Aim: To determine that the aetiology of cord compression in mucopolysaccharidoses (MPS) type VI. To illustrate the variability of this complication of mucopolysaccharidoses even within families. To report the youngest MPS VI patient yet described with spinal cord compression and to present the technique and results of spinal stabilisation.

Method: The course, clinical findings and management of three patients with MPS VI and two with MPS IV were reviewed.

Results: The patients with MPS VI demonstrated that the pathogenesis of spinal cord compression in this condition is complex, with elements of joint instability, bony disease and soft tissue compression. Two of the patients with MPS VI are siblings: the younger sibling was 30 months old when she required surgery. She is the youngest reported patient with this complication of MPS VI. The patients with MPS IV are presented to illustrate similarities and differences in the pathogenesis of the same problem in the two disorders. Results of cervical spine stabilisation were found to be satisfactory.

Conclusions: In both MPS IV and MPS VI spinal cord compression may be multi-factorial. This complication of the mucopolysaccharidoses needs to be considered even when the patient is very young.