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Key Points

  • Amniotic band syndrome is a group of congenital anomalies involving limbs, craniofacial region, or trunk.

  • Amniotic band syndrome can range from constrictive bands involving a digit to thoracoabdominoschisis, encephalocele, or the limb–body wall complex.

  • The incidence ranges widely from 1 in 1200 to 1 in 15,000 livebirths depending on how it is defined.

  • Ultrasound examination is the mainstay of diagnosis.

  • Amniotic bands can occur spontaneously or as a result of instrumentation of the pregnancy.

  • Fetoscopic release of amniotic bands may prevent limb amputation when it involves an extremity or may be lifesaving if they involve the umbilical cord.

  • Almost all cases of amniotic band syndrome deliver prematurely.


The amniotic band syndrome (ABS) is a group of sporadic congenital anomalies that involve the limbs, craniofacial regions, and trunk, ranging from constrictive bands, pseudosyndactyly to amputation, as well as multiple craniofacial, visceral, and body wall defects (Torpin, 1965; Jones et al., 1974; Higginbottom and Jones, 1979; Seeds et al., 1982; Ray et al., 1988; Lockwood et al., 1989; Seidman et al., 1989; Kulkarni and Gopal, 1990). The term amniotic band syndrome encompasses many congenital anomalies, including amniotic band disruption complex (Higginbottom and Jones, 1979), amniochorionic mesoblastic fibrous strings (Torpin, 1965), aberrant tissue bands (Jones et al., 1974), amniotic deformity, adhesion and mutilation (ADAM) complex (Keller et al., 1978; Orioli et al., 2003), amniotic adhesion malformation syndrome (Herva and Karkinen-Jaaskelainen, 1984), and the limb and/or body wall defect (Bamforth, 1992).


Several theories have been advanced to explain the occurrence of these anomalies but two are most commonly held. In 1930, Streeter proposed that a disruption in embryogenesis at the time of formation of the germ disk and the amniotic cavity initiated a chain of events leading to the multiple defects. He suggested that amniotic bands were the result, not the cause, of the pathologic process. In 1992, Bamforth reviewed this theory in a series of 54 cases of ABS and concluded that it may be caused by a localized disturbance in establishment of basic embryonic organization. The most widely accepted theory was proposed by Torpin in 1965. He examined the placenta and fetal membranes in a number of affected individuals and concluded that the disorder was caused by primary rupture of the amnion early in gestation (Keller et al., 1978; Higginbottom and Jones, 1979; Seeds et al., 1982; Herva and Karkinen-Jaaskelainen, 1984).


More recently, Moerman et al. (1992) proposed that the ABS is a collection of three distinct entities that can reconcile both Streeter’s and Torpin’s hypotheses. They suggested that ABS consists of three distinct lesions: (1) constrictive tissue bands; (2) amniotic adhesions; and (3) the more complex pattern of anomalies designated the limb-body wall complex (LBWC) (see Chapter 60). ...

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