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Bilateral cranial hemophilic pseudotumor
Qingsheng Xu1, Pan Wu, Yiping Feng
1From the Department of Neurosurgery, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou Zhejiang Province, People's Republic of China.
Hemophilic pseudotumor is a rare but well-known complication of hemophilia manifesting as recurrent hemorrhage and progressive enlargement of hematoma. A patient with severe hemophilia has 1% to 2% chance to develop pseudotumor. The chronic pressure of osseous hemorrhage usually results in bone destruction or resorption. Cranial hemophilic pseudotumors are extremely rare, with only 7 reported cases associated with mild or moderate factor VIII or IX deficiency. A 42-year-old man with a mild factor VIII deficiency developed a pseudotumor of the bilateral skull. Computed tomography and magnetic resonance imaging revealed an extra-axial lesion with bone destruction, and signal changes are consistent with chronic hemorrhage. With adequate factor-deficient replacement therapy, surgical removal was performed. Histologic examination disclosed old blood coagulum. No recurrence was observed in 3 years of follow-up. Cranial hemophilic pseudotumor is extremely rare, and with adequate factor-deficient replacement therapy, surgical management is a safe and effective way for cranial hemophilic pseudotumor treatment.
Hemophilic pseudotumor is a rare but well-known complication of hemophilia manifesting as recurrent hemorrhage and progressive enlargement of hematoma. A patient with severe hemophilia has 1% to 2% chance to develop pseudotumor. The chronic pressure of osseous hemorrhage usually results in bone destruction or resorption. Cranial hemophilic pseudotumors are extremely rare, with only 7 reported cases associated with mild or moderate factor VIII or IX deficiency. A 42-year-old man with a mild factor VIII deficiency developed a pseudotumor of the bilateral skull. Computed tomography and magnetic resonance imaging revealed an extra-axial lesion with bone destruction, and signal changes are consistent with chronic hemorrhage. With adequate factor-deficient replacement therapy, surgical removal was performed. Histologic examination disclosed old blood coagulum. No recurrence was observed in 3 years of follow-up. Cranial hemophilic pseudotumor is extremely rare, and with adequate factor-deficient replacement therapy, surgical management is a safe and effective way for cranial hemophilic pseudotumor treatment.
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