Aplasia Cutis Congenita with Ectrodactyly Skeletal Syndrome (ACCES, OMIM #619959) is an extremely rare multiple congenital anomalies syndrome caused by haploinsufficiency of the UBA2 gene. This syndrome presents with growth retardation, dysmorphic facial features, neurodevelopmental delay, skeletal problems including ectrodactyly, developmental dysplasia of the hip (DDH) and scoliosis, skin findings such as aplasia cutis, and some internal organ abnormalities. Our 13-year-old female patient and her 38-year-old father had a skeletal dysplasia phenotype with disproportionate short stature, bilateral DDH, mild epiphyseal involvement, scoliosis, and increased lumbar lordosis. Both were neurodevelopmentally normal and had mild dysmorphic facial features and mild ectodermal findings. The dominant inheritance pattern in the pedigree suggested a pre-diagnosis of spondyloepiphyseal dysplasia tarda. The exome sequencing analysis of the patient has identified a novel heterozygous variant, NM_005499.2:c.460-2A >G, in the UBA2 gene, and the father was found heterozygous either. The isolated spondyloepiphyseal involvement of our patients was an unusual presentation compared to patients with ACCES syndrome previously reported in the literature. Considering the highly variable expressiveness of ACCES syndrome and the co-occurrence of familial hip dysplasia and vertebral problems, we suggest that this syndrome can also be classified under \"Spondyloepi(meta)physial dysplasia (SE(M)D)\" in the nosology of genetic skeletal disorders.

UNASSIGNED: Surgical indications for Bernese periacetabular osteotomy (PAO) are well-established. However, the extent of postoperative functional recovery varies widely, as observed in clinical follow-ups. Thus, preoperative evaluation is crucial. This study aims to identify factors that influence functional recovery post-PAO and to develop a predictive nomogram.
UNASSIGNED: Retrospective data were collected between December 2016 and March 2022 at The First Affiliated Hospital of Shandong First Medical University. The dataset included demographic and imaging data of patients who underwent PAO. The least absolute shrinkage and selection operator (LASSO) regression was utilized to identify influencing factors, which were further analyzed using multivariate logistic regression to construct a predictive nomogram for post-PAO functional recovery.
UNASSIGNED: The analysis identified critical factors affecting functional recovery post-PAO, namely, the preoperative distance from the innermost surface of the femoral head to the ilioischial line, the surgical approach, preoperative acetabular depth, and the continuity of the preoperative Calve line. A nomogram was developed using these significant predictors. The model\'s validity was demonstrated by the receiver operating characteristic curve, with an area under the curve of 0.864. Additionally, the calibration curve confirmed the nomogram\'s accuracy, showing a strong correlation between observed and predicted probabilities, indicating high predictive accuracy.
UNASSIGNED: This predictive nomogram effectively identifies patients most suitable for PAO, providing valuable guidance for selecting surgical candidates and determining the appropriate surgical approach.

BACKGROUND: Developmental dysplasia of the hip (DDH) is a common cause of childhood disability, and the incidence of DDH shows significant familial aggregation. As the genetic factors of DDH remain unknown, the correlation between five candidate single nucleotide polymorphisms (SNPs) and DDH was evaluated in the Han Chinese population of Southwest China.
METHODS: A case‒control association study was conducted in 276 patients with DDH and 318 healthy controls. SNP genotyping in the case and control groups was performed by SNPshot and multiple PCR. SNPs were genotyped in the case and control groups by multiplex PCR. The relationship between DDH and candidate SNPs was evaluated using the χ2 test.
RESULTS: The genotype distributions of rs291412 in HIBCH and rs769956 in FTCDNL1 were different between the case and control groups (P < 0.05). After genetic model analysis, logistic regression analysis revealed that the C allele of rs291412 had a protective effect on DDH (OR = 0.605, P = 0.010) and that the G allele of rs769956 was a risk factor (OR = 2.939, P = 0.010).s.
CONCLUSIONS: These SNPs could be associated with susceptibility to DDH but larger population-based studies should confirm the current results.

OBJECTIVE: This study aimed to analyze and compare gait patterns and deviations at long-term follow-up in children who received medial open reduction (MOR) before 18 months for unilateral or bilateral hip developmental dysplasia (DDH).
METHODS: A retrospective chart review was conducted on children who underwent MOR. The study population was divided into two groups: the unilateral group, including unilateral (five children with unilateral) and bilateral (five children with bilateral DDH). Ten healthy children were recruited for the control group. Spatiotemporal, kinematic, stiff-knee gait (SKG), and kinetic gait characteristics were analyzed.
RESULTS: Stance time was significantly shorter in both the unilateral (median [IQR]; 590 ms, [560.0-612.5] and bilateral (575 ms, [550-637.5]) groups than in the control group (650, [602.5-677.5]) (p < 0.001), whereas swing time did not differ substantially (p = 0.065) There was no considerable difference in the mean knee flexion at swing between the unilateral (31.6°, [30-36]) and control (30.11°, [27.8-33.6] groups (p > 0.05), but the bilateral group (28.5°, [24.9-32.1]) showed the lower values than the other groups (p < 0.001 for bilateral vs unilateral group; p = 0.008 bilateral vs unilateral group). All the SKG parameters significantly differed among the groups in multi-group comparisons (p < 0.001 for each parameter). Three children had borderline SKG, and two had not-stiff limbs in the unilateral group. In the bilateral group, four children had stiff limbs, and one had borderline SKG. Most kinetic gait parameters were not statistically different between groups (p > 0.05).
CONCLUSIONS: This study has revealed notable deviations in gait patterns of children with DDH treated by MOR at long-term follow-up compared to healthy children\'s gait. MOR could negatively affect pelvic motion during gait due to impaired functions of the iliopsoas and adductor muscles, and SKG can be encountered secondary to iliopsoas weakness.

OBJECTIVE: Factors affecting direction of pelvic obliquity (PO) in dysplastic hip osteoarthritis (DHOA) remains unclear. This retrospective cohort study evaluates morphological characteristics, spinal alignment, and hip function in patients with unilateral DHOA.
METHODS: Between 2018 and 2022, 104 patients with unilateral DHA were enrolled. Patients were categorized into flat PO (F-PO group; PO < 2°), affected side PO (A-PO group; PO downward by ≥ 2°), and unaffected side PO (U-PO group; PO upward by ≥ 2°). Demographics, radiographic hip and lower limb parameters, spinal parameters, and functional scores were compared between the groups.
RESULTS: There were 39, 44, and 21 patients in the F-PO, A-PO, and U-PO group, respectively. The subluxation percentage of Crowe classification showed a significant difference among the three groups. The femoral head lateralization distance was significantly greater in the U-PO group than in the F-PO and A-PO groups. Furthermore, the hip adduction angle was significantly lower in the A-PO group than in the F-PO and U-PO groups. The lumbar scoliosis angle was significantly different between the groups. In multivariate analysis, hip adduction angle was extracted as an independent factor associated with the A-PO. Age, subluxation percentage, and hip adduction angle were identified as independent factors associated with the U-PO. Harris hip score was significantly poorer in U-PO group than in F-PO group.
CONCLUSIONS: Hip adduction angle influenced A-PO, while age, subluxation percentage, and hip adduction angle influenced U-PO; lumbar scoliosis angle was associated with PO direction. U-PO patients had poorer functional scores, indicating the impact of hip contracture and subluxation on PO direction in DHOA.

UNASSIGNED: Our purpose was to analyze the impact of Pavlik Harness treatment on children motor skills development, comparing to a control group.
UNASSIGNED: A total of 121 children were included: 55 cases (children with Developmental Dysplasia of the Hip) and 66 healthy controls. Cases were recruited from 2017 to 2021 and followed up to 2022. Controls (healthy children without orthopedic pathology) were recruited from 2020 to 2022. The primary endpoint was the time of achievement of three gross motor milestones (sitting without support, hands-and-knees crawling, and walking independently).
UNASSIGNED: The groups had no differences regarding sex distribution, gestational age, birth weight, and rate of twin pregnancy. The prevalence of positive family history of Development Dysplasia of the Hip (20.0% vs 3.0%, p < 0.003), breech presentation (38.2% vs 1.5%, p < 0.001), and C-section delivery (60.0% vs 19.7%, p < 0.001) was significantly higher in Development Dysplasia of the Hip group. Children with Development Dysplasia of the Hip achieved the three gross milestones evaluated 1 month later than healthy controls, although this was not statistically significant (p = 0.133 for sitting, p = 0.670 for crawling, and p = 0.499 for walking).
UNASSIGNED: Children with Development Dysplasia of the Hip, treated by Pavlik harness, do not have significant delays in motor skills acquisition.

BACKGROUND: Total hip arthroplasty (THA) has predominantly been utilized to treat older patients with primary osteoarthritis. However, recent improvements in surgical technique and implant materials have increased implant longevity, making THA a viable option for younger patients (< 30 years old). While trend analyses indicate an expanding utilization of THA in younger patients with non-OA diagnoses, current data on mid- and long-term THA outcomes in this population are limited. This study aims to describe the demographics, perioperative data, and implant survivorship of patients younger than 30 years undergoing THA.
METHODS: A retrospective study across two large urban academic medical institutions identified 135 patients aged < 30 years who underwent 155 primary THAs between 2012 and 2017, with up to 10-year clinical follow-up. Baseline demographics, surgical indications, intraoperative details, and postoperative outcomes were analyzed.
RESULTS: The mean age at surgery was 24.9 years (range 18-29). Osteonecrosis of the femoral head (55.5%), developmental hip dysplasia (28.3%), and arthritis (15.5%) were the primary indications. Most surgeries utilized the posterior approach (64.5%), manual technique (65.8%), and Ceramic-on-Polyethylene articulation (71.6%). Seven patients (4.5%) were readmitted within 90-days of surgery, including three non-orthopedic-related readmissions (1.9%) and four orthopedic-related readmissions (2.6%). Dislocations were reported in two patients (1.3%). There were six all-cause revisions (3.9%), four aseptic (2.6%) and two septic (1.3%). Kaplan-Meier analysis showed 10-year survival from implant retaining reoperation was 98.7%, 10-year survival from all-cause revision was 96.1%, and 10-year survival from aseptic revision was 97.4%.
CONCLUSIONS: With a 10-year aseptic implant survivorship rate of 97.4%, THA is a reliable surgical intervention for patients younger than 30 years of age who have severe hip pathology. Further studies are warranted for a more comprehensive understanding of mid- and long-term survivorship risk factors in this demanding population, facilitating improved risk assessment and informed surgical decisions.

Bimodal convolutional neural networks (CNNs) are frequently combined with patient information or several medical images to enhance the diagnostic performance. However, the technologies that integrate automatically generated clinical measurements within the images are scarce. Hence, we developed a bimodal model that produced automatic algorithm for clinical measurement (aaCM) from radiographic images and integrated the model with CNNs. In this multicenter research project, the diagnostic performance of the model was investigated with 813 radiographic hip images of infants at risk of developmental dysplasia of the hips (232 and 581 images of unstable and stable hips, respectively), with the ground truth defined by provocative examinations. The results indicated that the accuracy of aaCM was equal or higher than that of specialists, and the bimodal model showed better diagnostic performance than LightGBM, XGBoost, SVM, and single CNN models. aaCM can provide expert\'s knowledge in a high level, and our proposed bimodal model has better performance than the state-of-art models.

The direct anterior approach (DAA) has been increasing in popularity for primary total hip arthroplasty (THA). Despite previously documented anatomic limitations to its direct distal extension, alternative exposure methods have been described to safely access the femoral diaphysis and facilitate increasingly complex primary and revision THA scenarios. The DAA has several purported advantages compared to alternative approaches (eg, posterior and lateral-based), including its muscle-sparing nature, use of an internervous plane, and preservation of posterior stabilizing structures. Proponents of the DAA cite decreased postoperative pain, quicker recovery times, potentially lower dislocation rates, ease of intraoperative fluoroscopy, and improved implant placement/restoration of leg lengths. The current literature, however, is sparse when considering the use of this approach in the setting of severely dysplastic hips necessitating a concurrent subtrochanteric shortening osteotomy. When utilizing a posterior approach in this population, previous work from Ollivier and colleagues demonstrated high rates of cementless implant osseointegration and significantly improved clinical outcomes at long-term follow-up. Although relatively few reports of addressing this pathology via the DAA currently exist, initial results are promising. This study seeks to provide a detailed description of a surgical technique for performing primary THA and ipsilateral subtrochanteric shortening osteotomy in this patient population utilizing an extensile DAA.

To investigate the efficacy of chiropractic rehabilitation therapy in Crowe IV developmental dysplasia of the hip (DDH) patients after total hip arthroplasty. Seventy-two patients with Crowe IV type DDH hospitalized in the Department of Orthopedics I of Ya\'an Hospital of Traditional Chinese Medicine from January 2021 to June 2023 were selected for the study, and they were divided into 36 cases in the chiropractic rehabilitation therapy group (the treatment group) and 36 cases in the traditional rehabilitation therapy group (the control group) according to the method of randomized grouping. All patients were evaluated at preoperative, 1, 3, and 6 months postoperatively for follow-up, and the muscle strength of the affected limb, the patient\'s walking gait, the shortened length of the affected limb, the visual analog scale score (VAS score), the Oswestry Dysfunction Index Score (ODI score), the Harris Hip Score, and the degree of pelvic tilt were recorded to evaluate the results of the study. A total of 4 subjects withdrew from the study, 2 in the treatment group, and 2 in the control group. The muscle strength of the affected limb, walking gait, shortened length of the affected limb, VAS score, ODI score, Harris score, and pelvic tilt in the treatment and control groups improved significantly compared with the preoperative period. Comparisons between the 2 groups revealed that at the final follow-up visit, the limp gait of the patients in the treatment group was significantly reduced, the shortened length of the affected limb was significantly reduced, the VAS score was significantly reduced, and the ODI score was significantly reduced, in the treatment group relative to that of the control group, Harris Hip Score was significantly improved, and the degree of pelvic tilt was significantly reduced, but the improvement in muscle strength of the affected limb was not statistically significant. In future clinical practice, we recommend that chiropractic rehabilitation be used as a routine adjunctive treatment after TKA in patients with Crowe IV DDH to optimize outcomes and improve patients\' quality of life.