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.

Pediatric Orbital Juvenile Xanthogranuloma: A Case Series and Review of the Literature.

PURPOSE: Juvenile xanthogranuloma (JXG) is a subtype of histiocytosis characterised histologically by foamy non-Langerhan cells with Touton giant cells. It typically manifests as a single self-limiting cutaneous nodule in the pediatric population. Orbital JXG is extremely rare, and its clinical course and management are not well understood or defined. Herein we present 3 cases of orbital JXG and provide a detailed literature review. METHODS: Review of 3 cases with orbital JXG and literature review of all published cases. RESULTS: Three presented cases demonstrate the heterogeneous clinical course of orbital JXG. Although centred around the use of steroids, there is neither robust evidence nor consensus on its management. The wider JXG literature is currently concentrated around the classification of JXG with respect to histiocytosis, especially the exclusion of extracutaneous JXG as separate diseases. This separation is based on clinical, histopathological, and molecular findings. It is unclear where orbital JXG best fits in this emerging classification of JXG. CONCLUSION: Our review of the cases and literature on orbital JXG show that it may manifest with variable clinical course and its molecular pathogenic mechanism may be different to that of the cutaneous JXG.

Interrater reliability of photographic assessment of thyroid eye disease using the VISA classification.

PURPOSE: To determine the interrater reliability (IRR) of thyroid eye disease (TED) photographic assessment using the VISA classification. To assess whether a VISA grading atlas improves ophthalmology trainees' performance in photographic assessment of TED. METHODS: A prospective, partially randomized, international study conducted from September 2021 to May 2022. Online study invitation was emailed to a volunteer sample group of 68 ophthalmology college accredited consultants and trainees, and 6 were excluded from the study. Participants were asked to score 10 patient photographs of TED using only the inflammation and motility restriction components of the VISA classification. IRR was compared between groups of practitioners by their level of experience. A clinical activity grading atlas was randomized to 50% of the ophthalmology trainees. RESULTS: Overall rater ICC was 0.96 for inflammation and 0.99 for motility restriction. No statistically significant difference in IRR between rater groups was identified. Trainees with a grading atlas had the highest IRR for inflammation (ICC = 0.95). Each subcomponent of the inflammation and motility restriction components of VISA classification had an ICC considered good to excellent. The mean overall rater score was 4.6/9 for inflammation and 3.5/12 for motility restriction. For motility restriction there was a reduced mean score variance among all raters when scoring photographs with more severe motility restriction. CONCLUSION: IRR using the inflammation and motility restriction components of the VISA classification was excellent. A VISA grading atlas improved trainee performance in grading inflammation.

Odontogenic Lemierre's syndrome with septic superior ophthalmic vein and cavernous sinus thrombophlebitis complicated by blindness and ophthalmoplegia.

Lemierre's syndrome is a triad consisting of oropharyngeal infection, internal jugular vein thrombophlebitis, and systemic embolisation typically involving lung and brain. Orbital involvement in this life-threatening condition is rare but potentially blinding and may be an indicator of intracranial involvement. We describe a case of odontogenic Lemierre's syndrome complicated by extensive orbital and intracranial septic venous thrombosis, with optic and cranial neuropathy resulting in monocular blindness and ophthalmoplegia. A multidisciplinary approach with abscess drainage, antibiotic and antithrombotic therapy, and close radiological monitoring was critical for preserving contralateral vision and neurological function.

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.

Primary Extradural Ectopic Orbital Meningioma in a Child.

The authors describe a rare case of primary extradural ectopic meningioma of the orbit in a 13-year-old female managed with surgical excision, adding to the very limited literature of this diagnosis in children. This is the second case to show a radiological abnormality in the gyrus rectus and olfactory bulb; however, in our case, there was no radiological evidence of connection seen.

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.

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.

Orbital giant cell myositis is an unusual and potentially lethal cause of bilateral ophthalmoplegia - A case report and literature review.

We present a case of orbital giant cell myositis (OGCM), presenting with bilateral subacute progressive ophthalmoplegia and optic nerve dysfunction. An early extraocular muscle biopsy confirmed the diagnosis and guided appropriate management. Comprehensive investigation excluded any underlying systemic disease, including myocarditis. Twenty two months after presentation, the patient remains well on azathioprine with complete resolution of orbital signs.

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.

High nasolacrimal sac-duct junction anatomical variation - retrospective review of dacryocystography images.

PURPOSE: In assessing epiphora, dacryocystography (DCG) is often performed to determine the presence of any structural anomaly of the nasolacrimal drainage apparatus. We describe an anatomical variation of the nasolacrimal duct, termed high sac-duct junction (HSDJ) morphology, that is seen on DCG, which could lead to more difficult, and possibly unsuccessful, intubation of the nasolacrimal duct (NLD). METHODS: This was a multi-center, retrospective, observational case series. Seven hundred and thirty-four DCGs were included in this study, of which 45 were pediatric images. DCG images underwent a blinded review by an oculoplastic surgeon for the presence of HSDJ morphology. The direct extension of the NLD from the inferior border of the lacrimal sac is commonly regarded as a normal morphology. HSDJ was defined as NLD originating from the inferior aspect of the medial wall of the lacrimal sac. RESULTS: HSDJ was seen in 28/689 (4.1%) adult scans and 7/45 (15.6%) pediatric scans. In total, there were 35/734 (4.8%) DCGs demonstrating lacrimal sacs with HSDJ. CONCLUSIONS: We report the prevalence of an anatomical variant of the lacrimal sac and NLD, observed on DCG, in a population of patients with epiphora, where the NLD originates from the inferior aspect of the medial wall of the lacrimal sac. Knowledge of this anatomic variant may have management implications for patients with epiphora as it may make nasolacrimal intubation more technically difficult. Further studies are needed to correlate this anatomic variation with symptoms and management outcomes.

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.

Paediatric orbital conjunctival epithelial cyst with positive asialotransferrin.

Conjunctival orbital cysts are rare; they are typically either conjunctival dermoid or conjunctival epithelial cysts - congenital or acquired (inclusion). We describe the case of a 15-month-old girl presenting with strabismus and proptosis who had a retrobulbar intraconal cystic lesion displacing the optic nerve, with an adjacent middle cranial fossa anomaly. Aspiration of the orbital cyst tested positive for asialotransferrin, raising the suspicion of a direct communication with cerebrospinal fluid (CSF). Subsequent fine cut CT scanning disproved any connection with the intracranial space, and the cyst was excised complete and intact. Histopathology showed a conjunctival epithelial cyst. To our knowledge, this is the first case report in the literature of an asialotransferrin positive pediatric orbital conjunctival epithelial cyst. It is of clinical relevance as it explores the possibility of either a false positive asialotransferrin or potentially a prior developmental communication with the subarachnoid space. These two diagnostic possibilities are discussed.

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.

Spontaneous Orbital Hemorrhage Related to the Extraocular Muscles.

PURPOSE: The authors present the largest series of patients to date of spontaneous hemorrhage in relation to extraocular muscles (EOMs). METHODS: Observational retrospective case series. Institutional review board approval was obtained. RESULTS: Eighteen patients with a mean age of 71 years presented with 20 episodes of spontaneous orbital hemorrhage closely related to an EOM. Most patients woke with unilateral proptosis, pain, and diplopia. Vision was impaired in 25%. Imaging identified a characteristic well-circumscribed hematoma arising within the muscle sheath or intermuscular septum. Three episodes required surgical drainage. All patients had good recovery of vision. DISCUSSION: The authors describe a distinct clinical entity of idiopathic hemorrhages related to EOMs. No patient had an underlying vascular malformation or other lesion. The authors demonstrate that a proportion of these patients have vascular risk factors, namely, hypertension, hyperlipidemia, and antiplatelet use. This study suggests that inferior rectus is the most commonly affected EOM and shows that it is the muscle sheath or intermuscular septum of inferior rectus that is involved, rather than the muscle belly. Most patients can be managed conservatively with good visual outcomes. However, there were 3 exceptional cases that required surgical intervention. CONCLUSIONS: The findings that spontaneous orbital hemorrhages related to EOMs have certain predisposing factors, a characteristic radiological appearance, and a typically benign course will be helpful for clinicians in the management of this condition.

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.

Clinicopathological Features and Outcomes in Lymphoma of Extraocular Muscles.

PURPOSE: To describe the clinicopathological features and report the outcomes of treatment in lymphoma involving the extraocular muscles (EOM), the largest reported case-series. METHODS: A retrospective case series of patients with lymphoma involving the EOM from a single tertiary referral orbital center, between March 1992 and March 2018. Patients with other histopathologic diagnoses or who did not have an EOM biopsy were excluded. The main outcome measures were histologic evaluation and clinical follow-up including tumor response, recurrence, and survival. RESULTS: Twenty-five patients were included, 16 female; 9 male; median age 64.7 years (range 33.8-92.6 years). Unilateral involvement was present in 23 cases (92%). Lymphoma was found to be primary in 15 patients (60%), and any EOM could be affected by disease. Fourteen patients (56%) displayed impaired duction in the direction of the affected muscle. The commonest histologic type was extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue in 16 cases (64%) followed by follicular lymphoma in 3 cases (12%), diffuse large B-cell lymphoma in 2 cases (8%), gamma delta T-cell lymphoma in 2 cases (8%), small lymphocytic lymphom and lymphoplasmacytic lymphoma in 1 case each (4%). All 4 patients with high-grade lymphomas (diffuse large B-cell or T cell lymphoma) were found to have systemic disease. Patients were treated with combinations of radiotherapy, chemotherapy, or immunotherapy, with low toxicity. Two patients developed treatment-related complications. With a median follow-up of 26 months (range 3-108 months), there was only 1 recurrence, which was successfully salvaged with Rituximab, and no deaths due to lymphoma. CONCLUSIONS: Lymphoma of the EOM is a rare tumor with good response to treatment, few treatment-related complications and low risk of recurrence or mortality.A retrospective review of 25 patients with lymphoma of the extraocular muscles demonstrates excellent prognosis with a high rate of local remission (96%), 2 treatment-related complications, one recurrence, and no disease-related deaths.

Nasolacrimal Sac Diverticulum: A Case Series and Literature Review.

PURPOSE: To describe the demographics, presentation, treatment, and outcome in 10 cases of lacrimal sac diverticulum and to review the literature on this topic, and to determine the incidence of diverticula from a series of dacryocystograms. METHODS: Data were collected for all consecutive patients with lacrimal sac diverticulum seen between 2003 and 2015, including patient demographics, clinical presentation, imaging findings, treatment, and follow-up. A systematic literature review was performed for cases of lacrimal sac diverticulum. A retrospective review of 400 consecutive dacryocystograms was performed to determine the incidence of lacrimal sac diverticulum. RESULTS: Ten cases (9 patients) of lacrimal sac diverticulum are described, and a further 36 cases were discovered through a literature review. Epiphora, swelling, and dacryocystitis and/or diverticulitis were the most common presenting complaints. When all 46 cases are combined, lacrimal patency was demonstrated in 50% of cases. Dacryocystography diagnosed only 57% of patients with diverticulum, not helped by the addition of ultrasound or CT. The incidence of diverticulum was 3.2% on review of 400 consecutive dacryocystograms (1.6% per lacrimal system). Surgical management with dacryocystorhinostomy and/or diverticulum excision was effective in all 34 operated cases at resolving symptoms in the case series and literature review. CONCLUSIONS: Lacrimal sac diverticula present with epiphora, medial canthal swelling, dacryocystitis, and/or diverticulitis. Dacryocystography, despite being the most sensitive radiologic test, is only 59% sensitive, and adjunctive CT and ultrasound have limited use. Surgical management is recommended because conservative management often fails. Dacryocystorhinostomy, diverticulum excision, or a combination thereof appears to be equivalent in achieving resolution of symptoms.

Aetiology of acquired blepharoptosis in young adults.

PURPOSE: To identify the causes of blepharoptosis in young adults, and explore cases that do not fit into current diagnostic categories. METHODS: A retrospective cohort study of all patients aged 18-40 years ("young adults") with acquired blepharoptosis that presented to two specialist ocular plastics practices and a paediatric ophthalmologist over a period of up to 25 years. Each patient was classified according to diagnosis. Where the diagnosis was uncertain, the files were examined in detail to try and further establish a cause. RESULTS: A total of 266 young adult patients were included. The most common causes of acquired blepharoptosis were trauma-related (28.2%) and anophthalmic blepharoptosis (19.9%). In 12.4% of the cases, a definite diagnosis could not be made. Of these, one-third had a history of soft contact lens use, a possible etiologic factor. CONCLUSIONS: The cause of acquired blepharoptosis can usually be established by an appropriate history and examination, with additional diagnostic tests sometimes required. Nearly half of all young adult ptosis is related to trauma or acquired anophthalmos. Around one in eight young adults have blepharoptosis of unknown cause, a group warranting further study.