ABSTRACT
Intraorbital arteriovenous fistula is a rare vascular disease characterized by an acquired arteriovenous communication without direct cavernous sinus involvement. Intraorbital arteriovenous fistula may develop slowly and present similarly to other insidious orbitopathies, such as carotid-cavernous fistula. The authors present a case of a superficial temporal artery to superior ophthalmic vein fistula arising in the absence of trauma or prior surgery. This is the first report of a spontaneous intraorbital arteriovenous fistula arising between these vessels and further describes the rare occurrence of intraorbital arteriovenous fistula.
Subject(s)
Arteriovenous Fistula/diagnosis , Cavernous Sinus/abnormalities , Intracranial Arteriovenous Malformations/diagnosis , Temporal Arteries/abnormalities , Aged, 80 and over , Arteriovenous Fistula/therapy , Cerebral Angiography , Embolization, Therapeutic/methods , Female , Humans , Intracranial Arteriovenous Malformations/therapy , Tomography, X-Ray ComputedABSTRACT
PURPOSE: To assess the potential ocular toxicity of a combined BRAF inhibition (BRAFi) + MEK inhibition (MEKi) + hydroxychloroquine (HCQ) regime used to treat metastatic BRAF mutant melanoma. METHODS: Patients with stage IV metastatic melanoma and BRAF V600E mutations (n = 11, 31-68 years of age) were included. Treatment was with oral dabrafenib, 150 mg bid, trametinib, 2 mg/day, and HCQ, 400 mg to 600 mg bid. An ophthalmic examination, spectral domain optical coherence tomography, near-infrared and short-wavelength fundus autofluorescence, and static perimetry were performed at baseline, 1 month, and q/6 months after treatment. RESULTS: There were no clinically significant ocular events; there was no ocular inflammation. The only medication-related change was a separation of the photoreceptor outer segment tip from the apical retinal pigment epithelium that could be traced from the fovea to the perifoveal retina noted in 9/11 (82%) of the patients. There were no changes in retinal pigment epithelium melanization or lipofuscin content by near-infrared fundus autofluorescence and short-wavelength fundus autofluorescence, respectively. There were no inner retinal or outer nuclear layer changes. Visual acuities and sensitivities were unchanged. CONCLUSION: BRAFi (trametinib) + MEKi (dabrafenib) + HCQ causes very frequent, subclinical separation of the photoreceptor outer segment from the apical retinal pigment epithelium without inner retinal changes or signs of inflammation. The changes suggest interference with the maintenance of the outer retinal barrier and/or phagocytic/pump functions of the retinal pigment epithelium by effective MEK inhibition.
