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 Table of Contents  
CASE REPORTS
Year : 2022  |  Volume : 5  |  Issue : 1  |  Page : 137-141

C2 intraspinal osteochondroma causing spinal cord compression in a patient with multiple hereditary exostoses


Department of Orthopedics, Yenepoya Medical College, Mangalore, Karnataka, India

Date of Submission08-Jul-2020
Date of Decision04-Aug-2020
Date of Acceptance29-Dec-2020
Date of Web Publication02-Feb-2022

Correspondence Address:
Janardhana P Aithala
Department of Orthopedics, Yenepoya Medical College, Deralakatte, Mangalore 575018, Karnataka.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ISJ.ISJ_55_20

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  Abstract 

Intraspinal osteochondroma causing neurological manifestations is a rare condition and can present as either solitary osteochondroma or more commonly as a part of multiple hereditary exostoses. We report a case of osteochondroma arising from lamina of C2 in a 21-year-old patient with multiple hereditary exostoses causing spinal cord compression and cervical myelopathy. The patient presented with worsening neurological deficit and an inability to walk. Immediate laminectomy and surgical decompression were done after accurately localizing the lesion through computed tomography and magnetic resonance images. Following excision of the lesion, the patient recovered completely. The case report is followed by a review of literature highlighting the incidence of spinal osteochondroma, location preferences, clinical presentation, diagnosis, and results of decompression.

Keywords: Cervical spine, laminectomy, multiple hereditary exostoses, osteochondroma, spinal cord compression


How to cite this article:
Aithala JP. C2 intraspinal osteochondroma causing spinal cord compression in a patient with multiple hereditary exostoses. Indian Spine J 2022;5:137-41

How to cite this URL:
Aithala JP. C2 intraspinal osteochondroma causing spinal cord compression in a patient with multiple hereditary exostoses. Indian Spine J [serial online] 2022 [cited 2022 May 25];5:137-41. Available from: https://www.isjonline.com/text.asp?2022/5/1/137/337146




  Introduction Top


Boyer (1814) described the occurrence of multiple hereditary exostoses (MHE) in several members of a family, Solomon identified MHE as a hereditary disorder with autosomal-dominant inheritance.[1],[2] The involvement of the cervical spine is rare but can occur either as solitary osteochondroma (3%)[3],[4] or as a part of MHE (7–9%).[5] Intraspinal exostoses can cause spinal cord compression and neurological deficits.[3],[6] Early detection and excision of exostoses is the treatment of choice to prevent the progression of neurological deficit and ensure a good recovery.[5],[7] We report a case of MHE with a huge exostosis arising from C2 lamina with rapidly deteriorating neurological deficit which was treated with surgical decompression. A detailed review of literature highlighting the incidence of spinal osteochondroma, location preferences, clinical presentation, diagnosis, and results of decompression is also presented.


  Case Presentation Top


A 21-year-old male presented with inability to walk and sit, along with loss of bladder control for the previous two weeks. He had a history of tingling sensation in both upper and lower limbs for the last ten months but he had not sought any medical treatment. On examination, he had multiple exostoses arising from the left scapula [Figure 1], right proximal humerus, and proximal tibia on both sides. The patient was nonambulatory and his neurological examination revealed hypertonia with exaggerated reflexes, loss of proprioception, and motor power varying from grade 3 to 4 involving both upper and lower limb muscles including shoulder abduction. Laboratory values including full blood count, electrolytes, and inflammatory markers were all within normal limits.
Figure 1: MHE showing lesion at scapula and shoulder

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Although at first glance, his X-rays of the cervical spine looked normal, on closer examination we found some evidence of broadening with the irregularity of the posterior elements of the C2 vertebra [Figure 2]. Computed tomography (CT) scans showed an osseous growth originating from the lamina of the right C2 vertebra protruding into the spinal canal [Figure 3]. Magnetic resonance images (MRI) revealed the compression of the spinal cord at the C2 with myelomalacia changes of the spinal cord [Figure 4].
Figure 2: lateral view X-ray showing broadening of posterior elements of C2

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Figure 3: CT images showing intraspinal exostoses arising from lamina of C2 away from facet joint

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Figure 4: Axial image showing cartilage cap (A) and compression of cord (B), a sagittal image showing exostoses extending into the spinal canal (C)

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A diagnosis of cervical myelopathy caused by the compression of the spinal cord due to an intraspinal osteochondroma was made. The patient underwent C2 hemilaminectomy right side with a posterior approach. High-speed burr was used to make gutters medially and laterally (sparing facet joint). On the lateral side, the dura was identified first to ensure that there were no adhesions between dura and exostoses and then the superior and inferior border of lamina was freed from ligamentum flavum. Lamina was freed from all around and was slowly lifted along with complete exostoses. [Figure 5]. The histopathologic examination confirmed our diagnosis of a benign osteochondroma. Postoperatively, the patient reported a reduction in tingling sensation in both his upper and lower limbs immediately. The patient was mobilized on day ten with a walker and the patient regained full motor power and bladder control by the end of two months. The patient was followed up to 2 years and remains asymptomatic.
Figure 5: Excised exostoses mass

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  Discussion Top


Osteochondroma involving the spine is rare and accounts for about 7–9%[5] of all in the case of MHE and 3% in the case of solitary osteochondroma.[8] Cervical spine involvement is seen in 50% of spine cases with C2 being the most common.[3],[7],[9]

While most of the literature about cervical osteochondroma are case reports, few authors have done an extensive review of the literature. Lotfinia et al.[3] in their case series of eight cases found even distribution of lesions among cervical and lumbar spines. Authors made an extensive review of 176 cases published in literature till 2010 which included 79 cases of spine involvement with cord compression due to solitary osteochondroma (mean age 28 years) and 74 cases with MHE (mean age 17 years). Among solitary osteochondroma, 58% were in the cervical spine with 27% involving C2; C3 and C6 involvement were next common in that order. Among the MHE group, 68% had cervical spine involvement with cord compression, and 43% involved C2. There was family history in 56% of cases in the MHE group. In both categories, the thoracic spine was the second common site, while there were only 13 cases involving the lumbar spine. Albrecht et al.[6] in their review of 130 cases identified 32 cases with MHE and 96 patients with solitary osteochondromas. 50% of lesions in each category were in the cervical region and eight cases in each category were from C2. Yakkanti et al.[10] reviewed 132 cases of solitary osteochondroma of the spine in which 63 cases were found in the cervical spine (52.2%), the involvement of the posterior column in 85 cases (64.3%), and myelopathic symptoms in 36 patients (27.2%).

The cervical spine is the most commonly involved region in the spine and this may be due to the mobility of the cervical spine leading to greater stress which can cause microtrauma and displacement of epiphyseal cartilage.[6],[11],[12] Most of the osteochondromas were seen in posterior elements[3],[13] involving lamina, pedicle, and articular processes, while there are few reports of anterior osteochondroma causing hoarseness of voice and dysphagia.[14]

Most of the literature related to cervical osteochondroma is in the form of case reports and a review of the literature. However, since these are case reports of symptomatic cases, they cannot be used to calculate the actual incidence of exostoses in the spine especially in MHE. An imaging study of all MHE present in a population is required to calculate the incidence, but such studies are very rare. Roach et al.[15] studied 44 cases of MHE and found 30 patients with spinal lesions, out of which only 12 were encroaching on the spinal canal. This study highlights the fact that asymptomatic involvement of the spinal column is much higher than the quoted 7–9% spine involvement. Similarly, they feel that asymptomatic lesions can be found arising from the spinous process, transverse process and hence they may be the most common sites for spine exostoses.

The most common presentation of symptomatic exostoses is neurological involvement. Neurological deterioration can happen rapidly.[3],[6] There was a report of sudden death due to the involvement of odontoid.[16] Trauma or sudden hyperextension of the spine can precipitate acute neurological deficits.[6] Hence early detection and complete removal of exostoses give good results.[16] Patients with MHE can present early with higher rates of neurological involvement compared to the solitary osteochondroma group.[4] Lotfinia et al.[3] in their review found that majority have improved with surgery, while only two patients had a recurrence. There were six death reports (three in the solitary osteochondroma group and three in MHE group) in which five cases were not operated. Albrecht et al.[6] in their review of the literature found neurological deficit due to cervical spine involvement in 75% and 35% of patients, respectively, from the MHE group and solitary osteochondroma group. Roach et al.[15] in their study of 30 spinal lesions found that six patients required surgery (three patients due to neurological deficit and three patients due to the large size of exostoses). The authors opine that those lesions which are symptomatic and those lesions which are large and near the spinal cord need to be excised, while other asymptomatic lesions that are small need not be excised. Yakkanti et al.,[10] in their review of the literature of 36 cases of myelopathy, found that 34 cases showed improvement after surgery, while 2 patients showed deterioration, and 2 cases showed recurrence.

An X-ray may not help in identifying the lesion (21%).[6] Between CT and MRI, the cartilaginous cap is well appreciated in MRI, while an osseous lesion is better seen with CT. Moriwaka et al.[17] found comparable reliability between CT and MRI. Malignant transformation although rare needs to be considered while evaluating images.

The treatment of choice is laminectomy with high-speed burr and excision of the osteochondroma.[3],[6] Fusion may be required if facet joints are involved,[8] removal of large exostoses involves extensive laminectomy which can weaken the facet joints and lead to instability.[18] With complete excision of spinal osteochondroma, most patients show good recovery. Lotfinia et al. in their review of 176 cases found neurological improvement in 89% of cases. There were five deaths and seven cases of neurological deterioration. Lotfinia et al.[11] in a separate paper analyzed evidence from literature regarding C1 osteochondromas and found that 33% have neurological deficit and there was a recurrence of three cases. The recurrence rate varies from 0 to 4%.[3],[19] Osteochondroma needs to be excised completely to prevent a recurrence. Bess et al.[20] found that the recurrence rate is high if intralesional excision is done. Gille et al.[19] in their review of 150 cases of solitary osteochondroma found six cases (4%) of local recurrence and four cases (2.7%) of malignant transformation. In general, malignant transformation in SOC is 1% and 10% in MHE, but vertebral lesions may have a higher incidence.[3],[21]

Our case highlights the timely detection of neurological symptoms, the use of appropriate imaging modalities to make the diagnosis, and the results of early surgical excision. In our case, as the lesion was not extending to the facet joint, we preserved facet joints and hence the fusion of segment was not done. Using the burr, we could easily perform hemilaminectomy and as the osteochondroma was not adherent to the dura, it was easily separated and removed. This case also highlights the need for early decompression of the cord. The patient showed recovery in two months and remains asymptomatic until two years.


  Conclusion Top


Intraspinal osteochondroma leading to spinal cord compression needs to be considered in cases of MHE presenting with neurological symptoms and should be evaluated with adequate imaging modalities like CT and MRI. Laminectomy and surgical decompression by removing the osteochondroma completely at the earliest leads to complete neurological recovery.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Boyer A Traite des Maladies Chirurgicales, vol 3. Paris: Migneret 1964;1814. p. 594.  Back to cited text no. 1
    
2.
Soloman L Hereditary multiple exostoses. J Bone Joint Surg Br 1963;45:292-304.  Back to cited text no. 2
    
3.
Lotfinia I, Vahedi P, Tubbs RS, Ghavame M, Meshkini A Neurological manifestations, imaging characteristics, and surgical outcome of intraspinal osteochondroma. J Neurosurg Spine 2010;12:474-89.  Back to cited text no. 3
    
4.
Khosla A, Martin DS, Awwad EE The solitary intraspinal vertebral osteochondroma. An unusual cause of compressive myelopathy: Features and literature review. Spine (Phila Pa 1976) 1999;24:77-81.  Back to cited text no. 4
    
5.
Mikawa Y, Watanabe R, Nakashima Y, Hayashida T Cervical spinal cord compression in hereditary multiple exostoses. Report of a case and a review of the literature. Arch Orthop Trauma Surg 1997;116:112-5.  Back to cited text no. 5
    
6.
Albrecht S, Crutchfield JS, SeGall GK On spinal osteochondromas. J Neurosurg 1992;77:247-52.  Back to cited text no. 6
    
7.
Patel A, Thacker MM Cervical spinal canal compromise in a 14-year-old girl with hereditary multiple exostoses. Pediatr Radiol 2010;40(Suppl 1):S158.  Back to cited text no. 7
    
8.
Srikantha U, Bhagavatula ID, Atyanarayana S, Somanna S, Chandramouli BA Spinal osteochondroma: Spectrum of a rare disease Report of 3 cases. J Neurosurg Spine 2008;8:561-6.  Back to cited text no. 8
    
9.
Calhoun JM, Chadduck WM, Smith JL Single cervical exostosis. Report of a case and review of the literature. Surg Neurol 1992;37:26-9.  Back to cited text no. 9
    
10.
Yakkanti R, Onyekwelu I, Carreon LY, Dimar JR 2nd. Solitary osteochondroma of the spine-A case series: Review of solitary osteochondroma with myelopathic symptoms. Global Spine J 2018;8:323-39.  Back to cited text no. 10
    
11.
Lotfinia I, Vahedi A, Aeinfar K, Tubbs RS , Vahedi P . Cervical osteochondroma with neurological symptoms: Literature review and a case report. Spinal Cord Ser Cases 2017;3:16038.  Back to cited text no. 11
    
12.
Moon KS, Lee JK, Kim YS, Kwak HJ, Joo SP, Kim IY, et al. Osteochondroma of the cervical spine extending multiple segments with cord compression. Pediatr Neurosurg 2006;42:304-7.  Back to cited text no. 12
    
13.
George B, Atallah A, Laurian C, Tayon B, Mikol J Cervical osteochondroma (C2 level) with vertebral artery occlusion and second cervical nerve root irritation. Surg Neurol 1989;31:459-64.  Back to cited text no. 13
    
14.
Peck JH Dysphagia due to massive exostosis of the cervical spine. J Bone Joint Surg (Am) 1964;46:1379.   Back to cited text no. 14
    
15.
Roach JW, Klatt JW, Faulkner ND Involvement of the spine in patients with multiple hereditary exostoses. J Bone Joint Surg Am 2009;91:1942-8.  Back to cited text no. 15
    
16.
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.  Back to cited text no. 16
    
17.
Moriwaka F, Hozen H, Nakane K, Sasaki H, Tashiro K, Abe H Myelopathy due to osteochondroma: MR and CT studies. J Comput Assist Tomogr 1990;14:128-30.  Back to cited text no. 17
    
18.
Bhojraj SY, Panjwani JS A new management approach to decompression, posterior stabilization, and fusion for cervical laminar exostosis with cord compression in a case of diaphyseal aclasis. Case report and review of the literature. Spine 1993;18: 1376-9.  Back to cited text no. 18
    
19.
Gille O, Pointillart V, Vital JM Course of spinal solitary osteochondromas. Spine 2005;30:E13-9.  Back to cited text no. 19
    
20.
Bess RS, Robbin MR, Bohlman HH, Thompson GH Spinal exostoses: Analysis of twelve cases and review of the literature. Spine (Phila Pa 1976) 2005;30:774-80.  Back to cited text no. 20
    
21.
Jaffe HL Hereditary multiple exostoses with myelopathy. Arch Pathol 1943;36:335-57.  Back to cited text no. 21
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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