Stand-alone craniocervical decompression is feasible in children with mucopolysaccharidosis type I, IVA, and VI

Surgery in patients with MPS is associated with high morbidity and mortality of up to 4.2%. Because of the unique nature of the disease, recurring stenosis is inevitable. To shorten the procedure time and simplify the anticipated reoperation, we provide data that craniocervical decompression is feasible without the necessity of primary osteosynthesis. In the absence of craniocervical instability, decompression surgery without occipitocervical stabilization yields good postoperative results and challenges the long-standing paradigm of prophylactic craniocervical fixation.