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Disorder with an extremely variable clinical presentation characterised by the presence of partial to complete, congenital, fibrous, circumferential, constriction bands/rings on any part of the body, although a particular predilection for the upper or lower extremities is seen. Phenotypes range from only a mild skin indentation to complete amputation of parts of the fetus (for example digits, distal limb). Compression from the rings may lead to oedema, skeletal anomalies (for example fractures, foot deformities) and infrequently neural compromise.
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Disorder with an extremely variable clinical presentation characterized by the presence of partial to complete, congenital, fibrous, circumferential, constriction bands/rings on any part of the body, although a particular predilection for the upper or lower extremities is seen. Phenotypes range from only a mild skin indentation to complete amputation of parts of the fetus (for example digits, distal limb). Compression from the rings may lead to edema, skeletal anomalies (for example fractures, foot deformities) and infrequently neural compromise.
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