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Orthopaedics & Traumatology, Surgery &... Feb 2016Restoration of normal hip biomechanics is a key goal of total hip arthroplasty (THA) and favorably affects functional recovery. Furthermore, a major concern for both the... (Review)
Review
Restoration of normal hip biomechanics is a key goal of total hip arthroplasty (THA) and favorably affects functional recovery. Furthermore, a major concern for both the surgeon and the patient is preservation or restoration of limb length equality, which must be achieved without compromising the stability of the prosthesis. Here, definitions are given for anatomic and functional limb length discrepancies and for femoral and hip offset, determined taking anteversion into account. Data on the influence of operated-limb length and offset on patient satisfaction, hip function, and prosthesis survival after THA are reviewed. Errors may adversely impact function, quality of life, and prosthetic survival and may also generate conflicts between the surgeon and patient. Surgeons rely on two- or three-dimensional preoperative templating and on intraoperative landmarks to manage offset and length. Accuracy can be improved by using computer-assisted planning or surgery and the more recently introduced EOS imaging system. The prosthetic's armamentarium now includes varus-aligned and lateralized implants, as well as implants with modular or custom-made necks, which allow restoration of the normal hip geometry, most notably in patients with coxa vara or coxa valga. Femoral anteversion must also receive careful attention. The most common errors are limb lengthening and a decrease in hip offset. When symptoms are caused by an error in length and/or offset, revision arthroplasty may deserve consideration.
Topics: Arthroplasty, Replacement, Hip; Femur; Hip Prosthesis; Humans; Leg Length Inequality; Lower Extremity; Patient Satisfaction; Postoperative Complications; Preoperative Care; Prosthesis Failure; Quality of Life; Recovery of Function; Reoperation
PubMed: 26797005
DOI: 10.1016/j.otsr.2015.11.001 -
Diagnostics (Basel, Switzerland) Mar 2020Mucopolysaccharidosis type I (MPS I) is caused by the deficiency of α-l-iduronidase, leading to the storage of dermatan and heparan sulfate. There is a broad... (Review)
Review
Mucopolysaccharidosis type I (MPS I) is caused by the deficiency of α-l-iduronidase, leading to the storage of dermatan and heparan sulfate. There is a broad phenotypical spectrum with the presence or absence of neurological impairment. The classical form is known as Hurler syndrome, the intermediate form as Hurler-Scheie, and the most attenuated form is known as Scheie syndrome. Phenotype seems to be largely influenced by genotype. Patients usually develop several somatic symptoms such as abdominal hernias, extensive dermal melanocytosis, thoracolumbar kyphosis odontoid dysplasia, arthropathy, coxa valga and genu valgum, coarse facial features, respiratory and cardiac impairment. The diagnosis is based on the quantification of α-l-iduronidase coupled with glycosaminoglycan analysis and gene sequencing. Guidelines for treatment recommend hematopoietic stem cell transplantation for young Hurler patients (usually at less than 30 months of age). Intravenous enzyme replacement is approved and is the standard of care for attenuated-Hurler-Scheie and Scheie-forms (without cognitive impairment) and for the late-diagnosed severe-Hurler-cases. Intrathecal enzyme replacement therapy is under evaluation, but it seems to be safe and effective. Other therapeutic approaches such as gene therapy, gene editing, stop codon read through, and therapy with small molecules are under development. Newborn screening is now allowing the early identification of MPS I patients, who can then be treated within their first days of life, potentially leading to a dramatic change in the disease's progression. Supportive care is very important to improve quality of life and might include several surgeries throughout the life course.
PubMed: 32188113
DOI: 10.3390/diagnostics10030161 -
Clinical Orthopaedics and Related... Nov 2019Spastic hip subluxation or dislocation that is associated with an excessive coxa valga deformity is a common pathologic condition in children with cerebral palsy (CP)...
BACKGROUND
Spastic hip subluxation or dislocation that is associated with an excessive coxa valga deformity is a common pathologic condition in children with cerebral palsy (CP) that is often treated with large bone reconstructive procedures. Guided growth techniques (such as stapling, plate, or transphyseal screw) have been widely used to alter the growth axis in patients with a lower-limb deformity but only a few reports have described their use in patients with coxa valga deformities.
QUESTIONS/PURPOSES
(1) Does guided growth surgery using a transphyseal screw combined with adductor tenotomy prevent progressive coxa valga deformity and lateral hip subluxation in children with CP? (2) What factors influence the correction of coxa valga deformity and the success of hip stabilization? (3) What complications were associated with this operation and how often did children treated with it undergo reoperation?
METHODS
From 2012 to 2016, at our institution, three authors (H-CH, KNK, K-WW) retrospectively studied data on children with CP who underwent guided growth of the hip for progressive bilateral hip subluxation associated with coxa valga deformities. A single percutaneous screw was inserted across the inferomedial portion of proximal femoral physis in an AP view and centered along femoral neck in lateral view under fluoroscopy guidance. During the period, we treated 25 consecutive children with CP who had progressive hip subluxation with coxa valga deformities. The indications for surgery were migration percentage > 30% and head-shaft angle > 155° with at least 2 years growth remaining. Of those, 13 patients underwent guided growth alone, and 48% (12) underwent a combination of guided growth and adductor tenotomy. Of the 25 patients treated with this approach, 96% (24) were available for follow-up with complete data at a minimum of 2 years follow-up (mean 50 months; range 25 to 72). All children (17 boys and seven girls; 48 hips) underwent surgery at a mean age of 8 years (range 5 to 12). With regard to the gross motor function classification system, three patients were Level 1, four patients were Level II, seven patients were Level III, seven were Level IV, and three were Level V. Radiographic parameters including the head-shaft angle, Hilgenreiner's epiphyseal angle, acetabular index, and Reimer's migration percentage were assessed before surgery and at the latest follow-up examination by one author (H-CH). Complications and reoperations were assessed by chart review. During the period in question, we generally offered secondary reconstructive surgery to patients who underwent a guided growth procedure once their subluxation progressed.
RESULTS
With the data available, the coxa valga and lateral hip subluxation improved in terms of the reduction of head-shaft angle by a mean of 13° ± 7° (95% CI 11 to 15; p < 0.001) and the reduction of the migration percentage by 10% ± 11% (95% CI 7 to 13; p < 0.001). After controlling for potentially confounding variables like gender, gross motor function classification system, Hilgenreiner's epiphyseal angle and acetabular index, we found that longer follow-up duration (r = 0.234; p < 0.001) and a smaller preoperative migration percentage (r = -0.258; p = 0.004) were associated with larger changes in the head-shaft angle. In terms of complications, we found that the proximal femoral physis grew off the screw tip in 44% (21 of 48 hips) at a mean of 28 months. Among these, 31% of hips (15 of 48) in 33% of patients (eight of 24) underwent replacement with a longer screw. Among the 17% of hips (eight of 48) in 21% of patients (five of 24) who had progressive lateral subluxation and underwent secondary reconstructive surgery, we found that their preoperative acetabular index was higher (mean 29° versus 21°; p < 0.001), as was their head-shaft angle (mean 166° versus 162°; p = 0.045), and migration percentage (mean 54% versus 36 %; p < 0.001).
CONCLUSIONS
Although guided growth with single transphyseal screw did not create as large a degree of varus as proximal femoral osteotomy, it did stabilize the hip in children with cerebral palsy with migration percentage less than 50% in our series. It is a simple procedure that can be of benefit to children with cerebral palsy with unstable hip. Reoperation in patients where the physis has grown off the screw tip can be a problem; fortunately, it is a rather minor procedure to replace with a longer screw.
LEVEL OF EVIDENCE
Level III, therapeutic study.
Topics: Bone Screws; Cerebral Palsy; Child; Child, Preschool; Coxa Valga; Epiphyses; Female; Femur; Follow-Up Studies; Hip Dislocation; Humans; Male; Retrospective Studies; Treatment Outcome
PubMed: 31425278
DOI: 10.1097/CORR.0000000000000903 -
Bone & Joint Open Oct 2022To evaluate how abnormal proximal femoral anatomy affects different femoral version measurements in young patients with hip pain.
AIMS
To evaluate how abnormal proximal femoral anatomy affects different femoral version measurements in young patients with hip pain.
METHODS
First, femoral version was measured in 50 hips of symptomatic consecutively selected patients with hip pain (mean age 20 years (SD 6), 60% (n = 25) females) on preoperative CT scans using different measurement methods: Lee et al, Reikerås et al, Tomczak et al, and Murphy et al. Neck-shaft angle (NSA) and α angle were measured on coronal and radial CT images. Second, CT scans from three patients with femoral retroversion, normal femoral version, and anteversion were used to create 3D femur models, which were manipulated to generate models with different NSAs and different cam lesions, resulting in eight models per patient. Femoral version measurements were repeated on manipulated femora.
RESULTS
Comparing the different measurement methods for femoral version resulted in a maximum mean difference of 18° (95% CI 16 to 20) between the most proximal (Lee et al) and most distal (Murphy et al) methods. Higher differences in proximal and distal femoral version measurement techniques were seen in femora with greater femoral version ( > 0.46; p < 0.001) and greater NSA ( > 0.37; p = 0.008) between all measurement methods. In the parametric 3D manipulation analysis, differences in femoral version increased 11° and 9° in patients with high and normal femoral version, respectively, with increasing NSA (110° to 150°).
CONCLUSION
Measurement of femoral version angles differ depending on the method used to almost 20°, which is in the range of the aimed surgical correction in derotational femoral osteotomy and thus can be considered clinically relevant. Differences between proximal and distal measurement methods further increase by increasing femoral version and NSA. Measurement methods that take the entire proximal femur into account by using distal landmarks may produce more sensitive measurements of these differences.Cite this article: 2022;3(10):759-766.
PubMed: 36196582
DOI: 10.1302/2633-1462.310.BJO-2022-0102.R1 -
Molecular Genetics and Metabolism 2017Mucopolysaccharidosis IVA (MPS IVA; Morquio A: OMIM 253000) is a lysosomal storage disease with an autosomal recessive trait caused by the deficiency of... (Review)
Review
Mucopolysaccharidosis IVA (MPS IVA; Morquio A: OMIM 253000) is a lysosomal storage disease with an autosomal recessive trait caused by the deficiency of N-acetylgalactosamine-6-sulfate sulfatase. Deficiency of this enzyme leads to accumulation of specific glycosaminoglycans (GAGs): chondroitin-6-sulfate (C6S) and keratan sulfate (KS). C6S and KS are mainly produced in the cartilage. Therefore, the undegraded substrates are stored primarily in cartilage and in its extracellular matrix (ECM), leading to a direct impact on cartilage and bone development, and successive systemic skeletal dysplasia. Chondrogenesis, the earliest phase of skeletal formation, is maintained by cellular interactions with the ECM, growth and differentiation factors, signaling pathways, and transcription factors in a temporal-spatial manner. In patients with MPS IVA, the cartilage is disrupted at birth as a consequence of abnormal chondrogenesis and/or endochondral ossification. The unique skeletal features are distinguished by a disproportional short stature, odontoid hypoplasia, spinal cord compression, tracheal obstruction, pectus carinatum, kyphoscoliosis, platyspondyly, coxa valga, genu valgum, waddling gait, and laxity of joints. In spite of many descriptions of these unique clinical features, delay of diagnosis still happens. The pathogenesis and treatment of systemic skeletal dysplasia in MPS IVA remains an unmet challenge. In this review article, we comprehensively describe historical aspect, property of GAGs, diagnosis, screening, pathogenesis, and current and future therapies of MPS IVA.
Topics: Adolescent; Adult; Cartilage; Clinical Trials as Topic; Enzyme Replacement Therapy; Female; Genetic Therapy; Glycosaminoglycans; Humans; Male; Mucopolysaccharidosis IV; Orthopedic Procedures
PubMed: 27979613
DOI: 10.1016/j.ymgme.2016.11.007 -
Italian Journal of Pediatrics Nov 2018Mucopolysaccharidoses (MPS) are a group of diseases characterized by abnormal accumulation of glycosaminoglycans (GAGs). Although there are differences among the various... (Review)
Review
Mucopolysaccharidoses (MPS) are a group of diseases characterized by abnormal accumulation of glycosaminoglycans (GAGs). Although there are differences among the various disease types, the osteoarticular system is always involved. The aim of the present study was to establish a framework for MPS-related orthopaedic manifestations and for their treatment. The authors, affiliated to three different Italian Orthopaedic Centres, report data taken from the literature reviewed in light of their accumulated professional experience. Bone alterations make up what is known as dysostosis multiplex, involving the trunk and limbs and with typical radiological findings. Joints are affected by pathological tissue infiltrations. The cervical spinal cord is involved, with stenosis and cervical and occipitocervical instability. In MPS there is a much higher incidence of scoliosis compared with healthy subjects without any particular distinctive feature. Kyphosis of the spine is more frequent and also more severe because of its possible neurological complications, and it is localized at the thoracolumbar level with a malformed vertebra at the top of the deformity. Evolving forms, and those associated with neurological damage, require anteroposterior spine fusion. The hip is invariably involved, with dysplasia affecting the femoral neck (coxa valga), the femoral epiphysis (loss of sphericity, osteonecrosis), and the femoral acetabulum which is flared. All these features explain the tendency to progressive hip migration. Genu valgum is often found (a deviation of the physiological axis with an obtuse angle opening laterally). This deformity is often localized at the proximal tibial metaphysis; it causes functional limitations and leads to an irregular erosion of the articular cartilage. In young patients who still have the growth plate, it is possible to execute a medial hemiepiphysiodesis, a temporary inhibition of cartilage growth, with progressive axis correction. In this paper, the characterisation of clinical features and the review of treatments are divided into separate sections based on the part of the body involved. The conclusions of each section are presented as a summary. One section discusses the high risk of anaesthesia-related complications requiring the collaboration of specifically trained personnel.
Topics: Bone Diseases; Humans; Mucopolysaccharidoses; Orthopedic Procedures
PubMed: 30442173
DOI: 10.1186/s13052-018-0557-y -
BMC Musculoskeletal Disorders Jun 2021Gluteal muscle contracture (GMC) is a disease characterized by the limited function of the hip joint, knee pain, and abnormal gait. There is a lack of research on the...
BACKGROUND
Gluteal muscle contracture (GMC) is a disease characterized by the limited function of the hip joint, knee pain, and abnormal gait. There is a lack of research on the effect of GMC on the hip joint structure to date. This study aims to analyze the association between GMC and the deformity of the hip and pelvis.
METHODS
Standing anteroposterior pelvic radiographs of 214 patients (152 with gluteal muscle contracture and 62 without gluteal muscle contracture) were retrospectively collected. Neck-shaft angle, lateral center edge angle, Tönnis angle, femoral head coverage index, acetabular depth, Sacro-femoral-pubic angle, and obturator foramen ratio were respectively measured and included in the following statistical analysis. The collected data were analyzed using logistical regression and multiple linear regression to explore the factors influencing coxa valga and SFP angle.
RESULTS
GMC was identified as a common factor significantly associated with coxa valga and increased SFP angle. There is a difference of risk factors in logistic regression for coxa valga between the left and right sides.
CONCLUSION
GMC is a significant risk factor for coxa valga and increased SFP angle. Given that GMC can cause coxa valga and likely alter the pelvis's position, GMC should be paid attention to and treated early.
Topics: Contracture; Coxa Valga; Humans; Multivariate Analysis; Muscles; Retrospective Studies
PubMed: 34147092
DOI: 10.1186/s12891-021-04447-7 -
Medicine Oct 2023Coxa valga, measured as the neck-shaft angle (NSA) or head-shaft angle (HSA), is regarded as a potential risk factor for hip dislocation in patients with cerebral palsy.... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
Coxa valga, measured as the neck-shaft angle (NSA) or head-shaft angle (HSA), is regarded as a potential risk factor for hip dislocation in patients with cerebral palsy. We investigated the effects of a novel hip brace on coxa valga.
METHODS
A prospective, multicenter, assessor-blinded, randomized controlled trial was conducted from July 2019 to November 2021. Children with cerebral palsy aged 1 to 10 years with Gross Motor Function Classification System levels IV and V were recruited. The study group wore a hip brace for at least 12 hour a day. A lower strap of the hip brace was designed to prevent coxa valga biomechanically. The effectiveness of the hip brace on coxa valga was assessed by measurement of the NSA and head-shaft angle at enrollment and 6 and 12 months after enrollment.
RESULTS
Sixty-six participants were enrolled, and 33 patients were assigned to each group. Changes in the mean NSA of both sides and the NSA of left side showed significant differences between the 2 groups over 12 months (mean NSA of both sides, -1.12 ± 3.64 in the study group and 1.43 ± 3.75 in the control group, P = .023; NSA of the left side, -1.72 ± 5.38 in the study group and 2.01 ± 5.22 in the control group, P = .008).
CONCLUSIONS
The hip brace was effective in preventing the progression of coxa valga and hip displacement, suggesting that the prevention of coxa valga using hip brace is a contributing factor in prevention of hip displacement.
Topics: Humans; Child; Coxa Valga; Hip Dislocation; Cerebral Palsy; Prospective Studies; Hip Dislocation, Congenital
PubMed: 37904376
DOI: 10.1097/MD.0000000000035696