Ameloblastic Carcinoma With Orbital Invasion.

Ameloblastic carcinomas are malignant tumors arising from the odontogenic epithelium and defined as having features of ameloblastic differentiation in addition to cytological features of malignancy. Orbital involvement is rare and generally involves invasion of the orbital floor, apex, or soft tissue. This report describes an advanced presentation of ameloblastic carcinoma with orbital invasion and provides a review of the literature. A 58-year-old male presented with a 2-year history of a mid-facial mass, causing intracranial invasion and distortion of most skull foramina, nasopharynx, nasal cavity, and both orbits. Notably, there was an en-plaque pattern of circumferential tracking of the tumor along both orbital walls without invasion beyond the extraconal space, causing compression of the orbital apex and proptosis. Histology demonstrated nests of ameloblastic carcinoma and the advanced tumor was deemed nonresectable, with treatment being palliative.

Radiological measurements of lacrimal gland in thyroid eye disease.

PURPOSE: Lacrimal gland enlargement is a common feature of thyroid eye disease (TED) and has been positively correlated with the clinical activity score. Although lacrimal gland volume is the preferred measure of lacrimal gland size, its calculation is not clinically translatable due to the expertise, time and advanced software required. The aim of our study is to determine whether the lacrimal gland volume in patients with TED undergoing magnetic resonance imaging (MRI) can be estimated using simpler lacrimal gland linear and area measurements. METHODS: A retrospective review of 102 orbits (51 patients) with TED who underwent orbital MRI was conducted. The maximum length, width, and area of the lacrimal gland were measured in axial and coronal sections. Lacrimal gland volume was calculated by using a manual segmentation technique on all consecutive axial slices on commercially available software, OsiriX. All quantitative measurements were correlated with the lacrimal gland volume. RESULTS: Mean age of participants was 59 ± 16 years, and 67% (n = 34) were females. With multivariate analyses, combined lacrimal gland axial and coronal areas strongly correlated with volume (r = 0.843, p < 0.01). Strong univariate predictors of volume included axial area (r = 0.704, p < 0.01) and coronal area (r = 0.722, p < 0.01), while moderate predictors included axial length (r = 0.523, p < 0.01), axial width (r = 0.521, p < 0.01), coronal length (r = 0.450, p < 0.01), and coronal width (r = 0.649, p < 0.01). CONCLUSION: In patients with thyroid eye disease, lacrimal gland volume can be estimated using axial and coronal areas, which is simpler and more time efficient than calculating volumes.

Normal periocular anthropometric measurements in an Australian population.

PURPOSE: To report the normative ocular and periocular anthropometric measurements in an Australian cohort and investigate how these may be affected age, gender, and ethnicity. METHODS: Prospective study of patients presenting to the Royal Adelaide Hospital. Patient with orbital or eyelid disease, previous surgery, craniofacial abnormalities, pupil abnormalities, strabismus, and poor image quality was excluded. Standardised photographs were taken in a well-illuminated room. A green dot with a diameter of 24 mm was placed on the participant's foreheads for calibration between pixels and millimetres. Ocular and periocular landmarks were segmented to calculate the periorbital measurements. Independent sample t test was used to compare male and female subjects, Pearson's correlation was used to correlate periocular dimensions with age, and ANOVA with Bonferroni was used to compare periocular dimension between ethnic groups. RESULTS: Seven hundred and sixty eyes from 380 participants (215 female, mean age 58 ± 18 years) were included. The mean marginal reflex distance (MRD) 1 was 3.5 mm and decreased with increasing age (r = - 0.09, p = 0.01) and MRD 2 was 5.2 mm. Compared to Caucasians, African subjects had a significantly larger interpupillary distance and outer intercanthal distance, whereas East Asians had a significantly larger inner intercanthal distance (p < 0.05). The values of marginal reflex distance 2, palpebral fissure height, horizontal palpebral aperture, inner intercanthal distance, interpupillary distance and outer intercanthal distance were significantly higher in male subjects than female subjects (p < 0.05). CONCLUSIONS: Normative periocular dimensions may vary according to age, gender, and ethnicity. An understanding of normal periocular dimensions is important in the evaluation of orbital disease across different ethnic groups and may serve as reference points for oculoplastic surgery and industry.

Recurrent orbital inflammation associated with VEXAS syndrome.

VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) syndrome is a newly recognised adult-onset multisystem autoinflammatory disease caused by a somatic mutation in the UBA1 gene in myeloid or erythroid precursor cells. This report describes an atypical presentation of recurrent dacryoadenitis associated with VEXAS syndrome and provides a review of the literature. A 68-year-old male presented with three episodes of unilateral alternating dacryoadenitis followed by bilateral involvement over a 4-year period. Each episode of orbital inflammation was characterised by upper lid swelling, oedema and enlarged lacrimal glands. In addition, he experienced intermittent flares of angioedema-like lesions involving the face and extremities, recurrent jaw aches, rash, progressive pulmonary fibrosis, and myelodysplastic syndrome. His inflammatory symptoms lessened with prednisolone but were refractory to methotrexate. Mycophenolate was subsequently trialled with a reasonable clinical response. Genetic testing established the diagnosis of VEXAS syndrome and tofacitinib, a JAK inhibitor, was commenced with resolution of inflammatory symptoms.