1. The operation of pseudarthrosis of the hip joint is described and the results are assessed in ninety-three patients. 2. The result was good in eighty-three cases and poor in seven cases. Three patients died as a result of the operation. 3. Pseudarthrosis is the most satisfactory and the most reliable operation: 1) in ankvlosing spondylitis, and 2) in patients over sixty years of age with disabling osteoarthritis. 4. The more formidable operation of cup arthoplasty may prove to be superior in younger patients with osteoarthritis

Introduction: Spine fractures are common manifestation of osteoporosis. After an acute osteoporotic vertebral compression fracture pain persisting even after 3 months and clinical tenderness should raise the suspicion of pseudarthrosis. Pseudarthrosis is not a rare complication of a benign osteoporotic vertebral collapse occurs in about 10% of cases after an acute collapse. Treatment plan needs to be individualized. Cement augmentation procedures such as kyphoplasty and vertebroplasty can be performed in the absence of neurological deficit, whereas decompression and stabilization is necessary in presence of neurological deficit. Study Design: Prospective cohort study. Methods: 31 patients who were diagnosed to have an acute osteoporotic vertebral compression fracture were managed conservatively. Pain persisting after 3 months and clinical tenderness in 5 patients prompted further investigation, revealing pseudarthrosis. None of them had neurological deficit. Imaging of two patients revealed vacuum sign with intravertebral cleft on plain radiographs and on MRI. All of them were at the Dor-solumbar junction and of crush typeof VCF. Results: The incidence of pseudoarthrosis after an oste-porotic VCF was found to be 16.12%. One patient was treated with kyphoplasty, one with vertebroplasty with good pain relief and restoration of functional ability, and rest three are awaiting kyphoplasty. Conclusion: High suspicion of pseudarthrosis is to be kept in mind as it is not an uncommon complication of benign osteoporotic collapse. Vertebral augmentation procedures such as kyphoplasty and vertebroplasty are promising procedures for treatment in absence of neurological deficit

PURPOSE. To report the experience and evolution of treatment of Congenital Pseudarthrosis of the Tibia in a single surgeon, consecutive series of 19 patients. METHODS. A retrospective case series review, with radiological assessment and functional outcome scores including the Activity Scale for Kids (ASK) and the Oxford Ankle Foot Questionnaire (OAFQ) Child & Teenager version. Patients were classified as per Boyd & Andersen. Alteration in the Tibial mechanical axis was compared pre-treatment and at last review. RESULTS. The average follow up from presentation was 6.3 years, 7 patients had reached skeletal maturity. Union was obtained in 79% at the time of review with a mean time to union of an established pseudarthrosis of 15.2 months. 9 of 19 had an established diagnosis of Neurofibromatosis. 8 had a frank pseudarthrosis at presentation, 2 of which were present at birth. There were 3 re-fractures following treatment. One of the 19 required amputation after failed surgical treatment. Each patient had an average of 2.4 surgical procedures. 14 patients had either fixed or elongating rods, 10 had Ilizarov treatment. 2 have outstanding surgery planned. CONCLUSIONS. A philosophy of treatment has evolved which produces acceptable results. Splintage is used for antero-lateral bowing with corrective osteotomies and rodding, without extensive bone or periosteal resection, for significant or progressive deformity or fracture. Ilizarov treatment is used for recalcitrant cases over the age of 6 years. The technique has evolved over time to include bone graft wraps and the use of Bone Morphogenic protein in combination with the Ilizarov technique. Patients have below population and age matched average scores for both the ASK and OAFQ

Supraglenoidal dislocation of the humerus is uncommon. The anatomical findings in a bilateral example in which efficient pseudarthrosis had developed are described, and the probable evolution of the lesion is discussed.

1. Thirty-three cases of congenital pseudarthrosis of the clavicle are presented.

2. The lesions all occurred in the right clavicle and are thought to be due to abnormal intrauterine development rather than non-union of birth fractures.

3. Methods of treatment are reviewed. Excision and bone grafting is favoured.

1. Thirteen cases of congenital pseudarthrosis of the clavicle conforming to the classical picture are presented, together with five cases of pseudarthrosis occurring for various other reasons, and nine cases of congenital pseudarthrosis that form a special group because there was a strong family history.

2. Treatment, if desired by the patient, is easy, but should be deferred until the age of four or five years.

3. The etiology remains obscure. Our embryological study does not support the theory that the clavicle normally develops from two ossification centres that may fail to coalesce. Although in thirteen of our patients there was no family history of pseudarthrosis, there was another group of nine patients who presented a strong family history, and it seems certain that in some instances congenital pseudarthrosis of the clavicle can be transmitted genetically.

1. Three cases of infantile pseudarthrosis of the tibia treated successfully by delayed autogenous by-pass graft are reported.

2. The delayed autogenous graft is stouter, stronger and more easily handled and has enhanced osteogenic properties than a graft transferred immediately.

3. The by-pass graft commends itself, firstly, because it does not disturb the pseudarthrosis, which in consequence helps the immobilisation of the graft; secondly, because it is well embedded in healthy bone above and below, well away from the abnormal bone; thirdly, because it lies under compression and, ideally, is vertically disposed between the knee and the ankle; and fourthly, because there is no devitalising stripping of periosteum or introduction of foreign bodies.

4. Support to the grafted leg is needed for at least five years, but only by a polythene splint after four to six months.

5. With early grafting the deformity straightens out and shortening is overcome, as there is early return to normal use of the limb.

6. Prolonged follow-up is called for lest the basic lesion in the tibia should extend.

7. Fibrous dysplasia and similar fibrous lesions of bone account for many cases of infantile pseudarthrosis of the tibia. Many of these lesions are congenital and subsequently lead to fracture.

8. Postponement of surgery should not be countenanced.

1. Congenital pseudarthrosis of the clavicle is described.

2. The distinction from cranio-cleido-dysostosis and from birth fracture is stressed.

3. Details are given of two patients reported in the literature and a further nine patients are described.

4. The embryology, natural history and treatment of the condition are discussed.

1. The results of the by-pass grafting operation in eleven cases of pseudarthrosis of the tibia in childhood are presented.

2. The operation was successful in securing sound bone union in nine cases, and unsuccessful in two.

3. The causes of failure in the two unsuccessful cases are analysed.

4. The possible causes of occurrence and persistence of tibial pseudarthrosis in childhood are discussed.

5. It is suggested that the pseudarthrosis results from a fatigue fracture of a congenitally abnormal tibia, but that its persistence depends entirely on mechanical factors.

6. There is evidence to suggest that the underlying congenital abnormality may arise from dysplasia of the mesenchyme.

CPT is a uniquely difficult condition, often associated with Neurofibromatosis (NF1), where bone healing is compromised. Although rare, the severity of this condition and the multiple procedures often entailed in treating it, warrant research attention. As study material is limited, animal models of the disorder are desirable for testing new treatments.

We sought to create a model of CPT where both copies of the NF1 gene were ablated at the fracture site, as has been found in some clinical specimens. NF1 floxed mice had fracture surgery; both closed fracture and open osteotomy were performed. Either a Cre- or control GFP-adenovirus was injected into the fracture site at day zero. Recombination was confirmed in ZAP reporter mice. Additionally, cell culture studies were used to examine the possible responses of NF1+/+ (wild type) NF1+/− or NF1−/− to drugs which may rescue the dysregulated Ras/MAPK pathway in NF1.

In closed fractures, radiographic bridging was 100% in NF1+/+ calluses and <40% in NF1−/− calluses (P<0.05). In open fractures, radiographic bridging was 75% in NF1+/+ calluses and <30% in NF1−/− calluses (P<0.05). In both fracture repair models the NF1−/− state was associated with a significant up to 15-fold increase in fibrotic tissue invading the callus by week 3. In NF1−/− fractures, large numbers of TRAP+ cells were observed histologically in the fibrotic tissue. Closed fractures also showed a significant increase in BRDU labelled proliferating cells in the callus. In cell culture models of NF1 deficient osteogenesis, NF1−/− progenitors were found to be significantly impaired in their capacity to form a calcified matrix as measured by Alizarin Red S staining and osteogenic markers (Runx2, Osteocalcin, Alp expression). However, when differentiated calvarial NF1 floxed osteoblasts were treated with Cre adenovirus, mineralization was not affected, suggesting that NF1 impacts on osteogenic differentiation rather than mature cell function. Treatment with MEK inhibitor PD0325901 was found to rescue the NF1−/− progenitor differentiation phenotype and permit robust mineralization. Treatment with the JNK inhibitor SP600125 was also able to improve ALP activity and mineralization in NF1+/− osteoprogenitors compared to control cells.

This model of NF1 −/− induction at a fracture or osteotomy site closely replicates the clinical condition of CPT, with lack of bone healing and fibrous tissue invasion. Underlying defects in bone cell differentiation in NF1 deficiencies can be at least partially rescued by JNK and MEK inhibitors.

Congenital pseudarthrosis of the tibia (CPT) is a rare but well recognised condition. Obtaining union of the pseudarthrosis in these children is often difficult and may require several surgical procedures. The treatment has changed significantly since the review by Hardinge in 1972, but controversies continue as to the best form of surgical treatment. This paper reviews these controversies.

Cite this article: Bone Joint J 2013;95-B:1027–34.

1. The cases of four children who presented with bowing or pseudarthrosis of the fibula only, are described.

2. There is a gradation in the severity and significance of this condition. There may be fibular bowing without fibular pseudarthrosis; fibular pseudarthrosis without ankle deformity; fibular pseudarthrosis with deformity but without the late development of tibial pseudarthrosis; or fibular pseudarthrosis with the late development of tibial pseudarthrosis.

3. Proper management is dependent on a knowledge of this range of conditions.

The problem of retained drain fragments is a well known but under reported complication in the literature.

The authors present the case of a 66 years old male, who suffered a right distal humerus fracture luxation six years ago that was treated conservatively. He went to the emergency service with fever and right elbow purulent drainage.

Physical examination showed deformity, swelling and fluctuation of the right elbow with purulent drainage through cutaneous fistula. The x-ray showed instable inveterate pseudarthrosis of the distal humerus. Leucocytosis and neutrophylia with increased CRP were presente in the blood tests and the patient started empiric treatment with Ceftiaxone IV. A MRSA was isolated in cultural exam of the exsudate, and a six weeks treatment with Vancomycin IV was iniciated.

Exhaustive surgical cleaning was performed and two plastic foreing bodies (fragmented drains) were removed.

At the time of discharge the patient was afebrile, with normal analytical parameters and negative culture tests.

The orthopaedic surgeon should considerate the presence of a foreign body in patients with infected abcess and traumatic or surgery previous history.

Three or more years after completion of treatment, we re-examined 16 patients with orthopaedic problems associated with neurofibromatosis I (NF-I) who were treated at our institution between 1976 and 1999. Seven boys and five girls between the ages of 5 and 15 years presented with congenital pseudarthrosis of the tibia (CPT). All had undergone previous surgery elsewhere.

The patients had typical skin lesions and the associated radiological appearances of pseudarthrosis of the tibia. There were two cystic types of CPT, five hourglass and five normotrophic types, mostly at the level of the distal third of the tibia. Primary consolidation of the CPT was not obtained in any patient. Three patients underwent below-knee amputation after multiple surgical procedures. Eight had consolidations of the pseudarthrosis after multiple operations, but all had residual deformities and/or shortening. One patient remained with a non-consolidation. The surgical procedures included intramedullary rodding with or without bone grafting, fibular bypass grafting, Soffield turn-about rodding, electrical stimulation, and, in patients seen since 1989, various Ilizarov techniques including lengthening and bone transport.

Our results suggest that the best treatment of this perplexing paediatric orthopaedic problem remains undetermined. Considerations for the selection of treatment include the pathologic anatomic pattern of NF-I and the patient’s age and expectations. A National Orthopaedic Neurofibromatosis Register will be useful in the decision-making process.

Presenters Position:

Fourteen cases of pseudarthrosis of the tibia in childhood presented at a hospital in Burma over a period of eight years. The ages of the patients ranged from one month to seventeen years. Nine were treated by a pointed graft driven into the medullary cavity of the distal tibia, and usually across the ankle joint into the body of the talus, before fixation to the proximal tibia. In six of the nine union was secured, but one case required a second grafting. Transarticular segments of graft showed a marked tendency to undergo absorption. No significant deformity was observed to follow central penetration of the growth plate and epiphysis of the lower tibia.

1. The danger of fracture and subsequent pseudarthrosis in congenital anterior bowing of the tibia is emphasised. The fate of seven such patients is described.

2. With the object of preventing fracture, early and urgent bone grafting is advocated.