Vitreous, retinal and subretinal hemorrhages associated with von Willebrand's syndrome

Abstract

Two patients with von Willebrand's syndrome had intraocular hemorrhages without trauma or neovascularization. A 13-year-old Japanese girl complained of sudden spotted vision in her left eye. Vitreous, peripapillary retinal, and subretinal hemorrhages were seen by ophthalmoscopy in the affected eye. In addition, a 19-year-old Japanese man had repeated vitreous hemorrhage and epistaxis. Also noted was gyrate atrophy of the choroid and retina. Analysis of the coagulation-fibrinolysis system revealed decreased activities of Factor VIII and von Willebrand's factor in both patients. The amount of von Willebrand's factor antigen also was decreased. These results suggested that the patients had von Willebrand's syndrome. The bleeding tendency in the disorder appeared to be responsible for the pathogenesis of the intraocular hemorrhages seen in these patients.

Massive subhyaloidal hemorrhage associated with severe PAI-1 deficiency

“…This observation compares favorably with the results of Lee et al [3], who demonstrated a clear correlation between decreased plasma PAI-1 activity and hyperfibrinolytic bleeding. Moreover, the present case is in accordance with the findings of Shiono and coworkers, who reported on an association of spontaneous retinal hemorrhages in patients with von Willebrand disease [11], the most common congenital bleeding disorder.…”

“…ethanol, aspirin, warfarin) [5]. Interestingly, there have been a few reports on an association with hematologic disorders [1,11], including anemia. Patients with this condition have a substantially elevated risk of intraretinal hemorrhages or retinal vein occlusion [4].…”

Von Willebrand’s disease type I as a cause for subvitreal, retinal and subretinal haemorrhages

“…Although only two cases of intraocular bleedings [7] associated with VWD have been described in the literature, it might be expected that these intraocular bleedings happen more often and remain undiagnosed. The patient in our case report is the first non-Japanese patient described with subvitreal, retinal and subretinal haemorrhages associated with VWD.…”

“…Up to now, only two patients with retinal and vitreous haemorrhages associated with VWD have been described [7]; both were younger than 20 years. One patient was a 13-year-old Japanese girl with spontaneous unilateral vitreous, retinal and subretinal haemorrhages without prior history of epistaxis or recurrent haemorrhages.…”

“…The patient in our case report is the first non-Japanese patient described with subvitreal, retinal and subretinal haemorrhages associated with VWD. Up to now, she is also the patient with the latest onset of the first episode of subvitreal, retinal and subretinal haemorrhages (age 33 years as compared to 8 years or 13 years, respectively [7]). Finding the cause of vitreal, retinal and subretinal haemorrhages is a difficult task for every ophthalmologist.…”