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 Agree | Agree | Neutral | Disagree | Strongly Disagree | |
|---|---|---|---|---|---|
1.Spinal disorders are common in skeletal dysplasia. | 8 (80%) | 2 (20%) | 0 | 0 | 0 |
2.Spinal disorders can have an infantile onset (age 0–3 years) and are often progressive in nature. | 7 (70%) | 3 (30%) | 0 | 0 | 0 |
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%) | 0 | 0 | 0 | 0 |
5.Clinical evidence of myelopathy requires urgent evaluation and management. | 9 (90%) | 1 (10%) | 0 | 0 | 0 |
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%) | 0 | 0 | 0 |
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%) | 0 | 0 | 0 |
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%) | 0 | 0 | 0 |
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%) | 0 | 0 | 0 |
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%) | 0 | 0 | 0 |
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%) | 0 | 0 | 0 |
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%) | 0 | 0 | 0 | 0 |
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%) | 0 | 0 | 0 |
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%) | 0 | 0 | 0 |
15.Stenosis may occur at any level in the cervical spine in skeletal dysplasia. | 9 (90%) | 1 (10%) | 0 | 0 | 0 |
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%) | 0 | 0 | 0 |
17.Upper thoracic kyphosis occurs in skeletal dysplasia and can be associated with spinal cord injury during procedures requiring anesthesia. | 6 (60%) | 4 (40%) | 0 | 0 | 0 |
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%) | 0 | 0 | 0 |
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%) | 0 | 0 | 0 |
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%) | 0 | 0 | 0 |
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%) | 0 | 0 | 0 |
22.Respiratory function should be monitored in patients with thoracic spinal deformity. | 3 (30%) | 7 (70%) | 0 | 0 | 0 |
23.Brace or cast treatment in skeletal dysplasia is appropriate in young patients with progressive, flexible scoliosis. | 4 (40%) | 6 (60%) | 0 | 0 | 0 |
24.Early-onset scoliosis occurs in skeletal dysplasia and can be managed with surgical techniques that preserve spine growth. | 6 (60%) | 4 (40%) | 0 | 0 | 0 |
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%) | 0 | 0 | 0 |
26.Advanced imaging is strongly recommended prior to surgical instrumentation of the spine in skeletal dysplasia. | 8 (80%) | 2 (20%) | 0 | 0 | 0 |
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%) | 0 | 0 | 0 |
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%) | 0 | 0 | 0 |
29.Surgical decompression should be accompanied by instrumented fusion in skeletally immature patients with achondroplasia and progressive symptomatic spinal stenosis. | 4 (40%) | 6 (60%) | 0 | 0 | 0 |
30.In hypochondroplasia, symptomatic spinal stenosis can occur and should be monitored. | 6 (60%) | 4 (40%) | 0 | 0 | 0 |
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%) | 0 | 0 |