Hemihypertrophy is one of the congenital malformation syndromes. The disease is usually diagnosed in early childhood. With her, there is an uneven growth in size of the body or parts of it.
What is hemihypertrophy?
According to ABBREVIATIONFINDER, hemihypertrophy is also known as hemigiant growth. It is a very rare disease worldwide. It is diagnosed with a frequency of 1:1000,000. Hemihypertrophy is characterized by unilateral overgrowth of the body. Under normal circumstances, the body is symmetrical.
If there is a size deviation of five percent between the halves of the body, doctors speak of hemihypertrophy. The different height growth can also refer to individual parts of the body such as the extremities. The condition is usually a congenital anomaly.
Hemihypertrophy is a special form of hypertrophy. In this case, enlarged organs or tissue developments are found in the patient. Asymmetry of growth in hemihypertrophy in some cases can refer to paired internal organs. The hereditary disease can also lead to changes in the skin and teeth.
Hemihypertrophy is a complex clinical picture. It can occur as an isolated disease or be diagnosed as part of a syndrome. The disease can be congenital or develop during the first few years of life during the individual growth phases. In very few cases, hemihypertrophy develops due to inflammatory processes only at an older age.
Causes
The cause of the hemihypertrophy is classified as insufficiently known. It is believed to be a genetic predisposition. However, there are currently no specific findings. It is very likely that the disease is not hereditary.
In most cases, the malformation appears to develop in the first few days of pregnancy. When a cell changes, all of the cells that develop from it grow faster than the others. Depending on the time of the mutation, the strength of the expression of the growth in size is to be assessed.
Hemihypertrophy is usually congenital. However, it can also manifest itself in the first years of life during growth development. In addition, documented cases show that the disease can develop later in life through inflammatory processes. Proliferative processes such as osteomyelitis are known.
This is an infectious inflammation of the bone marrow in humans. In addition, hemihypertrophy can be diagnosed as part of other syndromes. These include Proteus syndrome, Silver-Russell syndrome, Beckwith-Wiedemann syndrome and Klippel-Trenaunay syndrome.
Symptoms, Ailments & Signs
The first signs of hemihypertrophy can appear shortly after birth. The overgrowth of one half of the body is already clearly visible in infants. The fundamental disturbance of the symmetry of the human body is a prominent symptom of the disease.
Soft tissues such as muscles, skin or tendons are involved in the process of size changes. Additional anomalies in the facial area, especially on the teeth or skin, are possible. Some patients also develop endocrine disorders such as thyroid disease or diabetes.
In some cases, the asymmetrical development of the body only emerges during the growth phases. Different leg or arm lengths are noticeable in the patient. Unequal development of the upper body is also possible. Other symptoms may include local size changes in paired organs.
Hemihypertrophy leads to a malformation of the blood vessels. Circulatory disorders are to be expected in the affected parts of the body. The skin is often described as marbled and slightly bluish. In addition, swelling of the skin and wound formation occur.
Diagnosis
Diagnosis of hemihypertrophy is initially made by visual contact with the asymmetry. The doctor then measures the affected body parts and documents the deviations. This is followed by examinations such as ultrasound, X -rays or various scans. This is used to determine the exact size differences.
Furthermore, the participation of the organs is checked. Since the anomaly no longer grows as a result of the asymmetric growth, the asymmetry can be expected to worsen as the child grows.
Complications
In most cases, hemihypertrophy causes asymmetry in the patient’s growth. One half of the body is affected by a strong overgrowth. The entire symmetry of the affected person is significantly disturbed by this disease and is no longer in balance. Muscles or eyes can be damaged, and the skin is often affected by symptoms.
In addition, malformations occur in the teeth, so that the intake of food or liquid is associated with considerable pain. It is not uncommon for patients to suffer from diabetes. The lengths of the arms and legs can be different on the different sides, which leads to significant restrictions in everyday life. There are also restrictions on movement.
In some cases, the internal organs can also be affected by hemihypertrophy. This leads to disturbances in blood circulation and severe swelling. In most cases, there are also psychological problems and inferiority complexes.
Especially in children, the disease often leads to teasing and bullying, which extremely restricts the quality of life. A direct treatment of the complaints is not possible. In some cases, the deformities can be surgically removed. There are no other complications.
When should you go to the doctor?
In order to avoid further complications and symptoms in adulthood from the disease, it should be treated by a doctor at the first sign. The first signs of hemihypertrophy can already appear after birth. The children suffer from significant overgrowth or asymmetry of the entire body. Doctors usually notice these symptoms after the baby is born. Furthermore, there are also malformations in the face, which can indicate hemihypertrophy.
The length of the legs and arms are also different, so that the child has to struggle with significant difficulties in everyday life. If these symptoms occur, an examination by a doctor is necessary. Swellings on the skin or circulatory disorders usually indicate hemihypertrophy.
The diagnosis can be made by a pediatrician or by a general practitioner. For further treatment, the patients are dependent on various specialists, since hemihypertrophy can only be treated symptomatically. Life expectancy is not reduced with hemihypertrophy.
Treatment & Therapy
The treatment of hemihypertrophy is very individual. If the balance is disturbed due to the different weight distribution in the body, the patient needs therapeutic support. Special training is carried out for this.
As far as possible, physical exercises are used to help correct misalignments and relieve the muscles. Tensions in the body should be reduced. Existing leg length differences can be compensated for with an orthopedic shoe fitting. Special shoes with an integrated shoe structure are made for this.
In addition, various surgical interventions can be performed. They pursue the goal of compensating for the difference in size between the halves of the body. In most cases, the patient suffers from pain due to the misalignment of the body. Therefore, drug treatment to alleviate the symptoms is very likely.
Tooth misalignments are accompanied orthodontically. This can lead to surgery or tooth correction with braces. If there are differences in the size of paired organs, it is checked to what extent these can be adjusted by means of a surgical intervention. Changes in the skin are usually corrected using cosmetic forms of treatment such as laser beam therapy or local surgery.
Outlook & Forecast
The prognosis of hemihypertrophy depends on its severity and the intensity of treatment. Although the disease is not fatal. But it always leads to asymmetrical body proportions. One-sided gigantism can be corrected by surgical measures. However, full body symmetry is not possible.
The uneven body growth favors the development of scoliosis. As a rule, scoliosis (curvature of the spine) can be treated well with physiotherapy or corset therapy. However, this is not possible with this form of hemihypertrophy due to the uneven growth.
Here, the curvature of the spine can only be corrected as part of an operation. A leg length discrepancy must also be corrected surgically in order not to risk further consequential damage such as scoliosis and severe postural damage.
The therapy also includes the individual manufacture of foot insoles and custom-made shoes. Hemihypertrophy is always associated with tooth misalignments. Their correction is also only possible surgically. In order to achieve an extensive alignment of both halves of the body, constant surgical interventions are necessary during growth. The uneven development of the body always leads to pain, which makes pain therapy necessary.
Although lifespan is not affected by hemihypertrophy, quality of life is reduced despite all treatment measures. In addition to the physical discomfort, those affected are often exposed to constant teasing. As a result of chronic pain and constant bullying, the development of mental illnesses is favored.
Prevention
The taking of preventive measures is not known in hemihypertrophy. However, since it is suspected that the disease increases the risk of developing a tumour, the patient should have preventive examinations in this regard. This applies in particular to cancers in the kidney area.
Aftercare
In the case of hemihypertrophy, the focus is on early detection and treatment of the disease so that there are no further complications or symptoms. The earlier the disease is recognized and treated, the better the further course in most cases. Therefore, the affected person should consult a doctor at the first symptoms or signs of the disease in order to prevent further complications or complaints.
The measures or options for aftercare are severely limited in the case of hemihypertrophy. In most cases, the symptoms are alleviated by surgery, with the interventions differing according to the type and severity of the exact symptoms. In general, after such a procedure, the person concerned should definitely rest and take care of his body.
Efforts or stressful and physical activities should be avoided. Physiotherapy measures can also be very helpful, and many of the exercises can also be carried out at home. Wearing special shoes can also relieve the symptoms. The life expectancy of those affected is usually not reduced by hemihypertrophy if the disease is recognized and treated early.
You can do that yourself
Due to hemihypertrophy, most of those affected suffer from an increased risk of tumor development. For this reason, affected patients should attend regular check-ups to avoid further complications.
The kidneys in particular must be examined, as they are particularly affected by the tumor diseases. If the person concerned suffers from dental problems or misaligned teeth, these are usually treated by a dentist.
A special self-help is not available to the person concerned, whereby the right care of the teeth can have a positive effect on these complaints. The misalignment of the body is treated with various therapies and exercises.
Most of these can also be done at home. The execution of various sports can also have a positive effect on this imbalance and eliminate it. Furthermore, in the case of hemihypertrophy, it is advisable to wear specially made shoes to strengthen the child’s posture.
In the case of psychological problems, talking to other people who are affected or to your own parents and friends can be very helpful. A psychologist should be consulted for severe depression. In many cases, the symptoms of hemihypertrophy can be alleviated relatively well.
