Definition
Definition
Craniosynostosis is a disorder of skull ossification in young children, characterized by the premature closure, by early fusion, of one or more sutures of the cranial vault.
Symptoms
Symptoms
Symptoms of craniosynostosis are:
- Visual disturbances.
- Exophthalmos (protrusion of the eyeballs).
- Headaches (cephalgia).
Pathophysiology
Craniosynostosis causes developmental arrest and underlying deformations of various parts of the skull, resulting in:
- A acrocephaly : congenital malformation of the skull also called oxycephaly, with very high development in height and flattened sides. The top of the skull can be flat, or pointed. It can be associated with other malformations, and sometimes leads to mental retardation. In this case, the forehead is high and the skull is rounded. This is a condition more common among North Africans.
- La trigonocephaly : cone-shaped skull. It is due to premature welding of the metopic suture (suture located between the two bones making up the front part of the skull, which is the frontal bone). The width of the forehead is reduced, with a median protrusion (in the middle) which gives the skull a triangular shape, the eyes are close together, and the face is narrow. This is a condition more common in boys, and very often associated with other malformations.
- La plagiocephaly : closing on one side of a coronal suture, which is the suture located between the frontal bone in front, and the parietal bone above the skull.
- La scaphocephaly : particularly frequent malformation due to premature closure of the sagittal suture (the suture located between the two parts of the parietal bone).
- La brachycephaly is secondary to early fusion of the coronal suture. This malformation can be observed in several members of the same family. A high paternal age has been found in a few cases, which supports the hypothesis of a dominant gene (a genetic anomaly carried by only one of the parents is sufficient to cause the disorder in the child).
Craniosynostosis can be discovered at any age and under any circumstances. Anatomical changes may be visible at birth or may become apparent soon afterward.
Cause
Cause
The main cause of craniosynostosis is genetic, probably through autosomal dominant transmission (it is enough for one of the two parents to carry the genetic anomaly on a non-sex chromosome for the child to have the anomaly). It is mainly encountered during:
- From Crouzon disease
- Apert's acrocephalopolysyndactyly.
- Saethre-Chotzen acrocephalopolysyndactyly.
- Acrocephalopolysyndactyly Peiffer.
- Antley-Bixler acrocephalopolysyndactyly.
- Baller-Gérold acrocephalopolysyndactyly.
- From Opitz acrocephalopolysyndactyly.
In these latter syndromes, it is associated with other bone malformations.
Treatment
Treatment
Treatment Distraction: A surgical technique used in maxillofacial surgery. Its aim is to make the body, and more specifically the skull, produce additional bone using a metal device consisting of a screw in a jack that allows for this gradual synthesis (1 months) of additional bone tissue between two previously sectioned parts.
Evolution
Premature
Complications It can cause eye complications, such as complete blindness. This is due to a deformation of the optic nerve (angulation) resulting from bone deformation and compression of the vessels. Mental retardation sometimes Intracranial hypertension (increased pressure inside the skull) mainly in oxycephaly and brachycephaly) which requires surgical intervention.