EC Paediatrics

Von Willebrand disease (VWD) is the most common inherited bleeding disorder, affecting almost 1% of the general population. Type 3 VWD is the most severe form, with severe bleeding phenotype that usually presents with mucocutaneous and other serious bleeding. Ocular bleeding, however, is rare in VWD, and hereby we report a three-year-old girl with type 3 VWD who developed traumatic hyphema; an extremely rare presentation. The child presented 2 days after blunt trauma to her left eye with periorbital ecchymosis, subconjunctival hemorrhage, total hyphema, loss of vision together with high intraocular pressure. She was managed conservatively with bed rest, eye shielding, VWF/FVIII concentrate infusion, tranexamic acid and topical treatment for high IOP. Initial improvement was followed by rebleeding with worsening hyphema in addition to new intravitreal hemorrhage. Her management was challenging in view of lack of cooperation, poor compliance with topical treatment, and the need to optimize blood tests for factor VIII and VWF recovery and trough levels. Her serious eye bleeding necessitated long term prophylaxis with VWF/FVIII concentrate. This case highlights the importance of early recognition, timely hemostatic intervention, and coordinated multidisciplinary management to prevent vision-threatening complications.

 Keywords: Von Willebrand Disease; Traumatic Hyphema; Case Report

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