Recurrent Spontaneous Spinal Epidural Haematoma leading to Compressive Myelopathy Rajendra Singh Jain DM, Rahul Handa MD, Kadam Nagpal MD, Swayam Prakash MD, Pankaj Kumar Gupta MD, Rakesh Agrawal MD PII: DOI: Reference:
S0735-6757(14)00004-7 doi: 10.1016/j.ajem.2013.12.061 YAJEM 54065
To appear in:
American Journal of Emergency Medicine
Received date: Accepted date:
24 December 2013 31 December 2013
Please cite this article as: Jain Rajendra Singh, Handa Rahul, Nagpal Kadam, Prakash Swayam, Gupta Pankaj Kumar, Agrawal Rakesh, Recurrent Spontaneous Spinal Epidural Haematoma leading to Compressive Myelopathy, American Journal of Emergency Medicine (2014), doi: 10.1016/j.ajem.2013.12.061
This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Title page Title- Recurrent Spontaneous Spinal Epidural Haematoma leading to Compressive Myelopathy
RI P
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Authors: Rajendra Singh Jain, DM Neurology, Professor, Department of Neurology, SMS Medical College, Jaipur,
SC
Rajasthan, India
Rahul Handa, MD Medicine, Senior Resident, Department of Neurology, SMS Medical College, Jaipur,
MA NU
Rajasthan, India
Kadam Nagpal, MD Medicine, Senior Resident, Department of Neurology, SMS Medical College, Jaipur, Rajasthan, India
Swayam Prakash, MD Medicine, Senior Resident, Department of Neurology, SMS Medical College, Jaipur,
ED
Rajasthan, India
Pankaj Kumar Gupta: MD Medicine, Senior Resident, Department of Neurology, SMS Medical College, Jaipur,
PT
Rajasthan, India
Rajasthan, India
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Rakesh Agrawal: MD Medicine, Senior Resident, Department of Neurology, SMS Medical College, Jaipur,
AC
Corresponding Author: Dr Rahul Handa, Address: Department of Neurology, SMS Medical College, Jaipur, Rajasthan, India; Phone number: +918696363322. E-mail: [email protected] We received no grant for this case. There is no financial and intellectual conflict of interest. Keywords: recurrent, spinal epidural haematoma, spontaneous, compressive myelopathy, paraparesis
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Recurrent Spontaneous Spinal Epidural Haematoma leading to Compressive Myelopathy Abstract:
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Spontaneous spinal epidural haematoma accounts for majority of cases of spinal epidural haematoma, but very few cases of recurrent spontaneous spinal epidural haematoma have been published till date. We herein report a
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case of a 20-year old male, who presented with ten days history of pain in the interscapular region, followed by paraparesis and sensory loss below umbilicus along with urinary retention. Patient gave history of two similar episodes in the last three months, with complete spontaneous recovery. His Magnetic ResonanceIimaging of dorsal spine was suggestive of sub acute spinal epidural haematoma. On reviewing previous two Magnetic Resonance Imagings of dorsal spine, it was apparent that the patient had suffered from recurrent haematoma at
ED
the same site. Thus, a provisional diagnosis of recurrent spontaneous spinal haematoma at the level of upper dorsal spine was kept and was managed accordingly. The patient with recurrent spontaneous spinal epidural
PT
haematoma, should be educated about the nature of the disease and advised to consult a neurosurgeon as early as possible in case of a relapse, so that they can undergo surgical management preferably within eight hours and
AC
Introduction:
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not later than 36 hours.
Spontaneous spinal epidural haematoma (SSEH) accounts for majority of cases of spinal epidural haematoma [1]. It is usually seen in the middle age group although cases have been described in children as well as elderly population [2]. Very few cases of recurrent SSEH have been published till date [3]. We herein report a case of recurrent SSEH and highlight the importance of suspecting and diagnosing this condition as early as possible so that the patient can undergo evacuation and decompression surgery within a narrow time window. Case report: A 20-year old male, not a known case of any chronic illness or on any medication, presented with ten days history of pain in the interscapular region followed by paraparesis and sensory loss below umbilicus along with urinary retention. Weakness progressed over first two days and then plateaued for five days and the patient
Recurrent Spontaneous Spinal Epidural Hematoma
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ACCEPTED MANUSCRIPT became bed bound. Over the next three days, motor power improved and the patient became ambulatory along with partial recovery in bladder dysfunction.
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improved completely over a period of one month with conservative treatment.
T
Patient gave history of two similar episodes in the last three months, one month apart, and both the episodes
On examination, patient had spasticity, power 4-/5 (MRC grade) with brisk reflexes and extensor plantar in both
SC
lower limbs. Pan-sensory loss was present below D2 vertebral level.
Magnetic resonance imaging (MRI) dorsal spine was done which revealed early sub acute spinal epidural
MA NU
haematoma at D1 and D2 vertebral level with cord compression (Figure 1). On reviewing previous two MRIs, it was apparent that the patient had suffered from recurrent haematoma at the same site (Figure 2). Thus, a provisional diagnosis of recurrent spontaneous spinal haematoma at the level of upper dorsal spine was kept and the patient was investigated for possible causes of bleeding disorders and vascular malformation. Patient’s
ED
coagulation profile including platelet count, bleeding time, clotting time, prothrombin time index (PTI), activated partial thromboplastin time (aPTT) and antiphospholipid antibody (APLA) were normal. Patient’s
PT
spinal digital subtraction angiography (DSA) was also normal (Figure 3). A final diagnosis of idiopathic recurrent SSEH was made. As the patient was improving, and ten days had already elapsed since the onset of
CE
weakness, we decided to manage the patient conservatively. On discharge, patient was advised to avoid heavy exercise and straining. He was educated about the nature of
AC
the disease and possibility of a relapse. He was advised to consult a neurosurgeon immediately in case of any relapse, so that timely intervention may be undertaken. Discussion: SSEH typically presents as acute onset pain in back, usually in interscapular area followed by features suggestive of compressive myelopathy [4,5]. Although, any part of spine may be involved, cervico-dorsal spine is the most common site of SSEH [4]. Due to the presence of epidural fat posteriorly, haematoma usually arise posterior to the spine. Most of the studies have postulated that the posterior vertebral venous plexus is the source of bleeding, however, some authors suggest that the epidural arteries are the origin of SSEH [6,7]. In a case of SSEH, all possible inherited and acquired coagulopathies and vascular malformations should be ruled out. In our patient spinal DSA and coagulation profile were normal and he was not on any anticoagulant
Recurrent Spontaneous Spinal Epidural Hematoma
Page 3
ACCEPTED MANUSCRIPT therapy. Thus, in our patient no etiological factor could be identified as a cause of recurrent haematoma. In approximately 30% of the patients with SSEH etiological factors remain unidentified [1].
T
Irrespective of the cause of compressive myelopathy, all the suspected cases should undergo MRI spine as early
RI P
as possible so that surgery can be performed within the narrow time window, during which the chances of improvement are highest. No cut-off time limit has been laid down, but various studies have shown that best results of decompressive surgery are seen within eight hours of onset of weakness and surgery done later than
SC
36 hours does not have much success [6,8].
MA NU
Conservative management can also be considered in patients with mild deficit or who have rapidly improving clinical course [8,9]. Our patient was managed conservatively as he presented to us ten days after the onset of weakness and during this time his weakness had improved significantly. He was investigated for any possible cause of recurrent SSEH, so that further attacks could be avoided. Even after extensive work up including spinal DSA, no possible cause of recurrent SSEH could be identified. To the best of our knowledge, only three cases
ED
reports of recurrent SSEH have been published so far out of which one case was in a child of 10 years of age
PT
[3]. Conclusion:
CE
Recurrent SSEH is a rarely reported entity but it is important to consider this as a cause of compressive myelopathy. All possible causes of spontaneous haematoma should be ruled out by extensive work-up including
AC
spinal DSA. The patient with recurrent SSEH, should be educated about the nature of the disease and advised to consult a neurosurgeon as early as possible in case of a relapse, so that they can undergo surgical management preferably within eight hours and not later than 36 hours. References: 1.
Rath SA: Spinal hematoma: a literature survey with meta-analysis of 613 patients. Neurosurg Rev 2003; 26:50.
2.
Foo D, Rosier AB: Preoperative neurological status in predicting surgical outcome of spinal epidural hematomas. Surg Neurol 1981; 15:389-401.
3.
Abram HS, DeLaHunt MJ, Merinbaum DJ, Hammond DN: Recurrent spontaneous spinal epidural hematoma in a child: first case report. Pediatr Neurol. 2007 Mar;36(3):177-80.
Recurrent Spontaneous Spinal Epidural Hematoma
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ACCEPTED MANUSCRIPT 4.
Patel H, Boaz JC, Phillips JP, Garg BP: Spontaneous spinal epidural hematoma in children. Pediatr Neurol 1998 Oct;19(4):302-7.
5.
Ravid S, Schneider S, Maytal J: Spontaneous spinal epidural hematoma: an uncommon
Beatty RM, Winston KR: Spontaneous cervical epidural hematoma. A consideration of etiology. J
RI P
6.
T
presentation of a rare disease. Childs Nerv Syst 2002 Jul;18(6-7):345-7.
Neurosurg 1984 Jul;61(1):143-8.
Groen RJ, Ponssen H: The spontaneous spinal epidural hematoma. A study of the etiology. J Neurol Sci 1990 Sep;98(2-3):121-38.
Groen RJ, van Alphen HA: Operative treatment of spontaneous spinal epidural hematomas: a
MA NU
8.
SC
7.
study of the factors determining postoperative outcome. Neurosurgery 1996 Sep;39(3):494-508. 9.
Kreppel D, Antoniadis G, Seeling W: Spinal hematoma: a literature survey with meta-analysis of 613 patients. Neurosurg Rev 2003 Jan;26(1):1-49.
ED
Legends:
Figure 1 (A and B): MRI Dorsal spine T1W and T2W sagital image suggestive of early sub-acute posterior
PT
epidural hematoma at the level of D1 and D2 vertebrae with cord compression (arrow).
CE
Figure 2 (A and B): MRI Dorsal spine T1W (A) and T2W (B) sagital image (done after the first episode: three months back) suggestive of late subacute posterior epidural hematoma at the level of D1 and D2
AC
vertebrae with cord compression (arrow). Figure 3 (A and B): MRI Dorsal spine T1W (A) and T2W (B) sagital image (done after the second episode one month back) suggestive of acute posterior epidural hematoma at the level of D1 and D2 vertebrae with cord compression (arrow). Figure 4: Digital Subtraction Angiography with catheter in right subclavian artery showing no vascular malformation
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S0735-6757(14)00004-7 doi: 10.1016/j.ajem.2013.12.061 YAJEM 54065
To appear in:
American Journal of Emergency Medicine
Received date: Accepted date:
24 December 2013 31 December 2013
Please cite this article as: Jain Rajendra Singh, Handa Rahul, Nagpal Kadam, Prakash Swayam, Gupta Pankaj Kumar, Agrawal Rakesh, Recurrent Spontaneous Spinal Epidural Haematoma leading to Compressive Myelopathy, American Journal of Emergency Medicine (2014), doi: 10.1016/j.ajem.2013.12.061
This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Title page Title- Recurrent Spontaneous Spinal Epidural Haematoma leading to Compressive Myelopathy
RI P
T
Authors: Rajendra Singh Jain, DM Neurology, Professor, Department of Neurology, SMS Medical College, Jaipur,
SC
Rajasthan, India
Rahul Handa, MD Medicine, Senior Resident, Department of Neurology, SMS Medical College, Jaipur,
MA NU
Rajasthan, India
Kadam Nagpal, MD Medicine, Senior Resident, Department of Neurology, SMS Medical College, Jaipur, Rajasthan, India
Swayam Prakash, MD Medicine, Senior Resident, Department of Neurology, SMS Medical College, Jaipur,
ED
Rajasthan, India
Pankaj Kumar Gupta: MD Medicine, Senior Resident, Department of Neurology, SMS Medical College, Jaipur,
PT
Rajasthan, India
Rajasthan, India
CE
Rakesh Agrawal: MD Medicine, Senior Resident, Department of Neurology, SMS Medical College, Jaipur,
AC
Corresponding Author: Dr Rahul Handa, Address: Department of Neurology, SMS Medical College, Jaipur, Rajasthan, India; Phone number: +918696363322. E-mail: [email protected] We received no grant for this case. There is no financial and intellectual conflict of interest. Keywords: recurrent, spinal epidural haematoma, spontaneous, compressive myelopathy, paraparesis
Recurrent Spontaneous Spinal Epidural Hematoma
Page 1
ACCEPTED MANUSCRIPT
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Recurrent Spontaneous Spinal Epidural Haematoma leading to Compressive Myelopathy Abstract:
SC
Spontaneous spinal epidural haematoma accounts for majority of cases of spinal epidural haematoma, but very few cases of recurrent spontaneous spinal epidural haematoma have been published till date. We herein report a
MA NU
case of a 20-year old male, who presented with ten days history of pain in the interscapular region, followed by paraparesis and sensory loss below umbilicus along with urinary retention. Patient gave history of two similar episodes in the last three months, with complete spontaneous recovery. His Magnetic ResonanceIimaging of dorsal spine was suggestive of sub acute spinal epidural haematoma. On reviewing previous two Magnetic Resonance Imagings of dorsal spine, it was apparent that the patient had suffered from recurrent haematoma at
ED
the same site. Thus, a provisional diagnosis of recurrent spontaneous spinal haematoma at the level of upper dorsal spine was kept and was managed accordingly. The patient with recurrent spontaneous spinal epidural
PT
haematoma, should be educated about the nature of the disease and advised to consult a neurosurgeon as early as possible in case of a relapse, so that they can undergo surgical management preferably within eight hours and
AC
Introduction:
CE
not later than 36 hours.
Spontaneous spinal epidural haematoma (SSEH) accounts for majority of cases of spinal epidural haematoma [1]. It is usually seen in the middle age group although cases have been described in children as well as elderly population [2]. Very few cases of recurrent SSEH have been published till date [3]. We herein report a case of recurrent SSEH and highlight the importance of suspecting and diagnosing this condition as early as possible so that the patient can undergo evacuation and decompression surgery within a narrow time window. Case report: A 20-year old male, not a known case of any chronic illness or on any medication, presented with ten days history of pain in the interscapular region followed by paraparesis and sensory loss below umbilicus along with urinary retention. Weakness progressed over first two days and then plateaued for five days and the patient
Recurrent Spontaneous Spinal Epidural Hematoma
Page 2
ACCEPTED MANUSCRIPT became bed bound. Over the next three days, motor power improved and the patient became ambulatory along with partial recovery in bladder dysfunction.
RI P
improved completely over a period of one month with conservative treatment.
T
Patient gave history of two similar episodes in the last three months, one month apart, and both the episodes
On examination, patient had spasticity, power 4-/5 (MRC grade) with brisk reflexes and extensor plantar in both
SC
lower limbs. Pan-sensory loss was present below D2 vertebral level.
Magnetic resonance imaging (MRI) dorsal spine was done which revealed early sub acute spinal epidural
MA NU
haematoma at D1 and D2 vertebral level with cord compression (Figure 1). On reviewing previous two MRIs, it was apparent that the patient had suffered from recurrent haematoma at the same site (Figure 2). Thus, a provisional diagnosis of recurrent spontaneous spinal haematoma at the level of upper dorsal spine was kept and the patient was investigated for possible causes of bleeding disorders and vascular malformation. Patient’s
ED
coagulation profile including platelet count, bleeding time, clotting time, prothrombin time index (PTI), activated partial thromboplastin time (aPTT) and antiphospholipid antibody (APLA) were normal. Patient’s
PT
spinal digital subtraction angiography (DSA) was also normal (Figure 3). A final diagnosis of idiopathic recurrent SSEH was made. As the patient was improving, and ten days had already elapsed since the onset of
CE
weakness, we decided to manage the patient conservatively. On discharge, patient was advised to avoid heavy exercise and straining. He was educated about the nature of
AC
the disease and possibility of a relapse. He was advised to consult a neurosurgeon immediately in case of any relapse, so that timely intervention may be undertaken. Discussion: SSEH typically presents as acute onset pain in back, usually in interscapular area followed by features suggestive of compressive myelopathy [4,5]. Although, any part of spine may be involved, cervico-dorsal spine is the most common site of SSEH [4]. Due to the presence of epidural fat posteriorly, haematoma usually arise posterior to the spine. Most of the studies have postulated that the posterior vertebral venous plexus is the source of bleeding, however, some authors suggest that the epidural arteries are the origin of SSEH [6,7]. In a case of SSEH, all possible inherited and acquired coagulopathies and vascular malformations should be ruled out. In our patient spinal DSA and coagulation profile were normal and he was not on any anticoagulant
Recurrent Spontaneous Spinal Epidural Hematoma
Page 3
ACCEPTED MANUSCRIPT therapy. Thus, in our patient no etiological factor could be identified as a cause of recurrent haematoma. In approximately 30% of the patients with SSEH etiological factors remain unidentified [1].
T
Irrespective of the cause of compressive myelopathy, all the suspected cases should undergo MRI spine as early
RI P
as possible so that surgery can be performed within the narrow time window, during which the chances of improvement are highest. No cut-off time limit has been laid down, but various studies have shown that best results of decompressive surgery are seen within eight hours of onset of weakness and surgery done later than
SC
36 hours does not have much success [6,8].
MA NU
Conservative management can also be considered in patients with mild deficit or who have rapidly improving clinical course [8,9]. Our patient was managed conservatively as he presented to us ten days after the onset of weakness and during this time his weakness had improved significantly. He was investigated for any possible cause of recurrent SSEH, so that further attacks could be avoided. Even after extensive work up including spinal DSA, no possible cause of recurrent SSEH could be identified. To the best of our knowledge, only three cases
ED
reports of recurrent SSEH have been published so far out of which one case was in a child of 10 years of age
PT
[3]. Conclusion:
CE
Recurrent SSEH is a rarely reported entity but it is important to consider this as a cause of compressive myelopathy. All possible causes of spontaneous haematoma should be ruled out by extensive work-up including
AC
spinal DSA. The patient with recurrent SSEH, should be educated about the nature of the disease and advised to consult a neurosurgeon as early as possible in case of a relapse, so that they can undergo surgical management preferably within eight hours and not later than 36 hours. References: 1.
Rath SA: Spinal hematoma: a literature survey with meta-analysis of 613 patients. Neurosurg Rev 2003; 26:50.
2.
Foo D, Rosier AB: Preoperative neurological status in predicting surgical outcome of spinal epidural hematomas. Surg Neurol 1981; 15:389-401.
3.
Abram HS, DeLaHunt MJ, Merinbaum DJ, Hammond DN: Recurrent spontaneous spinal epidural hematoma in a child: first case report. Pediatr Neurol. 2007 Mar;36(3):177-80.
Recurrent Spontaneous Spinal Epidural Hematoma
Page 4
ACCEPTED MANUSCRIPT 4.
Patel H, Boaz JC, Phillips JP, Garg BP: Spontaneous spinal epidural hematoma in children. Pediatr Neurol 1998 Oct;19(4):302-7.
5.
Ravid S, Schneider S, Maytal J: Spontaneous spinal epidural hematoma: an uncommon
Beatty RM, Winston KR: Spontaneous cervical epidural hematoma. A consideration of etiology. J
RI P
6.
T
presentation of a rare disease. Childs Nerv Syst 2002 Jul;18(6-7):345-7.
Neurosurg 1984 Jul;61(1):143-8.
Groen RJ, Ponssen H: The spontaneous spinal epidural hematoma. A study of the etiology. J Neurol Sci 1990 Sep;98(2-3):121-38.
Groen RJ, van Alphen HA: Operative treatment of spontaneous spinal epidural hematomas: a
MA NU
8.
SC
7.
study of the factors determining postoperative outcome. Neurosurgery 1996 Sep;39(3):494-508. 9.
Kreppel D, Antoniadis G, Seeling W: Spinal hematoma: a literature survey with meta-analysis of 613 patients. Neurosurg Rev 2003 Jan;26(1):1-49.
ED
Legends:
Figure 1 (A and B): MRI Dorsal spine T1W and T2W sagital image suggestive of early sub-acute posterior
PT
epidural hematoma at the level of D1 and D2 vertebrae with cord compression (arrow).
CE
Figure 2 (A and B): MRI Dorsal spine T1W (A) and T2W (B) sagital image (done after the first episode: three months back) suggestive of late subacute posterior epidural hematoma at the level of D1 and D2
AC
vertebrae with cord compression (arrow). Figure 3 (A and B): MRI Dorsal spine T1W (A) and T2W (B) sagital image (done after the second episode one month back) suggestive of acute posterior epidural hematoma at the level of D1 and D2 vertebrae with cord compression (arrow). Figure 4: Digital Subtraction Angiography with catheter in right subclavian artery showing no vascular malformation
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