Cystic squamous cell carcinoma of the orbit.

Both primary and secondary squamous cell carcinoma (SCC) of the orbit are rare entities, though cystic SCC is even more so. It may provide a significant diagnostic conundrum to oculoplastic surgeons. We present a case of an 86 year old male with a supero-medial transilluminating cystic lesion of the orbit. There was a preceding history of a moderately differentiated SCC of the cheek, excised 3 months prior. Computed tomography (CT) demonstrated no bone erosion. The cyst was excised aided by fibrin glue. This demonstrated a poorly differentiated cystic SCC with perineural infiltration. The patient elected for palliative aspirations of the cyst and is alive 12 months later. Cystic SCC of the orbit may present to a number of specialties, including maxillofacial and orbital surgeons. Both diagnosis and management may be challenging. We review common patterns in previous cases and discuss management.

Vismodegib for periocular basal cell carcinoma: an international multicentre case series.

INTRODUCTION: Vismodegib (Erivedge, Genentech) is a first-in-class inhibitor of the hedgehog (Hh) pathway, which is licensed for use in locally advanced basal cell carcinoma (BCC) and metastatic BCC. The National Institute for Health and Care Excellence withdrew recommendation for use of vismodegib secondary to a lack of data comparing vismodegib to standard supportive care. The purpose of this multicentre, international case series is to report outcomes of patients with locally advanced periocular BCC who have been treated with vismodegib. METHODS: The medical records of all patients treated with vismodegib were retrospectively reviewed across seven institutions in the United Kingdom, Australia, and New Zealand. RESULTS: Thirteen patients were identified. Seven (54%) patients were male. All BCCs were ill-defined, with seven (58%) having orbital involvement at presentation. Median treatment time was 7 months (range 2-36 months). Eleven out of 13 patients developed side effects, the most common being fatigue in six patients (46%). Median follow-up was 24 months (range 12-48 months). Complete response was found in 5/13 patients (38%) and a partial response in 8/13 patients (62%). Six patients had further surgery after vismodegib, with three classed as globe-sparing operations. Three patients developed recurrence (23%). Three patients (23%) ultimately underwent exenteration. DISCUSSION: This study demonstrates vismodegib to be a well-tolerated treatment which may, in some cases, facilitate globe-sparing surgery and hence avoid disfiguring operations such as exenteration. Uncertainty does remain regarding the long-term outcomes of patients treated with vismodegib.

The clinical, histological and immunohistochemical characteristics and nomenclature of meibomian gland ductal cysts (intratarsal keratinous cysts) and eyelid steatocystomas.

PurposeMeibomian gland ductal cysts (MGDCs) and steatocystomas are epithelial lined, keratin-containing lesions of the eyelids. MDGCs are variably called tarsal keratinous cysts, intratarsal keratinous cysts of the meibomian glands, intratarsal inclusion cysts, epidermal cysts and epidermoid cysts. Both lesions are poorly described in the literature. We report a series of seven MGDC and steatocystomas, and examine their clinical, pathological and immunohistochemistry features and their management and outcomes.Patients and methodsA retrospective review of case notes and histopathology slides of all MGDCs and steatocystomas identified at one major histopathology service in South Australia between 2013 and 2015.ResultsSeven cases were identified, with an average age of 64. The lesions range from 4 to 18 mm diameter and are firm, well-circumscribed and non-tender, and sometimes the keratin-filled cyst protrudes visibly under the tarsal conjunctiva. Two cases were previously misdiagnosed as chalazia but recurred after incision and curettage. Histologically, these lesions are lined by squamous epithelium but lack a well-formed stratum granulosum and can be distinguished by their immunohistochemical staining characteristics. Complete excision, including a wedge of underlying tarsal plate for MDGCs, is curative for with a follow up of 12-36 months.ConclusionsMGDCs and steatocystomas should be included in the differential of benign eyelid lesions. Diagnosing and differentiating these lesions from chalazia is important for determining the optimal management strategy.

Tinted contact lenses as an alternative management for photophobia in stationary cone dystrophies in children.

BACKGROUND: Intense photophobia is a debilitating symptom of the stationary cone dystrophies. The dark-tinted glasses with side-shields and floppy hats used to manage this are very conspicuous and can cause marked psychological morbidity to the children and their families. We assess the use of tinted contact lenses as an alternative management for the photophobia. METHODS: Three children, aged 5 to 13, with cone dystrophies, all with markedly reduced visual acuity, color vision, and profound photophobia were fitted with Lunelle ES70 Solaire 70% brown contact lenses. The child's parents completed two Children's Visual Function Questionnaires, the first pertaining to the period when the child wore tinted glasses; the second pertaining to the period of tinted contact lens wear. RESULTS: Subjectively, two of the children and their parents described a striking improvement in their quality of life, with improved confidence, interactions with other children, and cessation of name-calling and bullying. The Children's Visual Function Questionnaires gave objective evidence for this improvement in one child and was not significantly different in the other. The third child was reluctant to wear the contact lenses despite her parents' active encouragement. CONCLUSION: Tinted contact lenses offer an alternative management of the photophobia associated with stationary cone dystrophies in children. Marked improvements in their quality of life were observed in this case series.

Contamination of disposable tonometer prisms during tonometry.

AIM: Due to the theoretical possibility of prion transmission in applanation tonometry, many ophthalmological units in the United Kingdom now use disposable tonometer prisms. We have investigated the potential for bacterial and viral transmission from the health practitioner to the patient via disposable prisms. METHODS: All staff who perform applanation tonometry at the Sussex Eye Hospital (SEH) received a questionnaire to evaluate if the applanating face of the prism is touched during tonometry and the ease of use of the disposable prism compared to the reusable prisms that were previously used. We then cultured prisms handled by a random sample of staff members for common bacteria. Finally, we constructed a model to investigate the possibility of interpatient adenoviral transmission via disposable tonometer prisms. RESULTS: The questionnaire revealed that almost 50% of the staff admit to touching the applanating face of the tonometer prism prior to applanation. Cultures of the prisms grew a range of bacteria including Staphylococcus epidermidis, Staphylococcus aureus, and Bacillus species. The viral model suggested that adenovirus could be transmitted by applanation tonometry. CONCLUSION: The use of disposable prisms for applanation tonometry may reduce the risk of prion transmission but is not bacteriologically or virologically aseptic. This is a potential infection risk to patients.