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Amniotic Band Syndrome

Medically Reviewed by The Medindia Medical Review Team on Jun 18, 2022


What is Amniotic Band Syndrome?

Amniotic band syndrome (ABS) refers to various birth defects caused by fibrous bands from the amniotic sac wrapping around various parts of the fetus and preventing normal development.

Other names for the condition include congenital constriction band syndrome (CCBS), Adam complex, and Streeter dysplasia. The condition has been described since the time of Hippocrates and Aristotle (early 300 BC).


Studies have shown the prevalence to be about 7.7/10,000 live births, and as high as 178/10,000 among aborted fetuses.

Fetal Development within the Uterus - In Brief

To better understand how amniotic band syndrome occurs, it is necessary to know how a fetus develops in a mother's uterus. The fetus floats in the mother's womb surrounded by amniotic fluid. The fluid around the fetus is enclosed by a sac composed of two membranous layers. The outermost membrane is the chorion and lines the uterus. The inner layer closer to the fetus is the amnion.

Fibrous bands extending from the amnion entrap parts of the fetus and lead to wide range of anomalies depending on where the constriction occurs and how tight it is. The various abnormalities include mild constriction bands due to blood flow obstruction or amputation of digits, and occasionally even the entire limb. Severe cases may lead to death and miscarriage of the fetus.

What are the Causes of Amniotic Band Syndrome?

The exact cause is not known although several theories have been put forth. The two most popular theories include the following

1. Intrinsic theory:

This theory was proposed in 1930 by an embryologist at Carnegie Institute named George Streeter and referred to as Streeter dysplasia which suggests an internal (fetal) cause.

According to this theory, CCBS is essentially caused by a germ plasma defect. He suggested that some disrupting event during blastogenesis led to sloughing off of soft tissue. The healing process led to the formation of fibrous constriction bands and developmental abnormalities.

2. Extrinsic theory:

The extrinsic theory was proposed in 1965 by an obstetrician named Richard Torpin, which was first suggested by Hippocrates. Nowadays, Torpin's extrinsic theory is more widely accepted. This theory suggests that external agents, for example, maternal trauma, illness or drugs may play a role.

Torpin suggested that maternal trauma leads to rupture or tear of the amnion while the outer chorionic membrane is intact. The fetus still continues to float in the amniotic fluid, but is exposed to the sloughed off tissue from the amniotic membrane. This tissue can entrap and constrict various parts of the fetus, resulting in the Amniotic Band Syndrome.

However, it is possible that both intrinsic and extrinsic factors may play a role either alone or in combination and the cause may vary from one infant to another.


What are the Risk Factors of Amniotic Band Syndrome?

Several factors have been shown to be associated with an increased risk of ABS, but none are conclusively proved. These include the following:

What are the Symptoms and Signs of Amniotic Band Syndrome?

Amniotic Band syndrome shows varied range of presentation with no two cases similar. Several different patterns of amniotic band syndrome are seen. In general, the more severe forms of amniotic band syndrome occur when the constriction occurs in the first trimester.

The three most common patterns are:


The various clinical features encountered include the following:

How do you Diagnose Amniotic Band Syndrome?

After birth

Amniotic band syndrome is diagnosed typically soon after the baby is born based on characteristic physical findings. Minimal diagnostic criteria consist of the detection of characteristic defects of the arms, legs, fingers, and/or toes, such as ring-like constriction bands or amputation defects.

Diagnostic imaging including x-ray to determine the extent of bands into the deeper tissues and magnetic resonance imaging (MRI) or other scans may be necessary to see how the bands affect blood vessels and nerves.

Before birth

One extremely rare inherited disorder called Adams-Oliver syndrome (AOS) has symptoms similar to amniotic band syndrome and can make diagnosis difficult. AOS is characterized by defects of the scalp and abnormalities of the fingers, toes, arms, and/or legs.

Hence accurate diagnosis is important for management of the present pregnancy as well as counseling for future pregnancies.

How do you Treat Amniotic Band Syndrome?

If the bands are not deep and do not cause any symptoms or health problems, no treatment may be required. Treatment is mainly supportive and symptomatic.

Surgery

Some babies need surgery immediately after birth to release the bands if there is concern about damage to blood vessels or nerves and severe swelling (lymphedema) of the limb. In other cases, surgery may be delayed for upto six months of age. Surgery may be done to give the affected part a normal appearance.

There have also been reports of in-utero surgery performed to remove bands that threatened fetal development.

Other treatments:

Treatments under Investigation

Studies are ongoing to identify the presence of underlying risk factors such as genetic factors that may increase the chances of occurrence of amniotic band syndrome. These may shed light on the complex mechanisms involved in the development of amniotic band syndrome.

References:

  1. Prenatal diagnosis of amniotic band syndrome - (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4813076/)
  2. Amniotic Band Syndrome - (https://rarediseases.org/rare-diseases/amniotic-band-syndrome/)
  3. Epidemiology and risk factors of amniotic band syndrome, or ADAM sequence - (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3530965/)
  4. What is amniotic band syndrome (ABS)? - (http://www.chw.org/medical-care/fetal-concerns-center/conditions/infant-complications/amniotic-band-syndrome/)

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