Clinical History: None given.

Findings: Image #1 is an axial CT which demonstrates increased biparietal diameter in relationship to the AP diameter. Image #2 demonstrates rather shallow orbits. Image #3 is a 3-D reconstructed CT image, which demonstrates the increased biparietal diameter and coronal synostosis.

Diagnosis: Crouzon's Syndrome. (Coronal Synostosis)

Discussion: Crouzon's syndrome is a craniofacial abnormality consisting primarily of coronal synostosis as well as sagittal synostosis (which incidentally is not seen in this case). In addition, patients with Crouzon's syndrome often have shallow orbits producing exophthalmos, as well as maxillary hypoplasia. The coronal synostosis produces a skull, which is widened in its biparietal dimensions, while the AP diameter is decreased. With sagittal synostosis, the opposite would be true, that is, the AP diameter would be increased while the biparietal diameter would be decreased. The synostosis can be demonstrated as a straight, rather than serrated radiolucent line. Alternatively, there may be frank ossification of the suture. In addition, to coronal synostosis and sagittal synostosis, patients with Crouzon's syndrome often have lambdoid synostosis. Other associated findings include calcification of the stylohyoid ligament and deviation of the nasal septum. Hydrocephalus may occur.

References:

1. Swischuk L. Imaging of the Newborn, Infant and Young Child. Williams & Wilkins, Baltimore, Maryland; 1989:906-912.

2. Kirks D. Practical pediatric Imaging. Lippincott, Philadelphia; 1998:217-218.

3. Resnick D. Bone and Joint Imaging. W. B. Saunders, Philadelphia; 1996:1146-1147.