Congenital pseudarthrosis

Congenital pseudarthrosis of the clavicle (CPC) is a rare disorder with an unknown etiology, which is caused by a failure of the clavicle ossification nuclei union process. This is the first CPC twin instance documented in Saudi Arabia, and the fourth overall. In the 33rd week, a set of twins was born prematurely with respiratory distress syndrome. They were both admitted to the neonatal intensive care unit. X-rays of the chest were taken, and the clavicular deformity was discovered. Because the twins were born via a cesarean section, a traumatic clavicular fracture was ruled out...

BACKGROUND: Congenital pseudarthrosis of the tibia (CPT) is a rare disease. CPT is often unilateral and occurs between the middle and distal third of the tibia. Concurrent involvement of the fibula is present in more than half of cases. histologic studies indicate the presence of fibrous hamartoma tissue and a sick periosteum, which leads to recalcitrant bone fracture and, eventually, pseudoarthrosis. Although there are various surgical techniques, we intend to compare the 2 methods of external fixation versus internal plating...

Congenital pseudarthrosis of the tibia (CPT) is a rare disorder affecting the skeletal system in pediatric population with an estimated incidence of 1:140,000 to 1:250,000 newborns. It is characterized by deformity of the tibia, including anterolateral bowing of the bone diaphysis and/or narrowing of the medullary canal, leading to instability or fracture. CPT can be either idiopathic or associated with underlying conditions such as type 1 neurofibromatosis (NF1), fibrous dysplasia, or Campanacci's osteofibrous dysplasia...

CASE: We describe the surgical management of a girl with dwarfism and congenital spondyloepiphyseal dysplasia, who presented in adolescence with coxa vara and bilateral pseudarthrosis between the femoral neck and the diaphysis, with asymmetric distal migration at both sites and leg length discrepancy. The patient at 16 years underwent valgus osteotomy in situ and femoral neck fixation in the left hip. The right hip was operated 19 months later. CONCLUSION: Hip dysplasia is common in spondyloepiphyseal dysplasia, but a debilitating nontraumatic bilateral pseudarthrosis at the base of the femoral neck has not been previously reported...

BACKGROUND: Congenital pseudarthrosis of the tibia is a limb deformity, which can be distressing for the affected patients and the pediatric orthopedic surgeons involved. We hypothesized that the modified McFarland procedure would avoid fractures and even have a corrective effect on the affected tibia in congenital pseudarthrosis of the tibia patients. Toward this end, we evaluated the midterm results of treating congenital pseudarthrosis of the tibia patients of Crawford Type I and II with allograft bypass combined with long-term bracing...

The vascularized fibula transfer is a well-established technique for extremity reconstruction, but operative planning and patient selection remains crucial. Although recently developed techniques for bone reconstruction, such as bone segment transfer, are becoming increasingly popular, bone defects may still require vascularized bone grafts under certain circumstances. In this study, 41 cases, 28 (68%) men and 13 (32%) women (median age: 40 years), were retrospectively analyzed. Therapy-specific data (flap vascularity [free vs...

A 15-year-old girl with humeroradial synostosis since birth underwent a resection arthroplasty. A trapezoidal resection osteotomy of approximately 2 cm was performed at the anterior part of the bone flexure. This resulted at 18 months in an elbow arc of motion of 60°-110° and forearm pronation/supination of 40° and 60° without postoperative complications and improved disabilities of the arm, shoulder and hand and Hand 20 scores. Radiographic analysis revealed a humeroradial joint with a maintained pseudarthrosis and hinged motion at the humeroulnar joint...

Congenital pseudarthrosis of the tibia (CPT) is a rare, dysplastic condition that is characterized by a "false joint" in the tibia, leading to potential disability. We present a rare case report of a 12-year-old male from India with a history of neurofibromatosis type 1 (NF1) and anterolateral bowing of the tibia since birth. He sustained a tibial fracture during play. X-ray evaluation confirmed the fracture, and a clinical diagnosis of CPT was established. The treatment involved corticotomy for deformity correction and stabilization using Ilizarov's ring fixation...

OBJECTIVE: To explore the types of NF1 gene variants and clinical characteristics among patients with Neurofibromatosis type I (NF1). METHODS: Clinical data of 12 patients diagnosed at Ningbo Women and Children's Hospital between December 2019 and May 2022 were retrospectively analyzed. The probands and their family members were subjected to high-throughput sequencing, and candidate variants were verified by Sanger sequencing and chromosome microarray analysis. RESULTS: The 12 patients had ranged from 4 months to 27 years old, with a male-to-female ratio of 2 : 1...

OBJECTIVE: Correction of deformities around the knee joint in the frontal and sagittal axis, torsion, length and translation. INDICATIONS: Complex deformities of the proximal tibia, and to a lesser extent of the distal femur, which cannot be treated with acute correction using plate or nail osteosynthesis. CONTRAINDICATIONS: Nicotine abuse, soft tissue problems, lack of patient compliance. SURGICAL TECHNIQUE: First, mounting of the proximal ring of the ring fixator strictly parallel to the joint line in 2 planes, fixation with 3 or 4 pins or wires...

BACKGROUND: Vertebral column resection (VCR) is a powerful corrective technique for the management severe, rigid spinal deformities but does carry a relatively high complications rate. One of the feared complications is pseudarthrosis which places the patient at risk for implant failure. We present a single-center experience with post-operative computed tomography (CT) imaging at the osteotomy site to screen for impending pseudarthrosis. METHODS: A retrospective review of a single surgeon series of posterior-only VCR performed for severe pediatric and adolescent spinal deformities was performed...

OBJECTIVE: The purpose of this study was to explore the effect of fast-track surgery combined with a clinical nursing pathway on the recovery and complications of congenital pseudarthrosis of tibia. METHODS: 82 children with congenital pseudarthrosis of tibia admitted from January 2019 to December 2020 were selected as the study subjects. The control group received routine clinical nursing pathway while the intervention group received a fast-track surgery combined with a clinical nursing pathway...

Congenital pseudarthrosis of the tibia (CPT) is a refractory condition characterized by the decreased osteogenic ability in tibial pseudarthrosis repair. Periosteal mesenchymal stem cells (PMSCs) are multipotent cells involved in bone formation regulation. However, the mechanisms underlying its role in CPT remain unclear. In this study, we observed downregulation of circ_0000888 and pleiotrophin (PTN), as well as upregulation of miR-338-3p in CPT derived PMSCs (CPT-dPMSCs). Our results demonstrated that circ_0000888 and PTN likely enhanced the viability, proliferation, and osteogenic ability of PMSCs, while miR-338-3p had the opposite effect...

Objective: To compare the initial clinical efficacy of extensible intramedullary rod fixation and Williams intramedullary rod fixation combined with Ilizarov external fixation and wrapped autologous iliac bone grafting in the treatment of congenital tibial pseudarthrosis in children. Methods: A retrospective analysis was conducted on the clinical data of 60 children with Crawford Ⅳtype congenital pseudarthrosis of the tibia treated with combined surgery at the Orthopedics Department of Hunan Children's Hospital from January 2013 to December 2020...

Congenital pseudarthrosis of forearm fractures is rare and is strongly associated with neurofibromatosis type 1 (NF1). Our case report illustrates the progression of a non-union of the ulna after minor trauma in a twelve-year-old boy, newly diagnosed with NF1, and presents the technique of microsurgical bone reconstruction, including the growth plate. More than seven years after the first operation, follow-up presents a favorable outcome with a pain-free patient and unrestricted function of the forearm after a secondary correction of the remaining radial bowing...

The induced membrane technique is an innovative approach for repairing critical bone defects and has been applied recently in patients with congenital pseudarthrosis of the tibia (CPT). CPT is frequently associated with neurofibromatosis type 1 (NF1). Here, we briefly describe the clinical results of the induced membrane technique in NF1-deficient patients with CPT and in an animal model of CPT. Furthermore, we discuss the hypotheses used to explain inconsistent outcomes for the induced membrane technique in CPT-especially when associated with NF1...

Objective: To explore the long-term effect of combined surgery for the treatment of congenital tibial pseudarthrosis in children. Methods: The clinical data of 44 children with congenital tibial pseudarthrosis who underwent combined surgery (tibial pseudarthrosis tissue resection, intramedullary rod fixation, wrapped autologous iliac bone graft, Ilizarov external fixator fixation) from August 2007 to October 2011 at the Department of Pediatric Orthopedics, Hunan Children's Hospital were collected. There were 33 males and 11 females...

INTRODUCTION: Congenital pseudarthrosis of the fibula is not an uncommon condition to accompany congenital pseudarthrosis of the tibia. Persistence of the fibular pseudarthrosis has been linked to inferior outcomes including tibial union and alignment, refractures, and ankle alignment. In this report, we present the results of a pedicled periosteal propeller flap technique for the treatment of fibular pseudarthrosis. METHODS: Ten children with an average age at surgery of 5...

BACKGROUND: It is a rare condition defined by congenital pseudarthrosis of the fibula (CPF), which may be associated with neurofibromatosis (NF) and congenital pseudarthrosis of the tibia. A condition such as this may be symptomatic or asymptomatic, but it requires treatment because of the progression of signs such as ankle valgus and pseudarthrosis of the tibia. There are a number of surgical techniques that can be used to treat this condition, such as tibiofibular fusion, internal fixation, the Ilizarov method, and periosteal flap...

CASE: An 18-year-old woman with a history of congenital pseudarthrosis of the clavicle (CPC) presented with episodes of right upper extremity ischemia. Vascular studies demonstrated an extensive thrombus with complete occlusion of the brachial artery. She underwent urgent thrombectomy. Subsequently, she underwent first rib resection and scalenectomy as well as pseudarthrosis takedown and fixation. Postoperatively, she returned to Division I collegiate soccer with complete symptomatic resolution...