Cuvelier G D E, Davis J H, Purves E C, Wu J K
Division of Pediatric Hematology, Oncology and BMT, British Columbia Children's Hospital, Vancouver, BC, Canada.
Haemophilia. 2006 Nov;12(6):683-6. doi: 10.1111/j.1365-2516.2006.01323.x.
We describe the case of a spinal epidural haematoma in an infant with severe haemophilia A. Initial signs and symptoms were non-specific resulting in delay of the diagnosis and more definitive therapy. The patient eventually developed torticollis, acute flaccid paralysis of the upper extremities, and respiratory distress, prompting radiological examination of the spinal cord. The patient was treated with recombinant FactorVIII and laminectomy. Neurological recovery was complete 3 months following the event. We hypothesize that infants with haemophilia may be at higher risk for this rare complication because of their increasing mobility, frequent falls while cruising furniture, and lack of prophylactic factor replacement. Non-specific signs such as irritability without a focus should alert the clinician to this diagnostic possibility. Torticollis should prompt rapid radiological evaluation of the cervical spine with magnetic resonance imaging to avoid delay in diagnosis.
我们描述了一名患有严重甲型血友病的婴儿发生脊髓硬膜外血肿的病例。最初的体征和症状不具特异性,导致诊断和更确切治疗的延迟。患者最终出现斜颈、上肢急性弛缓性麻痹和呼吸窘迫,促使对脊髓进行放射学检查。患者接受了重组凝血因子VIII治疗和椎板切除术。事件发生3个月后神经功能完全恢复。我们推测,血友病婴儿可能因活动能力增强、在扶着家具学步时频繁跌倒以及缺乏预防性凝血因子替代治疗而面临这种罕见并发症的更高风险。诸如无明确病因的烦躁不安等非特异性体征应提醒临床医生考虑这种诊断可能性。斜颈应促使迅速通过磁共振成像对颈椎进行放射学评估,以避免诊断延误。