Congenital constriction ring syndrome
41-year-old white male. Incidental finding.
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The malformation was present since birth.
The pathogenesis of this disorder is not known for certain.
Patterson(1) included four types of deformity in CCRS:
- a simple ring usually occuring transversely around the limb or digit.
- a deeper ring often associated with abnormality of the part distally, usually lymphedema.
- fenestrated syndactyly or lateral fusion of adjacent digits at their distal ends with proximal fenestration between the intervening skin and soft tissue.
- intrauterine amputation, in which the soft tissue are more affected than the bone.
80% of patients with CCRS have been reported to have syndactyly, hypoplasia, brachydactyly, symphalangism and symbrachydactyly.
40% to 50% of patients have club foot, cleft lip, cleft palate and cranial defects.
1 Patterson TJS; Congenital ring constrictions, Br. J. Plast. Surg. 14:1, 1961
2 Mohammad Moosa et. al. The Internet Journal of Orthopedic Surgery, ISSN: 1531-2968