Bilateral Toe Walking as Presentation of Unilateral Dysplasia Epiphysealis Hemimelica of the Ankle: A Case Report.

CASE: We describe a case of dysplasia epiphysealis hemimelica (DEH) of the anterior tibiotalar joint that presented as toe walking in a 6-year-old boy. Radiographs and magnetic resonance images showed substantial exostosis at the anterior ankle that blocked dorsiflexion. He underwent surgical excision and casting for equinus, restoring ankle range of motion and gait. CONCLUSION: Although DEH is benign, it can cause major deficits and permanent damage to a joint when neglected. Recognition of subtle presentations of DEH, such as toe walking, is crucial. Early treatment can restore joint motion and prevent deformity and arthritis.

Can Adding BMP2 Improve Outcomes in Patients Undergoing the SUPERhip Procedure?

Congenital femoral deficiency (CFD) Paley type 1b is characterized by severe bony deformity of the upper femur, extra-articular contractures of the hip, and, delayed ossification of the femoral neck and/or subtrochanteric region. The Systematic Utilitarian Procedure for Extremity Reconstruction of the hip (SUPERhip) procedure for the correction of CFD deformities was developed in 1997. Initially, a non-fixed angle device (rush rod) was used for fixation. Late complications of persistent delayed ossification and recurrent varus deformity occurred. In order to reduce and treat such complications, fixation with a fixed angle device and the off-label use of BMP2 to induce ossification of the un-ossified femoral neck were employed. The purpose of this study is to determine if the use of a fixed angle device, and, BMP2 inserted into a drill hole in the cartilage of the femoral neck, decreases the incidence of these late complications. We retrospectively reviewed 72 SUPERhip procedures performed for Paley type 1b CFD between 1997 and 2012. Due to recurrent varus or persistent delayed ossification of the femoral neck, 34 revision SUPERhip procedures were performed. In total, 106 SUPERhip procedures were studied. Sixty-eight SUPERhips were performed using internal fixation without BMP2, while 38 SUPERhips were performed with both internal fixation and the addition of BMP2. Forty-one were performed using non-fixed angle internal fixation while 65 had fixed angle internal fixation. Fixed angle devices significantly reduced the incidence of recurrent varus compared with non-fixed angle devices. Inserting BMP2 in the femoral neck significantly reduced the incidence of persistent delayed ossification. Using only a fixed angle device but no BMP2 did not reduce the incidence of delayed ossification. The combination of both a fixed angle device and BMP2 reduced the incidence of recurrent coxa vara and persistent delayed ossification of the femoral neck. The SUPERhip procedure corrects the pathoanatomy of the proximal femur in CFD Paley type 1b but is associated with a very high risk of recurrence of coxa vara and persistence of femoral neck delayed ossification, unless, a fixed angle internal fixation device is used to prevent recurrent coxa vara and BMP2 is used to induce ossification of the femoral neck.

Cross-Union Surgery for Congenital Pseudarthrosis of the Tibia.

Congenital Pseudoarthrosis of the Tibia (CPT) is a rare condition with a reputation for recurrent fractures and failure to achieve union. A large variety of surgical procedures have been attempted for the treatment of fractured cases of CPT with an average rate of union without refracture of only 50%. Intentional cross-union between the tibia and fibula has been reported to improve these results to 100% union with no refractures. This is a retrospective study of 39 cases of CPT in 36 patients treated by the Paley cross-union protocol with internal fixation, bone grafting, zoledronic acid infusion and bone morphogenic protein 2 (BMP2) insertion. All 39 cases of CPT united at the tibia and developed a cross-union to the fibula. Two patients had a persistent fibular pseudarthrosis, one that was later treated at the time of planned rod exchange and one that has remained asymptomatic. There were few postoperative complications. There were no refractures during the up to 7-year follow-up period. The most common problem was the Fassier-Duval (FD) rod pulling through the proximal or distal physis into the metaphysis (66.7%). This did not negatively affect the results and was remedied at the time of the planned rod exchange. The Paley Cross-Union Protocol is very technically demanding, but the results have radically changed the prognosis of this once sinister disease.

Lengthening Reconstruction Surgery for Fibular Hemimelia: A Review.

Fibular hemimelia (FH) presents with foot and ankle deformity and leg length discrepancy. Many historic reconstructions have resulted in poor outcomes. This report reviews modern classification and reconstruction methods. The Paley SHORDT procedure (SHortening Osteotomy Realignment Distal Tibia) is designed to correct dynamic valgus deformity. The Paley SUPERankle procedure (Systematic Utilitarian Procedure for Extremity Reconstruction) is designed to correct fixed equino-valgus foot deformity. The leg length discrepancy in FH is successfully treated with serial lengthening and epiphysiodesis. Implantable intramedullary lengthening devices have led to all internal lengthenings. Recent advancements in techniques and implants in extramedullary implantable limb lengthening (EMILL) have allowed internal lengthenings in younger and smaller patients, who would traditionally require external fixation. These new internal techniques with lengthenings of up to 5 cm can be repeated more easily and frequently than external fixation, reducing the need to achieve larger single-stage lengthenings (e.g., 8 cm). Modern reconstruction methods with lengthening are able to achieve limb length equalization with a plantigrade-stable foot, resulting in excellent functional result comparable or better than a Syme's amputation with prosthetic fitting.