Amniotic Band Syndrome with Cranial Anomalies; Review of Two Cases

Aydin Atis Alev,Acar Zuhat,Ece Ermin,Topbas Selcuki Fitnat,Gurbuz Sümeyye,Acar Hicran

doi:10.31579/2690-4861/198

Abstract

Amniotic band sequence (ABS) results from in utero entrapment of fetal parts by fibrous bands, which leads to malformations that can affect multiple organ systems including the limbs, craniofacial regions and trunk, and central nervous system. These bands can adhere to anatomical structures of the fetus and cause vascular disruption which results in the amputation of the involved anatomic part. We present two cases of ABS both of which presented with craniofacial defects associated with ABS; one with apical encephalocel and extremity defects, the other one with schizencephaly with extremity defects and cleft lip & palate. ABS may represent itself not only with extremity defects but atypical forms in cranial vault. ABS must be kept in mind and sonography must be detailed when rare deformities like schizencephaly and extremity defects identified.

Highlights

Amniotic band sequence (ABS) is a congenital disorder presenting with fetal anomalies associated with fetal placental fibrous bands that may cause disruptions, deformations or malformations without a consistent anatomical pattern Amniotic band syndrome (ABS) is a prenatal complication with an incidence ranging from 1/1000 to 1/15000 in live births and 1/70 in stillbirths [1]
Constrictive rings, limb defects, neural or spine defects and craniofacial defects are the major classifications of ABS .Encephalocele, facial clefts and cleft lip and/or palate that are located non-midline atypical locations are among the craniofacial defects associated with ABS [3,4]
Amniotic band sequence (ABS) results from in utero entrapment of fetal parts by fibrous bands, which leads to a collection of malformations that can affect multiple organ systems