Polydactyly: the causes and symptoms, inheritance, treatment
Genetic disorder polydactyly of the limbs
A disease such as polydactyly, observed rarely. This term is understood an increase in the number of fingers.
This violation is not hazardous to life or health, but gives a person significant psychological discomfort.
To avoid this, it is necessary to conduct an operation to remove extension of the fingers.
General information about the disease
Polydactyly is the anatomical abnormality which is manifested in the form of increasing the number of fingers on the feet or hands. This pathology is congenital in nature and occurs equally often in boys and girls.
Polydactyly can be an independent disease or supplemented with other problems of locomotor apparatus.
Often this violation supplements dysplasia, brachydactyly, which consists of hypoplasia of the phalanges of the fingers, or syndactyly – fusion of fingers.
To related diseases are unnatural length of toes extremities — dolichostenomelia and underdevelopment of the fingers from ectrodactyly.
Polydactyly is not life-threatening but the disease can provoke the disruption of the extremities and present a psychological problem.
In addition, the disease often leads to impaired development. It can be a reason to use orthopedic shoes. In adulthood, one is faced with certain restrictions when choosing a career.
How is polydactyly?
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The main cause of this disease is hereditary predisposition. Familial forms of polydactyly are autosomal-dominant type of inheritance.
One of the parents can be a carrier of the gene of Megaplast, but the symptoms of polydactyly it missing. The probability that the child will inherit this disorder, to 50 %.
What causes pathology?
True causes that provoke the appearance of polydactyly is not always possible. The main cause of disorders is genetic predisposition.
Sometimes polydactyly becomes a manifestation of severe chromosomal or genetic disorders. In medicine, there are many syndromes, which are complemented by this disease.
However, in most cases to identify the causes of the disease impossible. There is a version that is a breach develops at 5-8 weeks of fetal life and is associated with an irregular increase in mesodermal cells.
What kinds of pathology are there?
There are different varieties of the disease, and the classification of the violation is carried out according to various criteria.
So, on the location of the extra fingers, there are types of pathology:
- Premaxilla. In such a situation is a repetition of parts of the thumb.
- Central. In this case, the violations took place two to four fingers.
- Postaxial. In this case, a duplication of the little finger.
Depending on the form of the double release:
- Type I – the extra finger is considered to be a rudiment that consists of soft tissue;
- Type II – violation is a consequence of the bifurcation of the main finger;
- Type III – the extra finger is considered complete.
The main manifestation of the disease is considered to be the level of deformation of the primary finger. This parameter affects the choice of tactics of surgical intervention.
It is worth considering that polydactyly usually appears on the hands, but in some cases it affects the toes. Also sometimes found mixed form of the disease, extra toes appear on the hands and feet.
As the disease manifests itself
The main symptom of polydactyly – presence of extra fingers on the feet or hands. They can remind rudimentry appendages or have the correct structure and dimensions.
Typically, these fingers are reduced in size and a smaller number of phalanges. Sometimes they don’t have the basics of bone and resemble soft formations. In some cases, doubles the nail phalanx.
Apart from increasing the number of fingers, thus violating deformed osteo-articular apparatus of the affected segments.
The aging of the body it progresses, which leads to the appearance of secondary disorders:
- In the case chondroectodermal dysplasia disease is complemented by short stature, limb shortening by changing the shape of the chest. There may also be heart disease.
- With the development of pathology of Laurence-bardet-moon-Biddle disease combined with the appearance of extra weight, deformation of the skull, mental retardation, underdevelopment of sexual characteristics, syndactyly.
- People with patau syndrome, in addition to polydactyly, have many defects. These include microcephaly, corneal opacity, mental retardation, disorders of different organs.
During clinical diagnosis of the child is examined by a traumatologist-orthopedist, which detects anatomical abnormalities and determines the deformation.
In addition to the orthopedist, need to be examined by a pediatrician. You should also obtain and genetics counseling.
In addition, it is necessary to conduct x-rays, which allows to identify anatomical features of bones and joints. To determine the condition of the cartilage, bone and soft tissues, it is necessary to perform magnetic resonance imaging.
In the survey used electrophysiological methods of diagnosis. These include electromyography, reovasography. They can be used to determine the condition of muscles and blood flow. Using biomechanical studies will be able to determine the static load on the legs and arms.
During a genetic survey carried out genealogical analysis, establish the type of inheritance, predict the risk of birth of a baby with polydactyly. If the disease is associated with chromosomal or genetic syndromes, it is necessary to conduct prenatal diagnosis.
With the development of isolated polydactyly pregnancy continue. In event of a severe chromosomal abnormalities often have to terminate the pregnancy.
Surgery is the only way
The only treatment of polydactyly is surgery, during which the removal of vestigial toes.
Usually such interference is carried out after the child reaches one year of age. If an extra toe is connected with the brush baffle out of the skin, it is removed within the first month of life.
The operation is usually performed under General anesthesia, although sometimes doctors recommend to choose a local anesthetic. Depending on the type of intervention there are several types of operations:
- removal of extra fingers without compromising the integrity of the main;
- the removal of the finger in conjunction with correction of the primary.
After the intervention the person shown rehabilitation, which includes physiotherapy, massage, physiotherapy.
Doctors do not advise to postpone the operation for a long time, because it can increase the symptoms of strain.
Such violations will require long-term treatment from the podiatrist.
Prognosis and prevention
Preventive measures consist in a detailed genetic survey of people who have close relatives who have symptoms of polydactyly.
People from such families will need to consult genetics. If pregnancy women are recommended to avoid any adverse effects, especially in the first trimester.
It is worth considering that polydactyly in humans is not a threat to life and health. After surgery children do not differ from peers.
They have over 15 years of experience from the podiatrist. Then the growth of the hands and feet ends, and therefore the probability of a deformation is missing.
Polydactyly – a rare violation, the exact cause of which is not yet established. It is believed that this pathology is associated with genetic predisposition.
To eliminate this disease can only be surgically removed, so when it is symptoms are advised to postpone the visit to the doctor.