Exertion-Induced Intrathecal Haematoma in Undiagnosed Von Willebrand Disease: A Case of Acute Spinal Cord Compression

未确诊的血管性血友病患者运动诱发的鞘内血肿:一例急性脊髓压迫病例

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Abstract

Intrathecal haematoma is a rare and potentially devastating cause of acute spinal cord compression. In the absence of trauma, anticoagulation, or a known bleeding disorder, diagnosis can be significantly delayed. This report presents an unusual case of an intrathecal haematoma precipitated by exertion in a previously undiagnosed case of von Willebrand disease. A 28-year-old previously healthy female presented with a sudden onset of thoracolumbar back pain following a chest press exercise. Initial assessment revealed no neurological deficits; however, she declined MRI and blood tests due to severe needle phobia and self-discharged. She re-presented hours later with progressive paraplegia, urinary incontinence, and sensory loss. MRI showed an intrathecal haematoma, prompting emergency surgical decompression. Intra-operative findings confirmed a dense intrathecal haematoma compressing the spinal cord. Coagulation studies postoperatively revealed von Willebrand disease, with prolonged activated partial thromboplastin time and low VWF antigen (31.3), VWF:RiCoF (10), and factor VIII (47.6). The patient achieved near-complete neurological recovery by post-operative day 34 and received factor replacement therapy and enhanced haematological follow-up. This case illustrates how exertion hypothetically may provoke spinal bleeding in individuals with latent coagulopathy, potentially via increased intrathoracic pressure. It also highlights the diagnostic challenges of intrathecal haematomas, particularly in the absence of trauma or known risk factors. Notably, the initial MRI report misclassified the haematoma location, corrected only via interdisciplinary imaging review intra-operatively. Intrathecal haematomas should be considered in the differential diagnosis for acute spinal cord compression, particularly when red flag symptoms are present. A detailed history, high index of suspicion, and dynamic radiological reassessment are critical. Early surgical intervention can lead to excellent neurological outcomes, even in severe cases.

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