Learning objectives
Our purposes are:
- to understand the complex relationships between the premature synostosis of the cranial sutures and craniofacial deformities in Patients with syndromic craniosysnostosis;
- to describe the normal sutures anatomy,
showing that the synostotic process is due not only to involvement of vault sutures but also to involvement of “minor” sutures of skull base.
We will assess the following steps:
- definition and classification of craniosynostosis;
- radiological assessment;
- normal sutures anatomy;
- skull and cranio-facial deformity;
- clinical presentation;
- surgical...
Background
- DEFINITION OF CRANIOSYNOSTOSIS
Craniosynostosis is the process of premature sutural closure,
taking place during a period of continuing brain growth; craniostenosis is the result of this process.
The premature fusion of the suture inhibits skull growth perpendicular to the affected sutures,
leading to skull base asymmetry,
cranio-facial asymmetry and limitation of brain growth.
Virchow in 1851 was the first to report the pathophysiology of craniosynostosis.
He recognized that ‘bony expansion ceases in a direction perpendicular to the synostosed suture,
with compensatory expansion in the...
Imaging findings OR Procedure details
-SKULL AND CRANIO-FACIAL DEFORMITY
Morphometric analysis of skull base and anatomic study of facial skeleton.
3D CT scanning is used to perform morphometric analysis of skull base and for evaluating facial skeleton deformity.
We evaluated the skull base asymmetry and the craniofacial deformities on the basis of the following measurements:
the direction and the angle of skull’s endocranial base deflection;
the angles between the petrosal pyramids and the midline (posterior petrosal sagittal angle: PPSA);
ethmoidal axis deviation;
“minor” sutures involvement;
nasal root deviation;
orbit height...
Conclusion
Premature fusion of cranial sutures in infants leads to profound changes in craniofacial shape and in skull base simmetry due to a close relationship between calvarium,
skull base and facial complex development.
HRCTwith bone definition algorithms and three-dimensional reconstruction is an excellent technique to identify premature sutural closure,
to evaluate cranio-facial deformities and to perform a morphometric analysis of skull base,
while MR is “gold standard” to detect parenchymal abnormalities.
In literature,
classifications of craniosynostosis are based on the involvement of the main skull sutures;...
References
Francel P.C.,
Park T.
S.,
Marsh J.
L.,
et al. Frontal plagiocephaly secondary to synostosis of the frontosphenoidal suture. J Neurosurg 83: 733-736,
1995.
Rogers G.
F.,
Proctor M.
R.,
Mulliken J.
B. Unilateral fusion of the frontosphenoidal suture: a rare case of synostotic frontal plagiocephaly.
Plast recostr surg 110: 1011-1021,
September 2002.
Dundulis J.
A.,
Becker D.
B.,
Govier D.
P.,
et al,
Coronal ring involvement in Patients treated for unilateral coronal craniosynostosis.
Plast recostr surg 114: 1695-1703, December 2004.
Rogers G.
F.,
Mulliken...
Personal Information
G.
D’Apolito,
MD
Department of Radiological Sciences and Bioimaging,
Catholic University of Rome
Largo A.
Gemelli,
8 - 00168,
Rome,
Italy.
mail to:
[email protected]