Bilateral Femoral Neck Pseudarthrosis in an Adolescent Girl With Congenital Spondyloepiphyseal Dysplasia. The Treatment Journey: A Case Report.

CASE: We describe the surgical management of a girl with dwarfism and congenital spondyloepiphyseal dysplasia, who presented in adolescence with coxa vara and bilateral pseudarthrosis between the femoral neck and the diaphysis, with asymmetric distal migration at both sites and leg length discrepancy. The patient at 16 years underwent valgus osteotomy in situ and femoral neck fixation in the left hip. The right hip was operated 19 months later. CONCLUSION: Hip dysplasia is common in spondyloepiphyseal dysplasia, but a debilitating nontraumatic bilateral pseudarthrosis at the base of the femoral neck has not been previously reported. Valgus corrective osteotomies of the femur in situ led to union and allowed the patient to walk.

A novel mutation in the proteoglycan 4 gene causing CACP syndrome: two sisters report.

BACKGROUND: Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome, caused by biallelic pathogenic mutations in the proteoglycan 4 (PRG4) gene, is characterized by early-onset camptodactyly, noninflammatory arthropathy, coxa vara deformity, and rarely, pericardial effusion. This syndrome can mimic juvenile idiopathic arthritis. CACP syndrome is caused by mutations in the proteoglycan 4 (PRG4) gene. To date, only 36 pathogenic mutations have been reported in this gene, but none have been reported from Azerbaijan. CASE PRESENTATION: Herein, we report two siblings presented with chronic polyarthritis, had a prior diagnosis of juvenile idiopathic arthritis, but was subsequently diagnosed as CACP syndrome with novel mutation in the PRG4 gene. CONCLUSION: Our report expands the knowledge of PRG4 mutations, which will aid in CACP patient counseling.

[A rare case of bilateral femoral neck fracture in a 5-year-old girl with autosomal dominant osteopetrosis type 2]. / Seltener Fall eines beidseitigen metaphysären Ermüdungsbruches des Schenkelhalses bei einem 5­jährigen Mädchen mit autosomal-dominant vererbter Osteopetrose Typ 2.

The rare case of a 5­year-old girl with autosomal dominant osteopetrosis type 2, who suffered metaphyseal fractures of the femoral neck on both sides within 6 months is described. On the right side, the diagnosis was made 3 months after the onset of symptoms, so that a coxa vara occurred. The treatment was surgically treated through a valgus osteotomy with fixation of the femoral head with K­wires. Three months after the operation, the girl complained of a painful restriction of movement on her left side. Radiologically, a metaphyseal femoral neck fracture without coxa vara was diagnosed and in situ fixated with 2 K wires. Two months after the second operation, there was a symmetrical free range of motion of the hips with no symptoms. The metaphyseal femoral neck fracture with verticalization of the growth plate is a serious disease in autosomal dominant osteopetrosis due to the development of a coxa vara, which, if diagnosed at an early stage, can be treated well with in situ fixation. If the coxa vara has already developed, a valgus osteotomy should be performed despite the risk of delayed bone healing.

Pediatric patient with a bilateral Salter-Harris II fracture and slipped capital femoral epiphysis secondary to autosomal recessive osteopetrosis.

Treatment of femoral neck fractures secondary to osteopetrosis is an uncertain and puzzled decision. Experience in the treatment, especially in the pediatric population, is scarcely reported. The duration of conservative treatment is prolonged and poses the risks of non-union and development of coxa vara deformity. The recommended treatment is closed reduction and internal fixation; however, surgery on osteopetrotic bone is challenging due to defective bone marrow function, delayed consolidation and higher risk of intraoperative fractures. Slipped capital femoral epiphysis secondary to osteopetrosis is very rarely reported. This article presents the case of a 5-year-old female patient with rapidly deteriorating physical function due to bilateral proximal femoral Salter-Harris type II fractures with associated slippage of the growth plates secondary to confirmed autosomal recessive osteopetrosis. Operative treatment was performed in a tertiary level orthopedic center with closed reduction and internal fixation with cannulated screws. A loss of fixation with coxa vara deformity was seen on the left side 7 months postoperatively with increasing pain. A revision surgery with reosteosynthesis and a valgus osteotomy was thus performed which showed good subjective and objective results 1 year postoperatively with complete bony union.

Single-stage surgical correction of a bilateral acquired coxa vara deformity in a young woman.

A 23-year-old female-treated patient of osteomalacia and secondary hyperparathyroidism with hypophosphatemia presented with a 5-year history of bilateral groin pain and stiffness of both hips and difficulty in walking. Plain radiographs of the pelvis showed bilateral coxa vara deformity. She was managed surgically by a single-stage bilateral subtrochanteric corrective osteotomy with the internal fixation. After the osteotomy healing at 3 months, the patient was pain free and walked comfortably with an increased range of hip motion.

Can we predict failure of percutaneous fixation of femoral neck fractures?

PURPOSE: This study evaluated a series of geriatric femoral neck fracture treated with closed reduction percutaneous pinning (CRPP) at a single level-1 trauma center to determine if there are any simple, reliable, radiographic characteristics that can be used to predict increased risk of post-operative failure in nondisplaced and valgus impacted fracture patterns. METHODS: We conducted a retrospective cohort study of all patients with femoral neck fractures (AO/OTA 31B) who underwent CRPP over a 12-year period at a single Level 1 trauma center. Failure was defined as radiographic failure within the first year after the index operation requiring revision surgery. Common patterns identified on initial review were the presence of a visible medial transcervical line (MTL) felt to indicate a tension-sided failure, a straight inferior calcar (SIC) indicating severe valgus impaction, and quality of intra-operative screw positioning. X-rays of patients were then reviewed for these characteristics in a blinded manner by three different trauma-fellowship trained orthopedic surgeons. Inter-rater reliability was calculated using Fleiss' Kappa Coefficient. Comparisons of failure rates between groups were made using a Fisher's Exact test. RESULTS: 139 patients who underwent CRPP for a femoral neck fracture and follow-up for at least 90 days were identified and reviewed. There were a total of 19 failures (13.6%) within one year. The patients with a varus fracture had a failure rate of 9/24 (37.5%). Of the valgus/nondisplaced fractures, MTL was identified in 42/115 (36%) patients. Inter-rater agreement was high for the presence of an MTL (84%, Kappa 0.69). Patients with an MTL had a fourfold increase in risk of failure (7/42=17% with an MTL vs. 3/73=4% without, p  0.03). The presence of a SIC and quality of screw placement were not predictive of failure. CONCLUSION: Varus femoral neck fractures fixed with CRPP have a high rate of failure (37.5%). Nondisplaced or valgus impacted fractures with the presence of a visible medial transcervical line on pre-operative radiographic imaging resulted in a fourfold increase in the risk of failure after CRPP. Identification of the MTL will help treating surgeons better council patients when making pre-operative decisions between arthroplasty and CRPP.

Adult Idiopathic Bilateral Coxa Vara with Hip Osteoarthritis Treated with Bilateral Proximal Femur Osteotomy: A Case Report.

CASE: We reported a case of a 25-year-old woman with idiopathic bilateral coxa vara who had initial presentation of hip osteoarthritis. She was later treated with bilateral subtrochanteric valgus osteotomy. A good functional outcome was recorded without nonunion or deformity recurrence. The arthritis of the hips also decelerated. CONCLUSIONS: Coxa vara first diagnosed in adulthood was relatively uncommon, and the cause in this present case was uncertain. Subtrochanteric valgus osteotomy seemed to be a suitable treatment for this case.

Schmid Type Metaphyseal Chondrodysplasia with a Novel COL10A1 Mutation.

Schmid type metaphyseal chondrodysplasia (SMCD) is a rare skeletal dysplasia, characterized by short stature, short limbs, bowing of the legs, and radiographic features of metaphyseal irregularities with fraying and splaying, more severe at the knee. It is caused by mutations of the COL10A1 gene. The authors present an Indian patient with a novel COL10A1 gene mutation.

Long-term Outcomes of Operative and Nonoperative Treatment of Congenital Coxa Vara.

BACKGROUND: Congenital coxa vara (CCV) is a rare hip condition with few long-term studies. The purpose of this study was to assess clinical, radiographic, and functional outcomes after operative and nonoperative treatment of CCV, assess reliability of radiographic parameters, and investigate risk factors for recurrence after surgery. METHODS: Retrospective review was performed of all CCV patients treated at 1 institution from 1980 to 2010. In addition, patients were recalled for additional follow-up x-rays, modified Harris Hip Score (mHHS), and gait analysis. Radiographic measurements [neck-shaft angle (NSA), head-shaft angle (HSA), Hilgenreiner-epiphyseal angle (HEA), and femoral neck length (FNL)] were assessed for reliability using intraclass correlation coefficients. Multivariate analysis was performed to identify risk factors for recurrence after surgery. RESULTS: Forty-six hips in 32 patients were reviewed. Mean age at presentation was 5.4±4.9 years. Mean follow-up was 11.8±5.8 years. Valgus proximal femoral osteotomy was performed in 27 hips (20 patients). Initial deformity was greater in the operative group (NSA 90±17 degrees, HEA 68±19 degrees) versus nonoperative patients (NSA 122±19 degrees, HEA 34±14 degrees) (P<0.0001), but radiographic outcomes were similar at follow-up. Most nonoperative hips had normal FNL growth rates (80%), but resolution of varus NSA occurred in only 21%. In contrast, 56% of operative hips showed decreased FNL growth rates. Interobserver reliability was excellent for HEA (0.98), NSA (0.90), and FNL (0.89), and good for HSA (0.79). Repeat osteotomy was performed in 6 cases (22%). No significant predictors for recurrence were identified. At long-term follow-up for recalled patients, 72% had significantly abnormal gait, and 50% had fair-poor functional outcomes (mHHS<79). CONCLUSIONS: Valgus osteotomy corrects severe deformity in CCV with improved clinical and radiographic outcomes. HEA and NSA are the most reliable radiographic measurements of proximal femoral deformity in CCV. Recurrence is not uncommon, but no predictors were identified. Many patients have persistent gait abnormalities and functional impairment at long-term follow-up, regardless of prior treatment. LEVEL OF EVIDENCE: Level III-retrospective cohort.

The orientation of the surgical epicondylar axis varies in varus and non-varus knees in the coronal plane.

PURPOSE: To investigate the orientations of the surgical epicondylar axis (SEA) of varus and non-varus knees in the coronal plane. METHODS: One-hundred and sixty-two knees from 81 Chinese patients undergoing total knee arthroplasty (TKA) were retrospectively investigated. The angle between the medial side of the femoral mechanical axis and the SEA (MA-SEA), as well as the physiological valgus angle, was measured in the coronal plane using three-dimensional reconstruction. The joint line angle (JLA) and hip-knee-ankle angle (HKAA) were measured in long-leg weight-bearing radiographs. The mean of each parameter was compared between the varus (HKAA < 177.0°) and the non-varus knees (HKAA ≥ 177.0°) using an independent t test. Linear regression was used to assess the correlation between MA-SEA with JLA and HKAA. RESULTS: A total of 42 non-varus knees (6 valgus and 36 neutral knees) and 98 varus knees were measured, as 22 knees were abandoned due to unrecognizable bony landmarks. The mean MA-SEA and JLA were significantly larger in non-varus knees (both, p < 0.01). The mean physiological valgus angle was 5.9 ± 1.0° for Chinese TKA patients and was significantly larger in varus knees (p < 0.01). There was a strong positive correlation between the MA-SEA and JLA (R 2 = 0.35, p < 0.05). CONCLUSIONS: There were significant differences in the orientation of the SEA between varus and non-varus knees, which was strongly correlated with the orientation of the femoral joint line. These findings will enhance the current knowledge of knee anatomy and should prove useful for coronal alignment in TKA. LEVEL OF EVIDENCE: III.

Management of Coxa Vara Septica and Pseudoarthrosis of the Femoral Neck: A Case Report.

BACKGROUND Coxa vara and pseudoarthrosis of the femoral neck after septic hip arthritis is a very rare disease. The aim of this study was to present a case with pseudoarthrosis of the femoral neck, with coxa vara after neonatal septic arthritis of the right hip, and treatment of the pseudarthrosis with proximal femoral valgus osteotomy. CASE REPORT A 12-month-old female, who had suffered neonatal septic arthritis with a five-day history of fever and painful right hip now demonstrated painless limping to the right hip, pelvic obliquity, Trendelenburg-Duchenne gait, limitation of hip abduction, lower-extremity length discrepancy with 3.0 cm of shortening of right femur, and walking on her tiptoes. In addition, a viable femoral head, coxa vara, and pseudoarthrosis of the femoral neck were observed. At the age of 26-months, the patient had a realignment valgus osteotomy of the proximal femur and adductor tenotomy was performed. At the last follow-up postoperative evaluation, at the age of four and a half years, the patient showed successful consolidation of pseudoarthrosis, correction of Trendelenburg gait, restored right lower extremity alignment, and corrected lower-extremity length discrepancy. CONCLUSIONS In the case of coxa vara and pseudoarthrosis of the femoral neck, realignment of the proximal femoral valgus osteotomy and bone grafting of the pseudoarthrosis resulted in ossification of the femoral neck as the epiphyseal plate was placed at right angle to the compressive forces.

Outcome of extensive varus and valgus stem alignment in short-stem THA: clinical and radiological analysis using EBRA-FCA.

INTRODUCTION: The principle of implanting a calcar-guided short stem consists of an individual alignment alongside the medial calcar providing the ability of reconstructing varus and valgus anatomy in a great variety. However, still, there are broad concerns about the safety of extensive varus and valgus positioning in regard to stability, bony alterations, and periprosthetic fractures. MATERIALS AND METHODS: 216 total hip arthroplasties using a calcar-guided short stem (optimys, Mathys Ltd.) in 162 patients were included. Depending on postoperative CCD angle, hips were divided into five groups (A-E). Varus- and valgus tilt and axial subsidence were assessed by "Einzel-Bild-Roentgen-Analyse"(EBRA-FCA, femoral component analysis) over a 2-year follow-up. The incidence of stress-shielding and cortical hypertrophy as well as clinical outcome [Harris Hip Score (HHS)] were reported. RESULTS: Postoperative CCD angles ranged from 117.9° to 145.6° and mean postoperative CCD angles in group A-E were 123.3°, 128.0°, 132.4°, 137.5°, and 142.5°, respectively. After 2 years, the mean varus/valgus tilt was -0.16°, 0.37°, 0.48°, 0.01°, and 0.86°, respectively (p = 0.502). Axial subsidence after 2 years was 1.20, 1.02, 1.44, 1.50, and 2.62 mm, respectively (p = 0.043). No periprosthetic fractures occurred and none of the stems had to be revised. Rates of stress-shielding and cortical hypertrophy as well as HHS showed no significant difference between the groups. CONCLUSIONS: Valgus alignment results in increased subsidence but does not affect the clinical outcome. There is no difference in stress shielding and cortical hypertrophy between the groups. The authors recommend long term monitoring of valgus aligned stems.

Coxa vara in postseptic arthritis of the hip in children.

The behavior and treatment of coxa vara and pseudarthrosis of the proximal femur secondary to sepsis is not well described. The aim of this study is to describe the pathoanatomy for coxa vara and pseudarthrosis in postseptic hips, evaluate progression of neck shaft angle (NSA), and discuss treatment. This is a retrospective case series of 20 patients (21 hips). There were 11 hips with predominant avascular necrosis of the capital femoral epiphysis without pseudarthrosis (type 1) and 10 with pseudarthrosis (type 2). The interobserver κ value was 0.79. There was a decrease in NSA from 110.3° to 99.3° during an average follow-up duration of 5.2 years (range: 2-14 years). The average change in NSA between the initial presentation and the final follow-up was 5.5° in type 1 and 17.1° in type 2. Nine patients underwent a surgical intervention. In cases where subtrochanteric valgus osteotomy was performed, the mean preoperative NSA was 94° and the mean NSA at the final follow-up was 128°; all operated pseudarthroses healed without bone grafting. Acetabuloplasty is not necessary in most cases.

Varus will have varus; a radiological study to assess and predict varus stem placement in uncemented femoral stems.

BACKGROUND: Varus inclination of the uncemented stem is not necessarily a technical error. The proximal femoral anatomy of hips with a coxa vara deformity frequently predisposes varus inclination. METHODS: We reviewed a series of 200 patients undergoing primary uncemented THA with the Corail® hip system. Preoperative data were based on patient demographics, diagnosis, and radiographic information (preoperative templating-CT measurements), and compared postoperative alignment for each stem and type of stem used. Proximal femoral traits which can alert surgeons, when templating preoperatively, to potential varus alignment were noted. RESULTS: All stems were inserted either in neutral or varus alignments. Low neck shaft angle is strongly predictive of increased varus stem alignment (p<0.001). Stems inserted with higher varus alignment were associated with the preoperative morphological traits associated with coxa vara hip deformities - increased femoral offset (p<0.001), greater trochanteric overhang (p<0.001), greater trochanteric height (p<0.046), and a lower canal flare index (p<0.046). CONCLUSIONS: Varus stem alignment is neither unexpected nor necessarily a technical failure when using this particular uncemented stem system. Coxa vara deformities, due to a combination of morphological traits, are more likely to be inserted with higher varus alignment than hips with normal or higher neck shaft angles. Surgeons need to be aware of this when carrying out preoperative templating and intraoperative assessment, in order to prevent over-compensation for offset, length or stability.

Acute, unstable slipped capital femoral epiphysis with associated congenital coxa vara.

Slipped capital femoral epiphysis in patients younger than 10 years is rare and is often associated with some identifiable metabolic or endocrinologic abnormality. We present a case of a 5-year-old girl with an acute, unstable, severe slipped capital femoral epiphysis associated with congenital coxa vara and its surgical management. This association has not been described in previous literature. Surgical treatment is proposed and described.

Two-stage surgical treatment of complex femoral deformities with severe coxa vara in polyostotic fibrous dysplasia.

BACKGROUND: In patients with polyostotic fibrous dysplasia with extensive femoral involvement, severe coxa vara may cause complex femoral deformities that are difficult to treat with a single-stage surgical procedure. We evaluated the results of treatment of such patients with a two-stage procedure. METHODS: Eleven patients with polyostotic fibrous dysplasia and severe coxa vara (including one who required bilateral treatment and one who required repeat treatment) were treated surgically at a mean age of fourteen years and four months. A two-stage surgical procedure was planned to correct the deformity. The first stage involved correction of the coxa vara and fixation with a hip plate. The second stage involved correction of a shepherd's crook deformity if present and definitive fixation with an interlocking cervicodiaphyseal nail. All patients were evaluated clinically and radiographically at a mean of four years and seven months after the second-stage procedure. RESULTS: The femoral neck-shaft angle averaged 83° before surgery and was corrected to a mean of 130° after the first-stage procedure. In two patients, cutout of the hip screw-plate caused acute postoperative loss of correction (by 40° in one patient and 20° in the other). After the second-stage procedure, the neck-shaft angle was either fully corrected or improved and the shepherd's crook deformity was fully corrected in all patients. At the time of the latest follow-up, the mean neck-shaft angle was 124°, with a mean loss of correction of 5° relative to the angle measured at the end of the second-stage procedure. The mean estimated blood loss was 625 mL in the first stage and 979 mL in the second. CONCLUSIONS: The two-stage surgical procedure designed to treat patients with polyostotic fibrous dysplasia with complex femoral deformity and severe coxa vara restored a nearly normal femoral alignment that was maintained at a mean of four years and seven months of follow-up. The clinical benefits were pain relief in all of the patients and gait normalization or improvement in most. The estimated blood loss was substantial in both stages. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Three-dimensional measurement of femoral neck anteversion and neck shaft angle.

We present a three-dimensional measurement technique for femoral neck anteversion and neck shaft angles which do not require alignment of the femoral and scanner axes. Two assessors performed the measurements on 11 patients (22 femurs). Repeatability between assessors was 2.7 degrees for femoral neck anteversion and 4.8 degrees for neck shaft angle. Measurements compared with an alternative single slice method were different by 2 degrees (3 degrees) in average. The method was repeatable and appropriate for clinical practice.