Congenital constriction ring syndrome

Case contributed by Dr Petralli Carlo Lucius E

Presentation

41-year-old white male. Incidental finding.

see below

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:

- 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.

References:

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

wym-1288284779555

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Case information

rID: 12192
Case created: 28th Oct 2010
Last edited: 26th Nov 2015
Inclusion in quiz mode: Included

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