Two-Stage Surgical Treatment of Complex Femoral Deformities with Severe Coxa Vara in Polyostotic Fibrous Dysplasia.

INTRODUCTION: For complex femoral deformities in patients affected by polyostotic fibrous dysplasia and coxa vara associated with shepherd's crook deformity of the femoral shaft, the correct treatment is stabilization with an interlocking cervicodiaphyseal nail after correction of all of the femoral deformities. STEP 1 THE FIRST-STAGE PROCEDURE—INTERTROCHANTERIC VALGUS OSTEOTOMY AND FIXATION WITH A HIP BLADE-PLATE PREOPERATIVE PLANNING: Evaluate the patient radiographically and select the hardware. STEP 2 PATIENT PREPARATION: Position the patient on the traction table. STEP 3 PREPARATION OF THE LATERAL ASPECT OF THE PROXIMAL PART OF THE FEMUR AND MARKING THE POSITION FOR BOTH THE OSTEOTOMY AND THE HIP BLADE-PLATE: Expose the lateral aspect of the proximal part of the femur and mark the position of both the blade-plate and the osteotomy. STEP 4 BLADE-PLATE POSITIONING OSTEOTOMY AND OSTEOTOMY FIXATION: Prepare the slot for the blade, perform the osteotomy, and correct the valgus alignment of the proximal part of the femur. STEP 5 POSTOPERATIVE MANAGEMENT: Check postoperative bleeding, provide blood transfusions if needed, and have the patient resume walking. STEP 6 THE SECOND-STAGE PROCEDURE—HIP PLATE REMOVAL OSTEOTOMY OF THE FEMORAL SHAFT TO CORRECT THE SHEPHERD’S CROOK DEFORMITY AND STABILIZATION WITH A CERVICODIAPHYSEAL NAIL PREOPERATIVE PLANNING: Study the patient radiographically, simulate the osteotomies, perform another assessment of lower-limb length, and select the hardware. STEP 7 PATIENT PREPARATION PLATE REMOVAL AND DRILLING OF KIRSCHNER WIRES TO MARK THE SITE OF THE PLANNED OSTEOTOMIES: Position the patient on the traction table and expose the lateral aspect of the femur from the tip of the greater trochanter to the level of the most distal planned osteotomy. STEP 8 PERFORM ALL OF THE OSTEOTOMIES AND DRILL A MEDULLARY CANAL IN THE OSTEOTOMY FRAGMENTS: All of the osteotomies are performed, and a medullary canal is drilled in each femoral fragment. STEP 9 NAIL AND SPIRAL BLADE INSERTION STABILIZATION OF OSTEOTOMIES AND CLOSURE: Introduce the nail into the osteotomy fragments and insert the spiral blade and distal locking screws. STEP 10 POSTOPERATIVE MANAGEMENT: Check postoperative bleeding, which is usually marked; administer blood transfusions as needed; and have the patient resume walking.
RESULTS: We performed this surgery on 13 femora in 11 patients.