The amniotic ligament 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?
According to PHONECATIONS.COM, 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 already be guessed at prenatally using ultrasound.
As amniotic ligament syndrome, malformations of newborns are known, which are due to mechanical influences 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 has no hereditary basis either. 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 and of themselves. However, depending on their location, they can prevent the fetus from turning over. In this scenario, the unborn child can become entangled in the strands. Under certain circumstances, the result can be a reduced blood supply to the trapped limbs. The syndrome is also known as constriction ring syndrome , intrauterine amputation , spontaneous amputation , or reductive limb malformation.
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 been known so far. However, based on the cases documented so far, various factors have been identified that 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 also appears that exposure to teratogenic influences during pregnancy can promote amniotic ligaments. Such influences include, for example, X-ray exposure or certain medication intake.
Genetic relationships may also have to be taken into account for the occurrence of amniotic ligaments. For example, some case documentation indicates inherited susceptibility 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 effects in the course of accidents during pregnancy.
Symptoms, ailments & signs
Fetuses can become entangled in amniotic ligaments. Entanglement in the ligaments can lead to 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 syndactylia in the sense of fingers or toes that have grown together. Also malformations of nails may 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 can differ on the sides and have different lengths.
In addition, there is sometimes distal lymphedema , which manifests itself in lymph swelling. Congenital ligament incisions or clubfoot can also characterize the amniotic ligament syndrome. An extreme case is when the head of the fetus is constricted. As a rule, neck 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 greatly facilitates the diagnosis. 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 called 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 pinched off.
In some cases, the syndrome also leads to malformations such as jaw dysplasia, cleft lip, open stomach and open back and distal lymphedema. The 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 concerned.
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 in addition to 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. Usually, however, amputation of the affected limb is necessary in this syndrome.
Treatment & Therapy
Treatment for amniotic band syndrome differs from case to case. As long as the fetus is not entangled in the ligaments, one 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 young 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 impair the infant’s ability to move. The separation of fused 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 childbirth. In the context of such an amputation, too, 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. Primarily, this also leads to a reduced blood flow in the body, which is unhealthy for the entire organism. The patient’s hands are particularly affected, which can lead to movement restrictions and other restrictions in everyday life. Furthermore, the malformations can also occur on the nails.
In amniotic ligament syndrome, the limbs themselves 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 through 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.
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
In the case of amniotic ligament syndrome, the options for follow-up care 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 with 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 to avoid unnecessary stress on the body.
Physiotherapy may also be necessary to completely relieve the symptoms of amniotic ligament syndrome and to restore movement of the body. Some exercises can also be done in your own home in order to speed up the treatment and reduce the discomfort.
Since the amniotic ligament syndrome can often lead to psychological complaints or depression, conversations with friends or with your own family are very helpful. However, contact with other sufferers of the 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 accordingly. Usually, newborn patients undergo the first surgical interventions as early as infants with the aim of correcting the deformities as a result of the prenatal constrictions.
The parents accompany the baby during the necessary hospital stays and after the hospital stay they 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 life, which causes emotional stress in the absence of surgical correction options. The constriction, which is perceived as unaesthetic, results in, for example, 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.
