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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.

Streeter anomaly

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 anomaly.

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 intubation.

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 44:280, 1992.  [PubMed: 1488974]
Lubinsky M, Sujansky E, Sanger W, et al: Familial amniotic bands. Am J Med Genet 14:81, 1983.  [PubMed: 6829612]
Stanek J, de Courten-Myers G, Spaulding AG, et al: Case of complex ...

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