Amniotic band syndrome is a malformation complex that results from the constriction of fetal limbs and is associated with amniotic bands. Amniotic bands result from a tear in the inner layer of the egg during pregnancy. Treatment of strangulated limbs depends on the severity of the malformation.
What is amniotic band syndrome?
Amniotic ligament syndrome is a malformation complex that results from the constriction of fetal limbs and is associated with amniotic ligaments. The manifestation of symptoms can be guessed prenatally by ultrasound. Amniotic ligament syndrome is the term used to describe malformations of newborns resulting from mechanical effects during the birth process. The syndrome is one of the other and congenital malformation syndromes of the musculoskeletal system. An exact prevalence of the condition is not known. The manifestation of symptoms occurs immediately after birth or can be guessed prenatally by ultrasound. Unlike other malformation syndromes, amniotic band syndrome is not due to genetic causes such as mutations and has equally little hereditary basis. However, some maternal risk factors contribute to the development of the syndrome. For example, the symptom complex is associated with several metabolic disorders of expectant mothers. Amniotic bands are ribbon-like strands of amino acids that are not in themselves a problem. However, depending on their location, they can hinder the fetus from turning around. In this scenario, the unborn child may become entangled in the strands. The result may possibly be reduced blood flow to the entangled limb. The syndrome is also known as lacing ring syndrome, intrauterine amputation, spontaneous amputation, or reductive limb malformation.
Causes
The bands of amino acids in amniotic band syndrome develop as part of tearing at the innermost layer of the egg, also known as the amnion. This tearing can occur at various stages of pregnancy and is due to as yet unknown causes. However, based on the cases documented to date, several factors have been identified that are believed to be associated with a higher likelihood of amniotic strands. These risk factors include, for example, metabolic diseases of the mother, so especially diabetes mellitus. Apparently, exposure to teratogenic influences during pregnancy may also promote amniotic bands. Such influences include, for example, exposure to X-rays or certain medications. Genetic correlations may also need to be considered for the occurrence of amniotic bands. For example, some case documentation indicates inherited susceptibilities to the formation of the bands. Tearing of the inner layer of the oviduct and the resulting strands of amino acids may also be associated with mechanical impact in the context of accidents during pregnancy.
Symptoms, complaints, and signs
Fetuses can become entangled in amniotic bands. Entanglement in the ligaments can result in inferior blood flow, which results in various birth defects. Usually, one of the limbs, a toe, or a finger becomes strangulated. The most common symptoms are seen in the hands. Amniotic strangulations manifest in different symptoms from case to case. Sometimes most commonly, the strangulations cause syndactyly in the sense of fused fingers or toes. Malformations of the nails may also be characteristic symptoms. In some cases, stunted growth has been observed in affected fetuses, manifesting mainly in the small bones. The individual limbs of affected fetuses may be laterally different and have varying lengths. In addition, distal lymphedema sometimes occurs, manifesting as lymphatic swelling. Congenital band incisions or clubfoot may also characterize amniotic band syndrome. An extreme case is when the fetal head is constricted. Usually, head constriction results in stillbirth. Amniotic band syndrome does not necessarily result in malformations in every case. Sometimes the constricted limbs develop completely normally and show only the lacing as a symptom.
Diagnosis and course
Amniotic band syndrome can be diagnosed prenatally by ultrasound.Postnatally, constrictions are still present in some cases, which greatly facilitates diagnosis. The postnatally visible malformations of the amniotic band syndrome must be differentially diagnosed from the malformations of many other syndromes, which are not due to mechanical constrictions but to genetic mutations or similar correlations. The prognosis for affected children depends on the severity of the constrictions.
Complications
Amniotic band syndromes are referred to as congenital birth defects. They consist of ribbon-like amino acid strands that wrap around the fetus. In the process, they can pinch off parts of the body, causing them to be malformed. In the worst case, they can cause intrauterine amputation. Mostly, however, fingers and toes are strangulated. In some cases, the syndrome also leads to malformations such as mandibular dysplasia, cleft lip, open abdomen, and open back and distal lymphedema. Amniotic band syndrome is not due to a genetic cause. However, there are women who belong to the risk-bearing group due to various metabolic diseases. Furthermore, exposure to X-rays, accidents during pregnancy, and certain medications may promote the symptom. Affected children have to suffer numerous complications and are often followed psychologically, medically and physiotherapeutically throughout their lives. Since every amniotic strangulation is different, the diagnosis is based on the malformation. Surgical corrective measures are taken only a few weeks after birth. For the parents this is a great burden, especially if in later years the child has to be provided with a prosthesis. If the fetus is threatened with strangulation of the head, prenatal microinvasive surgery is performed to rule out subsequent complications for mother and child. It also happens that despite the constriction, the limbs develop normally and only present the lacerations.
When should you go to the doctor?
In most cases, the complaints and symptoms of amniotic band syndrome are detected immediately after birth or even before birth. In this case, the affected persons suffer from various malformations and deformities. For this reason, it is usually no longer necessary to see a doctor to diagnose amniotic band syndrome. However, those affected are dependent on regular checks and examinations in order to prevent further complaints and complications in adulthood and thus make the life of the affected person easier. The treatment of the individual complaints is then carried out by the respective specialist. As a rule, amniotic band syndrome is diagnosed by a pediatrician or by a general practitioner. Not infrequently, not only the affected persons themselves, but also the parents and relatives are dependent on psychological treatment. This should be carried out in addition to the physical treatment, so that it does not lead to psychological complaints in adulthood. As a rule, a doctor should be consulted whenever the symptoms of amniotic band syndrome reappear and make everyday life difficult. However, amputation of the affected limb is usually necessary for this syndrome.
Treatment and therapy
Treatment of amniotic band syndrome differs from case to case. As long as the fetus does not become entangled in the ligaments, observation is sufficient. If prenatal ultrasound shows severe constriction, depending on its location, prenatal intervention may be required to free the fetus, especially in the case of constriction in the head region that endangers the life of the child. Such an intervention is also called prenatal surgery and is a rather young field of surgery. After birth, various treatment options are available to treat birth defects. Syndactyls, for example, can be surgically separated if they severely affect the infant’s ability to move. However, the separation of fused fingers or toes and the correction of deformities such as clubfeet only take place if the risk of surgery does not exceed the benefit to the patient. If necessary, the use of assistive devices can also take place, such as a prosthetic fitting. Prosthetic fittings take place under physiotherapeutic and psychological supervision.In rare cases, amputation of strangulated limbs is necessary after birth. Even in the context of such an amputation, the parents are usually accompanied by psychotherapy.
Outlook and prognosis
Due to this syndrome, most affected individuals usually suffer from various deformities and malformations. In the first place, there is also a reduced blood circulation in the body, which is unhealthy for the whole organism. It is mainly the patient’s hands that are affected, which can lead to restricted movement and other limitations in everyday life. Furthermore, the deformities can also occur on the nails. The limbs themselves can have different lengths in amniotic band syndrome. This can lead to teasing or bullying, especially in children, and thus significantly limit the patient’s quality of life. Furthermore, most affected individuals also develop a so-called clubfoot. However, these complications do not have to occur in every case, so that in some cases the child develops normally despite the syndrome. The treatment can take place with the help of surgical interventions or through various therapies and can limit the symptoms relatively well. There are no particular complications. Also, in most cases, the life expectancy of the patient is not reduced by the syndrome.
Prevention
Several preventive measures are available for amniotic band syndrome, although their effectiveness is controversial. For example, expectant mothers should reduce risk factors such as x-ray exposure or medication use during pregnancy.
Follow-up care
In amniotic band syndrome, the options for follow-up care are usually very limited. This syndrome is a congenital condition that occurs with malformations. However, these can only be treated symptomatically and not causally, so that even a complete cure cannot occur. In most cases, the symptoms of amniotic band syndrome are treated by surgery, which is intended to alleviate the individual malformations and symptoms. After the surgery, the affected person must rest and take care of the body. Athletic activities or other strenuous activities should be avoided in order not to put unnecessary strain on the body. Furthermore, physiotherapy may also be necessary to completely alleviate the discomfort caused by amniotic band syndrome and to restore the body’s movement. Some exercises can be performed in the patient’s own home to speed up treatment and reduce discomfort. Since it is not uncommon for amniotic band syndrome to cause psychological discomfort or depression, talking to friends or family can be very helpful. However, contact with other sufferers of amniotic band syndrome can also be sought, as this can lead to an exchange of information.
What you can do yourself
Patients with amniotic band syndrome suffer from constrictions on the body and limbs that manifest differently in each affected person. Because the constrictions occur in the womb, patients are already born with corresponding birth defects. Consequently, it is initially the parents who initiate adequate therapy for the infant and care for him or her according to the deformities. In most cases, the newborn patients undergo the first surgical interventions already at the age of infants with the aim of correcting the deformities resulting from the prenatal constrictions. Parents accompany the baby during the necessary hospitalization and also take care of the child at home according to the doctor’s instructions after the hospitalization. In addition to surgery, orthopedic insoles are also considered to alleviate symptoms, such as for patients with clubfoot. In addition, many affected patients receive physiotherapeutic treatment in order to train sufficient motor skills despite deformed limbs. For some patients, the condition represents a cosmetic blemish throughout their lives, which, in the absence of surgical correction options, creates a psychological burden. For example, the constriction, which is perceived as unaesthetic, results in inferiority complexes in some affected persons, so that consultation with a psychologist is urgently indicated.Partially, the flaw can be concealed by the choice of clothing.
