Amniotic rupture syndrome (ARS) is a group of fetal malformations and disruptions associated with in utero rupture of the amnion, with consequent formation of fibrous amniotic strands and membranes. Most often, it is associated with limb or digit reduction and atypical facial clefting, but central nervous system abnormalities may be the prominent or presenting abnormality on ultrasound or autopsy (Cignini et al. 2012; Orioli et al. 2003). Central nervous system abnormalities usually accompany craniofacial defects, and include encephaloceles, acalvaria, polymicrogyria, holoprosencephaly, aqueductal stenosis and mesencephalosynapsis, and a bewildering, chaotic array of post encephaloclastic as well as malformative changes. Diagnosis may require a high index of suspicion, careful gross examination and microscopic sampling of the transition between normal and abnormal body surfaces (Shannon 2020).

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Amniotic Band Syndrome/Amniotic Rupture Sequence

  • Patrick Shannon,
  • Sumit Das,
  • Yael Fisher

摘要

Amniotic rupture syndrome (ARS) is a group of fetal malformations and disruptions associated with in utero rupture of the amnion, with consequent formation of fibrous amniotic strands and membranes. Most often, it is associated with limb or digit reduction and atypical facial clefting, but central nervous system abnormalities may be the prominent or presenting abnormality on ultrasound or autopsy (Cignini et al. 2012; Orioli et al. 2003). Central nervous system abnormalities usually accompany craniofacial defects, and include encephaloceles, acalvaria, polymicrogyria, holoprosencephaly, aqueductal stenosis and mesencephalosynapsis, and a bewildering, chaotic array of post encephaloclastic as well as malformative changes. Diagnosis may require a high index of suspicion, careful gross examination and microscopic sampling of the transition between normal and abnormal body surfaces (Shannon 2020).