Título

CIRCUMSCRIBED AND DIFFUSE CHOROIDAL HEMANGIOMAS IN A PATIENT WITH BILATERAL STURGE-WEBER SYNDROME, A RARE PRESENTATION

Objetivo

To report a case of bilateral Sturge-Weber syndrome (SWS) with diffuse choroidal hemangioma in the right eye (OD) and circumscribed choroidal hemangioma in the left eye (OS).

Relato do Caso

20-year-old, female, with complain of low visual acuity in OD for 10 months. She reported episodes of seizure in childhood and bilateral trabeculectomy at 2 years old. In the central nervous system, she has anomalous venous in the cerebellum and reduction of parietal regions bilaterally with pial angiomatosis. On physical exam, she presented bilateral nevus flammeus on face. Best corrected visual acuity (BCVA) was hand motion in the OD and 20/25 in the OS. The fundoscopy of OD revealed extensive serous retinal detachment (SRD) and in the OS a reddish elevated area in the macula, without retinal detachment or macular edema. Ultrasonography (US) of OD presented a solid lesion with homogeneous high reflectivity and regular surface, suggestive of diffuse choroidal hemangioma, associated with total SRD. No disturbances were observed in the OS exam but the fluorescein angiography confirmed circumscribed choroidal hemangioma. The lesion of the OS was evaluated with DRI OCT (Triton, Topcon). The OCTA in the "flow image" of the choriocapillar showed a larger area of hyporeflectivity and smaller areas of hyporeflectivity. However the OCTA in face "intensity image" discards attenuation artifact. The structural B-scan OCT showed an elevated lesion in the choroid with thinning of the outer nuclear layer and loss of differentiation of the external layers of the retina. The patient underwent external beam radiotherapy on the OD with a dosage of 36 Gy, 20 applications of 180 cGy. After 2 months, she had BCVA maintenance in both eyes and the same findings at ophthalmoscopy observed in the first consultation. The control US of OD showed a reduction of height with smilar characteristics of the lesion compared to the previous test.

Conclusão

This is a rare SWS presentation, diffuse choroidal hemangioma in one eye and circumscribed in the other, since there is only one similar case reported.