Findings in Idiopathic Orbital Myositis.

PURPOSE: To report the histologic and clinical features of idiopathic orbital myositis (IOM) patients who underwent extraocular muscle (EOM) biopsy over 20 years, to provide the first methodical reference to the expected histopathologic findings, and to discuss the histopathologic differences from common differential diagnoses. DESIGN: Cohort study. PARTICIPANTS: All patients with a diagnosis of IOM who underwent EOM biopsy from 2000 through 2019 were included. Patients who had a different final diagnosis were excluded. METHODS: Tissue samples of EOM and medical records of all participants were reviewed. MAIN OUTCOME MEASURES: Histopathologic features of muscle biopsy, including tissue morphologic features and cellular composition. RESULTS: Thirteen patients met both inclusion and exclusion criteria, and their tissue samples were revisited. Nine patients showed histopathologic findings that suggested a conclusive diagnosis of IOM, and the study focused on them. The average age at presentation was 49 years, and 66.7% of patients were women. The most commonly biopsied EOM was the medial rectus (44.4%). The most common indications for biopsy were nonresolving orbital disease with inadequate response to corticosteroids (44.4%) or a high suspicion of malignancy because of known pre-existing systemic malignancy or the presence of an atypical orbital mass in addition to enlarged muscles (44.4%). The histopathologic findings that suggested a diagnosis of IOM were splaying of muscle fibers by inflammatory infiltrates (n = 9) and mild fibrosis (n = 8) in the endomysium or replacing muscle fibers, with no granulomas or vasculitis. The inflammatory infiltrates identified were of chronic inflammatory cells, consisting of lymphocytes (n = 9), plasma cells (n = 6), and histiocytes (n = 6). Other less commonly identified cells were eosinophils (n = 4), polymorphonuclears (n = 1), and giant cells (n = 1). Muscle fiber degeneration or regeneration was evident in 5 patients. Four patients from the initial cohort showed inconclusive histologic findings on revision and were reassigned as suspected IOM. CONCLUSIONS: The histopathologic features of involved muscles in IOM resemble those seen in idiopathic orbital inflammation and differ from those seen in common differential diagnoses. Extraocular muscle biopsy should be strongly considered whenever the presentation of orbital myositis is not typical or when significant underlying conditions are a possibility.

Extraocular Muscle Enlargement and Proptosis Associated with Chronic Inflammatory Demyelinating Polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an inflammatory neuropathy, which commonly causes peripheral neuropathy. It has rarely been associated with cranial nerve hypertrophy and neuro-ophthalmic manifestations. Proptosis secondary to cranial nerve hypertrophy has been reported in association with CIDP. The authors present a case of a 67-year-old man with CIDP who presented with bilateral proptosis, strabismus, and episodes of globe subluxation. The proptosis was mainly attributed to significant enlargement of the extraocular muscles, in addition to bilateral enlargement of the trigeminal nerves. There has been no published case of CIDP with associated enlargement of extraocular muscles without a history of underlying hyperthyroidism, inflammation, or malignancy. This may represent a new clinical finding in CIDP and adds to the limited literature on the neuro-ophthalmic and orbital associations of CIDP. The proptosis was managed with an uncomplicated bilateral orbital decompression.

Spheno-orbital Lesions-A Major Review of Nonmeningioma Causes.

PURPOSE: To review the imaging features of the broad range of nonmeningioma lesions of the greater wing of the sphenoid (GWS) bone and adjacent orbit to assist clinicians in differentiating these lesions from each other and from the most common lesion of the GWS and adjacent orbit, meningioma. METHODS: We reviewed 32 cases of spheno-orbital lesions involving the GWS from our own practice, as well as 109 published cases (total 141), with emphasis on available imaging features on computerized tomography (CT) and MRI. Features that might assist in differentiating meningioma from its mimics were analyzed for each lesion, including the presence of an osteoblastic or hyperostotic response, bone erosion or osteolysis, homogeneous hypo- or hyperintensity on T2-weighted MRI, leptomeningeal involvement, and the absence of a "dural tail" on contrast-enhanced MRI. The clinical and imaging features were also briefly summarized for each diagnostic group. RESULTS: The largest diagnostic group was metastasis (67 cases, 47.5%). The most useful imaging features that helped differentiate meningioma from its mimics were the presence of bone erosion and the absence of a "dural tail." Other features were helpful in a small minority of cases only. Metastatic prostate cancer was the largest single group (21 cases), and 18 (85.7%) of these were osteoblastic and most closely mimicked meningioma. Prostate cancer patients were generally older than males with GWS meningioma. Almost all other (44/46, or 95.7%) metastatic lesions showed evidence of bone erosion. Almost half (30 of 61, 49.2%) of patients with metastasis presented without a known diagnosis of malignancy. Among children 16 years of age and less, Langerhans cell histiocytosis (10 cases), dermoid cyst (5), and Ewing's sarcoma (5) were the most common diagnoses. CONCLUSIONS: A combination of a careful history and both CT and MRI gives information, which can best guide the management of patients with spheno-orbital lesions. Metastatic prostate cancer to the GWS most closely mimics GWS meningioma but can in most cases be differentiated on clinical and imaging features. Older males with hyperostotic lesions of the GWS should be investigated for prostate cancer. Other metastatic lesions and primary tumors of the GWS, as well as benign and structural lesions can readily be differentiated from meningioma on clinical and imaging features.

Primary Nasocutaneous Fistulae in Granulomatosis With Polyangiitis: A Case Series and Literature Review.

PURPOSE: To report the presentation, investigation, management, and outcomes of primary nasocutaneous fistulae in granulomatosis with polyangiitis and review the literature of this complication. METHODS: Retrospective series of 5 patients with granulomatosis with polyangiitis and nasocutaneous fistulae and the medical and surgical management strategies employed. RESULTS: Two cases presented with fistulae as the primary symptom of their systemic disease. Systemic management of granulomatosis with polyangiitis is a primary concern and two cases had inadequate control at presentation such that surgical intervention was deferred. Of the other 3 cases, 2 were closed using a median forehead flap and the other with an orbicularis advancement flap. An endonasal approach was attempted in 1 case but was unsuccessful due to the friable nature of the nasal mucosal tissue. CONCLUSIONS: Nasocutaneous fistulae should alert the clinician to underlying granulomatosis with polyangiitis, which may be a presentation of this condition. Systemic management of disease is often a barrier to surgical management. Myocutaneous flaps may be the most reliable management option to achieve closure.

Diplopia Following the Insertion of a Canalicular Bypass Tube: Etiology, Risk Factors, Management, and Outcomes.

PURPOSE: To report the etiology, management, and possible risk factors for diplopia after canalicular bypass surgery. METHODS: A multicenter retrospective, noncomparative case series of patients who developed diplopia following canalicular bypass surgery were assessed. RESULTS: Twenty-four cases of diplopia were identified across 12 institutions. Tubes were inserted as a primary procedure with external dacryocystorhinostomy (DCR) (1; 4%) or without DCR (10; 42%) or as a secondary procedure after external (8; 33%) or endonasal (5; 21%) DCR. Factors predisposing to local damage were noted in 17 (71%): these factors included preexisting autoimmune/inflammatory condition (7 cases), medial canthal tumor resection (5 cases), preoperative radiotherapy (2 cases), 2 drug treatments (topical and systemic), and 1 local surgery. Horizontal diplopia was due to restriction of abduction and first noted at a median of 3.5 months (mean: 17.8 months, range: 1 day to 112 months) and persisted in 23 (96%) cases with a mean restriction of -2, affecting primary gaze in 4 patients and activities of daily living in 13 (42%). Seventeen patients received various treatments: 10 were operated on resulting in cure in 1 and improvement in 9. A stable degree of diplopia persisted in all but one patient. CONCLUSIONS: Restriction of abduction causing horizontal diplopia is a rare complication with canalicular bypass surgery and a notably high proportion occurred after tube placement without DCR; carunculectomy was not ubiquitous. Although in some the diplopia may be improved with intervention, the chance of cure is low. This complication should probably be included during informed consent for canalicular bypass tubes.

Efficacy of porcine acellular dermal matrix in the management of lower eyelid retraction: case series and review of the literature.

PURPOSE: To investigate the efficacy of Permacol™, a decellularized porcine dermal-derived membrane, as a spacer in the management of lower eyelid retraction. The efficacy of sizing and insertion was investigated, as well as complications. The literature was also reviewed to compare this material with other porcine-derived grafts in use for the management of lower eyelid retraction. METHODS: This was a retrospective case series observing all patients who received lower eyelid Permacol implants by the two senior authors (AAM, TGH) for the management of lower eyelid retraction. Patient demographics, indications for surgery, graft size, degree of postoperative lid advancement, and complications were reviewed. RESULTS: A total of 12 patients (16 eyelids) received Permacol implants for correction of lower eyelid retraction during the study period of 18 months (January 2015 to July 2017). Ten procedures were related to thyroid eye disease, 3 for reconstruction, 2 postcosmetic lower lid blepharoplasty, and one acquired anophthalmic socket. The average preoperative inferior scleral show (ISS) was 1.74 mm, and the average postoperative ISS was 0.82 mm. There was a mean lower eyelid elevation of 0.91 mm (p < 0.005, Wilcoxon signed rank test) and mean ratio of graft height:preoperative ISS was 3.8:1 over a median of 8 months follow-up. CONCLUSIONS: Permacol is a safe and effective alternative to autologous tissues for use as a spacer in patients with lower eyelid retraction of varying etiologies. It does undergo some resorption with time, however this can be predicted and incorporated into surgical planning; we recommend an implant height:ISS ratio of 4:1.

Orbital Myositis: A Comprehensive Review and Reclassification.

PURPOSE: Orbital myositis occurs in typical and atypical forms. This review summarizes and updates the current state of knowledge of all forms of inflammation affecting extraocular muscle, excluding thyroid-associated orbitopathy. METHODS: A comprehensive literature review of orbital myositis was performed. RESULTS: Orbital myositis typically occurs in an idiopathic acute form, with painful diplopia due to inflammation in 1 or more extraocular muscles of young adult females, which usually responds to a course of oral corticosteroids. Atypical forms include idiopathic chronic or recurrent orbital myositis, and myositis related to systemic autoimmune, inflammatory, and infective conditions. The commonest associated autoimmune condition is inflammatory bowel disease. Immunoglobulin G4-related ophthalmic disease often affects extraocular muscle. Drug reactions and rarely paraneoplastic disease may also cause extraocular muscle inflammation. CONCLUSIONS: Orbital myositis occurs in a typical acute steroid responsive form, but atypical forms related to specific autoimmune and inflammatory conditions are increasingly recognized. Orbital myositis has many similarities to uveitis and would benefit from a systematic approach to nomenclature, diagnosis, and treatment.

Perioperative Prophylactic Antibiotics in 1,250 Orbital Surgeries.

PURPOSE: Intravenous antibiotic prophylaxis is used for many clean-contaminated surgeries or clean surgeries with an implant, but its value for clean orbital surgery has not been determined. This study investigated infection risks and adverse effects related to antibiotics in patients undergoing orbital surgery. METHODS: A prospective, nonrandomized comparative case series of all patients undergoing orbital surgery with participating surgeons between October 1, 2013, and March 1, 2015. Types of surgery, antibiotic regimens, corticosteroid use, antibiotic side effects, and surgical site infections (SSIs) were entered into an electronic database and subsequently analyzed. Cases in which patients received postoperative oral antibiotics were analyzed separately. RESULTS: Of 1,250 consecutive orbital surgeries, 1,225 met inclusion criteria. A total of 1208 patients were included in the primary analysis: 603 received no antibiotic prophylaxis (group A), and 605 received a single dose of intravenous antibiotic (group B). Five patients (0.42%) developed an SSI, 3 in group A and 2 in group B. The difference in SSI rates was not statistically significant between the 2 groups (p = 0.66). Antibiotic prophylaxis, alloplastic implants, paranasal sinus entry, and corticosteroid use were not associated with differences in SSI rates. All SSIs resolved on a single course of oral antibiotics; an implant was removed in 1 case. There were no complications associated with a single dose of intravenous prophylaxis. However, 12% of 17 patients (group C) who received 1 week of oral postoperative prophylactic antibiotics developed antibiotic-related complications (diarrhea, renal injury), yielding a number needed to harm of 8.5. CONCLUSIONS: In this large series, antibiotic prophylaxis does not appear to have reduced the already low incidence of SSI following orbital surgery. Given the detriments of systemic antibiotics, the rarity of infections related to orbital surgery, and the efficacy of treating such infections should they occur, patients undergoing orbital surgery should be educated to the early symptoms of postoperative infection and followed closely, but do not routinely require perioperative antibiotics.

Multiple myeloma manifesting as an ocular salmon patch - a case report.

Multiple myeloma (MM) is the second most prevalent hematologic malignancy after non-Hodgkin lymphoma and is currently considered incurable. Clinical ophthalmic manifestations of MM are rare but at the same time diverse. Ocular surface manifestations of multiple myeloma are uncommon. Conjunctival 'salmon patch' is a typical ocular surface ophthalmological sign with a distinct set of differential diagnoses, including most often ocular adnexal lymphoma. This case report presents a 33-year-old female with a relapse of MM manifesting as a conjunctival 'salmon patch'. The patient initially responded well to medical management including high dose melphalan supported by a third autologous stem cell transplantation (ASCT) and did not require further surgical excision of the ocular lesion. It is suggested that MM should be included in the differential diagnosis of 'salmon patch' conjunctival lesions.