A Rare Case of Amyloidosis of the Eyelid and Conjunctiva.

Amyloidosis of the eyelid is uncommon and is typically associated with systemic associations. In contrast, amyloidosis of the conjunctiva is often localised with no other associations. We present a rare case of a 92-year-old gentleman with both cutaneous lid lesions and conjunctival amyloid with no systemic involvement. Biopsy demonstrated the hallmarks of amyloid and treatment has remained conservative. He remains at the department to be monitored for secondary glaucoma.

Bisphosphonate-induced osteonecrosis of the maxilla presenting as a cicatricial ectropion.

CASE REPORT: A 63-year-old gentleman, who was being treated with bisphosphonates for multiple myeloma, presented with a cicatricial ectropion of the lower eyelid, without exposure keratopathy. A CT scan demonstrated extensive destruction of bone with an infraorbital fracture surrounded by sclerotic bony changes. The patient was managed conservatively with discontinuation of bisphosphonate therapy and topical ocular lubricants. The patient's condition remained unchanged a year after this initial management.

Merkel cell carcinoma of the eyelid: management and prognosis.

IMPORTANCE: The literature on Merkel cell carcinoma (MCC) of the eyelid remains scarce, and there has yet to be a study using the most up-to-date TNM staging system for this rare but aggressive tumor. OBJECTIVE: To analyze the TNM stage, management, and outcomes of patients with MCC of the eyelid. DESIGN, SETTING, AND PARTICIPANTS: Retrospective case series of 21 patients from 5 tertiary referral centers in the United Kingdom and Australia with primary MCC of the eyelid presenting at a median age of 77 years, with median follow-up of 54 months. Tumors were staged according to the American Joint Committee on Cancer, 7th edition, TNM criteria for eyelid carcinoma and MCC. MAIN OUTCOMES AND MEASURES: TNM stage, treatment modalities, and clinical outcome. RESULTS: The eyelid carcinoma TNM stages were T2aN0M0 for 5 patients, T2bN0M0 for 7 patients, T3aN0M0 for 4 patients, T3bN0M0 for 3 patients, T2bN1M0 for 1 patient, and T3aN1M0 for 1 patient. The MCC TNM stages were T1N0M0 for 12 patients, T2N0M0 for 7 patients, T1N1M0 for 1 patient, and T2N1M0 for 1 patient. One patient had a sentinel lymph node biopsy, and 8 patients underwent head/neck imaging. Eighteen patients underwent a wide local excision, 12 with a paraffin section and 6 with a frozen section. Two patients underwent Mohs surgery, 1 of whom required an orbital exenteration. Twelve patients (57%) received adjuvant radiotherapy, and 2 patients received chemotherapy. The local recurrence rate was 10%, the regional nodal recurrence rate was 10%, and the distant metastatic recurrence rate was 19%. The lowest T category tumor metastasizing to both regional nodes and distant locations was a T2a (eyelid TNM)/T1 (Merkel TNM) tumor measuring 8 mm. Two patients with T3a (eyelid TNM)/T2 (Merkel TNM) tumors died of metastatic MCC. CONCLUSIONS AND RELEVANCE: The majority of patients with MCC of the eyelid present with localized eyelid disease of T category T2 (eyelid TNM)/T1 (Merkel TNM). A wide local excision with margin control remains the mainstay of treatment, whereas the use of radiotherapy is institution specific. Tumors with a low T category are associated with regional nodal and distant metastatic disease. It may therefore be reasonable to consider a sentinel lymph node biopsy or strict regional lymph node surveillance for all MCCs of the eyelid, regardless of T category or size.

The significance of DNA mismatch repair genes in the diagnosis and management of periocular sebaceous cell carcinoma and Muir-Torre syndrome.

OBJECTIVE: The aims of this study were to determine the significance of expression of DNA mismatch repair proteins in detecting systemic malignancies in a series of patients with periocular sebaceous cell carcinoma and to determine the clinical characteristics and frequency of Muir-Torre syndrome in this cohort. DESIGN: The study was a retrospective non-comparative interventional case series. PARTICIPANTS: 31 patients with histologically proven sebaceous cell carcinoma of the eyelid participated in the study. METHODS: The authors made use of retrospective chart review and immunohistochemical staining of specimens. MAIN OUTCOME MEASURES: The main outcome measures are as follows: location, tumour size, sites of origin, growth patterns, management, histopathological and immunohistochemical findings, metastasis, other visceral malignancies and mortality. RESULTS: The median age of presentation of the 31 patients in this study was 71 years (range 35-92 years). There was a near-equal gender distribution (M:F-14:17). The average follow-up was 72 months. Seventeen patients had tumours arising from the upper lid, 13 from the lower lid and 1 from the caruncle. Nine patients had clinical Muir-Torre syndrome. Four patients were positive for microsatellite instability complexes and four were negative. Histologically, 14 patients had a high-grade tumour, 13 were intermediate grade and 4 were low grade. Based on the in situ pattern, six patients had a bowenoid pattern, five had both bowenoid and pagetoid patterns and two had a pagetoid pattern. Eighteen patients had no in situ disease detected. Twenty-one patients were alive without disease, and two were alive with disease. Six patients had died, five from other causes and one from the disease. CONCLUSIONS: Visceral malignancies are common in patients with periocular sebaceous cell carcinoma. Approximately one in eight demonstrated a heritable risk for further visceral malignancy through failure to express DNA mismatch repair proteins. Diagnosis of periocular sebaceous cell carcinoma should prompt physicians to search for other associated malignancies. Immunohistochemical characterisation of these sebaceous lesions is useful in identifying increased risk in affected patients and family members.

Linear scleroderma as a rare cause of enophthalmos: a case report.

INTRODUCTION: Enophthalmos is an important physical sign which can be easily missed. CASE PRESENTATION: A 64-year old female presented with painless and progressive shrinking of her right eye. Visual acuity was 6/6 in both eyes. The main clinical findings included exophthalmometry readings of 14 mm in the right eye and 22 mm in the left eye and a linear scar on her right forehead. This scar is a feature of linear scleroderma and called "en coup de sabre". She was referred to a dermatologist for further assessment. CONCLUSION: Enophthalmos is defined as the relative recession of the globe into the bony orbit and if measuring greater than 2 mm can give a noticeable cosmetic deformity. Scleroderma is a systemic or localised disease. Linear scleroderma has the following features-localised fibrosis of the skin, blood vessels, subcutaneous fat, muscle and sometimes bone. Histology shows an inflammatory and a sclerotic phase. Ophthalmic effects include enophthalmos, lash loss, lid induration or tightening and periorbital oedema.

Standards in consent for cataract surgery.

Consent forms for cataract surgery performed at Burnley General Hospital (BGH) and Blackburn Royal Infirmary (BRI) from October 4 to December 7, 2004, were prospectively reviewed to ensure that the East Lancashire Hospital's National Health Service (NHS) Trust Policy on consent to treatment and Department of Health (DoH) guidelines were being followed when seeking consent for cataract surgery. A set of 22 criteria derived as standards were formulated from the reference guide published by the DoH and from the East Lancashire trust policy document for consent to treatment. Each consent form was measured against these standards. Cases were randomly selected between BRI and BGH prospectively. All consent forms completed by physicians involved in formulating the standards were excluded. The review showed the NHS Trust Policy and DoH guidelines were largely followed when seeking consent for cataract surgery. However, certain areas were found to be deficient. If a health professional fails to obtain proper consent and the patient suffers harm as a result of treatment, it may be a factor in a claim of negligence against that health professional. Subsequent recommendations may include simple solutions that can be implemented to improve clinical practice when obtaining informed consent.