Congenital constriction ring syndrome

Case contributed by Dr Petralli Carlo Lucius E

Presentation

41-year-old white male. Incidental finding.

Modality: X-ray

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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