Standalone cranio-cervical decompression is feasible in children with mucopolysaccharidosis type I, IVA and VI

Publication date: Available online 9 April 2018
Source:The Spine Journal
Author(s): Harald Krenzlin, Ta-Chih Tan, Christina Lampe, Christian Lampe, Markus Knuf, Peter Horn, Manfred Schwarz
Background contextIn patients with Mucopolysaccharidosis (MPS), glycosaminoglycans deposits in the dura mater and supporting ligaments cause spinal cord compression and consecutive myelopathy, predominantly of the cranio-cervical junction. Disease characteristics of cranio-cervical stenosis (CCS) in MPS patients differ profoundly from other hereditary and degenerative forms.Due to high peri-procedural morbidity and mortality, MPS patients pose a substantial challenge to the unexperienced medical care provider. As literature remains scarce, we present our experience with a large cohort of MPS patients treated for CCS without atlanto-occiptal instrumentation.PurposeTo describe a save and least traumatic approach for treating CCS in children with MPS, avoiding primary instrumentation.Study designThis is a prospective follow-up (cohort) study.Patient samplesWe report 15 consecutive patients with CCS related to MPS, which were treated with standalone cervical decompression.Outcome measuresMyelopathy was assessed using MRI, somatosensory evoked potentials and clinical evaluation. Cervical instability was evaluated using plain x-ray and MRI. The disability status is quantified using either the Karnofsky- or Lansky Performance score.MethodsWe describe 15 consecutive patients treated with cranio-cervical decompression. Data was collected prospectively. The mean follow-up is 6 years (SD 5). The technique and treatment principles are described.ResultsThe overall clinical outcome in this patient cohort is good (mean Karnofsky Performance Score 80). No patient developed signs of C0-C1-C2 instability or progressive myelopathy. Re-stenosis occurred in 7 patients requiring a total of 8 re-operations.ConclusionsSurgery in patients with MPS is associated with high morbidity and a mortality of up to 4.2 %. Due to the unique nature of the disease, recurring stenosis is inevitable. In order to shorten the procedure time and simplify the anticipated re-operation, we provide data that cranio-cervical decompression is feasible without the necessity of primary osteosynthesis. In the absence of cranio-cervical instability, decompression surgery without occipito-cervical stabilisation yields good postoperative results and challenges the long-standing paradigm of prophylactic cranio-cervical fixation.



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