Hip Deformity Research Articles

Hip reduction surgery versus scoliosis correction in non-ambulant neuromuscular patients: which surgery is the first step?

Children and young adults with neuromuscular disorders have a high incidence of both spine and hip deformities. The aim of this study was to evaluate the outcome of either primary scoliosis or hip surgery in children and young adults with neuromuscular disorders. A retrospective study was conducted on all children and young adults with neuromuscular-related synchronous hip subluxation/dislocation and scoliosis undergoing hip or scoliosis surgery in our institution between 2012 and 2021 with a minimum follow-up of 24months. Demographic and operative data were collected; radiological parameters were measured preoperatively and postoperatively at final follow-up. Forty neuromuscular patients with synchronous hip displacement and scoliosis were included. Twenty patients with an average age of10.2years had hip correction surgery performed primarily, with a mean follow-up of 54.9 (24-96)months. The other 20 patients with an average age of 12.4years had scoliosis correction first, with a mean follow-up of 40 (24-60)months. In the "Hip first" group, pelvic obliquity, hip MP and Cobb angle were 16.8°, 71%, and 49°, respectively. At final follow-up, the mean pelvic obliquity and Cobb angles significantly progressed to 27.2° (p = 0.003) and 82.2° (p = 0.001), respectively. Eighteen patients (90%) required scoliosis correction after the hip surgery. In the "Scoliosis first" group, the mean pelvic obliquity, hip MP and Cobb angle were 21.2°, 49% and 65.5°, respectively. At final follow-up, both pelvic obliquity and Cobb angle significantly improved to 8.44° (p = 0.002) and 23.4° (p = 0.001), respectively. In 11/20 (55%) patients, the hip MP had significantly increased following the spinal surgery to 62% (p = 0.001), but only 5/20 (25%) patients underwent hip surgery after scoliosis correction. In neuromuscular patients presenting with synchronous hip displacement and scoliosis deformity, corrective scoliosis surgery is associated with a significant correction of pelvic obliquity and lower rates of secondary hip surgery. On the other hand, primary hip surgery does not reduce the risk of pelvic obliquity and scoliosis deformity progression.

The Preserved Thickness Ratio of the Femoral Head Contributes to the Collapse Predictor of Osteonecrosis.

The collapse of femoral head is a serious symptom of osteonecrosis of the femoral head (ONFH), resulting in hip pain and deformity. However, it is hardly possible to reestablish the femoral head nonoperatively once the collapse happens. Predicting femoral head collapse is of great value for the prognosis of ONFH. This study aimed to develop a new method to quantify the preserved thickness of femoral head and to assess its diagnostic contribution in predicting femoral head collapse on plain radiographs. This was a single-center retrospective study. A total of 101 hips (85 patients) with ARCO stage II from January 2008 to December 2016 were included in this study. The preserved thickness was measured on standard anteroposterior (AP) and frog-leg (FL) radiographs. The anteroposterior view's preserved thickness ratio (APTR) and the frog-leg view's preserved thickness ratio (FPTR) were calculated to show the preserved thickness ratio of the femoral head anteriorly and laterally. Univariate and multivariate logistic regression was performed to determine the risk factors for collapse. Sensitivity, specificity, and cut-off values for APTR and FPTR were determined by the receiver operating characteristic (ROC) curve analysis. Kaplan-Meier (K-M) analysis was applied to determine femoral head survival in ONFH patients. The mean age of the 27 females and 58 males was 38.93 years old. The mean follow-up time was 74.62 (36-124) months in the non-collapse group and 18.66 (3-82) months in the collapse group. Femoral head collapse was observed in 62 hips during the follow-up period. Logistic regression analysis and ROC results showed that APTR <24.79% and FPTR <10.62% were significantly correlated with femoral head collapse. The Kaplan-Meier survival curve suggested that the overall survival rate of APTR ≥24.79% was 68.2% at 5 and 10 years and FPTR ≥10.62% was 71.63% at 5 and 10 years. At the last follow-up, 26 hips had collapse on the anterior side of the femoral head, 12 hips occurred on the lateral side, and 24 hips happened to collapse on both anterior and lateral sides. Femoral head collapse predominantly occurred anteriorly rather than laterally in ONFH patients. The measurements of APTR and FPTR have noticeable implications for the prediction of femoral head collapse, and contribute to the selection of treatment options for ONFH patients with types B and C1 according to the JIC classification.

Higher femoral head–to–femoral neck ratio are associated with limited range of motion of hip joints in hereditary multiple exostosis patients

Hip involvement is one of the most common skeletal manifestations of hereditary multiple exostoses (HME) in 30-90% of the patients. The anatomical changes in the proximal femur in patients with HME, may lead to deformities and related functional deficits. Its effect on the range of motion (ROM) of the hip joint is not well described. The aim of this study was to evaluate the role of hip deformity of HME disease in the ROM of the hip joint. It was hypothesized that certain morphological parameters affect the ROM of the hip joint. Conclusively, 44 patients including 27 men and 17 women (88 hips) were evaluated. Hips are divided in Group 1 (no ROM limitation group) and Group 2 (ROM limitation group). The head-neck ratio (HNR), center edge angle (CE), Sharp’s acetabular angle (SAA), alpha angle (AA), neck shaft angle (NSA) and ROM parameters were measured. The correlations between radiological parameters (SAA, CE, NSA, AA and HNR), and the ROM data (F, E, Abd, Add, IR and ER) were statistically evaluated. All evaluated parameters (SAA, CE, NSA, AA) except the HNR do not show significant differences between study group 1 (no ROM limitation group) and group 2 (ROM limitation group). The means of the HNR in group 1 were significantly higher than those in group 2 (HNR: 1.23 vs. 0.92, p&lt;0.001). In group 2 HNR showed a strong positive correlation with Abd (PC 0.72, p&lt;0.001), E (PC 0.7, p&lt;0.001), ER (PC 0.67, p&lt;0.001), IR (PC 0.67, p&lt;0.001) and F (PC 0.66, p&lt;0.001). HME patients with hip involvement have better ROM of the hip joint, if they have the higher HNR. Additionally, a positive correlation between HNR and ROM capacity was determined.

Analysis of the epidemiological profile of congenital hip deformities, 2011-2021.

This study aimed to identify the relevance of hospitalizations for congenital hip deformities in Bahia. This is a retrospective epidemiological study in public databases. Descriptors in health sciences: "congenital hip dysplasia", "congenital hip dislocation", and "congenital dislocation hip". This is qualitative-quantitative research with the analysis of secondary data and cross-sectional typologies in the databases of the Ministry of Health - Health Information (TABNET), made available by the Department of Informatics of the Unified Health System (DATASUS). Bahia was the third Brazilian state with the highest number of hospitalizations, registering 1481 cases. The municipalities in Bahia with the highest prevalence were Itanhém, Salvador, and Barreiras, with 912, 445, and 20 cases, respectively. The elevated number of congenital hip deformities reflects a public health problem, requiring investments in public policies.

Results of Arthroscopic Treatment of Patients With Femoroacetabular Impingement Depending on the Type of Hip Deformity

Background. Femoroacetabular impingement (FAI) is one of the most common causes of pain syndrome and limited mobility in the hip joint among young and middle-aged individuals.&#x0D; The aims of this study: 1) To evaluate the impact of the type of hip joint deformity in patients with FAI on treatment outcomes; 2) To identify the type of hip joint deformity that is optimal for correction using arthroscopy; 3) To determine the impact of the acetabular structure in conditions of borderline dysplasia on the treatment outcome.&#x0D; Methods. A retrospective uncontrolled single-center study was conducted, including 121 patients (135 hip joints), among them 49 (40.5%) women and 72 (59.5%) men. Patients were divided into four groups based on the type of deformity. The first group included 33 (24.4%) joints with cam-type FAI, the second group — 72 (53.4%) joints with mixed-type, the third group — 17 (12.6%) joints with dysplasia (LCEA-O25°) and cam-type deformity of the femoral head-neck junction, and the fourth group — 13 (9.6%) joints with a combination of dysplasia, cam-type deformity, and retroversion of the acetabulum. All patients underwent physical examination and radiographic diagnostics. In patients with borderline dysplasia, the version of the acetabulum was additionally assessed. The iHOT-33 and HOS scales were used to evaluate the preoperative status and postoperative results.&#x0D; Results. The best treatment outcome was achieved in the first group of patients, which was statistically significantly different from the results in the third group. The treatment outcomes in the second group of patients did not show statistically significant differences from the first group according to the HOS questionnaire, but differed according to the iHOT-33 scale. The treatment outcomes in the fourth group of patients were almost indistinguishable on the iHOT-33 scale from the first group and on the HOS scale from the second group. In the third group, a statistically significant result on the HOS-Sport subscale was achieved in only 30% of patients, while in other groups it was not less than 58%. Other scales showed a slight superiority of treatment results in the first and fourth groups compared to the second and third groups. In the first years after surgery, all groups of patients showed a significant improvement in sports activity, but after 2 years, there was a tendency for a decrease in patients in the second and third groups.&#x0D; Conclusion. The highest results of arthroscopic treatment were shown by patients in the first group with isolated cam-type deformity, slightly worse were results by patients in the second group (with mixed-type). In patients with borderline dysplasia, the effectiveness of arthroscopy depended on the structure of the anterior wall of the acetabulum. The worst result was observed in patients with borderline dysplasia and insufficiently developed anterior wall of the acetabulum — in that group of patients, it is worth preferring isolated periacetabular osteotomy or in combination with arthroscopy.

Characteristics of Very Young Patients Undergoing Total Hip Arthroplasty: A Contemporary Assessment

BackgroundThis study aims to compare indications, patient characteristics, hospital factors, and complication rates between total hip arthroplasty (THA) patients aged 30 years or younger and those older than 30 years using a large national database. MethodsThe Premier Healthcare Database was utilized to identify primary THA patients from 2015 to 2021 who were aged ≤30 or >30 years. Patient demographics, hospital factors, and primary indications were compared for each cohort. Rates of complications and readmissions were assessed for each cohort by primary indication. Differences were assessed through univariate analysis. ResultsOverall, 539,173 primary THA patients were identified (age ≤30: 1849; >30: 537,234). Compared to the >30 cohort, the ≤30 cohort was more likely to be male (56.5% vs 44.9%, P < .001) and non-White (34.0% vs 14.2%, P < .001). The most common indications for THA in the ≤30 cohort were osteonecrosis (49.3%), osteoarthritis (17.8%), and congenital hip deformities (16.0%), and in the >30 cohort, they were osteoarthritis (77.0%), other arthritis (11.3%), and osteonecrosis (5.4%). Patients aged ≤30 years had lower rates of respiratory failure (0.16% vs 0.57%, P < .001), acute renal failure (0.32% vs 1.72%, P < .001), and urinary tract infection (0.38% vs 1.11%, P = .003) than those aged >30 years, but higher rates of wound dehiscence (0.59% vs 0.29%, P = .015) and transfusion (3.68% vs 2.21%, P < .001). There were no differences in 90-day readmission rates (P = .811) or 90-day in-hospital death (P = .173) between cohorts. ConclusionsYounger patients undergoing THA differed significantly in indication, patient characteristics, and hospital factors compared to the older population on univariate analysis. Despite differences in indications, the cohorts did not differ markedly with regard to complication rates in this study.

Clinical, genetic and structural delineation of RPL13-related spondyloepimetaphyseal dysplasia suggest extra-ribosomal functions of eL13

Spondyloepimetaphyseal dysplasia with severe short stature, RPL13-related (SEMD-RPL13), MIM#618728), is a rare autosomal dominant disorder characterized by short stature and skeletal changes such as mild spondylar and epimetaphyseal dysplasia affecting primarily the lower limbs. The genetic cause was first reported in 2019 by Le Caignec et al., and six disease-causing variants in the gene coding for a ribosomal protein, RPL13 (NM_000977.3) have been identified to date. This study presents clinical and radiographic data from 12 affected individuals aged 2–64 years from seven unrelated families, showing highly variable manifestations. The affected individuals showed a range from mild to severe short stature, retaining the same radiographic pattern of spondylar- and epi-metaphyseal dysplasia, but with varying severity of the hip and knee deformities. Two new missense variants, c.548 G>A, p.(Arg183His) and c.569 G>T, p.(Arg190Leu), and a previously known splice variant c.477+1G>A were identified, confirming mutational clustering in a highly specific RNA binding motif. Structural analysis and interpretation of the variants’ impact on the protein suggests that disruption of extra-ribosomal functions of the protein through binding of mRNA may play a role in the skeletal phenotype of SEMD-RPL13. In addition, we present gonadal and somatic mosaicism for the condition.

A bibliometric analysis andvisualization ofresearch trends onperiacetabular osteotomy.

Bernese periacetabular osteotomy (PAO) is a practical and safe acetabular reorientation technique used to correct structural hip deformities, and much relevant literature has been published over the past decades. This bibliometric study aims to determine the status of PAO research between 1994 and 2022. Information about PAO research publications from 1994 to 2022 was obtained from the Web of Science Core Collection. This bibliometric study is implemented and analyzed through three bibliometric tools with respect to publication counts, countries, institutions, authors, journals, funding agencies, references and keywords. In total, 634 publications concerning PAO were identified. The United States and Washington University in St. Louis have published the most literature in terms of country and institution. Additionally, Switzerland and the University of Bern are the country and the institution with the highest average number of citations in the PAO field. The most published and co-cited journal is Clinical Orthopaedics and Related Research. Burst keyword detection has discovered that 'patient-reported outcome', 'instability', 'risk', 'survivorship' and 'outcome' are regarded as the current research frontier. In summary, our findings provide insight into the bibliometric overview of research status in the PAO field, which may offer later investigators' references in exploring further research directions.

Hip-spine syndrome in adult spinal deformity patients

With the increasing prevalence of hip-spine syndrome, it is crucial to appreciate the biomechanical interplay between hip and spine degeneration or deformity and the consequential compensatory changes. Adult spinal deformity (ASD) patients are uniquely affected as a concomitant hip osteoarthritis (HOA) may impact their baseline and postoperative sagittal alignment. Similarly, severe HOA patients undergoing total hip replacement with concomitant spinal deformity may require personalized surgical planning for the placement of their acetabular component. If surgical intervention is required in ASD patients, the authors’ preferred sequence is to perform spinal realignment first. If major realignment is not planned, we recommend treating the most symptomatic pathology. Further investigation is required to evaluate the impact of hip pathology on radiographic and patient-reported outcomes following ASD surgery and vice versa.

Virtual 3D femur model to assess femoral version: comparison to the 2D axial slice approach.

Quantifying femoral version is crucial in diagnosing femoral version abnormalities and for accurate pre-surgical planning. There are numerous methods for measuring femoral version, however, reliability studies for most of these methods excluded children with hip deformities. To propose a method of measuring femoral version based on a virtual 3D femur model, and systematically compare its reliability to the widely used Murphy's 2D axial slice technique. We searched our imaging database to identify hip/femur CTs performed on children (<18 years old) with a clinical indication of femoral version measurement (September 2021-August 2022). Exclusion criteria were prior hip surgery, and inadequate image quality or field-of-view. Two blinded radiologists independently measured femoral version using the virtual 3D femur model and Murphy's 2D axial slice method. To assess intrareader variability, we randomly selected 20% of the study sample for re-measurements by the two radiologists >2 weeks later. We analyzed the reliability and correlation of these techniques via intraclass correlation coefficient (ICC), Bland-Altman analysis, and deformity subgroup analysis. Our study sample consisted of 142 femurs from 71 patients (10.6±4.4 years, male=31). Intra- and inter-reader correlations for both techniques were excellent (ICC≥0.91). However, Bland-Altman analysis revealed that the standard deviation (SD) of the absolute difference between the two radiologists for the Murphy method (mean 13.7°) was larger than that of the 3D femur model technique (mean 4.8°), indicating higher reader variability. In femurs with hip flexion deformity, the SD of the absolute difference for the Murphy technique was 17°, compared to 6.5° for the 3D femur model technique. In femurs with apparent coxa valga deformity, the SD of the absolute difference for the Murphy technique was 10.4°, compared to 5.2° for the 3D femur model technique. The 3D femur model technique is more reliable than the Murphy's 2D axial slice technique in measuring femoral version, especially in children with hip flexion and apparent coxa valga deformities.

Which Acetabular Measurements Most Accurately Differentiate Between Patients and Controls? A Comparative Study.

Which Acetabular Measurements Most Accurately Differentiate Between Patients and Controls? A Comparative Study.

Treatment of Symptomatic Residual Deformity in Legg-Calvé-Perthes Disease: Mid-Term Outcomes and Predictors of Failure After Surgical Hip Dislocation with Femoral-Head Reshaping and Relative Neck Lengthening.

Treating patients with symptomatic hips after healed Legg-Calvé-Perthes disease (LCPD) is challenging, mainly because of the complexity of the deformity. We performed a retrospective study to evaluate clinical and radiographic outcomes, measure the survival rate, and identify predictors of failure following a surgical hip dislocation (SHD) with femoral-head reshaping and relative femoral-neck lengthening for the treatment of symptomatic residual hip deformity after healed LCPD. We identified 60 patients undergoing SHD for the treatment of symptomatic residual LCPD deformity. Fifty-one (85%) of the patients (mean age, 16.3 ± 4.7 years; 21 male patients [41%]), were followed ≥4 years after surgery. We defined surgical failure as conversion to, or recommendation for, total hip arthroplasty (THA) or a Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) pain score of ≥10 at the most recent follow-up. We used a multivariable Cox proportional hazards model to identify factors that were predictive of failure. The rate of survival free from failure was estimated using a Kaplan-Meier curve. Twenty (39%) of the patients met 1 of the end-point criteria for surgical failure, while the hips of the remaining 31 (61%) of the patients were successfully preserved at a median follow-up of 10.2 years (interquartile range, 5.7 to 12.9 years). The estimated survival was 80% (95% confidence interval [CI] = 70% to 92%) at 5 years and 66% (95% CI = 53% to 81%) at 10 years. Independent factors associated with surgical failure were the severity of preoperative pain as assessed by the WOMAC pain score (hazard ratio [HR] = 1.16; 95% CI = 1.03 to 1.30; p = 0.01) and the severity of the deformity as assessed by the anteroposterior alpha angle (HR = 1.06; 95% CI = 1.01 to 1.11; p = 0.01). We found that SHD with relative femoral-neck lengthening and osteochondroplasty of the femoral head-neck junction was associated with improved deformity as assessed radiographically and decreased pain and symptoms of stiffness among patients with symptomatic residual LCPD deformity. Preoperative pain and deformity were identified as predictors of surgical failure. Further research is needed to establish the role of SHD and other procedures in preserving the hip over the long term. Therapeutic L evel IV . See Instructions for Authors for a complete description of levels of evidence.

Is the Acetabular Cup Orientation Different in Robot-Assisted and Conventional Total Hip Arthroplasty With Right-Handed Surgeons Using an Anterolateral Approach?

Introduction Total hip arthroplasty (THA) is one of the most successful orthopaedic procedures. Survival rates from 90% at 10 years to 93% at 20 years have been reported in different studies. Differences in implant and patient characteristics can undoubtedly explain some of this variability observed in prosthesis durability, but the effect of surgical technique and implant orientation cannot be ignored. Therefore, many intraoperative methods (anatomic landmarks, intraoperative x-ray, fluoroscopy, navigation, and robotic surgery) have been attempted to avoid acetabular component malpositioning.Although postoperative computed tomography (CT) is accepted as the gold standard for the measurement of acetabular anteversion, it remains controversial in respect of costs and radiation exposure. The aim of this study was to examine how acetabular component orientation was affected in robotic and conventional THA operations performed by two surgeons with right-hand dominance. Material and methods The study included 113 primary THA operations performed on 113 patients between 2017 and 2022 in two groups: (i) robotic THA (Mako, Stryker Corporation, Kalamazoo, Michigan, United States) (55 patients) and (ii) conventional THA (58 patients). The patients comprised 51 males and 62 females. THA was performed on 54 right-side hips and 59 left-side hips. The operations were performed by two orthopaedic surgeons, each with 20 years of arthroplasty experience, on all the patients in the lateral decubitus position with an anterolateral approach. In all the cases, the orientation of the acetabular component was 40° inclination and 20° anteversion. Difficult THA procedures (patients with developmental dysplasia of the hip (DDH), a history of hip surgery, revision THA, defect or deformity of the acetabulum, a history of scoliosis or lumbar posterior surgery, or those requiring proximal femoral osteotomy) were excluded from the study. Using the Liaw and Lewinnek methods, the acetabular component anteversion was measured on the radiographs taken in the optimal position postoperatively and the acetabular cup inclination angles were measured on the pelvis radiographs. The groups were compared using the Kolmogorov-Smirnov, Pearson Chi-square and Mann-Whitney U statistical tests. The limits were accepted as 40±5° for inclination and 20±5° for anteversion. Results No statistically significant difference was determined between the groups in respect of age, gender, or operated side. No statistically significant difference was determined between the optimal acetabular cup inclination angles of the robotic and conventional THA groups (p = 0.79). No statistically significant difference was determined between the optimal acetabular cup anteversion angles of the left and right conventional THA groups. Statistically significantly better results were determined in the robotic group in respect of acetabular cup anteversion (p<0,001). Conclusion The optimal orientation of the acetabular component is a key factor for successful THA. Otherwise, revision surgery is inevitable for reasons such as instability, impingement, or increased wear. The results of this study demonstrated that robotic surgery was superior to the conventional method in the placement of the acetabular component in the desired orientation.

Can We Extend the Indications for Multilevel Surgery to Non-Ambulatory Children with Neuromuscular Diseases? A Safety and Efficacy Study

A retrospective study that aims to analyze the safety and efficacy of single-event multilevel surgery (SEMLS) involving bifocal femoral osteotomy (BFO) performed in nine non-ambulatory children with neuromuscular diseases (NMD), with a median age of 12.86 years, to resolve both hip subluxation and ipsilateral knee flexion contracture that impaired standing, and to evaluate patient and caregiver satisfaction. Results: Median surgical time was 4 h 15 min (2 h 35 min–5 h 50 min). Hip flexion deformity improved by a median of 30° (15–35), while median improvement in knee flexion deformity was 30° (20–50). Only two patients could use a standing frame prior to surgery, although with increasing difficulty, while all children could use it following SEMLS. Mean follow-up was 27.47 months (24.33–46.9). Significant blood loss requiring transfusion was the only complication recorded (8/9). All caregivers reported slight, moderate, or significant improvement in all domains of the questionnaire, and all would undergo the procedure again and recommend it to others, as nearly all (8/9) were very satisfied. Conclusion: The findings of this study suggest that SEMLS including BFOs in non-ambulatory children with NMD can correct hip, knee, and foot deformities and simultaneously realign lower limbs to restore functional standing and wheelchair transfer. The functional results, safety, and degree of satisfaction achieved justify offering this strategy to families.

Role of POP Boot Traction in Lower Limb Deformities

Background: In India, postpolio residual paralysis (PPRP) patients, present with multiple deformities, which requires surgical intervention, and in rural setting, it poses a big challenge for doctors. Traction is one of the oldest treatments in fracture reduction and deformity correction. The objective of this study was to evaluate the clinical outcome of plaster of Paris (POP) boot traction for the correction of lower limb deformities in PPRP patients. Materials and Methods: This retrospective case series represents 14 cases of PPRP with hip and/or knee deformity treated with POP boot traction for deformity correction, followed by caliper application. Deformities were classified as mild, moderate, and severe and assessed weekly during treatment. Results: There were 8 females and 6 males. The mean age of participants was 22.21 ± 8.107 years, and the mean duration of traction was 5.21 ± 1.76 weeks. There were seven patients with unilateral knee deformity, two patients with bilateral knee deformity, two patients with unilateral hip and knee deformity, and three patients with bilateral hip and knee deformity. The mean baseline deformity angle was 37.41, and the mean postprocedure deformity angle was 15.89. Conclusions: POP boot traction is a low-cost intervention in PPRP patients and can give promising results in the correction of lower limb deformities.

Hospital Creates AI Prototype for Hip Deformities in Children

Biomedical Safety & Standards 53(8):p 57-59, May 01, 2023. | DOI: 10.1097/01.BMSAS.0000927980.26922.f7

Artificial Intelligence and 3D Imaging Explored for Addressing Pediatric Hip Deformities

Lippincott's Bone and Joint Newsletter 29(5):p 55-56, May 2023. | DOI: 10.1097/01.BONEJ.0000932052.51559.20

“Minimal Brain Dysfunction” (MBD) Influence on the Development of the Feet, Knees, Hips, Pelvis and Spine, Causes, Clinic, Therapy

In pathology of locomotor system, there are many causes but Minimal Brain Dysfunction (MBD) appears very frequently [1-3,6,7,20,24,25,31]. Between patients examined in years 1995 – 2023 this group counted 18 % – 20 % of cases. Mostly children with deformities of feet, knees, hips, pelvis and spine. Important is early therapy - than will be received “healthy status” of movement apparatus in adults.

The articular and the craniocervical abnormalities are of confusing age of onset in patients with Maroteaux-Lamy disease (MPS VI).

Maroteaux-Lamy disease (MPS Type VI) is an autosomal recessive lysosomal storage disorder. Skeletal abnormalities are vast. Early recognition may facilitate timely diagnosis and intervention, leading to improved patient outcomes. The most challenging is when patients manifest a constellation of craniocervical and articular deformities with variable age of onset. We collected 15 patients with MPS VI (aged from 6 years-58 years). From within our practice in Pediatric Orthopedics, we present patients with MPS type VI who were found to manifest a diverse and confusing clinical presentation of hip deformities and cervical cord compression. Stem cell transplants were proposed as treatment tool and enzyme replacement therapy has been instituted in some patients. The spectrum of the clinical involvement in our group of patients was supported firstly via the clinical phenotype followed by assessment of the biochemical defect, which has been detected through the deficiency of N-acetylgalactosamine-4-sulfatase (arylsulphatase B) leading to increased excretion of dermatan sulphate. Secondly, through the molecular genetic results, which showed homozygous or compound heterozygous mutation in the ARSB gene on chromosome 5q14. Hip replacements and decompression operations have been performed to restore function and to alleviate pain in the former and life saving procedure in the latter. The efforts in searching for the etiological diagnosis in patients with skeletal dysplasia/MPSs has not been rewarding as many had anticipated. This emerged from several facts such as improper clinical documentation, missing diagnostic pointers in radiographic interpretations, limited knowledge in skeletal dysplasia and its variants, and the reliance on underpowered studies. Physicians and radiologists are required to appreciate and assess the diverse phenotypic and the radiographic variability of MPS VI. The importance of considering MPS in the differential diagnosis of other forms skeletal dysplasia is mandatory. Finally, we stress that the value of early diagnosis is to overcome dreadful complications.

Hip dysplasia: What influence do age, arthrosis and concomitant diseases have on the treatment result?

Pelvic osteotomies are an established treatment for symptomatic adult hip dysplasia with apromising long-term outcome. Results depend not only on the achieved acetabular reorientation but also on patient-factors like preoperative joint condition (degree of osteoarthritis and joint congruency) and age. Additionally, the diagnosis and appropriate therapy of impingement-associated hip deformities is essential in order to achieve good mid- and long-term outcomes. The influence of chondrolabral pathology on the outcome of pelvic osteotomies is not yet defined. Symptomatic patients with residual dysplasia after previous pelvic or acetabular osteotomies can benefit from an additional osteotomy, although results can be worse in comparison to prior unoperated joints. Obesity can make surgery more demanding and increases the complication profile of PAO, although it has no influence on the postoperative outcome. Regarding the overall prognosis after an osteotomy, the consideration of combined risk factors is superior to the concentration on individual factors alone.