Anterior C1-2 osteochondroma presenting with dysphagia and sleep apnea

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terolateral transcondylar have been documented in the manage-ment of craniocervical and cervicothoracic junction neurentericcysts.4

In conclusion, neurenteric cysts are uncommon tumours ofdevelopmental origin. Presentation ranges from being asymptom-atic to profound neurological deficits, and is dependent on its loca-tion. Early surgical intervention and long-term follow-up in partialexcision is recommend, particularly to prevent long-termmorbidity.

doi:10.1016/j.jocn.2008.04.029

* Corresponding author. Tel.: +1 415 3539095; fax: +1 415 3533907.E-mail address: choud@neurosurg.ucsf.edu (D. Chou).

References

1. Wilkins RH. Intraspinal cysts. In: Wilkins RH, Rengachary SS, editors.Neurosurgery. 2nd ed. New York: McGraw-Hill; 1996. p. 3514–6.

2. Khosla A, Wippold FJ. CT myelography and MR imaging of extramedullary cystsof the spinal canal in adult and pediatric patients. AJR Am J Roentgenol2002;178:201–7.

3. Akoi S, Machida T, Sasaki Y, et al. Enterogenous cyst of the cervical spine: Clinicaland radiological aspects. Neuroradiology 1987;29:291–3.

4. Fuse T, Yamada K, Kamiya K, et al. Neuroenteric cyst at the craniovertebraljunction: Report of 2 cases. Surg Neurol 1998;50:431–6.

Anterior C1-2 osteochondroma presenting with dysphagia and sleep apnea

Vincent Wang, Dean Chou *

Department of Neurological Surgery, University of California San Francisco, 505 Parnassus Ave. Rm. M779, San Francisco, CA 94143-0112, USA

a r t i c l e i n f o

Article history:Received 12 January 2008Accepted 18 May 2008

Keywords:OsteochondromaC1 archTranscervical approach

a b s t r a c t

Spinal osteochondromas are rare, benign tumors of the bone. These tumors can manifest as solitarylesions or as part of a hereditary syndrome. Most spinal osteochondromas occur in the posterior cervicalspine and can cause myelopathy or radiculopathy. Osteochondromas of the anterior cervical spine thatcause respiratory or swallowing symptoms are rare. We present the unique case of a solitary osteochon-droma of the anterior C1 vertebral arch causing obstructive sleep apnea and dysphagia in a 16-year-oldfemale. The patient underwent resection of the tumor via a left anterior transcervical approach to thespine. The patient’s symptoms resolved completely after surgery. To our knowledge, this is the first caseof a sporadic osteochondroma arising from the anterior arch of the C1 vertebra causing dysphagia andobstructive apnea in a pediatric patient.

� 2008 Elsevier Ltd. All rights reserved.

1. Introduction

Osteochondroma is a benign tumor of the bone that can be sol-itary or part of a hereditary syndrome known as multiple heredi-tary exostoses (MHE). Most spinal osteochondromas occur in theposterior elements of the cervical spine.1 Osteochondromas arisingfrom the anterior elements are rare. In this report, we present thecase of a sporadic osteochondroma arising from the anterior archof the first cervical vertebra (C1) presenting with dysphagia,snoring, and sleep apnea in a 16 year-old girl without MHE.

2. Case report

Two years prior to presentation, the 16 year-old female noticeddifficulty swallowing and the new onset of snoring. On physicalexamination, there was a visible bulge behind her pharynx. MRIand CT of the cervical spine revealed a bony lesion projecting ante-riorly from the left side of the C1 arch and extending caudally tothe second cervical vertebra (C2) body (Figs. 1 and 2). The lesionhad high signal intensity on T1- and T2-weighted MRI and wasconsistent with osteochondroma.

Over the next two years, her symptoms worsened and a repeatMRI revealed that the lesion did not grow but had lost its attachmentto the C1 arch. The patient elected to have the lesion removed surgi-cally because she felt the lesion was causing her symptoms.

A transoral operation was initially considered because of thelesion’s location at C1; however, an en bloc resection would require

a mandibular osteotomy. Given the benign nature of the lesion, aleft anterior transcervical approach to the spine was choseninstead.

A transverse incision was used and cranial nerves XI, X, XII, thesuperior laryngeal neurovascular bundle, carotid artery, internaljugular vein, and great auricular nerve were identified and pre-served. The tumor was identified in the retropharyngeal space(Fig. 3). A straight osteotome was used to make a cut at the baseof the tumor and the bulk was removed in one piece. Further re-moval of tumor was performed in a piecemeal fashion with rong-eurs. All gross tumor was removed except for the small stumpemanating from the anterior arch of C1. Because of the benign nat-ure of osteochondroma, we did not pursue this small stump due tothe extreme angle of approach and risk of vascular injury.

Histological analysis confirmed the diagnosis of osteochon-droma. The patient did well postoperatively with minimal dyspha-gia that resolved after 2 weeks. A postoperative CT showedremoval of most of the tumor except for the small remnant inten-tionally left at the arch of C1. At 10 month follow-up CT, there wasno evidence of tumor growth, and the patient had significant alle-viation of her symptoms.

3. Discussion

Osteochondroma is the most common benign bone tumor, andusually occurs in long bones. Osteochondromas are thought toarise from a lateral displacement of a portion of the epiphysealgrowth cartilage to become a bone-like outgrowth capped bycartilage.1 Spinal osteochondromas are uncommon, and most casesoccur in patients with MHE.

Most patients have symptoms from months to years,1,2 as inthis case. Tumors arising from the posterior elements can causecord or nerve compression, leading to myelopathy or radiculopa-thy.1 Patients can also present with headache or neck pain.1,5 Sud-den death due to C1 osteochondroma has also been reported.6

Within the spine, about half of osteochondromas arise from thecervical spine, mostly from posterior elements.1 Tumors arisingfrom anterior elements are rare.4,8 Anterior cervical tumors can

cause dysphagia by mass effect against the esophagus, hoarsenessand lower cranial nerve abnormalities by nerve compression.4,7

Two other cases of osteochondromas from the anterior arch of C1have been reported. One occurred in a patient with MHE who pre-sented with dysphagia and another sporadic case was an elderlyfemale with obstructive sleep apnea.3,8 To our knowledge, thereare no reports of an anterior C1 osteochondroma causing dyspha-gia and sleep apnea in a pediatric patient without known MHE.

Workup of patients with spinal osteochondroma usually beginswith plain radiographs. On MRI, osteochondromas often have highsignal intensity on both T1- and T2-weighted sequences withoutcontrast enhancement.2 There is often a rim of low signal intensityrepresenting calcification.2 CT is the imaging modality of choice forevaluating osteochondromas.1,2 It can reveal the cartilaginous andbony details well.

Surgical resection of these lesions is the mainstay of treatment.Resection often leads to improvement of symptoms, even if thesymptoms have been present for years.1 Complete resection ofthe tumor is the goal, and removal of the entire cartilaginous capis recommended to prevent recurrence. There is a 1% chance thatthe residual tumor may have malignant transformation and re-growth is not uncommon.1 Thus, these patients should be followedby serial imaging.

References

1. Albrecht S, Crutchfield JS, SeGall GK. On spinal osteochondromas. J Neurosurg1992;77:247–52.

2. Aldea S, Bonneville F, Poirier J, et al. Acute spinal cord compression in hereditarymultiple exostoses. Acta Neurochir (Wien) 2006;148:195–8.

3. Barros Filho TE, Oliveira RP, Taricco MA, et al. Hereditary multiple exostoses andcervical ventral protuberance causing dysphagia. A case report. Spine1995;20:1640–2.

4. Grivas TB, Polyzois VD, Xarchas K, et al. Seventh cervical vertebral body solitaryosteochondroma. Report of a case and review of the literature. Eur Spine J2005;14:795–8.

5. Kouwenhoven JW, Wuisman PI, Ploegmakers JF. Headache due to anosteochondroma of the axis. Eur Spine J 2004;13:746–9.

6. Sakai D, Mochida J, Toh E, et al. Spinal osteochondromas in middle-aged toelderly patients. Spine 2002;27:E503–6.

7. Slavotinek JP, Brophy BP, Sage MR. Bony exostosis of the atlas with resultantcranial nerve palsy. Neuroradiology 1991;33:453–4.

8. Yoshida T, Matsuda H, Horiuchi C, et al. A case of osteochondroma of the atlascausing obstructive sleep apnea syndrome. Acta Otolaryngol 2006;126:445–8.

doi:10.1016/j.jocn.2008.05.024

Fig. 1. Axial T1-weighted MRI without gadolinium showing the lesion anterior toC2.

Fig. 2. Preoperative axial CT scan showing the lesion originating from the C1 arch.

Fig. 3. Intraoperative photo showing the lesion exposed by a standard transcervicalapproach. This figure is available in colour at www.sciencedirect.com.

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