Coxa Vara Deformity in Fibrous Dysplasia/McCune-Albright Syndrome: Prevalence, Natural History and Risk Factors: A Two-Center Study.

This study aimed to evaluate the prevalence of and risk factors for coxa vara deformity in patients with fibrous dysplasia/McCune-Albright syndrome (FD/MAS). This study was conducted at the National Institutes of Health and Leiden University Medical Center. All patients with any subtype of FD/MAS, FD involving the proximal femur, one or more X-rays available and age <30 years were included. X-rays were scored for the neck-shaft angle (NSA). Varus deformity was defined as NSA <110 degrees or >10 degrees below age-specific values. Risk factors for deformity were assessed by nested case-control analysis, comparing patients and femurs with and without deformity, and by linear mixed effects model, modeling temporal NSA decrease (the natural course of the NSA) in non-operated femurs with two or more X-rays. Assessed variables included growth hormone excess, hyperthyroidism, hypophosphatemia, >25% of the femur affected, calcar destruction, radiolucency, and bilateral involvement. In total 180 patients were studied, 57% female. Mean ± SD baseline age was 13.6 ± 7.5 years; median follow-up 5.4 (interquartile range [IQR], 11.1) years. Sixty-three percent (63%) were diagnosed with MAS. A total of 94 patients were affected bilaterally; 274 FD femurs were analyzed; 99 femurs had a varus deformity (36%). In the nested case-control analysis, risk factors were as follows: presence of MAS (p < 0.001), hyperthyroidism (p < 0.001), hypophosphatemia (p < 0.001), high percentage of femur affected (p < 0.001), and calcar destruction (p < 0.001). The linear mixed effects model included 114 femurs, identified risk factors were: growth hormone excess (ß = 7.2, p = 0.013), hyperthyroidism (ß = 11.3, p < 0.001), >25% of the femur affected (ß = 13.2, p = 0.046), calcar destruction (ß = 8.3, p = 0.004), radiolucency (ß = 3.9, p = 0.009), and bilateral involvement (ß = 9.8, p = 0.010). Visual inspection of the graph of the model demonstrated most progression of deformity if NSA <120 degrees with age < 15 years. In conclusion, in tertiary care centers, the prevalence of FD/MAS coxa vara deformity was 36%. Risk factors included presence of MAS, high percentage of femur affected, calcar destruction, radiolucency, NSA <120 degrees and age < 15 years. © 2023 The Authors. Journal of Bone and Mineral Research published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research (ASBMR).

Value in Scoliosis Surgery: Coordinated Surgical and Anesthetic Techniques Avoid Blood Transfusion without Fibrinolytic Medications or Red Blood Cell Salvage.

STUDY DESIGN: Retrospective chart review. OBJECTIVE: The aim of this study was to document the impact of coordinated surgical and anesthetic techniques on estimated blood loss (EBL) and subsequent need for transfusion. SUMMARY OF BACKGROUND DATA: Scoliosis surgery is typically associated with large quantities of blood loss, and consequently blood transfusion may be necessary. Many strategies have been employed to minimize blood loss, including blood collection with reinfusion ("cell-saver") and the use of antifibrinolytic drugs. We reviewed our experience with methods to minimize blood loss to show that transfusion should be a rare event. METHODS: One hundred and thirty consecutive cases of spine fusion for adolescent idiopathic scoliosis utilizing pedicle screw fixation were reviewed from March 2013 to October 2019. The senior author was the primary surgeon for all cases. Data were collected from the electronic medical record, including age, sex, weight, number of instrumented levels, EBL, total fluids administered during surgery, pre- and postoperative hemoglobin, and procedure duration. RESULTS: The average EBL was 232 ±â€Š152 mL (range 37-740 mL). The average preoperative hemoglobin was 13.4 ±â€Š1.2 g/dL and the average postoperative hemoglobin (last measured before discharge) was 9.0 ±â€Š1.2 g/dL. One patient received a transfusion of 270 mL homologous blood. Blood salvage and reinfusion ("cell-saver") was not used. No patient was managed with antifibrinolytic drugs. CONCLUSION: Minimizing blood loss using a combination of surgical and anesthesia techniques can effectively eliminate the need for blood transfusion. The elimination of costly adjuncts increases the value of a complex orthopedic procedure.Level of Evidence: 5.

The surgical management of fibrous dysplasia of bone.

The surgical management of Polyostotic Fibrous Dysplasia (FD) of bone is technically demanding. The most effective methods to manage the associated bone deformity remain unclear. The marked variation in the degree and pattern of bone involvement has made it difficult to acquire data to guide the surgeon's approach to these patients. In light of the paucity of data, but need for guidance, recognized experts in the management of these patients came together at the National Institutes of Health in Bethesda, Maryland as part of an International meeting to address issues related to fibrous dysplasia of bone to discuss and refine their recommendations regarding the surgical indications and preferred methods for the management of these challenging patients. The specific challenges, recommended approaches, and "lessons learned" are presented in hopes that surgeons faced with typical deformities can be guided in the surgical reconstruction of both children and adults with FD.

Surgical management of fibrous dysplasia in McCune-Albright syndrome.

Polyostotic Fibrous Dysplasia associated with McCune-Albright Syndrome presents extreme challenges for the treating physician. The bone deformities, frequent fractures and weight bearing bone pain all combine to adversely affect the child's ability to engage in the normal activities of childhood. All too often the family is told that there is no real treatment for the bone disease. As a result of the rare nature of this syndrome, few orthopedic surgeons have had extensive experience with reconstructions for the skeletal deformities. Early surgery to address the upper femoral deformity and the innovative use of off-label internal fixation devices can have a profound effect on these young patients. The parents and patient must have a complete understanding of the effect of further growth on the reconstructions and be prepared to undergo repeated procedures as the deformities change and recur. Bone grafting and the use of common internal fixation devices (plates and screws) are almost always doomed to early failure. The innovative use of intramedullary devices can lead to prolonged ambulation and the eradication of weight bearing bone pain in selected patients. Frequent fractures and deformity can be effectively managed in most patients with these techniques.

Transphyseal fracture-dislocation of the femoral neck: a case report and review of the literature.

We describe a case of transphyseal hip fracture-dislocation in a 7.5-year-old patient who was treated initially by open reduction and internal fixation. Soon after the injury, the femoral head developed avascular necrosis. The treatment was focused on maintaining adequate hip range of motion and providing femoral head containment with a combined subtrochanteric femoral osteotomy and shelf acetabuloplasty. The patient's young age and good hip remodeling potential contributed to the favorable clinical outcome 3 years after the injury. The long-term prognosis remains guarded, however.

Factors influencing the development of osteonecrosis in patients treated for slipped capital femoral epiphysis.

BACKGROUND: Osteonecrosis is a serious complication of the treatment of slipped capital femoral epiphysis. The purpose of the present study was to identify factors influencing the development of osteonecrosis. METHODS: Two hundred and forty patients who had been treated for slipped capital femoral epiphysis between 1965 and 1999 were retrospectively evaluated. Treatment included stabilization with a spica cast or fixation with one to four pins or screws. Radiographs that had been made at the time of presentation, before and after the operation, and at consecutive follow-up examinations were reviewed. Osteonecrosis was defined retrospectively on the basis of radiographic evidence of sclerosis and collapse of the femoral head. The risk of development of osteonecrosis was correlated with various clinical and radiographic parameters. RESULTS: All twenty-one patients in whom osteonecrosis developed had presented with an unstable slipped capital femoral epiphysis. None of the 204 patients who had presented with a stable slipped capital femoral epiphysis, regardless of grade, had development of osteonecrosis. In the group of patients who had presented with an unstable slipped capital femoral epiphysis, the risk of development of osteonecrosis increased with the severity (grade) of the slip. Osteonecrosis was more likely to develop in patients who had been treated with multiple pins than in those who had been treated with a single cannulated screw. CONCLUSIONS: Patients who have a stable slipped capital femoral epiphysis are not at risk for the development of osteonecrosis when treated with pinning in situ. Patients who have an unstable slipped capital femoral epiphysis have a decreased risk of osteonecrosis when treated with pinning in situ. Complete or partial reduction of an unstable slipped capital femoral epiphysis increases the risk of development of osteonecrosis. Pinning in situ without reduction with a single cannulated screw is the method of choice for the treatment of a slipped capital femoral epiphysis.

Multicentric giant cell tumor of bone: a case report and review of the literature.

Multicentric giant cell tumor of bone is the rare variant of a lesion that is relatively common in a skeletally mature population. An otherwise healthy 13-year-old boy presenting with this entity was followed up for 6 years. During this period, the patient was diagnosed with and treated for six individual lesions. One recurrence required resection, Ilizarov bone lengthening, and subsequent ankle arthrodesis. He remains fully active and free of distant metastasis.

Aneurysmal bone cyst in 29 children.

The purpose of this study was to review longitudinally the clinical features, method of treatment, and recurrence rates of 40 cases of aneurysmal bone cyst in children treated at one institution. Twenty-nine patients with histologic confirmation of the diagnosis and minimum follow-up of 2 years were included. Thirteen patients were less than 10 years of age, and 16 were in the second decade of life. The most frequent location of the lesion was the tibia (seven cases). Patients were treated with curettage, curettage and bone grafting, or resection. The overall recurrence rate was 27.5%. Five lesions recurred once, and three recurred twice. The average time before recurrence was 18.7 months. Complications occurred in six cases, three of them with physeal arrest. The lesion occurred more commonly in females in the second decade of life and was not associated with a pre-existing tumor. The recurrence rate dropped after the use of a high-speed bur.