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Constriction ring syndrome : ウィキペディア英語版
Constriction ring syndrome

Constriction ring syndrome (CRS), also known or misnamed as "ADAM Complex", "amniotic band sequence", "amniotic band syndrome", "amniotic disruption complex", "amniochorionic mesoblastic fibrous strings", "congenital amputation", "constriction band syndrome", "congenital constriction bands", "Streeter bands", "tissue bands", is a congenital disorder with unknown etiology. Because of the unknown etiology there are many different, and sometimes wrong names, (see etiology). It is a malformation due to intrauterine bands or rings that give deep grooves in, most commonly, distal extremities like fingers and toes. In rare cases the constriction ring can form around other parts of the fetus and cause amputation or even intrauterine death (see Types and Classification). The anatomy proximal to the site of constriction (or amputation) is developmentally normal.
CRS can be associated with other malformations with club foot being most common.
(see also Types and Classification).
The precise configuration of the bands, lymphedema, and character of the amputations are not predictable and vary with each individual patient. Also more than one extremity is usually affected, and it is rare for only one ring to present as an isolated malformation with no other manifestation of this syndrome.〔〔
==Diagnosis==
thumb
The diagnosis of constriction ring syndrome can be confirmed with an ultrasonography. The clinical manifestations can be extremely variable. It could be a single or multiple manifestation. This can be confirmed at the end of the first trimester or at the beginning of the second trimester. But not every patient will be diagnosed at that moment, most will get this diagnosis at birth.
Differential Diagnosis;

The differential diagnosis includes;
*Symbrachydactyly
*Chorionic villus sampling
*Congenital amputations
*Hypoplasias of hand, digit, thumb
*Adams-Oliver syndrome
*ADAM complex
ADAM Complex; CRS is sometimes mislabeled as ADAM complex. ADAM is an abbreviation for Amniotic Deformity, Adhesions Mutilations. CRS is the malformation due to a constriction ring around mostly a limb. ADAM-complex is the association of limb defects (caused by constriction rings) and certain craniofacial clefts
Adams-Oliver syndrome is often mislabeled as CRS and consists of cutis aplasia of the scalp in which a longitudinal defect can vary in size and can often be associated with full-thickness skullcap loss. The distal digital or toe hypoplasia-aplasia is often confused with CRS. Constriction rings with or without edema are not present. The digital or toe hypoplasia-aplasia usually contains diminutive nails or nail folds”.〔

抄文引用元・出典: フリー百科事典『 ウィキペディア(Wikipedia)
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