Emad Anam

Background: Progressive pseudorheumatoid dysplasia (PPD) is a rare genetic skeletal disorder that is frequently missed or misdiagnosed due to overlapping features with juvenile idiopathic arthritis. This study aimed to assess the knowledge, diagnostic capabilities, and management practices of Saudi pediatric orthopedic surgeons in identifying and treating PPD. Materials and Methods: A cross-sectional survey was distributed to 120 pediatric orthopedic surgeons in Saudi Arabia, with 54 responses collected (response rate: 45%). The survey covered demographics, awareness, diagnostic knowledge, and clinical management of PPD. Descriptive statistics were used to summarize the data, and chi-square tests assessed associations between experience and confidence in managing PPD. Results: Only 31.5% of participants were aware of PPD, and 24.1% correctly identified WISP3 as the causative gene. Confidence in managing PPD was low, with only 7.4% of participants feeling very confident in long-term care. A large proportion of respondents (55.6%) were unsure about the pattern of joint involvement. While 51.9% of participants endorsed a multidisciplinary approach to management, misconceptions about laboratory and radiographic findings were common. The chi-square test showed no significant association between years of experience and confidence in managing PPD ( P = 0.21). Conclusion: This study highlights a considerable gap in knowledge and confidence among pediatric orthopedic surgeons in diagnosing and managing PPD. Implementing enhanced training programs, targeted educational initiatives, and multidisciplinary clinical guidelines is essential to improving awareness and patient outcomes. Integrating rare disease education into orthopedic residency programs could facilitate earlier diagnosis and appropriate management approaches.

Background/Objectives: Patellofemoral joint (PFJ) replacement is a bone-preserving option for isolated patellofemoral osteoarthritis; however, reported survivorship and failure patterns remain variable. This study evaluated implant survivorship, functional outcomes, reoperations, and failure mechanisms following PFJ replacement using standard second-generation implant systems, with or without patellar resurfacing. Methods: We retrospectively reviewed a consecutive cohort of 39 patients (48 knees) who underwent PFJ replacement for isolated patellofemoral osteoarthritis between 2011 and 2021. Median age at surgery was 59 years, and median body mass index (BMI) was 31 kg/m². Median follow-up for clinical and revision surveillance was 9 years (IQR 8–10). Functional outcomes were assessed using the Oxford Knee Score (OKS) and SF-12 Physical and Mental Component Scores (PCS and MCS). Implant survivorship was analyzed using Kaplan–Meier methodology, with conversion to total knee arthroplasty (TKA) as the endpoint. Statistical analyses were primarily descriptive and exploratory because only five TKA revisions occurred. Results: Median OKS improved from 19 (IQR 16–24) preoperatively to 36 (IQR 24–42) at the latest follow-up, with a median paired improvement of 17 points. SF-12 PCS improved from 25 to 47, and SF-12 MCS from 36 to 55. Eight knees (16.7%) underwent non-revision reoperation, and five knees (10.4%) underwent conversion to TKA. All TKA revisions were performed for the progression of tibiofemoral osteoarthritis. Kaplan–Meier survivorship free from TKA revision was 89.6% at 9 years (95% CI 76.8–95.5). No clear difference in TKA-free survivorship was detected between resurfaced and non-resurfaced knees. Conclusions: PFJ replacement demonstrated substantial functional improvement and mid- to long-term survivorship comparable to published registry ranges in a selected cohort with isolated patellofemoral osteoarthritis. TKA revision was uncommon and was attributable to the progression of tibiofemoral osteoarthritis. Because of the retrospective design, small cohort size, bilateral cases, and limited number of revision events, subgroup and risk-factor analyses should be interpreted as exploratory.

Sickle cell disease (SCD) is a genetic disorder characterized by chronic hemolysis and vaso-occlusive crises (VOCs), leading to musculoskeletal complications that significantly affect quality of life. Among these, shoulder complications are a concern, with humeral head avascular necrosis (AVN) being the second most common site of involvement after the femoral head. Other shoulder pathologies, including osteomyelitis and septic arthritis, further contribute to morbidity. However, these conditions remain underdiagnosed and understudied, often due to overlapping symptoms with VOC-related bone infarctions. Imaging, particularly magnetic resonance imaging, is crucial for early diagnosis and accurate differentiation. Management strategies range from conservative pain control to surgical interventions, including core decompression for early-stage AVN and arthroplasty for advanced joint destruction. Surgical outcomes in SCD, however, remain inconsistent due to higher complication rates and a lack of standardized guidelines. Despite advancements in diagnosis and treatment, shoulder pathology in SCD remains an area of limited research. This review highlights the need for larger, long-term studies with a homogeneous etiology to support and refine current treatment strategies and improve patient outcomes.