Case Reports / Journal of Clinical Neuroscience 22 (2015) 1493–1495
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Transient monoplegia and paraesthesia after an epidural blood patch for a spinal cerebrospinal fluid leak Alvin Ho-Kwan Cheung a, Lai-Fung Li a, Vincent Ching So b, May Ka-Mei Leung b, Wai-Man Lui a,⇑ a b
Division of Neurosurgery, Department of Surgery, Queen Mary Hospital, 102 Pok Fu Lam Road, Pok Fu Lam, Hong Kong Department of Anesthesiology, Queen Mary Hospital, Pok Fu Lam, Hong Kong
a r t i c l e
i n f o
Article history: Received 23 November 2014 Accepted 3 March 2015
Keywords: Cerebrospinal fluid fistula Epidural blood patch Intracranial hypotension
a b s t r a c t We describe the very rare complication of new onset complete paralysis and numbness of one limb after an epidural blood patch in a 36-year-old woman. Intracranial hypotension resulting from a spinal cerebrospinal fluid fistula may be treated by epidural injection of autologous blood that is, a blood patch. This is usually a safe and effective procedure. The woman’s muscle strength of hip flexion, extension, ankle dorsiflexion and plantarflexion decreased from 5/5 to 0/5 following the procedure. After symptom onset, an MRI of her spine showed no compressive or ischaemic lesions amenable to urgent intervention. The cause of neurological deficit was at that time unknown and steroids were administered. Her symptoms persisted for about 2 days and gradually improved. In this paper, the management plan and the course of this rare and alarming complication is reported. Ó 2015 Published by Elsevier Ltd.
1. Introduction Young patients suffering from non-traumatic subdural haematomas may have underlying intracranial hypotension which may cause the brain to collapse beneath the dura with tearing of bridging veins [1]. A spinal cerebrospinal fluid (CSF) fistula, which allows leakage of CSF from the spinal subarachnoid space to the epidural space, is a possible etiology leading to intracranial hypotension [2]. Classically, epidural injection of autologous blood, that is an epidural blood patch, is a relatively safe and effective treatment option [3–5]. Nevertheless, this case report describes an unusual complication after an epidural blood patch in a woman with a spinal CSF fistula.
2. Case report A 36-year-old woman with 2 months history of postural headache and neck pain was admitted to the medical ward because of vomiting over the past few days. Her occipital headache worsened with standing and was relieved by recumbence, suggestive of intracranial hypotension. Her neck pain had been previously investigated in the Outpatient Department and degenerative changes were found on a cervical spine radiograph. The woman was not taking anti-thrombotics. On examination, she had no neurological deficits in terms of power, sensation or deep tendon reflexes. There was no sign of bleeding diathesis. Her platelet count and clotting profile were normal, and electrolyte, renal and liver function tests were unremarkable. A CT scan of her brain showed a 10 mm thick left fronto-parietal chronic subdural haematoma (CSDH) which failed to resolve on serial reassessment. Her brain CT scan after two weeks is shown in Figure 1. The woman was transferred to the neurosurgical unit for further management. An MRI of her whole spine revealed a rim of epidural fluid at the posterior aspect of the thecal sac at the C3 and C4 level (Fig. 2). This finding was confirmed by a CT myelogram of the cervical spine where abnormal contrast pooling
⇑ Corresponding author. Tel.: +852 2255 3368; fax: +852 2817 5475. E-mail address: [email protected] (W.-M. Lui).
occurred at the C3 and C4 level (Fig. 3). Her diagnosis was a cervical CSF fistula. The CSDH was managed with a conservative approach since she had no neurological deficit with minimal mass effect. Meanwhile, the options of lumbar epidural blood patch or open dural repair were offered to prevent further CSF leak. The woman, who was still ambulatory in the ward without any neurological deficits, consented to the former. Anaesthetists performed an epidural puncture at the L3–L4 interspace with aseptic technique. Continuous pressure was applied to a saline-filled syringe and the epidural space was identified when a loss of resistance to saline occurred. No CSF could be aspirated. A catheter of 13 cm in length was placed into the epidural space and 20 mL of fresh autologous blood was then collected and injected via the catheter. The woman assumed a head down posture to allow the epidural blood to reach the cervical CSF fistula. After the slow injection of blood, she complained of a mild lower back fullness. The procedure was concluded with the smooth removal of the epidural catheter. One and a half hours later she suddenly developed left lower limb monoplegia and paraesthesia. On physical examination, her Medical Research Council muscle power grading was 0/5 for hip flexion and extension, ankle dorsiflexion and plantarflexion. There were no sphincter disturbances. An urgent lumbosacral spine MRI showed no haematoma anywhere, including in the subcutaneous, epidural, subdural, or subarachnoid spaces. There was no compression or abnormal signal seen to affect the spinal cord or the conus medullaris, which ended at L1–L2 interspace. An urgent brain CT scan was also performed which ruled out worsening CSDH or other acute intracranial bleeding. Dexamethasone was initiated empirically to treat the suspected inflammatory response on the spinal nerves. Approximately 24 hours later, the woman gradually regained power of the monoplegic limb to grade 3. Impaired sensation to light touch and pin-prick sensation persisted however. On the following day, she had further recovery with her power increasing up to 4 and the numbness gradually subsided. She felt that her headache had markedly improved. She could also start walking with assistance and using a stick. She had complete resolution of the CSDH and complete neurological recovery 2 weeks later, and she was able to continue a course of uneventful rehabilitation.
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Case Reports / Journal of Clinical Neuroscience 22 (2015) 1493–1495
Fig. 3. Axial cervical spine CT myelogram showing abnormal contrast pooling at the posterior epidural space (arrow).
Fig. 1. Axial brain CT scan showing a left parietal chronic subdural hematoma.
Fig. 2. Sagittal spinal T2-weighted MRI showing posterior epidural fluid collection at the C3 and C4 levels (arrow) suggestive of a spinal cerebrospinal fluid fistula.
3. Discussion Transient monoplegia has been reported after epidural anaesthesia or steroid injection, but it is rare following an epidural blood patch. In the former situation, nerve roots may be affected by the steroid or anaesthetic agents, causing symptoms of radiculopathy [6–8]. Haematoma, injected substances, or other forms of compression to the spinal cord and cauda equina are frequently evident [9,10]. In this report, we describe a woman with a cervical CSF leak who developed acute left lower limb monoplegia and paraesthesia
hours after her lumbar epidural blood patch, with no evidence of compression on MRI. We first questioned whether the site of injection was accurate. Inadvertent injection of blood into the subarachnoid space may result in signs of radiculopathy, meningism, neck stiffness or fever [3]. These additional symptoms were not prominent in our patient. The epidural space was defined by the loss of saline resistance and the inability to aspirate CSF. There was no evidence to suggest that the needle had entered the thecal sac, an incident which may cause direct trauma to the cauda equina. An isolated L3 root puncture was also unlikely given her presentation. However, there was also a chance of direct trauma from the lumbar puncture, a much more common procedure than the epidural blood patch. But even in lumbar punctures, the incidence of lower limb paralysis or numbness is exceedingly low. The injected blood product was the most likely culprit which caused the transient neurological dysfunction. The extent of left L2 to S2 neurological symptoms suggested that either the cord, cauda equina or multiple roots were affected. However, the woman’s MRI showed no evidence of blood collection at any of the intradural spaces which may have caused spinal cord compression. Another postulation was spinal cord ischaemia triggered by the blood patch. Cerebral vasospasm usually occurs a few days after subarachnoid hemorrhage [11], and so the very early onset of neurological deficit in this woman argued against vasospasm as the cause of her transient monoplegia. Also, we would expect bilateral neurological disturbance if her deficit was related to spinal cord ischaemia. We suggest that the most probable explanation is pooling of the epidural blood around the left spinal nerves. During the procedure, the woman was placed in the left lateral position with her right side up. By gravity, the blood would sink and tend to collect on the left side of the epidural space. Theoretically, the blood would have mainly collected at the L2 to S2 level immediately after the blood patch injection in this patient. A few hours later, the blood would irritate these left sided nerve roots and cause transient neurological dysfunction. Fortunately, the symptoms gradually improved when the blood started to resolve. A less likely possibility, but worth mentioning, is irritation of the nerve roots by the 13 cm long epidural catheter which was
Case Reports / Journal of Clinical Neuroscience 22 (2015) 1495–1498
directed cranially to enable the injected blood to track upwards and reach the cervical CSF fistula. However, this argument is deemed less likely as the laterality of symptoms and delayed presentation cannot be explained by this mechanism. New onset neurological deficit after epidural blood patch may cause much distress to both the patient and the surgeon. The priority is to rule out compressive [9] or ischaemic [12] lesions by an urgent spinal MRI and brain CT scan. These lesions may be treated by surgery, systemic steroid, inotropes or anti-thrombotics. T1-, T2- and diffusion-weighted MRI should provide adequate information in the urgent setting. Electrophysiological investigations may be of interest but these take time to arrange and perform. It is highly likely that new neurological symptoms without imaging abnormalities will improve with time. This complication after epidural blood patch is fortunately rare and runs a benign course.
Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.
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References [1] Miyazawa K, Shiga Y, Hasegawa T, et al. CSF hypovolemia vs intracranial hypotension in ‘‘spontaneous intracranial hypotension syndrome’’. Neurology 2003;60:941–7. [2] Schievink WI, Meyer FB, Atkinson JL, et al. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. J Neurosurg 1996;84:598–605. [3] Wilkinson HA. Lumbosacral meningismus complicating subdural injection of ‘‘blood patch’’: case report. J Neurosurg 1980;52:849–51. [4] Olsen KS. Epidural blood patch in the treatment of post-lumbar puncture headache. Pain 1987;30:293–301. [5] Waguri N, Tomita M, Hayatsu K, et al. Epidural blood patch for treatment of spontaneous intracranial hypotension. Acta Anaesthesiol Scand 2002;46: 747–50. [6] Horlocker TT, Wedel DJ. Neurologic complications of spinal and epidural anesthesia. Reg Anesth Pain Med 2000;25:83–98. [7] Kane RE. Neurologic deficits following epidural or spinal anesthesia. Anesth Analg 1981;60:150–61. [8] Epstein NE. The risks of epidural and transforaminal steroid injections in the Spine: Commentary and a comprehensive review of the literature. Surg Neurol Int 2013;4:S74–93. [9] Tekkök IH, Carter DA, Brinker R. Spinal subdural haematoma as a complication of immediate epidural blood patch. Can J Anaesth 1996;43:306–9. [10] Kalina P, Craigo P, Weingarten T. Intrathecal injection of epidural blood patch: a case report and review of the literature. Emerg Radiol 2004;11:56–9. [11] Weir B, Grace M, Hansen J, et al. Time course of vasospasm in man. J Neurosurg 1978;48:173–8. [12] Huntoon MA, Martin DP. Paralysis after transforaminal epidural injection and previous spinal surgery. Reg Anesth Pain Med 2004;29:494–5.
http://dx.doi.org/10.1016/j.jocn.2015.03.015
Multimodality management of a giant skull base hemangioendothelioma of the sphenopetroclival region Amol Raheja a, Ashish Suri a,⇑, Shuchita Singh b, Rajeev Kumar b, Rakesh Kumar b, Aruna Nambirajan c, Meher C. Sharma c a
Department of Neurosurgery and Gamma Knife, All India Institute of Medical Sciences, Room 712, Ansari Nagar, New Delhi 110029, India Department of Otorhinolaryngology, All India Institute of Medical Sciences, New Delhi, India c Department of Pathology, All India Institute of Medical Sciences, New Delhi, India b
a r t i c l e
i n f o
Article history: Received 22 November 2014 Accepted 3 March 2015
Keywords: Epithelioid Hemangioendothelioma Multimodality management Skull base
a b s t r a c t A 20-year-old man presented with proptosis, nasal obstruction, vision loss and cavernous sinus syndrome, ongoing for 6 years. Imaging and biopsy confirmed a middle skull base epithelioid hemangioendothelioma arising from the left sphenopetroclival region with infratemporal fossa and intracranial-intradural extension into the left temporal lobe. Preoperative embolization of the left internal maxillary artery followed by a combined neurosurgical (front-temporal orbito-zygomatic craniotomy) and otorhinolaryngology (maxillary swing) approach was performed for tumor debulking. Postoperative radiotherapy and maintenance interferon chemotherapy was given to achieve a favorable outcome at 6 months follow-up. We describe the pertinent clinical, genetic, radiological and histopathological features, along with the available therapeutic modalities for a primary giant skull base hemangioendothelioma. Ó 2015 Elsevier Ltd. All rights reserved.
1. Introduction Epithelioid hemangioendothelioma (EH), coined by Weiss and Enzinger [1] in 1982, is a rare vascular tumor (
1493
Transient monoplegia and paraesthesia after an epidural blood patch for a spinal cerebrospinal fluid leak Alvin Ho-Kwan Cheung a, Lai-Fung Li a, Vincent Ching So b, May Ka-Mei Leung b, Wai-Man Lui a,⇑ a b
Division of Neurosurgery, Department of Surgery, Queen Mary Hospital, 102 Pok Fu Lam Road, Pok Fu Lam, Hong Kong Department of Anesthesiology, Queen Mary Hospital, Pok Fu Lam, Hong Kong
a r t i c l e
i n f o
Article history: Received 23 November 2014 Accepted 3 March 2015
Keywords: Cerebrospinal fluid fistula Epidural blood patch Intracranial hypotension
a b s t r a c t We describe the very rare complication of new onset complete paralysis and numbness of one limb after an epidural blood patch in a 36-year-old woman. Intracranial hypotension resulting from a spinal cerebrospinal fluid fistula may be treated by epidural injection of autologous blood that is, a blood patch. This is usually a safe and effective procedure. The woman’s muscle strength of hip flexion, extension, ankle dorsiflexion and plantarflexion decreased from 5/5 to 0/5 following the procedure. After symptom onset, an MRI of her spine showed no compressive or ischaemic lesions amenable to urgent intervention. The cause of neurological deficit was at that time unknown and steroids were administered. Her symptoms persisted for about 2 days and gradually improved. In this paper, the management plan and the course of this rare and alarming complication is reported. Ó 2015 Published by Elsevier Ltd.
1. Introduction Young patients suffering from non-traumatic subdural haematomas may have underlying intracranial hypotension which may cause the brain to collapse beneath the dura with tearing of bridging veins [1]. A spinal cerebrospinal fluid (CSF) fistula, which allows leakage of CSF from the spinal subarachnoid space to the epidural space, is a possible etiology leading to intracranial hypotension [2]. Classically, epidural injection of autologous blood, that is an epidural blood patch, is a relatively safe and effective treatment option [3–5]. Nevertheless, this case report describes an unusual complication after an epidural blood patch in a woman with a spinal CSF fistula.
2. Case report A 36-year-old woman with 2 months history of postural headache and neck pain was admitted to the medical ward because of vomiting over the past few days. Her occipital headache worsened with standing and was relieved by recumbence, suggestive of intracranial hypotension. Her neck pain had been previously investigated in the Outpatient Department and degenerative changes were found on a cervical spine radiograph. The woman was not taking anti-thrombotics. On examination, she had no neurological deficits in terms of power, sensation or deep tendon reflexes. There was no sign of bleeding diathesis. Her platelet count and clotting profile were normal, and electrolyte, renal and liver function tests were unremarkable. A CT scan of her brain showed a 10 mm thick left fronto-parietal chronic subdural haematoma (CSDH) which failed to resolve on serial reassessment. Her brain CT scan after two weeks is shown in Figure 1. The woman was transferred to the neurosurgical unit for further management. An MRI of her whole spine revealed a rim of epidural fluid at the posterior aspect of the thecal sac at the C3 and C4 level (Fig. 2). This finding was confirmed by a CT myelogram of the cervical spine where abnormal contrast pooling
⇑ Corresponding author. Tel.: +852 2255 3368; fax: +852 2817 5475. E-mail address: [email protected] (W.-M. Lui).
occurred at the C3 and C4 level (Fig. 3). Her diagnosis was a cervical CSF fistula. The CSDH was managed with a conservative approach since she had no neurological deficit with minimal mass effect. Meanwhile, the options of lumbar epidural blood patch or open dural repair were offered to prevent further CSF leak. The woman, who was still ambulatory in the ward without any neurological deficits, consented to the former. Anaesthetists performed an epidural puncture at the L3–L4 interspace with aseptic technique. Continuous pressure was applied to a saline-filled syringe and the epidural space was identified when a loss of resistance to saline occurred. No CSF could be aspirated. A catheter of 13 cm in length was placed into the epidural space and 20 mL of fresh autologous blood was then collected and injected via the catheter. The woman assumed a head down posture to allow the epidural blood to reach the cervical CSF fistula. After the slow injection of blood, she complained of a mild lower back fullness. The procedure was concluded with the smooth removal of the epidural catheter. One and a half hours later she suddenly developed left lower limb monoplegia and paraesthesia. On physical examination, her Medical Research Council muscle power grading was 0/5 for hip flexion and extension, ankle dorsiflexion and plantarflexion. There were no sphincter disturbances. An urgent lumbosacral spine MRI showed no haematoma anywhere, including in the subcutaneous, epidural, subdural, or subarachnoid spaces. There was no compression or abnormal signal seen to affect the spinal cord or the conus medullaris, which ended at L1–L2 interspace. An urgent brain CT scan was also performed which ruled out worsening CSDH or other acute intracranial bleeding. Dexamethasone was initiated empirically to treat the suspected inflammatory response on the spinal nerves. Approximately 24 hours later, the woman gradually regained power of the monoplegic limb to grade 3. Impaired sensation to light touch and pin-prick sensation persisted however. On the following day, she had further recovery with her power increasing up to 4 and the numbness gradually subsided. She felt that her headache had markedly improved. She could also start walking with assistance and using a stick. She had complete resolution of the CSDH and complete neurological recovery 2 weeks later, and she was able to continue a course of uneventful rehabilitation.
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Case Reports / Journal of Clinical Neuroscience 22 (2015) 1493–1495
Fig. 3. Axial cervical spine CT myelogram showing abnormal contrast pooling at the posterior epidural space (arrow).
Fig. 1. Axial brain CT scan showing a left parietal chronic subdural hematoma.
Fig. 2. Sagittal spinal T2-weighted MRI showing posterior epidural fluid collection at the C3 and C4 levels (arrow) suggestive of a spinal cerebrospinal fluid fistula.
3. Discussion Transient monoplegia has been reported after epidural anaesthesia or steroid injection, but it is rare following an epidural blood patch. In the former situation, nerve roots may be affected by the steroid or anaesthetic agents, causing symptoms of radiculopathy [6–8]. Haematoma, injected substances, or other forms of compression to the spinal cord and cauda equina are frequently evident [9,10]. In this report, we describe a woman with a cervical CSF leak who developed acute left lower limb monoplegia and paraesthesia
hours after her lumbar epidural blood patch, with no evidence of compression on MRI. We first questioned whether the site of injection was accurate. Inadvertent injection of blood into the subarachnoid space may result in signs of radiculopathy, meningism, neck stiffness or fever [3]. These additional symptoms were not prominent in our patient. The epidural space was defined by the loss of saline resistance and the inability to aspirate CSF. There was no evidence to suggest that the needle had entered the thecal sac, an incident which may cause direct trauma to the cauda equina. An isolated L3 root puncture was also unlikely given her presentation. However, there was also a chance of direct trauma from the lumbar puncture, a much more common procedure than the epidural blood patch. But even in lumbar punctures, the incidence of lower limb paralysis or numbness is exceedingly low. The injected blood product was the most likely culprit which caused the transient neurological dysfunction. The extent of left L2 to S2 neurological symptoms suggested that either the cord, cauda equina or multiple roots were affected. However, the woman’s MRI showed no evidence of blood collection at any of the intradural spaces which may have caused spinal cord compression. Another postulation was spinal cord ischaemia triggered by the blood patch. Cerebral vasospasm usually occurs a few days after subarachnoid hemorrhage [11], and so the very early onset of neurological deficit in this woman argued against vasospasm as the cause of her transient monoplegia. Also, we would expect bilateral neurological disturbance if her deficit was related to spinal cord ischaemia. We suggest that the most probable explanation is pooling of the epidural blood around the left spinal nerves. During the procedure, the woman was placed in the left lateral position with her right side up. By gravity, the blood would sink and tend to collect on the left side of the epidural space. Theoretically, the blood would have mainly collected at the L2 to S2 level immediately after the blood patch injection in this patient. A few hours later, the blood would irritate these left sided nerve roots and cause transient neurological dysfunction. Fortunately, the symptoms gradually improved when the blood started to resolve. A less likely possibility, but worth mentioning, is irritation of the nerve roots by the 13 cm long epidural catheter which was
Case Reports / Journal of Clinical Neuroscience 22 (2015) 1495–1498
directed cranially to enable the injected blood to track upwards and reach the cervical CSF fistula. However, this argument is deemed less likely as the laterality of symptoms and delayed presentation cannot be explained by this mechanism. New onset neurological deficit after epidural blood patch may cause much distress to both the patient and the surgeon. The priority is to rule out compressive [9] or ischaemic [12] lesions by an urgent spinal MRI and brain CT scan. These lesions may be treated by surgery, systemic steroid, inotropes or anti-thrombotics. T1-, T2- and diffusion-weighted MRI should provide adequate information in the urgent setting. Electrophysiological investigations may be of interest but these take time to arrange and perform. It is highly likely that new neurological symptoms without imaging abnormalities will improve with time. This complication after epidural blood patch is fortunately rare and runs a benign course.
Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.
1495
References [1] Miyazawa K, Shiga Y, Hasegawa T, et al. CSF hypovolemia vs intracranial hypotension in ‘‘spontaneous intracranial hypotension syndrome’’. Neurology 2003;60:941–7. [2] Schievink WI, Meyer FB, Atkinson JL, et al. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. J Neurosurg 1996;84:598–605. [3] Wilkinson HA. Lumbosacral meningismus complicating subdural injection of ‘‘blood patch’’: case report. J Neurosurg 1980;52:849–51. [4] Olsen KS. Epidural blood patch in the treatment of post-lumbar puncture headache. Pain 1987;30:293–301. [5] Waguri N, Tomita M, Hayatsu K, et al. Epidural blood patch for treatment of spontaneous intracranial hypotension. Acta Anaesthesiol Scand 2002;46: 747–50. [6] Horlocker TT, Wedel DJ. Neurologic complications of spinal and epidural anesthesia. Reg Anesth Pain Med 2000;25:83–98. [7] Kane RE. Neurologic deficits following epidural or spinal anesthesia. Anesth Analg 1981;60:150–61. [8] Epstein NE. The risks of epidural and transforaminal steroid injections in the Spine: Commentary and a comprehensive review of the literature. Surg Neurol Int 2013;4:S74–93. [9] Tekkök IH, Carter DA, Brinker R. Spinal subdural haematoma as a complication of immediate epidural blood patch. Can J Anaesth 1996;43:306–9. [10] Kalina P, Craigo P, Weingarten T. Intrathecal injection of epidural blood patch: a case report and review of the literature. Emerg Radiol 2004;11:56–9. [11] Weir B, Grace M, Hansen J, et al. Time course of vasospasm in man. J Neurosurg 1978;48:173–8. [12] Huntoon MA, Martin DP. Paralysis after transforaminal epidural injection and previous spinal surgery. Reg Anesth Pain Med 2004;29:494–5.
http://dx.doi.org/10.1016/j.jocn.2015.03.015
Multimodality management of a giant skull base hemangioendothelioma of the sphenopetroclival region Amol Raheja a, Ashish Suri a,⇑, Shuchita Singh b, Rajeev Kumar b, Rakesh Kumar b, Aruna Nambirajan c, Meher C. Sharma c a
Department of Neurosurgery and Gamma Knife, All India Institute of Medical Sciences, Room 712, Ansari Nagar, New Delhi 110029, India Department of Otorhinolaryngology, All India Institute of Medical Sciences, New Delhi, India c Department of Pathology, All India Institute of Medical Sciences, New Delhi, India b
a r t i c l e
i n f o
Article history: Received 22 November 2014 Accepted 3 March 2015
Keywords: Epithelioid Hemangioendothelioma Multimodality management Skull base
a b s t r a c t A 20-year-old man presented with proptosis, nasal obstruction, vision loss and cavernous sinus syndrome, ongoing for 6 years. Imaging and biopsy confirmed a middle skull base epithelioid hemangioendothelioma arising from the left sphenopetroclival region with infratemporal fossa and intracranial-intradural extension into the left temporal lobe. Preoperative embolization of the left internal maxillary artery followed by a combined neurosurgical (front-temporal orbito-zygomatic craniotomy) and otorhinolaryngology (maxillary swing) approach was performed for tumor debulking. Postoperative radiotherapy and maintenance interferon chemotherapy was given to achieve a favorable outcome at 6 months follow-up. We describe the pertinent clinical, genetic, radiological and histopathological features, along with the available therapeutic modalities for a primary giant skull base hemangioendothelioma. Ó 2015 Elsevier Ltd. All rights reserved.
1. Introduction Epithelioid hemangioendothelioma (EH), coined by Weiss and Enzinger [1] in 1982, is a rare vascular tumor (
