Type:
Educational Exhibit
Keywords:
Developmental disease, Congenital, Education, Diagnostic procedure, MR, CT, Conventional radiography, Paediatric, Head and neck, Bones
Authors:
A. Martin, D. Petruneac; Targu-Mures/RO
DOI:
10.1594/ecr2018/C-2331
Background
Virchow introduced in 1851 the term craniosynostosis to describe a variety of abnormalities in calvarial growth,
resulting in skull deformity.
Craniosynostosis or craniostenosis,
are the premature fusion of one or more of the cranial sutures,
restricting cranial growth perpendicular to the affected suture with compensatory overgrowth along the other patent sutures.
The clinical outcome varies between minor cosmetic deformity to severe head growth restriction with increased intracranial pressure,
mental retardation and cranial palsies. The cranial vault surrounds and encloses the brain,
and is formed from several plates of bone which meet at sutural joints,
unique to the skull.
The brain has usually reached adult size by the age of 7 years but the sutures normally persist long after this.
In neonates they appear wide open and fuse with age in an interval ranging from 9 months to 30-40 years of age.
The classification of skull deformities in craniosynostoses is based on the involved suture: metopic,
sagittal,
coronal,
lambdoid,
squamosal,
transverse or occipital.
Craniosynostoses are usually classified into syndromic craniosynostoses when they are associated with other abnormalities (as Crouzon,
Apert,
and Pfeiffer syndromes) and non-syndromic craniosynostoses when they are isolated.
However 85% of craniosynostoses are isolated,
only 15% are syndromic.