Intracranial Subdural Haemophilic Pseudotumor: Case Report


Hemophilia is an autosomal recessive disease linked to the X chromosome. Type A is the most frequent one and is caused by Factor VIII deficiency [1,2]. Type B has Factor IX deficiency, and is less common. Hemophilic pseudotumors are an infrequent complication of this disease. Starker was the first to report a case in a 14 year old boy, in 1918 [3]. In 1965, Fernandez de Valderrama and Matthews described the condition as a “chronic cystic inflammation of the muscle, caused by recurrent bleedings, and associated with radiological evidence of compromised bone structure” [4,5]. Based on the scarce published information, the estimated incidence is of 1-2% in severe hemophiliacs, although some pseudotumors have been reported in moderate cases [3,4,6-9]. This kind of tumor can be more frequently found in long bones such as femur and humerus, pelvis, and small bones of hands and feet [3,4]. Cases have been reported in the cranium, orbit and facial bones, but always characteristically included bone structures [10,11]. There is only one intracranial case reported in 2009. We present a case of an intracranial subdural hemophilic pseudotumor in a patient with severe factor VIII deficiency.

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@inproceedings{Uberti2017IntracranialSH, title={Intracranial Subdural Haemophilic Pseudotumor: Case Report}, author={Micaela Uberti and Mat{\'i}as Burroni and Lucas Capo and Alejandra I Baques and Patricia R Do scimento and Silvio Grisendi and Mar{\'i}a Sol Serrano and Javier Salazar}, year={2017} }