amniotic band sequence
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Definition: Amniotic band sequence is a disruption sequence having a broad spectrum of clinical manifestations ranging from partial amputations to major craniofacial and limb-body wall defects.
Amniotic bands are the result of adhesions between the amnio and embryonic or fetal parts. Their frequency is comprised between 1/1200 to 1/15 000 births. Amniotic bands cause masive malformations with limb amputations, sever abdominal or cranial wall defects. Two different theories have been proposed: constrictive amniotic bands secondary to early amnion rupture, and vascular disruption events.
Associations
short umbilical cord
early amnion rupture-oligohydramnios disruption (EAROD)
amniotic deformity-adhesion mutilation (ADAM)
Synopsis
fetal malformations
- limb anomalies
- intrauterine amputations
- limb constriction rings
- pseudosyndactyly
- club feet
- abnormal dermatoglyphs
- craniofacial anomalies and cranial defects
- facial clefts (cleft lip, cleft palate)
- microphtalmia
- abnormal skull calicification
- anencephaly
- exencephaly
- encephalocele
- decapitation
- pterygium
- abdominal wall anomalies
- ompahlocele
- gastroschisis
- abdominal constriction ring
constriction of the abdominal cord
The anomalies are characteristically asymmetrical.
Prediposing factors
familial predisposition
amniocentesis
Differential diagnosis
Adams-Oliver syndrome (aplasia cutis-limb defects)
- scalp and/or skull defects at the vertex are associated with often asymmetric transverse limb defects.
- autosomal dominant disease is usually diagnosed after birth
other congenital transverse deficiencies not related with band constriction
Case records
Case #11370: Amniotic band sequence with exencephaly
Case #13378: Amniotic band sequence
See also