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 PROCEDURE­INTERTROCHANTERIC 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 PROCEDURE­HIP 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.

Internal fixation after fracture or osteotomy of the femur in young children with polyostotic fibrous dysplasia.

Children from 4 to 7 years of age with polyostotic fibrous dysplasia (PFD) may need internal fixation of the femur for either fracture or osteotomy. At that age, the small size of the femur allows only the use of small intramedullary nails. However, titanium elastic nails and rigid intramedullary pediatric interlocking femoral nails - good for fracture or osteotomy fixation in the normal femur - are not indicated in PFD. From 2009 to 2011, we treated eight cases of PFD femoral fracture and deformity by internal fixation with a custom-modified adult humeral nail to which a spiral blade was connected. The 7-mm thick nail fit properly into the small femoral shaft and the spiral blade conferred ideal mechanical support to the femoral neck. Three cases had replacement of the humeral nail with a more appropriate adult femoral nail 3 years after the index procedure when the femur had reached an adequate size. In the maximum follow-up period of 4 years, few complications were observed.

Valgus extension femoral osteotomy to treat "hinge abduction" in Perthes' disease.

PURPOSE: "Hinge abduction" is a complication of Perthes' disease caused by impingement of the extruded superolateral portion of the femoral head against the lateral lip of the acetabulum. Catterall first described femoral valgus extension osteotomy (VGEO) to treat this condition. We report the results of this operation in 16 cases of Perthes' disease with "hinge abduction". METHODS: Sixteen hips in 16 patients affected by Perthes' disease and "hinge abduction" were operated on at a mean age of 10.1 years and followed up an average of 6.5 years later. Before surgery, the mean Iowa hip score was 44.4 points. Preoperative radiographs were taken with the affected hip in maximum adduction in order to calculate the amount of valgus correction. The osteotomy was performed between the greater and the lesser trochanter, and it was fixed with a hip plate. RESULTS: All the osteotomies healed uneventfully. At follow-up, no patient complained of pain and hip abduction ranged from 20° to 45°. Four out of the 16 patients had a moderate limp, and 12 had an improvement in gait pattern compared to preoperatively. At follow-up, the Iowa hip score totaled a mean of 83.6 points, with a statistically significant improvement in comparison to the preoperative evaluation. At follow-up, two hips were classified as Stulberg II-III, ten hips as Stulberg III, and four as Stulberg IV. CONCLUSIONS: In our hands, VGEO was an effective procedure to treat "hinge abduction" in severe Perthes' disease with satisfactory results. The main limitation of our study is its short follow-up.