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Amniotic band syndrome
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Amniotic band syndrome

Contributors: Craig N. Burkhart MD, Dean Morrell MD, Lowell A. Goldsmith MD, MPH
Other Resources UpToDate PubMed

Synopsis

Amniotic bands result from early rupture or tears in the amnion. These fibrous bands entangle parts of the developing fetus resulting in adhesions or constrictions. The rings encircle and can constrict the limbs, face, or trunk and characteristically involve a distal extremity. It is primarily a sporadic disorder, although a few familial cases have been reported.

Congenital constriction bands are the most typical cutaneous finding of amniotic band syndrome and have associated premature termination of the involved extremities. Deep lesions on an extremity may be associated with distal lymphedema, nerve compression, deformity, or intrauterine amputation. Other findings in amniotic band syndrome can include pseudoainhum, cutis aplasia, stellate scarring, alopecia, cleft palate and lip, and microdactyly.

Maternal risk factors include epidermolysis bullosa, connective tissue disorders, abdominal trauma, uterine malformations, primigravida mothers younger than 25 years, and amniocentesis.

Codes

ICD10CM:
L94.6 – Ainhum

SNOMEDCT:
765206003 – Constriction ring syndrome

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Last Updated:01/11/2022
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Amniotic band syndrome
Amniotic band syndrome : Constricting band, Present at birth
Clinical image of Amniotic band syndrome
A deep constriction band around the lower leg.
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