Bisphosphonate-Induced Orbital Inflammation: A Case Series and Review.

INTRODUCTION: To present a series of patients with bisphosphonate induced orbital inflammation, and to review the clinical presentation, radiological features, treatment options and outcomes. METHODS: We present a multicentre, retrospective case series review of patients with a clinico-radiological diagnosis of bisphosphonate induced orbital inflammation and review all the reported cases of this complication in the literature. RESULTS: Four new patients with bisphosphonate induced orbital inflammation were added to the 25 cases in the literature. Intravenous zoledronate was the commonest precipitant (22/29, 75.9%) and inflammation occurred 1-28 (mean 3) days post-infusion. Orbital imaging identified orbital inflammation in 22/29 cases and extra-ocular muscle enlargement in 8/29. Five patients presented with reduced vision of which one - with anterior ischaemic optic neuropathy - did not resolve. The vision resolved in all except one patient, with most requiring steroid treatment. CONCLUSIONS: Bisphosphonates have a pro-inflammatory effect, which can precipitate orbital inflammation. This rare, but potentially serious complication of bisphosphonate treatment should be considered by clinicians using bisphosphonate treatment and by ophthalmologists seeing patients with orbital inflammatory disease.

Mucus extravasation into the orbit during frontal sinus irrigation.

During minitrephination and irrigation of the frontal sinus, mucus extravasated into the orbit through a defect in the sinus floor. The mucus incited a foreign body reaction and became encapsulated within the orbit necessitating excision via an anterior orbitotomy.

Hepatocellular carcinoma metastasis to sphenoid wing.

We present a case report where hepatocellular carcinoma metastasis to the sphenoid wing was the initial presentation of an occult primary.

Endogenous Streptococcus mitis panophthalmitis in a patient visiting the Dominican Republic.

A 67-year-old woman presented with painful, acute vision loss after 5 days of fever and muscle aches while visiting the Dominican Republic. She had no recent history of ocular surgery, dental work or recent trauma. Anterior chamber aspiration confirmed an initial diagnosis of endogenous endophthalmitis, positive for Streptococcus mitis that progressed to panophthalmitis on return to Canada. Treatment included systemic antibiotics, intravitreal antibiotics and intravitreal dexamethasone. Despite the best medical treatment, the left eye progressed to corneal perforation 5 weeks after presentation. An evisceration with fitted orbital implant was successful in alleviating pain following the surgery. S. mitis is a rare, but possible cause of endogenous endophthalmitis and panophthalmitis. It was important to work with a multidisciplinary and global team to coordinate and offer appropriate treatment measures. Although vision was lost, evisceration of the left eye provided ocular comfort and good cosmetic outcomes for the patient.

Natural history of primary paediatric optic nerve sheath meningioma: case series and review.

PURPOSE: To study the natural history, clinical and radiological characteristics of primary paediatric optic nerve sheath meningioma (PPONSM). METHODS: Retrospective study of eight paediatric patients who were treated between 1994 and 2016 at the University Hospital Zurich, Switzerland and the Royal Adelaide Hospital, Australia. Clinical records and imaging studies were reviewed. RESULTS: The mean age at presentation was 11 years (range: 6-17 years). There were six female patients and two male patients. 2/8 patients had associated neurofibromatosis type 2. Patients were followed up for 71-297 months (mean 156±70 months). 6/8 patients were observed through the course of their disease and 2/8 patients were treated with radiotherapy. 2/8 patients who were observed had minimal change in vision and did not experience tumour growth after long-term follow-up. CONCLUSIONS: This is the largest PPONSM case series with long-term data on patients treated conservatively. We highlight that a small subset of these tumours are indolent and can be managed using observation alone.

Lymphoid hyperplasia of the orbit and ocular adnexa: A clinical ​pathologic review.

Lymphoid hyperplasia (LH) is a benign lymphoproliferative disorder that, in a minority of cases, may be associated with concurrent or metachronous non-Hodgkin lymphoma. LH cases are further subdivided into "reactive" and "atypical" categories based on the presence or absence of unequivocal malignant features. With improving molecular diagnostic technologies, "reactive" LH is by far the most common category of LH, with atypical LH accounting for only a small minority of specimens. Similarly, lesions previously diagnosed as LH are now being revised as low-grade B-cell non-Hodgkin lymphoma or diagnosed as newly described benign conditions such as IgG4-related disease. Additional differential diagnoses include specific and nonspecific orbital inflammations, infiltrative processes, and depositions. Hence, there are emerging changes in the patterns and proportions of entities that fall within the spectrum of lymphoproliferative disorders of the orbit and ocular adnexa. Reactive LH and low-grade malignant lymphoproliferative disorders in the orbit and ocular adnexa are clinically and radiologically indistinguishable from each other, requiring tissue biopsy in all cases. The prognosis of ocular adnexal LH is generally favorable, but the small risk of non-Hodgkin lymphoma mandates follow-up for at least 5 years. We summarize the current state of knowledge on LH occurring in the orbit and ocular adnexa.

Clinico-radiological features of primary lacrimal gland pleomorphic adenoma: an analysis of 37 cases.

PURPOSE: To present the radiological and clinical features of primary lacrimal gland pleomorphic adenoma (PLGPA). METHODS: Thirty-seven consecutive PLGPAs presenting to two hospitals in Japan were reviewed. RESULTS: PLGPA cases had 15 men and 22 women with a mean age of 51.9 years. Common presenting features were ptosis (83 %), diplopia (78 %) and globe displacement (78 %). Twenty-two percent of cases reported symptom duration of less than 6 months, but only 5.6 % of cases had pain. Sixteen percent of PLGPAs were centered in the palpebral lobe; none of which were associated with globe indentation or lacrimal fossa expansion. Bony excavation was observed in 84 % of orbital lobe PLGPA; the bone margin was well demarcated and the character of excavation was more frequently smooth than scalloped (2:1). The presence of globe indentation or bony excavation was associated with increased tumor size (p = 0.003). An enhancing rim was visible on T1-weighted fat-suppressed gadolinium-enhanced magnetic resonance imaging (T1FS-Gad MRI) in 27 %. Five (19 %) enclosed cystic spaces were bright on T2-weighted MRI and non-enhancing. Calcification of tumor parenchyma was common in cystic cases but rare in non-cystic cases (p = 0.01). CONCLUSIONS: PLGPAs have varied clinical and radiological features. Common radiological features are a heterogeneous internal architecture on T2-weighted MRI, an enhancing rim on T1FS-Gad MRI, smooth or scalloped bony excavation with intact cortical bone, and globe indentation. Cystic spaces, calcification, and symptom duration less than 6 months are common, but pain is rare. Awareness of the clinico-radiological variants of PLGPA is important when considering incisional biopsy of a lacrimal gland mass.

Prospective evaluation of digital non-stereo color fundus photography as a screening tool in age-related macular degeneration.

PURPOSE: To evaluate mydriatic, non-stereo digital color fundus photographs as a screening tool for identifying and classifying exudative age-related macular degeneration (AMD). DESIGN: Prospective case series. METHODS: Digital color fundus photographs were obtained from patients seen in the AMD screening clinic over a 9-month period at the Ivey Eye Institute in London, Ontario. Photographs for eligible patients were separated by eye, cataloged, blinded, and randomly labeled before interpretation by an experienced vitreoretinal surgeon. Exact agreement, sensitivity, specificity, positive predictive value, and negative predictive value of the fundus photographs in diagnosing, classifying, and managing cases of suspected exudative AMD were then calculated against gold standard clinical examination and fluorescein angiography. RESULTS: A total of 223 images were used from 118 eligible patients. Exact agreement between photographic evaluation and gold standard ranged from 89.2% (presence of pigment epithelial detachment (PED)) to 82.5% (evidence of retinal pigment epithelium geographic atrophy). Sensitivities ranged from 89.2% (presence of choroidal neovascular membrane (CNVM)) to 40.0% (presence of PED). Specificities ranged from 94.1% (presence of PED) to 86.8% (presence of retinal pigment epithelium geographic atrophy). Positive predictive value ranged from 86.1% (presence of CNVM) to 40.0% (presence of PED). Negative predictive value ranged from 94.1% (presence of PED) to 88.9% (presence of CNVM). As a screening tool for high-risk dry changes and active exudative changes, overall sensitivity specificity, positive predictive value, and negative predictive value were 82.1%, 79.1%, 70.4%, and 88.0%, respectively. CONCLUSIONS: Digital, non-stereo color fundus photographs are highly sensitive and have high negative predictive value as a screening tool. Very few treatable lesions are missed using telemedicine in age-related macular degeneration.

Sebaceous carcinoma in Japanese patients: clinical presentation, staging and outcomes.

AIM: To describe the clinical features, management and correlation of the American Joint Committee on Cancer tumour node metastasis (TNM) staging for eyelid carcinoma with outcomes in Japanese patients with sebaceous carcinoma. METHODS: Multicentre retrospective review of 63 Japanese patients. Tumours were staged using the American Joint Committee on Cancer 7th edition TNM criteria. RESULTS: A distinct mass was the initial presentation in 94% and correct initial diagnosis made in 57% patients. Most tumours (60%) presented at stage T2aN0M0. The remaining TNM stages were: T2bN0M0 (25%); T3aN0M0 (9%); T3bN0M0 (2%); T2bN1M0 (2%); T3bN1M1 (2%). Frozen section controlled excision was performed in 81%. One patient required an orbital exenteration. Median follow-up was 4.2 years. Local recurrence occurred in four patients treated with frozen section controlled excision. Five patients had regional nodal metastases, two of which had T2aN0M0 lesions. T3a tumours and greater were significantly associated with local recurrence but not regional nodal metastasis. One patient died due to disease. One patient is alive with disease, and remaining patients were alive without disease at last follow-up. CONCLUSIONS: In this Japanese cohort, an eyelid mass was the main clinical presentation of sebaceous carcinoma. Contrary to previous reports, T2a tumours smaller than 10 mm were associated with regional nodal metastases.