Congenital constriction ring syndrome

Case contributed by Petralli Carlo Lucius E
Diagnosis probable


Incidental finding.

Patient Data

Age: 40 years
Gender: Male

Right hand


The middle finger shows short middle and distal phalanges, with surrounding soft tissue swelling.

Case Discussion

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:

  1. a simple ring usually occurring transversely around the limb or digit
  2. a deeper ring often associated with abnormality of the part distally, usually lymphedema
  3. fenestrated syndactyly or lateral fusion of adjacent digits at their distal ends with proximal fenestration between the intervening skin and soft tissue
  4. intrauterine amputation, in which the soft tissue is 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.

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