EPOS

European Paediatric Ophthalmological Society

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Choroidal haemangioma associated with Sturge-Weber Syndrome treated with low dose ocular irradiation

O'Keefe Michael, Soeldner Hansjorg, Lanigan Bernadette
¹The Children's Hospital, Department of Paediatric Ophthalmology, Temple Street, Dublin 9, Ireland

Purpose:This case study of a patient with a choroidal haemangioma associated with Sturge-Weber syndrome describes the clinical features of this disorder and the clinical course after low dose ocular irradiation. Methods: The retrospective case history of an 8-year-old female patient with a choroidal haemangioma is reported. Results:A girl who was born with Sturge-Weber Syndrome developed a left congenital glaucoma. At the age age of 4 months a successful trabeculectomy of the left eye was performed. Both eyes developed a visual acuity of 20/20 with correction (Right eye plano, left eye mixed astigmatism: -2.00Dsph, +3.00Dcyl x 90 degrees). In the subsequent follow-up period the intraocular pressures remained at a satisfactory level without further treatment and no choroidal haemangioma was detected. At the age of 8 years she presented with a total retinal detachment.An ultrasound examination showed a large choroidal haemangioma and exudative retinal detachment. Low dose external beam ocular irradiation of 6 Gy in three fractions was applied. Subsequently there was only a slight reduction of the subretinal fluid. The visual acuity of the left eye remained perception of light with intact light projection. Conclusion: Large choroidal haemangiomas associated with Sturge-Weber Syndrome are difficult to treat and low dose ocular irradiation showed only limited success.