Abstract
Crouzon syndrome is the most common form of craniofacial dysostosis, characterised
by a classical triad of abnormal skull shape, abnormal facies, and exophthalmos. The
clinically overt dental abnormalities in these patients, distracts clinicians from
the developmental neurological defects and therefore this entity remains relatively
under - highlighted in radiology literature. We report and highlight the role of imaging
in diagnosis of Crouzon syndrome in two patients, and discuss the relevant differential
diagnosis. Moreover, our report is among the few Indian studies in which Crouzon syndrome
was confirmed by genetic studies. The classical clinical triad of Crouzon syndrome
was observed in both patients. The skull radiographs and cranial CT with 3D reconstruction
VRT (Volume rendered technique), revealed characteristic radiological features. Genetic
studies reconfirmed the clinical and radiological diagnosis of Crouzon syndrome, in
both patients.
Keywords
Crouzon Syndrome - dental abnormalities - Imaging - Skull radiographs - Cranial CT
- Volume rendered technique - Genetic studies
