The masquerades of a childhood ciliary body medulloepithelioma: a case of chronic uveitis, cataract, and secondary glaucoma.

Ciliary body medulloepitheliomas in childhood often masquerade other intraocular conditions due to its insidious nature as well as its secondary effects on proximal intraocular tissues in the anterior chamber. We report a case where a ciliary body medulloepithelioma in a two-year-old boy presents with chronic uveitis, cataract, and an uncontrolled secondary glaucoma after an innocuous blunt ocular trauma. The diagnosis was only made after the occurrence of a ciliary body mass. We discuss the clinical features of ciliary body medulloepitheliomas, the implications of a delayed diagnosis and treatment as well as the concern of periorbital tumor seeding with the use of an aqueous shunt implant in this case.

Efficacy and complications of super-selective intra-ophthalmic artery melphalan for the treatment of refractory retinoblastoma.

PURPOSE: To report the efficacy of super-selective intra-ophthalmic artery melphalan (IAM) for the treatment of refractory retinoblastoma and any associated complications of this treatment. DESIGN: A prospective case series. PARTICIPANTS: Eyes with retinoblastoma that had been treated with systemic chemotherapy or local therapy and had a relapse of their condition. METHODS: All patients receiving IAM between May 2009 and September 2010 were included in the study. Intra-ophthalmic artery melphalan was offered to patients who had failed to respond adequately to systemic chemotherapy and local treatment where appropriate or because of a new recurrence of retinoblastoma that could not be treated with local therapies. None of the patients were excluded because of central nervous system abnormalities. Patients received 2 treatments of IAM given 4 weeks apart. All patients received an orthoptic assessment 3 weeks after each treatment and an examination under anesthesia (EUA). A third treatment was given if an unsatisfactory response was observed on EUA after 2 treatments. MAIN OUTCOME MEASURES: The response of the retinoblastoma tumor(s) and any associated local side effects from the treatment. RESULTS: A total of 15 eyes in 14 patients were treated with IAM during the study period. The mean age at the time of IAM was 31.5 months (median 17.3, range 11.2-150.7 months), and the mean follow-up was 8.7 months (3-16.3 months). Tumor control was achieved in 12 eyes (80%), and 12 eyes (80%) had local side effects that included third cranial nerve palsy in 6 (40%), orbital edema in 3 (20%), permanent retinal detachment in 1 (7%), and vitreous hemorrhage in 4 (27%). Seven eyes (47%) developed significant retinal pigment epithelium changes. CONCLUSIONS: Intra-ophthalmic artery melphalan is an effective treatment for retinoblastoma, achieving a high level of remission in refractory tumors. It can be associated with significant local side effects that can result in loss of vision and possible amblyogenesis. Clinicians and parents need to consider the benefits and potential local side effects before embarking on treatment.

Quality of optometry referrals to neovascular age-related macular degeneration clinic: a prospective study.

OBJECTIVES: To evaluate the quality of referrals to a neovascular age-related macular degeneration clinic from optometrists using the standard Rapid Access Referral Form (RARF) from the Royal College of Ophthalmologists. DESIGN: A prospective study. Prospective data were gathered from all optometry referrals using the RARF, between the periods of December 2006 to August 2009. These were assessed for accuracy of history, clinical signs and final diagnosis as compared to a macula expert. SETTING: Highlands NHS Trust. PARTICIPANTS: All patients referred to the eye department at NHS Highlands Trust using the RARF. MAIN OUTCOME MEASURES: The symptoms of neovascular age-related macular degeneration correctly identified by optometrists, and the signs of neovascular age-related macular degeneration correctly identified by optometrists. RESULTS: Fifty-four RARFs were received during this period, there was an overall agreement with symptomatology in 57.4% of cases. Optometrists scored less well in recognizing the clinical signs of neovascular age-related macular degeneration, with the poorest scores for recognizing macular oedema (44.4%) and drusen (51.9%). Twenty (37%) patients referred had neovascular age-related macular degeneration. CONCLUSIONS: RARFs make up the minority of referrals to the neovascular age-related macular degeneration clinic. Optometrists find it difficult to accurately elicit the signs of macula disease.

Unsuspected periventricular leukomalacia in children with strabismus: a case series.

PURPOSE: We report a case series of seven patients who presented with strabismus and no apparent known neurological deficit. METHODS: A retrospective review of notes was performed on all patients presenting at the Royal Liverpool Children's Hospital (Alder Hey) with strabismus, in whom subsequent investigation revealed the presence of periventricular leukomalacia (PVL). RESULTS: All seven patients presented with esotropia. One patient also had dissociated vertical deviation. A history of premature birth was elicited in six cases; however, one patient had been born at 42 weeks. Apparently uncomplicated routine surgery produced unexpectedly poor results. CONCLUSIONS: Patients with undiagnosed PVL may present to the ophthalmologist with strabismus and no other apparent neurological abnormality. Where it is suspected that the patient may have suffered a significant hypoxic-ischaemic event, the ophthalmologist should have a low threshold for investigating with magnetic resonance imaging, particularly if the patient shows unexpected outcomes following appropriate treatment. Patients and parents should be counselled on possible suboptimal results prior to the offer of surgical correction of strabismus.

Adverse effects of fluorescein angiography in hypertensive and elderly patients.

PURPOSE: To evaluate adverse effects in patients undergoing fundus fluorescein angiography (FA), and to assess the level of these adverse effects in hypertensive and elderly patients. METHOD: We carried out a retrospective study of 358 patients undergoing FA. They were assessed for adverse effects and their relationship to medical history, pre- and post-FA blood pressure and age. RESULTS: The overall adverse reaction rate (AR) was 11.2%, the majority (80%) of which involved nausea. There were no mortalities and no cases of anaphylaxis or cardiac arrest. Hypertension (systolic or diastolic) did not increase the AR. Interestingly, raised initial systolic blood pressure (BP) > or = 160 showed a statistically significant decreased AR compared with that for BP < 160 (7% versus 15%, respectively; p = 0.04). There was no statistical correlation between AR and age. CONCLUSIONS: FA is a relatively safe procedure, but serious side-effects have been reported in the past and therefore adequate measures need to be in place to cope with such emergencies. In our study, there was no increase in the incidence of adverse effects in patients who were hypertensive or elderly. Therefore, these patients should not be denied an important diagnostic procedure that may improve their quality of life purely because of hypertension or age.