Parent-patient Discrepancies in the Quality of Life Assessment of Early-onset Scoliosis: A Comparison Between 2 Questionnaires Completed on 2 Different Time Points - A Preliminary Report.

BACKGROUND: EOSQ-24 is a parent proxy questionnaire designed to assess the health-related quality of life (HRQoL) of early-onset scoliosis (EOS) patients during their childhood years. EOSQ-SELF, a novel self-reported questionnaire, assesses HRQoL in older children (>8 y) and adolescents. So far, the same group of EOS patients has not been evaluated with both EOSQ-24 and EOSQ-SELF. The aim of this study was to evaluate how the same pathology was reflected in the parent and patient at different time points by comparing the answers to the common questions between EOSQ-24 and EOSQ-SELF. METHODS: A group of otherwise healthy EOS patients whose parents filled out EOSQ-24 at the early phase of growth-friendly treatment was re-tested by the EOSQ-SELF questionnaire at the end of treatment. Both EOSQ-24 and EOSQ-SELF are validated in Turkish. Inclusion criteria were patients with EOS, independent ambulation, age of 8 years or older at EOSQ-SELF enrollment, literacy in Turkish, no apparent intellectual impairment, and a minimum of 24 months after graduation. The common questions between the 2 surveys with nearly identical phrasings were extracted. Common items from the 2 tests were compared with a Wilcoxon signed rank test. RESULTS: Twenty-one patients (15 females, 6 males) who previously filled out EOSQ-24 met the inclusion criteria. The mean age of the group was 10 (5 to 16) years at EOSQ-24 participation and 18 (13 to 24) at the final analysis. Fourteen questions were found common in 10 domains. The scores were significantly different in 5 questions of 4 domains. EOSQ-SELF had significantly less favorable scores in the pain/discomfort, pulmonary function, and fatigue/energy level domains. Scores in the parental burden/relationships domain were significantly higher (P<0.05). CONCLUSIONS: The self-reported group had a general trend of worse results. Parents and caregivers may not accurately perceive the problems of EOS patients. Our findings indicate a disconnect between caregivers and the patients, as both parties underreported the other side in some domains. These findings suggest the challenges faced by EOS patients are not adequately reflected on proxy questionnaires that assess the HRQoL of children. LEVEL OF EVIDENCE: Diagnostic Level I.

Early results of a novel modular knee arthrodesis implant after uncontrolled periprosthetic knee joint infection.

AIM: The aim of this study is to evaluate the functional outcomes and complications after non-fusion knee arthrodesis with a modular segmental intramedullary implant used for infected total knee arthroplasty revisions. METHODS: A retrospective review of the patients who had been surgically treated with a modular intramedullary arthrodesis implant for recurrent infection after revision TKA between January 2016 and February 2020 were included. The indications for arthrodesis were failed infected TKA with massive bone loss, deficient extensor mechanism and poor soft tissue coverage that precluded joint reconstruction with revision TKA implants. Clinical outcomes were assesed with visual analogue scale for pain (pVAS), Oxford knee score (OKS) and 12-item short form survey (SF-12). Full-length radiographs were used to verify limb length discrepancies (LLD). RESULTS: Fourteen patients (4 male and 10 female) patients with a mean age of 69.3 (range, 59 to 81) years at time of surgery were available for final follow-up at a mean of 28.8 months (range, 24-35 months). All clinical outcome scores improved at the final follow-up (pVAS, 8.5 to 2.6, p = .01; OKS, 12.6 to 33.8, p = .02; SF-12 physical, 22.9 to 32.1, p = .01 and SF-12 mental, 27.7 to 40.2, p = .01). The mean LLD was 1.0 cm (range, + 15 - 2.3 cm). Re-infection was detected in three patients (21.4%). Two patients were managed with suppressive antibiotic treatment and a third patient required repeat 2-stage revision procedure. In one patient, a periprosthetic femur fracture was observed and treated with plate osteosynthesis. CONCLUSION: Uncontrolled infection after total knee arthroplasty can be effectively treated with arthrodesis using a modular intramedullary nail and satisfactory functional results can be obtained. LEVEL OF EVIDENCE: Level 4, Retrospective cohort study.

Outcomes of Limb Lengthening With Computer-assisted Hexapod External Fixators in the Treatment of Fibular Hemimelia.

BACKGROUND: Fibular hemimelia (FH) represents the most common deficiency of the long bones and is associated with multiple deformities. Reconstructive treatment with external fixators in FH restores normal lower extremity alignment and length with plantigrade feet for a balanced and effective gait. The aim of this study is to evaluate the outcomes of lower limb lengthening and simultaneous tri-plane deformity correction with a computer-assisted hexagonal external fixator in children with FH. METHODS: A retrospective review was performed for FH cases treated with a computer-assisted hexagonal external fixator in a tertiary referral center. Leg length discrepancy (LLD), interphyseal angles, tibiocalcaneal distances, healing index (HI), and callus shapes were analyzed for radiologic evaluation, and the Pediatric Quality of Life Inventory (PedsQL) was used for functional assessment. Limbs with HI <50 days/cm, PedsQL >75, and without regenerate fractures were considered successful lengthenings. RESULTS: Twenty-four limbs of 23 patients were included. The limbs were lengthened for a mean of 7.24 cm (range, 4.7 to 15.6). The initial LLD of 5.6 cm (range, 0.5 to 19 cm) increased to 1.7 cm (range, 0.1 to 6 cm), and the mean interphyseal angle was 12.7 degree (range, 1.5 to 54.2 degree), tibiocalcaneal distance was 0.85 cm (range, 0.1 to 1.7) at final follow-up. The most common regenerated bone morphology was cylindrical, as seen in 11 limbs (45.8%). The average PedsQL score was 83.5 (range, 69.5 to 96.7). Sixteen limbs (66.7%) had successful lengthening at their first, and 4 limbs (80%) had successful lengthening at their second surgeries. Seven limbs had complications requiring surgical intervention (29.1%), with 3 (12.5%) regenerate fractures after external fixators removal. CONCLUSIONS: Limb reconstruction with computer-assisted hexapod fixators is a successful and reliable option for the treatment of LLD in FH, and patients demonstrate good functional outcomes. Surgeons should be aware of potential complications and should utilize prophylactic measures when necessary. LEVELS OF EVIDENCE: Level III, retrospective comparative study.

A Comprehensive Assessment of Psychosocial Well-being Among Growing Rod Graduates: A Preliminary Investigation.

BACKGROUND: Traditional growing rods (TGR) encompass a long process, in which patients experience physical and psychosocial difficulties. However, the effect of repeating surgeries on the overall psychological functioning of graduated patients has not been thoroughly investigated in the literature. The aim of this study is to evaluate the psychological well-being of graduated idiopathic early-onset scoliosis patients in terms of psychopathology, neurocognition, and psychosocial functioning, and determine the accuracy of scoliosis outcome questionnaires in these regards. METHODS: TGR graduates with idiopathic early-onset scoliosis without known intellectual disabilities or neuromuscular impairments were included. Patients were thoroughly evaluated using psychological instruments [Wechsler Adult Intelligence Scale, Auditory Consonant Trigram Test, Verbal Fluency Test, Beck Depression Inventory, Beck Anxiety Inventory, Rosenberg Self-Esteem Scale, Symptom Checklist-90, Post-Traumatic Growth Inventory, Strengths and Difficulties Questionnaire, Spinal Appearance Questionnaire, Scoliosis Research Society 22-item questionnaire (SRS-22)]. Results were compared with normative data when available. Spearman correlations were performed between the results of these tests, the total treatment duration, and the number of spinal surgeries. RESULTS: Of the 15 patients included in the study, 9 were females, and the mean age was 18.73 (16 to 23). The mean age at index surgery was 6.38 (3 to 10) whereas that of graduation was 14.00 (12 to 16). The average number of spinal surgeries was 14.28 (7 to 20). Two patients performed below the range of adult intellectual functioning. Auditory Consonant Trigram Test showed normal verbal working memory and attention control. Six patients had abnormal Verbal Fluency Test performance. Eight patients had abnormal ratings on at least one of the assessment scales of psychopathology (Symptom Checklist-90, Beck Depression Inventory, and Beck Anxiety Inventory). Eight patients had low-to-moderate self-esteem (Rosenberg self-esteem scale). The median spinal appearance questionnaire and SRS-22 scores were 34 and 4.18, respectively. Pain and function subdomains of SRS-22 scored higher than self-image and mental health. No correlation was found between the treatment duration and number of surgeries and test scores. SRS-22 showed correlations with multiple psychological tests. CONCLUSION: Completed TGR treatment yields acceptable correction of deformities and surgical outcomes, however, may fail to improve psychological well-being. This is the first study to find various psychosocial abnormalities in two-thirds of cases. LEVEL OF EVIDENCE: Level IV; cross-sectional study.

Primary Cutaneous Adenoid Cystic Carcinoma in a Rare Location With an Immune Response to a BNT162b2 Vaccine: A Case Report.

CASE: In this report, a rare case of primary cutaneous adenoid cystic carcinoma (PCACC) localized in the subcutaneous tissue of the scapular region that grew after BNT162b2 corona virus disease of 2019 (COVID-19) vaccination is presented and may be explained by CD4 and CD8 cell infiltration. The BNT162b2 mRNA vaccine has been associated with a multisystem inflammatory syndrome (MIS-V). A comparable immune reaction could potentially enhance tumor growth rate. CONCLUSION: Primary cutaneous adenoid cystic carcinomas are rare tumors with unique locations. Further studies with case series are required to establish management algorithms for PCACC and investigate the potential effect of vaccination.

Comprehensive single cell transcriptomics analysis of murine osteosarcoma uncovers Skp2 function in metastasis, genomic instability and immune activation and reveals additional target pathways.

Osteosarcoma (OS) is the most common primary pediatric bone malignancy. One promising new therapeutic target is SKP2, encoding a substrate recognition factor of the SCF E3 ubiquitin ligase responsible for ubiquitination and proteasome degradation of substrate p27, thus driving cellular proliferation. We have shown previously that knockout of Skp2 in an immunocompetent transgenic mouse model of OS improved survival, drove apoptosis, and induced tumor inflammation. Here, we applied single-cell RNA-sequencing (scRNA-seq) to study primary OS tumors derived from Osx-Cre driven conditional knockout of Rb1 and Trp53. We showed that murine OS models recapitulate the tumor heterogeneity and microenvironment complexity observed in patient tumors. We further compared this model with OS models with functional disruption of Skp2: one with Skp2 knockout and the other with the Skp2-p27 interaction disrupted (resulting in p27 overexpression). We found reduction of T cell exhaustion and upregulation of interferon activation, along with evidence of replicative and endoplasmic reticulum-related stress in the Skp2 disruption models, and showed that interferon induction was correlated with improved survival in OS patients. Additionally, our scRNA-seq analysis uncovered decreased activities of metastasis-related gene signatures in the Skp2-disrupted OS, which we validated by observation of a strong reduction in lung metastasis in the Skp2 knockout mice. Finally, we report several potential mechanisms of escape from targeting Skp2 in OS, including upregulation of Myc targets, DNA copy number amplification and overexpression of alternative E3 ligase genes, and potential alternative lineage activation. These mechanistic insights into OS tumor biology and Skp2 function suggest novel targets for new, synergistic therapies, while the data and our comprehensive analysis may serve as a public resource for further big data-driven OS research.

Postoperative bleeding adversely affects total knee arthroplasty outcomes in hemophilia.

BACKGROUND: Hemophilic arthropathy can result in severe degenerative arthritis and functional limitations in the knees of relatively young patients. Total knee arthroplasty (TKA) provides pain relief and gain of function in advanced-stage hemophilic arthropathy cases. However, little is known about the long-term effects of early major postoperative bleeding (MPOB) in people with hemophilia (PWH). The aim of this study was to evaluate the effects of early MPOB on the final functional outcome, complications, and implant survival of TKA in a single-center hemophilia cohort. METHOD: PWH who underwent TKA between 1998 and 2019 in a single center were reviewed. Demographic data, clinical data, and radiographic images were evaluated. Hospital for Special Surgery (HSS), Knee Society Score (KSS), and Knee Society Function Score (KSS-F) scores were used to determine function. Patients with early bleeding complications (wound dehiscence, ecchymosis, hemarthrosis, hematoma formation, prolonged or recurrent bleeding attacks) were defined as the bleeding group. Patients who did not experience these complications were assigned to the control group. The bleeding group was compared with controls. Survival of the primary arthroplasty was analyzed by Kaplan-Meier curves. RESULTS: Forty-five TKAs in 29 patients were included in the study. TKA led to an increase in the mean range of motion from 46.08° to 84.59° (P < 0.01). HSS scores increased from 48.33 preoperatively to 82.67 postoperatively (P < 0.01). There were improvements in both KSS and KSS-F scores from 34.22 and 53.3 preoperatively to 82.00 and 84.63 (P < 0.01), respectively. Ten patients (10 TKAs) (34%) experienced major bleeding during the postoperative period. Six of these patients had moderate hemophilia, and four had severe hemophilia. Three of these patients had hemarthroses (10.2%), one patient had a hematoma (3.4%), one patient had hemorrhagic bullae formation (3.4%), and five had excessive/prolonged bleeding from the wound (17%). The bleeding group (34%) had significantly worse HSS (63.78 vs 92.75, P < 0.001), KSS (61.78 vs 93.25, P < 0.001), and KSS-F (60.71 vs 96.25, P = 0.005) scores compared with controls. Preoperative and postoperative flexion contractures were positively correlated (+0.33, P = 0.003). One of the patients with postoperative hemarthrosis also had an accompanying transient common peroneal nerve palsy, and one patient (3.4%) had a periprosthetic fracture. Three knees (6.6%), two of whom were in the bleeding group, developed periprosthetic infections. Four knees (8.8%) in three patients underwent revision surgery, and two knees (4.4%) ended up in arthrodeses. Kaplan-Meier analysis revealed a mean survival duration of 17.04 years for the bleeding group and 22.15 years for the control group (P = 0.83). Survival rates were 80.0% for the bleeding group and 96.4% for the control group (P = 0.83). CONCLUSIONS: In this study, MPOB after TKA in PWH was common and led to significantly worse function. MPOB after TKA in PWH was associated with a higher rate of complications and lower survival rates, although the differences were not statistically significant. Efforts must be made to avoid MPOB after TKA in PWH.

Hip-preserving reconstruction of the proximal femur with a vascularized fibula autograft and liquid-nitrogen-treated tumor bearing bone.

A five-year-old boy with Ewing sarcoma of the proximal femur was operated at our institution with limb-sparing surgery and biological reconstruction of the proximal femur with a vascularized fibular autograft. During this procedure, the proximal femur was soaked in liquid nitrogen which was subsequently fixed to the fibular autograft, while the epiphysis of the femoral head was spared. To the best of our knowledge, this is the first report of epiphysis-sparing surgery of the proximal femur via a vascularized fibular autograft surrounded by a liquid nitrogen-treated tumor bearing bone autograft. Three years postoperatively, the patient remains disease-free, has a full weight-bearing extremity with good function, and a remodeled proximal femur with minimal deformity. There are no radiological or clinical signs indicative of femoral head osteonecrosis. In conclusion, hip-sparing biological reconstruction is a successful method of limb preservation as an alternative to tumor endoprostheses.

The effect of the COVID-19 pandemic on orthopedic surgeries in a tertiary referral center.

OBJECTIVES: The aim of this study is to investigate the effect of the novel coronavirus-2019 (COVID-19) pandemic on the operational trends in the orthopedic surgery department of a tertiary referral center. PATIENTS AND METHODS: A total of 305 orthopedic surgical procedures in 245 patients (136 males, 109 females; mean age: 34±26.6 years; range, 0 to 91 years) between March 16th and June 27th, 2020 were retrospectively analyzed. The same period of the year before including 860 procedures in 783 patients (364 males, 419 females; mean age: 33.6±25.8 years; range, 0 to 95 years) was also reviewed as a pre-pandemic control group. Patient demographics, surgical indications, COVID-19 polymerase chain reaction (PCR) test status, method of anesthesia, surgical subspecialties (trauma, sports, etc.), trauma mechanisms, and surgical priorities were evaluated. The pandemic and the pre-pandemic periods were compared. RESULTS: The rate of elective surgeries decreased compared to the previous year, and priority C type surgeries had the highest frequency (42.5%). Orthopedic trauma was the leading subspecialty with 91 (29.8%) cases and had a higher share, compared to the pre-pandemic period (17.0%). Hip fractures (18.7%) were the most common cause of trauma surgery, and simple falls (42.3%) composed the largest group of trauma mechanisms, which was similar to the pre-pandemic period (hip fractures, 13.6%; simple falls, 42.5%). The distribution of surgical urgency levels and subspecialties differed significantly between the pre-pandemic and pandemic periods (p<0.001). Post-hoc analysis of subspecialty distribution revealed a significant decrease in arthroplasty (p=0.002) and hand surgery (p<0.001), and a significant increase in trauma (p<0.001) and the "other" category (p<0.001). CONCLUSION: Our experience in a tertiary referral center illustrated a shift toward performing emergent and urgent surgeries, when the severity of the outbreak increased. Prioritizing surgical urgencies during the outbreak changed the orthopedic surgery practice with an emphasis on trauma and oncology surgeries. Hip fractures were the most common cause of trauma surgery, and simple falls composed the largest group of trauma mechanisms.

Preliminary results of a new intercalary modular endoprosthesis for the management of diaphyseal bone metastases.

OBJECTIVES: This study aims to evaluate functional outcomes of patients and to analyze complication rates of modular intercalary endoprosthetic reconstruction after resection of metastatic diaphyseal bone lesions. PATIENTS AND METHODS: Between December 2017 and February 2020, 22 patients (15 males, 7 females; median age: 64.2 years; range, 49 to 91) who underwent reconstruction with modular intercalary endoprostheses for metastatic bone tumors at five different centers were retrospectively analyzed. Age, sex, diagnosis, follow-up duration, previous treatments of patients, and resection lengths were recorded. The Musculoskeletal Tumor Society Scores (MSTS) were used to assess functional status of available patients at the final follow-up. Failures were categorized according to the Henderson classification. RESULTS: Locations of the resected tumors included 10 humeri (45.5%), five tibiae (22.7%), and seven femurs (31.8%). The length of the resected tissues ranged from 35 mm to 180 mm. Seven patients (31.8%) died of disease, and one patient died of pneumonia within follow-up period. The functional outcomes of surviving patients were satisfying with a median MSTS score of 86.9% (range, 70 to 100%) at a median follow-up of 17 (range, 8 to 26) months. There were two cases of type II (9%), one cases of type IIIa (4.5%), two cases of type IIIb (9%), and one case of type IV (4.5%) failure. Complications were most commonly observed in tibial reconstructions. CONCLUSION: The good short-term functional results were achieved in surviving patients. Uncomplicated patients were able to perform daily living activities without limitations. The overall rate of complications was relatively low and, among them, mechanical problems were the most commonly encountered problems.