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Bilateral congenital pseudarthrosis of the olecranon.

A case of bilateral congenital pseudarthrosis of the olecranon is reported in a 15-month-old boy. This abnormality was noted at birth because of a fixed flexion deformity of both elbows. The left side was operated on at the age of 16 months and the right side at 21 months. Surgical exploration showed a mobile pseudarthrosis with a 2-cm gap between the tip of the olecranon and the nucleus, each one covered with cartilage. This cartilage was excised, the triceps tendon was lengthened to allow the lowering of the nucleus, and both fragments were fixed with nylon threads. The elbow was kept extended for 30 days in a plaster cast. Fusion was obtained shortly. At the 2-year follow-up, the function was nearly normal as was the radiological appearance. This abnormality is very rarely reported in the literature, and we emphasize the necessity of an early operation.

Diagnosis, Differential↗

Congenital pseudarthrosis of the tibia: history, etiology, classification, and epidemiologic data.

Congenital pseudarthrosis of the tibia is a rare disease with a variable history. The pseudarthrosis is usually not present at birth (and therefore is not truly congenital) but occurs during the first decade of life. Paget in 1891 was the first to describe a case. The etiology is unknown. Neurofibromatosis plays a role in approximately 50% of patients. In the others, hereditary and mechanical factors are debated. Various (morphologic) classification systems have been proposed (Andersen, Boyd, Crawford). Because the appearance changes during the course of the disease, all classification systems have limited value: the determining factor is the stage of the disease at which it was classified. Because of the rarity of the disease and the variability of its history, the European Pediatric Orthopaedic Society decided to carry out a multicenter study on this disease. This paper presents the epidemiologic data on the patients involved. Data have been gathered on 340 patients from 13 countries. Two hundred patients were male (58.8%), 140 were female (41.2%). The right side was affected in 165 patients (48.5%) and the left side in 172 (50.6%); 3 patients had bilateral disease. Symptoms of neurofibromatosis were present in 54.7%. Histologic examinations in 192 patients showed a nonspecific appearance in 45.3%; in 15.6% the ultrastructure resembled fibrous dysplasia, and in 39% there was histologic evidence of neurofibromatosis. Most of the lesions were initially localized in the middle or distal third of the tibia. In 29% the localization changed during the course of the disease.

Child↗

Pseudarthrosis following treatment of idiopathic scoliosis by Harrington instrumentation and fusion without added bone.

A retrospective review was conducted to assess the incidence of pseudarthrosis among 381 patients who had surgical treatment of idiopathic scoliosis by Harrington distraction rod and posterior fusion without added bone. In 260 patients where the lowest level of fusion was at or above the third lumbar vertebra, there were no cases of pseudarthrosis. In the remaining 121 patients with fusion extending below the third lumbar level, there were eight cases, an incidence of 6.6%, for an overall incidence of 2.5%.

Adolescent↗

Treatment of congenital pseudarthrosis with the Ilizarov technique.

Four patients with congenital pseudarthrosis in neurofibromatosis were treated with percutaneous transosseous fixation according to the Ilizarov method. Three tibias were first treated unsuccessfully with a Sofield procedure. After application of the Ilizarov fixator, consolidation was obtained in two cases after 6 and 19 months, respectively. A fourth case consisted of a congenital pseudarthrosis of the ulna, primarily treated with the Ilizarov system, which showed advanced consolidation after 4 months.

Child↗

Congenital pseudarthrosis of the clavicle: a review of the literature and surgical results of six cases.

This article is a retrospective review of six symptomatic patients who, when treated surgically, all healed with excision of pseudarthrosis, bone grafting, and plating. We discuss congenital pseudarthrosis of the clavicle with emphasis on etiologies, clinical presentation, differential diagnosis, and method of treatment. Our patients' chief complaints were cosmesis, pain, or shoulder deformity.

Adolescent↗

Operative treatment of congenital pseudarthrosis of the clavicle.

Eight children were treated operatively with resection of fibrous pseudarthrosis and sclerotic bone ends, careful dissection and preservation of the periosteal sleeve to maintain continuity, and approximation of bone ends. None had additional bone grafts or internal fixation. All had bridging ossification 6-8 weeks postoperatively, and all were solidly healed by 14 weeks after surgery. Follow-up has ranged from 2 to 14 years, with no evidence of recurrence. Remodeling of the prominence occurred slowly in 2-5 years, with the distal clavicle variably underdeveloped in all patients. Early resection of fibrous pseudarthrosis probably does not require the extensive grafting and internal fixation that has been recommended for older children.

Bone Development↗

Congenital pseudarthrosis of the tibia: treatment by transfer of the ipsilateral fibula with vascular pedicle.

We performed five transfers of the ipsilateral fibula in the treatment of congenital pseudarthrosis of the tibia. In all cases, conventional treatment by intramedullary-rod and autogenous-iliac bone grafting had failed at least once. We hoped to avoid the use of contralateral-fibular grafting or other methods that have not yet been proven effective. The ipsilateral fibula was transferred, and its vascular pedicle left intact and secured to both fragments of the tibial pseudarthrosis by cerclage wires. All five transfers have united and hypertrophied with growth. We believe this salvage method is superior to others not only because of the successful results, but also because the intact limb is left undisturbed.

Child↗

Pseudarthrosis of the first rib in the overhead athlete.

Fractures of the first rib are uncommon in athletes and present a different clinical entity from traumatic first rib fracture associated with high energy thoracic trauma. These fractures are stress induced and precipitated by chronic muscular forces acting on the first rib. Typically they heal with conservative treatment. This report describes a fracture of the first rib in a tennis player that developed into a symptomatic pseudarthrosis as a result of persistent overhead activities. Symptoms mimicked ipsilateral shoulder injury. Pseudarthrosis of the first rib should be included in the differential diagnosis of chronic persistent shoulder pain in the overhead athlete.

Adult↗

A case of pseudarthrosis of the capitate.

Pseudarthrosis of the capitate bone is extremely rare. In this case, the injury and pseudarthrosis was so old, the bone with a nutrient vessel was grafted, and bone union and excellent results are obtained.

Adult↗

Pseudarthrosis following lumbar fusion: detection by direct coronal CT scanning.

Twenty patients with fusions of the lumbar spine (seven with pseudarthrosis, 11 with solid bony fusions, and two with fusions that appeared solid but assessment was complicated by the presence of surgical hardware) underwent computed tomographic (CT) scanning in the supine position in the axial plane and, employing a specially designed seat, in the coronal plane. Three-millimeter contiguous sections were acquired. The direct coronal images were compared with those reformated from the axial images. The higher-resolution direct coronal images facilitated the diagnosis of pseudarthrosis and increased confidence in interpretation of normal studies. Direct coronal imaging is easy to perform and in many cases requires fewer scans and less radiation than reformations. Because it provides more useful diagnostic information, direct coronal imaging should replace current methods for evaluating fusions of the lumbar spine.

Adult↗

A case of congenital pseudarthrosis of the tibia treated with pulsing electromagnetic fields. 17-year follow-up.

Congenital pseudarthrosis of the tibia presents surgeons with one of the most challenging of all orthopedic problems. Various surgical treatments have succeeded only rarely. We report long-term follow-up of a patient with congenital pseudarthrosis of the tibia treated with pulsed electromagnetic fields (PEMF) and bone grafting. In this severe case, Bassett type III and Boyd type II, encouraging results were achieved with Boyd's dual onlay grafts and PEMF. Seven years after surgery, skeletal maturity was complete and an unacceptable degree of leg shortening had been avoided.

Adolescent↗

Congenital pseudarthrosis of the tibia.

Forty cases of congenital pseudarthrosis of the tibia were reviewed. The results were assessed so as to emphasise function rather than simply the presence or absence of union. At the time of review, 14 patients had undergone amputation; eight others had non-union or tenuous union. No surgical procedure except the Farmer operation (a composite skin and bone pedicle graft from the other leg) showed any clear superiority. Among the factors associated with a poor result were considerable shortening, older children, and rapid resorption of the bone graft. It is felt that congenital pseudarthrosis of the tibia is a biological problem and not merely a mechanical one; consequently biological approaches to its treatment are needed.

Adolescent↗

Congenital pseudarthrosis of the ulna due to neurofibromatosis.

Two cases of congenital pseudarthrosis of the ulna due to neurofibromatosis are reported. Similar radiographic changes in the ulna were found, with distortion of the capitulum and part of the trochlea and bowing of the radius. In one patient the head of the radius was dislocated and in the other it dislocated readily during pronation. A possible mechanism to explain the findings was suggested. On the basis of these and a review of the literature it is recommended that the main aim of treatment should be maintenance of the normal relative lengths of the radius and ulna by early excision of the pseudarthrosis to remove the restraining effect of the abnormal ulna. This will allow normal development of the lower end of the humerus and radius and prevent dislocation of the radial head.

Adult↗

Free vascularised fibular graft in the treatment of congenital pseudarthrosis of the tibia.

Five cases of congenital pseudarthrosis of the tibia successfully treated by a free vascularised fibular graft are described. Follow-up ranged from 5 to 34 months with a mean of 17.5 months. The technique, which includes radical excision of abnormal bone and soft tissue around the pseudarthrosis, also permits primary bone lengthening, and correction of deformity. The early results indicate that satisfactory bony union is achieved in a relatively short period of time.

Child↗

Bilateral congenital pseudarthrosis of the olecranon.

A case of bilateral congenital pseudarthrosis of the olecranon is described. The clinical features, radiological appearance and prognosis are distinct from the type of congenital pseudarthrosis of the forearm reported previously. The lesion appeared to be a localised failure of ossification within the proximal ulnar metaphysis. Excision, stabilisation and bone grafting led to rapid fusion.

Child, Preschool↗

Extending intramedullary rods in congenital pseudarthrosis of the tibia.

Five patients with Boyd type II congenital pseudarthrosis of the tibia underwent excision of the pseudarthrosis and double onlay bone grafting. Stability was maintained by extending intramedullary rods. Clinical union was achieved in all cases at a mean of 8.6 months (range six to 11). The rods extended by 15.7% (range 2% to 31.4%) as growth occurred. One rod was removed because of infection and a vascularised free fibular graft was subsequently performed. The extending rods provided stability while union occurred and did not require revision as the legs grew. The rods can be removed easily and have not jeopardized further surgical options.

Bone Transplantation↗

Free vascularised fibular grafts for congenital pseudarthrosis of the tibia.

We describe 11 patients with congenital pseudarthrosis of the tibia treated by a free vascularised fibular graft (FVFG) and followed up from 10 to 64 months (mean 38). Bony union was achieved in nine of the 11 cases: two failures required amputation. The mean time for union in the successful cases was five months. Nine of the 11 patients had had an average of four surgical procedures before the FVFG, so the graft was a salvage procedure for which the only alternative was amputation. FVFG is recommended as a primary procedure for the treatment of congenital pseudarthrosis of the tibia if there is a large tibial defect (over 3 cm) or shortening of more than 5 cm. The primary use of this operation is not advised for cases in which standard orthopaedic procedures are expected to succeed. For a small defect with a favourable prognosis (Boyd and Sage 1958), we recommend conventional bone grafting, intramedullary nailing and electrical stimulation.

Adolescent↗