Objective: To review of an uncommon deformity arising in four patients. Method: A clinical and radiological review. Conclusions: heightened awareness and early treatment with monitoring is required. 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 deficiency. 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 benefit 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.
To assess the performance and success of joint sparing limb salvage surgery in high grade malignancy, in terms of function, complications, recurrence and survival, as compared to joint resection. We report a ten-year experience of twenty patients with high grade malignancies of bone which did not cross the epiphyseal plate. They underwent not only limb salvage surgery but also joint preservation. The aim of this is to preserve function in the joint and to prevent the inevitable wear of prosthetic joints requiring revision surgery. The age range was 4 - 25 years (mean 13. 5). The Diagnoses were 14 Osteosarcomas and 6 Ewings sarcomas. Mean follow up was 49 months. There were 13 femoral &
7 tibial malignancies. 12 underwent complex biological fixation with a combination of reimplanted autoclaved or irradiated bone; vascularised fibular graft; femoral or humeral allograft. In 8 cases custom made hydroxyapatite coated prostheses were used to replace the resected bone. This surgery must clearly be evaluated in the context of recurrence, particularly as this is associated with an increased risk of metastases and death. Analysis of our results to date has not shown a greater rate of complications. We experienced one recurrence, and one death. The custom prostheses group had fewer complications and operations. Functionally these patients report near normal limbs and joints and do not report any limitation of activities. Joint sparing limb salvage surgery is extremely worthwhile as it produces a significantly better functioning limb and lower morbidity, with less likelihood of revision surgery. We have not found a higher risk of post-operative complications, recurrence or death. Furthermore massive prosthetic replacement is quicker, osseointegrates reliably and is associated with a lower complication and further operation rate.