Haemophilic pseudotumour of the carotid artery

Panel A

A 62-year-old Greek man was referred for right carotid endarterectomy for symptomatic internal carotid artery stenosis (> 70%) after recovering from a mild right hemispheric infarct 2 weeks previously. The patient was known to have moderate haemophilia B (factor IX deficiency). A conventional right carotid endarterectomy, using a Dacron patch for closure, was performed under general anaesthesia with shunt placement. Recombinant factor IX replacement (BeneFIX^®; Wyeth Pharmaceuticals Inc.) was commenced with an intravenous infusion of 5000 IU with additional continuous infusion of 6 IU/kg/h (2.1 ml/h) intraoperatively and for 48 hours postoperatively. Postoperative recovery was unremarkable. Three weeks later, the patient presented with a right-sided neck swelling immediately deep to the surgical wound. On palpation the swelling was firm and tender. Duplex ultrasonography showed a 4 cm diameter haematoma compressing the external carotid artery (Panel A). The patient was managed conservatively with observation. Eight weeks later, the patient presented in follow-up with progression of the neck mass to a size of 13 cm (length) by 12 cm (width) by 6 cm (depth). Computed tomographic angiography showed evidence of a pseudotumour arising from the right internal carotid (Panel B, arrows). It was decided to surgically excise the pseudotumour under continuous factor IX cover. Incision through the previous endarterectomy scar revealed the large pseudotumour arising from the internal carotid artery (Panel C, 1). Histology showed dense adherent thrombus composing the wall of the haemophilic pseudotumour (Panel C, 2). The bifurcation of the common carotid artery was dissected out from dense adhesions, and three-vessel control obtained. The tumour was opened and the contents evacuated. Postoperative recovery was uneventful and no recurrence was noted at 6 months.

Panel B Panel C

Haemophilic pseudotumour is a rare but well-documented complication of haemophilia.^1,2 Recurrent episodes of haemorrhage into an encapsulated haematoma result in an enlarging mass, which can compress local structures. Most haemophilic pseudotumours arise from the skeleton, ² but we present a unique report of a carotid pseudotumour. Surgical excision provides curative treatment.^3,4

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