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Congenital pseudarthrosis of the forearm represents a rare entity which is often associated with neurofibromatosis type I. Isolated pseudarthrosis of the ulna is the most frequent deformity, followed by pseudarthrosis of the radius and both forearm bones. The treatment of these deformities remains difficult: conventional bone grafts rarely lead to complete fusion. The reconstruction by a vascularized fibular graft represents the treatment of choice if the radial head is not dislocated. This procedure allows restoration of a balance between radius and ulna during growth. The presence of a vascularized graft enhances the potential for bony fusion in a fibrous environment. In cases of isolated ulna pseudarthrosis, a luxation of the proximal radial epiphysis may occur when the ulnar deformity increases with time. The single-bone forearm represents a salvage procedure that creates a stable upper limb with a satisfactory wrist and elbow function. The two clinical cases of a pseudarthrosis of both forearm bones and of an ulna pseudarthrosis are presented.

We report a case of congenital pseudarthrosis of the forearm associated with neurofibromatosis type 1 which was treated by free vascularized periosteal flap transplant and repeated bone grafting. The young female patient recovered good hand and forearm function. A review of the literature revealed the rare occurrence of this disease (approximately 100 cases reported to date), which probably explains the wide variety of surgical proposals.

During a 3-year period, three patients developed arterial complications related to congenital or post-traumatic old pseudarthrosis of the clavicle. Arterial complications of pseudarthrosis of the clavicle presenting as a thoracic outlet syndrome are very rare. Symptoms are variable and occur late. Without treatment, the prognosis is poor with spontaneous development of gangrene. Arterial morphology investigations should be undertaken in patients with pseudarthrosis of the clavicle or isolated arterial symptoms involving the upper limb whose radial pulse disappears during postural tests. Duplex Doppler of the subclavian artery is an excellent screening exam but selective arteriography is the gold standard. It shows proximal arterial lesions (embolytic stenosis of the subclavian artery with post-stenotic dilatation), as well as distal embolic complications. Both static and postural tests must be performed to unmask subclavian restriction by the clavicle, proving its causal effect in the arterial complications. There are four clinical varieties: chronic thrombosis of the subclavian artery, distal arterial micro emboli, acute thrombosis of proximal arteries of the upper limb, and subclavian aneurysm. These lesions are thought to be due to chronic constriction and repeated arterial microtrauma. Congenital or post-traumatic pseudarthrosis, hypertrophic callus, arterial restriction by a screw in a clavicular plate, usually explain the arterial lesions. Bone tumors and Paget's disease are potential but exceptional clavicular etiologies. Surgical treatment is always necessary. Clavicular resection is usually needed in case of pseudarthrosis; there is no functional handicap. Plate fixation and autologous grafting, or open reduction and internal fixation are other valid surgical treatments; The embolytic lesions must be treated to prevent recurrence of distal embolization: graft resection and thromboendarteriectomy have been described. Neurological and venous decompression may be associated at the same time. Complementary treatment can be associated: distal bypass, cervicothoracic sympathectomy, in situ thrombolysis or thrombectomy. Endovascular treatment is not indicated. Optimal treatment of clavicular fractures is required to prevent the development of thoracic outlet syndrome.

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.

Limb salvage surgery is the standard care for most malignant tumor affecting the extremities in the child, and a vascularized fibula transfer is probably the most popular microsurgical option to reconstruct long-bone defects. Between 1994 and 1999, nine children with intractable diseases of the upper limb were treated using free vascularized fibula grafts (one patient had resection in 1983 and initially prosthetic reconstruction, then fibula transplant in 1996). There were 6 boys and 3 girls. Mean age was 10 years (between 6 and 16). Eight patients had defects after sarcoma resection, one had an aggressive enchondroma. The reconstructed sites were the humerus (= 6), the radius (n = 3). The length of the bone defect ranged from 8 to 19 cm (mean: 14.4 cm). The fibula head with the cartilage and the growth plate was used in 3 children. One girl, 4.5 years old with congenital pseudoarthrosis of radius and cubitus had a resection and reconstruction with a U shaped fibula transplant. One patient died from lung and brain metastasis, two years after the reconstruction. There were no local recurrences. The complications were numerous but usually benign; fracture of the grafted fibula n = 7, necessity of additional bone grafts (n = 4) malunion (n = 1) needed reoperation, pseudoarthrosis (n = 2) with reoperation, ankle valgus (n = 1) required reoperation, necrosis of the fibula head (n = 1), radial inclination (n = 1). The ten patients had bone union. The mean period required to obtain radiographic bone union was 5 months. The functional results of the remaining patients were evaluated according to the scale of ENNEKING. The results ranged from 21 to 30 points. Our results were satisfactory with regard to pain, emotional acceptance, manual dexterity. The vascularized fibula graft is indicated in children with large bone defects, more than 8 cm in the humerus, radius and ulna.

Introduction: Risks and benefits of using Ilizarov apparatus in the treatment of congenital tibial or fibular pseudarthrosis (CTFP) are presented in this retrospective study., Materials and Methods: We reviewed with an average follow-up of 3 years and 4 months, the outcome of twenty consecutive patients treated between 1985 and 1993, for a CTFP using the Ilizarov apparatus. Sixteen patients were treated for non union of both tibia and fibula, 1 patient for an isolated non union of the fibula, and 3 patients for correction of a previously treated, malunited pseudarthrosis. The apparatus was used in four different ways: Realignement, end to end compression, and leg lengthening in 14 cases, Simple external fixation in association with another method of treatment in 2 cases, Progressive correction of malunion in 3 cases, Progressive diaphyseal reconstruction in 1 case (fibula)., Results: The mean fixation duration was 7.3 months. Union was achieved with the initial treatment in 11 out of 20 cases (including the 3 cases of malunion correction). Bone grafting was used in 7 out of the 9 remaining cases, and led to bone healing in 3 of them. Five complications were encountered: deep infection in 1 case, repeated stress fracture in 1 case, repeated fracture of the pins in 1 case, malunion in 6 cases, and less than 3 cm leg length discrepancy in 4 cases., Discussion: Ilizarov external fixator is an efficient solution for many cases of CTFP, in which healing did not occur with other methods of treatment. The best indication for its use are the normotrophic and the hypertrophic types of non union (Apoil II), after the age of 4 or 5. Secondary massive bone grafting is to be considered in some cases, since it can either achieve bone union or strengthen it. The major disadvantage of this method is the lack of excision of the dystrophic tissue at the non union site. So, even after the non union is healed, the bone remains dystrophic and fragile, and necessitates a permanente protective orthosis, until the end of bone growth.

The association pseudarthrosis of the clavicle and the thoracic outlet syndrome is rare. The author describe a case in a 64 years old man, found before embolic complications. Shoulder X-ray and artériography démonstrate the lesion. The etiopathogenic hypothesis are called up. In case of complication, the surgical treatment is made of osteo synthesis or partial or total resection of the clavicle, associated to a vascular repair in case of need. At long term the result is excellent.

A new case of congenital nonunion of the clavicle is reported with a histological description. Medullar drilling and bone grafting "in loco" was performed during the surgical procedure. The etiologic hypotheses are discussed. The authors compare congenital nonunions between the pathologies associated with the necessity of medullar drilling for consolidation.

Congenital pseudarthrosis of the tibia is nearly always the result of a congenital bowing of the tibia when it is unilateral and with an antero-external angle. There is no possible spontaneous consolidation and there may be several associated sequellae (shortening and trophic disorders of the limb). The etiological and therapeutic aspects of the affection are reviewed.

The authors have analysed 19 cases of cervical spine fusion in children and teenagers with a review of the literature. In the present series, the levels fused were between the occiput, C1 and C2. The ages of the patients ranged between 2 months and 18 years. There was a great variety of indications for these operations, the majority being congenital deformities or Morquio's disease. The main disabilities were neurological impairment, or instability or deformity of the neck. Recent advances in radiological investigation made it possible to make a more precise diagnosis of lesions with bony deformities, fibrous bands or instability. In 9 instances, a fusion alone was made and in 10 it was associated with cord decompression. The results were analysed in relation to the clinical features, the post-operative mobility of the neck and the incorporation of the graft.

The authors report 7 new cases of congenital pseudarthrosis of the clavicule. Clinical features are bearing out classical data of litterature. The disease is discovered during first months by the way of a clavicular swelling which settles mostly on the right side. But according to the authors the surgical treatment ought to be systematically purposed, restoring a normal morphology and avoiding any functional trouble. It consists in resecting the pseudarthrosis and grafting. This act is easy if done early, the ideal age being around 3 or 5.

Reporting a clinical observation, the authors emphasize upon the extreme scarceness of this variety of so called "congenital" pseudarthrosis of the odontoid process. The congenital origin, due to a lack of fusion, or the acquired one by avascular necrosis following a trauma is explained. The diagnosis may be clinical, consisting either on a vertebral syndrome occurring in childhood, or on a neurological syndrome in the adult age. It is mainly radiological : A scanner investigation is presented here. Owing to the potential neurological severity of the disease, the posterior fusion has to be recommanded.

Eighteen cases of congenital pseudarthrosis of the leg in 17 children were treated by nailing and tibio-fibular graft with a mean follow-up of six years (from one to twelve years). Six patients had reached the end of growth. Primary union was obtained in 17 cases in a mean time of 6.5 months (from 3.5 to 18 months), but one case developed a new pseudarthrosis at the level of an associated upper tibial osteotomy. Only one case required a supplementary graft. Walking was usually achieved without any apparatus after five-and-a-half months, the tibia being protected by a nail which was left in place until the end of growth. In two cases, a fracture produced by trauma occurred in the limb long after consolidation. These two fractures healed in the normal time. Inequality of limb length was present in all the cases, but its progress varied. On three occasions it was treated by femoral lengthening. Stiffness of the ankle and subtaloid joints resulted from nailing of the hindfoot in nine cases and improved very little after freeing of the joints. The use of telescopic rods avoids the need for further operations to change the rod during growth. We consider that this is a reliable method but that, in certain cases, the effects on growth and on the foot can be diminished by appropriate modifications of technique.

Four cases of congenital pseudarthrosis were treated by the Ilizarov method and consolidation obtained by 3.5 months in three patients who had all undergone several previous surgical procedures. Although the length of follow up is short (maximum 3.5 years) consolidation occurred quicker than by traditional methods. This technique also permitted both simultaneous correction of axial malalignment and leg lengthening. We think that this method has a place as a salvage procedure except where the distal fragment is very thin or dystrophic.