Item | Guiding Principle | Vote (%) | Level of agreement (mean; range) |
---|---|---|---|
A | All infants should be monitored clinically for foramen magnum stenosis every 3–4 months from birth to the age of 1 year, thereafter every 3–6 months until the age of 3 years. After the age of 3 years, monitoring for foramen magnum stenosis should be based on individual need and local protocols | 100 | 8.4 (4–10) |
B | MRI imaging should be undertaken as routine monitoring for foramen magnum stenosis at 3–6 months of age and repeated according to findings in other routine assessments | 89 | 7.9 (2–10) |
C | Where signs of compression are observed on screening, infants should be referred as soon as possible to a neurosurgery specialist in a centre experienced in the management of achondroplasia | 97 | 7.6 (1–10) |
D | The decision to decompress the foramen magnum should be made using a combination of clinical, neurological and imaging assessments, evaluated by a neurosurgical specialist experienced in the management of achondroplasia | 93 | 8.9 (5–10) |
E | The decision to proceed to decompression of the foramen magnum should be made jointly by a neurosurgeon experienced in achondroplasia, the individual’s family and the wider multidisciplinary team | 96 | 8.4 (2–10) |
F | Older children and adults with previously undetected foramen magnum stenosis should be managed on an individual basis by the multidisciplinary team | 96 | 8.3 (5–10) |