Utilidad de la imagen multimodal en el diagnóstico de las complicaciones del síndrome de Grönblad – Stranberg

Resumo

Introduction: The elastic pseudoxanthoma (EPX) is a systemic disease characterized by the progressive degeneration of the elastic fibers from the connecting tissue. The ocular injuries most frequently associated with EPX are the angioid streaks (AS) which form the Grönblad –Strandberg syndrome, that can complicate developing a choroidal neovascularization (CNV). New image-based diagnose tests, applied in combination; allow us a better knowledge about the physiopathology, monitoring of the illness development, as well as the reaction to the treatment (2-5). Clinical case: We present the case of a 56-year-old woman, diagnosed with EPX, referred to us with metamorphopsias in her right eye (RE) for 1 week. Her visual acuity (VA) was 0.5 in the RE. In the funduscopic examination, angioid streaks were found, associated to a hemorrhage in the macular area. The Optical Coherence Tomography (OCT) showed an image that suggests a macular CNV and subretinal liquid, while the autofluorescence (AF) confirmed the existence of a hipoautofluorescent injury with hiperautofluorescent borders. Finally, a OCT- Angiography (OCT-A) was carried out, in which we could identify the neovascular formation. The patient was treated with intravitreous. Ranibizumab. Conclusions: The use of multimodal fundus images in patients suffering from PXE allows a more precise and objective study of the ophthalmological findings. It helps us identify possible complications, and in necessary situations, to establish an appropriate treatment