Faculty Bibliography

PURPOSE OF REVIEW/OBJECTIVE:The pathogenesis of dedifferentiated chondrosarcoma is controversial, and no genetic abnormality has consistently been identified in the disease. Focusing on the diagnostic challenges encountered in dedifferentiated chondrosarcoma, the following review aims at summarizing the tumor's active neoplastic pathways while highlighting therapeutic modalities that could potentially be explored to enhance patient survivorship. RECENT FINDINGS/RESULTS:Owing to the challenging examination of small needle biopsy sampling as well as the disease's overlapping morphological and immunohistochemical features with other bone and soft-tissue sarcomas, the diagnosis of dedifferentiated chondrosarcoma can be problematic. While combined doxorubicin- and cisplatin-based regimens remain the first-line systemic chemotherapy in the disease, ~50% of tumors carry EXT1/2 or IDH1/2 mutations, advancing EXT or IDH inhibitors as potential alternative therapies, respectively. Despite systemic chemotherapy, dedifferentiated chondrosarcoma remains an aggressive tumor with dismal prognosis and limited survival. A multidisciplinary collaboration across multiple cancer centers is warranted to yield an accurate diagnosis, understand the disease's underlying pathogenesis, develop adequate treatment, and improve patient survivorship.

BACKGROUND:This investigation aimed to determine the degree of correlation among 3 functional scales for evaluating the hip in pediatric patients and determine the correlation between measures of global function and outcome. METHODS:We performed a prospective study of 173 consecutive patients (Mage = 13 ± 3 y) being followed for developmental dysplasia of the hip (n = 122, 71%), slipped capital femoral epiphysis (n = 31, 18%), or Legg-Calve-Perthes disease (n = 20, 12%). We evaluated patients clinically, and we compiled scores for the Iowa Hip Score (IHS), Harris Hip Score (HHS), and Children's Hospital Oakland Hip Evaluation Scale (CHOHES). Patients concomitantly completed the Pediatric Outcomes Data Collection Instrument (PODCI) at the same clinic visit. We assessed Global Functioning Scale the and the Sports and Physical Functioning Core Scale of the PODCI. We determined the degree of correlation between the functional hip scales and between each scale and the PODCI scales using Spearman rank correlation coefficients. RESULTS:The correlations between the IHS, HHS, and CHOHES scores were robust (IHS and HHS ρ = 0.991; IHS and CHOHES ρ = 0.933; HHS and CHOHES ρ = 0.938; all P < 0.001). The correlation between the Global Functioning Scale of the PODCI and the 3 hip scores was ρ = 0.343 for the IHS, ρ = 0.341 for the HHS, and ρ = 0.352 for the CHOHES (all P < 0.001). The correlation between the sports and physical functioning core scale of the PODCI and the 3 hip scores was ρ = 0.324 for the IHS, ρ = 0.329 for the HHS, and ρ = 0.346 for the CHOHES (all P < 0.001). CONCLUSIONS:In a pediatric population with diverse hip pathology, there was a very strong correlation between scores on the IHS, HHS, and CHOHES. However, none of these 3 most commonly used hip scores correlated strongly with the global functioning scale or sports and physical functioning core scale of the PODCI. The most frequently used scores to determine the outcome of pediatric patients with hip pathology correlate strongly with each other but do not necessarily relate to global functional results. LEVEL OF EVIDENCE/METHODS:Level IV.

BACKGROUND:Children with suspected fractures are typically evaluated with multiple x-rays. This approach can add time, discomfort, and radiation exposure without clinical benefit. The purpose of this study was to determine whether a focused radiographic protocol could reduce the number of x-rays performed without missing any fractures. METHODS:Pediatric patients presenting at 2 sites within an academic medical center [urgent care (UC) and the emergency department(ED)] for a suspected fracture were identified. There were 495 patients (UC, 409; ED, 86) over a 3-month period. A retrospective chart review was performed to characterize the standard x-rays performed. Using this data, a simplified protocol was developed and distributed. Subsequently, 333 patients (UC, 259; ED, 74) were evaluated over the same period 1-year later. The main outcome measures included the final diagnosis, the total number of x-rays, the number of anatomic areas imaged, visit length, and the time for additional trips to radiology. Charts were reviewed to identify any missed injuries. Welch 2-sample t tests and Fisher exact tests were used for statistical analysis. RESULTS:After implementing the radiographic protocol, there was a significant reduction in the number of x-ray views, 3.4 versus 5.1 (P<0.001). There was a decrease in imaging of multiple anatomic areas with the largest reduction occurring in patients presenting with elbow injuries (9% vs. 44%, P<0.001). No difference was found in the rate of patients sent back to radiology (6% vs. 7%, P=0.67). However, among patients presenting with outside imaging, significantly fewer were sent to radiology for additional x-rays (29% vs. 50%, P<0.01). CONCLUSION/CONCLUSIONS:A simple radiographic protocol for evaluating pediatric patients with suspected fractures safely led to a decrease in the overall number of x-rays without missing any injuries. LEVEL OF EVIDENCE/METHODS:Level III, retrospective comparative study.

Background: The formation of destructive pseudotumors is a well-documented, albeit rare, complication of total hip arthroplasties. They tend to be progressive and, if left untreated, can result in extensive periprosthetic bony destruction. The current case presents a large benign mass consistent with a pseudotumor on both imaging and intraoperative findings but histologic findings demonstrating chronic hematoma. Case Description: An 86-year-old female with a metal-on-polyethylene total hip presented with a massive pseudotumor accompanied by extensive bony lysis. Due to pain and chronic anemia, a palliative debulking procedure was undertaken as a palliative measure. At one year follow-up, the patient reported significant pain relief and was able to ambulate safely with gait aids. Her hemoglobin stabilized post-operatively and ongoing transfusions were not required. Final pathology was not supportive of particle disease despite this being the leading diagnosis. Microscopic sections showed tissue mostly composed of fibrin and blood with multiple foci of calcification and reactive papillary endothelial hyperplasia which can be seen in chronic hematomas. Conclusions: This case presents the diagnostic dilemma of a large benign mass consistent with a pseudotumor on both imaging and intraoperative findings but histologic findings consistent with a chronic hematoma. It highlights the importance of close follow-up and early intervention when periprosthetic osteolysis is detected.

Venous malformations, the most common type of vascular malformation, are slow-flow lesions resulting from disorganized angiogenesis. The International Society for the Study of Vascular Anomalies (ISSVA) classification offers a categorization scheme for venous malformations based on their genetic landscapes and association with congenital overgrowth syndromes. Venous malformations present as congenital lesions and can have broad physiologic and psychosocial sequelae depending on their size, location, growth trajectory, and tissue involvement. Diagnostic evaluation is centered around clinical examination, imaging evaluation with ultrasound and time-resolved magnetic resonance imaging, and genetic testing for more complex malformations. Interventional radiology has emerged as first-line management of venous malformations through endovascular treatment with embolization, while surgery and targeted molecular therapies offer additional therapeutic options. In this review, an updated overview of the genetics and clinical presentation of venous malformations in conjunction with key aspects of diagnostic imaging and treatment are discussed.

Expandable endoprostheses provide a limb salvage option for skeletally immature patients with bone sarcoma of the lower extremities. Initial reports of the Repiphysis prosthesis were encouraging; however, medium-term follow-up revealed high complication rates. We report on the long-term follow-up of a cohort of patients treated with the Repiphysis prosthesis. Eleven patients were included in the study. Data collected included sex, age at surgery, duration of follow-up, site of disease, histologic diagnosis, number of lengthening sessions, amount lengthened, postoperative complications, endoprosthetic failure, mode of endoprosthetic failure, duration from index surgery to failure and to revision, type of revision surgery and final limb-length discrepancy. The average duration of follow-up from the time of surgery was 180 months (range, 144-215 months). Fifteen Repiphysis implants were used in 11 patients. All implants failed with an average time from surgery to failure of 36 months (range, 3-72 months). Twenty-four complications were observed: one wound dehiscence, two deep infections, 18 mechanical failures, implant collapse with destruction of proximal tibia epiphysis in two and one periprosthetic proximal femur fracture with dislodgement of the stem. Despite being an option for limb salvage, the Repiphysis prosthesis has a high rate of mechanical failure and need for revision, similar to other expandable implants. The authors, therefore, recommend full disclosure of the potential short- and long-term complications and need for revision, as well as alternative treatment options if their use is considered. Level of evidence: IV (Therapeutic).

PURPOSE/OBJECTIVE:Tranexamic acid (TXA), a synthetic lysine analogue, has been used in orthopedic procedures to limit blood loss and prevent allogeneic blood transfusions. However, data are scarce on its use in hip reconstruction of patients with cerebral palsy (CP). This study examines the effect of TXA on patients with CP undergoing hip reconstruction with at least two osteotomies. METHODS:This is a single-center, retrospective study of patients with CP who underwent hip reconstruction with two or more osteotomies from January 2013 to April 2019. There were 43 patients, with a mean age of 9.9 years. Age, procedure, preoperative and postoperative hemoglobin/hematocrit, estimated blood loss (EBL), transfusions and length of stay were recorded. The patients were split into the following two groups: 24 patients who had received intraoperative TXA and 19 who had not. RESULTS:Age, EBL, mean preoperative and postoperative hemoglobin or hematocrit, preoperative to postoperative hematocrit drop, and length of stay were similar for the two groups (p > 0.05). The risk for intraoperative transfusion (21 vs. 17%), postoperative transfusion (26 vs. 8%), and any transfusion (42 vs. 21%) appeared to be greater in the group that did not receive TXA, but this difference did not achieve statistical significance. CONCLUSION/CONCLUSIONS:This pilot study shows patients with CP undergoing hip reconstruction with two or more osteotomies; the use of TXA, while not statistically significant, shows a trend toward a decreased need for allogeneic blood transfusion.

Osteomyelitis represents inflammation and infection of bone tissue by a pathogen. Acute osteomyelitis is more likely to be unifocal compared to a chronic process which tends to be multifocal and recurrent. Early diagnosis, aggressive appropriate antibiotic therapy and a multidisciplinary approach are essential for a satisfactory prognosis and improved outcome. We report an atypical case of acute multifocal methicillin-sensitive Staphylococcus aureus (MSSA) osteomyelitis.