Streeter Anomaly Syndrome

At a glance

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.

Synonyms

Congenital Constricting Bands; Amniotic Band Sequence; Streeter Dysplasia Syndrome; ADAM Complex; Terminal Transverse Defects of Arm.

Incidence

Reported as 1:1,200 live births in some populations. No sex predilection.

Genetic inheritance

Unknown.

Pathophysiology

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).

Clinical aspects

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.

Precautions before anesthesia

Assess for clinical associated features such as cardiac, neurological, and respiratory abnormalities. Assess the airway for potential difficulty with direct laryngoscopy and tracheal intubation.

Anesthetic considerations

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.

Other conditions to be considered

• ☞Adams-Oliver Syndrome: 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 (Dactylolysis Spontanea): Narrow strip of hardened skin, a constricting ring formation on the little toe at the level of digitoplantar fold leading progressively to spontaneous amputation.

References