Abstract
Abstract: :
Purpose:To assess the value of annual fundoscopy for the detection of retinal angiomas in patients with Von Hipple Lindau disease. Methods:This was a longitudinal, single center study. Patients with Von Hipple Lindau disease underwent annual surveillance for retinal angiomas as part of the Cambridge screening protocol. All patients included in this study met the clinical criteria for the diagnosis of Von Hipple Lindau disease. The length of follow up, age of the patient and number of new retinal angiomas was recorded. The annual incidence of new angiomas was calculated. Results:45 patients were included in this study, male to female ratio 1:1.5. The median age at referral was 29 years (range 6–64). The median follow up period was 7 years (range 1–30 years). 18 patients (40%) had retinal angiomata at the first fundal examination (5 optic disc angiomas, 22 peripheral retinal angiomas). 8 patients, with normal fundi initially, developed retinal angiomas. 26 new angiomas were discovered during the period of follow up (3 optic disc and 23 peripheral retina), 12 of these were found in patients who had no previous retinal angiomas. The median age at presentation of the first angioma was 25 years (range 10–34). The median age at presentation of a second angioma was 29.5 years (range 11–51). The annual incidence of retinal angiomas was 0.07 per year (7 angiomas per 100 patients per year.) Conclusions: Patients with Von Hipple Lindau disease are at risk of visual morbidity from retinal angiomas. Given the high incidence of retinal angiomas in these patients annual surveillance is justified and should be continued long term.
Keywords: clinical (human) or epidemiologic studies: prevalence/incidence • oncology • retina