Abstract
BackgroundAlthough the clinical efficacy of laminoplasty in adult cervical spondylotic myelopathy or ossification of posterior longitudinal ligament has been frequently reported, there are only few reports of laminoplasty for patients with lysosome storage diseases (LSDs). Therefore, this study aimed to report the midterm clinical and radiological outcomes of patients with LSDs after cervical laminoplasty.MethodsSix patients with LSD who underwent laminoplasty with/without C1 laminectomy for cervical myelopathy were enrolled. Clinical evaluations, including the cervical Japanese Orthopedic Association (cJOA) score and visual analog scale (VAS) scores for upper extremity numbness, and radiographic parameters, including C2–C7 lordotic angle, atlanto-dens interval (ADI), and ⊿ADI, were evaluated preoperatively, at 2 years postoperatively, and at the final follow-up.ResultsFive patients had mucopolysaccharidoses (type I: n = 1, II: n = 3, VII: n = 1) and one patient had mucolipidoses type III. The mean age of patients at surgery was 27.5 years, and the mean postoperative follow-up period was 61 months. All mucopolysaccharidoses cases required C1 posterior arch resection with C2–C7 laminoplasty. No critical complications were observed postoperatively. There were no significant differences in C2–C7 angle (p = 0.724) and ⊿ADI (p = 0.592) between the preoperative and final follow-ups. The cJOA score and VAS for numbness significantly improved at the final follow-up (p = 0.004 and p = 0.007, respectively).ConclusionsThe cervical myelopathy in patients with LSD could be safely and effectively treated with laminoplasty with/without C1 posterior arch resection after excluding patients with atlantoaxial instability. Atlantoaxial stability and symptom improvement could be maintained at an average of 5 years postoperatively.
Highlights
The clinical efficacy of laminoplasty in adult cervical spondylotic myelopathy or ossification of posterior longitudinal ligament has been frequently reported, there are only few reports of laminoplasty for patients with lysosome storage diseases (LSDs)
All patients with MPS required C1 posterior arch resection accompanied by C2-C7 laminoplasty, whereas the patient with ML was treated with C2-C7 laminoplasty without C1 posterior arch resection
Cervical range of motion (ROM) significantly decreased from 58.3°±13.7° preoperatively to 35.5° ± 10.7° at the final follow-up (p = 0.003)
Summary
The clinical efficacy of laminoplasty in adult cervical spondylotic myelopathy or ossification of posterior longitudinal ligament has been frequently reported, there are only few reports of laminoplasty for patients with lysosome storage diseases (LSDs). This study aimed to report the midterm clinical and radiological outcomes of patients with LSDs after cervical laminoplasty. Mucopolysaccharidoses (MPS) and mucolipidoses (ML) comprise a group of rare inherited lysosomal storage diseases (LSDs). The incidence of MPS is 1.53 per 100,000 live births, that of overall LSDs is one per 7000 individuals [1,2,3]. The accumulation of undegraded cholesterol, phospholipid, and. Terai et al Orphanet J Rare Dis (2021) 16:401 Patient no. Medical therapy cJOA score (points) VAS score (mm).
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