Surgical decompression of thoracic spinal stenosis in achondroplasia: indication and outcome.
OBJECTIVE
The achondroplastic spinal canal is narrow due to short pedicles and a small interpedicular distance. Compression of neural structures passing through this canal is therefore regularly encountered but rarely described. Symptomatology, radiological evaluation, and treatment of 20 patients with achondroplasia who underwent decompression of the thoracic spinal cord are described and outcome is correlated with the size of the spinal canal and the thoracolumbar kyphotic angle.


METHODS
Scores from the modified Japanese Orthopaedic Association scale, Nurick scale, European Myelopathy scale, Cooper myelopathy scale for lower extremities, and Odom criteria before and after surgery were compared. Magnetic resonance imaging was evaluated to determine the size of the spinal canal, spinal cord compression, and presence of myelomalacia. The thoracolumbar kyphotic angle was measured using fluoroscopy.


RESULTS
Patient symptomatology included deterioration of walking pattern, pain, cramps, spasms, and incontinence. Magnetic resonance images of all patients demonstrated spinal cord compression due to degenerative changes. Surgery resulted in a slight improvement on all the ranking scales. Surgery at the wrong level occurred in 15% of cases, but no serious complications occurred. The mean thoracolumbar kyphotic angle was 20°, and no correlation was established between this angle and outcome after surgery. No postoperative increase in this angle was reported. There was also no correlation between size of the spinal canal and outcome.


CONCLUSIONS
Decompressive surgery of the thoracic spinal cord in patients with achondroplasia can be performed safely if anatomical details are taken into consideration. Spondylodesis did not appear essential. Special attention should be given to the method of surgery, identification of the level of interest, and follow-up of the thoracolumbar kyphotic angle.
Department of Neurosurgery, Leiden University Medical Centre, Leiden, The Netherlands. cvleggeert@lumc.nl
Carmen
C
Vleggeert-Lankamp
Wilco
W
Peul
D000130:Achondroplasia / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D000328:Adult; D000368:Aged; D019299:Decompression, Surgical / Q000379:methods*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011184:Postoperative Period; D012720:Severity of Illness Index; D013117:Spinal Cord Compression / Q000175:diagnosis / Q000601:surgery*; D013130:Spinal Stenosis / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D013904:Thoracic Vertebrae / Q000601:surgery; D016896:Treatment Outcome; D055815:Young Adult