Utility of optic pathway glioma screening in young children with neurofibromatosis type I: questions generated by a clinical audit.

AIM: Neurofibromatosis type I (NFI) is a phakomatosis that affects approximately 1 per 3000 live births. About 15% children with NFI develop optic pathway glioma (OPG). The Neurofibromatosis UK society recommend annual ophthalmic screening to identify those children who may have OPG affecting vision and refer for investigation and treatment as necessary. METHODS: We undertook a retrospective audit with three aims: (1) to elicit if departmental screening practice of children with NFI for OPG meets current guidelines, (2) to document the age at which tests of visual function are useful in the diagnosis and screening of OPG, and (3) to document the contribution eye screening has made to the diagnosis of OPG. RESULTS: A total of 37 children were identified from the clinic register. Overall 43% children met the criterion for an appropriate number of screening episodes. All the children met the visual acuity and optic disc assessment criteria; 84% met the pupil-testing criterion. No child was mature enough to perform visual fields or colour vision testing. CONCLUSION: Further education is required to encourage patients to attend eye clinic for screening as the majority of patients failing to reach the standard were due to non-attendances. No OPGs were detected during 128 screening episodes over approximately 7 years of screening. The authors question the usefulness of including visual field and colour vision assessment in the protocol for this age group.

Social and visual function in nystagmus.

AIM: To investigate the influence of nystagmus on visual and social function and determine if parents are able to assess visual and social function in children with nystagmus. METHOD: A postal questionnaire comprising 14 questions related to visual function (VF-14) and questions pertaining to social function were sent to all 1013 members of the Nystagmus Network-a UK based organisation for nystagmus sufferers and their families. Visual and social function scores were compared by regression analysis. RESULTS: 180 adult, 233 parent, and 124 child questionnaires were returned. Idiopathic nystagmus was the most common cause. In adults the mean VF-14 score indicated very low visual function, in the same range as patients assessed in low vision services. Children's visual function scored better than adults, between scores of patients with age related macular disease and corneal grafts. There was a strong correlation between perceived visual and social function for adults (p<0.001) and parental assessment of their children (p<0.001), but not between child self assessment of visual and social function. There was strong correlation between parental and child assessment of visual and social function (p<0.001, p<0.001) CONCLUSION: Questionnaires indicated that nystagmus is associated with very low visual function. There is a strong correlation between visual and social impairment. The authors have shown for the first time in an ophthalmic disease that parents are able to estimate the impact of nystagmus on their child both in terms of visual and social functioning, although they underestimate the impact of nystagmus on emotional aspects of wellbeing.

Non-traumatic sixth nerve palsy in a young patient.

Non-traumatic sixth nerve palsy in the 20-50 years old group is unusual and a detailed history is often needed to identify serious and potentially reversible causes. We present a case of sixth sense nerve palsy in a young female with eczema for which she was treated with low-dose cyclosporin. We review the indications for and ocular side effects of cyclosporin, a drug with which most ophthalmologists will be unfamiliar.

Attendance at the Eye Department Harare, Zimbabwe: the spectrum of clinical presentations.

OBJECTIVE: To present data on presentation patterns of ophthalmic disease in a developing country, highlight areas of change and suggest policy to improve the current situation. DESIGN: Cross sectional study. SETTING: Sekuru Kaguvi Eye Department, Parirenyatwa Hospital, Harare, Zimbabwe. SUBJECTS: All new patients presenting to the department during a one week period. MAIN OUTCOME MEASURES: Referral route, time from onset of symptoms to presentation and mode of injury where appropriate as stated by the patient; diagnosis made by trained ophthalmologist. RESULTS: 196 patients presented during the five day period, 61.2% male, 38.8% female. The majority of patients were males of working age, presenting themselves or referred from an urban clinic. The most common diagnostic group was trauma (21.9%) usually occurring in the workplace, of which 53.5% were corneal foreign bodies. 37% of trauma cases presented the same day, and over 50% within 24 hours of injury. CONCLUSION: The study was carried out as a pilot audit project and shows there have been some noticeable changes to the presentation of eye disease over the last 20 years, with patients presenting sooner with trauma and more frequently overall. The introduction of policies concerning the use of safety goggles at work may begin to reduce eye disease due to trauma.