Multicentre approach to epidemiological aspects of craniosynostosis in Germany

Publication date: Available online 22 October 2018

Source: British Journal of Oral and Maxillofacial Surgery

Author(s): C. Neusel, D. Class, A.W. Eckert, R. Firsching, P. Göbel, D. Götz, R. Haase, G. Jorch, A. Köhn, S. Kropf, L. Patzer, I. Schanze, C. Zahl, A. Rissmann

Abstract

We know of no current published data on the prevalence of craniosynostosis in Germany, so our objective in this study was to contribute to the limited knowledge of its epidemiology by assessing time trends, the frequency of prenatal diagnosis, and the timing of diagnosis and treatment. Data were collected in Saxony-Anhalt during the period 2000–17, and we designed a retrospective multicentre cohort study. The prevalence was 4.8 cases of craniosynostosis/10 000 births, and did not increase during that time. We compared the data of 91 patients with those of 273 controls. There were 75 boys and 16 girls (ratio 4.7:1). Fifty-one children had isolated craniosynostosis, consisting of 46 with a single-suture, and five with a multisuture, synostosis. Twenty-nine were associated with other congenital malformations, and 11 were syndromic. Three cases had been diagnosed prenatally, and 34 had skull deformities diagnosed immediately after birth at a mean (SD) age of 3.4 (4.7) months. The mean (SD) age at the time of first admission to hospital in one of the three surgical centres of Saxony-Anhalt was 5.9 (5.5) months, and 65 patients were operated on at a mean age of 9.1 (6.3) months. In contrast to published reports we found a prevalence of 4.8 cases of craniosynostosis/10 000 births that did not increase during the period 2000–16. Although we found a low prenatal detection rate, the diagnosis and treatment in this cohort study seemed timely.

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