Abstract

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

INTRODUCTION

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.[ 19 , 31 , 34 ] Conservative treatment (i.e., infusion of recombinant coagulation factors, corticosteroids) may suffice where there are no major neurological deficits, while surgery is warranted for those with significant paresis.[ 4 , 6 , 8 ] Here, a 1-year-old hemophiliac male with a significant C5-T10 SSEH that contributed to a severe quadriparetic deficit warranted emergent surgical decompression.

CASE DESCRIPTION

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 [ Figures 1a and b ]. An urgent C5-T10 right hemilaminectomy was performed along with the simultaneous administration/infusion of VIII replacement. The 48-h postoperative cervicothoracic magnetic resonance imaging documented complete epidural hematoma removal with no increased signal in the cord (i.e., consistent with the absence of a significant ischemic injury) [ Figure 2 ]. Postoperatively, the factor VIII deficit was confirmed and he was treated with continued VIII factor infusions (i.e., 500 UI twice a day, for a total of 6 days). Five years later, he exhibited no residual deficit and demonstrated no spinal deformity.

Figure 1:

Preoperative holo-spine magnetic resonance imaging showing a large cervicothoracic posterior epidural hematoma, hyperintense in T2-weighted images (a, red arrow in axial plane) and hypointense in T1-weighted images (b, red arrows in sagittal plane).

Figure 2:

Postoperative holo-spine T1-weighted magnetic resonance imaging sequences showing a complete spontaneous spinal epidural hematomas removal and decompression of the spinal cord.

DISCUSSION

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.[ 20 , 22 , 30 , 34 ] Kiehna et al. strongly suggested conservative treatment for SSEH in hemophiliac pediatric patients; they cited their high success rates for medical therapy alone.[ 19 ] Other studies also advocated conservative treatment of SSEH utilizing factor VIII replacement infusions; these measures avoided potential surgical complications.[ 21 ] Further, CarlhanLedermann et al. presented a case of SSEH successfully treated conservatively, but also noted that some patients’ hemorrhages/severe neurological deficits warranted acute surgical intervention.[ 8 ]

Surgery

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.[ 23 ] Notably, in the pediatric population, multilevel surgical decompressions post a greater risk of future instability/ deformity. In our case, the urgent C5-T10 surgery consisted of a right-side partial hemilaminectomy, and the patient, 5 years later, exhibited no deformity. In a review of the literature, 28 of 38 pediatric patients with SSEH were successfully managed conservatively, while only 10 cases warranted surgery [ Table 1 ].[ 1 - 3 , 5 , 7 , 9 - 18 , 24 - 29 , 32 , 33 , 35 - 40 ] Notably, there was just one death (i.e., expiration due to a postoperative complication) and just one other patient who requiring additional surgery to address a recurrent spinal epidural hematoma [ Table 2 ].

Table 1:

Literature review of reported cases of hemophilic pediatric patients with SSEH.

Table 2:

Summary of literature review data.

CONCLUSION

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.

Ethics approval

There is no ethical issue in this paper.

Declaration of patient consent

Patient’s consent not required as patient’s identity is not disclosed or compromised.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Disclaimer

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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