- Department of Head and Neck Surgery, Neurosurgery Unit, Garibaldi Hospital, Catania, Italy
- Department of Experimental Biomedicine and Clinical Neurosciences, School of Medicine, Postgraduate Residency Program in Neurological Surgery, Neurosurgical Clinic, AOUP “Paolo Giaccone”, Palermo, Italy
- Department of Radiology, ARNAS Garibaldi, Catania, Italy
- Department of Neurosurgery, Cannizzaro Hospital, Catania, Italy.
Gianluca Scalia, Department of Head and Neck Surgery, Neurosurgery Unit, Garibaldi Hospital, Catania, Italy.
DOI:10.25259/SNI_82_2023Copyright: © 2023 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Gianluca Scalia1, Massimiliano Porzio2, Roberta Costanzo2, Domenico Gerardo Iacopino2, Gianluca Galvano3, Giovanni Federico Nicoletti1, Giuseppe Emmanuele Umana4. Spontaneous spinal epidural hematoma in a pediatric hemophiliac. 24-Feb-2023;14:69
How to cite this URL: Gianluca Scalia1, Massimiliano Porzio2, Roberta Costanzo2, Domenico Gerardo Iacopino2, Gianluca Galvano3, Giovanni Federico Nicoletti1, Giuseppe Emmanuele Umana4. Spontaneous spinal epidural hematoma in a pediatric hemophiliac. 24-Feb-2023;14:69. Available from: https://surgicalneurologyint.com/surgicalint-articles/12169/
Background: Spontaneous spinal epidural hematomas (SSEH), unrelated to trauma, epidural anesthesia, or surgery, are rare in the pediatric population. Here, a 1-year-old male with hemophilia presented with a magnetic resonance (MR)-documented SSEH and was successfully treated with a C5-T10 right hemilaminectomy.
Case Description: A 1-year-old male with hemophilia presented with quadriparesis. The holo-spine magnetic resonance imaging with contrast showed a posterior cervicothoracic compressive epidural lesion extending from C3 to L1 consistent with an epidural hematoma. He underwent a C5 to T10 right-sided hemilaminectomy for clot removal, following which his motor deficits fully resolved. A literature review of SSEH attributed to hemophilia revealed that 28 of 38 cases were effectively treated conservatively, while only 10 cases warranted surgical decompression.
Conclusion: Select patients with SSEH attributed to hemophilia with severe MR-documented cord/cauda equina compromise and significant accompanying neurological deficits may require emergent surgical decompression.
Keywords: Epidural, Hematoma, Hemophilia, Pediatric, Spine
Spontaneous spinal epidural hematomas (SSEH) that develop in the extradural space without accompanying histories of trauma, epidural anesthesia, or surgery represent <1% of all space-occupying spinal lesions. In adults, SSEH occurs with a frequency of 0.1 cases/100,000/year, and account for 0.6% of all spinal hemorrhages; in children, their incidence remains unclear.[
A 1-year-old male with hemophilia A (i.e., severe deficit of factor VIII) presented with the acute and spontaneous onset of severe quadriparesis. Laboratory tests revealed a low hemoglobin (8.5 g/dL), low hematocrit (25.6%), a mildly elevated international normalized ratio (1.34) and partial thromboplastin time (37 s), with reduced prothrombin activity (65 s). The holo-spine magnetic resonance (MR) with contrast showed a large cervicothoracic posterior epidural compressive lesion extending from C3 to L1 that was consistent with an epidural bleed; it was hypointense on T1, and hyperintense on T2-weighted images [
Hemophilia is one of the most common hereditary X-linked bleeding disorders attributed to a deficiency of factor VIII. Some patients with hemophilia acutely develop SSEH and present with the rapid onset of paralysis correlated with the levels of MR-documented spinal hemorrhages.[
Most adults with SSEH will undergo decompressive laminectomies. However, children may occasionally be managed with less extensive partial laminectomies or single/multiple short-segment laminoplasties.[
A 1-year-old male with hemophilia presented with acute quadriparesis attributed to a MR-documented C5-T10 thoracic SSEH resulting in marked cord compression. Following a right-sided C5-T10 hemilaminectomy, the patient was neurologically intact and remained intact for 5 subsequent years during which he developed no spinal deformity.
There is no ethical issue in this paper.
Patient’s consent not required as patient’s identity is not disclosed or compromised.
There are no conflicts of interest.
The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.
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