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Table 2 Statements that reached 80% agreement in Round 2. Final Guidelines

From: Best practice guidelines for management of spinal disorders in skeletal dysplasia

 Strongly AgreeAgreeNeutralDisagreeStrongly Disagree
1.Spinal disorders are common in skeletal dysplasia.8 (80%)2 (20%)000
2.Spinal disorders can have an infantile onset (age 0–3 years) and are often progressive in nature.7 (70%)3 (30%)000
3.Spinal cord compression and myelopathy are common manifestations of spinal disorders in skeletal dysplasia.3 (30%)7 (70%)   
4.Myelopathic findings on history and physical exam (e.g. poor balance, broad based gait, extremity weakness, upper motor neuron signs, urinary incontinence) should raise suspicion of spinal cord compression/injury in patients with skeletal dysplasia.10 (100%)0000
5.Clinical evidence of myelopathy requires urgent evaluation and management.9 (90%)1 (10%)000
6.In patients with skeletal dysplasia and “spine-at-risk”* findings, neuromonitoring should be considered for all surgical procedures to minimize the risk of spinal cord injury.3 (30%)7 (70%)000
7.Skeletal dysplasia should be considered in individuals with radiographic findings of vertebral anomalies such as platyspondyly and/or anterior vertebral body beaking.6 (60%)4 (40%)000
8.Achondroplasia or hypochondroplasia are likely diagnoses if there narrowing of the interpedicular distance in the lumbar spine (from L1 to L5) on AP radiographs.6 (60%)4 (40%)000
9.Flexion/extension plain radiographs of the cervical spine should be considered for all patients with known risk of C1-C2 instability or unclassified skeletal dysplasia.9 (90%)1 (10%)000
10.Vertebral artery and upper cervical anatomy is variable in skeletal dysplasia; therefore advanced imaging is recommended prior to upper cervical spinal surgery.6 (60%)4 (40%)000
11.Flexion-extension CT scan or MRI can be very useful adjuncts in evaluating cervical instability in patients with skeletal dysplasia.8 (80%)2 (20%)000
12.Cervical instability or evidence of significant spinal cord compression on imaging associated with myelopathic changes on physical exam should be considered for surgical management.10 (100%)0000
13.Prophylactic C1-C2 fusion for an individual at risk for cervical instability is not indicated without evidence of spinal cord compression or myelopathic changes.7 (70%)3 (30%)000
14.There are several effective techniques for stabilization of the cervical spine in patients with skeletal dysplasia. Treating surgeons should be prepared for unusual anatomy in this patient population.9 (90%)1 (10%)000
15.Stenosis may occur at any level in the cervical spine in skeletal dysplasia.9 (90%)1 (10%)000
16.Cervical kyphosis can be seen in skeletal dysplasia. Repeated evaluation is indicated as progression may occur and lead to spinal cord injury if untreated.9 (90%)1 (10%)000
17.Upper thoracic kyphosis occurs in skeletal dysplasia and can be associated with spinal cord injury during procedures requiring anesthesia.6 (60%)4 (40%)000
18.Thoracolumbar kyphosis in infants with achondroplasia improves in most cases without bracing or surgery, but prolonged unsupported sitting is discouraged.8 (80%)2 (20%)000
19.Thoracolumbar kyphosis can be seen in skeletal dysplasia. Repeated evaluation is indicated as progression may occur and lead to neurologic symptoms or back pain if untreated.9 (90%)1 (10%)000
20.Surgical stabilization of thoracolumbar kyphosis in skeletal dysplasia is appropriate for deformities that are progressive, result in neurologic compromise, or associated with back pain not responsive to non-operative interventions.7 (70%)3 (30%)000
21.Instrumented fusion with or without decompression for thoracolumbar kyphosis in skeletal dysplasia is most successful when sagittal alignment and balance are achieved.6 (60%)4 (40%)000
22.Respiratory function should be monitored in patients with thoracic spinal deformity.3 (30%)7 (70%)000
23.Brace or cast treatment in skeletal dysplasia is appropriate in young patients with progressive, flexible scoliosis.4 (40%)6 (60%)000
24.Early-onset scoliosis occurs in skeletal dysplasia and can be managed with surgical techniques that preserve spine growth.6 (60%)4 (40%)000
25.Surgical management of scoliosis and kyphosis in skeletal dysplasia is associated with a higher complication rate compared to the general population.5 (50%)5 (50%)000
26.Advanced imaging is strongly recommended prior to surgical instrumentation of the spine in skeletal dysplasia.8 (80%)2 (20%)000
27.In achondroplasia, symptomatic spinal stenosis can present in the upper and lower extremities. Symptoms and signs include decreased strength or mobility, neurogenic claudication, back and leg pain, and/or upper and lower motor neuron findings.8 (80%)2 (20%)000
28.Progressive symptoms and signs of spinal stenosis causing reduced physical function in achondroplasia should be treated surgically by decompression when appropriate non-operative measures are ineffective.3 (30%)7 (70%)000
29.Surgical decompression should be accompanied by instrumented fusion in skeletally immature patients with achondroplasia and progressive symptomatic spinal stenosis.4 (40%)6 (60%)000
30.In hypochondroplasia, symptomatic spinal stenosis can occur and should be monitored.6 (60%)4 (40%)000
31.Increased lumbar lordosis can be associated with hip flexion contractures. Realignment of the hip deformity can improve sagittal alignment of the spine.7 (70%)2 (20%)1 (10%)00