Droplet-Shaped Deep Intraretinal Hemorrhage as Initial Presentation of Von Willebrand Disease Type 1

Von Willebrand disease (VWD) is characterized by bleeding symptoms that result in easy bruising, skin bleeding, prolonged bleeding from mucosal surfaces, heavy menstrual bleeding, and serious bleeding from the gastrointestinal tract. Early diagnosis and treatment are life-saving. This report is on a 47-year-old Taiwanese male with spontaneous, unilateral, droplet-shaped deep intraretinal hemorrhage. The patient presented in this case had no prior history of trauma, bleeding tendency, or coagulopathy. A diagnosis of VWD type 1 was made after von Willebrand multimer analysis. This is the first case reported to have intraretinal hemorrhage only as the first manifestation of VWD type 1. This report demonstrates that patients with spontaneous retinal hemorrhage should be evaluated for a possible bleeding disorder. Detailed clinical history and ophthalmologic examination can lead to the early diagnosis of VWD, before serious any complications occur. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:1044-1048.].

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