Abstract
Introduction. Amniotic band syndrome and sequence are a relatively rare condition in which congenital anomalies occur as a result of the adherence and entrapment of fetal parts with coarse fibrous bands of the amniotic membrane. A large percentage of reported cases have an atypical gestational history. The frequency of this obstetric complication is not affected by fetal gender, genetic abnormality, or prenatal infection. Case. A 21-year-old, G1P0 female parturient at 18 weeks and 5 days with a single intrauterine gestation during a routine ultrasound evaluation was noted to have amniotic band sequence. The pregnancy was subsequently complicated by preterm premature rupture of membranes with oligohydramnios, resulting in a surviving neonate scheduled for rehabilitative treatment. Conclusion. Amniotic band syndrome is an uncommon congenital anomaly resulting in multiple disfiguring and disabling manifestations. Several theories are proposed with most involving early rupture of the amnion and entanglement of fetal parts by amniotic bands. This syndrome can be manifested by development of multiple malformations, with the majority of the defects being limb abnormalities of a disorganized nature, as in the case we present. In the absence of a clear etiology of consequential congenital abnormalities, obstetric management guidelines should use shared decision models to focus on the quality of life for the offspring.
Highlights
Amniotic band syndrome and sequence are a relatively rare condition in which congenital anomalies occur as a result of the adherence and entrapment of fetal parts with coarse fibrous bands of the amniotic membrane
When not diagnosed until postpartum, bands that interfere with drainage of the limb resulting in venous congestion or lymphedema can produce extreme pain, due to peripheral nerve compression requiring early surgical intervention repeated over several stages to improve long-term function [1, 28, 30,31,32,33]
Due to the perinatal complications associated with intrauterine band lysis, multidisciplinary meeting consisting of family, obstetricians, neonatologist, psychologist, social worker, and other relevant entities should take place to discuss the options for continuation of pregnancy, perinatal hospice care, and pregnancy termination
Summary
The clinical manifestations are primarily distal deformities, such as constriction of limbs and fingers, syndactyly, acrosyndactyly, phalangeal hypoplasia, pseudoainhum, and amputation of limbs and fingers [1, 2]. The thumb is less vulnerable since it lies protected within the palm of the hand in utero, compared to the longer digits which are more exposed leading to amputations distal to the level of the proximal phalanx [3,4,5,6] Multiple malformations such as clubfoot (30% of patients), leg length discrepancies (24%), other bone anomalies (12%), special craniofacial defects such as cleft lip and palate (8%), visceral and body wall defects, and anencephaly (5%) have been detected in 70% of infants with the disorder [7,8,9,10,11]
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