- Email: [email protected]
Exostosis as a cause of spinal cord compression
Surg Neurol 1986;26:581-4
581
Exostosis as a Cause of Spinal Cord Compression A l b e r t o G o t t l i e b , M . D . , P a o l o S e v e r i , M . D . , A n t o n i o R u e l l e , M . D . , a n d G i o v a n n i Lasio, M . D . Division of Neurosurgery, Ospedali Galliera, Genoa, Italy
Gottlieb A, Severi P, Ruelle A, Lasio G. Exostosis as a cause of spinal cord compression. Surg Neurol 1986;26:581-4.
A patient harboring multiple hereditary exostoses with a long-lasting tetraparesis owing to intraspinal osteochondroma is described. The results of surgical treatment were poor in contrast to the results achieved by other previous authors performing decompression of the spinal cord. The related literature is briefly reviewed and conclusions are drawn in favor of computed tomographic examination of the spine of patients with vertebral complaints. KEYWORDS: Osteochondroma; Spinal cord compression; Multiple hereditary exostoses
Multiple hereditary exostoses (osteochondrosis multipla) is one of the most frequent dysplasias of bone, mainly affecting the diaphysal parts (i.e., the part of long bones which is preformed as cartilage). Vertebral complaints are present in a small percentage of cases (7% according to Stocks and Barrington [25]). Most vertebral exostoses grow from the external parts of the laminae. In some rare cases exostoses grow into the spinal canal, compressing the spinal roots and cord. We have found in the related literature 33 cases of cord compression caused by this condition, 15 of which were in the cervical region [3,4,6,10,14,15,17-20,23, 24,27,29]. Case R e p o r t A 31-year-old man was admitted to our department for a slowly progressing tetraparesis with sensory disturbances in both legs of 16 years' duration. The patient's father, paternal grandmother, and uncle had had multiple hereditary exostoses (Figure 1). H e himself had been operated on for bone protusions in the ribs, hips, and knees during childhood. At the age of 14 years, the patient, while diving into shallow water, sustained a se-
Address reprint requests to: Dr. Paolo Severi, Division of Neurosurgery, Ospedali Galliera, via A. Volta 8, 16128 Genova, Italy.
© 1986 by ElsevierSciencePublishingCo., Inc.
vere head injury accompanied by a sudden tetraparesis, which cleared in a few minutes. No further examination was performed at that time. The neurological examination disclosed spastic tetraparesis more severe on the left side: he could stand or walk only with the help of crutches and his gait was chronically spastic. A dense superficial hypoesthesia below C-4 was present, more evident on the right side of the body. The patient did not complain of bladder disturbances or sexual dysfunction. Hyperreflexia was present in the upper and lower limbs; more so on the left side. Bilateral pathological plantar responses were also present. Plain X-rays of the skeleton revealed several unevenly distributed exostoses; the skull was unaffected. The films of the cervical spine showed hypertrophy of the lateral masses, which were probably fused. The spinal tap showed normal protein content of the fluid. There was no evidence of blockage. Myelography showed a round filling defect slightly left of the midline; at level of C-4 to C-5 the cord was pushed to the right (Figure 2). Computed tomography (CT) scanning showed a bony protusion into the spinal canal, arising from the lamina (Figure 3A). During operation an exostosis was found growing from the posterior arch of C-4, slightly left of the midline and infolding the dura mater, whose outer layer was adherent to the tumor (Figure 3B). Complete piecemeal removal of the tumor was performed. The histologic examination showed an outer layer of cartilage with a central ossifying core. The postoperative course was uneventful; when discharged, the patient's neurological examination gave unchanged results and I year later, despite continuing physiotherapy, he had attained only a slight improvement of gait.
Discussion In a thorough review of the relevant literature we found only 33 previous cases of spinal cord compression owing to osteochondromas (Table 1). In 25 cases the intraspinal tumor was accompanied by general bone complaints (multiple hereditary exostoses), whereas in nine patients [ 10,11,13,14,16,20,27,28], the only detectable 0090-3019/86/$3,50
Figure 1. Patient's family," members with multiple hereditary exostoses are shaded.
C) female
[ ] male
Figure 2. Myelography showing a round filling defect.
Spinal Exostosis
Surg Neurol 1986;26:581-4
583
1. Distribution of Exostoses along the Spine as Found in the Literature
Table
Level
A
B
Figure 3. (A) Computed tomography scan and (B) surgeon's impression of the anatomical situation of the fourth cervical vertebra.
osteochondroma was spinal, and no family history of multiple hereditary exostoses was present. In these patients diagnosis was obviously more difficult. Twenty-six out of the 34 patients were in their twenties or thirties, as is to be expected with an inherited disease (Figure 4). More men than women were affected, thus confirming that autosomic gene trasmission postulated for multiple hereditary exostoses has a greater recurrence in males, who account for as many as 73.5% of cases [7,12,18,19]. Osteochondromas did not appear to be evenly distributed along the spine: they often affected the cervical and upper thoracic regions; 31 out of 34 cases were located between C-1 and D-5 (12 vertebrae) and only 3 between D-5 and the sacrum. We also tried to learn from the literature the vertebral sites of origin of these tumors, but in cases of large lesions the authors' descriptions did not provide a precise location. Undoubtedly, however, those parts of the vertebra that derived from the neural arch were most affected, i.e., the laminae, pedicles, articular processes, and lateral posterior
No. of cases
Authors
C-1 C-2
2 7
C-4 C-5
1 3
C-6
2
C-7
1
D-1
2
D-2 D-3 D-4
1 2 5
D-5
5
D-10
1
Sam and Howard [21], Signargout et al [22] Palmer and Blum [19] Goncharova et al [9], Larson et al [12] Adam and Morin [1], Buur and Mcrch [2], Loftus et al [13], Karaian et al [11], Twersky et al [28] Goldenberg et al [8], Natarajan et al [16], Signargout et al [22], Thierry et al [26], Twersky et al [28] Decker and Wei [5]
L-3 L-4
1 1
Gokay and Bucy [7] Twersky et al [28]
Chiurco [4], Julien et al [10] Carmel [3], Ferrari et al [6], MacGee [14], Madigan et al [15], Ochsner and Rothstein [17], Rose and Fekete [20], Vinstein and Franken [29] Ours O'Connor and Roberts [18], Singh et al [23], Slepian and Hamby [24] Palmer and Blum [19], Thomas and Andress [27] Palmer and Blum [19]
parts of the vertebral body. O f these, 12 rose from the laminae, 2 from the pedicle-articular complex, 4 from the costovertebral junction and 7 from the vertebral body. The duration of symptoms varied widely, from no Figure 4. Distribution according to sex and decades of life of the exostoses. N. of c88e8
12
6
~
~
10
20
11
23
Female
Male
4
~
~
~
60 Age
584
Surg N e u r o l 1986;26:581-4
previous complaint, to sudden death from bulbar compression [20], to 32 years of progressive worsening [21]. The average duration of the complaint was 10 months. Plain X-rays of the spine are usually diagnostic or highly indicative in this condition. In only one patient out of those found in the relevant literature [26] were the plain roentgenograms normal, as was the case with our patient. Computed tomography scanning, when performed, was diagnostic in all cases. Surgical treatment in most cases resulted in cure. Our patient, on the contrary, achieved only slight improvement after operation, because of the long lasting and severe compression that his spinal cord had sustained.
Conclusion The increasing number of cases of cord compression owing to exostoses published in recent years since the CT scan has been available probably means that cord compression in the course of multiple hereditary exostoses is not as rare as the number of such cases, quoted in the literature, would imply. The ease of performing a spinal CT scan and the positive results of early operation suggest that all patients harboring spinal exostoses should be investigated by this means in order to detect possible endorachidial tumor growth even if there is no sign of cord compression at the affected vertebral level.
The authors thank Professor G. C. Andrioli for his assistance in the preparation of this paper.
References 1. Adam H, Morin P. Compression m~dullaire par exostose au cours d'une maladie exostosante. Presse Med 1966;74:660. 2. Buur T, M~rch MM. Hereditary multiple exostoses with spinal cord compression. J Neurol Neurosurg Psychiatry 1983;46:96-8. 3. Carmel PW, Cramer FJ. Cervical cord compression due to exostosis in a patient with hereditary multiple exostoses. Case report. J Neurosurg 1968;28:500-3. 4. Chiurco AA. Multiple exostoses of bone with fatal spinal cord compression: report of a case and brief review of the literature. Neurology 1970;20:275-8. 5. Decker RE, Wei WC. Thoracic cord compression from multiple hereditary exostoses associated with cerebellar astrocytoma. J Neurosurg 1969;30:310-2. 6. Ferrari G, Taddei L, Vivenza C, Rossi G. Paraparesis in hereditary multiple exostoses: case report. Neurology 1979;29:973-7. 7. Gokay H, Bucy PC. Osteochondroma of the lumbar spine. Report of a case. J Neurosurg 1955;12:72-8.
Gottlieb et al
8. Goldenberg DB, RienhoffWF III, Rao PS. Osteochondroma with spinal cord compression: report of a case. J Assoc Can Radiol 1968; 19:192-4. 9. Goncharova RF, Dogaeka MA, Karaidi LS. Spinal cord compression by exostosis. Vopr Neirokhir 1965;29:60. 10. Julien J, Riemens V, Vital CI, Lagueny A. Cervical cord compression by solitary osteochondroma of the atlas. J Neurol Neurosurg Psychiatry 1978;41:479-81. 11. Karaian JM, DeFilipp G, Bucheit WA, Bonakdarpour A, Eckhardt W. Vertebral osteochondroma causing spinal cord compression: case report. Neurosurgery 1984; 14:483-4. 12. Larson NE, Dodge HW Jr, Rushton JG, Dahlin DC. Hereditary multiple exostoses with compression of the spinal cord. Mayo Clin Proc 1957;32:728-34. 13. Loftus CM, Rozario RA, Prager R, Scott RM. Solitary osteochondroma of T4 with thoracic cord compression. Surg Neurol 1980;13:355-7. 14. MacGee EE. Osteochondroma of the cervical spine: a cause of transient quadriplegia. Neurosurgery 1979;4:259-60. 15. Madigan R, Worral T, McClain EJ. Cervical cord compression in hereditary multiple exostosis. J Bone Joint Surg (Am) 1974;56A:401-4. 16. Natarajan M, Balakrishnan D, Srinivasan V. Solitary osteochondroma causing spinal cord compression. Int Surg 1976;61:494-5. 17. Ochsner EH, Rothstein T. Multiple exostoses, including an exostosis within the spinal canal with surgical and neurological observations. Ann Surg 1907;46:608-16. 18. O'Connor GA, Roberts TS. Spinal cord compression by an osteochondroma in a patient with multiple osteochondromatosis. J Neurosurg 1984;60:420-3. 19. Palmer FJ, Blum PW. Osteochondroma with spinal cord compression. Report of three cases. J Neurosurg 1980;52:842-5. 20. Rose EF, Fekete A. Odontoid osteochondroma causing sudden death. Report of a case and review of the literature. Am J Clin Pathol 1964;42:606-9. 21. Sam UH, Howard LL. Hereditary multiple exostoses with myelopathy. Arch Neurol 1979;36:714. 22. Signargout J, Guegan Y, Le Marec B, Simon J. Les parapl6gies de la maladie des exostoses multiples. Apropos de deux observations. J Radiol Electrol Med Nucl 1973;54:403-7. 23. Singh DS, Srinivasa Rao S, Srivastava KK, Yadav SS. Cervical cord compression from multiple hereditary exostoses. J Assoc Physicians India 1980;28:535-7. 24. Slepian A, Hamby WB. Neurological complications associa:ed with hereditary deforming chondrodysplasia: review of the literature and a report on two cases occurring in the same family. J Neurosurg 1951;8:529-35. 25. Stocks P, Barrington A. Hereditary disorders of bone development. In: Treasury of human inheritance. Cambridge: Cambridge Univ Press 1925;3:183. 26. Thierry A, Aimard G, Bochu M, Rambaud G, Rochet M, Brunon J. Parapl6gie au cours d'une maladie exostosante multiple. J Med Lyon 1970;1473-6. 27. Thomas ML, Andress MR. Osteochondroma of the cervical spine causing cord compression. Br J Radiol 1971;44:549-50. 28. Twersky J, Kassner EG, Tenner MS, Camera A. Vertebral and costal osteochondroma causing spinal cord compression. AJR 1975;124:124-8. 29. Vinstein AL, Franken EA Jr. Hereditary multiple exostoses. Report of a case with spinal cord compression. AJR 1971;112:405-7.
581
Exostosis as a Cause of Spinal Cord Compression A l b e r t o G o t t l i e b , M . D . , P a o l o S e v e r i , M . D . , A n t o n i o R u e l l e , M . D . , a n d G i o v a n n i Lasio, M . D . Division of Neurosurgery, Ospedali Galliera, Genoa, Italy
Gottlieb A, Severi P, Ruelle A, Lasio G. Exostosis as a cause of spinal cord compression. Surg Neurol 1986;26:581-4.
A patient harboring multiple hereditary exostoses with a long-lasting tetraparesis owing to intraspinal osteochondroma is described. The results of surgical treatment were poor in contrast to the results achieved by other previous authors performing decompression of the spinal cord. The related literature is briefly reviewed and conclusions are drawn in favor of computed tomographic examination of the spine of patients with vertebral complaints. KEYWORDS: Osteochondroma; Spinal cord compression; Multiple hereditary exostoses
Multiple hereditary exostoses (osteochondrosis multipla) is one of the most frequent dysplasias of bone, mainly affecting the diaphysal parts (i.e., the part of long bones which is preformed as cartilage). Vertebral complaints are present in a small percentage of cases (7% according to Stocks and Barrington [25]). Most vertebral exostoses grow from the external parts of the laminae. In some rare cases exostoses grow into the spinal canal, compressing the spinal roots and cord. We have found in the related literature 33 cases of cord compression caused by this condition, 15 of which were in the cervical region [3,4,6,10,14,15,17-20,23, 24,27,29]. Case R e p o r t A 31-year-old man was admitted to our department for a slowly progressing tetraparesis with sensory disturbances in both legs of 16 years' duration. The patient's father, paternal grandmother, and uncle had had multiple hereditary exostoses (Figure 1). H e himself had been operated on for bone protusions in the ribs, hips, and knees during childhood. At the age of 14 years, the patient, while diving into shallow water, sustained a se-
Address reprint requests to: Dr. Paolo Severi, Division of Neurosurgery, Ospedali Galliera, via A. Volta 8, 16128 Genova, Italy.
© 1986 by ElsevierSciencePublishingCo., Inc.
vere head injury accompanied by a sudden tetraparesis, which cleared in a few minutes. No further examination was performed at that time. The neurological examination disclosed spastic tetraparesis more severe on the left side: he could stand or walk only with the help of crutches and his gait was chronically spastic. A dense superficial hypoesthesia below C-4 was present, more evident on the right side of the body. The patient did not complain of bladder disturbances or sexual dysfunction. Hyperreflexia was present in the upper and lower limbs; more so on the left side. Bilateral pathological plantar responses were also present. Plain X-rays of the skeleton revealed several unevenly distributed exostoses; the skull was unaffected. The films of the cervical spine showed hypertrophy of the lateral masses, which were probably fused. The spinal tap showed normal protein content of the fluid. There was no evidence of blockage. Myelography showed a round filling defect slightly left of the midline; at level of C-4 to C-5 the cord was pushed to the right (Figure 2). Computed tomography (CT) scanning showed a bony protusion into the spinal canal, arising from the lamina (Figure 3A). During operation an exostosis was found growing from the posterior arch of C-4, slightly left of the midline and infolding the dura mater, whose outer layer was adherent to the tumor (Figure 3B). Complete piecemeal removal of the tumor was performed. The histologic examination showed an outer layer of cartilage with a central ossifying core. The postoperative course was uneventful; when discharged, the patient's neurological examination gave unchanged results and I year later, despite continuing physiotherapy, he had attained only a slight improvement of gait.
Discussion In a thorough review of the relevant literature we found only 33 previous cases of spinal cord compression owing to osteochondromas (Table 1). In 25 cases the intraspinal tumor was accompanied by general bone complaints (multiple hereditary exostoses), whereas in nine patients [ 10,11,13,14,16,20,27,28], the only detectable 0090-3019/86/$3,50
Figure 1. Patient's family," members with multiple hereditary exostoses are shaded.
C) female
[ ] male
Figure 2. Myelography showing a round filling defect.
Spinal Exostosis
Surg Neurol 1986;26:581-4
583
1. Distribution of Exostoses along the Spine as Found in the Literature
Table
Level
A
B
Figure 3. (A) Computed tomography scan and (B) surgeon's impression of the anatomical situation of the fourth cervical vertebra.
osteochondroma was spinal, and no family history of multiple hereditary exostoses was present. In these patients diagnosis was obviously more difficult. Twenty-six out of the 34 patients were in their twenties or thirties, as is to be expected with an inherited disease (Figure 4). More men than women were affected, thus confirming that autosomic gene trasmission postulated for multiple hereditary exostoses has a greater recurrence in males, who account for as many as 73.5% of cases [7,12,18,19]. Osteochondromas did not appear to be evenly distributed along the spine: they often affected the cervical and upper thoracic regions; 31 out of 34 cases were located between C-1 and D-5 (12 vertebrae) and only 3 between D-5 and the sacrum. We also tried to learn from the literature the vertebral sites of origin of these tumors, but in cases of large lesions the authors' descriptions did not provide a precise location. Undoubtedly, however, those parts of the vertebra that derived from the neural arch were most affected, i.e., the laminae, pedicles, articular processes, and lateral posterior
No. of cases
Authors
C-1 C-2
2 7
C-4 C-5
1 3
C-6
2
C-7
1
D-1
2
D-2 D-3 D-4
1 2 5
D-5
5
D-10
1
Sam and Howard [21], Signargout et al [22] Palmer and Blum [19] Goncharova et al [9], Larson et al [12] Adam and Morin [1], Buur and Mcrch [2], Loftus et al [13], Karaian et al [11], Twersky et al [28] Goldenberg et al [8], Natarajan et al [16], Signargout et al [22], Thierry et al [26], Twersky et al [28] Decker and Wei [5]
L-3 L-4
1 1
Gokay and Bucy [7] Twersky et al [28]
Chiurco [4], Julien et al [10] Carmel [3], Ferrari et al [6], MacGee [14], Madigan et al [15], Ochsner and Rothstein [17], Rose and Fekete [20], Vinstein and Franken [29] Ours O'Connor and Roberts [18], Singh et al [23], Slepian and Hamby [24] Palmer and Blum [19], Thomas and Andress [27] Palmer and Blum [19]
parts of the vertebral body. O f these, 12 rose from the laminae, 2 from the pedicle-articular complex, 4 from the costovertebral junction and 7 from the vertebral body. The duration of symptoms varied widely, from no Figure 4. Distribution according to sex and decades of life of the exostoses. N. of c88e8
12
6
~
~
10
20
11
23
Female
Male
4
~
~
~
60 Age
584
Surg N e u r o l 1986;26:581-4
previous complaint, to sudden death from bulbar compression [20], to 32 years of progressive worsening [21]. The average duration of the complaint was 10 months. Plain X-rays of the spine are usually diagnostic or highly indicative in this condition. In only one patient out of those found in the relevant literature [26] were the plain roentgenograms normal, as was the case with our patient. Computed tomography scanning, when performed, was diagnostic in all cases. Surgical treatment in most cases resulted in cure. Our patient, on the contrary, achieved only slight improvement after operation, because of the long lasting and severe compression that his spinal cord had sustained.
Conclusion The increasing number of cases of cord compression owing to exostoses published in recent years since the CT scan has been available probably means that cord compression in the course of multiple hereditary exostoses is not as rare as the number of such cases, quoted in the literature, would imply. The ease of performing a spinal CT scan and the positive results of early operation suggest that all patients harboring spinal exostoses should be investigated by this means in order to detect possible endorachidial tumor growth even if there is no sign of cord compression at the affected vertebral level.
The authors thank Professor G. C. Andrioli for his assistance in the preparation of this paper.
References 1. Adam H, Morin P. Compression m~dullaire par exostose au cours d'une maladie exostosante. Presse Med 1966;74:660. 2. Buur T, M~rch MM. Hereditary multiple exostoses with spinal cord compression. J Neurol Neurosurg Psychiatry 1983;46:96-8. 3. Carmel PW, Cramer FJ. Cervical cord compression due to exostosis in a patient with hereditary multiple exostoses. Case report. J Neurosurg 1968;28:500-3. 4. Chiurco AA. Multiple exostoses of bone with fatal spinal cord compression: report of a case and brief review of the literature. Neurology 1970;20:275-8. 5. Decker RE, Wei WC. Thoracic cord compression from multiple hereditary exostoses associated with cerebellar astrocytoma. J Neurosurg 1969;30:310-2. 6. Ferrari G, Taddei L, Vivenza C, Rossi G. Paraparesis in hereditary multiple exostoses: case report. Neurology 1979;29:973-7. 7. Gokay H, Bucy PC. Osteochondroma of the lumbar spine. Report of a case. J Neurosurg 1955;12:72-8.
Gottlieb et al
8. Goldenberg DB, RienhoffWF III, Rao PS. Osteochondroma with spinal cord compression: report of a case. J Assoc Can Radiol 1968; 19:192-4. 9. Goncharova RF, Dogaeka MA, Karaidi LS. Spinal cord compression by exostosis. Vopr Neirokhir 1965;29:60. 10. Julien J, Riemens V, Vital CI, Lagueny A. Cervical cord compression by solitary osteochondroma of the atlas. J Neurol Neurosurg Psychiatry 1978;41:479-81. 11. Karaian JM, DeFilipp G, Bucheit WA, Bonakdarpour A, Eckhardt W. Vertebral osteochondroma causing spinal cord compression: case report. Neurosurgery 1984; 14:483-4. 12. Larson NE, Dodge HW Jr, Rushton JG, Dahlin DC. Hereditary multiple exostoses with compression of the spinal cord. Mayo Clin Proc 1957;32:728-34. 13. Loftus CM, Rozario RA, Prager R, Scott RM. Solitary osteochondroma of T4 with thoracic cord compression. Surg Neurol 1980;13:355-7. 14. MacGee EE. Osteochondroma of the cervical spine: a cause of transient quadriplegia. Neurosurgery 1979;4:259-60. 15. Madigan R, Worral T, McClain EJ. Cervical cord compression in hereditary multiple exostosis. J Bone Joint Surg (Am) 1974;56A:401-4. 16. Natarajan M, Balakrishnan D, Srinivasan V. Solitary osteochondroma causing spinal cord compression. Int Surg 1976;61:494-5. 17. Ochsner EH, Rothstein T. Multiple exostoses, including an exostosis within the spinal canal with surgical and neurological observations. Ann Surg 1907;46:608-16. 18. O'Connor GA, Roberts TS. Spinal cord compression by an osteochondroma in a patient with multiple osteochondromatosis. J Neurosurg 1984;60:420-3. 19. Palmer FJ, Blum PW. Osteochondroma with spinal cord compression. Report of three cases. J Neurosurg 1980;52:842-5. 20. Rose EF, Fekete A. Odontoid osteochondroma causing sudden death. Report of a case and review of the literature. Am J Clin Pathol 1964;42:606-9. 21. Sam UH, Howard LL. Hereditary multiple exostoses with myelopathy. Arch Neurol 1979;36:714. 22. Signargout J, Guegan Y, Le Marec B, Simon J. Les parapl6gies de la maladie des exostoses multiples. Apropos de deux observations. J Radiol Electrol Med Nucl 1973;54:403-7. 23. Singh DS, Srinivasa Rao S, Srivastava KK, Yadav SS. Cervical cord compression from multiple hereditary exostoses. J Assoc Physicians India 1980;28:535-7. 24. Slepian A, Hamby WB. Neurological complications associa:ed with hereditary deforming chondrodysplasia: review of the literature and a report on two cases occurring in the same family. J Neurosurg 1951;8:529-35. 25. Stocks P, Barrington A. Hereditary disorders of bone development. In: Treasury of human inheritance. Cambridge: Cambridge Univ Press 1925;3:183. 26. Thierry A, Aimard G, Bochu M, Rambaud G, Rochet M, Brunon J. Parapl6gie au cours d'une maladie exostosante multiple. J Med Lyon 1970;1473-6. 27. Thomas ML, Andress MR. Osteochondroma of the cervical spine causing cord compression. Br J Radiol 1971;44:549-50. 28. Twersky J, Kassner EG, Tenner MS, Camera A. Vertebral and costal osteochondroma causing spinal cord compression. AJR 1975;124:124-8. 29. Vinstein AL, Franken EA Jr. Hereditary multiple exostoses. Report of a case with spinal cord compression. AJR 1971;112:405-7.