Occipitocervical stabilization using bilateral laminar C2 screws in children with mucopolysaccharidosis IVA

European Spine Journal : Official Publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society
Petr VanekJiri Zeman

Abstract

Mucopolysaccharidosis IVA (MPS IVA) is a multisystemic storage disorder. Patient's disability and life expectancy depends upon skeletal complications, including cervical myelopathy due to upper cervical compression or instability. Posterior decompression followed by occipitocervical fixation or C1-2 fusion are the most frequently recommended surgical interventions. The bony elements of C1 and C2 are often inadequately developed making routine screw insertion difficult. The main purpose of this work was to present novel technique of occipitocervical fixation using two C2 laminar screws. Four children with MPS IVA underwent decompression and C0-C2 instrumented fusion using two C2 bilateral laminar screws. The dimensions of the C2 lamina were measured. Clinical and radiological results were monitored prospectively for a minimum 3 years. The mean laminar length was 24 ± 1.15 mm, width 6.15 ± 0.55 mm and height 7.4 ± 0.6 mm. Patients remained in a stable neurological condition. The mean antero-posterior diameter of the spinal canal on the pre-operative MR was 6.2 ± 0.74 mm and it was enlarged to 11.4 ± 0.8 mm after 3 years. All screws were placed adequately. In all patients, the control CT scan 2 years post-operatively revealed a st...Continue Reading

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Citations

Nov 10, 2017·European Journal of Orthopaedic Surgery & Traumatology : Orthopédie Traumatologie·D ChytasV S Nikolaou
Feb 14, 2020·International Journal of Molecular Sciences·Hidetomi Terai, Hiroaki Nakamura
Jun 28, 2018·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·A BroomfieldR Ramirez
Mar 17, 2020·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·EunJi MoonJin Hoon Park
Sep 15, 2017·Journal of Children's Orthopaedics·N WilliamsD M Eastwood

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