Beta Thalassemia Major as a Rare Cause of Spontaneous Spinal Epidural Hemorrhage
Spontaneous spinal epidural hematoma (SSEH) is a rare neurosurgical emergency with paucity of data in understanding the pathogenesis. SSEH can be devastating to the patients as it causes permanent paraplegia if not treated in a timely manner. SSEH has been described in various coagulopathic disorder...
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| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Wolters Kluwer Medknow Publications
2025-01-01
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| Series: | Indian Spine Journal |
| Subjects: | |
| Online Access: | https://doi.org/10.4103/isj.isj_56_23 |
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| Summary: | Spontaneous spinal epidural hematoma (SSEH) is a rare neurosurgical emergency with paucity of data in understanding the pathogenesis. SSEH can be devastating to the patients as it causes permanent paraplegia if not treated in a timely manner. SSEH has been described in various coagulopathic disorders but there have been no case reports of spontaneous epidural hematoma occurring in patients with beta thalassemia. We present a case report illustrating clinical features, magnetic resonance imaging findings, operative intervention, and outcome after surgery in a 17-year-old boy with beta thalassemia major, who presented to us with epidural hematoma in dorsolumbar spine, requiring emergency long segment laminotomy and evacuation of the epidural clot. The operative procedure resulted in a good outcome over a period of 6 months. We have proposed pathophysiology of formation of SSEH in beta thalassemia patients. The pathophysiology behind the hemorrhagic manifestation is not completely understood and requires further studies. Timely diagnosis is important to avoid life-threatening morbidity or mortality in such patients because surgical decompression with physiotherapy leads to good outcomes. |
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| ISSN: | 2589-5079 2589-5087 |