Amniotic band syndrome

The Amniotic band syndrome is a malformation complex that results from the constriction of fetal limbs and is associated with amniotic ligaments. Amniotic ligaments are caused by a tear in the inner layer of the egg membrane during pregnancy. The treatment of the pinched limbs depends on the severity of the malformation.

What is Amniotic Band Syndrome?

The amniotic ligament syndrome is a malformation complex that results from the constriction of fetal limbs and is associated with amniotic ligaments. The manifestation of the symptoms can be guessed at prenatally using ultrasound.

The amniotic ligament syndrome is known as malformations in newborns that are caused by mechanical effects during the birth process. The syndrome is one of the other and congenital malformation syndromes of the musculoskeletal system. The exact prevalence of the disease is not known.

The manifestation of the symptoms occurs immediately after birth or can be guessed at prenatally using ultrasound. In contrast to other malformation syndromes, the amniotic ligament syndrome is not due to genetic causes such as mutations and is also not hereditary. However, some of the mother's risk factors contribute to the development of the syndrome. For example, the symptom complex is associated with various metabolic diseases in expectant mothers.

Amniotic ligaments are ribbon-like strands made of amino acids that do not pose a problem in themselves. However, depending on their location, they can prevent the fetus from turning over. In this scenario, the unborn child can get caught in the strands. Under certain circumstances, the result can be a reduced blood supply to the trapped limbs. The syndrome is also called Lacerating ring syndrome, intrauterine amputation, Spontaneous amputation or reductive limb malformation known.

causes

In amniotic ligament syndrome, the bands of amino acids arise from tears on the innermost layer of the egg, also known as the amnion. This tear can occur in different phases of pregnancy and is decreasing for reasons that have not yet been identified. However, various factors have been identified on the basis of the cases documented so far, which are presumably associated with a higher probability of amniotic cords.

These risk factors include, for example, metabolic diseases in the mother, especially diabetes mellitus. It appears that exposure to teratogenic influences during pregnancy can also promote amniotic ligaments. Such influences include, for example, X-ray exposure or certain medication intake.

Genetic relationships may also have to be considered for the occurrence of amniotic ligaments. Some case documentation suggests inherited susceptibilities to the formation of the ligaments. A tear in the inner layer of the membrane and the resulting strands of amino acids can also be associated with mechanical impacts in the course of accidents during pregnancy.

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Symptoms, ailments & signs

Fetuses can become entangled in amniotic ligaments. Entanglement in the ligaments can result in reduced blood flow, which can result in various birth defects. Usually one of the limbs, a toe, or a finger is pinched off. The most common symptoms are on the hands. Amniotic constrictions manifest themselves in different symptoms from case to case.

Occasionally the most common symptoms of the constrictions are syndactylies in the sense of fingers or toes that have grown together. Malformations of the nails can also be characteristic symptoms. In some cases, inhibited growth has been observed in the affected fetuses, which manifests itself primarily in the small bones. The individual limbs of affected fetuses may differ on the sides and have different lengths.

In addition, there is sometimes distal lymphedema, which manifests itself in lymph swelling. Amniotic ligament syndrome can also be characterized by congenital ligament incisions or clubfoot. An extreme case is when the head of the fetus is constricted. As a rule, head constriction leads to stillbirth. Amniotic ligament syndrome does not have to result in malformations in every case. Sometimes the constricted limbs develop completely normally with only the lacing furrows as a symptom.

Diagnosis & course

The amniotic ligaments of the syndrome can be diagnosed prenatally using ultrasound. In some cases postnatal constrictions are still present, which makes the diagnosis much easier. The postnatally visible malformations of the amniotic ligament syndrome must be differentiated from the malformations of many other syndromes, which are not due to mechanical constrictions, but to genetic mutations or similar relationships. The prognosis for affected children depends on the severity of the constriction.

Complications

Amniotic band syndromes are known as congenital birth defects. They consist of ribbon-like strands of amino acids that wrap around the fetus. In doing so, they can pinch off parts of the body so that they are malformed. In the worst case, they can cause an intrauterine amputation. Mostly, however, fingers and toes are tied off.

In some cases, the syndrome also leads to malformations such as jaw dysplasia, cleft lip, open abdomen and back and distal lymphedema. Amniotic ligament syndrome cannot be traced back to a genetic cause. However, there are women who belong to the risk-bearing group due to various metabolic diseases.

X-ray exposure, accidents during pregnancy, and certain medication intake can also promote the symptom. Affected children have to suffer numerous complications and are often accompanied psychologically, medically and physiotherapeutically for a lifetime. Since every amniotic constriction is different, the diagnosis is based on the malformation.

Surgical corrective measures are taken just a few weeks after the birth. This is a great burden for the parents, especially if the child has to be provided with a prosthesis in later years. If the fetus is threatened with constriction of the head, a prenatal micro-invasive procedure is carried out in order to rule out subsequent complications for mother and child. It also happens that the limbs develop normally despite the constriction and only show the lacing furrows.

When should you go to the doctor?

In most cases the symptoms of amniotic ligament syndrome are diagnosed immediately after birth or even before birth. Those affected suffer from various deformities and malformations. For this reason, a doctor usually no longer needs to be consulted to diagnose amniotic ligament syndrome. However, those affected are dependent on regular checks and examinations in order to avoid further complaints and complications in adulthood and thus to make life easier for the person affected.

The treatment of the individual complaints is then carried out by the respective specialist. Usually, amniotic ligament syndrome is diagnosed by a pediatrician or a general practitioner. Not infrequently, not only those affected, but also parents and relatives are dependent on psychological treatment. This should be carried out alongside the physical treatment so that there are no psychological complaints in adulthood. As a rule, a doctor should always be consulted if the symptoms of amniotic ligament syndrome reappear and make everyday life difficult. As a rule, however, amputation of the affected limb is necessary in this syndrome.

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Treatment & Therapy

Treatment for amniotic ligament syndrome differs from case to case. As long as the fetus is not entangled in the ligaments, observation is sufficient. If the prenatal ultrasound shows severe constrictions, depending on their location, prenatal intervention to free the fetus may be necessary, especially in the case of constrictions in the head area that endanger the life of the child.

Such an intervention is also known as prenatal surgery and is a relatively new field of surgery. After the birth, different treatment options are available for the treatment of birth defects. Syndactylia, for example, can be surgically separated if they severely affect the infant's mobility. The separation of overgrown fingers or toes and the correction of misalignments such as clubfeet only take place if the risk of the operation does not outweigh the benefit for the patient.

If necessary, aids can also be used, for example a prosthesis. Prosthetic restorations take place in physiotherapeutic and psychological care. In rare cases, an amputation of a pinched-off limb is required after birth. In the context of such an amputation, the parents are usually accompanied by psychotherapy.

Outlook & forecast

As a result of this syndrome, most of those affected usually suffer from various malformations and deformities. In the first place, there is also a reduced blood supply to the body, which is unhealthy for the entire organism. The patient's hands are primarily affected, so that movement restrictions and other restrictions in everyday life can occur. The malformations can also occur on the nails.

The limbs themselves in amniotic ligament syndrome can be of different lengths. This can lead to teasing or bullying, especially in children, and thus significantly reduce the patient's quality of life. Most of those affected also develop what is known as clubfoot. However, these complications do not have to occur in every case, so that in some cases the child will develop normally despite the syndrome.

The treatment can take place with the help of surgical interventions or various therapies and limit the symptoms relatively well. There are no particular complications. In most cases, the life expectancy of the patient is also not reduced by the syndrome.

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prevention

Various preventive measures are available for amniotic ligament syndrome, the effectiveness of which is, however, controversial. For example, expectant mothers should reduce risk factors such as x-ray exposure or medication use during pregnancy.

Aftercare

With amniotic ligament syndrome, follow-up care options are usually very limited. This syndrome is a congenital disease that occurs with malformations. However, these can only be treated symptomatically and not causally, so that complete healing cannot occur.

In most cases, the symptoms of amniotic ligament syndrome are treated by a surgical procedure that is intended to alleviate the individual malformations and complaints. After the surgery, the person affected must rest and take care of the body. Sports or other strenuous activities should be avoided in order not to unnecessarily burden the body.

Physiotherapy may also be necessary to completely alleviate the symptoms of amniotic ligament syndrome and to restore movement of the body. Some exercises can also be done at home to speed up treatment and reduce discomfort.

Since the amniotic ligament syndrome can often lead to psychological complaints or depression, discussions with friends or with your own family are very helpful. However, contact with other sufferers of amniotic ligament syndrome can also be sought, as this enables information to be exchanged.

You can do that yourself

Patients with amniotic ligament syndrome suffer from constrictions on the body and limbs, which express themselves differently for each person affected. Since the constrictions take place in the womb, the patients are born with the corresponding birth defects.

As a result, it is first and foremost the parents who initiate adequate therapy for the infant and take care of the deformities. Usually, newborn patients undergo the first surgical interventions as early as infants with the aim of correcting the deformities resulting from the prenatal constrictions.

The parents accompany the baby during the necessary hospital stays and after the hospital stay also look after the child at home according to the doctor's instructions. In addition to operations, orthopedic insoles can also be used to alleviate the symptoms, for example for patients with clubfoot. In addition, many sufferers receive physiotherapy treatment in order to train sufficient motor skills despite deformed limbs.

For some patients, the disease represents a cosmetic blemish throughout their life, which in the absence of surgical correction can cause emotional stress. The constriction, which is perceived as unaesthetic, results in inferiority complexes in some affected persons, so that the consultation of a psychologist is urgently indicated. The flaw can partly be concealed by the choice of clothing.