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Role of Diffusion MRI Tractography in Endoscopic Endonasal Skull Base Surgery
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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.

The Journal of Craniofacial Surgery
|July 10, 2014
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Summary

Hemophilic pseudotumor, a rare complication of hemophilia, can occur in the skull. Surgical removal with factor replacement therapy offers a safe and effective treatment for cranial hemophilic pseudotumors.

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Area of Science:

  • Hematology
  • Neurosurgery
  • Radiology

Background:

  • Hemophilic pseudotumors are rare complications of hemophilia, arising from recurrent hemorrhage and hematoma enlargement.
  • Patients with severe hemophilia have a 1-2% chance of developing pseudotumors, which can cause bone destruction.
  • Cranial hemophilic pseudotumors are exceptionally rare, with only seven cases reported previously, typically in mild or moderate factor deficiencies.

Observation:

  • A 42-year-old male with mild factor VIII deficiency presented with a bilateral cranial pseudotumor.
  • Imaging revealed an extra-axial lesion with bone destruction and signs of chronic hemorrhage.
  • Histological examination confirmed old blood coagulum.

Findings:

  • The patient underwent surgical removal after receiving adequate factor VIII replacement therapy.
  • No recurrence was observed during a 3-year follow-up period.
  • This case highlights the successful management of an extremely rare cranial hemophilic pseudotumor.

Implications:

  • Cranial hemophilic pseudotumors, though rare, require prompt diagnosis and management.
  • Adequate factor replacement therapy is crucial for successful surgical intervention.
  • Surgical management, combined with factor therapy, is a safe and effective treatment for cranial hemophilic pseudotumors.