Ocular Myasthenia Gravis Associated With Thymic Amyloidosis.

A 45-year-old woman with ptosis and diplopia was found to have myasthenia gravis (MG) associated with amyloidosis of the thymus gland. Systemic MG is frequently associated with thymomas or thymic hyperplasia but has only once previously been reported in association with amyloidosis of the thymus. This case demonstrates that isolated ocular MG rarely may also be associated with amyloidosis of the thymus.

Impact of supply problems of preservative-free glaucoma medications on patients and hospital staff.

PURPOSE: Glaucoma is a chronic ocular disease, which is usually managed with long-term daily medical therapy, in the form of eye drops. Patients who are intolerant to preservatives in topical medicines require preservative-free versions. From early 2011 patients attending Moorfields Eye Hospital, London, UK, started to report recurring problems with the supply of the following preservative-free glaucoma medications: Timolol 0.25% (Timoptol 0.25%, MSD UK); Dorzolamide (Trusopt, MSD UK); Dorzolamide and Timolol 0.5% (Cosopt, MSD UK). This study investigates the impact of the supply problems of these medications at Moorfields Eye Hospital from a patient, administrative and clinical perspective. METHODS: Information was sought by interviewing both patients and hospital staff, and by a retrospective case note review between April 2010 and May 2013. RESULTS: Many hospital roles, both administrative and clinical, were involved in attempting to resolve the impact of the supply problems. All staff reported a considerable increase in their workload. At the peak of the problem, the glaucoma secretaries received about 150 enquiries per week. A review of 83 sets of patient notes, retrieved from a random sample of 125 patients, showed that 22% encountered a supply problem. Of these, more than one-third attended Moorfields Eye Hospital Accident & Emergency (A&E) for repeat supplies and 89% eventually had their medication changed. In telephone interviews with 39 of a random sample of 50 patients (a subset of the 83 notes retrieved), 59% of the interviewees reported a supply problem. Of these, one-third attended Moorfields Eye Hospital A&E for repeat supplies and half eventually required an alternative medication. Some patients reported going to considerable lengths to obtain ongoing supplies in the community. CONCLUSIONS: This study shows that medication supply problems can have a major impact on patients and hospital services. Supply problems occur across many fields of medicine and with increasing frequency. The findings of this study highlight the importance of early communication of impending shortages between manufacturers and the Department of Health, as recommended in the best practice guidelines. In order to minimise the impact of medicine shortages on patients, clinicians and administrative staff, hospitals need immediate notification of potential supply problems and clear updates on supply resolution. In addition, hospitals should consider nominating an individual as a contact point for patient enquiries regarding medicine supply problems.

Blepharoptosis and HAART related mitochondrial myopathy.

PURPOSE: To report a case of blepharoptosis in a patient with human immunodeficiency virus (HIV) on highly active antiretroviral therapy (HAART) with biopsy confirmed mitochondrial deletions consistent with HAART related myopathy. METHODS: A 51-year-old man with HIV demonstrated visually significant ptosis after being on HAART therapy for over 20 years. RESULTS: Muscle tissue biopsy following blepharoplasty was analyzed and found to have significant mitochondrial deletions. CONCLUSIONS: This patient represents a case of isolated ptosis consistent with acquired myopathy secondary to mitochondrial dysfunction without systemic manifestations otherwise seen in inherited mitochondrial disorders.

Juvenile iridoschisis and incomplete plateau iris configuration.

INTRODUCTION: Iridoschisis is a rare condition in which there is localized cleavage of the iris stroma into 2 layers. It can be associated with plateau iris and more often with either angle closure or open-angle glaucoma. It usually presents in later life as a bilateral progressive condition, however, in this article we present a young adult with bilateral iridoschisis and incomplete plateau iris configuration. CASE PRESENTATION: We report the case of a 22-year-old male with bilateral sensorineural hearing loss who presented to clinic for general ophthalmology evaluation. He had no prior ocular history. Examination findings revealed bilateral iridoschisis with incomplete plateau iris configuration. His ocular examination was otherwise unremarkable except for bilateral retinal pigment epithelium mottling. His work-up for glaucoma including optic disc evaluation, visual field testing, and imaging was all within normal limits. CONCLUSIONS: This case is unusual in its presentation of a young patient with bilateral iridoschisis and incomplete plateau iris configuration. Iridoschisis is more commonly a senile process and the presence of iridoschisis in a young adult prompts a review of congenital causes and associations. Although this patient had no evidence of glaucoma at this time, given the associated risk, it is important that he continue to have regular follow-up at this time.

Treatment of Refractory Persistent Corneal Epithelial Defects: A Standardized Approach Using Continuous Wear PROSE Therapy.

PURPOSE: To evaluate continuous wear of a fluid-ventilated, gas-permeable scleral PROSE device using a standardized protocol as treatment for refractory persistent corneal epithelial defects in patients with severe ocular surface disease. METHODS: Retrospective review of eight eyes of seven consecutive patients with persistent epithelial defects refractory to traditional therapies. The standardized treatment regimen consisted of: (1) 24-hour-a-day PROSE wear until re-epithelialization was achieved, (2) brief daily device removal, cleaning, disinfection, and reservoir fluid replacement, (3) addition of a benzalkonium chloride (BAK)-free fourth-generation fluoroquinolone antibiotic drop to the reservoir, and (4) transition to long-term, daytime PROSE wear upon re-epithelialization. RESULTS: All eight eyes exhibited resolution of the persistent epithelial defect. No eyes developed microbial keratitis. Four eyes exhibited recurrences; all recurrences promptly responded to reinstitution of continuous wear. CONCLUSIONS: Continuous wear of a PROSE device, using a strictly standardized regimen, constitutes an effective, safe treatment option for refractory persistent epithelial defects.

Ultra-widefield fluorescein angiography reveals retinal phlebitis in Susac's syndrome.

A 23-year-old woman with history of headaches and auditory changes presented with acute-onset visual field loss in the right eye. The combination of multiple retinal branch artery occlusions of the right eye on funduscopic examination, characteristic white matter lesions in the corpus callosum on magnetic resonance imaging, and hearing loss on audiometric testing led to a diagnosis of Susac's syndrome. Ultra-widefield fluorescein angiography revealed involvement of the retinal veins, which has not been previously reported with this condition. Additionally, ultra-widefield indocyanine green angiography demonstrated changes in the choroidal circulation, which are controversial in this syndrome.