Atraumatic spinal epidural hematoma in an infant with hemophilia A

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INTRODUCTIONSpinal epidural hematoma is a serious entity that occurs only rarely. Hemophiliacs are at increased risk for bleeding in various anatomic sites, including the spinal epidural space. Only a few cases of atraumatic spinal epidural bleeding have been reported in the pediatric population (1–4). Typically, the diagnosis of epidural hematoma depends on the classic clinical triad of localized spine pain, radicular pain, and sensorimotor deficits (2–4). Infants are often unable to localize or express pain in any way except a very general means, making this triad difficult to discern, at best. We present this case of an uncommon entity to further illustrate the difficulties in diagnosis and the resultant delay in management that can occur. A high level of clinical suspicion for bleeding even in atypical locations must be maintained in hemophiliac patients, especially in infants, in whom usual signs and symptoms may be missing.CASEE.S. was a 10-month-old boy who was diagnosed with moderate hemophilia A, with factor VIII levels at 2 to 6% of normal, at 8 months of age. He had had a subgaleal bleed in the neonatal period following vacuum extraction. He had been treated with factor VIII replacement therapy for hemarthroses on three occasions, the last time 1.5 weeks prior to initial presentation. He presented to the emergency department (ED) with a 1-day history of irritability. He refused to stand or crawl at home. He was eating less, but there were no other systemic or focal complaints. There was no history of significant trauma, although developmentally the patient was pulling to a stand and beginning to cruise, with the attendant small falls. The patient had been in his usual good health prior to presentation. On physical examination, the patient was held in his mother’s arms, was alert and interactive with examiners, and his vital signs were stable. It was noted that the patient disliked being supine. No external signs of a bleed were appreciated, and there was no joint or bony tenderness. Abdomen was soft, nondistended, and nontender. The neurologic examination revealed no focal deficits. No meningismus was appreciated. His tone and strength were normal and symmetric in the upper and lower extremities. Deep tendon reflexes were 2+/4, and his toes were down going. Imaging for occult bleed was obtained; computed tomography (CT) of the abdomen and of the head was normal. An abdominal obstruction series was also normal. The patient was discharged with the presumed diagnosis of a viral infection.The patient presented to the ED a second time 4 days later. He had continued irritability, appearing uncomfortable with movement and refusing to be placed on his stomach. He frequently drew his legs up toward his abdomen. There was no documented history of constipation prior to this presentation. He had a low-grade temperature of 99.2° F and had had several loose bowel movements. The neck was supple, although he did not actively move it. Tone, sensation, and strength were normal, and deep tendon reflexes were 1+/4 in the lower extremities. The abdominal examination was guarded, with an impression of abdominal pain. Surgery and Gastroenterology were consulted in the ED. An obstruction series was normal, and an air enema showed no intussusception. The patient was admitted for hydration, pain relief, and further evaluation.After admission, factor VIII replacement therapy was initiated. His admission factor VIII level was 2%. Inhibitors were present at 0.7 Bethesda units. Diagnostic lumbar puncture revealed xanthochromic fluid with a markedly elevated protein of 1500 mg/dL.

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