A Pediatric Radiology Textbook and Pediatric Radiology Digital Library
Amniotic Band Syndrome
Etiology: — Exogenous theory – disruption of amnion early in pregnancy allows embryo and fetus and fetal parts to enter chorionic cavity and become tangled or entrapped by proteinaceous bands which disrupt tissue growth — Endogenous theory – vascular insult — Wide variety of deformities depending on what parts are trapped by amnion
Imaging: Asymmetric pattern with single or multiple defects — Head or face entrapment: Acrania, anencephaly, facial clefts, nasal deformities, asymmetric microphthalmos — Truncal entrapment: Abdominal wall defects, rib clefting, congenital scoliosis, ambiguous genitalia, imperforate anus — Extremity entrapment: Variable levels of limb amputation, variable levels of limb constriction with possible distal atrophy, lymphedema distal to the level of band constriction, clubfoot, phocomelia
DDX:
Complications: Cause soft tissue constriction, swelling, amputations
Treatment:
Clinical:
Radiology Cases of Amniotic Band Syndrome
AP radiograph of the hand shows the presence of the first proximal phalanx. All the other phalanges are absent.AP radiograph of the feet shows in the left foot there is amputation of most of the phalanges while in the right foot there is amputation of most of the first distal phalanx and the second middle and distal phalanges.