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| CASE REPORT |
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| Year : 2021 | Volume
: 12
| Issue : 2 | Page : 112-114 |
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Spontaneous spinal epidural hematoma in a known case of hemophilia a managed conservatively: A rare case report
Ashish Acharya, Ravindra Kumar Bind, Sarvpreet Singh Grewal, Paul Sudhakar John
Department of Neurosurgery, Christian Medical College and Hospital, Ludhiana, Punjab, India
| Date of Submission | 11-Sep-2020 |
| Date of Decision | 11-Sep-2020 |
| Date of Acceptance | 25-Sep-2020 |
| Date of Web Publication | 06-Aug-2021 |
Correspondence Address:
Dr. Ashish Acharya
Department of Neurosurgery, Christian Medical College, Ludhiana, Punjab
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/joah.joah_137_20
Spontaneous spinal extradural hematomas are rarely attributed to hemophilia. When it occurs, magnetic resonance imaging (MRI) best establishes the diagnosis that should prompt immediate medical or surgical management. Here, we present a case with large spinal epidural hematomas (EDH) which was managed conservatively with good neurological outcome. A 12-year-old male who is a known case of hemophilia A was admitted with a complaint of neck pain, was neurologically intact on examination with no motor or sensory deficits. MRI cervical spine revealed a large spinal EDH in the cervical region compressing the cord posteriorly. The patient was managed conservatively with tranexamic acid and factor VIII replacement and observed over few days. He recovered well with interval resolution of hematoma. this case depicts the role of conservative management of spontaneous EDH in patients with Hemophilia A utilizing factor replacement therapy, steroids, and tranexamic acid and emphasizes that surgery is not always required even in such radiologically large appearing lesions.
Keywords: Hemophilia A, spinal epidural hematoma, tranexamic acid
How to cite this article:
Acharya A, Bind RK, Grewal SS, John PS. Spontaneous spinal epidural hematoma in a known case of hemophilia a managed conservatively: A rare case report. J Appl Hematol 2021;12:112-4 |
How to cite this URL:
Acharya A, Bind RK, Grewal SS, John PS. Spontaneous spinal epidural hematoma in a known case of hemophilia a managed conservatively: A rare case report. J Appl Hematol [serial online] 2021 [cited 2023 Mar 27];12:112-4. Available from: https://www.jahjournal.org/text.asp?2021/12/2/112/323323 |
| Introduction | |  |
Spontaneous spinal epidural hematomas (EDH) rarely occur with Hemophilia A. In fact, central nervous system hemorrhages are found in only 2%–8% of children with hemophilia, and <10% are intraspinal.[1] Urgent diagnosis with magnetic resonance (MR) is the key to successful management with conservative modalities to avoid rapid expansion of hematomas to the point where they warrant surgery.
| Case Report | | |
This is the case of a 12-year-old male with hemophilia A, diagnosed at the age of 18 months, with 5 days of cervical pain, and the inability to extend his neck. On examination, he exhibited the onset of a mild myelopathy (motor preservation 5/5, normal sensory function) characterized by diffuse lower extremity hyperactive reflexes (3+) with bilateral Babinski signs, consistent with an ASIA SCORE of “E.”
The patient did not have any first-degree relatives with hemophilia A. The patient has been receiving factor replacement therapy intermittently. Three months previously, he had received a Factor VIII transfusion.
On examination, the patient showed exaggerated reflexes in bilateral lower extremities and upgoing plantars with motor and sensory preservation on neurological examination.
Magnetic resonance cervical spine
A cervical-spine MRI was done which revealed a large heterogeneous hyperintense posterior epidural lesion, extending from C2 to D8 resulting in marked cord compression. There were also milder posterior epidural signal changes involving the upper lumber epidural compartment. Hyperintensities were also seen in the intra-spinous and supraspinous soft tissues, typical of acute blood [Figure 1]a and [Figure 1]b. | Figure 1: T1(1a) and T2(1b) -weighted imaging on magnetic resonance imaging of cervical spine showing hetero-intense foci involving the posterior epidural space, extending from C2 to D8 level, with milder signal changes in the upper lumber posterior epidural space. It was seen to cause severe narrowing of the spinal canal with significant cord compression
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Hematological evaluation
The hematological investigations revealed a negative inhibitor screen (Inhibitor Screen Fresh Mix (APTT seconds): 44.9 Inhibitor Screen Incubated Mix (APTT seconds): 45.4, normal platelet count.
Diagnosis in such scenarios should be prompt as any delay may result in permanent disabling deficits and resultant poor quality of life. Access to MRI should be available and one must maintain a high index of suspicion for hemorrhagic sequalae.
Conservative management
In consultation with the hematology department, the patient was started on factor VIII replacement therapy (i.e., dose of 1000 Units OD for 2 days, then 500 units bid for 6 days, and reduced to 250 units OD for last 2 days). The patient was also given tranexamic acid (i.e., dose of 500 mg 8 hourly which was continued at discharge). Further, dexamethasone was started at a dose of 2 mg q8 h, and tapered over 7 days. The patient was at complete bed rest for 7 days, and the neurological status was monitored closely.
Outcome
The cervical pain improved, while within 3 days, the tone in the left leg normalized (i.e., the left sided Babinski resolved). The follow-up MRI performed 7 days later showed near-total resolution of EDH with resolved cord compression [Figure 2]a and [Figure 2]b. Prothrombin time international normalised ratio and factor VIII levels were monitored regularly. The patient was discharged on the 9th day and was neurologically fully intact. Oral tranexamic acid was continued on discharge and the patient was observed to be doing well at last follow-up, 4 month later. | Figure 2: T1(2a) and T2(2b)-weighted imaging on magnetic resonance imaging of cervical spine depicting interval resolution of heterogeneous signal changes and compression of thecal sac
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| Discussion | | |
Hemophilia is the most common hereditary bleeding disorder, with an incidence of 0.7–0.8/10,000.[2] Spinal EDH are rare in patients with Hemophilia A. Patients typically present with the acute onset of neck and/or back pain and paresis, followed by sensory deficits as well as bowel or bladder dysfunction. However, subset with spinal EDH may, if caught early enough, be treated conservatively. MR studies are best at determining the size, location, and severity of EDH to determine whether nonsurgical versus surgical management is warranted. Availability of MRI is hence crucial to such diagnosis, and functional neurosurgical unit at standby in the case of surgery needed for deterioration/expansion of hematoma.
Evaluation of medical therapy
Medical management includes replacement of deficient factors.[3] Kiehna et al.[1] studied pediatric hemophilia patients with spinal EDH and found that that observation and correction of coagulopathy was safe and effective for those with stable neurological examinations. They also concluded that this required multidisciplinary approach to maximize safety/efficacy (e.g., hematologist, neurosurgeon, and pediatric intensivist to ensure timely correction of the coagulation disorder; maintenance of adequate factor levels; and close hemodynamic and neurological monitoring). Kubota and Miyajima,[4] concluded that unless the neurological deficiency is progressing rapidly, nonsurgical management is safe for these patients versus attempting high-risk surgical management without adequate management of their coagulation status.
Connelly et al.[5] state that coagulopathy-induced spinal bleeds allow for conservative treatment, as hematoma remains in liquid form for a longer time compared with cases with normal clotting, enabling spread of the hematoma in the spinal epidural space. However, in cases with prolonged bleeding, one might expect a larger hematoma to occur, with more extensive compression of the neural structures.
Crabbe et al. further suggested that immobilization of the neck and administration of steroids might result in rapid improvement of the neurological deficit in cervical SSEH.[6]
| Conclusion | | |
For patients with hemophilia who develop acute spinal pain and neurological deficits, emergent MR studies will help determine whether conservative management (i.e., coagulation factor replacement therapy, steroids) or surgery is warranted to avoid permanent injury.
Hereby, this case depicts the role of conservative management of spontaneous EDH in patients with hemophilia A utilizing factor replacement therapy, steroids, and tranexamic acid and emphasizes that surgery should not always be rushed for even in such radiologically large appearing lesions until the patient is neurologically intact.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Kiehna EN, Waldron PE, Jane JA. Conservative management of an acute spontaneous holocord epidural hemorrhage in a hemophiliac infant. J Neurosurg Pediatr 2010;6:43-8.  |
| 2. | Agrawal D, Mahapatra AK. Spontaneous subdural hematoma in a young adult with hemophilia. Neurol India 2003;51:114-5. [ PUBMED] [Full text] |
| 3. | Narawong D, Gibbons VP, McLaughlin JR, Bouhasin JD, Kotagal S. Conservative management of spinal epidural hematoma. Pediatr Neurol 1988;4:169-71. |
| 4. | Kubota T, Miyajima Y. Spinal extradural haematoma due to haemophilia A. Arch Dis Child 2007;92:498. |
| 5. | Connelly ES, Winfree CJ, McCormick PC. Management of spinal epidural hematoma after tissue plasminogen activator. Spine 1996;21:1694-8. |
| 6. | Crabbe DC, Mendelow AD, Pharoh P. Cervical spinal extradural hamatoma causing a transient Brown-Sequard syndrome. JNNP 1992;55:239. |
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