Pediatric Neurology 51 (2014) 858e859

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Pediatric Neurology
journal homepage: www.elsevier.com/locate/pnu

Visual Diagnosis

Multiple Exostoses Presenting With High Cervical Myelopathy

in the First Decade of Life
Vykuntaraju K. Gowda DM a, *, Praveen-kumar Srikanteswara DM b,
Rudra Prasad DNB c
a

Department of Pediatric Neurology, Indira Gandhi Institute of Child Health, Bangalore, India
Department of Neurology, Bangalore Medical College and Research Institute, Bangalore, India
c
Department of Orthopaedics, Indira Gandhi Institute of Child Health, Bangalore, India
b

Hereditary multiple exostoses (HMEs) is a benign

condition characterized by the presence of multiple exostoses.1 Spinal cord compression resulting from vertebral
osteochondroma is a rare complication of this entity, and
such a presentation in the rst decade of life is even
rarer.2
A 9-year-old girl presented with multiple bony swellings
adjacent to the joints during the last 5 years and progressive
difculty in walking for 6 months before presentation. On
examination, multiple bony swellings were observed over
the medial side of the left knee and lower thighs, lateral side
of the right elbow, the anterior aspect of right arm, and the
lateral side of right wrist (Fig 1). She had spasticity of all
limbs and motor power was 3 of 5 (Medical research council

web 4C=FPO

FIGURE 2.
Radiograph of the knees demonstrates bilateral exostoses.

FIGURE 1.
Clinical photographs demonstrate bony swelling around the knees and
elbow. (The color version of this gure is available in the online edition.)
* Communications should be addressed to: Dr. Vykuntaraju K Gowda,
Bangalore Child Neurology and Rehabilitation Center, HANS complex, 8/
A 1st Main 1st Cross, Manuvana, Near Adhichunchanagiri Choultry,
Vijayanagar, Bangalore, 560040, India. Tel.: 080-23301212, 919535212556; fax: 080-26541799.
E-mail address: drknvraju@hotmail.com
0887-8994/$ - see front matter 2014 Elsevier Inc. All rights reserved.
http://dx.doi.org/10.1016/j.pediatrneurol.2014.08.030

grade). She had exaggerated deep tendon reexes. Knee

radiographs revealed exostoses (Fig 2). Magnetic resonance
imaging of the cervical spine demonstrated an osteochondroma arising from the C2 lamina causing spinal canal
narrowing and compression of spinal cord (Fig 3). Motor
power improved after removal of the cervical exostosis and
spinal cord decompression.
The exostoses commonly involve metaphysis of long
bones and less frequently vertebrae.2 It is inherited as an
autosomal dominant trait with variable penetrance. There
were no affected family members, suggesting sporadic
occurrence. Neurological dysfunction is most often caused

V.K. Gowda et al. / Pediatric Neurology 51 (2014) 858e859

859

FIGURE 3.
Midsagittal and parasagittal T2 magnetic resonance images of the cervical spine and brainstem demonstrate osteochondroma arising from the right C2
lamina with severe spinal canal narrowing, spinal cord compression, and spinal cord signal changes.

by mechanical irritation, rarely because of nerve root and

spinal cord compression.3
Reid et al4 were rst to describe myelopathy associated with HME. In the vertebral column, the cervical
spine is commonly affected. In our patient too, C2 was
involved. The mean age of presentation is 20 years. Our
patient and that of Labram et al.2 are the only examples
of presentation in the rst decade of life. Compressive
myelopathy due to osteochondroma is an uncommon
condition in patients with HME and can result in
serious neurological sequelae if not diagnosed and
treated early.

References
1. Atabay H, Kuyucu Y, Korkmaz O, Iplikcioglu AC. Myelopathy due to
hereditary multiple exostoses: CT and MR studies. Clin Neurol Neurosurg. 1996;98:186-188.
2. Labram EK, Mohan J. Diaphyseal aclasis with spinal cord compression. Report of two cases and review of the literature. J Neurosurg.
1996;84:518-521.
3. Carmel PW, Cramer FJ. Cervical cord compression due to exostosis in
a patient with hereditary multiple exostoses. Case report. J Neurosurg.
1968;28:500-530.
4. Reid J. Case of disease of the spinal cord from an exostosis of the
second cervical vertebra. Lond Edinb Mon J Med Sci. 1843;3:194-198.