CRANEOSINOSTOSIS DEFINICION PDF

Las uniones entre dichos huesos, formadas inicialmente por tejido conjuntivo si bien a lo largo de nuestra vida se van osificando, son las denominadas suturas y fontanelas. No es raro que se produzca microcefalia y que no se desarrollen correctamente distintas estructuras cerebrales. En definitiva se trata de un trastorno que puede provocar severos problemas para el menor y su correcto desarrollo. Tipos de craneosinostosis Dependiendo de los huesos que se suturen prematuramente, podemos encontrar diversos tipos de craneosinostosis. Se produce cuando la sutura sagital se cierra de manera temprana. Plagiocefalia Este tipo de craneosinostosis es la que se produce cuando la sutura coronal se cierra prematuramente.

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Head of plagiocephalic baby viewed from above Kinds of craniosynostosis Children born with craniosynostosis have a distinct phenotype , i. A facial feature of metopic synostosis is hypotelorism ; in the frontal view, it can be seen that the width between the eyes is smaller than usual.

Plagiocephaly can be subclassified as anterior plagiocephaly or posterior plagiocephaly. Anterior plagiocephaly[ edit ] Anterior plagiocephaly is a clinical description of unilateral coronal synostosis.

This fact immediately raises an important point. Unlike closure of the sagittal or the metopic suture, right and left are not the same in unilateral coronal synostosis. Growth is arrested in the plane perpendicular to the fused suture and the forehead is flattened, but only at the ipsilateral side of the head. Compensatory growth occurs in a parallel plane, as well as in a perpendicular plane. Assessment of the skull from a top view shows asymmetry of the frontal bones, an increased width of the skull and a forward displacement of the ear at the ipsilateral side of the head.

The difference is that this time, the deformity mostly shows at the occiput. This growth pattern exerts an effect at the base of the skull, which is not even when the child is assessed from a point of view standing behind the child, as well as on the cervical spine, which shows a curvature.

The appearance can be the same as that seen with primary microcephaly : a markedly small head, but with normal proportions. Other craniosynostosis types and their signs[ edit ] Apert syndrome : an abnormal skull shape, small upper jaw, and fusion of the fingers and toes.

Crouzon syndrome : A craniofacial birth abnormalities with bilateral coronal suture fusion. Anterior and posterior of skull shortness, flat cheek bones and a flat nose are their features. Pfeiffer syndrome : abnormalities of the skull, hands, and feet wide-set, bulging eyes, an underdeveloped upper jaw, beaked nose.

Saethre—Chotzen syndrome : short or broad head. This is especially true in the cases with syndromic craniosynostosis. Findings include elevation of the intracranial pressure; obstructive sleep apnea OSA ; abnormalities in the skull base and neurobehavioral impairment.

Elevated intracranial pressure[ edit ] When the ICP is elevated the following symptomes may occur: vomiting, visual disturbance, bulging of the anterior fontanel, altered mental status, papilledema and headache. Other symptoms can be difficulty in breathing, snoring, day-time sleepiness and perspiration.

Other factors, such as a micrognathism and adenoid hypertrophy , are likely to contribute in causing OSA. Hydrocephalus is seen in 6. Some evidence for this statement has been provided by studies using computed tomographic CT scans and magnetic resonance imaging MRI to identify differences between the structures of the brains of healthy children and those affected with craniosynostosis.

Multiple potential causes of premature suture closure have been identified, such as the several genetic mutations that are associated with syndromic craniosynostosis. Brain structures of children with craniosynostosis were evaluated using magnetic resonance imaging. Biomechanical factors[ edit ] Biomechanical factors include fetal head constraint during pregnancy. These last two are both important factors influencing bone development.

Several research groups have found evidence that these environmental factors are responsible for an increase in the risk of craniosynostosis, likely through effects on fibroblast growth factor receptor genes. Moloney et al. Cranial sutures[ edit ] Fontanelles and sutures as seen from above and to the left of the cranium of a child.

The mesenchyme above the meninges undergoes intramembranous ossification forming the neurocranium. The three main elements of analysis include medical history, physical examination and radiographic analysis. Medical history should in any case include questions about risk factors during pregnancy, the familial rate and the presence of symptoms of elevated intracranial pressure ICP.

Elevated ICP[ edit ] Symptoms of increased intracranial pressure — such as headache and vomiting — should be questioned after. Physical examination[ edit ] Fundoscopy should always be performed in children with craniosynostosis. The goal is to assess the shape of the forehead, the skull length, the width of the skull, position of the ears and the symmetry of the frontal bones and [occiput].

The second is looking at the patient from behind while preferably the child is in the same position as described above. It is important to look at the skull base to determine whether it is level or not , the position of the ears and to the mastoid to spot the possible presence of a bulge. The third point of view is the frontal view. The points to look at are: eye position, eye symmetry and twisting of the nasal tip. Syndromal craniosynostosis presents with a skull deformity as well as deformities affecting other parts of the body.

For example, one can consider the number of closed sutures. This will be further discussed under phenotype. A third classification involves the presence or absence of an identified craniofacial syndrome. More than identified syndromes show deformations due to craniosynostosis.

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