AMNIOTIC BAND SYNDROME

Pragya Raj, Amity University Kolkata 

Amniotic Band Syndrome (ABS) is the congenital malformations related to the amniotic bands which entangle the fetal parts during the pregnancy term, results in a wide range of anatomical disturbances like constriction rings, bizarre multiple congenital anomalies. ABS occurs rarely but should be considered with a newborn with congenital anomalies. Both genders are equally affected. There are various theories related to the cause of ABS, but the exact reason for its occurrence is still unknown. Diagnosis of ABS can be done prenatally by ultrasound and if undiagnosed, could result in defects that could be seen after birth. The examination is usually done before the 20^th weeks of gestation. The ultrasound report analysis allows detection by visualization of amniotic bands attached to the fetus. 

Defects related to ABS:

Congenital anomalies could be a possible result of the vascular disruptive process during gestation, which includes gastroschisis, terminal transverse limb reduction defects, microtia, small intestinal atresia, etc. These defects occur due to the presence of ‘amniotic bands’ which are fibrous tissues that occur in the amniotic lining which have also been attributed to vascular disruption. An example supporting this is the association between Septo-optic dysplasia (SOD) and the syndrome of amniotic bands. SOD is characterized by features such as pituitary gland dysfunction, optic nerve hypoplasia, a mutation in a single gene, exposure to teratogens, etc. Furthermore, p63 mutations are also related to limb anomalies. Anomalies related to the cleft palate comprises of a different condition which could be caused by IRF6, disorganization, p63. 

ABS occurs infrequently which can be approximately 1 in 15,000 to around 1 in 10,000. Several other anomalies related to the ABS include syndactyly, club-foot, limb length discrepancy, peripheral nerve palsy, cleft-lip palate, spina bifida. The fetal death related to Amniotic Band Strangulation of the umbilical cord has also been reported. This syndrome occurs with no connection with genetic or chromosomal disorders and the syndrome could be considered sporadic. 

Risk Factors:

The various risk factors associated with ABS are young maternal age, unplanned pregnancy, hyperthermia, non-steroidal anti-inflammatory drug usage. As the exact reason for this syndrome is unknown, however the antenatal risk factors associated with ABS include prematurity of the fetus of fewer than 37 weeks, low birth weight, maternal illness during the pregnancy term. 

It is difficult to visualize the amniotic bands in the first trimester and it becomes more difficult to identify these bands if they are present in the extremities. In the second and third trimester, it becomes easier to detect the anomalies by ultrasonography. 

Treatment:

Depending on the anatomical structure involved, patients can consult with an orthopedic surgeon, pediatric general surgeon, or craniofacial surgeon. While it can be easy to detect the extremities, if the band involves the abdomen, neck, or chest, it may comprise imaging studies, which would require a radiologist as well. Currently, there is no specific treatment of Amniotic Band Syndrome even when the diagnosis is done prenatally. All patients should receive counseling related to fetal abnormalities and the consequences they might face post-birth. Consulting with specialties is highly recommended. 

Also read: Coronavirus – UK launches clinical trial of BCG vaccine

SOURCES:

1. Pietro Cignini,¹ Claudio Giorlandino,¹ Francesco Padula, et al.; Epidemiology and Risk Factors of Amniotic Band Syndrome, or ADAM Sequence; 2012; Journal of Prenatal Medicine; Vol: 6, no.4, pp: 59-63; URL: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3530965/ 

2. Ajay Pratap Singh and Sudheer R. Gorla; Amniotic Band Syndrome; 2020; StatPearls; URL: https://www.ncbi.nlm.nih.gov/books/NBK545283/

3. Cihan Inan, N Cenk Sayin, Hakan Gurkan, et al.; Unusual facio-upper arm band of a fetus mimicking amniotic band syndrome; 2019; Journal of Obstetrics and Gynaecology Research; Vol: 45, no. 4, pp. – 927-30; DOI: http://10.1111/jog.13905.

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