Tetraparesis due to exostotic osteochondroma at upper cervical cord in a patient with multiple exostoses-mental retardation syndrome (Langer-Giedion syndrome).
METHODS
Case report of a severe upper cervical cord compression and tetraparesis by a massive cervical exostotic osteochondroma in a patient with multiple exostoses-mental retardation syndrome (Langer-Giedion syndrome; LGS).


OBJECTIVE
To describe this very rare pathological condition and the results of surgical intervention.


METHODS
Gifu, Japan.


METHODS
A 23-year-old man was referred to our clinic because of progressing tetraparesis. He had previously been diagnosed with hereditary multiple exostoses and mental retardation. As he had not complained of any symptoms, his family only noticed the tetraparesis after advanced deterioration. His face possessed the pathognomic features of LGS. A postmyelogram CT scan demonstrated an exostotic mass arising from the left-side C2 pedicle with associated severe spinal cord compression. He was diagnosed with LGS. Hemilaminectomy on the left side and resection of the osteochondroma were performed.


RESULTS
At 5 years postoperatively, a neurological examination showed the full return of all motor functions. The CT scan revealed no intracanalar recurrence of the tumor.


CONCLUSIONS
In this case of severe tetraparesis due to cervical osteochondroma, decompression by hemilaminectomy provided excellent results. In patients with LGS and intracanalar osteochondroma, the neurological deficit may be masked by mental retardation. Hence, awareness of this pathological condition will help clinicians diagnose it at an early stage.
Miyamoto
K
K
Department of Orthopaedic Surgery, Gifu University School of Medicine, Yanagido, Gifu-City, Gifu-Prefecture, Japan.
Sakaguchi
Y
Y
Hosoe
H
H
Mori
A
A
Yamazaki
S
S
Hattori
S
S
Shimizu
K
K
D000328:Adult; D002574:Cervical Vertebrae / Q000000981:diagnostic imaging / Q000601:surgery; D005097:Exostoses, Multiple Hereditary / Q000000981:diagnostic imaging* / Q000601:surgery*; D006801:Humans; D007796:Laminectomy; D015826:Langer-Giedion Syndrome / Q000000981:diagnostic imaging* / Q000601:surgery*; D008297:Male; D011782:Quadriplegia / Q000000981:diagnostic imaging* / Q000601:surgery*; D011859:Radiography; D016896:Treatment Outcome