Anoftalm is the oftalmopatologiya which is characterized by lack of an eyeball in an orbit. Clinical displays of a disease – irreversible loss of visual functions on the party of defeat, narrowing of borders of the visual field, violation of spatial perception and bystry fatigue when performing visual work as a healthy eye. Diagnostics includes external examination, eye biomicroscopy, an ultrasonic and patomorfologichesky research. Step prosthetics is the cornerstone of specific treatment. Instillations of antiseptics are in addition shown. Surgical correction of cosmetic defects is carried out according to individual indications.
Anoftalm – often found oftalmopatologiya. According to statistical data, prevalence of a congenital form makes 1-2,1 on 10 thousand population. At mature age this indicator fluctuates from 21 to 22,3 on 10 thousand as within every year the patient carries out about 12 000 enukleation. For patients of children's age the disease is diagnosed in 0,4% of cases. Removal of an eyeball – a compulsory measure at 5-12% of patients with a severe injury of eyes in the anamnesis. Today the number of the patients living in the territory of Russia and needing eye prosthetics increased up to 300 thousand people.
Reasons of an anoftalm
Anoftalm carry to number of polietiologichesky diseases. The starting factor of development of a congenital form in most cases precisely does not manage to be established. As the main reasons for emergence of the congenital and acquired forms of pathology are considered:
- Pre-natal infections. The Anoftalmichesky syndrome can be a consequence of action of viruses of measles, rubella or the surrounding herpes at an embryogenesis stage.
- Amniotic tyazh. A syndrome of amniotic banners – the reason of multiple pathologies of an organ of vision. Depending on on what term of a gestation they were formed, develops or easier forms of damage of eyes (a gipertelorizm, a strabizm, ).
- Genetic syndromes. Anoftalm often arises in total with multiple anomalies of development against the background of chromosomal pathologies (Patau, Lenz, Lokhmann's syndromes) or mutations of genes of PAX6, SOX2, OTX2 and VSX2.
- Action of teratogenny factors. Teratogenny action concerning an organ of vision ionizing radiation, the use of alcoholic drinks, some pharmacological medicines, drugs possesses.
- Traumatic damages. Can lead home or production accidents to development of an anoftalm. Resort to an enukleation because of emergence of a post-traumatic atrophy, a chronic uveit or high risk of accession of a sympathetic oftalmiya.
- Oncological diseases. Removal of an eyeball can be made in the presence of a malignant tumor.
Congenital anomaly of development of an eyeball arises at action of teratogenny factors on 2-6 week of an embryogenesis. In this period process of laying of an organ of vision is broken. At defect of a structure of rudiments process of formation of an eye bubble and its further transition to an eye glass is impossible. If influence of pathogenic factors falls on the moment of already educated eye glass, then in an orbital cavity it is possible to find its low-differentiated rudiment. Anoftalm can be early manifestation of the genetic syndrome caused by a gene or chromosomal mutation. The acquired pathology develops as a result of massive injury or surgical removal of an eyeball.
classify by emergence time on congenital and acquired. It is possible one - and bilateral option. This type of systematization is used at a diagnosis stage. From the clinical point of view in practical ophthalmology allocate the following forms of pathology:
- True. The disease is combined with lack of an external cranked body, a hiazma, an optic nerve and its channel. The true type happens exclusively congenital and often meets along with other malformations.
- Imaginary. Total absence of an eye comes to light. The normal anatomo-physiological structure is characteristic of other structures of an orbit. Radiographic methods of diagnostics allow to visualize the channel of an optic nerve.
Symptoms of an anoftalm
Anoftalm is shown by irreversible loss of visual functions. At a unilateral form of a disease visual acuity of other eye can meet standard. Loss of binocular sight leads to narrowing of borders of visual fields, violation of spatial perception. Patients show the astenopichesky complaints connected with bystry exhaustion of the akkomodatsionny device. Adaptation to monocular deep sight takes place in young age better. When carrying out an enukleation in connection with a blindness adaptation occurs in process of progressing of visual dysfunction. The combination of an anoftalm to defeat of the nervous device results in sharp morbidity in an eye-socket projection zone with irradiation in a nape.
Lack of timely step prosthetics at a congenital form of an anoftalm leads to asymmetry of the person. On the party of defeat omission of an eyebrow is noted, pripodnimany covered a nose and a corner of a mouth. In process of growth of the child expressiveness of deformation increases that involves malfunction of breath and the act of chewing. Existence in a cavity of an eye-socket of a rudiment of an eyeball of low extent of differentiation often is the reason of accession of complications of the infectious and inflammatory nature. In a row a case retrobulbarny abscess or phlegmon of an orbit develops.
Visually lack of an eye in an orbit cavity is defined. The shape of a conjunctival cavity is brought closer to conic, reduced in a size. Narrowing of an eye crack is combined with an internal epikantus, entropiony or total absence of a fold of an upper eyelid. Tool diagnostics includes:
- Eye biomicroscopy. The research allows to reveal disproportionality of the conjunctival arches, the narrowed eye crack, the wrong situation a century, multiple hems and solderings.
- Brain KT. KT is applied to differential diagnostics between an imaginary and true form of an anoftalm and mikroftalmy. At an imaginary form the channel of an optical nerve is visualized. Distinctive feature of a mikroftalm – existence of the low-differentiated rudiment in an orbital cavity.
- Ultrasonography of an eye. Use of a technique is recommended for assessment of a condition of a cavity of an orbit, definition of an ekhogennost of contents of an eye-socket.
- Patomorfologichesky research. The method gives the chance to the ophthalmologist to establish degree of a reduction of an eye and its correlation with an embryogenesis stage.
Consultation of the geneticist as can act as early display of genetic pathology is shown to all patients with a congenital form of a disease. Accession of neurologic symptomatology, morbidity and asymmetry persons demand inspection from the neurologist.
Treatment of an anoftalm
It is impossible to restore visual acuity at an anoftalma. The treatment purpose – to eliminate cosmetic defect and to prevent complications. Surgical tactics comes down to step prosthetics which needs to be carried out at early children's age with gradual replacement by an artificial limb of bigger diameter. Eye prosthetics is carried out in several stages:
- Preparation. Radical intervention – an enukleation or removal of a rudiment of an eye glass is shown. At the expressed defect of walls of an eye-socket surgical plasticity is carried out them.
- Individual prosthetics. Development of an artificial limb taking into account the color and relief characteristics suitable the patient.
Conservative therapy at an anoftalma comes down to daily instillations of solutions of anti-septic tanks. At the expressed defects the century is carried out expeditious correction of a lagoftalm, entropion. Implementation of a kantotomiya is justified only after achievement of 18 years by the patient as earlier intervention becomes complicated entropiony.
Forecast and prevention
The principle of step prosthetics in treatment of patients with anoftalmy allows to eliminate cosmetic defects and to prevent formation of bone deformations of front department of a skull. It is impossible to restore visual functions at this pathology, however the disease does not pose threat for the patient's life. Specific preventive measures it is not developed. Nonspecific prevention of the acquired form comes down to observance of safety measures on production and in life. For decrease in risk of development of a congenital anoftalm it is necessary to avoid teratogenny influence of various factors from 2 to 6 week of pregnancy.