IgG4-related Orbital Disease Presenting as an Orbital Apex Lesion.

IgG4-related disease is an uncommon multisystem inflammatory disease characterized by tissue infiltration by IgG4 plasma cells, extensive fibrosis, and sclerosing inflammation. Diffuse orbital involvement extending to the orbital apex and cavernous sinus has been reported, but a solitary well-circumscribed lesion at the apex has not been previously reported. Herein, we report a unique case of IgG4-related orbital disease presenting as a solitary well-circumscribed orbital apex lesion causing subacute visual decline in a 45-year-old male. MRI demonstrated bilateral lacrimal gland and infraorbital nerve enlargement and an 18 × 7 mm left orbital apex lesion demonstrating homogeneous contrast enhancement and isointense signal on T2. He was initially treated with tapering systemic corticosteroid therapy with improvement in his vision; however, he experienced visual deterioration 3 months later with recurrent inflammation. He subsequently commenced high-dose systemic corticosteroid therapy and rituximab infusions with improvement of left visual acuity to 6/7.5 + 2.

Extraocular muscle enlargement in dysthyroid optic neuropathy.

OBJECTIVE: To investigate extraocular muscle volumes in thyroid eye disease (TED) patients with and without dysthyroid optic neuropathy (DON). DESIGN: Retrospective cohort study. PARTICIPANTS: TED patients who had computed tomography of the orbits. METHODS: The extraocular muscles were manually segmented in consecutive axial and coronal slices, and the volume was calculated by summing the areas in each slice and multiplying by the slice thickness. Data were collected on patient demographics, disease presentation, thyroid function tests, and antibody levels. RESULTS: Imaging from 200 orbits was evaluated. The medial rectus, lateral rectus, superior muscle group, inferior rectus, and superior oblique volumes were significantly greater in orbits with DON compared with TED orbits without DON (p < 0.01 for all). There was no significant difference in the inferior oblique muscle volume (p = 0.19). Increase in volume of the superior oblique muscle showed the highest odds for DON. Each 100 m3 increase in superior oblique, lateral rectus, inferior rectus, medial rectus, and superior muscle group volume was associated with 1.58, 1.25, 1.20, 1.16, and 1.14 times increased odds of DON. CONCLUSION: All extraocular muscle volumes except for the inferior oblique were significantly greater in DON patients. Superior oblique enlargement was associated with the highest odds of DON, suggesting superior oblique enlargement to be a novel marker of DON.

DCR for nasolacrimal duct stenosis may be less effective than for complete obstruction.

OBJECTIVES: To ascertain the success of endo-DCR in nasolacrimal duct stenosis (NLDS) versus nasolacrimal duct obstruction (NLDO). METHODS: Consecutive adult patients with epiphora attending a tertiary lacrimal clinic from February 2012 to February 2021 were reviewed. NLDS was diagnosed by patent lacrimal syringing and combined dacryocystography (NLD stenosis) and dacryoscintigraphy (post-sac delay) findings in all eyes. Cases with evidence of canalicular stenosis or other identifiable causes of epiphora were excluded. The epiphora resolution and improvement rates following endo-DCR were compared between NLDS and complete NLDO cases. RESULTS: DCRs in 24 NLDS (23 patients, 69.6% females, mean age 61.0 ± 17.07) and 58 NLDO (56 patients, 69.6% females, mean age 61.9 ± 17.4) were included. Resolution of epiphora was achieved in 10 (41.7% [95% CI 0.24-0.61]) of the NLDS cases compared to 40 (69.0% [95% CI 0.56-0.79]) in NLDO (p = 0.021). Improvement of epiphora (i.e., either improvement or resolution) was noted in 17 (70.8% [95% CI 0.51-0.85]) of NLDS and 53 (91.4% [95% CI 0.81-0.96]) of NLDO cases (p = 0.034). Three patients (12.5%) with NLDS had subsequent lacrimal procedures (one DCR revision, two Jones tube) at a median of 14 (range 11-21) months. 71.4% of the NLDS patients responded to a phone questionnaire at a median of 93 months postoperatively. Of these, 46.7% reported resolution or significant improvement, and 33.3% reported slight improvement. 64.3% said they would recommend DCR to others suffering from epiphora. CONCLUSION: Endo-DCR may benefit approximately 70% of patients with NLDS. The success of endo-DCR in complete NLDO may be higher.

Soft stop on syringing and probing may have a high false-positive rate in diagnosing pre-sac obstruction.

PURPOSE: To determine the diagnostic value of 'soft stops' encountered during lacrimal syringing and probing. METHODS: Single-center retrospective review. Adult patients with epiphora attending a tertiary lacrimal clinic from May 2010 to April 2021 were reviewed. Cases with evidence of soft stop encountered during lacrimal syringing/probing were included, and patients with possible canaliculitis or a history of lacrimal surgery were excluded. Findings of syringing/probing consistent with pre-sac obstruction were correlated with dacryocystography (DCG) and surgical findings. RESULTS: 53 (10.2%) canalicular systems had soft stops on syringing/probing and were included in the analysis. The mean age of the patients was 63.8 ± 15.6 (range 28-87) years, and 27 (65.9%) were females. Intraoperative examination findings were available for 27 of 30 cases that underwent lacrimal surgery and DCG was available for 40 systems. Pre-sac obstruction found on syringing/probing was confirmed in 40% and 37% of cases on DCG and surgery, respectively. The correlation between syringing/probing and DCG was stronger for canalicular than for common canalicular location (p = 0.016). Canalicular stenosis on syringing/probing manifested as pre-sac abnormality on DCG in 5/7 (71.4%) compared to 0/6 common canalicular stenosis cases (p = 0.021). Based on the surgical findings, the false-positive rate of a soft stop on syringing/probing was highest for common canalicular 'stenosis' (100%) and lowest for canalicular 'block' (45.5%; p = 0.093). Findings of pre-sac obstructions on DCG were confirmed in 85.7% of the cases intraoperatively (p = 0.035 compared to syringing/probing alone). CONCLUSIONS: Soft stops on probing showed poor correlation with DCG and surgical findings, particularly in common canalicular location.

Radiological analysis of orbital lymphoma histological subtypes.

PURPOSE: To characterise the radiological features of orbital lymphoma subtypes. METHODS: This was a multicentre, retrospective study to analyse radiological and clinical characteristics of orbital lymphoma by histological subtype across two sites within Australia and the United States. RESULTS: A total of eighty-eight patients were included. The most common subtypes were extranodal marginal zone lymphoma [EMZL] (48, 54.5%), follicular lymphoma [FL] (16, 18.2%), and diffuse large B-cell lymphoma [DLBCL] (15, 17.0%). Clinically, significant associations were found between DLBCL and vision change (p < .01), pain (p < .01), extraocular movement limitation (p = .01), and optic neuropathy (p = .01). Radiologically, there was no significant difference between the individual histopathological sub-groups with respect to bone destruction (p = .30), optic nerve involvement (p = .30) and diffuse appearance (p = .84). However, if categorised as either aggressive or indolent, aggressive lymphoma subtypes were significantly more likely to demonstrate globe indentation radiologically than indolent subtypes (Odds Ratio = 5.5, 95% CI: 1.3, 23.3; p = .02). CONCLUSIONS: DLBCL was significantly associated with vision change, pain, extraocular movement limitation and optic neuropathy clinically. Aggressive lymphoma subtypes were significantly more likely to demonstrate globe indentation radiologically. Otherwise, there were no significant differences between lymphoma subtypes and radiological findings on MRI and CT.

Optic nerve infiltration in systemic non Hodgkin lymphoma.

Optic nerve infiltration secondary to systemic non-Hodgkin lymphoma (NHL) is a rare phenomenon. We present a 55-year-old man with low-grade systemic NHL who initially presented with an isolated optic neuropathy and non-specific neurological symptoms. We further present a literature review of systemic NHL with radiological evidence of optic nerve infiltration. On magnetic resonance imaging, the characteristic features include optic nerve enhancement and enlargement, while leptomeningeal enhancement is uncommon. Cerebrospinal fluid analysis and optic nerve sheath biopsy can return false negative results, and when such investigations are inconclusive, biopsy of the optic nerve substance has a high diagnostic yield. Although rare, lymphomatous optic nerve infiltration must be considered in the differential diagnosis of a pale swollen optic disc.

Bilateral lacrimal gland disease: clinical features and outcomes.

BACKGROUND: Patients with bilateral lacrimal gland disease are a unique subset of patients where there is a paucity of literature. This presentation often represents systemic disease or malignancy and can cause diagnostic difficulties. We aim to describe the diagnoses and features of bilateral lacrimal gland disease. METHOD: Retrospective multi-centre case series involving 115 patients with bilateral lacrimal gland disease from 1995 to 2020. RESULTS: 115 patients were included. Their ages ranged from 9 to 85 (mean 47.3 years) with a female predominance (73, 63.5%). The most common category of diagnosis was inflammatory (69, 60%) followed by lymphoproliferative (23, 20%), structural (17, 14.8%) and other conditions (6, 5.2%). The five most common specific diagnoses were IgG4 related disease (20, 17.4%) and idiopathic orbital inflammatory disease (20, 17.4%), lymphoma (16, 13.9%), lacrimal gland prolapse (13, 11.3%), and sarcoidosis (11, 9.6%). Corticosteroid treatment was used most commonly (29, 25.2%) followed by observation (25, 21.7%). At last follow up, the majority of patients had complete resolution, significant improvement with mild residual disease or stable disease without further progression (104, 90.4%). CONCLUSION: Bilateral lacrimal disease may be due to a range of aetiologies, most of which are systemic. The most common are inflammatory and lymphoproliferative conditions. Due to the wide range of aetiologies of bilateral lacrimal gland disease, it is extremely difficult to accurately determine a cause based on clinical findings alone, highlighting the vital role of lacrimal gland biopsy in patients presenting with bilateral lacrimal gland disease.

Intraorbital and intracranial extension of adenoid cystic carcinoma without clinical or radiological lacrimal gland involvement.

Adenoid cystic carcinoma (AdCC) is a rare epithelial neoplasm of the head and neck, most commonly found in the salivary glands. Orbital AdCC is an uncommon clinical entity arising from the lacrimal glands, however primary orbital AdCC has been previously described in a small number of case reports. The exact origin of the neoplasm with uninvolved lacrimal gland in the orbit is unknown, however it may arise from ectopic lacrimal or salivary gland tissue, or extension from nearby epithelial structures. We describe the clinical characteristics, investigations and management of a 55-year-old man presenting with vertical diplopia, found to have left posterior orbital AdCC invading the skull base with intracranial extension involving the inferotemporal fossa, pterygopalatine fossa, left carotid artery, cavernous sinus and temporal lobe dura, without clinical or radiological lacrimal gland involvement or systemic metastases.

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.

Outcomes of surgical resection of sphenoid-orbital meningiomas with Sonopet ultrasonic aspirator.

Purpose: Surgical resection is the mainstay of treatment for spheno-orbital meningiomas. The Sonopet® is an ultrasonic aspirator device that provides several advantages over the traditional standard suction techniques and bone drill, including decreased collateral soft tissue damage, more precise bone removal and a clearer operative field. The purpose of the study was to examine the treatment outcomes of Sonopet®-assisted resection of spheno-orbital meningiomas.Methods: A retrospective chart review was conducted in seven patients with spheno-orbital meningioma in a single institution who underwent surgical resection with the Sonopet®. Pre-operative and post-operative data included the assessment of visual acuity, relative afferent pupillary defect (RAPD), Ishihara score, proptosis, fundus examination, computerised visual fields and the presence or absence of diplopia, headache, and other neuro-ophthalmic complications.Results: Nine Sonopet®-assisted procedures were performed on seven patients. Post-operatively, 89% of cases had stabilization or improvement of visual acuity and colour vision, whilst 29% had improved visual fields with the remainder being stable. Proptosis improved in all patients. Five of nine cases (44%) had new post-operative cranial nerve palsies, of which 75% were transient. One patient had tumour recurrence after 14 months, requiring further tumour resection and radiotherapy.Conclusion: Sonopet®-assisted resection of spheno-orbital meningiomas has comparable outcomes of visual improvement and complication rates to traditional resection techniques. Longer periods of post-operative observation and follow-up are recommended to observe long-term benefits.

Clinico-radiological Diagnosis of Optic Nerve Choristomas.

Optic nerve choristomas are very rare lesions. They are characterised by the presence of fat cells, mesodermal collection of fibrous tissue and smooth muscle, and atrophic optic nerve tissue. Although the condition can be diagnosed on histology, it is not always possible to carry out surgical exploration for tissue diagnosis in view of the apical location of the lesion and the inherent risk to vision from surgery. Detailed neuro-imaging becomes vital in these cases for diagnosis. We report a case of a patient with an optic nerve choristoma diagnosed on neuroimaging, review the current literature related to this condition, and discuss the key clinico-radiological features of optic nerve choristomas.

Nasolacrimal duct obstruction secondary to lacrimal sac involvement by sebaceous carcinoma.

Sebaceous carcinoma (SC) is the third most common eyelid malignancy in Australia, and is potentially fatal. It usually presents as a nodule or diffuse eyelid thickening, and is commonly misdiagnosed. We describe a case of SC with lacrimal sac involvement, presenting with clinical features of nasolacrimal duct obstruction. At the time of endoscopic dacryocystorhinostomy (DCR), nasal endoscopy revealed a polypoid mass of the opened lacrimal sac. Biopsy of the mass showed poorly differentiated SC. The lacrimal drainage apparatus was later excised via a combined external and endoscopic approach. Conjunctival map biopsies showed extensive intraepithelial disease, which was treated with topical mitomycin C. At three-month follow-up, there was no evidence of residual disease on nasal endoscopy or repeat conjunctival biopsy.

Bilateral orbital compartment syndrome in a patient with disseminated intravascular coagulation.

A 39-year-old male developed bilateral periorbital oedema and tense orbits in keeping with orbital compartment syndrome (OCS) shortly after presenting to the emergency department for uncontrollable epistaxis. Bilateral lateral canthotomy and inferior cantholysis was performed within 30 minutes of onset, with the left side further decompressed via superior cantholysis. Computed tomography demonstrated bilateral proptosis and optic nerve stretch, but no intraorbital haemorrhage or haematoma. Laboratory findings were consistent with disseminated intravascular coagulation (DIC) and sepsis of unknown origin. The right visual acuity recovered to 6/6 -2 from counting fingers, but the left eye failed to improve beyond light perception. This unique case of OCS is the first associated with DIC which had no evidence of intraorbital haemorrhage.