EPIDURAL HEMATOMA OF THE CAUDA-EQUINA IN A CHILD WITH HEMOPHILIA-A

Purpose: The presenting signs, treatment, and outcome of an epidural h ematoma of the cauda equina in a child with severe hemophilia are repo rted for the first time. Patients and Methods: A 20-month-old boy with severe hemophilia A (factor VIII < 0.01 U/ml) presented with a 12-day history of refusal to stand and constipation of 5-7 days duration. He had normal deep tendon reflexes with normal sensation and withdrawal to pinprick of his lower extremities bilaterally. He stood on his righ t leg, but had inversion of his left foot and refused to bear weight o n his left leg. MRI revealed an epidural hematoma of the cauda equina and a distended bladder. Factor VIII replacement therapy and lumbosacr al laminectomy with evacuation of the hematoma resulted in recovery of a normal gait, but bladder dysfunction persisted for 11 months. Clean intermittent catheterization (CIC) was required until bladder functio n returned. Results: Complete neurologic recovery occurred 11 months a fter presentation. Conclusions: This case demonstrates the following p oints: (a) an epidural hematoma of the cauda equina in a child with se vere hemophilia can present with neurologic findings that are as subtl e as those seen in normal children; (b) CIC can be performed safely ov er an extended period without factor VIII replacement; and (c) complet e recovery is possible, despite prolonged bladder dysfunction and a 12 -day interval between the onset of symptoms and treatment.