Early neurosurgical intervention in spondyloepiphyseal dysplasias.
OBJECTIVE
The aim of this study is to evaluate the benefits of early intervention in two major spondyloepiphyseal dysplasias of Saudi Arabia, namely, multiple sulfatase deficiency (MSD, Austin's disease) and Morquio's disease. The MSD is encountered frequently in the Kingdom and poses significant health risk to the child because of cord compression. The clinics of this hospital have several Austin's patients.


RESULTS
This study indicates that early intervention before serious irreversible damage to the cervical cord occurs improves the neurological course of the patient; no patient had a worse outcome. On the other hand, neurosurgical intervention after the neurological symptoms of cord compression occurs was not as rewarding.


CONCLUSIONS
Morquio's disease is more common outside the Kingdom. The results in this study also confirm that early intervention in this disease is beneficial. Other surgeons make a similar recommendation for Morquio's disease. However, their experience with Austin's disease is not reported due to the rarity of this disease elsewhere.
Al-Shail
E
E
Department of Neurosurgery, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
Al-Odaib
A
A
Ozand
P T
PT
D000367:Age Factors; D002574:Cervical Vertebrae; D002648:Child; D002675:Child, Preschool; D019299:Decompression, Surgical / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D009085:Mucopolysaccharidosis IV / Q000150:complications*; D052517:Multiple Sulfatase Deficiency Disease / Q000150:complications*; D009809:Odontoid Process / Q000473:pathology; D010009:Osteochondrodysplasias / Q000201:enzymology / Q000209:etiology / Q000601:surgery*; D012529:Saudi Arabia; D013117:Spinal Cord Compression / Q000209:etiology / Q000517:prevention & control / Q000601:surgery*; D013997:Time Factors; D016896:Treatment Outcome