Functional and Radiological Outcomes of Surgically Treated Coxa-vara in Children.

BACKGROUND: Coxa vara is a condition in which the neck-shaft angle of the femur decreases below its normal value (<110 degrees) based on age. The deformity can be primarily caused by a congenital defect of the femoral neck cartilage or by secondary causes related to skeletal dysplasia, trauma, infection, and pathological bone disorders. The surgical treatment aims to restore the normal anatomy, thereby improving the mechanics and function of the hip joint. Generally, the HE angles should be corrected to <35 to 40 degrees, converting the shear forces into compressive forces. Our study aims to report the surgical outcome of coxa vara with various pathologies using radiographic measurement and functional scoring. METHODS: Thirty-eight hips in 28 patients who underwent surgery for treatment of coxa-vara from 2013 to 2022 were included. Radiographic parameters like Hilgenreiner-epiphyseal angle and neck-shaft angle were assessed preoperatively and at the latest follow-up. Harris hip score was used for functional outcome. RESULTS: The mean age was 11.13 (5 to 18). The average follow-up was 48 (ranging from 24 to 96) months. The mean preoperative neck-shaft angle was 82 degrees, and the Hilgenreiner-epiphyseal angle was 71 degrees. At the latest follow-up, the mean neck-shaft angle was 127 degrees, and the Hilgenreiner-epiphyseal angle was 33 ( P <0.001). The Harris hip score improved from 63 preoperatively to 92 at the latest follow-up ( P <0.001). There was no recurrence of deformities at the mean follow-up of 37.87 months. Four complications occurred with k-wire fixation in whom revalgus osteotomy was done. CONCLUSIONS: Surgical correction of coxa vara in various pathologies can be done successfully with the Hilgenreiner-epiphyseal angle corrected to ≤ 35 degrees or the neck shaft angle corrected to >110 degrees to prevent deformity recurrence. The majority of the patients reported improvement in hip function. However, a longer-term follow-up is required to determine further outcomes regarding the recurrence of the deformity.

Treatment of Delbet II/III Pediatric Femoral Neck Fractures With Proximal Femoral Locking Plate Versus Cannulated Screws.

INTRODUCTION: Complications following operative treatment of pediatric femoral neck fractures include nonunion, coxa vara, and avascular necrosis (AVN). Proximal femoral locking plates (PFLPs) provide a fixed-angle construct that may reduce the rates of coxa vara, but their use in pediatric femoral neck fractures has not been studied. The purpose of this study was to evaluate rates of union, coxa vara, and AVN in traumatic pediatric femoral neck fractures treated with PFLP or cannulated screws (CS). METHODS: We retrospectively reviewed all traumatic, nonpathologic Delbet II/III femoral neck fractures in patients below 18 years of age treated with PFLP or CS. All cases had ≥6 months of radiographic follow-up to evaluate for osseous union and AVN. Changes in proximal femoral alignment were determined by measuring injured and contralateral femoral neck-shaft angle and articulotrochanteric distance (ATD) between 6 and 12 months postoperatively. RESULTS: Forty-two patients were identified with mean age at surgery of 10.7±2.9 years (range 3.3 to 16.3 years) and mean follow-up of 36±27 months. Sixteen patients (38%) underwent PFLP fixation, whereas 26 patients (62%) underwent CS fixation. When compared with the CS cohort, the PFLP cohort had a greater proportion of males (87.5% vs. 50%, P =0.02) and Delbet III fractures (68.8% vs. 15.4%, P <0.001). There was no difference between PFLP and CS cohorts with respect to rates of union (81% vs. 88%, respectively, P =0.66), AVN (25% vs. 35%, respectively, P =0.73), or secondary surgery (62% vs 62%, P =0.95). There was no significant difference in neck-shaft angle between injured and contralateral hips in those patients treated with PFLP ( P =0.93) or CS ( P =0.16). However, the ATD was significantly decreased in hips treated with CS compared with the contralateral hip (18.4±4.6 vs. 23.3±4.2 mm, P =0.001), with no significant difference in the PFLP group ( P =0.57). CONCLUSIONS: This study suggests that the use of a PFLP in Delbet II/III femoral neck fractures does not appear to significantly increase nonunion rates or AVN and maintains anatomic ATD when compared with screw fixation. LEVEL OF EVIDENCE: Level III-therapeutic study.

Quadruped Gait and Regulation of Apoptotic Factors in Tibiofemoral Joints following Intra-Articular rhPRG4 Injection in Prg4 Null Mice.

Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome leads to diarthrodial joint arthropathy and is caused by the absence of lubricin (proteoglycan 4-PRG4), a surface-active mucinous glycoprotein responsible for lubricating articular cartilage. In this study, mice lacking the orthologous gene Prg4 served as a model that recapitulates the destructive arthrosis that involves biofouling of cartilage by serum proteins in lieu of Prg4. This study hypothesized that Prg4-deficient mice would demonstrate a quadruped gait change and decreased markers of mitochondrial dyscrasia, following intra-articular injection of both hindlimbs with recombinant human PRG4 (rhPRG4). Prg4-/- (N = 44) mice of both sexes were injected with rhPRG4 and gait alterations were studied at post-injection day 3 and 6, before joints were harvested for immunohistochemistry for caspase-3 activation. Increased stance and propulsion was shown at 3 days post-injection in male mice. There were significantly fewer caspase-3-positive chondrocytes in tibiofemoral cartilage from rhPRG4-injected mice. The mitochondrial gene Mt-tn, and myosin heavy (Myh7) and light chains (Myl2 and Myl3), known to play a cytoskeletal stabilizing role, were significantly upregulated in both sexes (RNA-Seq) following IA rhPRG4. Chondrocyte mitochondrial dyscrasias attributable to the arthrosis in CACP may be mitigated by IA rhPRG4. In a supporting in vitro crystal microbalance experiment, molecular fouling by albumin did not block the surface activity of rhPRG4.

[Migration of the Tips of Metal Implants during Treatment of Coxa Vara Adolescentium (Slipped Capital Femoral Epiphysis)]. / Migrace spicek kovových implantátu v prubehu lécby u coxa vara adolescentium.

PURPOSE OF THE STUDY This paper aims to detect, through a retrospective study, the migration of the tips of used metal implants (K-wires or a screw) in the direction out from the proximal femoral epiphysis as a part of studied basic radiometric characteristics of the cohort, with no intention of the authors to evaluate the therapy outcomes. MATERIAL AND METHODS It was a retrospective multicentre study including patients of two orthopaedic clinics and one department of orthopaedics treated in the period 2005-2018. The same treatment procedure was used in all three centres. The "in situ" fixation was indicated in patients, in whom the Southwick angle in anteroposterior and Lauenstein views was not much greater than 30°, whether primarily due to a mild slip or thanks to careful reduction either in acute or acute-on-chronic forms. All X-rays were measured by a single author (M.S.). In AP and Lauenstein view, overlap of the implant tip (K-wire or a screw) above the subcapital growth plate, the height of epiphysis and Southwick angle are measured at the beginning and at the end of treatment. In a smaller group of patients, also the inter-observer error (M.S. and J.P.) was identified. RESULTS K-wire transfixation was used in 43 patients (50 joints), with the mean age of 11.7 years and the mean duration of transfixation of 18.2 months. The slip of the implant tip out of the head, assessed separately for each introduced K-wire and then averaged, was in both views considered statistically significant (in AP view the level of significance was 5% (p-value = 1.393 x 10^(-6) < 0.05) , in Lauenstein view the level of significance was also 5% (p-value = 0.0001652 < 0.05)). The Wilcoxon signed rank test with continuity correction was used. Transfixation by screw alternatively with one K-wire was used in 23 patients (28 joints), with the mean age of 12.4 years and the mean duration of transfixation of 14.4 months. The slip of the screw tip outside the head was assessed as significant (in AP view at the level of significance of 5% (p-value = 9.41 x 10^(-5) < 0.05), in Lauenstein view at the level of significance of 5% (p-value = 0.003557 < 0.05)). The Wilcoxon signed rank test with continuity correction was used. DISCUSSION This paper aims to detect, through a retrospective study, the so-called migration of the tips of used metal implants (K-wires or a screw) outside the femoral head. Smooth and thin implants such as Kirschner wires should not compromise the continuing growth from subcapital growth plate contrary to the AO screw with threads in the femoral head, the screw head rested against the lateral cortical bone and the screw inserted as a compression one. Nonetheless, with some exceptions, the literature confirms the continued growth of the femoral neck even in the case of screws. In general, implants that do not compromise femoral neck growth provide an opportunity to remodel the anterolateral prominence of the femoral metaphysis, especially in younger patients. In agreement with other authors, the data from our study confirmed, even after a short period of time, a certain degree of proximal femoral remodelling expressed by changes in the Southwick angle. CONCLUSIONS Our study confirmed that in the case of "smooth" K-wires as well as cannulated screws the tips of both implants migrate outside the head. The differences were statistically significant. Therefore, the introduction of a conventional cannulated screw cannot be claimed to immediately produce the effect of epiphyseodesis. Yet, smooth implants less compromise the growth of the femoral neck, which is why they have recently been preferred. Key words: coxa vara adolescentium, metal implants, migration.

Retrospective analysis of 35 pediatric femoral neck fractures.

OBJECTIVE: Pediatric proximal femur fracture is extremely rare trauma comparing to other fractures. The proximal femur fracture is 1% of all pediatric fractures. The aim of current study is to compare the incidence of early complications and outcomes of pediatric proximal femur fractures regarding fracture types retrospectively. DESIGN: Our study includes 35 cases which are criticized by Delbet classification system, modalities of treatment, duration of waiting for surgery, duration of follow-up and also complications. Our inclusion criteria are age below 16 years old, proximal femur fractures with no evidence of tumoral, romathologic and methabolic conditions. Age, sex and surgery type (open-closed) were noted, and the data were statistically assessed. Assessment of the final outcome was made at the last follow-up visit using the Ratliff's method. RESULTS: The mean of age of patients in our study is 9.5 ± 5.06 years. The average follow-up was 25.6 ± 13.2 months. 15 patients (42.9%) are type 2 Delbet fracture, 5 patients (14.2%) are Delbet type 3 and 15 patients (42.9%) are Delbet type 4 fracture. Using the Ratliff's method, 25 patients (71.4%) had satisfactory outcomes. Ten patients (28.6%) had unsatisfactory outcomes. The complications as AVN, coxa vara and premature closure of physis, non-union and postoperative infections have been detected in this study. AVN was seen in four (11.4%) patients. In addition, coxa vara was seen in six (17.2%) patients. CONCLUSION: Pediatric femoral neck fractures are extremely rare fractures and can be treated with low complication rates in cases with early treatment and anatomic reduction.

"Lessons from Rare Forms of Osteoarthritis".

Osteoarthritis (OA) is one of the most prevalent conditions in the world, particularly in the developed world with a significant increase in cases and their predicted impact as we move through the twenty-first century and this will be exacerbated by the covid pandemic. The degeneration of cartilage and bone as part of this condition is becoming better understood but there are still significant challenges in painting a complete picture to recognise all aspects of the condition and what treatment(s) are most appropriate in individual causes. OA encompasses many different types and this causes some of the challenges in fully understanding the condition. There have been examples through history where much has been learnt about common disease(s) from the study of rare or extreme phenotypes, particularly where Mendelian disorders are involved. The often early onset of symptoms combined with the rapid and aggressive pathogenesis of these diseases and their predictable outcomes give an often-under-explored resource. It is these "rarer forms of disease" that William Harvey referred to that offer novel insights into more common conditions through their more extreme presentations. In the case of OA, GWAS analyses demonstrate the multiple genes that are implicated in OA in the general population. In some of these rarer forms, single defective genes are responsible. The extreme phenotypes seen in conditions such as Camptodactyly Arthropathy-Coxa Vara-pericarditis Syndrome, Chondrodysplasias and Alkaptonuria all present potential opportunities for greater understanding of disease pathogenesis, novel therapeutic interventions and diagnostic imaging. This review examines some of the rarer presenting forms of OA and linked conditions, some of the novel discoveries made whilst studying them, and findings on imaging and treatment strategies.

Syndrome of progressive deforming non-inflammatory arthritis of childhood: two patients of camptodactyly-arthropathy-coxa vara-pericarditis syndrome.

Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome is a rare familial arthropathy of childhood, commonly misdiagnosed as juvenile idiopathic arthritis. It is characterized by non-inflammatory arthropathy, coxa vara deformity, and sterile pericarditis. We describe two children with CACP syndrome who were referred to the rheumatology clinic for the suspicion of inflammatory arthritis. A literature search was carried out using PubMed/ Medline and Embase databases. English language reports of mutation-proven cases of CACP syndrome reported until 31 March 2020 were retrieved and analysed. Both the children had a delay in diagnosis (age at diagnosis- 12 and 13 years, respectively) and had received immunomodulatory therapy for suspected inflammatory arthritis. Presence of symmetrical arthropathy of large joints, camptodactyly, and normal inflammatory parameters are clues that indicated CACP syndrome. One child with a novel variant in PRG4 also had associated mitral valve prolapse and regurgitation. Both had severe constrictive pericarditis requiring pericardiectomy. On literature review, a total of 98 mutation-proven cases of CACP syndrome have been reported till date. Arthropathy in CACP syndrome mainly involves knees, wrists, ankles, and hips. Pericarditis is usually mild, however, can present rarely with severe symptoms requiring surgical intervention. CACP syndrome can closely mimic inflammatory arthritis and early clinical recognition is important to avoid misdiagnosis. Molecular confirmation is essential for early diagnosis and future genetic counselling for affected families.

Safety and efficacy of tranexamic acid in children with cerebral palsy undergoing femoral varus derotational osteotomy: a double cohort study.

AIMS: The safety and efficacy of tranexamic acid (TXA) in reducing blood loss after total joint arthroplasty and spinal fusion surgery has been well documented. However, little data exist regarding the effectiveness of intraoperative TXA in children with cerebral palsy (CP). The aim of this double cohort study is to investigate the safety and efficacy of intraoperative TXA in reducing blood loss and transfusion requirements for children with CP undergoing a proximal unilateral or bilateral femoral varus derotational osteotomy (VDRO). PATIENTS AND METHODS: A retrospective review was performed of all paediatric theatre lists between May 2012 and January 2019 for all paediatric (< 16 years old) CP patients who underwent unilateral or bilateral VDRO combined with soft tissue release at our institution. Fifty-one patients were included in our study further subdivided into two individual groups, unilateral and bilateral VDRO. RESULTS: No statistically significant differences were found in demographics such as age, weight, ASA, GMFCS and antiepileptic medication between the groups. However, there were significant statistically differences in TBL and transfusion rates between the groups that received TXA and those that did not, both in unilateral [241 ml (TXA) vs. 369 ml (non-TXA)] and bilateral [287 ml (TXA) vs. 467 ml (non-TXA)] operations. CONCLUSION: TXA successfully reduced TBL (in both TXA subgroups) and the transfusion rates without associated complications. TXA's safety and efficacy should be explored further in adequately powered randomized controlled trials.

Preoperative valgus deformity has twice the risk of failure as compared to varus deformity after total knee arthroplasty.

PURPOSE: The aim of this study was to assess whether preoperative valgus or varus deformity affected survivorship after total knee arthroplasty (TKA) and to quantify the risk factors for implant failure in a registry-based population. METHODS: The Emilia-Romagna Registry of Prosthetic Orthopedic Implants was examined regarding TKAs performed on patients with a preoperative diagnosis of valgus or varus deformity. Demographics, implant characteristic and survivorships were investigated and compared. A total of 2327 TKA procedures performed from 2000 to 2016 were included in the study. Six hundred and forty primary TKAs with a diagnosis of valgus deformity were evaluated with a median follow-up of 3.3 years; 1687 primary TKAs with a diagnosis of varus deformity were evaluated with a median follow-up of 2.5 years. RESULTS: Bi-compartmental, cemented posterior stabilised fixed-bearing implants were preferred. For both diagnoses, the implant survivorship rate was greater than 98% in the first year. However, the survival curve of the TKAs implanted for valgus deformity showed a greater slope in the first 3 years as compared to the survival curve of those implanted for varus deformity. Valgus deformity had a 2.1-fold higher risk for revision as compared with varus deformity. Infection was a major cause of implant failure in TKAs for varus deformity, 9/24 (37.5%), while its incidence was lower for valgus deformity, 1/21 (4.8%). CONCLUSIONS: Preoperative valgus alignment showed a twofold risk of failure as compared to varus alignment after TKA. This should be considered in daily practice, and surgeons are called on to pay more attention when performing TKAs on such patients. Prospective randomised controlled trials are, therefore, necessary to better understand the role of preoperative coronal knee deformity in implant failure. LEVEL OF EVIDENCE: Prognostic study, level III.

Valgus Hip Osteotomy in Children With Spondyloepiphyseal Dysplasia Congenita: Midterm Results.

BACKGROUND: Coxa vara has been frequently reported in spondyloepiphyseal dysplasia congenita (SEDC), and proximal femoral osteotomy has been described as a useful treatment. The aim of this study was to discuss the clinical, radiographic, and gait outcomes after valgus extension osteotomy of the proximal femur. Changes of lumbar lordosis, associated with coxa vara correction, are reported as well as the outcome differences between different ages. METHODS: Records of children with SEDC, who were followed at our institution between 2004 and 2014, were reviewed; and children had hip surgery were identified. Hip pain and passive range of motion, radiographic neck shaft angle (NSA), and Hilgenreiner trochanteric (H-T) angle, sagittal spinopelvic parameters, and gait data were recorded. Preoperative and last follow-up data were compared. Outcomes were also compared between 3 age groups. RESULTS: Of the 79 children with SEDC, 26 children (12 boys and 14 girls) had hip osteotomy in 48 hips. Mean age at surgery was 9.6 years and the mean follow-up was 5 years. Preoperative hip pain was noted in 30 hips. At the last follow-up, 3 hips were painful at the extreme range of motion. Passive range of motion, NSA, and H-T improved postoperatively. Although NSA was maintained over the follow-up, H-T deterioration was noted. Spinopelvic measurements changed significantly and gait data remained stable except pelvic tilt that reduced significantly after surgery. The changes of radiographic measurements in each age group were similar to the total group of patients. CONCLUSIONS: In children with SEDC, surgical correction of coxa vara, by proximal femoral valgus osteotomy, is an effective treatment that improves hip pain and range of motion in addition to the radiographic alignment of the proximal femur and the sagittal spinopelvic alignment. Children are expected to maintain their level of function after surgery and to have good results over the midterm regardless of their age at surgery. LEVEL OF EVIDENCE: Level IV-therapeutic study.

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.

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.

Using a Tibial Short Extension Stem Reduces Tibial Component Loosening After Primary Total Knee Arthroplasty in Severely Varus Knees: Long-term Survival Analysis With Propensity Score Matching.

BACKGROUND: Patients with severe preoperative varus deformity have been reported to have high rates of loosening after total knee arthroplasty (TKA), primarily on the tibial side. This study investigated whether a short extension stem for the tibial component in severely varus knees would reduce the failure rate due to loosening on the tibial side. METHODS: Patients who underwent TKA, performed by a single surgeon using a single implant between November 1998 and January 2009, were retrospectively evaluated. Patients diagnosed with primary osteoarthritis, having a hip-knee-ankle axis greater than varus 8° on preoperative long-film radiographs, and postoperatively followed up for more than 2 years were included. Patients were divided into "stem" and "nonstem" groups, followed by 1:1 propensity score matching according to age, gender, body mass index, preoperative mechanical axis, and postoperative alignment. Tibial loosening rates in the 2 groups were compared. RESULTS: The study cohort included 602 patients, divided into "stem" and "nonstem" groups. Propensity score matching yielded 88 pairs of patients. Mean follow-up duration was similar in the stem and nonstem groups (109.22 vs 103.81 months, P = .451). None of the patients in the stem group, compared with 5 in the nonstem group, experienced aseptic loosening. The overall implant survival rate was significantly higher in the stem group than in the nonstem group (P = .0201). CONCLUSION: Using a short extension stem for the tibial component in primary TKA in patients with severe varus deformity greater than 8° may reduce the rate of loosening of the tibial side and increase the longevity of the implant. LEVEL OF EVIDENCE: Level III.

Trochantoplasty for Total Hip Arthroplasty in Patients With Coxa Vara Deformity.

BACKGROUND: In total hip arthroplasty (THA) of hips with coxa vara, the femoral stems might be inserted in a varus alignment. To avoid varus insertion, we designed a technique, which we termed "trochantoplasty." In this procedure, the medial half of the greater trochanter was removed during THA. METHODS: We evaluated 30 patients (31 hips) who had coxa vara deformity and underwent THA using trochantoplasty at the mean follow-up of 5 years (range, 3-9 years). RESULTS: All stems were inserted in the neutral position. One Vancouver type 1 periprosthetic femoral fracture occurred after a fall at postoperative 2 months. At the latest follow-up, the mean power of abductor was 4.3 (range, 3-5). Four patients had moderate limp whereas 26 patients had slight limp. The abduction at 90° flexion ranged from 15° to 45° (mean, 35°). There was no revision. All prostheses had bone-ingrown stability without any detectable wear or osteolysis. The mean Harris hip score was improved from 66.9 to 89.4 points. CONCLUSION: Trochantoplasty can be used to avoid varus insertion of the femoral stem while performing THA in patients with coxa vara deformity without compromising the abductor mechanism.

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.

Transfixing Kirshner wires for fixation of intertrochanteric valgus osteotomies in management of pediatric coxa vara.

BACKGROUND: Coxa vara is a radiological term describing a decrease in the neck-shaft angle to 120° or less. Coxa vara is associated with pathomechanical changes that can manifest clinically. If left untreated, coxa vara can affect the normal development of the pediatric hip. Valgus osteotomy is the standard surgical treatment for coxa vara, but there is no consensus regarding the optimal osteotomy technique and fixation method. The work reported here aimed to highlight transfixing wires as a fixation method for valgus osteotomy applied as treatment for various types of pediatric coxa vara. MATERIALS AND METHODS: This study included 16 cases of pediatric coxa vara with different etiologies in 9 patients with a mean age of 39.9 ± 15.2 months. Radiological and clinical evaluations and scoring of the condition of each patient according to the Iowa Hip Score were performed pre- and postoperatively. Transfixing wires and a protective spica were used for the fixation of a V-shaped, laterally based, closing-wedge valgus osteotomy in all cases. The postoperative follow-up period ranged from 14 to 102 months, with a mean duration of 33.3 ± 27.7 months. RESULTS: The mean Hilgenreiner epiphyseal angle (HEA) was corrected from 81.7 ± 2.2° to 24.3 ± 3.5° and the mean femoral neck-shaft angle (FNSA) was improved from 86.9 ± 4.2° to 138.6 ± 3.5°. No recurrence of the deformity was observed during the follow-up periods considered here. The osteotomy site united after an average of 11.7 ± 2.2 weeks with no secondary displacement, and in cases of developmental coxa vara there was progressive ossification of the neck defect with no surgery-related complications. Clinical results were markedly improved by the osteotomy, with a mean postoperative Iowa Hip Score at last follow-up of 95.06 ± 2.6, compared to a mean preoperative score of 57.4 ± 3.6. CONCLUSIONS: Transfixing wires protected in a hip spica cast represent a simple, easy, and reliable fixation method for valgus osteotomies performed to correct pediatic coxa vara. It assures stable fixation and rapid healing of the osteotomy without loss of the achieved correction, it completely avoids the femoral neck affording marked protection to the growth plate. LEVEL OF EVIDENCE: IV.

Spondyloepiphyseal dysplasia congenita caused by double heterozygous mutations in COL2A1.

Spondyloepiphyseal dysplasia congenita (SEDC) is a group of rare inherited chondrodysplasias characterized by short stature, abnormal epiphyses, and flattened vertebral bodies. SEDC is usually caused by substitution of glycine residue with another amino acid in the triple helical domains of alpha 1 chains, which consist of type II collagen (COL2A1). Herein, we describe a unique case of SEDC with mild coxa vara (SEDC-M) caused by double de novo COL2A1 mutations located on the same allele. One mutation, p.G504S, was previously described in patients with SEDC, whereas the other, p.G612A, was a novel mutation; both were located in the triple helical domain. Neither mutation was identified in the parents and appeared to be de novo. To the best of our knowledge, this is the first study involving a patient with a type II collagenopathy with two COL2A1 mutations on the same allele. The case was characterized by a more severe phenotype compared with previously reported cases involving a single p.G504S mutation, which may have been the result of the double mutation.

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.

Spinal Cord Injury After Extremity Surgery in Children With Thoracic Kyphosis.

BACKGROUND: Spinal cord injury is a rare complication after lower extremity surgery in children with skeletal dysplasia and thoracic kyphosis. We encountered two patients who had this complication, from among 51 (39 from Nemours/Alfred I. duPont Hospital for Children and 12 from Seattle Children's Hospital) who underwent lower extremity surgery during an 8.5-year period (June 2004 to December 2012). Because spinal cord injury is a devastating complication likely not known to most physicians treating patients with skeletal dysplasias, we sought to examine factors that may contribute to this rare complication. CASE DESCRIPTION: We performed a retrospective review of two patients with skeletal dysplasia who had paraplegia develop after extremity surgery. Outcome measures included operative time, vital signs, and postsurgery recovery of neurologic deficit. MR images were reviewed. Two patients were found-an 8.5-year-old boy with spondyloepiphyseal dysplasia congenita with a 76°-thoracic kyphosis apex at T4 and a 6.5-year-old boy with mucopolysaccharidosis type 1-H with an 80°-thoracic kyphosis apex at T2. Bilateral proximal femoral osteotomies or bilateral innominate and proximal femoral osteotomies had been performed. The spinal cord injuries occurred at the apex of the kyphosis as determined by clinical examination and MRI assessment. In both patients, the mean arterial blood pressure decreased below 50 mm Hg and might be a factor in the etiology of the paralysis. The first patient recovered motor function in 5 months; the second had no recovery. LITERATURE REVIEW: Paraplegia is extremely rare after nonspine operations. Many factors contribute to the risk for a spinal cord event: low mean arterial pressure, duration of the surgery, position on the operating table, the kyphotic spine deformity, or unappreciated vascular disease. Motor-evoked potentials and somatosensory-evoked potentials together potentially provide high sensitivity and specificity for predicting a postoperative neurologic deficit. CLINICAL RELEVANCE: Based on our two patients with skeletal dysplasia and a literature review of patients with hyperkyphosis undergoing extremity surgery, the surgeon must be aware of the risk of spinal cord injury. Careful preoperative assessment possibly including MRI of the spine is recommended. Mean arterial pressure should be maintained at a safe level; neuromonitoring should be considered.

Novel mutations in PRG4 gene in two Indian families with camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome.

BACKGROUND & OBJECTIVES: Camptodactyly--arthropathy-coxa vara-pericarditis (CACP) syndrome is an autosomal recessive disorder caused by mutations in the PRG4 (proteoglycan 4) gene. Hallmarks of the syndrome include congenital or early-onset camptodactyly and arthropathy with synovial hyperplasia, progressive coxa vara deformity and non-inflammatory pericardial effusions. Till date only around 25 pathogenic mutations have been reported in this gene and none have been reported from India. We report here the mutations in the PRG4 gene in three patients of CACP from two unrelated families from India. METHODS: Molecular genetic studies were done for the three patients with the CACP syndrome, from two unrelated Indian families, through sequence analysis of all coding exons and the exon-intron boundaries of the PRG4 gene. RESULTS: Two novel frame-shift deletion mutations leading to premature protein termination were found. One patient was identified to be homozygous for a 2 base pair deletion in exon 6 (c.2645_2646delGA) and the two affected siblings from the other family were found to be homozygous for a 4 base pair deletion in exon 6 (c.2883_2886delAAGA). CONCLUSIONS: This is perhaps the first report of PRG4 mutations from India. Further mutation studies in Indian CACP cases will help to determine the mutation spectrum of the PRG4 gene in the Indian population and also help to further elucidate the molecular pathology and the genotype-phenotype correlation of this rare disease.