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Intraspinal Hematomas in Hemophilia

We report herein a case of a intraspinal hematoma in a 9-year-old boy with factor IX deficiency. Replacement of factor IX resulted in resolution of symptoms. The most frequent presentations of intraspinal hematomas are neck or back pain, paresis, sensory impairment, and urinary retention. Intraspinal hematomas may have devastating sequelae, including hemiplegia and quadriplegia. The occurrence or development of sequelae are related to the length of time between onset of symptoms and factor replacement. Whenever the physician suspects intraspinal hematoma, immediate replacement should be given to obtain levels of 80–100% prior to any imaging studies. Factor levels should be maintained at 30–50% for 10–14 days while the patient is monitored closely with serial neurological examinations. Most patients respond to factor replacement, but laminectomy should be considered for intractable or progressive cases.