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PubMed2003

Residual challenges after healing of congenital pseudarthrosis in the tibia.

The purpose of the current study was to evaluate the clinical results of the Ilizarov bone transport method in the treatment of congenital pseudarthrosis in the tibia. In seven patients operated on between 2.6 and 7.8 years of age, primary healing of the pseudarthrosis was achieved in all patients (after additional bone grafting in two patients). Within a followup of 6 to 8 years, major complications occurred in all patients. Five refractures occurred, and in one patient the refracture did not heal. At the last followup, axial deformities and an abnormal malalignment test with lateral mechanical axis deviation of 10 mm or greater was found in all patients. Three patients had leg length inequality of 20 mm or more. The Ilizarov bone transport method is useful in achieving primary healing in congenital pseudarthrosis of the tibia, but residual challenges with secondary reconstructive surgery caused by refracture and postoperative deformities must be expected.

Bone Transplantation↗
PubMed2006

Treatment of a congenital pseudarthrosis of the tibia by osteogenic protein-1 (bone morphogenetic protein-7): a case report.

Congenital pseudarthrosis of the tibia remains one of the most difficult orthopaedic problems to treat. Before the use of a recombinant bone morphogenetic protein (bone morphogenetic protein-7; osteogenic protein-1) the patient with congenital pseudarthrosis of the tibia in this report had had 12 unsuccessful surgeries. Five months after radical resection of sclerotic tibial segments, Ilizarov fixation and administration of osteogenic protein-1 osteogenic device, the congenital pseudarthrosis of the tibia healed; at 45 months the tibia increased in size and the patient was fully weight bearing.

Bone Morphogenetic Protein 7↗
PubMedDate not supplied

Use of free vascularized fibular graft for congenital ulnar pseudarthrosis: surgical decision making in the growing child.

Congenital pseudarthrosis of the ulna may cause growth disturbance and progressive forearm deformity, leading to functional compromise of the upper extremity. Treatment is challenging, and surgical decision making must take into account three goals of treatment: bony healing, distal radioulnar joint (DRUJ) stability, and continued skeletal growth. Four cases of congenital ulnar pseudarthrosis treated with free vascularized fibular graft are presented here. In two cases, the vascularized fibular graft included the proximal fibular epiphysis to reconstruct the DRUJ and ulnocarpal joints. Average age of the four patients at time of vascularized fibular grafting was 10 years (range 3-16 years). Patients had undergone up to three previous failed operations. A step-cut osteotomy technique with rigid internal fixation was used in all patients. Donor-site distal tibiofibular arthrodesis was performed in skeletally immature patients when appropriate. At average follow-up of 60 months (range 33-83 months), all patients achieved bony union with full wrist range of motion compared with the contralateral extremity. The DRUJ was stable in all patients. Two skeletally immature patients with concomitant epiphyseal transfer showed continued skeletal growth. Two patients nearing skeletal maturity achieved revascularization of the distal ulna. Free vascularized fibular grafting is a successful option in the treatment of congenital ulnar pseudarthrosis. Reconstruction of the distal radioulnar and ulnocarpal joints using concomitant proximal fibular epiphyseal transfer should be considered in the skeletally immature patient with distal ulnar involvement.

Adolescent↗
PubMedDate not supplied

Isolated congenital pseudarthrosis of the fibula: clinical course and optimal treatment.

The purposes of this study were to determine the clinical course of isolated congenital pseudarthrosis of the fibula (ICPF) and to establish optimal treatment guidelines for this disease. Thirteen fibulae in 12 patients with ICPF were followed for an average of 6.2 years. Twelve of the 13 fibulae were regarded to originally have had a pre-pseudarthrotic condition. Five fibulae remained intact during follow-up, but 8 developed pseudarthrosis. Two cases with established pseudarthrosis were observed for ankle alignment change without intervention, and in 4 cases successful fibular osteosynthesis maintained the ankle alignment. The remaining 2 adolescent cases presenting with ankle valgus were treated using the Ilizarov technique and distal tibiofibular synostosis. ICPF follows a unique clinical course, which should be taken into consideration along with fibular status, ankle alignment, and patient age when planning treatment.

Adolescent↗
PubMed2000

Treatment approaches for congenital pseudarthrosis of tibia: results of the EPOS multicenter study. European Paediatric Orthopaedic Society (EPOS).

This study was designed to analyze the different therapeutic methods used by European Paediatric Orthopaedic Society members from 13 countries for congenital pseudarthrosis of tibia. The treatment data of 340 patients who underwent 1287 procedures for this condition were analyzed. The essential findings were that the method of choice needed to approach the biological problem with the aims of: (1) resecting the pseudarthrosis to provide stability, the basic requirement for bony consolidation; (2) correcting length discrepancy and axial deformity; (3) achieving fusion; and (4) solving the additional problems around the main deformity such as alignment, leg length discrepancy and ankle valgus. The Ilizarov technique emerged as being the optimal method, having the highest rate of fusion (75.5%) of pseudarthrosis and rate of success in correction of the additional deformities. There was also consensus that surgery should be avoided before the third year of life.

Adolescent↗
PubMedDate not supplied

Congenital pseudarthrosis of the ulna: a report of two cases and a review of the literature.

Two cases of congenital pseudarthrosis of the ulna in patients with neurofibromatosis were followed up for 15 and 8 years, respectively. In both cases conventional bone grafting failed. The solution in one case was production of a one-bone forearm. In the other, osteotomy of the radius, resection of the ulnar pseudarthrosis, and stabilization of the distal radioulnar joint achieved a good result. The literature suggests that free vascularized bone grafting, electrical stimulation, formation of a one-bone forearm, or osteotomy of the uninvolved bone with or without resection of the pseudarthrosis should be considered as treatment alternatives.

Adolescent↗
PubMed1997

Pseudarthrosis: US evaluation after posterolateral spinal fusion: work in progress.

PURPOSE: To determine the value of ultrasound (US) in evaluation of pseudarthrosis after posterolateral spinal fusion. MATERIALS AND METHODS: In 10 patients who had undergone bilateral posterolateral thoracic or lumbar fusion with autologous bone graft and instrumentation more than 9 months previously, US was performed within 1 week before second-look surgery. A total of 20 sites each side of midline were evaluated for the presence of bone graft, solid fusion, clefts, fluid collections, and hardware visibility. US findings were compared with those at surgery. In three patients, standard radiographs were reviewed before US; blinded US evaluation was performed in the remaining seven patients. RESULTS: At US, all 10 sites of pseudarthrosis seen at surgery were identified correctly. Of 10 sites with solid fusion at surgery, US depicted six. At four sites (two patients), fusion was mistaken for or obscured by hardware. Overall, sensitivity was 100%, specificity was 60%, and accuracy was 80%. CONCLUSION: US can play a valuable role in the detection of posterolateral bone graft pseudarthrosis, especially when hardware is present.

Adult↗
PubMed2006

[Congenital pseudarthrosis of the clavicle: a review of nine cases].

BACKGROUND: Congenital pseudarthrosis of the clavicle (CPC) is a rare malformation of the scapular waist of unknown etiology. About 200 cases have been reported. The lesion is usually right-sided and unilateral. Physical examination reveals swelling over the midportion of the clavicle, usually asymptomatic, and the diagnosis is confirmed by plain radiology. The differential diagnosis includes posttraumatic pseudarthrosis, cleidocranial dysostosis and neurofibromatosis. Surgical treatment is indicated in symptomatic patients or those with major deformity. MATERIAL AND METHODS: We performed a retrospective review of the medical records of 9 patients with CPC managed in our hospital. RESULTS: We studied 5 girls and 4 boys with unilateral right CPC. None of them had a familial history of CPC. Four complained of local pain, without functional impairment, and clavicular asymmetry on x-ray ranging from 0.5 to 1 cm, without accompanying pathologic findings. Surgical treatment was performed in 5 patients, with resection of fibrous pseudarthrosis, internal fixation, and iliac bone graft. In 4 patients callus response was achieved at 2 months. The remaining patient required withdrawal of orthopaedic material 2 months after surgery because of its protrusion through the skin, without the need for further surgical treatment to date. CONCLUSIONS: Although rare, CPC should be included in the differential diagnosis of clavicle abnormalities in children. Good results are achieved with current surgical techniques in patients with clear indication.

Child, Preschool↗
PubMed1981

The detection of spinal pseudarthrosis in ankylosing spondylitis.

Spinal pseudarthrosis in ankylosing spondylitis can be difficult to detect radiologically. This paper assesses the value of bone radioscintigraphy in relation to radiological examination. The patterns of tracer uptake in the spine were recorded in 16 patients in whom established ankylosing spondylitis was complicated by pseudarthrosis. Twenty such lesions were identified in 16 patients, mostly occurring at one level in a junctional region of the spine. Four patients had two lesions. Standard radiography failed to demonstrate this abnormality in seven patients and in these, tomography was required to confirm the scintigraphic findings. On the other hand, bone scanning was much less accurate in detecting the minor forms of marginal vertebral end plate destruction (spondylo-discitis) than conventional radiology. This study suggests that 99TcmMDP scanning is a useful primary investigation for the detection of spinal pseudarthrosis in patients with chronic ankylosing spondylitis who suffer late onset back pain.

Adolescent↗
PubMed1993

Late-onset pseudarthrosis of the dysplastic tibia.

Eleven children in whom a tibial fracture occurred after minor trauma had pre-existing dysplastic changes evident radiographically. These changes included cortical tapering, sclerosis, and formation of a cyst in the region of the medullary canal. Ten of the eleven patients had had no more fractures an average of fifteen years after the most recent fracture. Six of the fractures healed following prolonged immobilization in a cast, but four of the six tibiae were abnormally bowed anteriorly, and it was thought that a stress fracture could occur in the future. Four of the patients had a clinically straight tibia and radiographic evidence of thick cortices following corrective osteotomy, intramedullary fixation with bone-grafting, and prolonged immobilization in a cast. At the most recent follow-up examination, the eleventh patient had a persistent pseudarthrosis despite several operative procedures. While there were too few patients in this series for us to draw definite conclusions, our findings suggest that late-onset pseudarthrosis of a dysplastic tibia has a better prognosis than does congenital pseudarthrosis.

Bone Diseases, Developmental↗
PubMed1988

Spinal pseudarthrosis complicating ankylosing spondylitis: comparison of CT and conventional tomography.

Pseudarthrosis, a functional false joint, is an important mechanical complication of advanced ankylosing spondylitis. For correct determination of prognosis and therapy, the presence and extent of the abnormality must be assessed accurately. CT findings in 18 patients with 22 pseudarthroses were compared with the results of conventional tomography. The condition was confirmed by surgery in nine patients (50%). CT scans clearly characterized the pseudarthrosis, providing data not provided by conventional tomography in 17 (77%) of the 22 lesions. CT showed irregular diskovertebral osteolysis with reactive sclerosis and more frequently detected the vacuum phenomenon and paraspinal swellings. In all cases, CT showed either a fracture of mobile facet joints in the posterior elements and allowed clear differentiation between the two. In four cases, such differentiation was impossible on conventional tomograms. Associated spinal stenosis was suspected on conventional tomograms in three lesions, but it was shown clearly by CT in 10 lesions. Our results suggest that CT offers considerable advantages over conventional tomography in the investigation of spinal pseudarthrosis complicating ankylosing spondylitis.

Adult↗
PubMed2001

Congenital pseudarthrosis of the tibia in pediatric patients: MR imaging.

OBJECTIVE: The purpose of this study was to describe the characteristics of congenital pseudarthrosis of the tibia on MR images of infants and children and to assess the value of MR imaging in evaluating this disease. CONCLUSION: MR imaging of congenital pseudarthrosis allows assessment of the type and extension of the disease. It is especially recommended for the evaluation of periosteal and soft-tissue changes near the pseudarthrosis.

Child, Preschool↗
PubMed1999

Congenital pseudarthrosis of the tibia. Treatment and outcome at skeletal maturity in 10 children.

We present the clinical characteristics of 14 children with congenital pseudarthrosis of the tibia (CPT) treated in northern Finland at Oulu University Hospital during the years 1968-1996. The incidence of CPT in northern Finland was 3.5/10(5) during years 1962-1996, which is higher than in previous reports. The estimated prevalence of pseudarthrosis was 1.8/10(5). The first 10 children with a mean follow-up time of 19 years from the first grafting procedure and 14 years from the last grafts, all had a solid bony union and could walk without external support at the last follow-up. The best treatment, especially in unfavorable types, seems to be a radical resection of the pseudarthrosis and reconstruction with a free vascularized fibular graft.

Age Determination by Skeleton↗
PubMed1999

Bilateral congenital pseudarthrosis of the clavicle report of a case with clinical, radiological and neurophysiological evaluation.

Congenital pseudarthrosis of the clavicle is a rare condition. Bilateral involvement is extremely rare: only seven cases have been reported in the literature. Although the anatomy of the thoracic outlet can be markedly altered by the hypermobility of the shoulder and although few cases with mild symptoms suggesting brachial plexus impairment have been reported, the neurological status of the brachial plexus in congenital pseudarthrosis of the clavicle has not been well assessed. We report a case of bilateral congenital pseudarthrosis of the clavicle in which clinical, neurophysiological and radiological evaluations were performed.

Child, Preschool↗
PubMedDate not supplied

Congenital pseudarthrosis of the tibia: a retrospective review.

Treatment of congenital pseudarthrosis of the tibia has been notoriously very difficult. This retrospective review of 36 cases (33 patients) seen at the Shriners Hospital (Springfield, Massachusetts Unit), from 1927 to the present evaluates the different forms of treatment and their long-term results. Adequate information was obtained from 30 patients (33 cases) to include them in this study. Twenty-nine patients (32 cases) underwent 154 surgical procedures, of which 138 were to achieve union of the pseudarthrosis (4.3 per case), and one parent could not recall how many surgeries were performed on her son. Sixteen surgeries were performed to correct leg-length discrepancy. Four cases healed with bracing alone. Of the 30 patients (33 cases), 14 patients (15 cases) required amputation. These patients had undergone multiple attempts at union (average, 4.7 procedures). Patients who went on to union averaged 2.8 surgical procedures. Even with newer techniques available to the trained pediatric orthopaedist, treatment of congenital pseudarthrosis of the tibia remains an elusive problem.

Amputation, Surgical↗
PubMed1999

The value of scintigraphy in the diagnosis of pseudarthrosis after spinal fusion surgery.

The utility of planar bone scintigraphy was evaluated for discerning bony union after spinal fusion surgery, especially in cases of clinically and radiologically suggested pseudarthrosis. Between 1991 and 1996, the authors performed bone scintigraphy on 42 patients (21 women, 21 men; mean age, 42 years) after spinal fusion surgery (32 posterolateral, 10 combined) and just before their admission to the hospital for material removal. The fusions consisted of 29 lumbosacral, 6 thoracolumbar, 3 lumbar, 2 thoracolumbosacral, 1 thoracic, and 1 cervical. The mean fusion spanned four segments, and the mean time between spinal fusion and material removal was 27 months. The scintigraphy was performed using the tracer Tc-99m. Based on the scintigraphy data, the radiologist suspected pseudarthrosis in five patients (12%), and the condition was confirmed in four patients during operation (10%), two diagnosed and two undiagnosed. The accuracy of the method was 88%; sensitivity, 50%, specificity, 93%; positive predictive value, 40%; and negative predictive value, 95%. The sensitivity and positive predictive value of bone scintigraphy are low for possible instability after spinal fusion. The method is not sufficient to reliably diagnose pseudarthrosis after spondylodesis.

Adult↗
PubMed2000

Pseudarthrosis repair. Autogenous iliac crest versus femoral ring allograft.

Pseudarthrosis repair in the lumbar spine is one of the most challenging problems faced by spine surgeons. Historically high failure rates with posterior repair have led to the use of anterior lumbar interbody fusion with tricortical iliac crest autograft in these difficult cases. More recently, femoral ring allografts packed with autograft bone have been advocated as another method that would decrease donor site morbidity. Two series of patients underwent anterior lumbar interbody fusion with anterior instrumentation to repair pseudarthrosis (Group I, 33 patients with tricortical autogenous iliac crest and Group II, 20 patients with femoral ring allografts). At minimum 2-year followup, there was no difference in fusion rates (Group I, 32 of 33 versus Group II, 20 of 20). Patients in Group I had radiographic fusion develop more rapidly than patients in Group II (12 months versus 18 months), but a significant proportion of patients in Group I (35%) had an average of 2 mm of graft subsidence. Despite excellent fusion rates in both groups, functional outcomes were not as good with only 28% of patients in Group I and 36% of patients in Group II returning to work. Using anterior instrumentation, anterior interbody fusion offers an excellent method to repair pseudarthrosis using femoral ring allografts or autogenous iliac crest. However, femoral ring allografts offer the potential to decrease donor site morbidity, allowing the surgeon to treat multiple spine levels.

Bone Transplantation↗
PubMed2000

[Congenital pseudarthrosis of the clavicle: 25 childhood cases].

PURPOSE OF THE STUDY: Congenital pseudarthrosis of the clavicle is rare, only 200 cases having been reported. Based on 25 personal cases and an overview of the literature, we try to explain the etiology of this condition and the different kinds of treatment. MATERIAL AND METHODS: A retrospective analysis was performed on twenty-five children (16 females, 9 males, mean age at the end of the follow up - 11.5 yrs) from three different centers. We assessed the outcome of surgical and nonsurgical procedures, based on pain, functional ability, cosmetic results, and x-ray examination. RESULTS: A family background was noted in three children. The lesion always involved the right side. Twenty patients presented a bump over the middle third of the clavicle, thirteen a foreshortened shoulder girdle, three complained of discomfort. In two cases, palpation of the clavicular area was painful. No neurovascular compressive syndrome was reported. None of the patients complained of a decrease in the range of motion or in the strength of the upper limb. X-rays showed a middle third defect. In five cases we found abnormal first ribs. Seventeen patients underwent surgery, at a mean age of 6 years and 4 months. The procedure always included excision of the pseudarthrosis at both ends and internal fixation with a wire or a plate. In only eight cases a bone graft was used. Healing was achieved in fourteen patients. Three patients needed a second surgical procedure. In these 3 cases we had not used bone grafting. All patients had a normal range of shoulder motion, except a twelve year old girl who complained of discomfort of the right upper limb. The cosmetic result was good in eleven cases, one surgical wound was noted as hypertrophic, and one developed a keloid. An asymmetry of the trunk was still noted in seven cases. The x-rays showed symmetric clavicles in ten cases. Eight patients were treated conservatively. All of them had a normal range of motion of the shoulder, six had a good cosmetic result and two cases a poor one. DISCUSSION: According to Alldred, the anomaly results from the failed coalescence of the two primary ossification centers of the clavicle. The overview of the literature and our findings (in one case) confirm that the cartilage which covers both ends of the bone is made of growth cartilage. However, the true mechanism of the nonunion is still unknown. The three familial cases of our work suggest a possible genetic transmission of the disease. The diagnosis is based on the following criteria: right side lesion, found in infancy, without previous fracture, increasing size with growth, without major functional consequences, without neurofibromatosis or cleidocranial dysostosis symptom. X-rays or histologic examination will confirm the diagnosis showing the usual findings described above. Complications of the pseudarthrosis of the clavicle are rare and late. Conservative management appears to give good results as seen with our eight patients. However surgical treatment ensures symmetrical shoulder girdles and good function with few complications. Therefore, we recommend performing an excision of the cartilaginous caps, followed by an iliac bone graft and an internal fixation with wire. Surgical management will be preferred in symptomatic patients, in the case of major or increasing deformity, or on parental request.

Arm↗