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Purpose: Some articles have focused on the effectiveness of the Pavlik harness treatment for unstable and dislocated hips, yet data on monitoring its effectiveness with the acetabular index remains elusive. Therefore, this study aimed to assess Pavlik harness effectiveness in infants ≤6 months diagnosed with grade I developmental dysplasia of the hip using acetabular index improvement and identify the possible predictors of successful Pavlik harness treatment. Methods: A retrospective review of infants with grade I acetabular dysplasia treated with a Pavlik harness and monitored with anteroposterior pelvis X-rays at presentation and follow-up. Successful treatment was defined as achieving an acetabular index <30°. Results: A total of 231 infants with acetabular dysplasia were treated with a Pavlik harness. A successful outcome was achieved in 135 infants (58.4%). Younger age, lower initial acetabular index, and patients with unilateral developmental dysplasia of the hip were significant predictors of a successful outcome. An age of 4.5 months or older was found to be the threshold for an unsuccessful result following Pavlik harness treatment, with a sensitivity of 65.2% and specificity of 57.3%. An initial acetabular index of 35.5° was found to be the threshold for an unsuccessful result, with a sensitivity of 83.7% and specificity of 61.5%. Conclusion: Pavlik Harness's success in correcting the acetabular index in acetabular dysplasia patients was related to unilateral cases, a younger age at presentation, and a lower initial acetabular index. The thresholds for unsuccessful treatment were an age of 4.5 months or greater and an acetabular index of 35.5° or higher.
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OBJECTIVE: Recurrence is the main hinderance in treatment of aneurysmal bone cysts (ABCs). Therefore, several treatment modalities and adjuvant therapies have been proposed. In this study, we aim to evaluate the long-term effectiveness of percutaneous curettage and allogenic bone grafting as a new, minimally invasive modality in treating ABCs. METHODS: We performed a retrospective review of the medical records of patients diagnosed with primary ABCs at a university hospital over a 10-year period (2000-2010). We selected all patients who were diagnosed with primary ABC in the extremities and pelvis, treated with the same surgical procedure, and were followed for at least 2 years postoperatively. All patients underwent the same procedure of percutaneous curettage and impaction of allogenic pulverized fine bone fragments (harvested from locally stored femoral heads) mixed with autologous bone marrow aspirate from the iliac bone. We reported patient's characteristics (age and gender), site and size of the lesion, presenting symptoms, Capanna classification, follow-up duration, and post-operative complications. Assessment of cyst healing was based on the appearance on radiographs according to the modified Neer classification. RESULTS: Nineteen patients were included in this study; 10 patients were males and nine were females. The mean age was 9.6 years (range 3-15). The location of the lesions was as follows: femur (eight), tibia (four), pelvis (four), proximal humerus (one), distal radius (one), and calcaneus (one). The most common presenting symptom was pain in the involved area. Pathological fracture was the presenting feature in two patients. The mean follow-up duration was 6.4 years (range 2-18). The earliest radiological sign of incorporation of the allograft was seen at 3 months after surgery. All patients showed bone remodeling and radiographic resolution (classified as either A or B on the modified Neer classification) of their cystic lesions within 6 months. No local recurrence, infection, or pathological fractures occurred during the follow-up period. CONCLUSION: Percutaneous curettage and impaction of allogenic bone graft mixed with autogenic bone marrow aspirate is an efficient, minimally invasive, reproducible, and affordable procedure for the treatment of primary ABCs.
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Cistos Ósseos Aneurismáticos , Transplante Ósseo , Humanos , Pré-Escolar , Criança , Adolescente , Cistos Ósseos Aneurismáticos/diagnóstico por imagem , Cistos Ósseos Aneurismáticos/cirurgiaRESUMO
Purpose: Closed reduction (CR) is a standard treatment for developmental dysplasia of the hip (DDH) after failed conservative treatment. After CR, the affected hip is held in the reduced position by a spica cast that typically extends below the knee (long). Above knee (short) spica cast is an alternative technique utilized by some pediatric orthopedic surgeons. We aimed to compare short versus long spica cast after CR in terms of success rate and complications. Methods: Patients who underwent CR with short or long hip spica cast over a 3-year period (2016-2019) were evaluated for the success (sustainability of the reduction) and complications. The acute and long-term success were recorded retrospectively. Acute success was defined as concentric reduction of the hip confirmed by intraoperative arthrogram and immediate postoperative CT scan. Long-term success was defined as maintained reduction at 12 months' post reduction. Results: Forty-seven patients were included in our study. Long spica casts were used in 24 patients and short ones in the remaining 23. The overall acute and long-term success rates were 83% and 66%, respectively. The acute success rate of long spica was 87.5%, while short spica achieved 78.2%. On the long term, the success rate of short spica was higher than long one (73.9% vs 58.3%). Cox regression analysis showed that the type of cast (short vs long spica) was not correlated with acute success (P = 0.405), long-term success (P = 0.263), residual dysplasia (P = 0.405), or avascular necrosis (P = 0.053). Conclusion: CR in DDH is an important line of management in the younger patient population and can save them an invasive open surgery later in life. A short leg spica could represent an easier and likely as successful alternative to the traditional long spica. More prospective future research is needed to validate our observational findings. Level of Evidence: III.
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Background: Several treatment modalities have been reported to minimize the recurrence after surgical treatment of benign bone cysts. In this study, we evaluated local tumor control, recurrence rate, and bone healing of benign bone cysts after treatment with a simple technique, percutaneous curettage and a local autologous cancellous bone graft. Methods: Retrospective analysis of the records of 16 patients diagnosed with benign bone cysts between 2003 and 2010. We documented the demographic data, radiographic signs of healing (progressive decrease in radiolucency, remineralisation, ossification, consolidation of the cyst, and reconstitution of the bone), healing rate, postoperative complications, and recurrence. Results: Seven of the 16 patients (43.75%) were diagnosed with a simple bone cyst (SBC), while nine (56.25%) had an aneurysmal bone cyst (ABC). On average, radiographic signs of healing were present within 3-6 months, but in two patients these signs presented after 16 months. During the follow-up period, there was no difference in the healing rate between patients with SBC and ABC; no signs of deep or superficial wound infection, no postoperative fracture, and no recurrence in any case over an average of 6.3 years of follow-up. Conclusion: Treatment of benign bone cysts (SBC/ABC) with minimally invasive percutaneous curettage and a local autologous cancellous bone graft is a simple and effective modality with a promising outcome in the local control of recurrence and in enhancing bony consolidation.
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PURPOSE: Proximal femoral osteotomy in cerebral palsy patients is a demanding procedure. The fixation of the osteotomy can fail due to the weak osteoporotic bone. The LCP pediatric hip plate with its good grip makes these procedures safe. The aims of the present study are to evaluate the radiological outcome of proximal femoral osteotomy that was fixed with the pediatric LCP in cerebral palsy patients and to raise safety issues regarding its low rate of complications. PATIENTS AND METHODS: Sixteen patients with cerebral palsy who were operated in our department were included in this retrospective study. Data collected from medical charts and X-ray measurements retrospectively reviewed. RESULTS: In total, 16 patients (21 hips), ie, 9 males and 7 females, were included in this analysis. The mean age at the time of the index surgery was 11.9 years (5.9-18.0). The mean follow-up period was 4.78 years (1.5-8.0). Five patients had bilateral hip involvement and 11 had unilateral involvement. All patients had spastic cerebral palsy. The mean values of varus correction and de-rotation were 25° (0°-45°) and 35° (20°-50°), respectively. Neck shaft angle and the Reimer's migration index were significantly improved postoperatively (p<0.01 for both). Seventeen hips showed complete consolidation within 14 weeks of fixation and four hips needed 16 weeks. These four hips were in three patients who were 16 years of age or older. The correlation between age at surgery and the time needed for consolidation was statistically significant (p=0.005). Avascular necrosis, revision surgeries, failure of fixation, acetabular penetration, screw loosening or fracture of the femur were not seen in this study. CONCLUSION: The LCP pediatric hip plate can be used safely in CP patients. The plate provides a strong stable fixation on osteoporotic bone with a low rate of complications.
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BACKGROUND AND AIMS: The relationship between intra-operative hypotension and post-operative complications has been recently studied in non-cardiac surgery. Little is known about this relationship in traumatic hip surgery. Our study aimed to investigate this relationship. METHODS: A retrospective study was conducted on patients who underwent surgical correction of traumatic hip fracture between 2010 and 2015. We reviewed the perioperative blood pressure readings and the episodes of intra-operative hypotension. Hypotension was defined as ≥30% decrease in the pre-induction systolic blood pressure sustained for ≥10 min. The relationship between intra-operative hypotension and post-operative complications was evaluated. Post-operative complications were defined as new events or diseases that required post-operative treatment for 48 h. Factors studied included type of anaesthesia, blood transfusion rate, pre-operative comorbidities and delay in surgery. We used the Statistical Package for Social Sciences (SPSS, IBM 25) to perform descriptive and non-parametric statistics. RESULTS: A total of 502 patients underwent various types of traumatic hip surgery during the study period. Intra-operative hypotension developed in 91 patients (18.1%) and 42 patients (8.4%) developed post-operative complications. Significantly more patients with hypotension developed post-operative complications compared to patients with stable vitals (18.7% vs. 6.1; P < 0.001). There was no statistically significant difference in the incidence of post-operative complication in patients receiving general or spinal anaesthesia. Pre-operative comorbidities had no significant relationship with post-operative complications. Intra-operative blood transfusion was related to both intra-operative hypotension and post-operative complications. CONCLUSION: There was an association between intra-operative hypotension and post-operative complications in patients undergoing traumatic hip surgery.
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BACKGROUND Kaposiform hemangioendothelioma is a rare locally aggressive vascular endothelial-derived spindle cells neoplasm. Herein, we report a rare case of bifocal tibial kaposiform hemangioendothelioma. CASE REPORT A 9-year-old female presented with a 2-year history of pain and swelling in the left leg. The patient had a high plasma level of the D-dimer and fibrinogen. Radiography revealed a centric lytic lesion on the left proximal tibial metaphysis and an eccentric lateral distal tibial metaphyseal. Histopathologic examination of the sample taken from the distal tibia revealed a dense spindle cell tumor with lobular architecture composed of compact spindle cells compressing small slit-like vascular spaces, forming glomeruloid nests. No necrosis was identified. Based on these findings and the positive immunohistochemical staining for CD31, CD34, and D2-40, the patient was diagnosed with kaposiform hemangioendothelioma. Treatment was started by using vincristine chemotherapy, after which the patient developed temporary peroneal neuropathy, which improved over the next 3 months. CONCLUSIONS Bifocal tibial kaposiform hemangioendothelioma lesions are unique in pediatric patients and can be successfully treated with vincristine chemotherapy. In these cases, the treating physician should be aware of peroneal neuropathy as a potential complication of vincristine administration.
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Antineoplásicos Fitogênicos/uso terapêutico , Neoplasias Ósseas/tratamento farmacológico , Hemangioendotelioma/tratamento farmacológico , Síndrome de Kasabach-Merritt/tratamento farmacológico , Sarcoma de Kaposi/tratamento farmacológico , Tíbia/patologia , Vincristina/uso terapêutico , Neoplasias Ósseas/diagnóstico por imagem , Criança , Feminino , Hemangioendotelioma/diagnóstico por imagem , Humanos , Síndrome de Kasabach-Merritt/diagnóstico por imagem , Imageamento por Ressonância Magnética , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Radiografia , Sarcoma de Kaposi/diagnóstico por imagem , Tíbia/diagnóstico por imagem , Vincristina/efeitos adversosRESUMO
Aim: Variations in the clinical outcomes using mesenchymal stem cells (MSCs) treatments exist, reflecting different origins and niches. To date, there is no consensus on the best source of MSCs most suitable to treat a specific disease. Methods: Total transcriptome analysis of human MSCs was performed. MSCs were isolated from two adult sources bone marrow, adipose tissue and two perinatal sources umbilical cord and placenta. Results: Each MSCs type possessed a unique expression pattern that reflects an advantage in terms of their potential therapeutic use. Advantages in immune modulation, neurogenesis and other aspects were found. Discussion: This study is a milestone for evidence-based choice of the type of MSCs used in the treatment of diseases.
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Perfilação da Expressão Gênica , Regulação da Expressão Gênica , Células-Tronco Mesenquimais/metabolismo , Feminino , Humanos , Masculino , Células-Tronco Mesenquimais/citologia , Especificidade de ÓrgãosRESUMO
Low-density lipoprotein receptor-related protein 4 (LRP4) is a multi-functional protein implicated in bone, kidney and neurological diseases including Cenani-Lenz syndactyly (CLS), sclerosteosis, osteoporosis, congenital myasthenic syndrome and myasthenia gravis. Why different LRP4 mutation alleles cause distinct and even contrasting disease phenotypes remain unclear. Herein, we utilized the zebrafish model to search for pathways affected by a deficiency of LRP4. The lrp4 knockdown in zebrafish embryos exhibits cyst formations at fin structures and the caudal vein plexus, malformed pectoral fins, defective bone formation and compromised kidney morphogenesis; which partially phenocopied the human LRP4 mutations and were reminiscent of phenotypes resulting form a perturbed Notch signaling pathway. We discovered that the Lrp4-deficient zebrafish manifested increased Notch outputs in addition to enhanced Wnt signaling, with the expression of Notch ligand jagged1b being significantly elevated at the fin structures. To examine conservatism of signaling mechanisms, the effect of LRP4 missense mutations and siRNA knockdowns, including a novel missense mutation c.1117C > T (p.R373W) of LRP4, were tested in mammalian kidney and osteoblast cells. The results showed that LRP4 suppressed both Wnt/ß-Catenin and Notch signaling pathways, and these activities were perturbed either by LRP4 missense mutations or by a knockdown of LRP4. Our finding underscore that LRP4 is required for limiting Jagged-Notch signaling throughout the fin/limb and kidney development, whose perturbation representing a novel mechanism for LRP4-related diseases. Moreover, our study reveals an evolutionarily conserved relationship between LRP4 and Jagged-Notch signaling, which may shed light on how the Notch signaling is fine-tuned during fin/limb development.
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Regulação da Expressão Gênica no Desenvolvimento , Proteínas Relacionadas a Receptor de LDL/genética , Receptores Notch/metabolismo , Proteínas Serrate-Jagged/metabolismo , Transdução de Sinais , Proteínas de Peixe-Zebra/genética , Peixe-Zebra/genética , Nadadeiras de Animais/embriologia , Nadadeiras de Animais/metabolismo , Animais , Extremidades/embriologia , Extremidades/fisiologia , Técnicas de Silenciamento de Genes , Células HEK293 , Humanos , Rim/embriologia , Rim/metabolismo , Proteínas Relacionadas a Receptor de LDL/metabolismo , Mutação , Mutação de Sentido Incorreto , Organogênese , Via de Sinalização Wnt , Peixe-Zebra/embriologia , Peixe-Zebra/metabolismo , Proteínas de Peixe-Zebra/metabolismoRESUMO
ABSTRACTS: A 28-year-old woman presented with a mass in her right leg, which, on plain radiograph, appeared as a lytic lesion in the mid-shaft of the tibia with cortical thinning but without periosteal reaction. Tc-MDP 3-phase bone scan demonstrated intense uptake in the periphery of the mass with a photopenic center, the so-called doughnut sign. Histopathology revealed a capillary hemangioma. This case demonstrates that the doughnut sign on bone scan can be caused by capillary hemangioma in addition to other reported bone pathologies, including aneurysmal bone cyst, giant cell tumor, and telangiectatic osteosarcoma.
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Neoplasias Ósseas/diagnóstico por imagem , Hemangioma Capilar/diagnóstico por imagem , Adulto , Feminino , Humanos , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Compostos Radiofarmacêuticos , Medronato de Tecnécio Tc 99mRESUMO
Charcot-Marie-Tooth disease comprises a group of disorders characterized by progressive muscle weakness and wasting. Reviewing the anaesthetic literature produced conflicting reports about the best anaesthetic options for patients with CMTD; as they are at increased risk of prolonged response to muscle relaxants, malignant hyperthermia and risks of regional anaesthesia. We present a case of the successful use of total intravenous anaesthesia with dexmedetomidine and propofol combined with caudal block using bupivacaine mixed with dexmedetomidine without any complications, for a 17 year old male patient with Charcot Marie-Tooth disease who underwent a lower limb orthopedic surgery.
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Anestesia/métodos , Doença de Charcot-Marie-Tooth/fisiopatologia , Adolescente , Anestesia Intravenosa , Humanos , MasculinoRESUMO
OBJECTIVES: To describe the pattern of developmental dysplasia of the hip (DDH) in late presenting Jordanian male patients and identify the risk factors and associated findings. METHODS: This is a retrospective study of 1145 male patients who attended the Pediatric Orthopedic Clinic for a DDH check up. This study was carried out in the Orthopedic Section, Special Surgery Department, Faculty of Medicine, The University of Jordan, Amman, Jordan between March 2011 and October 2014. Data was collected from medical records, and x-ray measurements were evaluated. RESULTS: Of the 1145 male patients, 43 (3.75%) with 70 involved hips were diagnosed with late- presenting DDH. Being a first-born baby resulted in 41.9% increased risk for DDH. Cesarian delivery was significantly associated with an increased risk of hip dislocation (p=0.004) while normal delivery was significantly associated with acetabular dysplasia (p=0.004). No predictable risk factors were found in 44.2% patients with DDH. Bilateral cases were more common than unilateral cases: (26 [60.5%] versus 17 [39.5%]). Limited abduction was a constant finding in all dislocated hips (p less than 0.001). Associated conditions, such as club foot and congenital muscular torticollis were not observed. CONCLUSION: Cesarian section is a significant risk for dislocated hips while normal delivery is significantly associated with acetabular dysplasia. Bilateral DDH is more common than the unilateral. Club foot and torticollis were not observed in this series.