Medical Information - Crouzon Syndrome

CROUZON syndrome is an inherited syndrome of Craniofacial Dysmorphology or abnormal craniofacial appearance, which was originally described in 1912 and is now well recognised. Children who have Crouzon syndrome have a range of problems of variable severity, from mild facial symptoms of a mainly cosmetic nature to symptoms affecting breathing, feeding, vision and brain development.

The child with Crouzon syndrome usually enters a programme of care involving many different clinical specialities, which often continues from birth to the later teenage years.

Crouzon syndrome predominantly affects the appearance of the head and face.

• regressed mid-face and shallow orbits (eye sockets), which may be present at birth or become more evident; seen from the side the face has a concave appearance and the shallow orbits result in prominent eyeballs (proptosis); the arrangement of the teeth (dentition) is also affected; and
• the abnormal skull shape may result in raised intracranial pressure and require surgery to protect the restricted brain.

Crouzon syndrome may involve any combination of cranial sutures, most commonly including the coronal and sagittal sutures.

Common terms for head shape:
Brachycephaly = flat forehead,
Scaphocephaly = a boat shaped skull,
Turricephaly = a tower shaped skull.

• View the Crouzon Syndrome information leaflet (pdf)
• Contact one of the UK National Craniofacial Units