Soft-tissue textiloma: a potential diagnostic pitfall.

Klippel–Trenaunay syndrome has 3 essential features: cutaneous hemangiomas, varicose veins and hypertophy of the involved limbs in length or girth, or both. Both bones and soft tissue are usually affected by the hypertrophy. The arteriovenous malformation effects are polysystemic. Patients with this syndrome are at risk of thromboembolic disease. Baskerville’s report shows that 7 (14%) of 49 patients with Klippel–Trenaunay syndrome had pulmonary emboli and 8 (16%) of 49 had DVT. Muluk and colleagues also suggested that patients with Klippel–Trenaunay syndrome are at risk for pulmonary emboli and DVT. Our patient was given anticoagulation prophylactically and has no evidence of DVT. Surgery for patients with Klippel– Trenaunay syndrome has been recommended only for disabling problems when the benefit is fairly predictable. Our patient clearly had a severe degenerative arthritis secondary to the underlying arteriovenous malformations. The radiographic findings of an enlarged and squared patella and femoral condyles, and generalized osteoporosis are similar to the radiographic findings of hemophiliac arthropathy. The grey-black synovium found in our patient is also similar to the synovial siderosis found in hemophilia. In both hemophilia and Klippel–Trenaunay syndrome, chronic recurrent intra-articular bleeding is thought to result in these changes. The siderosis and arteriovenous malformations found within the synovium of our patient support this theory. There are no known previous reports of total knee arthroplasty in patients with Klippel–Trenaunay syndrome. Since the radiographic and histologic findings of knee arthropathy in this case of Klippel–Trenaunay syndrome bears a great similarity to hemophiliac arthropathy, it is reasonable to assume that the longterm results of total knee arthroplasty in this syndrome will be similar to those in hemophilia. Most hemophiliacs with total knee arthroplasty have a good to excellent long-term result. Long-term follow-up of our patient will reveal if this assumption is correct.