Spontaneous bleeding from external ear canal as a presenting symptom in a child with von Willebrand disease

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Summary 

Type 1 von Willebrand Disease is caused by a deficiency of von Willebrand factor and most commonly results in epistaxis, easy bruising, and postoperative bleeding. In this case, we reported an infant with von Willebrand Disease who presented with spontaneous bleeding from external ear canal and PT, PTT and bleeding time values within normal range.

Keywords: von Willebrand disease, Bleeding, External ear canal

A 5-month-old male infant presented with the 3 days-history of bleeding from nose and right ear. He had been previously evaluated by ENT clinic for recurrent nose and ear bleeding, and suggested as normal. Parental history for bleeding diathesis was unremarkable. Physical examination was unremarkable except a clot in the right ear canal. Laboratory evaluation demonstrated hemoglobin of 11g/dL and platelet of 386,000mm⁻³. Prothrombin time, activated partial thromboplastin time, bleeding time, AST and ALT values were in normal range. Factor XIII deficiency was eliminated by urea clot lysis assay. Platelet aggregation tests by collagen, adenosine diphosphate and ristocetin were normal. Bleeding from right ear canal repeated four times a week. His mother denied any history of trauma. There was no lesion leading to bleeding on repeated otoscopic examination. On day 8, his von Willebrand factor antigen (vWF Ag) and ristocetin cofactor activity (vWF R:Co) were reported as 3 and 1%, respectively. The diagnosis was made as von Willebrand Disease (vWD).

vWD is the most common hereditary bleeding disorder caused by a deficiency (type 1), dysfunction (type 2) or complete absence (type 3) of vWF. Deficiency of vWF most commonly results in epistaxis, easy bruising and postoperative bleeding [1], [2]. However, spontaneous bleeding from external ear tract as a presenting symptom was not previously reported in children with vWD. Hemostatic screening studies including PT, PTT and bleeding time may be normal in type 1 disease. Hence, it s important to demonstrate the plasma levels of vWF (vWF Ag and vWF R:Co) in children with a clinical bleeding history [1].

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References 

1. Lillicrap D, Dean J, Blanchette VS. von Willebrand disease. In:  Lilleyman J,  Hann I,  Blanchette V editor. Pediatric Hematology. second ed.. China: Churchill Livingstone; 2000;p. 601–609
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2. Ziv O, Ragni MV. Bleeding manifestations in males with von Willebrand disease. Haemophilia. 2004;10:162–168
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