Abstract
Aim: To review an uncommon deformity arising in four patients. Method: A clinical and radiological review. Abstract: We describe four cases of distal radial epiphyseal dysplasia associated with a localised area of cutis aplasia congenita (CAC) over the dorsum of the distal forearm. The cutis aplasia was diagnosed at birth in all cases, but the radial dysplasia was not recognised until presentation to our orthopaedic department between the ages of 5 and 10 years. Radial dysplasia describes a spectrum of osseous, musculotendinous, and neurovascular dysplasias of the pre-axial border of the upper limb, and is the most common form of longitudinal deþ ciency. Cutis aplasia congenita involves an ulcerated area lacking in normal skin formation, present at birth. The most common site is on the scalp, but it has been described on the extremities, and overlying embryological malformations. This association, the long-term implications and the requirement for follow-up until skeletal maturity have not previously been described. We emphasise the importance of continued monitoring of these patients as the effects of radial dysplasia did not become obvious for several years, and the potential beneþt from achieving early skin cover with grafting rather than allowing healing by secondary intention is discussed. It is important to increase awareness of this condition so that early orthopaedic and plastic surgical opinions can be sought, in order to reduce the disabling effect on the underlying radius. Conclusions: Heightened awareness and early treatment with monitoring is required.
Theses abstracts were prepared by Professor Dr. Frantz Langlais. Correspondence should be addressed to him at EFORT Central Office, Freihofstrasse 22, CH-8700 Küsnacht, Switzerland.
