This is the Mount Sinai Division of Plastic and Reconstructive Surgery. In this video series, we will augment our continuing medical education article on amniotic band syndrome for PRS, with additional visual aids to better characterize clinical manifestations and treatment strategies for the management of amniotic band syndrome. In this video, we will discuss important background information for the provider to be aware of when first establishing care for pediatric patients presenting with amniotic band syndrome. Patients are often referred to the plastic surgeon by a primary care pediatrician who first notices the defect. We aim to provide a basic overview in order for the plastic surgeon to be prepared when first meeting these patients in the office. Amniotic band syndrome refers to a congenital condition characterized by the presence of constrictive fibrous bands with associated malformations, deformations, neurovascular compromise, and or amputations observed distal to the area of constriction, resulting in subsequent loss of function and reduction in quality of life. There is no genetic predisposition, and it occurs sporadically rather than being inherited. Incidence is reported to be from 1 in 1,200 to 1 in 15,000 live births, and approximately 1.8% of stillbirths. There is a very extensive range of congenital defects that are known to occur in association with amniotic band syndrome, and these defects have been found all over the body, including the craniofacial region, those involving the trunk and abdomen, and the upper and lower extremities. Here in our paper, you can see an illustrated synopsis in this figure of the more frequently reported amniotic band syndrome manifestations in the literature. This reflects the heterogeneous nature of the features of this condition. There are two main theories for the proposed mechanism for amniotic band syndrome. First, the intrinsic theory, originally proposed by Streeter in 1930, posits that amniotic band syndrome occurs due to an underlying genetic insult that causes priming of the germinal disk for the development of fetal raw surfaces that are predisposed to band adhesion during development in utero. The other theory, the extrinsic theory, originally proposed by Torpin in 1965, suggests that amniotic band syndrome develops as a result of amniotic rupture in utero, leaving the external fetal surfaces exposed and susceptible to adhering to the sticky bands and the subsequent associated compressive and constrictive effects on the involved appendages. While amniotic band syndrome is known to be a sporadic condition, there are several known risk factors for the development of amniotic band syndrome. These include low birth weight and prematurity, trauma, hemorrhage, amniocentesis, unplanned pregnancy, prematurity, living at high altitudes, maternal drug use, including tobacco, and maternal illnesses during pregnancy, most commonly pre-gestational diabetes, anorexia, and infection. Amniotic band syndrome can be diagnosed clinically by the presence of a constrictive circumferential band of soft tissue with associated indentation of neighboring soft tissue deep to the band. As you can see in the figure on the right. Proximal structures are usually anatomically normal, while soft tissues distal to the band can either be normal or present with additional defects. Of note, amniotic band syndrome usually prevents with maximum severity at birth and does not clinically worsen with time. Additionally, amniotic band syndrome can be diagnosed prenatally with routine prenatal ultrasound, although importantly, one should note that the presence of the bands themselves on the ultrasound does not confer a diagnosis of amniotic band syndrome. There must also be associated defects, most commonly extremity asymmetry, syndacty, or amputation also noted on the prenatal ultrasound scan at that time for a diagnosis of amniotic band syndrome to be made.

amniotic band syndrome

congenital condition

plastic and reconstructive surgery

prenatal diagnosis

risk factors