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Outcomes from 18 years of cervical spine surgery in MPS IVA: a single centre's experience
A Broomfield1, K Zuberi2, J Mercer3
1Willink Biochemical Genetics Unit, Manchester Centre for Genomic Medicine, St Mary's Hospital, Central Manchester Foundation Trust, Manchester, M13 9WL, UK. alexander.broomfield@cmft.nhs.uk.
Insights
Cervical spine surgery in Mucopolysaccharidosis type IVA (MPS IVA) patients shows limited neurological improvement once symptoms appear. Early surgical intervention is crucial, even without clear MRI evidence, to manage progressive spinal stenosis.
Area of Science:
- Orthopedics
- Neurology
- Genetics
Background:
- Mucopolysaccharidosis type IVA (MPS IVA) is a rare genetic disorder affecting skeletal development, particularly the cervical spine.
- Cervical spine instability and stenosis are common complications in MPS IVA, leading to potential neurological deficits.
Purpose of the Study:
- To evaluate the long-term outcomes of cervical spine surgery in pediatric patients with MPS IVA.
- To identify factors influencing surgical outcomes in this patient population.
Main Methods:
- Retrospective review of MPS IVA patients who underwent cervical spine surgery.
- Assessment of preoperative neurological status, growth, genotype, and radiological findings.
- Documentation of long-term surgical, radiological, and neurological outcomes.
Main Results:
- 31% of MPS IVA patients underwent cervical spine surgery, with a median age of 6.1 years.
- Preoperative myelopathy was present in 5 patients; 6 symptomatic patients lacked overt cord signal change on MRI.
- No significant neurological progression occurred in asymptomatic patients post-surgery, while 70% of symptomatic patients continued to deteriorate.
Conclusions:
- Neurological deterioration tends to progress in MPS IVA patients with evident neurological signs, even after surgical intervention.
- Lack of T2 spinal cord signal change on MRI should not preclude surgical consideration for symptomatic patients or those with progressive stenosis/instability.
Purpose:
This study examines the long-term outcomes of paediatric Morquio (MPS IVA) patients undergoing cervical spine surgery and evaluates the factors that impacting this.
Methods:
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.
Results:
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.
Conclusions:
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.
