- Email: [email protected]
Metastatic saccrococcygeal chordoma
Journal of Clinical Neuroscience 23 (2016) 149–152
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case Reports
Metastatic saccrococcygeal chordoma Mostafa Fatehi Hassanabad a, Alireza Mansouri b, Naif M. Alotaibi b, Lili-Naz Hazrati c, Mark Bernstein b,d,⇑ a
Division of Neurosurgery, University of British Columbia, Vancouver, Canada Division of Neurosurgery, University of Toronto, Canada c Division of Pathology, University Health Network, Toronto, Canada d Division of Neurosurgery, Toronto Western Hospital, Toronto, Canada b
a r t i c l e
i n f o
Article history: Received 26 April 2015 Accepted 2 May 2015
Keywords: Brain metastases Cerebral metastases Sacrococcygeal chordomas
a b s t r a c t We report a 46-year-old man who presented with a 2 week history of worsening headaches and acute onset left sided hemiplegia. He had undergone a surgical resection of a sacral chordoma 13 years prior, followed by adjuvant radiotherapy and chemotherapy. MRI revealed multiple enhancing lesions in the brain, and the two largest were resected. The histopathology was consistent with chordoma. Sacrococcygeal chordomas are locally invasive notochord-related sarcomas. They rarely metastasize to the brain, and only eight patients have been reported. While currently available adjuvant radiotherapy and systemic chemotherapeutic regimens can be implemented in the management of these rare patients, they have shown limited success. The newer strategies that are reported here have also been disappointing. Ó 2015 Elsevier Ltd. All rights reserved.
1. Introduction
2. Case report
Sacrococcygeal chordomas are slow growing tumors with a propensity for local extension and recurrence. Successful treatment is limited by the difficulty of a gross total resection (GTR), and there is a 50% local recurrence rate after surgery [1–3]. Although adjuvant radiotherapy has been used to decrease local recurrence, chordomas are traditionally considered to be radioresistant and doses greater than 60–65 Gy are necessary [4]. Proton beam radiation permits the administration of higher radiation doses in sensitive areas and is now the gold standard modality for treatment of cranial base chordomas [4]. Systemic therapeutic options are an area of active research and recent studies have shown the receptor tyrosine kinase (RTK) inhibitor imatinib (Novartis AG, Basel, Switzerland) to stabilize or decrease the size of some locally advanced chordomas [3]. This effect is likely mediated through the inhibition of the plateletderived growth factor receptor beta (PDGFRb), which is commonly overexpressed in chordomas [5]. A recent prospective study showed disease stabilization in 72% and tumor shrinkage in 16% of patients [3]. Metastasis to the brain appears to be rare, and there are only eight case reports of this in the literature [1,3,6–11]. We report a 46-year-old man who presented with diffusely metastatic chordoma, originating from the sacrococcygeal region. A surgical resection and other subsequent treatments could not contain the spread of this tumor.
A 46-year-old man initially presented in October 2001 with an 8 month history of slowly progressing urinary incontinence and worsening back pain. The imaging at that time demonstrated a multilobulated tumor of the sacral area (Fig. 1A, B). A tumor debulking was attempted in October 2001, but the extensive neurovascular involvement precluded a GTR. Therefore, he underwent adjuvant radiotherapy (70 Gy/35 fractions via intensity modulated radiation therapy) from 2001–2002. Postoperatively, his back pain improved significantly but the urinary incontinence persisted. The residual tumor was initially found to be stable, however, local progression was found during the annual monitoring in 2009. Due to the significant morbidity of a potential resection, the patient chose to proceed with medical therapy with imatinib instead of repeat surgery. This treatment stabilized the local growth but a pulmonary metastasis was noted on imaging in December 2012. The patient was enrolled in an open label Phase 1 dose finding clinical trial with a new FAK inhibitor (BI 1300.2 trial), but he experienced no clinical effect on his disease. Due to the inexorable progression of his disease, systemic medical therapy was deemed ineffective and was discontinued after five cycles in November 2013. In February 2014, the patient acutely developed left sided hemiplegia (0/5 strength in arm and leg) after a 2 week history of worsening headaches and nausea. Brain MRI studies confirmed multiple brain lesions in the right hemisphere (Fig. 1C, D). The two largest tumors were in the right premotor and parietooccipital regions. The patient underwent resection of the two lesions with the greatest mass effect. The histopathological assessment confirmed
⇑ Corresponding author at: Division of Neurosurgery, Toronto Western Hospital, Toronto, Canada. Tel.: +1 416 6036499. E-mail address: [email protected] (M. Bernstein).
150
Case Reports / Journal of Clinical Neuroscience 23 (2016) 149–152
Fig. 1. (A) Sagittal T2-weighted MRI of the lumbosacral region showing a large sacrococcygeal chordoma. (B) Axial T2-weighted MRI of the sacrum. The arrow indicates the destroyed sacrum (December 2001). (C) Preoperative sagittal T1-weighted contrast-enhanced MRI of the brain showing multiple lesions in keeping with metastatic spread of chordoma. (D) Preoperative axial T2-weighted brain MRI showing the same foci. There is no enhancement with contrast (February 2014). (E) Sagittal and (F) axial T1weighted contrast-enhanced MRI. These images indicate a new partially enhancing lesion in the left occipital lobe (arrows; July 2014).
that the tumors where metastatic chordomas. Hemotoxylin and eosin staining showed lobules of physaliferous cells separated by fibrous septa and extensive myxoid stroma (Fig. 2A). Moreover, the cells expressed S100 protein focally and the epithelial membrane antigen diffusely (Fig. 2B–D). The repeat MRI of the brain in July 2014 demonstrated multiple new lesions bilaterally and his clinical course continued to decline (Fig. 1E, F). The patient passed away in December 2014.
3. Discussion Chordomas are sarcomatous lesions which account for almost 5% of malignant bone tumors [12]. The most important prognostic
factor in developing metastases is the feasibility of GTR. In fact, patients who need adjuvant radiotherapy, ostensibly due to incomplete resection, have a higher reported rate of metastasis. Kamel et al. suggest that a partial resection exposes residual tumor to the bloodstream, subsequently predisposing a metastasis [1]. Despite their propensity for widespread metastasis, sacrococcygeal tumors rarely spread to the brain. Table 1 shows a brief summary of reported patients with metastases. The overall prognosis of patients with chordomas remains fairly poor. The current treatment paradigm for sacrococcygeal tumors is wide surgical resection and postoperative external beam radiation therapy [13]. Unfortunately, the aggressive local extension of the disease leads to an inexorable decline in the functional capacity of most patients.
151
Case Reports / Journal of Clinical Neuroscience 23 (2016) 149–152
Fig. 2. (A) Hemotoxylin and eosin staining of a tumor slide shows physaliphorus cells with vacuolated cytoplasm and myxoid intercellular matrix. (B) Chordoma cells expressed the epithelial membrane antigen on immunostaining. The tumor slides were found to positively stain with (C) pan-keratin and (D) S-100 protein.
Table 1 Summary of reported patients with metastatic sacrococcygeal chordoma to the brain Patient
Report
Age (years), sex
Original treatment
Time from diagnosis to metastasis
Sites of metastases
1 2 3 4 5 6 7
Kamel et al. [1] Hall et al. [2] Chambers et al. [6] Fichardt et al. [7] Fox et al. [8] Morris et al. [10] Liao CM et al. [11]
59, 29, 59, 41, 58, 27, 30,
Partial sacrectomy, local radiotherapy Complete resection NR Resection, local radiotherapy NR Resection, local radiotherapy Resection, local radiotherapy, chemotherapy
2 years 16 months 4 years 2 months 5 years 18 months 2 months
Right occipital lobe Left occipital lobe, lungs Brain Adrenal glands, pituitary Lungs, lymph node, right parietal lobe Lungs, lymph nodes, left frontal lobe Lungs, cerebellum
M M M M F F M
F = female, M = male, NR = not reported.
We described a patient with a sacrococcygeal tumor which metastasized to the lungs and brain. Unlike previous reported patients with brain metastases, our patient underwent extensive systemic therapy, both with imatinib and experimental medications. Unfortunately, disease progression and metastasis was observed while the patient was receiving systemic chemotherapy. As evidenced here and in earlier reports, the treatment of patients with chordomas is quite challenging. Surgical resection remains the cornerstone of therapy, and achievement of a GTR is an important prognostic factor. However, new molecular research has revealed abnormal cellular pathways and signaling cascades which will be targeted by novel therapeutic approaches. For instance, almost all chordomas exhibit activated PDGFRb, and many have an activated mammalian target of rapamycin (mTOR) pathway. Several clinical trials are studying the efficacy of RTK and mTOR inhibitors in disease stabilization [3,13]. These trials may provide the theoretical foundation for effective therapeutics.
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. References [1] Kamel MH, Lim C, Kelleher M, et al. Intracranial metastasis from a sacrococcygeal chordoma. Case report. J Neurosurg 2005;102:730–2. [2] Hall WA, Clark HB. Sacrococcygeal chordoma metastatic to the brain with review of the literature. J Neurooncol 1995;25:155–9. [3] Stacchiotti S, Longhi A, Ferraresi V, et al. Phase II study of imatinib in advanced chordoma. J Clin Oncol 2012;30:914–20. [4] Foweraker KL, Burtn KE, Maynard SE, et al. High-dose radiotherapy in the management of chordoma and chondrosarcoma of the skull base and cervical spine: Part 1 – Clinical outcomes. Clin Oncol (R Coll Radiol) 2007;19:509–16. [5] Stacchiotti S, Casali PG. Systemic therapy options for unresectable and metastatic chordomas. Curr Oncol Rep 2011;13:323–30. [6] Chambers PW, Schwinn CP. Chordoma. A clinicopathologic study of metastasis. Am J Clin Pathol 1979;72:765–76.
152
Case Reports / Journal of Clinical Neuroscience 23 (2016) 152–159
[7] Fichardt T, de Villiers PC. Chordoma. S Afr Med J 1974;48:383–91. [8] Fox JE, Batsakis JG, Owano LR. Unusual manifestations of chordoma. A report of two cases. J Bone Joint Surg Am 1968;50:1618–28. [9] Higinbotham NL, Phillips RF, Farr HW, et al. Chordoma. Thirty-five-year study at Memorial Hospital. Cancer 1967;20:1841–50. [10] Morris AA, Rabinovitch R. Malignant chordoma of the lumbar region; report of a case with autopsy; comment on unusual metastases to the brain, lungs, pancreas, sacrum and axillary and iliac lymph nodes. Arch Neurol Psychiatry 1947;57:547–64.
[11] Liao CM, Kao HW, Hsueh C, et al. Cerebellar metastasis from extraosseous chordoma of the coccygeal region: a case report. Chin J Radiol 2006;31:309–15. [12] Heffelfinger MJ, Dahlin DC, MacCarty CS, et al. Chordomas and cartilaginous tumors at the skull base. Cancer 1973;32:410–20. [13] Walcott BP, Nahed BV, Mohyeldin A, et al. Chordoma: current concepts, management, and future directions. Lancet Oncol 2012;13:e69–76.
http://dx.doi.org/10.1016/j.jocn.2015.05.036
Management of delayed posttraumatic cervical kyphosis Alejandro J. Lopez a, Justin K. Scheer a, Kingsley Abode-Iyamah b, Zachary A. Smith a, Patrick W. Hitchon b, Nader S. Dahdaleh a,⇑ a b
Department of Neurological Surgery, Northwestern University, Feinberg School of Medicine, Suite 2210, 676 North Saint Clair Street, Chicago, IL 60611, USA Department of Neurosurgery, University of Iowa Hospitals and Clinics, Carver School of Medicine, Iowa City, IA, USA
a r t i c l e
i n f o
Article history: Received 29 January 2015 Accepted 2 May 2015
Keywords: Cervical fracture Kyphosis Post traumatic kyphosis Traction Unstable cervical fractures
a b s t r a c t We describe three patients with misdiagnosed unstable fractures of the cervical spine, who were treated conservatively and developed kyphotic deformity, myelopathy, and radiculopathy. All three patients were then managed with closed reductions by crown halo traction, followed by instrumented fusions. Their neurologic function was regained without permanent disability in any patient. Unstable fractures of the cervical spine will progress to catastrophic neurologic injuries without surgical fixation. Posttraumatic kyphosis and the delayed reduction of partially healed fracture dislocations by preoperative traction are not well characterized in the subaxial cervical spine. The complete evaluation of any subaxial cervical spine fracture requires CT scanning to assess for bony fractures, and MRI to assess for ligamentous injury. This allows for assessment of the degree of instability and appropriate management. In patients with delayed posttraumatic cervical kyphosis, preoperative closed reduction provided adequate realignment, facilitating subsequent operative stabilization. Ó 2015 Elsevier Ltd. All rights reserved.
1. Introduction Unstable spinal fractures require surgical fixation to prevent neurological injury [1]. Up to 29% of patients with a delayed diagnosis of an unstable cervical fracture will proceed to catastrophic neurologic deficit or mortality [2,3]. The incidence of delayed treatment of unstable cervical spine fractures has been reported to vary from 4.9–22.9% [4,3], depending on the institution [5]. In the past, the initial evaluation was performed by radiography. A three view radiograph series, consisting of lateral, open mouth, and anteroposterior views, was found to be sufficient for diagnosing greater than 99% of posttraumatic unstable cervical spine fractures [6]. However, inadequate evaluation of radiologic studies has contributed the largest percentage of delayed diagnoses [4]. An altered mental status of the patient may mask neurologic deficits and can contribute significantly to early diagnostic failures as well [4]. CT scanning is now considered the standard of care for evaluating traumatic spinal injury due to increased sensitivity and reduced reader error [7]. It also has the advantage of detecting upper cervical spine injuries, such as condylar fractures, and lower subaxial cervical spine injuries that often cannot be detected with plain radiography. In addition, an evaluation with MRI is crucial in
⇑ Corresponding author. Tel.: +1 312 695 6200; fax: +1 312 695 0225. E-mail address: [email protected] (N.S. Dahdaleh).
order to assess for ligamentous injury, as the discoligamentous and posterior ligamentous complexes contribute to spinal stability [8–10]. Unlike the thoracolumbar spine, delayed posttraumatic instability and kyphosis of the subaxial spine is not well described in the literature as a separate entity, despite being encountered in clinical practice [11–14]. Few studies have examined the delayed management of these conditions, which often present with myelopathy and radiculopathy in a subacute or chronic fashion [15]. While closed cranial traction is common practice for reducing acute fracture dislocations of the spine [16–18], its role in partially healed fractures, as in patients with delayed posttraumatic cervical kyphosis, has not been described. We report three patients who presented with myelopathy due to cervical kyphosis following the initial nonsurgical treatment of missed unstable subaxial cervical spine fractures. We detail the management of these patients, emphasizing the role of preoperative traction.
2. Materials and methods A retrospective case series of three patients with delayed diagnoses of unstable cervical spine fractures was performed. The preoperative, postoperative, and follow-up reports were retrieved, including imaging studies. The course of disease and management of each patient were summarized, including outcome measures at presentation and follow-up.
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case Reports
Metastatic saccrococcygeal chordoma Mostafa Fatehi Hassanabad a, Alireza Mansouri b, Naif M. Alotaibi b, Lili-Naz Hazrati c, Mark Bernstein b,d,⇑ a
Division of Neurosurgery, University of British Columbia, Vancouver, Canada Division of Neurosurgery, University of Toronto, Canada c Division of Pathology, University Health Network, Toronto, Canada d Division of Neurosurgery, Toronto Western Hospital, Toronto, Canada b
a r t i c l e
i n f o
Article history: Received 26 April 2015 Accepted 2 May 2015
Keywords: Brain metastases Cerebral metastases Sacrococcygeal chordomas
a b s t r a c t We report a 46-year-old man who presented with a 2 week history of worsening headaches and acute onset left sided hemiplegia. He had undergone a surgical resection of a sacral chordoma 13 years prior, followed by adjuvant radiotherapy and chemotherapy. MRI revealed multiple enhancing lesions in the brain, and the two largest were resected. The histopathology was consistent with chordoma. Sacrococcygeal chordomas are locally invasive notochord-related sarcomas. They rarely metastasize to the brain, and only eight patients have been reported. While currently available adjuvant radiotherapy and systemic chemotherapeutic regimens can be implemented in the management of these rare patients, they have shown limited success. The newer strategies that are reported here have also been disappointing. Ó 2015 Elsevier Ltd. All rights reserved.
1. Introduction
2. Case report
Sacrococcygeal chordomas are slow growing tumors with a propensity for local extension and recurrence. Successful treatment is limited by the difficulty of a gross total resection (GTR), and there is a 50% local recurrence rate after surgery [1–3]. Although adjuvant radiotherapy has been used to decrease local recurrence, chordomas are traditionally considered to be radioresistant and doses greater than 60–65 Gy are necessary [4]. Proton beam radiation permits the administration of higher radiation doses in sensitive areas and is now the gold standard modality for treatment of cranial base chordomas [4]. Systemic therapeutic options are an area of active research and recent studies have shown the receptor tyrosine kinase (RTK) inhibitor imatinib (Novartis AG, Basel, Switzerland) to stabilize or decrease the size of some locally advanced chordomas [3]. This effect is likely mediated through the inhibition of the plateletderived growth factor receptor beta (PDGFRb), which is commonly overexpressed in chordomas [5]. A recent prospective study showed disease stabilization in 72% and tumor shrinkage in 16% of patients [3]. Metastasis to the brain appears to be rare, and there are only eight case reports of this in the literature [1,3,6–11]. We report a 46-year-old man who presented with diffusely metastatic chordoma, originating from the sacrococcygeal region. A surgical resection and other subsequent treatments could not contain the spread of this tumor.
A 46-year-old man initially presented in October 2001 with an 8 month history of slowly progressing urinary incontinence and worsening back pain. The imaging at that time demonstrated a multilobulated tumor of the sacral area (Fig. 1A, B). A tumor debulking was attempted in October 2001, but the extensive neurovascular involvement precluded a GTR. Therefore, he underwent adjuvant radiotherapy (70 Gy/35 fractions via intensity modulated radiation therapy) from 2001–2002. Postoperatively, his back pain improved significantly but the urinary incontinence persisted. The residual tumor was initially found to be stable, however, local progression was found during the annual monitoring in 2009. Due to the significant morbidity of a potential resection, the patient chose to proceed with medical therapy with imatinib instead of repeat surgery. This treatment stabilized the local growth but a pulmonary metastasis was noted on imaging in December 2012. The patient was enrolled in an open label Phase 1 dose finding clinical trial with a new FAK inhibitor (BI 1300.2 trial), but he experienced no clinical effect on his disease. Due to the inexorable progression of his disease, systemic medical therapy was deemed ineffective and was discontinued after five cycles in November 2013. In February 2014, the patient acutely developed left sided hemiplegia (0/5 strength in arm and leg) after a 2 week history of worsening headaches and nausea. Brain MRI studies confirmed multiple brain lesions in the right hemisphere (Fig. 1C, D). The two largest tumors were in the right premotor and parietooccipital regions. The patient underwent resection of the two lesions with the greatest mass effect. The histopathological assessment confirmed
⇑ Corresponding author at: Division of Neurosurgery, Toronto Western Hospital, Toronto, Canada. Tel.: +1 416 6036499. E-mail address: [email protected] (M. Bernstein).
150
Case Reports / Journal of Clinical Neuroscience 23 (2016) 149–152
Fig. 1. (A) Sagittal T2-weighted MRI of the lumbosacral region showing a large sacrococcygeal chordoma. (B) Axial T2-weighted MRI of the sacrum. The arrow indicates the destroyed sacrum (December 2001). (C) Preoperative sagittal T1-weighted contrast-enhanced MRI of the brain showing multiple lesions in keeping with metastatic spread of chordoma. (D) Preoperative axial T2-weighted brain MRI showing the same foci. There is no enhancement with contrast (February 2014). (E) Sagittal and (F) axial T1weighted contrast-enhanced MRI. These images indicate a new partially enhancing lesion in the left occipital lobe (arrows; July 2014).
that the tumors where metastatic chordomas. Hemotoxylin and eosin staining showed lobules of physaliferous cells separated by fibrous septa and extensive myxoid stroma (Fig. 2A). Moreover, the cells expressed S100 protein focally and the epithelial membrane antigen diffusely (Fig. 2B–D). The repeat MRI of the brain in July 2014 demonstrated multiple new lesions bilaterally and his clinical course continued to decline (Fig. 1E, F). The patient passed away in December 2014.
3. Discussion Chordomas are sarcomatous lesions which account for almost 5% of malignant bone tumors [12]. The most important prognostic
factor in developing metastases is the feasibility of GTR. In fact, patients who need adjuvant radiotherapy, ostensibly due to incomplete resection, have a higher reported rate of metastasis. Kamel et al. suggest that a partial resection exposes residual tumor to the bloodstream, subsequently predisposing a metastasis [1]. Despite their propensity for widespread metastasis, sacrococcygeal tumors rarely spread to the brain. Table 1 shows a brief summary of reported patients with metastases. The overall prognosis of patients with chordomas remains fairly poor. The current treatment paradigm for sacrococcygeal tumors is wide surgical resection and postoperative external beam radiation therapy [13]. Unfortunately, the aggressive local extension of the disease leads to an inexorable decline in the functional capacity of most patients.
151
Case Reports / Journal of Clinical Neuroscience 23 (2016) 149–152
Fig. 2. (A) Hemotoxylin and eosin staining of a tumor slide shows physaliphorus cells with vacuolated cytoplasm and myxoid intercellular matrix. (B) Chordoma cells expressed the epithelial membrane antigen on immunostaining. The tumor slides were found to positively stain with (C) pan-keratin and (D) S-100 protein.
Table 1 Summary of reported patients with metastatic sacrococcygeal chordoma to the brain Patient
Report
Age (years), sex
Original treatment
Time from diagnosis to metastasis
Sites of metastases
1 2 3 4 5 6 7
Kamel et al. [1] Hall et al. [2] Chambers et al. [6] Fichardt et al. [7] Fox et al. [8] Morris et al. [10] Liao CM et al. [11]
59, 29, 59, 41, 58, 27, 30,
Partial sacrectomy, local radiotherapy Complete resection NR Resection, local radiotherapy NR Resection, local radiotherapy Resection, local radiotherapy, chemotherapy
2 years 16 months 4 years 2 months 5 years 18 months 2 months
Right occipital lobe Left occipital lobe, lungs Brain Adrenal glands, pituitary Lungs, lymph node, right parietal lobe Lungs, lymph nodes, left frontal lobe Lungs, cerebellum
M M M M F F M
F = female, M = male, NR = not reported.
We described a patient with a sacrococcygeal tumor which metastasized to the lungs and brain. Unlike previous reported patients with brain metastases, our patient underwent extensive systemic therapy, both with imatinib and experimental medications. Unfortunately, disease progression and metastasis was observed while the patient was receiving systemic chemotherapy. As evidenced here and in earlier reports, the treatment of patients with chordomas is quite challenging. Surgical resection remains the cornerstone of therapy, and achievement of a GTR is an important prognostic factor. However, new molecular research has revealed abnormal cellular pathways and signaling cascades which will be targeted by novel therapeutic approaches. For instance, almost all chordomas exhibit activated PDGFRb, and many have an activated mammalian target of rapamycin (mTOR) pathway. Several clinical trials are studying the efficacy of RTK and mTOR inhibitors in disease stabilization [3,13]. These trials may provide the theoretical foundation for effective therapeutics.
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. References [1] Kamel MH, Lim C, Kelleher M, et al. Intracranial metastasis from a sacrococcygeal chordoma. Case report. J Neurosurg 2005;102:730–2. [2] Hall WA, Clark HB. Sacrococcygeal chordoma metastatic to the brain with review of the literature. J Neurooncol 1995;25:155–9. [3] Stacchiotti S, Longhi A, Ferraresi V, et al. Phase II study of imatinib in advanced chordoma. J Clin Oncol 2012;30:914–20. [4] Foweraker KL, Burtn KE, Maynard SE, et al. High-dose radiotherapy in the management of chordoma and chondrosarcoma of the skull base and cervical spine: Part 1 – Clinical outcomes. Clin Oncol (R Coll Radiol) 2007;19:509–16. [5] Stacchiotti S, Casali PG. Systemic therapy options for unresectable and metastatic chordomas. Curr Oncol Rep 2011;13:323–30. [6] Chambers PW, Schwinn CP. Chordoma. A clinicopathologic study of metastasis. Am J Clin Pathol 1979;72:765–76.
152
Case Reports / Journal of Clinical Neuroscience 23 (2016) 152–159
[7] Fichardt T, de Villiers PC. Chordoma. S Afr Med J 1974;48:383–91. [8] Fox JE, Batsakis JG, Owano LR. Unusual manifestations of chordoma. A report of two cases. J Bone Joint Surg Am 1968;50:1618–28. [9] Higinbotham NL, Phillips RF, Farr HW, et al. Chordoma. Thirty-five-year study at Memorial Hospital. Cancer 1967;20:1841–50. [10] Morris AA, Rabinovitch R. Malignant chordoma of the lumbar region; report of a case with autopsy; comment on unusual metastases to the brain, lungs, pancreas, sacrum and axillary and iliac lymph nodes. Arch Neurol Psychiatry 1947;57:547–64.
[11] Liao CM, Kao HW, Hsueh C, et al. Cerebellar metastasis from extraosseous chordoma of the coccygeal region: a case report. Chin J Radiol 2006;31:309–15. [12] Heffelfinger MJ, Dahlin DC, MacCarty CS, et al. Chordomas and cartilaginous tumors at the skull base. Cancer 1973;32:410–20. [13] Walcott BP, Nahed BV, Mohyeldin A, et al. Chordoma: current concepts, management, and future directions. Lancet Oncol 2012;13:e69–76.
http://dx.doi.org/10.1016/j.jocn.2015.05.036
Management of delayed posttraumatic cervical kyphosis Alejandro J. Lopez a, Justin K. Scheer a, Kingsley Abode-Iyamah b, Zachary A. Smith a, Patrick W. Hitchon b, Nader S. Dahdaleh a,⇑ a b
Department of Neurological Surgery, Northwestern University, Feinberg School of Medicine, Suite 2210, 676 North Saint Clair Street, Chicago, IL 60611, USA Department of Neurosurgery, University of Iowa Hospitals and Clinics, Carver School of Medicine, Iowa City, IA, USA
a r t i c l e
i n f o
Article history: Received 29 January 2015 Accepted 2 May 2015
Keywords: Cervical fracture Kyphosis Post traumatic kyphosis Traction Unstable cervical fractures
a b s t r a c t We describe three patients with misdiagnosed unstable fractures of the cervical spine, who were treated conservatively and developed kyphotic deformity, myelopathy, and radiculopathy. All three patients were then managed with closed reductions by crown halo traction, followed by instrumented fusions. Their neurologic function was regained without permanent disability in any patient. Unstable fractures of the cervical spine will progress to catastrophic neurologic injuries without surgical fixation. Posttraumatic kyphosis and the delayed reduction of partially healed fracture dislocations by preoperative traction are not well characterized in the subaxial cervical spine. The complete evaluation of any subaxial cervical spine fracture requires CT scanning to assess for bony fractures, and MRI to assess for ligamentous injury. This allows for assessment of the degree of instability and appropriate management. In patients with delayed posttraumatic cervical kyphosis, preoperative closed reduction provided adequate realignment, facilitating subsequent operative stabilization. Ó 2015 Elsevier Ltd. All rights reserved.
1. Introduction Unstable spinal fractures require surgical fixation to prevent neurological injury [1]. Up to 29% of patients with a delayed diagnosis of an unstable cervical fracture will proceed to catastrophic neurologic deficit or mortality [2,3]. The incidence of delayed treatment of unstable cervical spine fractures has been reported to vary from 4.9–22.9% [4,3], depending on the institution [5]. In the past, the initial evaluation was performed by radiography. A three view radiograph series, consisting of lateral, open mouth, and anteroposterior views, was found to be sufficient for diagnosing greater than 99% of posttraumatic unstable cervical spine fractures [6]. However, inadequate evaluation of radiologic studies has contributed the largest percentage of delayed diagnoses [4]. An altered mental status of the patient may mask neurologic deficits and can contribute significantly to early diagnostic failures as well [4]. CT scanning is now considered the standard of care for evaluating traumatic spinal injury due to increased sensitivity and reduced reader error [7]. It also has the advantage of detecting upper cervical spine injuries, such as condylar fractures, and lower subaxial cervical spine injuries that often cannot be detected with plain radiography. In addition, an evaluation with MRI is crucial in
⇑ Corresponding author. Tel.: +1 312 695 6200; fax: +1 312 695 0225. E-mail address: [email protected] (N.S. Dahdaleh).
order to assess for ligamentous injury, as the discoligamentous and posterior ligamentous complexes contribute to spinal stability [8–10]. Unlike the thoracolumbar spine, delayed posttraumatic instability and kyphosis of the subaxial spine is not well described in the literature as a separate entity, despite being encountered in clinical practice [11–14]. Few studies have examined the delayed management of these conditions, which often present with myelopathy and radiculopathy in a subacute or chronic fashion [15]. While closed cranial traction is common practice for reducing acute fracture dislocations of the spine [16–18], its role in partially healed fractures, as in patients with delayed posttraumatic cervical kyphosis, has not been described. We report three patients who presented with myelopathy due to cervical kyphosis following the initial nonsurgical treatment of missed unstable subaxial cervical spine fractures. We detail the management of these patients, emphasizing the role of preoperative traction.
2. Materials and methods A retrospective case series of three patients with delayed diagnoses of unstable cervical spine fractures was performed. The preoperative, postoperative, and follow-up reports were retrieved, including imaging studies. The course of disease and management of each patient were summarized, including outcome measures at presentation and follow-up.