Background: Cranial sutures and synchondroses tend to close prematurely in patients with Crouzon syndrome. This influences their skull vault and skull base development and may involve in common disturbances such as increased intracranial pressure and cerebellar tonsillar herniation. The authors’ hypothesis was that Crouzon patients patients have a smaller foramen magnum than controls because of premature fusion of the intraoccipital synchondroses, putting them at risk for cerebellar tonsillar herniation. Therefore, foramen magnum size and time of intraoccipital synchondroses closure were evaluated and were related to the presence and degree of cerebellar tonsillar herniation.
Methods: The foramen magnum surface area and anteroposterior diameter were measured on three-dimensional computed tomographic scans of 27 Crouzon patients and 27 age-matched controls. Scans had a slice-thickness between 0.75 and 1.25 mm and were aligned in a three-dimensional reformatting platform. The t test was used to study size differences. Synchondroses were graded as described by Madeline and Elster and studied with ordinal logistic regression analysis.
Results: Crouzon patients had a smaller foramen magnum surface area (602 mm2 versus 767 mm2, p < 0.001) and anteroposterior diameter (31 mm versus 35 mm, p < 0.001) compared with controls. Differences stayed constant over time. Intraoccipital synchondroses closed 3 to 9 months earlier in Crouzon patients than in controls (p < 0.05).
Conclusions: Since intraoccipital synchondroses close earlier in Crouzon patients, from early life on their foramen magnum is smaller compared with controls. Within Crouzon patients, the presence of cerebellar tonsillar herniation could not be related to foramen magnum size.
CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.
Rotterdam, The Netherlands
From the Departments of Plastic, Reconstructive and Hand Surgery, Paediatric Radiology, Biostatistics, and Paediatric Neurosurgery, Dutch Craniofacial Center, Erasmus Medical Center Sophia Children’s Hospital.
Received for publication June 6, 2013; accepted July 1, 2013.
Disclosure: The authors have no financial interest to declare in relation to the content of this article.
Bianca F. M. Rijken, M.D., Department of Plastic, Reconstructive and Hand Surgery, Room Ee 15.91, Erasmus Medical Center Rotterdam, P.O. Box 2040, 3000 CA Rotterdam, The Netherlands, firstname.lastname@example.org