Correction of knee deformity in patients with Ellis-van Creveld syndrome

Six knees in three patients with Ellis-van Creveld syndrome were treated with lateral soft tissue release and corrective osteotomy of the tibia at 10 years of age on average. The main feature was valgus deformity with lateral dislocation of the patella. All patellae were reduced. The valgus deformity improved from 35 degrees (range, 48 degrees-20 degrees) to 17 degrees (range, 35 degrees-5 degrees) of the femorotibial angle (FTA) on average, although the FTA in five of six knees was < 5 degrees after surgery. There was one recurrent case and one transient peroneal nerve palsy. The reason for undercorrection was a depression of the lateral tibial plateau. The deformity of the articular surface is the most important problem in correcting the valgus deformity of the knee in this syndrome.