Hypertelorism is a congenital pathology, which consists in an abnormally large distance between paired organs. Often there is visual or ocular hypertelorism - in which the distance between the bone structures of the orbit is significantly increased in comparison with the norm. Often, the anomaly is not a separate disease, but is part of some genetic pathologies, for example, hypertelorism is often found in Edwards syndrome, neurofibromatosis.
- 1Definition of disease
- 4Possible complications
Definition of disease
According to statistics,orbital hypertelorism is the most common deformity of the skull, which affects not only the bony, but also the soft facial structures.The disease began to study relatively recently, the first descriptions of a similar anomaly appeared in medical literature in 1924. Since that time, pathology has been treated separately, differentiating it from a similar disease - a telecanthus, which has similar
- True. The glazes are planted widely due to the anomalously wide distance of the bony formations between them;
- False hypertelorism or epicanthus -this distance between the medial angles of the eyes is much higher than normal, but there is no change directly in the bones of the skull.
The development of pathology is associated with the excessive growth of small wings of the sphenoid bone in the embryonic period. Because of this, there is a characteristic distance between the eyes, the nose is much wider than the norm, and the back of the nose is flat.
Depending on the external features, two forms of a similar anomaly are distinguished:
- Symmetric. There are no other manifestations other than the pathological distance between the eyes;
- Asymmetric. The eyes are located at an excessive distance from each other, and there are differences in their position in height, can be expressed strabismus.
Hypertelorism is not defined as a single disease and is a characteristic feature of more than 130 diseases and syndromes.
Hypertelorism is an innate pathology.Pathology is formed during the approach of the eye sockets during embryonic development of the fetus. It is believed that the violation is triggered by exposure to the mother's organism of pathogenic factors, for example, excessive alcohol, toxic substances, some hormonal drugs, as well as due to the transferred infectious diseases. All this can lead to the following congenital problems, in which there is a possibility of development of orbital hypertelorism:
- Craniostenosis or earlier infection of the cranial suture;
- Cranial cerebral hernia and cleft;
- Various craniofacial vascular malformations;
- Other conditions in which the normal formation of the cranium does not occur - tumors, changes in bones after trauma.
In recent years, the occurrence of ocular hypertelorism due to genetic pathology has increased, which can occur even if all the prescriptions are observed during pregnancy. For example, the increased distance between the eyes is almost always diagnosed in Down's syndrome.
Hypertelorism can be easily determined by visual inspection.Always there are signs characteristic for the disease, the severity of which may differ depending on the degree of development of pathology.
- Excessive distance between the medial gaps of the eyes;
- It is possible to deform the eye slits - they may have an irregular shape, be too wide or narrow;
- Abnormal structure of the nose- too wide nose bridge and flat back;
- Multidirectional movements of eyeballs;
- There is an inherent strabismus,deformation of the optic nerve and other ophthalmic diseases.
In most cases, hypertelorism is accompanied by impaired intelligence, underdevelopment of the jaw apparatus, abnormal development of the feet.
In rare cases, orbital hypertelorism can lead to serious complications.Usually this formation of craniocerebral hernia, the development of any form of cranial dysplasia. If you do not perform surgery in time, then in due course ophthalmic diseases may develop - acquired strabismus, optic nerve atrophy, increased intraocular pressure. Ocular hypertelorism is also a risk factor for reducing visual acuity and developing glaucoma.
To date, the only method of treatment is surgical plastic surgery.The appointment of a surgical intervention is possible starting from two years. In this case, the methods and technology of the procedure completely depend on the severity of the pathology and the degree of development of ophthalmic and neurologic symptoms. As a rule, an early operation helps prevent complications and reduces the risk of social pressure.
Conservative treatment is necessary only in cases where orbital hypertelorism is accompanied by various complications.
Usually prescribe drugs to reduce intraocular pressure, moisturizing drops, as well as antispasmodics to prevent pain caused by disruption of the location of the eyeball.
There are no effective ways to prevent the development of this serious pathology.As a preventive measure of fetal development disorders, it is necessary to adhere to all the recommendations and prescriptions during the entire pregnancy period. Of great importance is the observance of sleep and rest, the rejection of toxic, narcotic and hormonal substances, as well as balanced nutrition. Beginning with the first trimester, it is necessary to undergo regular medical examinations in time to notice the presence of abnormal development of the craniofacial apparatus..
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Ocular hypertelorism is a common symptom that is characteristic of many genetic diseases. Due to the fact that there are no effective ways of treating and preventing the disease, it is necessary to approach the pregnancy responsibly starting from the planning stage. And if the pathology still could not be avoided, it is necessary to raise the question of surgical correction as soon as possible, which allows reducing the risk of complications and facilitating the social adaptation of the child.
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