Constricting amniotic bands leading to amputation with
scarring, distal syndactyly, cleft lip and palate, anencephaly,
encephalocele, hydrocephaly, omphalocele, and gastroschisis. Other internal
anomalies involve the head, heart, lungs, diaphragm, kidneys, and gonads.
Although the peripheral defect is often minimal, a thorough examination is mandatory.
Amniotic bands led to partial amputation of the end phalanx of the fifth
finger and stricture of the fourth finger in this child with Streeter
Congenital Constricting Bands; Amniotic Band Sequence or
Disruption; Streeter Dysplasia Syndrome; ADAM Complex; Terminal Transverse
Defects of Arm.
Reported as 1:1200 live births in some populations.
Possibly autosomal recessive, but in general
the cause of amnion disruption is unknown.
Unknown. Various theories include these
mechanisms: vascular, mechanical, genetic disruption, or germ disc
disruption (Streeter's hypothesis). The amniotic band so formed disrupts
early embryonic growth and results in unusual fetal malformations. It has
also been suggested that the defect occurs prior to 26 days postconception
and before the establishment of effective embryonic circulation (because of
the involvement of several internal organs).
Wide case-to-case variation. Typically include
amputation of digits and limbs from ring-like band constriction in most
cases. The described ADAM Complex (Amniotic Deformity, Adhesions, and
Mutilation) includes amniotic deformity, cleft lip and palate, and other
facial malformations. The described LBWD (Limb and Body Wall Defect) Complex
occurs in severe cases with hypoplasia of all major internal organs, major
limb defects, and death soon after birth.
Assess for clinical associated features
such as cardiac, neurological, and respiratory abnormalities.
Assess the airway for potential difficulty with direct laryngoscopy and tracheal
Difficult peripheral intravenous access
may be encountered because of the self-mutilation.
Special attention must be given to positioning because of vascular compromise in the
extremities. In the presence of facial malformations, the potential for difficult airway
management must be anticipated.
Adams-Oliver Syndrome (AOS): A very rare inherited disorder
characterized by defects of the scalp and associated with multiple scars and
hairless areas that usually have dilated blood vessel directly under the
skin. Scalp defects are already present at birth. The extremities are either
short (hypoplastic fingers and toes) or characterized by absent hands and
lower legs. Congenital heart defect must be ruled out.
Ainhum: A narrow strip of hardened skin, a constricting ring
formation on the little toe at the level of digitoplantar fold leading
progressively to spontaneous amputation.
Bamforth J: Amniotic band sequence: Streeter's hypothesis re-examined.
Am J Med Genet
Lubinsky M, Sujansky E, Sanger W, et al: Familial amniotic bands. Am J Med Genet
Stanek J, de Courten-Myers G, Spaulding AG, et al: ...