Optical frequency domain imaging (OFDI) represents a novel technique for the diagnosis of giant cell arteritis.

BACKGROUND/OBJECTIVES: Giant cell arteritis (GCA) is an inflammatory vascular disease in which prompt and accurate diagnosis is critical. The efficacy of temporal artery biopsy (TAB) is limited by 'skip' lesions and a delay in histological analysis. This first-in-man ex-vivo study aims to assess the accuracy of optical frequency domain imaging (OFDI) in diagnosing GCA. SUBJECTS/METHODS: 29 TAB samples of patients with suspected GCA were submerged in 0.9% sodium chloride and an OFDI catheter was passed through the lumen to create cross-sectional images prior to histological analysis. The specimens were then preserved in formalin for histological examination. Mean intimal thickness (MIT) on OFDI was measured, and the presence of both multinucleate giant cells (MNGCs) and fragmentation of the internal elastic lamina (FIEL) was assessed and compared with histology, used as the diagnostic gold standard. RESULTS: MIT in patients with/without histological evidence of GCA was 0.425 mm (±0.43) and 0.13 mm (±0.06) respectively compared with 0.215 mm (±0.09) and 0.135 mm (±0.07) on OFDI. MIT measured by OFDI was significantly higher in patients with histologically diagnosed arteritis compared to those without (p = 0.0195). For detecting FIEL and MNGCs, OFDI had a sensitivity of 75% and 28.6% and a specificity of 100% and 77.3% respectively. Applying diagnostic criteria of MIT > 0.20 mm, or the presence of MNGCs or FIEL, the sensitivity of detecting histological arteritis using OFDI was 91.4% and the specificity 94.1%. CONCLUSIONS: OFDI provided rapid imaging of TAB specimens achieving a diagnostic accuracy comparable to histological examination. In-vivo imaging may allow imaging of a longer arterial section.

Histopathological features of asymmetric lacrimal gland enlargement in patients with thyroid eye disease.

PURPOSE: Lacrimal gland enlargement can be a feature of thyroid eye disease (TED). Unilateral or asymmetric lacrimal gland enlargement is poorly described and may impede diagnosis. We present the histological and clinical findings of four patients with asymmetric lacrimal gland enlargement. METHODS: A retrospective case note review was performed for patients over two tertiary orbital clinics (Royal Adelaide Hospital, South Australia and the Sussex Eye Hospital, Brighton, United Kingdom) presenting with an asymmetrical lacrimal gland enlargement with a background of TED that underwent biopsy to exclude alternate diagnoses. Baseline data was collected for each patient and histopathological images and reports were reviewed. RESULTS: All four patients were hyperthyroid at time of lacrimal gland biopsy. Biopsy demonstrated nonspecific, lymphoid aggregates, typically of B cell type, with no diagnostic findings to support lymphocyte clonality or IgG4-related disease. One biopsy specimen demonstrated evidence of some fibrosis. CONCLUSION: Asymmetrical lacrimal gland enlargement can occur as part of the TED spectrum but may require biopsy to exclude alternate pathology. Histology demonstrates a non-specific lymphocytic infiltrate.

Acute Bilateral Orbital Myositis Following Covid 19 Vaccination.

The authors present a case of acute bilateral orbital myositis occurring 24 hours after the administration of the mRNA1273 vaccination for COVID 19. The patient was presented with right proptosis, with orbital imaging demonstrating bilateral enlargement of all the extraocular muscles. Serological investigation did not reveal a precipitating cause or underlying disease process. The presenting features resolved entirely following treatment with methylprednisolone and the patient remains asymptomatic.

Malignant lesions of the caruncle.

Caruncle malignancy is rare, but signs of disease can be easily missed by both patients and clinicians. There is significant potential for significant morbidity and even mortality from delayed diagnosis and treatment. Clinical features of primary malignant cancer include rapid growth, pigment deposition, ulcerated surface and bleeding. Malignant diagnoses include lymphoproliferative disease, basal cell carcinoma, squamous cell carcinoma, sebaceous carcinoma and malignant melanoma. Increased pigmentation is associated with melanoma, yellow coloured deposition with sebaceous carcinoma and a salmon-pink hue with lymphoproliferative disease. Treatment involves excision with margin control which may necessitate exenteration. Metastases to cervical and preauricular lymph nodes has been reported.

Spontaneous periocular ecchymosis: a major review.

Periocular ecchymosis, or periocular bruising, is a common clinical finding. Periocular skin is thin with an extensive vascular network, making this anatomical region prone to bruising. The most common etiology is trauma, but rarely, patients can present with spontaneous periocular ecchymosis (SPE). The pathophysiology of SPE is complex and varied. In this literature review of 121 articles, we assessed the frequency and variety of causation of this infrequent entity. The main finding was that by far the most common diagnosis causing SPE is amyloidosis and neoplasm, most notably neuroblastoma. Amyloidosis accounted for 23% articles (28/121) and neuroblastoma for 17% articles (21/121). Overall, neoplastic processes accounted for 30% of the articles (36/121), raised intracranial pressure and vascular malformations for 19% of the articles (23/121), migraine and atypical headache for 7% of the articles (8/121), while iatrogenic accounted for 5% of the articles (6/121). Through exploration and appreciation of the pathophysiology, we hope to foster a greater understanding in the clinician to establish underlying etiology, from benign to life-threatening, when presented with SPE.

Acute Leukemia Presenting in the Pediatric Orbit.

We present a case series to evaluate the clinical features of acute leukemia presenting with primary orbital manifestations. We undertook a retrospective case review of primary orbital presentations of acute myeloid leukemia (AML) and acute lymphocytic leukemia (ALL) over a 10-year period at two hospital sites (Hereford County Hospital and Leicester Royal Infirmary). Our case series included four patients - two with AML and two with ALL. Patients were young (mean age of four years and five months) at presentation, all with unilateral disease, and presented with orbital signs. Although there was some confusion with the diagnosis at the time of referral, a suspicion of malignancy was made rapidly once ophthalmic review was initiated. All four cases were diagnosed with the assistance of peripheral blood film and bone marrow biopsy, without the need for orbital biopsy. All four cases had resolution of the orbital mass and remain disease-free.

Probable IGG4 related ophthalmic disease presenting with uveitis.

We present a case of an uncommon presentation of IgG4-related ophthalmic disease (ROD). A 58-year-old female presented with unilateral acute anterior uveitis of the right eye, which progressed to scleritis with the development of an associated orbital mass despite treatment with oral glucocorticoid. Initial histopathology of an orbital biopsy was non-diagnostic and continued progression of the disease lead to complete loss of vision in the right eye. The development of uveitis in the previously unaffected left eye led to the decision for enucleation of the right globe and further orbital biopsy. Histopathology revealed features supporting IgG4-related ophthalmic disease. Oral glucocorticoid therapy failed to induce remission, and rituximab therapy was initiated, leading to a rapid resolution in her symptoms. Other cases with a similar presentation report a poor visual prognosis, highlighting the need for prompt diagnosis and treatment of uveitis associated with signs of orbital or scleral involvement.

Periocular Trichoblastoma: A Diagnostic Dilemma.

A 41-year-old woman presented with a large medial canthal lesion that extended over the midline. Excisional biopsy revealed a multinodular basaloid tumor, located within the dermis and subcutaneous fat that extended into skeletal muscle at the deep and peripheral margin. Histology was consistent with trichoblastoma. A review of the literature reveals a lack of specific and sensitive immunohistochemistry markers to establish the diagnosis of trichoblastoma. Following reconstruction, the patient has shown no signs of recurrence of the lesion at 24 months.