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278
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of intramedullary tumor surgery is gross total resection. However, this must not be achieved at the cost of
neurological deterioration. Ependymoma (Fig. 19.35),
contrary to infiltrative astrocytoma, is a surgical disease and patients after gross total resection may enjoy
cure or at least a long recurrence-free survival (with
the exception of rare high-grade lesions) [24, 46, 57,
84]. On the other hand, astrocytoma (Fig. 19.36) can
seldom be cured by surgery, recurrence is always a
possibility, and thus surgical strategy must respect this
distinction [11].
Other extra- and intramedullary lesions are rare
(Fig. 19.37) and detailed discussion would be beyond
the scope of this chapter.
Fig. 19.35 Huge
ependymoma spreading from
brain stem to C6 microsurgically removed with survival
more than 10 years without
recurrence. (a) Sagittal MRI
showing the extent of tumor.
(b) Postoperative MRI
without marks of residual
tumor on sagittal view. (c, d)
Axial cuts depicting “banana
skin” like cord appearance
after tumor resection
19.2 Intradural Tumors (Extramedullary, Intramedullary)
Fig. 19.36 Inoperable
infiltrative cystic spinal cord
astrocytoma
279
Fig. 19.37 Intramedullary cavernous hemangioma unusually located behind C3 vertebra. (a) Preoperative MRI in T1 sequence. (b)
Preoperative MRI in T2 sequence. (c) MRI performed 3 months after surgery without sign of residual lesion
280
19 Tu mor s
References
1. Abdu, W.A., Provencher, M.: Primary bone and metastatic
tumors of the cervical spine. Spine (Phila Pa 1976) 23,
2767–2777 (1998)
2. Abe, E., Sato, K., Tazawa, H., et al.: Total spondylectomy
for primary tumor of the thoracolumbar spine. Spinal Cord
38, 146–152 (2000)
3. Ameli, N.O., Abbassioun, K., Saleh, H., et al.: Aneurysmal
bone cysts of the spine. Report of 17 cases. J Neurosurg 63,
685–690 (1985)
4. Amendola, B.E., Amendola, M.A., Oliver, E., et al.:
Chordoma: role of radiation therapy. Radiology 158,
839–843 (1986)
5. Atanasiu, J.P., Badatcheff, F., Pidhorz, L.: Metastatic lesions
of the cervical spine. A retrospective analysis of 20 cases.
Spine (Phila Pa 1976) 18, 1279–1284 (1993)
6. Azouz, E.M., Kozlowski, K., Marton, D., et al.: Osteoid
osteoma and osteoblastoma of the spine in children. Report
of 22 cases with brief literature review. Pediatr Radiol 16,
25–31 (1986)
7. Bacci, G., Toni, A., Avella, M., et al.: Long-term results in
144 localized Ewing’s sarcoma patients treated with combined therapy. Cancer 63, 1477–1486 (1989)
8. Bailey, C.S., Fisher, C.G., Boyd, M.C., et al.: En bloc marginal excision of a multilevel cervical chordoma. Case
report. J Neurosurg Spine 4, 409–414 (2006)
9. Barsa, P., Suchomel, P., Lukas, R., et al.: Percutaneous
CT-guided radiofrequency ablation in spinal osteoid osteoma
treatment. Acta Chir Orthop Traumatol Cech 74, 401–405
(2007)
10. Barwick, K.W., Huvos, A.G., Smith, J.: Primary osteogenic
sarcoma of the vertebral column: a clinicopathologic correlation of ten patients. Cancer 46, 595–604 (1980)
11. Benes 3rd, V., Barsa, P., Benes Jr., V., et al.: Prognostic factors in intramedullary astrocytomas: a literature review. Eur
Spine J 18, 1397–1422 (2009)
12. Bertram, C., Madert, J., Eggers, C.: Eosinophilic granuloma
of the cervical spine. Spine (Phila Pa 1976) 27, 1408–1413
(2002)
13. Blaylock, R.L., Kempe, L.G.: Chondrosarcoma of the cervical spine. Case report. J Neurosurg 44, 500–503 (1976)
14. Bohlman, H.H., Sachs, B.L., Carter, J.R., et al.: Primary
neoplasms of the cervical spine. Diagnosis and treatment of
twenty-three patients. J Bone Joint Surg Am 68, 483–494
(1986)
15. Boriani, S., Bandiera, S., Biagini, R., et al.: Chordoma of the
mobile spine: fifty years of experience. Spine (Phila Pa
1976) 31, 493–503 (2006)
16. Boriani, S., Biagini, R., De Iure, F., et al.: Primary bone
tumors of the spine: a survey of the evaluation and treatment
at the Istituto Ortopedico Rizzoli. Orthopedics 18, 993–1000
(1995)
17. Boriani, S., Biagini, R., De Iure, F., et al.: En bloc resections
of bone tumors of the thoracolumbar spine. A preliminary
report on 29 patients. Spine (Phila Pa 1976) 21, 1927–1931
(1996)
18. Boriani, S., Capanna, R., Donati, D., et al.: Osteoblastoma of
the spine. Clin Orthop Relat Res 278, 37–45 (1992)
19. Boriani, S., De Iure, F., Campanacci, L., et al.: Aneurysmal
bone cyst of the mobile spine: report on 41 cases. Spine
(Phila Pa 1976) 26, 27–35 (2001)
20. Boriani, S., Nandiera, S., Weinstein, J.N.: Primary malignant tumors of the cervical spine. In: Clark, C.R., Benzel,
E.C., Currier, B.L., et al. (eds.) The cervical spine, vol. 4, pp.
840–857. Lippincott, Philadelphia (2004)
21. Boriani, S., Weinstein, J.N., Biagini, R.: ) Primary bone
tumors of the spine. Terminology and surgical staging. Spine
(Phila Pa 1976) 22, 1036–1044 (1997)
22. Brada, M., Pijls-Johannesma, M., De Ruysscher, D.: Proton
therapy in clinical practice: current clinical evidence. J Clin
Oncol 25, 965–970 (2007)
23. Brihaye, J., Ectors, P., Lemort, M., et al.: The management
of spinal epidural metastases. Adv Tech Stand Neurosurg
16, 121–176 (1988)
24. Brotchi, J., Dewitte, O., Levivier, M., et al.: A survey of 65
tumors within the spinal cord: surgical results and the importance of preoperative magnetic resonance imaging.
Neurosurgery 29, 651–657 (1991)
25. Bruneau, M., Polivka, M., Cornelius, J.F., et al.: Progression
of an osteoid osteoma to an osteoblastoma. Case report. J
Neurosurg Spine 3, 238–241 (2005)
26. Burn, S.C., Ansorge, O., Zeller, R., et al.: Management of
osteoblastoma and osteoid osteoma of the spine in childhood. J Neurosurg Pediatr 4, 434–438 (2009)
27. Camins, M.B., Duncan, A.W., Smith, J., et al.:
Chondrosarcoma of the spine. Spine (Phila Pa 1976) 3, 202–
209 (1978)
28. Cantwell, C.P., O’Byrne, J., Eustace, S.: Radiofrequency
ablation of osteoid osteoma with cooled probes and impedance-control energy delivery. AJR Am J Roentgenol 186,
S244–S248 (2006)
29. Cantwell, C.P., Obyrne, J., Eustace, S.: Current trends in
treatment of osteoid osteoma with an emphasis on radiofrequency ablation. Eur Radiol 14, 607–617 (2004)
30. Capanna, R., Albisinni, U., Picci, P., et al.: Aneurysmal bone
cyst of the spine. J Bone Joint Surg Am 67, 527–531 (1985)
31. Caudell, J.J., Ballo, M.T., Zagars, G.K., et al.: Radiotherapy
in the management of giant cell tumor of bone. Int J Radiat
Oncol Biol Phys 57, 158–165 (2003)
32. Chabot, M., Herkowitz, H.N.: Spine tumors: Patient evaluation. In: Weisel, S. (ed.) Seminars in spine surgery, vol. 7,
pp. 260–268. Saunders, Philadelphia (1995)
33. Chakravarti, A., Spiro, I.J., Hug, E.B., et al.: Megavoltage
radiation therapy for axial and inoperable giant-cell tumor of
bone. J Bone Joint Surg Am 81, 1566–1573 (1999)
34. Chan, P., Boriani, S., Fourney, D.R., et al.: An assessment of
the reliability of the Enneking and Weinstein-BorianiBiagini classifications for staging of primary spinal tumors
by the Spine Oncology Study Group. Spine (Phila Pa 1976)
34, 384–391 (2009)
35. Choi, D., Melcher, R., Harms, J., et al.: Outcome of 132
operations in 97 patients with chordomas of the craniocervical junction and upper cervical spine. Neurosurgery 66,
59–65 (2010). discussion 65
36. Cohen, Z.R., Fourney, D.R., Marco, R.A., et al.: Total cervical spondylectomy for primary osteogenic sarcoma. Case
report and description of operative technique. J Neurosurg
97, 386–392 (2002)
References
281
37. Constans, J.P., de Divitiis, E., Donzelli, R., et al.: Spinal
metastases with neurological manifestations. Review of 600
cases. J Neurosurg 59, 111–118 (1983)
38. Dahlin, D.C., Cupps, R.E., Johnson Jr., E.W.: Giant-cell
tumor: a study of 195 cases. Cancer 25, 1061–1070 (1970)
39. Dahlin, D.C., McLeod, R.A.: Aneurysmal bone cyst and
other nonneoplastic conditions. Skeletal Radiol 8, 243–250
(1982)
40. de Kleuver, M., van der Heul, R.O., Veraart, B.E.:
Aneurysmal bone cyst of the spine: 31 cases and the importance of the surgical approach. J Pediatr Orthop B 7, 286–
292 (1998)
41. Di Lorenzo, N., Delfini, R., Ciappetta, P., et al.: Primary
tumors of the cervical spine: surgical experience with 38
cases. Surg Neurol 38, 12–18 (1992)
42. Dominguez, C.J., Martin-Ferrer, S., Rimbau, J., et al.: Upper
cervical chondrosarcoma. Neurocirugia (Astur) 16, 261–265
(2005). discussion 265
43. Dreghorn, C.R., Newman, R.J., Hardy, G.J., et al.: Primary
tumors of the axial skeleton. Experience of the Leeds
Regional Bone Tumor Registry. Spine (Phila Pa 1976) 15,
137–140 (1990)
44. Enneking, W.F.: A system of staging musculoskeletal neoplasms. Clin Orthop Relat Res 204, 9–24 (1986)
45. Enneking, W.F., Spanier, S.S., Goodman, M.A.: A system
for the surgical staging of musculoskeletal sarcoma. Clin
Orthop Relat Res 153, 106–120 (1980)
46. Epstein, F.J., Farmer, J.P., Freed, D.: Adult intramedullary
spinal cord ependymomas: the result of surgery in 38
patients. J Neurosurg 79, 204–209 (1993)
47. Feigenberg, S.J., Marcus Jr., R.B., Zlotecki, R.A., et al.:
Megavoltage radiotherapy for aneurysmal bone cysts. Int J
Radiat Oncol Biol Phys 49, 1243–1247 (2001)
48. Fidler, M.W.: Surgical treatment of giant cell tumours of the
thoracic and lumbar spine: report of nine patients. Eur Spine
J 10, 69–77 (2001)
49. Fisher, C.G., Keynan, O., Boyd, M.C., et al.: The surgical
management of primary tumors of the spine: initial results of
an ongoing prospective cohort study. Spine (Phila Pa 1976)
30, 1899–1908 (2005)
50. Fourney, D.R., Rhines, L.D., Hentschel, S.J., et al.: En bloc
resection of primary sacral tumors: classification of surgical
approaches and outcome. J Neurosurg Spine 3, 111–122
(2005)
51. Frank, E., Chamberland, D., Ragel, B.: A proposed technique for intraoperative measurement of cervical spine stiffness. Neurosurgery 39, 147–150 (1996)
52. Fujita, T., Kawahara, N., Matsumoto, T., et al.: Chordoma in
the cervical spine managed with en bloc excision. Spine
(Phila Pa 1976) 24, 1848–1851 (1999)
53. Gabrielsen, T.O., Seeger, J.F.: Vertebral angiography in the
diagnosis of intraspinal masses in upper cervical region.
Neuroradiology 5, 7–12 (1973)
54. Gebes, S., Winking, M.: Plasmacytoma involving the atlas
and axis. Neurochirurgia (Stuttg) 32, 187–188 (1989)
55. George, B., Lot, G., Boissonnet, H.: Meningioma of the
foramen magnum: a series of 40 cases. Surg Neurol 47, 371–
379 (1997)
56. George, B., Lot, G., Velut, S., et al.: French language Society
of Neurosurgery. 44th Annual Congress. Brussels, 8-12 June
1993. Tumors of the foramen magnum. Neurochirurgie
39(1), 1–89 (1993)
57. Guidetti, B., Mercuri, S., Vagnozzi, R.: Long-term results of
the surgical treatment of 129 intramedullary spinal gliomas.
J Neurosurg 54, 323–330 (1981)
58. Harrington, K.D.: The use of methylmethacrylate for vertebral-body replacement and anterior stabilization of pathological fracture-dislocations of the spine due to metastatic
malignant disease. J Bone Joint Surg Am 63, 36–46 (1981)
59. Harrop, J.S., Schmidt, M.H., Boriani, S., et al.: Aggressive
“benign” primary spine neoplasms: osteoblastoma, aneurysmal bone cyst, and giant cell tumor. Spine (Phila Pa 1976)
34, 39–47 (2009)
60. Hastings, D.E., Macnab, I., Lawson, V.: Neoplasms of the
atlas and axis. Can J Surg 11, 290–296 (1968)
61. Hay, M.C., Paterson, D., Taylor, T.K.: Aneurysmal bone
cysts of the spine. J Bone Joint Surg Br 60, 406–411 (1978)
62. Hosalkar, H.S., Jones, K.J., King, J.J., et al.: Serial arterial
embolization for large sacral giant-cell tumors: mid- to longterm results. Spine (Phila Pa 1976) 32, 1107–1115 (2007)
63. Hug, E.B., Fitzek, M.M., Liebsch, N.J., et al.: Locally challenging osteo- and chondrogenic tumors of the axial skeleton: results of combined proton and photon radiation therapy
using three-dimensional treatment planning. Int J Radiat
Oncol Biol Phys 31, 467–476 (1995)
64. Hyun, S.J., Rhim, S.C., Riew, K.D.: A combined posterior,
lateral, and anterior approach to ventrolaterally situated
chordoma of the upper cervical spine. Surg Neurol 72,
409–413 (2009). discussion 413
65. Jackson, R.P.: Recurrent osteoblastoma: a review. Clin
Orthop Relat Res 131, 229–233 (1978)
66. Jackson, R.P., Reckling, F.W., Mants, F.A.: Osteoid osteoma
and osteoblastoma. Similar histologic lesions with different
natural histories. Clin Orthop Relat Res 128, 303–313
(1977)
67. Jaffe, H., Lichtenstein, I.: Solitary unicameral bone cyst with
emphasis on the roentgen picture, the pathologic appearance
and the pathogenesis. Arch Surg 44, 1004–1025 (1942)
68. Kaibara, T., Hurlbert, R.J., Sutherland, G.R.: Transoral
resection of axial lesions augmented by intraoperative magnetic resonance imaging. Report of three cases. J Neurosurg
95, 239–242 (2001)
69. Kaiser, T.E., Pritchard, D.J., Unni, K.K.: Clinicopathologic
study of sacrococcygeal chordoma. Cancer 53, 2574–2578
(1984)
70. Khan, D.C., Malhotra, S., Stevens, R.E., et al.: Radiotherapy
for the treatment of giant cell tumor of the spine: a report of
six cases and review of the literature. Cancer Invest 17,
110–113 (1999)
71. Kingdom, T.T., Nockels, R.P., Kaplan, M.J.: Transoraltranspharyngeal approach to the craniocervical junction.
Otolaryngol Head Neck Surg 113, 393–400 (1995)
72. Konya, D., Ozgen, S., Gercek, A., et al.: Transmandibular
approach for upper cervical pathologies: report of 2 cases
and review of the literature. Turk Neurosurg 18, 271–275
(2008)
73. Kostuik, J., Weinstein, J.N.: Differential diagnosis and surgical treatment of metastatic spine tumors. In: Frymoyer, J.
(ed.) The adult spine: principles and practice, pp. 861–888.
Raven, New York (1991)
282
19 Tu mor s
74. Laus, M., Albisinni, U., Alfonso, C., et al.: Osteoid osteoma
of the cervical spine: surgical treatment or percutaneous
radiofrequency coagulation? Eur Spine J 16, 2078–2082
(2007)
75. Lee, F.Y., Mankin, H.J., Fondren, G., et al.: Chondrosarcoma
of bone: an assessment of outcome. J Bone Joint Surg Am
81, 326–338 (1999)
76. Levine, A.M., Boriani, S.: Benign tumors of cervical spine.
In: Clark, C.R., Benzel, E.C., Currier, B.L., et al. (eds.) The
cervical spine, vol. 4, pp. 816–839. Lippincott, Philadelphia
(2005)
77. Levine, A.M., Boriani, S., Donati, D., et al.: Benign tumors
of the cervical spine. Spine (Phila Pa 1976) 17, 399–406
(1992)
78. Lichtenstein, L., Jeffe, H.L.: Eosinophilic granuloma of
bone: with report of a case. Am J Pathol 16(595–604), 593
(1940)
79. Lichtenstein, L., Sawyer, W.R.: Benign osteoblastoma.
Further observations and report of twenty additional cases.
J Bone Joint Surg Am 46, 755–765 (1964)
80. Lis, E., Bilsky, M.H., Pisinski, L., et al.: Percutaneous
CT-guided biopsy of osseous lesion of the spine in patients
with known or suspected malignancy. AJNR Am J Neuroradiol
25, 1583–1588 (2004)
81. Lucas, D.R., Unni, K.K., McLeod, R.A., et al.: Osteoblastoma:
clinicopathologic study of 306 cases. Hum Pathol 25, 117–
134 (1994)
82. Marmor, E., Rhines, L.D., Weinberg, J.S., et al.: Total en
bloc lumbar spondylectomy. Case report. J Neurosurg 95,
264–269 (2001)
83. Marsh, B.W., Bonfiglio, M., Brady, L.P., et al.: Benign
osteoblastoma: range of manifestations. J Bone Joint Surg
Am 57, 1–9 (1975)
84. McCormick, P.C., Torres, R., Post, K.D., et al.: Intramedullary
ependymoma of the spinal cord. J Neurosurg 72, 523–532
(1990)
85. McLain, R.F., Weinstein, J.N.: Solitary plasmacytomas of
the spine: a review of 84 cases. J Spinal Disord 2, 69–74
(1989)
86. Munzenrider, J.E., Liebsch, N.J.: Proton therapy for tumors
of the skull base. Strahlenther Onkol 175(Suppl 2), 57–63
(1999)
87. Murphey, M.D., Andrews, C.L., Flemming, D.J., et al.: From
the archives of the AFIP. Primary tumors of the spine: radiologic pathologic correlation. Radiographics 16, 1131–1158
(1996)
88. Murphy, W.A., Strecker, E.B., Schoenecker, P.L.:
Transcatheter embolisation therapy of an ischial aneurysmal
bone cyst. J Bone Joint Surg Br 64, 166–168 (1982)
89. Nagashima, H., Nishi, T., Yamane, K., et al.: Case report:
osteoid osteoma of the C2 pedicle: surgical technique using
a navigation system. Clin Orthop Relat Res 468, 283–288
(2010)
90. Nesbit Jr., M.E., Gehan, E.A., Burgert Jr., E.O., et al.:
Multimodal therapy for the management of primary, nonmetastatic Ewing’s sarcoma of bone: a long-term follow-up
of the First Intergroup study. J Clin Oncol 8, 1664–1674
(1990)
91. Neumann, D., Dorn, U.: Osteoid osteoma of the dens axis.
Eur Spine J 16(Suppl 3), 271–274 (2007)
92. Noel, G., Feuvret, L., Calugaru, V., et al.: Chordomas of the
base of the skull and upper cervical spine. One hundred
patients irradiated by a 3D conformal technique combining
photon and proton beams. Acta Oncol 44, 700–708 (2005)
93. Nyholm, K.: Eosinophilic xanthomatous granulomatosis
and Letterer-Siwe’s disease. Acta Pathol Microbiol Scand
Suppl 216, 211+ (1971)
94. Ono, K., Ebara, S., Fuji, T., et al.: Myelopathy hand. New
clinical signs of cervical cord damage. J Bone Joint Surg Br
69, 215–219 (1987)
95. Otani, S., Ehrlich, J.C.: Solitary granuloma of bone:
Simulating primary neoplasm. Am J Pathol 16(479–490),
477 (1940)
96. Peraud, A., Drake, J.M., Armstrong, D., et al.: Fatal ethibloc
embolization of vertebrobasilar system following percutaneous injection into aneurysmal bone cyst of the second cervical vertebra. AJNR Am J Neuroradiol 25, 1116–1120
(2004)
97. Phillips, E., Levine, A.M.: Metastatic lesions of the upper
cervical spine. Spine (Phila Pa 1976) 14, 1071–1077 (1989)
98. Pierot, L., Boulin, A.: Percutaneous biopsy of the thoracic
and lumbar spine: transpedicular approach under fluoroscopic guidance. AJNR Am J Neuroradiol 20, 23–25
(1999)
99. Prasad, V.S., Raju, B.S., Sundaram, C.: Plasmacytoma of
dens as a cause of atlanto-axial instability. Spinal Cord 36,
661–663 (1998)
100. Rao, S., Badani, K., Schildhauer, T., et al.: Metastatic
malignancy of the cervical spine. A nonoperative history.
Spine (Phila Pa 1976) 17, 407–412 (1992)
101. Raskas, D.S., Graziano, G.P., Herzenberg, J.E., et al.:
Osteoid osteoma and osteoblastoma of the spine. J Spinal
Disord 5, 204–211 (1992)
102. Raycroft, J.F., Hockman, R.P., Southwick, W.O.: Metastatic
tumors involving the cervical vertebrae: surgical palliation.
J Bone Joint Surg Am 60, 763–768 (1978)
103. Rhines, L.D., Fourney, D.R., Siadati, A , et al.: En bloc resec-
tion of multilevel cervical chordoma with C-2 involvement.
Case report and description of operative technique. J Neurosurg
Spine 2, 199–205 (2005)
104. Rich, T.A., Schiller, A., Suit, H.D., et al.: Clinical and
pathologic review of 48 cases of chordoma. Cancer 56,
182–187 (1985)
105. Robins, S.L.: Pathology, 3rd edn, pp. 1649–1650. Saunders,
Philadelphia (1967)
106. Rose, E.F., Fekete, A.: Odontoid osteochondroma causing
sudden death. Report of a case and review of the literature.
Am J Clin Pathol 42, 606–609 (1964)
107. Samii, M., Klekamp, J., Carvalho, G.: Surgical results for
meningiomas of the craniocervical junction. Neurosurgery
39, 1086–1094 (1996). discussion 1094–1085
108. Sar, C., Eralp, L.: Transoral resection and reconstruction
for primary osteogenic sarcoma of the second cervical vertebra. Spine (Phila Pa 1976) 26, 1936–1941 (2001)
109. Schellinger, K.A., Propp, J.M., Villano, J.L., et al.:
Descriptive epidemiology of primary spinal cord tumors. J
Neurooncol 87, 173–179 (2008)
110. Sciubba, D.M., Okuno, S.H., Dekutoski, M.B., et al.: Ewing
and osteogenic sarcoma: evidence for multidisciplinary
management. Spine (Phila Pa 1976) 34, 58–68 (2009)
References
283
111. Shives, T.C., Dahlin, D.C., Sim, F.H., et al.: Osteosarcoma
of the spine. J Bone Joint Surg Am 68, 660–668 (1986)
112. Shives, T.C., McLeod, R.A., Unni, K.K., et al.:
Chondrosarcoma of the spine. J Bone Joint Surg Am 71,
1158–1165 (1989)
113. Simmons, E.D., Zheng, Y.: Vertebral tumors: surgical versus nonsurgical treatment. Clin Orthop Relat Res 443,
233–247 (2006)
114. Suchomel, P., Buchvald, P., Barsa, P., et al.: Single-stage
total C-2 intralesional spondylectomy for chordoma with
three-column reconstruction. Technical note. J Neurosurg
Spine 6, 611–618 (2007)
115. Suit, H.D., Goitein, M., Munzenrider, J., et al.: Definitive
radiation therapy for chordoma and chondrosarcoma of base
of skull and cervical spine. J Neurosurg 56, 377–385 (1982)
116. Sundaresan, N., Boriani, S., Okuno, S.: State of the art
management in spine oncology: a worldwide perspective
on its evolution, current state, and future. Spine (Phila Pa
1976) 34, 7–20 (2009)
117. Sundaresan, N., Galicich, J.H., Chu, F.C., et al.: Spinal
chordomas. J Neurosurg 50, 312–319 (1979)
118. Suttner, N.J., Chandy, K.J., Kellerman, A.J.: Osteoid osteomas of the body of the cervical spine. Case report and
review of the literature. Br J Neurosurg 16, 69–71 (2002)
119. Talac, R., Yaszemski, M.J., Currier, B.L., et al.: Relationship
between surgical margins and local recurrence in sarcomas
of the spine. Clin Orthop Relat Res 397, 127–132 (2002)
120. Thiry, S., Steenebruggen, A., Hotermans, J.M., et al.:
Complete destruction of the body of the axis by a myeloplasmacytoma. Resection by the transoral route and reconstruction of the vertebral body by a bone graft. Presentation
of a case 2 years after intervention. Neurochirurgie 14,
799–808 (1968)
121. Thomson, A.D., Turner-Warwick, R.T.: Skeletal sarcomata
and giant-cell tumour. J Bone Joint Surg Br 37-, 266–303
(1955)
122. Tokuhashi, Y., Matsuzaki, H., Oda, H., et al.: A revised scoring system for preoperative evaluation of metastatic spine
tumor prognosis. Spine (Phila Pa 1976) 30, 2186–2191
(2005)
123. Tokuhashi, Y., Matsuzaki, H., Toriyama, S., et al.: Scoring
system for the preoperative evaluation of metastatic spine
tumor prognosis. Spine (Phila Pa 1976) 15, 1110–1113
(1990)
124. Tomita, K., Kawahara, N., Baba, H., et al.: Total en bloc
spondylectomy. A new surgical technique for primary
malignant vertebral tumors. Spine (Phila Pa 1976) 22,
324–333 (1997)
125. Tomita, K., Kawahara, N., Kobayashi, T., et al.: Surgical
strategy for spinal metastases. Spine (Phila Pa 1976) 26,
298–306 (2001)
126. Torma, T.: Malignant tumours of the spine and the spinal
extradural space; a study based on 250 histologically verified cases. Acta Chir Scand Suppl 225, 1–176 (1957)
127. Verbiest, H.: Giant-cell tumours and aneurysmal bone cysts
of the spine. With special reference to the problems related
to the removal of a vertebral body. J Bone Joint Surg Br 47,
699–713 (1965)
128. Vergel De Dios, A.M., Bond, J.R., Shives, T.C., et al.:
Aneurysmal bone cyst. A clinicopathologic study of 238
cases. Cancer 69, 2921–2931 (1992)
129. Weber, K.L.: Current concepts in the treatment of Ewing’s
sarcoma. Expert Rev Anticancer Ther 2, 687–694 (2002)
130. Weiner, H.L., Freed, D., Woo, H., et al.: Intra-axial tumors
of the cervicomedullary junction: surgical results and longterm outcome. Pediatr Neurosurg 27, 12–18 (1997)
131. Weinstein, J.M.: Spine neoplasms. In: Weinstein, J.M. (ed.)
The pediatric spine, pp. 887–916. Raven, New York
(1994)
132. Wilner, D.: Radiology of bone tumors and allied disorders,
vol. 2. Saunders, Philadelphia (1982)
133. Yamazaki, T., McLoughlin, G.S., Patel, S., et al.: Feasibility
and safety of en bloc resection for primary spine tumors: a
systematic review by the Spine Oncology Study Group.
Spine (Phila Pa 1976) 34, 31–38 (2009)
134. Zileli, M., Cagli, S., Basdemir, G., et al.: Osteoid osteomas
and osteoblastomas of the spine. Neurosurg Focus 15, E5
(2003)
Congenital and Developmental Abnormalities
P. Suchomel and O. Choutka
20
Developmental anomalies of CVJ were first discovered by anatomists and pathologists. Ackerman is
credited for being the first to recognized the basilar
impression in 1790 as cited by McGregor [26].
According to Gladstone and Erickson, the case of
“occipital vertebrae” was described by Meckel in 1815
[13]. Rokitanski, a Czech pathologist working in
Vienna, was probably the first one to described the
basilar invagination as a developmental deformity in
1844, as cited by Ebenius [9]. Boogaard measured the
angle of the clivus related to foramen magnum (FM)
diameter on cadavers in 1865 [26]. In 1880, Grawitz
contributed with anatomic description of six skulls
with basilar impression [5]. Homén discovered, in
1901, the narrowing of FM by the odontoid and
cephalic bulge of clivus as a possible cause of death,
documented by a drawn picture [18]. The first diagnosis of basilar impression in a living patient documented
by a radiograph was reported by Schüler in 1905 [31].
Basilar invagination is the most common abnormality (38–74%) of CVJ followed by atlas assimilation,
atlantoaxial (AA) dislocation, Klippel-Feil syndrome,
hindbrain herniation, and hydromyelia [8, 10, 14, 27].
In the majority of symptomatic and operated cases, a
combination of various abnormalities is observed,
however.
P. Suchomel
Department of Neurosurgery,
Neurocenter, Regional Hospital Liberec,
Husova St. 10, 46063 Liberec, Czech Republic
O. Choutka
University of Cincinnati, Medical Center,
Department of Neurosurgery,
Albert Sabin Way 231,
Cincinnati, OH 45267-0515, USA
20.1 Etiology
A detailed description of embryological failures of
CVJ is beyond the scope of this chapter. A significant number of CVJ deformities occur due to failure
in development and correct connection of CVJ structures before the birth. As examples of these congeni-
tal anomalies, proatlas segmentation failure, basilar
invagination, atlas assimilation, condylar hypoplasia,
odontoid aplasia, hemivertebrae, and segmentation
failures may be mentioned [27].
Anomalies developing during the period of unfinished bone growth postnatally are referred to as
developmental, including os odontoideum, basilar
impression, and syndromal abnormalities [27]. Both
congenital and developmental anomalies are considered primary CVJ abnormalities [2, 26].
Failure in formation of UCS and CVJ during childhood can be caused by a variety of reasons including
genetic or secondary factors. The distorted CVJ development can be a part of rare syndromes like osteogenesis
imperfecta, skeletal dysplasia, Goldenhar’s syndrome,
Conradi syndrome, Down syndrome, and spondyloepiphyseal dysplasia, with possible combination of pre- and
postnatal developmental failures and later, decompensation caused by axial load [27].
The abnormalities described above, often, present
in various combinations with neural anomalies. Chiari
malformation and syringomyelia are frequently
reported as a part of complex congenital or developmental CVJ deformity.
Some deformities of CVJ can arise secondarily
as a result of disease affecting the bone: hyperparathyroidism, osteomalacia, osteoporosis, Paget’s disease, rheumatoid arthritis (RA), trauma, and tumors
[2, 30].
P. Suchomel and O. Choutka, Reconstruction of Upper Cervical Spine and Craniovertebral Junction,
DOI: 10.1007/978-3-642-13158-5_20, © Springer-Verlag Berlin Heidelberg 2011
285
286
20 Congenital and Developmental Abnormalities
20.2 Clinical Appearance
As we deal only with adult patients in our department,
failures in CVJ development presenting clinically in
children will not be discussed in this chapter.
There is a great variability in clinical presentation
of developmental disorders of CVJ. Menezes stated
that the most interesting feature of clinical presentation is its diversity [27]. Besides pain, most other
symptoms are related to compression of neural structures, e.g., spinal cord, brainstem, cerebellum, and
lower cranial nerves. Some clinical signs can be related
to vascular compromise.
Motor and/or sensory abnormalities and sphincter
dysfunction related to myelopathy; nystagmus, ataxia,
dysmetria caused by brainstem and cerebellar compression; dysphagia, speech irregularity, and tongue
atrophy due to inferior cranial nerve compromise may
be present. The clinical symptoms usually progress
slowly, although acute deterioration is possible, particularly if exacerbated by trauma [14]. Starting with
mild symptoms like vertigo, torticollis, and/or ataxia,
the patients may finally suffer from serious quadrusymptomatology including respiratory disturbances.
Risk of sudden death cannot be excluded [29].
Pain, common to other diseases of UCS, is present
nearly in all cases, but is usually less dominant in adult
patients as they have been living with their deformity
for a long time. The patients with combined CVJ
anomalies often have short neck, limited neck movement, and low hairline (typical of Klippel-Feil syndrome). Other developmental abnormalities such as
cleft palate, face asymmetry, scoliosis, urinary tract
abnormities etc. may also be present.
20.3 Radiology
Since Schüler’s report in 1905 [31] describing an
X-ray of a living patient with basilar invagination, it
was necessary to develop a standardized algorithm to
diagnose the vertical migration of UCS and other CVJ
anomalies.
Chamberlain [5], in his classical work, described a
line drawn between hard palate and posterior FM margin (opisthion) on lateral X-rays and called it “the
basal line.” He also suggested modifying the occipital
X-rays to so called “fronto-vertex” projection in order
to see directly the FM and its deformity on radiograms.
Chamberlain’s idea was practised in his four patients
and confirmed by autopsy in two of them who later
died, one following posterior surgical decompression.
According to his original paper, the atlas and the whole
axis should be located below the basal line in healthy
individuals. Analyzing 100 plain lateral radiographsfrom normal adults, Saunders later added the numerical value to Chamberlain’s statement [30]. He reported
that the tip of the odontoid process should be located
below the Chamberlain’s basal line on lateral projections in mean distance 1.0 mm (SD = 3.6 mm). This
value is still valid even for MRI and CT craniometry
with the only difference in larger range of acceptable
variability (3–7 mm) [2, 23, 34].
McGregor recognized that it is not always easy or
even possible to correctly establish the posterior FM lip
(opisthion) on lateral films and suggested to use the
lowermost occipital skull point as another end of the
line drawn from the hard palate [26]. He used 204 lateral films from mixed race South African population for
his evaluation and after statistical analysis he stated that
the odontoid tip should not lie more than 4.5 mm above
the base line. He found 7 mm as the maximal acceptable
normal value. Not frequently cited but a very important
part of his work, however, is the measurement of the
basal angle formed by intersection of clival line and
anterior skull base line. He measured mean angle 134°
(range 121°–148°), which was comparable to the values
obtained by Brailsford, earlier [3].
McRae studied the CVJ anomalies with tomography (“laminagraphy”) and described 25 cases of atlas
assimilation. He defined the line connecting the basion
with opisthion as an important parameter when locating the FM.
Many more parameters describing the normal and
pathological radiological anatomy of CVJ derived
from plain radiograms or tomograms were later developed. Only some of them are still used today in the era
of CT and MRI, when used, however, their significance
is similar [34].
Commonly used Wackenheim’s clivus line drawn
along the posterior clivus should cross over the odontoid tip [37]. The angle formed by Wackenheim’s clivus
line and line along posterior C2 wall called “clivuscanal angle” should not be less than 150° in flexion and
is reaching up to 180° in extension. The abnormal flattening of the skull base (platybasia) is considered to be
present when the basal angle (as described previously)
exceeds 140°. The atlantooccipital (AO) joint angle in
20.7 Basioccipital Hypoplasia
coronal plane ranges between 124°–127°. The condylar
hypoplasia may be expected in cases of more obtuse
angle [34]. Also, other parameters originally used for
atlanto-occipital dislocation (AOD) can be applied in
CVJ craniometrics. As an example, the basion-dens
interval (BDI) of Harris, which normally measures
7.4 mm (SD = 4.3 mm) can be distorted [17].
Cervicomedullary angle (CMA) is an angle between
anterior borders of medulla oblongata and spinal cord
at the level of FM as measured on MRI [4, 28]. This
angle should be between 135°and 175° in normal subjects and measured less than 135° is considered as predictive of myelopathic progression [4, 24]. However,
Abumi et al. [1] obtained values from 50 healthy
Japanese individuals and established the angle of 163°
as a normal value (range 154°–179°).
Nowadays, plain radiography serves only for pri-
mary screening of CVJ and UCS anomalies. MRI and
CT are the mainstay methods, showing exactly the pathological anatomy in case of CVJ anomaly. Nevertheless,
the more sophisticated modalities of contemporary
imaging techniques are often requested by a surgeon in
order to recognize the reducibility or instability of the
deformity. Dynamic MRI directly shows the location
and extent of neural compression [27] whereas dynamic
CT exactly depicts the instability as well as the reducibility of bone dislocation during traction and/or flexion
and extension [16]. MR- or CT-angiography contribute
to identifying the frequently present abnormality in
vascular supply [38]. If surgery is indicated, the 3D-CT
modeling is useful for planning of trajectory of fixation
screws and help to avoid an unintended surgical injury
to neural or vascular structures.
20.4 Anomalies of the Occiput
Abnormal development of occipital bone is often associated with decreased height of skull base and basilar
invagination.
287
Fig. 20.1 Condylus tertius, partial posterior atlas assimilation,
pseudoarthrosis of anterior C1 arch
(Fig. 20.1), although multiple ossicles can be present.
It can create a joint or pseudojoint with odontoid or
anterior C1 arch. Currently, CT or MRI techniques will
display this abnormity, often presenting together with
os odontoideum. If large enough, the condylus tertius
can limit flexion movement in the AO joint.
20.6 Condylar Hypoplasia
Insufficient development of one or both condyles, logically, decreases the odontoid distance from FM and
basilar invagination is therefore often present, simultaneously. Also, condylar dysplasia is often combined
with atlas assimilation. In the past, this deformity was
diagnosed due to distortion of the AO joint angle on
AP films. Today, CT scan in frontal plane will show
exactly the shape and the size of condyles (Fig. 20.2).
Consequently, the movement in AO joint is limited,
resulting in restricted flexion and extension of the
head.
20.5 Condylus Tertius
This anomaly is a result of failed integration of proatlas
(the fourth occipital sclerotome) to occipital bone. The
anteriorly located remnants may form the third condyle
composed of one piece of bone in majority of cases
20.7 Basioccipital Hypoplasia
Dysplastic changes in basiocciput can be mild or
severe, resulting in shortening of the clivus. Because
of frequent odontoid invagination and/or synostosis, it
is very difficult to reveal this anomaly from plain
radiographs. MRI and CT (Fig. 20.3) will show it