Amniotic Band Syndrome with CTEV and Meningocele: A Rare Case Report

Amniotic band syndrome (ABS) is a group of rare congenital abnormalities caused by wrapping of parts of the foetus by fibrous amniotic bands during intrauterine life. It can be seen in infants without any known genetic mutations. Band formation most frequently affects the distal segments, including the hand. Here, we report a case of a neonate who presented with multiple congenital abnormalities and clinical features that suggest the Amniotic Band Syndrome. It was delivered by a 17-year-old female patient at 28 weeks period of gestation, who had a medical abortion.


INTRODUCTION
Amniotic band sequence (ABS) is a constellation of complex fetal morphological abnormalities associated with intrauterine amniotic bands. 1 It is a rare disorder and with varied degrees of abnormalities. 2 The clinical aspects of the limbs are varied with presence of cutaneous grooves of amputation, pseudo-syndactyly and club feet. 3he incidence varies from 1 in 1,200 to 1 in 15,000 live births. 4Both sexes are equally impacted. 5Anomalies can range in severity from simple craniofacial clefts that are ultimately life-incompatible to single-digit amputations. 6rtal first described this malformation in 1685. 7ABS has several names that have been reported in the literature, including congenital ring constriction, constriction ring syndrome, amnion rupture sequence, amniotic band sequence, and amniotic band disruption complex. 2,8,9everal explanations have been put out regarding this disease that includes: I. membrane disruption linked to the development of the amniotic band; II.genetic factors; and III.vascular abnormality resulting in an ischaemic event. 10

CASE REPORT
An 18 year old primigravida who was 28 weeks pregnant came to Karnali Academy of Health Sciences (KAHS) with history of decreased fetal movement and vaginal leak since one day.There was no previous prenatal checkup.There was no significant past medical history for the patient and drug history during the pregnancy and history of consanguinity.Also, congenital anomalies were not found in the family in last 3 generations.A uterus of around 28 weeks' size was noted during an obstetric examination with absent fetal heart sound.Ultrasonography at the time of examination showed intra uterine fetal demise (IUFD) with multiple anomalies.Written consent was taken before the induction with misoprostol regarding the prognosis of outcome.The patient responded well to the induction, giving birth vaginally to a stillbirth boy with a birth weight of 1200 grams (Figure 1).
Clinical examination showed partial sectioning of the index finger, middle finger and ring finger of right hand along with syndactyly of the stumps and normal thumb and little finger (Figure 1).The left hand was unremarkable.Congenital Talipes Equino Varus (CTEV) was seen in right foot (Figure 2) whereas the left leg was normal.A sac protruding from the spinal column likely meningocele was noted (Figure 3).There was flat nasal bridge and low set ears.The fetopathological examination was not done as the parents did not give consent to do so.

DISCUSSION
Amniotic band syndrome is a complex disorder that can result in a number of fetal defects.Clinically, it can be identified by the presence of bands, cutaneous strictures, amputations of the extremities, and pseudosyndactylies, among other pathognomonic symptoms. 11S etiopathogenesis is still unknown, but there are two main theories. 12The first is the intrinsic explanation, which George Streeter put forth in 1930.According to this view, an abnormality in embryogenesis would result in a faulty histogenesis of the fetal tissue, which would result in the fibrous bands.However, it is unable to explain the majority of the anomalies observed. 13The extrinsic idea, put forth by Torpin in 1965, claims that amnion rupture would lead to the production of fibrous tissue strings that would be responsible for constriction bands, amputation, and other deformities. 14Congenital abnormalities of the illness can range from limb or extremity amputations to potentially fatal craniofacial, thoracic, and abdominal problems.Amputated limbs or digits, contractures in the limbs, Congenital Talipes Equino Varus (CTEV) also known as club feet, syndactyly, and absent limbs are among the reported defects in the extremities. 15Craniofacial abnormalities have been reported as encephalocele, anencephaly, cleft lip and cleft palate whereas Thoraco-abdominal complications include omphalocele and gastroschisis. 16enatal ultrasound can be used to identify ABS.This test may occasionally reveal amniotic bands, but more frequently it will detect abnormalities that are characteristic of ABS, such as oligoamnios and a decrease in fetal activity. 17The most crucial and important ultrasound diagnostic criteria are constriction rings on the extremities, visible amniotic bands, and irregularly amputated fingers and/or toes with terminal syndactyly. 18e appropriate course of action relies on the nature of the anomalies that are present, which can range from lifethreatening to functionally and aesthetically detrimental.Because there are so few reliable studies, prenatal therapy, such as fetoscopy to remove constriction bands, is still debatable.In addition, prenatal procedures demand a multidisciplinary organization. 8

CONCLUSION
Amniotic band syndrome is a rare congenital anomaly affecting the fetus during pregnancy.When fetal deformities are recognized as being incompatible with life, a medical termination of pregnancy may be recommended.And multidisciplinary monitoring is necessary for patients who survive in order to enhance their quality of life.
Data Availability: Related photograph of this case will be available on request Informed Consent: Consent from patient's father as well

Figure 2 : 1 :
Figure 2: CTEV in right foot Figure 1: Partial sectioning of the index finger middle finger and ring finger of right hand along with syndactyly of the stumps