Constriction rings syndrome

Orpha code: 295000OMIM code: 217100

Definition

Constriction rings syndrome is a congenital limb malformation 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 (e.g. digits, distal limb). Compression from the rings may lead to edema, skeletal anomalies (e.g. fractures, foot deformities) and, infrequently, neural compromise.

Disease data
Classification

Malformation syndrome

Synonyms
Amniotic band sequence
Amniotic band syndrome
Congenital ring constrictions
Constriction band syndrome
Streeter dysplasia
Amniotic band sequence
Amniotic band syndrome
Congenital ring constrictions
Constriction band syndrome
Streeter dysplasia
ORPHA code
295000
OMIM code
217100
ICD10 code
Q79.8
ICD11 code
LD26.5

No additional description.

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