In the article by Pollack et al1 on "Ocular Complications in Ehlers-Danlos Syndrome Type IV," the authors noted ocular complications from an attempted repair on a carotid-cavernous sinus fistula. Their conclusion stated that the diagnosis of Ehlers-Danlos syndrome type IV should be "considered in any young or middle-aged patient seen with spontaneous direct CCF [carotid-cavernous sinus fistula]." We have recently seen a patient with Ehlers-Danlos syndrome type IV and suggest that subretinal hemorrhage should be considered as a diagnosis in these patients when a mass in the choroid simulating a melanoma is detected.
We examined a 45-year-old man with Ehlers-Danlos syndrome type IV who had a subconjunctival hemorrhage and an intraocular mass at the region of the equator (Figure 1). The mass itself measured 7.5×8.0 mm, was 3.9 mm high, and exhibited medium to high reflectivity on ultrasonography (Figure 2) A fluorescein angiogram was not obtained because of the Ehlers-Danlos
Abramson DH, Servodidio CA, Giovinazzo V, Pullman M, Budinger KS. Pseudomelanoma in Ehlers-Danlos Syndrome Type IV. Arch Ophthalmol. 1997;115(9):1216. doi:10.1001/archopht.1997.01100160386032