SuddenCervicalPain: Spontaneous Cervical Epidural Hematoma BERTRAND DEMIERRE, MD,* PIERRE-F. UNGER, MD,t FLAW0 BONGIOANNI, MD* Three cases of cenical epidurai hematoma are reported. Acute neck pain usually associated with a mild effort, closely followed by ndlcular paln and a neurologlcdeficit below the lesion is the typical presentationof this extremely rare and difficult diagnosis. As prognosis depends on preoperative neuroioglcstate, the authorsemphasize the Importance of prompt idenllfiwtion of tfiis lesion. The diagnosis is confirmed by computed tomography, and emergency neumsurglcai iaminectomy is mandatory. (Am J Emerg Bled lggl;g:B4-56. Copyright 0 1991 by W.B. Saunders Company)
and concomitant paresthesiae of both hands. Clinical examination revealed a Brown-Sequard syndrome with left C3 sensory level and rapidly progressing right motor deficit. Air myelogram showed anterior shift of spinal cord at C3 to C5 level. A right posterolateral epidural hematoma was evacuated by an emergency cervical laminectomy. Partial recovery ensued but was rapidly followed by tetraplegia and death within the next 24 hours. Necropsy identified an epidural rebleeding extending up to Cl level.
Since Jackson’s original observation, more than 200 cases of spontaneous spinal epidural hematomas have been reported in the literature.’ Only 25% of these were located in the cervical region.* A sudden cervical pain followed fairly rapidly by neurologic deficit are always the main signs of the spontaneous cervical epidural hematoma (SCEH). Nonspontaneous cases have been described in association with coagulopathies, arteriovenous malformations, tumors and pregnancy.3-8 Although an extremely rare entity, this pathology is recognized with increasing frequency since the advent of computed tomography (CT) scan. This study reports three cases of SCEH; its aim is to describe the clinical signs and symptoms and possible pathogenesis of a clinical entity that requires prompt identification to prevent serious morbidity and sometimes death.
CASE 3
CASE 1 A 28-year-old teacher with previous history of cervical pain awoke one morning with severe neck pain followed within a few hours by a radiation in the left shoulder. The symptoms were not alleviated by physical therapy and weakness of left arm supervened over the next 24 hours. Clinical examination showed torticollis, a Brown-Sequard syndrome with CS right sensory level, left pyramidal signs and a left Homer syndrome. Air myelogram demonstrated an anterolateral displacement of the spinal cord between the third and seventh cervical vertebrae. Laminectomy was carried out 4 days later and evacuation of a left posterolateral epidural hematoma was performed. Two weeks later, neurologic recovery was complete.
CASE 2 A 70-year-old diabetic, hypertensive woman was awakened one morning by an excruciating cervical pain radiating in both shoulders From the ‘Department of Neurosurgery and the tPoiiclinic of Medicine, University Hospital of Geneva, Geneva, Switzerland. Manuscript received January 30,199O; revision accepted June 5, 1990. Address reprint requests to Dr Demierre: Department of Neurosurgery, University Hospital of Geneva, 1211 Geneva 4, Switzerland. Key Words: Cervical epidurai hematoma, cervical spine, emergency, computed tomography. Copyright 0 1991 by W.B. Saunders Company 0735-6757/91/0901-0016$5.00/0 54
A 64-year-old woman with treated hypertension developed a sudden right cervico-brachial pain after a mild effort, soon accompanied by numbness of the last fingers of the right hand, followed shortly by progressive weakness of the right limbs. Objective findings included fixed torticollis with global right hemiparesis without sensory deticit. CT showed a biconvex right posterolateral hyperdensity from C3 to Dl level (Figure 1). Emergency decompressive laminectomy allowed removal of a fresh epidural hematoma and rapid neurologic recovery. Ten days later the patient was discharged with a moderate residual neck stiffness.
DISCUSSION Cervical pain of sudden onset is not a specific finding of SCEH. Differential diagnosis includes frequent disorders, such as herniated cervical disc, torticollis, and subarachnoid bleeding as well as less frequent pathologies such as aortic dissection, hydromyelia, acute transverse myelitis, spinal cord ischemia, epidural abscess, or Paget’s disease.3*9m’6Because of the gravity of the SCEH evolution, the diagnosis should be done quickly. According to Beatty and Winston,” women are more often affected than are men (65%), lower cervical region is more frequently involved than is the upper cervical spine. When it extends above CS level, epidural hematoma carries a mortality of approximately 30%. Before the CT-scan era, air myelogram was the only reliable diagnostic too1.‘5,‘8 Even if the cervico-brachialgia is accompanied by a slight deficit, which is always present in case of SCEH, a cervical CT scan should be performed before secondary aggravation: emergency CT scan has now become the procedure of choice for early diagnosis.‘7,‘9-25 The demonstration of a biconvex hyperdensity posterolateral to the spinal cord is classical (Figure 1). Anterior location is less frequent.26 MRI is nowadays a promising altemative.27 Because symptoms are often triggered by effort, as described in the third case reported here, this type of lesion has been coined by Gauthier “effort epidural hematoma.“18 The origin of the spontaneous hematoma of the spine is subject to controversy: for some it may be related to a sudden increase in epidural venous pressure, an idea that has been challenged by Beatty and Winston, who argue that venous pres-
DEMIERRE, UNGER, AND BONGIOANNI n SPONTANEOUS CERVICAL EPIDURAL HEMATOMA
55
and Miss Georges and Miss Lanoe for preparation of this manuscript.
REFERENCES
FIGURE 1. CT-scan of the third patient showing a biconvex hyperdensity posterolateral to the spinal cord at the C5 level.
sure cannot be higher than intrathecal pressure and therefore cannot be responsible for a spinal cord compression.1~‘o~13~‘6~‘7~2*~29 In line with Crock and Yonekawa’s work, these authors suggest that bleeding might stem from fragilized meningeal arteries located on postero-superior side of cervical roots.30 This hypothesis might account for the high frequency of associated radicular pain and posterolateral location of SCEH. The only available treatment is emergency decompressive laminectomy, particularly if neurologic deficit is incomplete. Markham reports 100% mortality for unoperated cases.” According to Foo and Rossier, prognosis depends on preoperative neurologic deficit: when complete, a quarter of patients die within the 4 months following surgical treatment, whereas an incomplete neurologic deficit is associated with 7% mortality and a better functional outcome on the whole.3’ Most authors emphasize the need for rapid diagnosis, as the quality of neurosurgical recovery is closely correlated to the readiness of surgical decompression.32.33 Outcome correlates well with early laminectomy only in cases of progressing neurologic impairment. The fact that the neurologic deficits can be discrete initially, suggests that the emergency physician can miss this diagnosis. Therefore a sudden cervical pain even with slight neurologic impairments should always be investigated by a CT scan, because of secondary aggravation.
CONCLUSION Sudden acute cervical pain, sometimes after a mild effort followed by radiculalgia should raise the suspicion of an epidural cervical hematoma, when associated with neurologic deficit. Although prognosis depends on pre-operative neurologic state, we emphasize the need for rapid diagnosis with CT scan and early laminectomy to avoid increased morbidity when deficits are incomplete. The authors thank Dr T. Cunningham, Department of Rheumatology, University of Geneva, for reviewing the final translation,
1. Jackson R: Case of spinal apoplexy. Lancet 1869;2:5-6 2. Correa AV, Beasley BAL: Spontaneous cervical epidural hematoma with complete recovery. Surg Neurol 1978;10:227228 3. Combelles G, Blond S, Lesoin F, et al: Hematomes extraduraux rachidiens sans lesion osseuse traumatique. A propos de 9 cas. Neurochirurgie 1983;29:417-422 4. Foo D, Chang YC, Rossier AB: Spontaneous cervical epidural hemorrhage, anterior cord syndrome and familial vascular malformation: case report. Neurology 1980;30:308-311 5. Harris DJ, Fornasier VL, Livingston KE: Hemangiopericytoma of the spinal canal. Report of three cases. J Neurosurg 1978;49:914-920 6. Jacobson I, McCabe JJ, Harris P, et al: Spontaneous spinal epidural haemorrhage during anticoagulant therapy. Br Med J 1966; 1522-523 7. Yonekawa Y, Mehdorn HM, Nishikawa M: Spontaneous spinal epidural hematoma during pregnancy. Surg Neurol 1975;3:327-328 8. Zuccarello M, Scanarini M, D’Avell D, et al: Spontaneous spinal extradural hematoma during anticoagulant therapy. Surg Neurol 1980;14:411-413 9. Bidzinski J: Spontaneous spinal epidural hematoma during pregnancy: case report. J Neurosurg 1966;24:1017 10. Cooper DW: Spontaneous spinal epidural hematoma. Case report. J Neurosurg 1967;26:343-345 11. Cube HM: Spinal extradural hemorrhage. J Neurosurg 1962;19:171-172 12. Lee KS, McWhorter JM, Angelo JN: Spinal epidural hematoma associated with Paget’s Disease. Surg Neurol 1988; 30:131-134 13. Lowrey JJ: Spinal epidural hematomas. Experiences with three patients. J Neurosurg 1959;16:508-513 14. Locke GE, Giorgio AJ, Biggers SL, et al: Acute spinal epidural hematoma secondary to aspirin-induced prolonged bleeding. Surg Neurol 1976;5:293-296 15. Markham JW, Lynge HN, Stahlmange 8: The syndrome of spontaneous spinal epidural hematoma. Report of three cases. J Neurosurg 1967;26:334-342 16. Pear BL: Spinal epidural hematoma. AJR 1972;115:155164 17. Beatty RM, Winston KR: Spontaneous cervical epidural hematoma. J Neurosurg 1984;61:143-148 18. Gauthier G: L’hematome extra-dural rachidien sans fracture de la colonne. Revue de 56 cas verifies. Psychiat Neurol (Basel) 1963;146:149-175 19. Bareno EU, Schlamich MA: L’hematome epidural cervical spontane. A propos d’un cas. Neurochirurgie 1987;33:66-70 20. Costabile G, Husag L, Probst C: Spinal epidural hematoma. Surg Neurol 1984;21:489-492 21. Haykal HA, Wang AM, Zamani AA, Rumbaugh CL: Computed tomography of spontaneous acute cervical epidural hematoma. J Comput Assist Tomogr 1984;8:229-231 22. Lanzieri CF, Sacher M, Solodnik P, Moser F: CT myelography of spontaneous spinal epidural hematoma. Case report. J Comput Assist Tomogr 1985;9:393-394 23. Levitan LH, Wiens CW: Chronic lumbar extradural hematoma: CT findings. Radiology 1983;148:707-708 24. Nehls DG, Shetter AG, Hodak JA, et al: Chronic spinal epidural hematoma presenting as lumbar stenosis: clinical myelographic and computed tomographic features. A case report. Neurosurgery 1984;14:230-233 25. Post MJ, Seminer DS, Quencer RM: CT diagnosis of spinal epidural hematoma. AJNR 1982;3:190-192 26. Philips TW, Kling TF, McGilligudy JE: Spontaneous ventral spinal epidural hematoma with anterior cord syndrome: report of a case. Neurosurgery 1981;9:440-443
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AMERICAN JOURNAL OF EMERGENCY MEDICINE n Volume 9, Number 1 n January 1991
27. Zabala AB, Gonzalez MG, Allut AG, et al: Spontaneous spinal epidural hematoma diagnosed by MRI. Case report. J Neurosurg Sci 1988;32:153-155 28. Ainslie JP: Paraplegia due to spontaneous exdural or subdural haemorrhage. Br J Surg 1958;45:565-567 29. Amyes EW, Vogel PJ, Raney RB: Spinal cord compression due to spontaneous epidural hemorrhage. Report of three cases. Bull Los Angeles Neurol Sot 1955;20:1-8 30. Crock HV, Yoshizawa H: The Blood Supply of the Verte-
bral Column and Spinal Cord in Man. New York, NY, SpringerVerlag, 1977 31. Foo D, Rossier AB: Preoperative neurological status in predicting surgical outcome of spinal epidural hematomas. Surg Neurol 1981;15:389-401 32. McQuarie IG: Recovery from paraplegia caused by spontaneous spinal epidural hematoma. Neurology 1978;28:224-228 33. Lepoire J, Tridon P, Montaut J, et al: L’hematome extradural radidien spontone. Neurochirurgie 1961;7:298-313
and concomitant paresthesiae of both hands. Clinical examination revealed a Brown-Sequard syndrome with left C3 sensory level and rapidly progressing right motor deficit. Air myelogram showed anterior shift of spinal cord at C3 to C5 level. A right posterolateral epidural hematoma was evacuated by an emergency cervical laminectomy. Partial recovery ensued but was rapidly followed by tetraplegia and death within the next 24 hours. Necropsy identified an epidural rebleeding extending up to Cl level.
Since Jackson’s original observation, more than 200 cases of spontaneous spinal epidural hematomas have been reported in the literature.’ Only 25% of these were located in the cervical region.* A sudden cervical pain followed fairly rapidly by neurologic deficit are always the main signs of the spontaneous cervical epidural hematoma (SCEH). Nonspontaneous cases have been described in association with coagulopathies, arteriovenous malformations, tumors and pregnancy.3-8 Although an extremely rare entity, this pathology is recognized with increasing frequency since the advent of computed tomography (CT) scan. This study reports three cases of SCEH; its aim is to describe the clinical signs and symptoms and possible pathogenesis of a clinical entity that requires prompt identification to prevent serious morbidity and sometimes death.
CASE 3
CASE 1 A 28-year-old teacher with previous history of cervical pain awoke one morning with severe neck pain followed within a few hours by a radiation in the left shoulder. The symptoms were not alleviated by physical therapy and weakness of left arm supervened over the next 24 hours. Clinical examination showed torticollis, a Brown-Sequard syndrome with CS right sensory level, left pyramidal signs and a left Homer syndrome. Air myelogram demonstrated an anterolateral displacement of the spinal cord between the third and seventh cervical vertebrae. Laminectomy was carried out 4 days later and evacuation of a left posterolateral epidural hematoma was performed. Two weeks later, neurologic recovery was complete.
CASE 2 A 70-year-old diabetic, hypertensive woman was awakened one morning by an excruciating cervical pain radiating in both shoulders From the ‘Department of Neurosurgery and the tPoiiclinic of Medicine, University Hospital of Geneva, Geneva, Switzerland. Manuscript received January 30,199O; revision accepted June 5, 1990. Address reprint requests to Dr Demierre: Department of Neurosurgery, University Hospital of Geneva, 1211 Geneva 4, Switzerland. Key Words: Cervical epidurai hematoma, cervical spine, emergency, computed tomography. Copyright 0 1991 by W.B. Saunders Company 0735-6757/91/0901-0016$5.00/0 54
A 64-year-old woman with treated hypertension developed a sudden right cervico-brachial pain after a mild effort, soon accompanied by numbness of the last fingers of the right hand, followed shortly by progressive weakness of the right limbs. Objective findings included fixed torticollis with global right hemiparesis without sensory deticit. CT showed a biconvex right posterolateral hyperdensity from C3 to Dl level (Figure 1). Emergency decompressive laminectomy allowed removal of a fresh epidural hematoma and rapid neurologic recovery. Ten days later the patient was discharged with a moderate residual neck stiffness.
DISCUSSION Cervical pain of sudden onset is not a specific finding of SCEH. Differential diagnosis includes frequent disorders, such as herniated cervical disc, torticollis, and subarachnoid bleeding as well as less frequent pathologies such as aortic dissection, hydromyelia, acute transverse myelitis, spinal cord ischemia, epidural abscess, or Paget’s disease.3*9m’6Because of the gravity of the SCEH evolution, the diagnosis should be done quickly. According to Beatty and Winston,” women are more often affected than are men (65%), lower cervical region is more frequently involved than is the upper cervical spine. When it extends above CS level, epidural hematoma carries a mortality of approximately 30%. Before the CT-scan era, air myelogram was the only reliable diagnostic too1.‘5,‘8 Even if the cervico-brachialgia is accompanied by a slight deficit, which is always present in case of SCEH, a cervical CT scan should be performed before secondary aggravation: emergency CT scan has now become the procedure of choice for early diagnosis.‘7,‘9-25 The demonstration of a biconvex hyperdensity posterolateral to the spinal cord is classical (Figure 1). Anterior location is less frequent.26 MRI is nowadays a promising altemative.27 Because symptoms are often triggered by effort, as described in the third case reported here, this type of lesion has been coined by Gauthier “effort epidural hematoma.“18 The origin of the spontaneous hematoma of the spine is subject to controversy: for some it may be related to a sudden increase in epidural venous pressure, an idea that has been challenged by Beatty and Winston, who argue that venous pres-
DEMIERRE, UNGER, AND BONGIOANNI n SPONTANEOUS CERVICAL EPIDURAL HEMATOMA
55
and Miss Georges and Miss Lanoe for preparation of this manuscript.
REFERENCES
FIGURE 1. CT-scan of the third patient showing a biconvex hyperdensity posterolateral to the spinal cord at the C5 level.
sure cannot be higher than intrathecal pressure and therefore cannot be responsible for a spinal cord compression.1~‘o~13~‘6~‘7~2*~29 In line with Crock and Yonekawa’s work, these authors suggest that bleeding might stem from fragilized meningeal arteries located on postero-superior side of cervical roots.30 This hypothesis might account for the high frequency of associated radicular pain and posterolateral location of SCEH. The only available treatment is emergency decompressive laminectomy, particularly if neurologic deficit is incomplete. Markham reports 100% mortality for unoperated cases.” According to Foo and Rossier, prognosis depends on preoperative neurologic deficit: when complete, a quarter of patients die within the 4 months following surgical treatment, whereas an incomplete neurologic deficit is associated with 7% mortality and a better functional outcome on the whole.3’ Most authors emphasize the need for rapid diagnosis, as the quality of neurosurgical recovery is closely correlated to the readiness of surgical decompression.32.33 Outcome correlates well with early laminectomy only in cases of progressing neurologic impairment. The fact that the neurologic deficits can be discrete initially, suggests that the emergency physician can miss this diagnosis. Therefore a sudden cervical pain even with slight neurologic impairments should always be investigated by a CT scan, because of secondary aggravation.
CONCLUSION Sudden acute cervical pain, sometimes after a mild effort followed by radiculalgia should raise the suspicion of an epidural cervical hematoma, when associated with neurologic deficit. Although prognosis depends on pre-operative neurologic state, we emphasize the need for rapid diagnosis with CT scan and early laminectomy to avoid increased morbidity when deficits are incomplete. The authors thank Dr T. Cunningham, Department of Rheumatology, University of Geneva, for reviewing the final translation,
1. Jackson R: Case of spinal apoplexy. Lancet 1869;2:5-6 2. Correa AV, Beasley BAL: Spontaneous cervical epidural hematoma with complete recovery. Surg Neurol 1978;10:227228 3. Combelles G, Blond S, Lesoin F, et al: Hematomes extraduraux rachidiens sans lesion osseuse traumatique. A propos de 9 cas. Neurochirurgie 1983;29:417-422 4. Foo D, Chang YC, Rossier AB: Spontaneous cervical epidural hemorrhage, anterior cord syndrome and familial vascular malformation: case report. Neurology 1980;30:308-311 5. Harris DJ, Fornasier VL, Livingston KE: Hemangiopericytoma of the spinal canal. Report of three cases. J Neurosurg 1978;49:914-920 6. Jacobson I, McCabe JJ, Harris P, et al: Spontaneous spinal epidural haemorrhage during anticoagulant therapy. Br Med J 1966; 1522-523 7. Yonekawa Y, Mehdorn HM, Nishikawa M: Spontaneous spinal epidural hematoma during pregnancy. Surg Neurol 1975;3:327-328 8. Zuccarello M, Scanarini M, D’Avell D, et al: Spontaneous spinal extradural hematoma during anticoagulant therapy. Surg Neurol 1980;14:411-413 9. Bidzinski J: Spontaneous spinal epidural hematoma during pregnancy: case report. J Neurosurg 1966;24:1017 10. Cooper DW: Spontaneous spinal epidural hematoma. Case report. J Neurosurg 1967;26:343-345 11. Cube HM: Spinal extradural hemorrhage. J Neurosurg 1962;19:171-172 12. Lee KS, McWhorter JM, Angelo JN: Spinal epidural hematoma associated with Paget’s Disease. Surg Neurol 1988; 30:131-134 13. Lowrey JJ: Spinal epidural hematomas. Experiences with three patients. J Neurosurg 1959;16:508-513 14. Locke GE, Giorgio AJ, Biggers SL, et al: Acute spinal epidural hematoma secondary to aspirin-induced prolonged bleeding. Surg Neurol 1976;5:293-296 15. Markham JW, Lynge HN, Stahlmange 8: The syndrome of spontaneous spinal epidural hematoma. Report of three cases. J Neurosurg 1967;26:334-342 16. Pear BL: Spinal epidural hematoma. AJR 1972;115:155164 17. Beatty RM, Winston KR: Spontaneous cervical epidural hematoma. J Neurosurg 1984;61:143-148 18. Gauthier G: L’hematome extra-dural rachidien sans fracture de la colonne. Revue de 56 cas verifies. Psychiat Neurol (Basel) 1963;146:149-175 19. Bareno EU, Schlamich MA: L’hematome epidural cervical spontane. A propos d’un cas. Neurochirurgie 1987;33:66-70 20. Costabile G, Husag L, Probst C: Spinal epidural hematoma. Surg Neurol 1984;21:489-492 21. Haykal HA, Wang AM, Zamani AA, Rumbaugh CL: Computed tomography of spontaneous acute cervical epidural hematoma. J Comput Assist Tomogr 1984;8:229-231 22. Lanzieri CF, Sacher M, Solodnik P, Moser F: CT myelography of spontaneous spinal epidural hematoma. Case report. J Comput Assist Tomogr 1985;9:393-394 23. Levitan LH, Wiens CW: Chronic lumbar extradural hematoma: CT findings. Radiology 1983;148:707-708 24. Nehls DG, Shetter AG, Hodak JA, et al: Chronic spinal epidural hematoma presenting as lumbar stenosis: clinical myelographic and computed tomographic features. A case report. Neurosurgery 1984;14:230-233 25. Post MJ, Seminer DS, Quencer RM: CT diagnosis of spinal epidural hematoma. AJNR 1982;3:190-192 26. Philips TW, Kling TF, McGilligudy JE: Spontaneous ventral spinal epidural hematoma with anterior cord syndrome: report of a case. Neurosurgery 1981;9:440-443
56
AMERICAN JOURNAL OF EMERGENCY MEDICINE n Volume 9, Number 1 n January 1991
27. Zabala AB, Gonzalez MG, Allut AG, et al: Spontaneous spinal epidural hematoma diagnosed by MRI. Case report. J Neurosurg Sci 1988;32:153-155 28. Ainslie JP: Paraplegia due to spontaneous exdural or subdural haemorrhage. Br J Surg 1958;45:565-567 29. Amyes EW, Vogel PJ, Raney RB: Spinal cord compression due to spontaneous epidural hemorrhage. Report of three cases. Bull Los Angeles Neurol Sot 1955;20:1-8 30. Crock HV, Yoshizawa H: The Blood Supply of the Verte-
bral Column and Spinal Cord in Man. New York, NY, SpringerVerlag, 1977 31. Foo D, Rossier AB: Preoperative neurological status in predicting surgical outcome of spinal epidural hematomas. Surg Neurol 1981;15:389-401 32. McQuarie IG: Recovery from paraplegia caused by spontaneous spinal epidural hematoma. Neurology 1978;28:224-228 33. Lepoire J, Tridon P, Montaut J, et al: L’hematome extradural radidien spontone. Neurochirurgie 1961;7:298-313
