Outcomes from 18 years of cervical spine surgery in MPS IVA: a single centre’s experience
This study examines the long-term outcomes of paediatric Morquio (MPS IVA) patients undergoing cervical spine surgery and evaluates the factors that impacting this.
A retrospective review was performed on all MPS IVA patients undergoing cervical spine surgery, since the introduction of standardised neuroradiological screening. The impact of preoperative neurological status, growth, genotype and radiological status on outcome is assessed, whilst long-term surgical, radiological and neurological outcomes are documented.
Twenty-six of the eighty-two MPS IVA patients (31%) reviewed underwent cervical spine surgery at a median age of 6.1 years (range, 1.45 to 15.24). Preoperatively, cord signal change was seen in 11 patients with 5 being myelopathic; however, 6 clinically manifesting patients had no overt cord signal change. Postoperatively, none of the 14 preoperatively clinically asymptomatic patients followed long term progressed neurologically during a median follow-up of 77.5 months (range = 18–161). Of the ten preoperatively clinically symptomatic patients who were followed up for the same duration, seven continued to deteriorate, two initially improved and one remained stable. Radiological follow-up performed for a median duration of 7 years (range = 0.5–16) has shown a degree of stenosis at the level immediately caudal to the termination of the graft in 76% of patients, though only one has become clinically symptomatic and required revision.
Once clinically elicitable neurological signs become evident in patients with MPS IVA, they tend to progress despite surgical intervention. Referring clinicians should also not be falsely reassured by the lack of T2 spinal cord signal change but should consider surgical intervention in the face of new clinical symptomology or radiological signs of progressive canal stenosis or instability.
KeywordsMorquio syndrome Dysostosis multiplex Cervical spine Magnetic resonance imaging Myelopathy
The authors would like to acknowledge both Professor JE Wraith and Mr. R Cowie, who between them cared for and operated on the majority of these patients and whose dedication underlies the majority of this manuscript. We would also like to acknowledge the contribution of Mr. J Thorne whose work and advice was integral to many patients on this paper.
Compliance with ethical standards
Conflicts of interest
Dr. Broomfield, Mr. Zuberi, Miss Mercer, Miss Moss and Dr. Jones have received financial support for attending symposia from Biomarin. The other authors declare that they have no conflict of interest.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. As this is a retrospective study, formal consent is not required.
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