Assessment of Epidemiological Trends in Craniosynostosis: Limitations of the Current Classification System.

Abstract

Background:Craniosynostosis affects 1 in 2,000 live births, which makes it one of the most common craniofacial abnormalities in the United States. Despite this fact, few national epidemiologic reports exist, although US and European studies have reported an increased incidence of metopic craniosynostosis. The aim of our study is to analyze the National Inpatient Sample (NIS) to support those conclusions.Methods:We identified hospitalizations from 1998 to 2012 by using the ICD-9-CM diagnosis code for congenital anomalies of skull and face bones (756.0) and procedure codes related to craniosynostosis repair (2.01, 2.03, 2.04, 2.06).Results:We analyzed data from 37,815 hospitalizations and 49,505 reconstructive procedures. There was a 61.6% increase in the number of hospitalizations related to craniosynostosis repairs. There was a 180% increase in bone graft to skull procedures, 109% increase in other cranial osteoplasty, 54% increase in formation of cranial bone flap, and a 6% decrease in opening of cranial suture.Conclusions:We observed a steady rise in the number of craniosynostosis repairs performed, but whether this is a result of a true increase in incidence, better diagnosis, or change in treatment patterns needs further research. The current classification system does not provide information about the specific suture affected (metopic, sagittal, etc.), the type of repair performed (endoscopic, fronto-orbito advancement, etc.), and whether the repair is a primary procedure or a revision. More descriptive diagnosis and procedural codes are imperative to improve the epidemiologic and outcomes data of craniosynostosis in the United States.