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: Monali M. Patil, Pandurang Barve, Trimurti D. Nadkarni, Karansinh R. Parve Patil
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2025-01-01
Series:Indian Spine Journal
Subjects:
Online Access:https://doi.org/10.4103/isj.isj_56_23
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author Monali M. Patil
Pandurang Barve
Trimurti D. Nadkarni
Karansinh R. Parve Patil
author_facet Monali M. Patil
Pandurang Barve
Trimurti D. Nadkarni
Karansinh R. Parve Patil
author_sort Monali M. Patil
collection DOAJ
description 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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publishDate 2025-01-01
publisher Wolters Kluwer Medknow Publications
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series Indian Spine Journal
spelling doaj-art-ed9d5e93df1145908cfb96ac69ff7a9c2025-08-20T02:06:43ZengWolters Kluwer Medknow PublicationsIndian Spine Journal2589-50792589-50872025-01-0181818410.4103/isj.isj_56_23Beta Thalassemia Major as a Rare Cause of Spontaneous Spinal Epidural HemorrhageMonali M. PatilPandurang BarveTrimurti D. NadkarniKaransinh R. Parve PatilSpontaneous 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.https://doi.org/10.4103/isj.isj_56_23beta thalassemiaepidural hematomaparaplegia
spellingShingle Monali M. Patil
Pandurang Barve
Trimurti D. Nadkarni
Karansinh R. Parve Patil
Beta Thalassemia Major as a Rare Cause of Spontaneous Spinal Epidural Hemorrhage
Indian Spine Journal
beta thalassemia
epidural hematoma
paraplegia
title Beta Thalassemia Major as a Rare Cause of Spontaneous Spinal Epidural Hemorrhage
title_full Beta Thalassemia Major as a Rare Cause of Spontaneous Spinal Epidural Hemorrhage
title_fullStr Beta Thalassemia Major as a Rare Cause of Spontaneous Spinal Epidural Hemorrhage
title_full_unstemmed Beta Thalassemia Major as a Rare Cause of Spontaneous Spinal Epidural Hemorrhage
title_short Beta Thalassemia Major as a Rare Cause of Spontaneous Spinal Epidural Hemorrhage
title_sort beta thalassemia major as a rare cause of spontaneous spinal epidural hemorrhage
topic beta thalassemia
epidural hematoma
paraplegia
url https://doi.org/10.4103/isj.isj_56_23
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AT pandurangbarve betathalassemiamajorasararecauseofspontaneousspinalepiduralhemorrhage
AT trimurtidnadkarni betathalassemiamajorasararecauseofspontaneousspinalepiduralhemorrhage
AT karansinhrparvepatil betathalassemiamajorasararecauseofspontaneousspinalepiduralhemorrhage