Craniovertebral abnormalities in Type VI mucopolysaccharidosis (Maroteaux-Lamy syndrome).
BACKGROUND
The craniovertebral abnormalities found in patients with Type VI mucopolysaccharidosis (Maroteaux-Lamy syndrome) are described, and the indications for and outcomes of surgery in this group are assessed.


METHODS
The clinical histories and radiological findings in all patients with Type VI mucopolysaccharidosis treated at Royal Manchester Children's Hospital during the past 10 years were reviewed.


RESULTS
The typical findings in patients with this disease are of canal stenosis at the level of the foramen magnum and upper cervical spine with or without cord compression. The stenosis is secondary to thickening of the posterior longitudinal ligament. Atlantoaxial instability is rare. Of nine patients under regular clinical review, four underwent decompressive surgery for cervical cord compression. Three of the four showed improvement in their neurological symptoms and signs postoperatively. Of the children reviewed, six had radiological evidence of cord compression, although only those with neurological signs or symptoms were treated surgically.


CONCLUSIONS
Despite the often formidable anesthetic challenge, surgery is indicated in those patients who present with progressive neurological deficit due to cervical myelopathy. Surgery can be undertaken safely if the associated medical problems in these children are recognized and managed appropriately.
Thorne
J A
JA
Department of Neurosurgery, Royal Manchester Children's Hospital, England. johnathorne@yahoo.co.uk
Javadpour
M
M
Hughes
D G
DG
Wraith
E
E
Cowie
R A
RA
D000293:Adolescent; D001268:Atlanto-Axial Joint / Q000473:pathology / Q000601:surgery; D002574:Cervical Vertebrae / Q000473:pathology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D019299:Decompression, Surgical; D005260:Female; D005539:Foramen Magnum / Q000473:pathology / Q000601:surgery; D006801:Humans; D007593:Joint Instability / Q000175:diagnosis / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008297:Male; D009087:Mucopolysaccharidosis VI / Q000175:diagnosis / Q000601:surgery*; D009460:Neurologic Examination; D011183:Postoperative Complications / Q000175:diagnosis; D012189:Retrospective Studies; D013117:Spinal Cord Compression / Q000175:diagnosis / Q000601:surgery*; D013130:Spinal Stenosis / Q000175:diagnosis / Q000601:surgery*; D014057:Tomography, X-Ray Computed