Childs Nerv Syst DOI 10.1007/s00381-014-2441-4
CASE REPORT
Multilevel thoracic hemangioma with spinal cord compression in a pediatric patient: case report and review of the literature Jacob Cherian & Christina M. Sayama & Adekunle M. Adesina & Sandi K. Lam & Thomas G. Luerssen & Andrew Jea
Received: 7 May 2014 / Accepted: 12 May 2014 # Springer-Verlag Berlin Heidelberg 2014
Abstract Purpose Vertebral hemangiomas are common benign vascular tumors of the spine. It is very rare for these lesions to symptomatically compress neural elements. If spinal cord compression does occur, it usually involves only a single level. Multilevel vertebral hemangiomas causing symptomatic spinal cord compression have never been reported in the pediatric population to the best of our knowledge. Methods We report the case of a 15-year-old boy presenting with progressive paraparesis due to thoracic spinal cord compression from a multilevel thoracic hemangioma (T5–T10) with epidural extension. Results Because of his progressive neurological deficit, he was initially treated with urgent multilevel decompressive laminectomies from T4 to T11. This was to be followed by radiotherapy for residual tumor, but the patient was unfortunately lost to follow-up. He re-presented 3 years later with recurrent paraparesis and progressive disease. This was treated with urgent radiotherapy with good response. As of 6 months follow-up, he has made an excellent neurological recovery. Conclusions In this report, we present the first case of a child with multilevel vertebral hemangiomas causing symptomatic spinal cord compression and review the literature to detail the pathophysiology, management, and treatment of other cases of spinal cord compression by vertebral hemangiomas.
Keywords Thoracic spine . Vertebral hemangioma . Spinal cord compression
Introduction Hemangiomas are the most common benign tumor of the vertebrae and are often asymptomatic [4]. There are three histological types of vertebral hemangiomas: capillary, cavernous, and mixed [7, 12]. In rare cases, these neoplasms can cause spinal cord compression and result in progressive neurological deficits, usually at a single vertebral level [3, 4, 11, 17]. Extensive vascularity of these lesions suggests aggressive behavior, but the natural history of these lesions remains unknown [4, 9, 10, 13]. Often, multilevel involvement of vertebral hemangiomas has been documented [4]. In this study, we report a unique case of multilevel (T5-T10) thoracic hemangiomas with epidural extension causing symptomatic spinal cord compression in a 15-yearold boy. To the best of our knowledge, this is the first case description of its kind in the pediatric age group. In addition, we review the literature to discuss the natural history, management, and treatment options of vertebral hemangiomas.
Case presentation J. Cherian : C. M. Sayama : S. K. Lam : T. G. Luerssen : A. Jea (*) Division of Pediatric Neurosurgery, Texas Children’s Hospital, Department of Neurosurgery, Baylor College of Medicine, 6621 Fannin Street, CCC 1230.01, 12th Floor, Houston, TX 77030, USA e-mail: [email protected] A. M. Adesina Division of Neuropathology, Texas Children’s Hospital, Department of Pathology, Baylor College of Medicine, 6621 Fannin Street, CCC 1230.01, 12th Floor, Houston, TX 77030, USA
History and examination This previously healthy 15-year-old Hispanic boy initially presented with a 5-month history of progressive lower extremity weakness. His symptoms started with numbness, tingling, and stiffness in his left thigh and gradually progressed so that he was unable to walk unassisted and needed to use crutches due to weakness with hip flexion and leg abduction on the left side. Approximately 2 weeks prior to his initial
Childs Nerv Syst
Fig. 1 Cutaneous hemangioma
presentation, the patient experienced fecal urgency, urinary urgency, and an episode of urinary incontinence. Upon examination, the patient had full power in his upper extremities. Left iliopsoas, quadriceps, and extensor hallicus longus power were rated at 3/5. Right iliopsoas power was 4+/ 5 with the other tested groups at full power on the right side. Patellar reflexes were hyperreflexic bilaterally with spread. Hoffman’s sign was negative bilaterally. Babinski’s sign was positive bilaterally. Sensation to light touch was intact throughout. There was no saddle anesthesia, and he had good rectal tone. A large cutaneous hemangioma was noted overlying his left flank and lumbosacral region (Fig. 1). There were no other stigmata of dysraphism. MR imaging of the spine showed an extensive enhancing infiltrative lesion with epidural extent from T2 to T12 with a possible separate focus involving the inferior end plate of L4 (Figs. 2a and 3a). Spinal cord compression was noted, as well as extension of the enhancing lesion from the epidural space Fig. 2 (a) Sagittal T2-weighted MRI, pre-operative; (b) sagittal T2-weighted MRI, post-operative
through the neural root foramina. CT imaging studies demonstrated hypoplastic left-sided pedicles throughout the lower thoracic spine extending T6–T12 with hypertrophy of the right-sided pedicles associated with expansion of the rightsided ribs. The spinal canal was deformed owing to the hemihypertrophy on the right side. Also, there was an abnormal trabecular pattern involving most of the thoracic vertebrae T4–T12. Spinal angiography demonstrated an increased capillary blush throughout the soft tissues from the upper thoracic spine to approximately L1 on the right side, without arterial venous shunting. Given his progressive symptoms and spinal cord compression, the decision was made to decompress his neural elements through multilevel laminectomies (Fig. 4). He underwent T4–T11 laminectomies removed en bloc with the assistance of a craniotome. There was abnormal tissue in the epidural space, both dorsally and laterally, on the right side of the spinal cord with ventral extension. The mass was relatively nonvascular; however, there was some capillary oozing when the mass was subtotally debulked. The patient recovered well from surgery with no immediate change in his neurological status.
Pathological findings Intraoperative cytologic preparations were paucicellular. Frozen and permanent sections demonstrated multiple dilated, congested, and thin-walled vascular channels containing serum and blood. This vascular proliferation within the fibroadipose and dense fibroconnective tissues was noted to extend into bone. Sections from the thoracic vertebrae demonstrated bone marrow infiltration and partial replacement by vascular proliferation. Uninvolved bone marrow was appropriately cellular for age and revealed maturing hematopoiesis.
Childs Nerv Syst
Fig. 3 Axial view. T2-weighted MRI at T7 at (a) presentation, (b) post-operative, (c) recurrence, (d) post-radiotherapy
The vascular endothelial cells were positive for CD34 and negative for D2-40 and Glut-1. This immunohistochemical staining pattern is consistent with a benign cavernous hemangioma (Fig. 5).
Follow-up and representation MR imaging 5 months after follow-up demonstrated partial resection of the lesion and resolution of cord compression (Figs. 2b and 3b). In addition, there was radiographic improvement of spinal cord edema with decreased T2 signal. The patient had no complaints of back or leg pain, demonstrated near-normal strength in his lower extremities, and was
able to ambulate without assistance. He was given follow-up instructions for radiotherapy to address his residual disease. Unfortunately, the patient was lost to follow-up, and no radiotherapy was delivered. Three years later, the patient represented with 1 month of worsening leg weakness and a need for crutches while walking. On motor confrontation, strength in the illiopsoas, hamstrings, and extensory hallicus longus were noted to be 2/5 bilaterally. Sustained clonus was elicited with bilateral ankle jerks, and the left patella reflex was hyperreflexic with spread. He had diminished proprioception in his bilateral lower extremities. Thoracolumbar MR revealed a significant increase in his known diffuse angiomatosis with a substantial increase in the epidural component associated with progressive cord compression (Figs. 3c and 6a). Given the prior history of bony decompression and infeasibility of surgical cure, the patient was referred urgently for radiotherapy. Steroids were started and tapered off with the completion of radiotherapy. Aggressive inpatient rehabilitation was simultaneously pursued. At 6 months follow-up, the patient had recovered full 5/5 strength in all groups except for mild 4+/5 power in his right hip flexors. He is now fully ambulatory without assistance. Interval MR imaging has demonstrated decreased cord compression with very small residual disease (Figs. 3d and 6b).
Discussion
Fig. 4 En bloc multilevel laminectomy performed for the decompression of the spinal cord
Little is known about the natural history of vertebral hemangiomas. These benign neoplasms have a wide range of clinical presentations, from incidental lesions to symptomatic lesions with moderately aggressive behavior to rare biologically active lesions that compress the spinal cord or nerve root(s) and require aggressive surgical treatment [4, 9, 10, 13], as in our case. New-onset back pain followed by subacute progression of thoracic myelopathy is the most common clinical presentation in patients with neurological deficits [4]. Four different pathophysiological mechanisms have been described as to cause spinal cord compression by vertebral hemangiomas [4]. The most common mechanism is encroachment of the spinal canal due to tumoral enlargement of the vertebral body.
Childs Nerv Syst Fig. 5 Composite image showing a cavernous vascular channels filled with blood, b CD34-positive immunostain in the endothelial cells, and negative immunostain for (c) D2-40 and (d) Glut-1
Other mechanisms include tumor extension into the epidural space (as was the case in our patient), compression fracture, and bleeding from the mass into the epidural space [2–4, 16]. Most vertebral hemangiomas that cause spinal cord compression are located in the thoracic spine [10]. Multilevel involvement has been reported; but to the best of our knowledge, there has been only one report associated with spinal cord compression in an adult patient, a 41-year-old Fig. 6 (a) Sagittal T2-weighted MRI, recurrence preradiotherapy; (b) sagittal T2weighted MRI, post-radiotherapy
man with Klippel-Weber-Trenaunay syndrome [5]. Vertebral hemangiomas, usually benign vascular tumors, must be differentiated from vascular myelopathies, which are associated with vascular malformations of the spine, such as capillary telangectasias, AVMs, AVFs, capillary angiomas, and aneurysms, as may be seen in metameric angiomatosis (Cobb syndrome) and disseminated angiomatosis (Rendu-Osler-Weber syndrome). In our patient, both Klippel-Trenaunay-
Childs Nerv Syst
Weber and Cobb syndromes were considered, the former due to the finding of vertebral hemihypertrophy and the latter because of the large neurocutaneous hemangioma in association with underlying spinal involvement. Unfortunately, clinical genetic testing was not available to test for these two syndromes in this case. Diagnosis of vertebral hemangioma is made by radiologic evaluation. If at least one-third of the vertebral body is involved, a honeycomb appearance and compression fractures may be seen on plain X-rays. Thickened vertical trabeculae may be seen on axial CT slices and may be described as “polka dot” [2, 13]. MRI plays an important role in the diagnosis of hemangiomas. The differentiation between intra- and extraosseous hemangiomas is important, as extraosseous hemangiomas are associated more frequently with symptomatic lesions. Inactive hemangiomas have predominantly fatty avascular stromata showing high signal intensity on both T1- and T2-weighted MRI, but active lesions have soft tissue hypervascular stromata showing low signal intensity on T1-weighted MRI [1, 9, 14]. Treatment for vertebral hemangiomas is indicated if symptoms, such as neurological deficits or severe pain, develop. The most common treatment option for vertebral hemangioma associated with pain is radiotherapy. If radiotherapy is used as a single treatment modality, a total dose of 30–40 Gy in 15–20 fractions is recommended [6, 15]. Endovascular treatment via embolizaton is another option. However, embolization is not routinely used as the sole modality to manage vertebral hemangiomas. Embolization may be used as a pre-operative adjunct, but it is associated with significant risks, especially in the watershed area of the midthoracic spine [1]. Surgical intralesional resection, either anteriorly or posteriorly, has also been advocated. Corpectomy with strut graft may be the most appropriate intervention, based on the degree of anterior vertebral involvement [4]. In cases of spinal cord compression, a decompressive laminectomy is usually performed [8], especially in multilevel involvement as demonstrated in our case. Subtotal resection is usually followed by post-operative radiotherapy, since post-operative radiotherapy can significantly decrease the number of recurrences [2]. Had post-operative radiotherapy, therefore, been performed in this case as was planned, recurrence may have been avoided. From our case study, we found that a child who has multilevel vertebral hemangiomas causing symptomatic spinal cord compression can be safely and effectively treated with multilevel decompressive laminectomy in the acute setting. Surgery, however, as this case highlights, is not curative, and recurrent cord compression can occur. Therefore, surgery should be followed by radiotherapy to address residual disease.
Conclusions Vertebral hemangiomas are common and may be multilevel in location. Those that cause compressive neurological symptoms are rare and usually slow in onset. In cases of progressive neurological deficits, surgical decompression— usually in the form of laminectomy—is indicated. Subtotal resection should be followed by radiotherapy to decrease the chance of recurrence. To the best of our knowledge, this is the first case to highlight how to treat children with multilevel vertebral hemangiomas causing symptomatic spinal cord compression. In our case study, we found that multilevel decompressive laminectomy can safely and effectively resolve spinal cord compression, but it should be followed by radiotherapy to address residual disease. Acknowledgments We appreciate the editorial assistance provided by Ms. Kristine Eco. Ethical standards This study was approved by the BCM IRB. All persons gave written consent prior to inclusion in the study. Conflict of interest None of the authors have any conflicts of interest.
References 1. Aksu G, Fayda M, Saynak M, Karadeniz A (2008) Spinal cord compression due to vertebral hemangioma. Orthopedics 31:169 2. Bremnes RM, Hauge HN, Sagsveen R (1996) Radiotherapy in the treatment of symptomatic vertebral hemangiomas: technical case report. Neurosurg 39:1054–1058 3. Castel E, Lazennec JY, Chiras J, Enkaoua E, Saillant G (1999) Acute spinal cord compression due to intraspinal bleeding from a vertebral hemangioma: two case reports. Eur Spine J 8:244–248 4. Fox MW, Onofrio BM (1993) The natural history and management of symptomatic and asymptomatic vertebral hemangiomas. J Neurosurg 78:36–45 5. Grau SJ, Holtmannspoetter M, Seelos K, Tonn JC, Siefert A (2009) Giant multilevel thoracic hemangioma with spinal cord compression in a patient with Klippel-Weber-Trenaunay syndrome: case report. Spine 34:498–500 6. Guedea F, Majó J, Guardia E, Canals E, Craven-Bartle J (1994) The role of radiation therapy in vertebral hemangiomas without neurological signs. Int Orthop 18:77–79 7. Hillman J, Bynke O (1991) Solitary extradural cavernous hemangiomas in the spinal canal: report of five cases. Surg Neurol 36:19–24 8. Krueger EG, Sobel GL, Weinstein C (1961) Vertebral hemangioma with compression of spinal cord. J Neurosurg 18:331–338 9. Laredo JD, Assouline E, Gelbert F, Wybier M, Merland JJ, Tubiana JM (1990) Vertebral hemangiomas: fat content as a sign of aggressiveness. Radiology 177:467–472 10. Laredo JD, Reizine D, Bard M, Merland JJ (1986) Vertebral hemangiomas: radiologic evaluation. Radiology 161:183–189 11. Lee S, Hadlow AT (1999) Extraosseous extension of vertebral hemangioma, a rare cause of spinal cord compression. Spine 24: 2111–2114
Childs Nerv Syst 12. Nguyen JP, Djindjian M, Gaston A, Gherardi R, Benhaiem N, Caron JP et al (1987) Vertebral hemangiomas presenting with neurologic symptoms. Surg Neurol 27:391–397 13. Pastushyn AI, Slin’ko EI, Mirzoyeva GM (1998) Vertebral hemangiomas: diagnosis, management, natural history and clinicopathological correlates in 86 patients. Surg Neurol 50:535–547 14. Ross JS, Masaryk TJ, Modic MT, Carter JR, Mapstone T, Dengel FH (1987) Vertebral hemangiomas: MR imaging. Radiology 165:165–169
15. Schild SE, Buskirk SJ, Frick LM, Cupps RE (1991) Radiotherapy for large symptomatic hemangiomas. Int J Radiat Oncol 21:729–735 16. Schnyder P, Fankhauser H, Mansouri B (1986) Computed tomography in spinal hemangioma with cord compression: report of two cases. Skeletal Radiol 15:372–375 17. Yazici M, Iyigun OL, Gulman B, Rakunt C, Cizmeli O (1996) Vertebral hemangioma presenting with intermittent claudication. Eur Spine J 5: 131–133
CASE REPORT
Multilevel thoracic hemangioma with spinal cord compression in a pediatric patient: case report and review of the literature Jacob Cherian & Christina M. Sayama & Adekunle M. Adesina & Sandi K. Lam & Thomas G. Luerssen & Andrew Jea
Received: 7 May 2014 / Accepted: 12 May 2014 # Springer-Verlag Berlin Heidelberg 2014
Abstract Purpose Vertebral hemangiomas are common benign vascular tumors of the spine. It is very rare for these lesions to symptomatically compress neural elements. If spinal cord compression does occur, it usually involves only a single level. Multilevel vertebral hemangiomas causing symptomatic spinal cord compression have never been reported in the pediatric population to the best of our knowledge. Methods We report the case of a 15-year-old boy presenting with progressive paraparesis due to thoracic spinal cord compression from a multilevel thoracic hemangioma (T5–T10) with epidural extension. Results Because of his progressive neurological deficit, he was initially treated with urgent multilevel decompressive laminectomies from T4 to T11. This was to be followed by radiotherapy for residual tumor, but the patient was unfortunately lost to follow-up. He re-presented 3 years later with recurrent paraparesis and progressive disease. This was treated with urgent radiotherapy with good response. As of 6 months follow-up, he has made an excellent neurological recovery. Conclusions In this report, we present the first case of a child with multilevel vertebral hemangiomas causing symptomatic spinal cord compression and review the literature to detail the pathophysiology, management, and treatment of other cases of spinal cord compression by vertebral hemangiomas.
Keywords Thoracic spine . Vertebral hemangioma . Spinal cord compression
Introduction Hemangiomas are the most common benign tumor of the vertebrae and are often asymptomatic [4]. There are three histological types of vertebral hemangiomas: capillary, cavernous, and mixed [7, 12]. In rare cases, these neoplasms can cause spinal cord compression and result in progressive neurological deficits, usually at a single vertebral level [3, 4, 11, 17]. Extensive vascularity of these lesions suggests aggressive behavior, but the natural history of these lesions remains unknown [4, 9, 10, 13]. Often, multilevel involvement of vertebral hemangiomas has been documented [4]. In this study, we report a unique case of multilevel (T5-T10) thoracic hemangiomas with epidural extension causing symptomatic spinal cord compression in a 15-yearold boy. To the best of our knowledge, this is the first case description of its kind in the pediatric age group. In addition, we review the literature to discuss the natural history, management, and treatment options of vertebral hemangiomas.
Case presentation J. Cherian : C. M. Sayama : S. K. Lam : T. G. Luerssen : A. Jea (*) Division of Pediatric Neurosurgery, Texas Children’s Hospital, Department of Neurosurgery, Baylor College of Medicine, 6621 Fannin Street, CCC 1230.01, 12th Floor, Houston, TX 77030, USA e-mail: [email protected] A. M. Adesina Division of Neuropathology, Texas Children’s Hospital, Department of Pathology, Baylor College of Medicine, 6621 Fannin Street, CCC 1230.01, 12th Floor, Houston, TX 77030, USA
History and examination This previously healthy 15-year-old Hispanic boy initially presented with a 5-month history of progressive lower extremity weakness. His symptoms started with numbness, tingling, and stiffness in his left thigh and gradually progressed so that he was unable to walk unassisted and needed to use crutches due to weakness with hip flexion and leg abduction on the left side. Approximately 2 weeks prior to his initial
Childs Nerv Syst
Fig. 1 Cutaneous hemangioma
presentation, the patient experienced fecal urgency, urinary urgency, and an episode of urinary incontinence. Upon examination, the patient had full power in his upper extremities. Left iliopsoas, quadriceps, and extensor hallicus longus power were rated at 3/5. Right iliopsoas power was 4+/ 5 with the other tested groups at full power on the right side. Patellar reflexes were hyperreflexic bilaterally with spread. Hoffman’s sign was negative bilaterally. Babinski’s sign was positive bilaterally. Sensation to light touch was intact throughout. There was no saddle anesthesia, and he had good rectal tone. A large cutaneous hemangioma was noted overlying his left flank and lumbosacral region (Fig. 1). There were no other stigmata of dysraphism. MR imaging of the spine showed an extensive enhancing infiltrative lesion with epidural extent from T2 to T12 with a possible separate focus involving the inferior end plate of L4 (Figs. 2a and 3a). Spinal cord compression was noted, as well as extension of the enhancing lesion from the epidural space Fig. 2 (a) Sagittal T2-weighted MRI, pre-operative; (b) sagittal T2-weighted MRI, post-operative
through the neural root foramina. CT imaging studies demonstrated hypoplastic left-sided pedicles throughout the lower thoracic spine extending T6–T12 with hypertrophy of the right-sided pedicles associated with expansion of the rightsided ribs. The spinal canal was deformed owing to the hemihypertrophy on the right side. Also, there was an abnormal trabecular pattern involving most of the thoracic vertebrae T4–T12. Spinal angiography demonstrated an increased capillary blush throughout the soft tissues from the upper thoracic spine to approximately L1 on the right side, without arterial venous shunting. Given his progressive symptoms and spinal cord compression, the decision was made to decompress his neural elements through multilevel laminectomies (Fig. 4). He underwent T4–T11 laminectomies removed en bloc with the assistance of a craniotome. There was abnormal tissue in the epidural space, both dorsally and laterally, on the right side of the spinal cord with ventral extension. The mass was relatively nonvascular; however, there was some capillary oozing when the mass was subtotally debulked. The patient recovered well from surgery with no immediate change in his neurological status.
Pathological findings Intraoperative cytologic preparations were paucicellular. Frozen and permanent sections demonstrated multiple dilated, congested, and thin-walled vascular channels containing serum and blood. This vascular proliferation within the fibroadipose and dense fibroconnective tissues was noted to extend into bone. Sections from the thoracic vertebrae demonstrated bone marrow infiltration and partial replacement by vascular proliferation. Uninvolved bone marrow was appropriately cellular for age and revealed maturing hematopoiesis.
Childs Nerv Syst
Fig. 3 Axial view. T2-weighted MRI at T7 at (a) presentation, (b) post-operative, (c) recurrence, (d) post-radiotherapy
The vascular endothelial cells were positive for CD34 and negative for D2-40 and Glut-1. This immunohistochemical staining pattern is consistent with a benign cavernous hemangioma (Fig. 5).
Follow-up and representation MR imaging 5 months after follow-up demonstrated partial resection of the lesion and resolution of cord compression (Figs. 2b and 3b). In addition, there was radiographic improvement of spinal cord edema with decreased T2 signal. The patient had no complaints of back or leg pain, demonstrated near-normal strength in his lower extremities, and was
able to ambulate without assistance. He was given follow-up instructions for radiotherapy to address his residual disease. Unfortunately, the patient was lost to follow-up, and no radiotherapy was delivered. Three years later, the patient represented with 1 month of worsening leg weakness and a need for crutches while walking. On motor confrontation, strength in the illiopsoas, hamstrings, and extensory hallicus longus were noted to be 2/5 bilaterally. Sustained clonus was elicited with bilateral ankle jerks, and the left patella reflex was hyperreflexic with spread. He had diminished proprioception in his bilateral lower extremities. Thoracolumbar MR revealed a significant increase in his known diffuse angiomatosis with a substantial increase in the epidural component associated with progressive cord compression (Figs. 3c and 6a). Given the prior history of bony decompression and infeasibility of surgical cure, the patient was referred urgently for radiotherapy. Steroids were started and tapered off with the completion of radiotherapy. Aggressive inpatient rehabilitation was simultaneously pursued. At 6 months follow-up, the patient had recovered full 5/5 strength in all groups except for mild 4+/5 power in his right hip flexors. He is now fully ambulatory without assistance. Interval MR imaging has demonstrated decreased cord compression with very small residual disease (Figs. 3d and 6b).
Discussion
Fig. 4 En bloc multilevel laminectomy performed for the decompression of the spinal cord
Little is known about the natural history of vertebral hemangiomas. These benign neoplasms have a wide range of clinical presentations, from incidental lesions to symptomatic lesions with moderately aggressive behavior to rare biologically active lesions that compress the spinal cord or nerve root(s) and require aggressive surgical treatment [4, 9, 10, 13], as in our case. New-onset back pain followed by subacute progression of thoracic myelopathy is the most common clinical presentation in patients with neurological deficits [4]. Four different pathophysiological mechanisms have been described as to cause spinal cord compression by vertebral hemangiomas [4]. The most common mechanism is encroachment of the spinal canal due to tumoral enlargement of the vertebral body.
Childs Nerv Syst Fig. 5 Composite image showing a cavernous vascular channels filled with blood, b CD34-positive immunostain in the endothelial cells, and negative immunostain for (c) D2-40 and (d) Glut-1
Other mechanisms include tumor extension into the epidural space (as was the case in our patient), compression fracture, and bleeding from the mass into the epidural space [2–4, 16]. Most vertebral hemangiomas that cause spinal cord compression are located in the thoracic spine [10]. Multilevel involvement has been reported; but to the best of our knowledge, there has been only one report associated with spinal cord compression in an adult patient, a 41-year-old Fig. 6 (a) Sagittal T2-weighted MRI, recurrence preradiotherapy; (b) sagittal T2weighted MRI, post-radiotherapy
man with Klippel-Weber-Trenaunay syndrome [5]. Vertebral hemangiomas, usually benign vascular tumors, must be differentiated from vascular myelopathies, which are associated with vascular malformations of the spine, such as capillary telangectasias, AVMs, AVFs, capillary angiomas, and aneurysms, as may be seen in metameric angiomatosis (Cobb syndrome) and disseminated angiomatosis (Rendu-Osler-Weber syndrome). In our patient, both Klippel-Trenaunay-
Childs Nerv Syst
Weber and Cobb syndromes were considered, the former due to the finding of vertebral hemihypertrophy and the latter because of the large neurocutaneous hemangioma in association with underlying spinal involvement. Unfortunately, clinical genetic testing was not available to test for these two syndromes in this case. Diagnosis of vertebral hemangioma is made by radiologic evaluation. If at least one-third of the vertebral body is involved, a honeycomb appearance and compression fractures may be seen on plain X-rays. Thickened vertical trabeculae may be seen on axial CT slices and may be described as “polka dot” [2, 13]. MRI plays an important role in the diagnosis of hemangiomas. The differentiation between intra- and extraosseous hemangiomas is important, as extraosseous hemangiomas are associated more frequently with symptomatic lesions. Inactive hemangiomas have predominantly fatty avascular stromata showing high signal intensity on both T1- and T2-weighted MRI, but active lesions have soft tissue hypervascular stromata showing low signal intensity on T1-weighted MRI [1, 9, 14]. Treatment for vertebral hemangiomas is indicated if symptoms, such as neurological deficits or severe pain, develop. The most common treatment option for vertebral hemangioma associated with pain is radiotherapy. If radiotherapy is used as a single treatment modality, a total dose of 30–40 Gy in 15–20 fractions is recommended [6, 15]. Endovascular treatment via embolizaton is another option. However, embolization is not routinely used as the sole modality to manage vertebral hemangiomas. Embolization may be used as a pre-operative adjunct, but it is associated with significant risks, especially in the watershed area of the midthoracic spine [1]. Surgical intralesional resection, either anteriorly or posteriorly, has also been advocated. Corpectomy with strut graft may be the most appropriate intervention, based on the degree of anterior vertebral involvement [4]. In cases of spinal cord compression, a decompressive laminectomy is usually performed [8], especially in multilevel involvement as demonstrated in our case. Subtotal resection is usually followed by post-operative radiotherapy, since post-operative radiotherapy can significantly decrease the number of recurrences [2]. Had post-operative radiotherapy, therefore, been performed in this case as was planned, recurrence may have been avoided. From our case study, we found that a child who has multilevel vertebral hemangiomas causing symptomatic spinal cord compression can be safely and effectively treated with multilevel decompressive laminectomy in the acute setting. Surgery, however, as this case highlights, is not curative, and recurrent cord compression can occur. Therefore, surgery should be followed by radiotherapy to address residual disease.
Conclusions Vertebral hemangiomas are common and may be multilevel in location. Those that cause compressive neurological symptoms are rare and usually slow in onset. In cases of progressive neurological deficits, surgical decompression— usually in the form of laminectomy—is indicated. Subtotal resection should be followed by radiotherapy to decrease the chance of recurrence. To the best of our knowledge, this is the first case to highlight how to treat children with multilevel vertebral hemangiomas causing symptomatic spinal cord compression. In our case study, we found that multilevel decompressive laminectomy can safely and effectively resolve spinal cord compression, but it should be followed by radiotherapy to address residual disease. Acknowledgments We appreciate the editorial assistance provided by Ms. Kristine Eco. Ethical standards This study was approved by the BCM IRB. All persons gave written consent prior to inclusion in the study. Conflict of interest None of the authors have any conflicts of interest.
References 1. Aksu G, Fayda M, Saynak M, Karadeniz A (2008) Spinal cord compression due to vertebral hemangioma. Orthopedics 31:169 2. Bremnes RM, Hauge HN, Sagsveen R (1996) Radiotherapy in the treatment of symptomatic vertebral hemangiomas: technical case report. Neurosurg 39:1054–1058 3. Castel E, Lazennec JY, Chiras J, Enkaoua E, Saillant G (1999) Acute spinal cord compression due to intraspinal bleeding from a vertebral hemangioma: two case reports. Eur Spine J 8:244–248 4. Fox MW, Onofrio BM (1993) The natural history and management of symptomatic and asymptomatic vertebral hemangiomas. J Neurosurg 78:36–45 5. Grau SJ, Holtmannspoetter M, Seelos K, Tonn JC, Siefert A (2009) Giant multilevel thoracic hemangioma with spinal cord compression in a patient with Klippel-Weber-Trenaunay syndrome: case report. Spine 34:498–500 6. Guedea F, Majó J, Guardia E, Canals E, Craven-Bartle J (1994) The role of radiation therapy in vertebral hemangiomas without neurological signs. Int Orthop 18:77–79 7. Hillman J, Bynke O (1991) Solitary extradural cavernous hemangiomas in the spinal canal: report of five cases. Surg Neurol 36:19–24 8. Krueger EG, Sobel GL, Weinstein C (1961) Vertebral hemangioma with compression of spinal cord. J Neurosurg 18:331–338 9. Laredo JD, Assouline E, Gelbert F, Wybier M, Merland JJ, Tubiana JM (1990) Vertebral hemangiomas: fat content as a sign of aggressiveness. Radiology 177:467–472 10. Laredo JD, Reizine D, Bard M, Merland JJ (1986) Vertebral hemangiomas: radiologic evaluation. Radiology 161:183–189 11. Lee S, Hadlow AT (1999) Extraosseous extension of vertebral hemangioma, a rare cause of spinal cord compression. Spine 24: 2111–2114
Childs Nerv Syst 12. Nguyen JP, Djindjian M, Gaston A, Gherardi R, Benhaiem N, Caron JP et al (1987) Vertebral hemangiomas presenting with neurologic symptoms. Surg Neurol 27:391–397 13. Pastushyn AI, Slin’ko EI, Mirzoyeva GM (1998) Vertebral hemangiomas: diagnosis, management, natural history and clinicopathological correlates in 86 patients. Surg Neurol 50:535–547 14. Ross JS, Masaryk TJ, Modic MT, Carter JR, Mapstone T, Dengel FH (1987) Vertebral hemangiomas: MR imaging. Radiology 165:165–169
15. Schild SE, Buskirk SJ, Frick LM, Cupps RE (1991) Radiotherapy for large symptomatic hemangiomas. Int J Radiat Oncol 21:729–735 16. Schnyder P, Fankhauser H, Mansouri B (1986) Computed tomography in spinal hemangioma with cord compression: report of two cases. Skeletal Radiol 15:372–375 17. Yazici M, Iyigun OL, Gulman B, Rakunt C, Cizmeli O (1996) Vertebral hemangioma presenting with intermittent claudication. Eur Spine J 5: 131–133
