Jaffe-Campanacci syndrome; a case series and review of the literature.

BACKGROUND: Jaffe-Campanacci syndrome is a rare syndrome, characterized by multiple non-ossifying fibromas (NOF) and cafe-au-lait patches. The name was coined in 1982 by Mirra after Jaffe who first described the case in 1958. Although it's suggested there is a relation with Neurofibromatosis type 1, there is still no consensus on whether Jaffe-Campanacci syndrome is a subtype or variant of neurofibromatosis-1(NF-1). CASE PRESENTATION: In this article, we present a case series of 2 patients. The first case is a 13-year-old male with Jaffe-Campanacci syndrome who presented with a distal femur fracture. His father had positive features of both Jaffe-Campanacci syndrome and NF-1, while his sister only had features of NF-1, so we presented both. CONCLUSION: Jaffe-Campanacci has a clear relationship with type 1 neurofibromatosis, which still has to be genetically established. Due to the presence of several large non-ossifying fibromas of the long bones, it is linked to a significant risk of pathological fractures. We concur with previous authors, that an osseous screening program should be performed for all patients with newly diagnosed type 1 neurofibromatosis, to identify non-ossifying fibromas and assess the potential for pathological fracture. Moreover, siblings of patients with NF-1 should be screened for multiple NOFs that may carry a high risk of pathological fractures.

Comparative Study Between Single-limb Versus One-and-a-Half Hip Spica Cast in Fracture Femur in Young Children.

INTRODUCTION: Femoral fractures account for ∼1% to 2% of all pediatric bone injuries and are a common occurrence in children. The conservative approach, employing either a single or one-and-a-half spica casts, has been traditionally favored, yielding satisfactory outcomes in select cases. This study aims to compare both procedures regarding functional and radiologic outcomes, complications, and parents' satisfaction. METHODS: In this randomized controlled trial, we enrolled 84 pediatric patients, aged between 2 and 6 years, presenting with femoral fractures. Participants were randomly allocated into 2 groups; one receiving single-limb spica cast fixation (n=42) and the other receiving one-and-a-half spica cast fixation (n=42). We assessed postprocedural functional and radiologic outcomes. Other evaluations focused on parental ease in maintaining hygiene for the casted child, the child's mobility capabilities including standing and crawling, and the incidence of skin complications. RESULTS: No significant variance was observed between the 2 groups concerning the time to bone union, and the overall complication rates. Parental satisfaction was notably higher in the single-limb spica group, particularly regarding the ease of maintaining hygiene for the casted child and the child's mobility while encased in the cast (P<0.001). Furthermore, a significant correlation was identified between the one-and-a-half spica application and the increased occurrence of skin pressure ulcers (P<0.001). CONCLUSION: Both single-limb and one-and-a-half spica cast applications demonstrated comparable results in functional and radiologic outcomes, as well as in complication rates. However, parents favored the single-limb method due to its facilitation of a more manageable lifestyle for both the child and their parents. These considerations suggest that the single-limb hip spica cast fixation may be preferable in managing pediatric femoral fractures. LEVEL OF EVIDENCE: Level II.

Are positional screws superior to lag screws in fixation of medial malleolar fractures? A randomized controlled trial.

PURPOSE: There is no consensus about the best fixation method for medial malleolar fractures. This study was conducted to compare the clinical and radiological results of positional (fully threaded) screws and lag (partially threaded) screws when used to fix medial malleolar fractures. METHODS: This prospective, randomized clinical trial included 68 patients with closed isolated medial malleolar fractures. Each study arm included 34 patients who were randomly treated with either fully threaded screws (FT group) or partially threaded screws (PT group). Patients were evaluated radiologically for union and clinically using the American Orthopaedic Foot and Ankle Society (AOFAS) ankle hindfoot score. RESULTS: After a 12-month follow-up, the mean AOFAS ankle hindfoot score was 92.1 ± 3.5 in the FT group and 91.8 ± 5.4 in the PT group. Union was achieved in the FT group after a mean of 6.2 ± 1.3 weeks and after a mean of 6.5 ± 1.5 weeks in the PT group. CONCLUSION: Both fully threaded and partially threaded screws yielded good comparable clinical and radiological outcomes when used to fix isolated medial malleolar fractures.

Femoral Lengthening in Children with Congenital Femoral Deficiency: A Systematic Review.

BACKGROUND: Congenital femoral deficiency (CFD) presents a significant challenge in pediatric orthopaedics, characterized by a spectrum of congenital anomalies ranging from mild femoral shortening to complete absence of the proximal femur and hip joint. This review aims at reviewing the latest concepts of femoral limb lengthening modalities in treating CFD, to explore the efficacy, complications, and long-term outcomes of various surgical techniques. METHODS: A comprehensive search of the literature was performed for clinical studies involving lengthening in patients with proximal focal femoral deficiency (PFFD) in several databases. RESULTS: We analyze the evolution of limb lengthening procedures, from the Wagner and Ilizarov methods to the latest advancements in distraction osteogenesis, and assess their role in addressing the functional needs of patients. We also analyze the possible risk factors for the occurrence of complications with each method and alternatives to avoid them. CONCLUSION: The review highlights the importance of individualized treatment plans, considering factors such as the degree of femoral deficiency and the potential for achieving a functional limb length; however, it requires a multidisciplinary approach and careful preoperative planning to optimize patient outcomes. The review underlines the need for ongoing research to refine surgical techniques and to compare them and improve the quality of life for individuals with PFFD. LEVEL OF EVIDENCE: Level III. See Instructions for Authors for a complete description of levels of evidence.

Blalock-Taussig Shunt versus Ductal Stent in the Palliation of Duct Dependent Pulmonary Circulation; A Systematic Review and Metanalysis.

In infants with ductal dependent pulmonary blood flow, Blalock-Taussig (BT) shunt and Patent Ductus Arteriosus (PDA) stent, are two palliative procedures aimed to restore circulation. A systematic review and metanalysis was performed on studies comparing PDA stents and BT shunts, in accordance with PRISMA guidelines. Meta-analysis revealed the following; (1) a reduced risk of mortality [RR = 0.585 [0.399-0.859], (P = 0.006)], (2) a reduced risk of complications [RR = 0.523 [0.318-0.860], (P = 0.011), and (3) a reduced risk of ECMO use [R = 0.267 [0.101-0.706] (P = 0.008)], all in the stent group. Additionally, stent group showed higher post procedure oxygen saturation [SMD = 1.307 [95% CI 1.065-1.550], (P < 0.001)], and Nakata index [SMD = 0.679 95% CI [0.513 to 0.845], (P < 0.001)]. PDA stenting presents a viable alternative to BT shunt procedure with better post procedure stability.