THE PATHOLOGY OF VALGUS KNEE IN ELLIS-VAN-CREVELD SYNDROM, AND ITS SURGICAL TREATMENT. COMPARISON TO THE EQUIVALENT PATHOLOGY IN BLOUNT’S DISEASE.

Abstract

Introduction: The usual surgical treatment of valgus knee in Ellis van Creveld Syndrom (EVC), is high tibial osteotomy. However, this approach failed to achieve the expected goal of lasting correction. Based on Dr. Paley’s observations, and our previous unsuccessful treatment of valgus knee in EVC syndrome, we changed the surgical approach. The aim of the surgical treatment is to eradicate all the elements causing sever valgus knee: 1) dysplasic lateral tibial condyle, 2) progressive depression of the lateral tibial plateau, 3) short fibula, 4) short and contracted fascia lata, 5) short lateral collateral ligament and biceps femoris, 6) short lateral head of gastrocnemius, and 7) contracted lateral knee capsule and lateral retinaculum. In many aspects the pathology of Blount’s disease grade 5 or 6 is similar, but located at the medial tibia causing genu vara.

Materials and Methods: Three valgus knees of EVC syndrome and two varus knees of Blount’s disease grade 5 & 6 underwent surgical treatment by a unique surgical approach to address all pathologies which contribute to the deformity. In the cases of EVC syndrome the first stage operation included: 1) peroneal nerve release and soft tissue release including T.F.L., B.F., L.C.L., capsule and gastrocnemius, 2) arthrogram, 3) fibulectomy, 4) elevation of lateral tibial plateau with bone graft, 5) reconstruction of L.C.L. and B.F., 6) lateral release of retinacula and rerouting of patella, and 7)cast application. In the second stage operation of these cases a corrective high tibial osteotomy was performed. In the Blount’s disease knees the operative treatment was performed in one stage and included: 1) arthrogram, 2) elevation of the medial tibial plateau, 3) fibulotomy, 4) closing wedge tibial osteotomy based laterally, 5) transfer of the bony wedge under the elevated plateau and fixation.

Results: All deformities were corrected with no recurrence, and stability of the knees persisted. We had one common peroneal nerve neuropraxia that recovered and one wound dehiscence.

Conclusions: In both conditions, EVC syndrome and Blount’s disease, corrective high tibial osteotomy does not address the pathology, and recurrence is to be expected. The described surgical technique fulfills that target – eradication of the pathologic elements that lead to valgus or varus.