OBJECTIVE The goal of this study was to determine the rates of screening and surgery for foramen magnum
stenosis in children with
achondroplasia in a large, privately insured healthcare network. METHODS Rates of screening and surgery for foramen magnum
stenosis in children with
achondroplasia were determined using de-identified insurance claims data from a large, privately insured healthcare network of over 58 million beneficiaries across the United States between 2001 and 2014. Cases of
achondroplasia and screening and surgery claims were identified using a combination of International Classification of Diseases diagnosis codes and Current Procedural Terminology codes. American Academy of Pediatrics (
AAP) practice guidelines were used to determine screening trends. RESULTS The search yielded 3577 children age 19 years or younger with
achondroplasia. Of them, 236 met criteria for inclusion in the screening analysis. Among the screening cohort, 41.9% received some form of screening for foramen magnum
stenosis, whereas 13.9% of patients were fully and appropriately screened according to the 2005 guidelines from the
AAP. The screening rate significantly increased after the issuance of the
AAP guidelines. Among all children in the cohort, 25 underwent cervicomedullary
decompression for foramen magnum
stenosis. The incidence rate of undergoing cervicomedullary
decompression was highest in infancy (28 per 1000 patient-years) and decreased with age (5 per 1000 patient-years for all other ages combined). CONCLUSIONS Children with
achondroplasia continue to be underscreened for foramen magnum
stenosis, although screening rates have improved since the release of the 2005
AAP surveillance guidelines. The incidence of surgery was highest in infants and decreased with age.