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Purpose: To access the long-term outcomes for patients with arthrogryposis multiplex congenita at adult age., Materials and Methods: The cases were traced for most of them thanks to direct contact maintained from child hood, from colleagues interested from other parts of the country, and from the list from Alliance arthrogryposis association (parents and patients). The methods used were: mostly direct clinical examination, some phone calls or email. All answered a questionnaire for general life and mainly for function. One of these questions was: what is the function you missed most during your life?, Results: 65 patients( 41 females and 24 males) were reviewed at adult age from 22 to 65 years. For the personal life: 38 are married and had 34 children with only 4 having arthrogryposis. Only 27 (15 F/12 M) were living alone. Self-sufficiency was observed in 35, partial in 20, total dependence in 10 people. 38 reached university level, 20 had secondary school level, 10 had primary school level. Ambulation was made possible with wheelchair: permanent for 18, partial for 9 ambulating at home only, 8 ambulating outside with crutches and 29 were free walkers. 36 patients out of 65 were driving a car sometimes modified with special equipment. The involvement of spine was seen in 26 patients requiring surgical treatment sometimes complex combining anterior and posterior approach in 14 cases. Surgery of the lower limbs (hips, knee, feet) was very often repeated, with almost always stability, pain free and function. The most important finding was that 52 patients had more or less severe involvement of the upper limbs which was considered by the majority of the patients to be the most disabling, more than the absence of walking!, Conclusion: Finally, it appeared that for the care of these patients, priority goes to the upper limbs function, because majority of these patients have a high level of intelligence. A remarkable fact is that many of these patients had to spend a lot of time during infancy and childhood in rehabilitation centers with education adapted for schools and teachers. Finally, they are grateful for that, telling often that it would have been much more difficult if not impossible to have such a treatment and education at home.

The few number of severe thoracic scoliosis requiring surgical treatment makes the description of its therapeutic course difficult. Twenty-one cases of severe thoracic scoliosis with Cobb's angle >90 degrees were treated surgically in the past 20 years. Surgical treatment was performed because of an evolutive scoliosis or in case of respiratory or functional impairment. All patients underwent posterior approach after a preoperative preparation by the use of halo-traction. In 6 cases, a previous surgical posterior spinal release was performed before the halo-traction period. Fusion achieved in all cases and the final correction was generally better than preoperative reducibility. The surgical course was guided by the poor functional status of these patients, especially concerning respiratory function. Spinal instrumentation was made up of 3 rods with few spinal implants in the majority of the cases. Surgical correction was made by means of rods narrowing without any rod's rotation procedures. Functional improvement, especially respiratory was noted in all the cases. Nevertheless, postoperative spirometries were not significantly different from the preoperative ones. The aesthetic improvement of the rib hump was disappointing making necessary a complementary thoracoplasty in two cases. Surgical treatment of severe thoracic scoliosis remains justified in adults because of a progressive functional and radiological deterioration with respiratory compromise. We performed spinal fusion by posterior approach only, after a preoperative period of halo-traction. The final correction depends on the preoperative reducibility. We noted a functional and respiratory improvement in all the cases.

Introduction: We report our experience of five cases in adolescents with spastic quadriplegia who had primarily sagittal plane deformity with hyperlordosis of the lumbar spine, an uncommon condition in cerebral palsy spinal deformity., Methods: Three boys and two girls, 13 to 19 years old, were surgically treated for excessive lumbar lordosis which made sitting difficult. Bilateral hip flexion contractures were present in all cases with a very horizontal sacrum and, in four cases, associated with pelvic anteversion. Two patients were surgically treated by posterior spinal fusion and instrumentation, and three patients had anterior discectomy and fusion prior to posterior fusion and instrumentation., Conclusion: Hyperlordosis was reduced by 26 to 48 degrees , and the horizontal sacrum was corrected by 15 to 35 degrees , which enabled the patients to sit comfortably.

Study Design: Retrospective study., Objective: To report nine cases of Charcot's joint of the spine, to clarify the difficulty in diagnosis and treatment, and to analyze the literature., Summary of Background: Charcot's joint of the spine, also known as spinal neuropathic or neurogenic arthropathy, is a destructive condition that affects the intervertebral disc and the adjacent vertebral bodies. It is the result of a loss of joint protection mechanisms, generally secondary to a spinal cord lesion. We report a series of nine patients treated surgically., Methods: Eight men and one woman suffering from paraplegia or tetraplegia were reviewed. The time interval between the neurologic disorder and the diagnosis of neuropathic spinal arthropathy was 10 to 36 years. The most frequent presenting symptom was an evolutive thoracolumbar kyphosis, sometimes associated with back pain or increased spasticity in the lower limbs. The neuropathic arthropathy involved the thoracic spine in four patients and the lumbar spine in four other patients. The remaining patient presented two arthropathies, one thoracic and one lumbosacral. A percutaneous vertebral biopsy was performed in five patients suspected to have an infection or a tumor. Treatment was always surgical. In eight cases, a circumferential fusion was performed in the area of the dislocated vertebral levels. The postoperative follow-up was from 3 years to 10 years., Results: A solid and stable circumferential fusion of the spine was obtained in all patients. The functional status improved in all patients. Pain and sagittal imbalance were successfully treated. The increased spasticity observed at the initial examination improved in all patients who returned to the neurological deficit initially present before the onset of Charcot's arthropathy., Conclusions: The diagnosis of Charcot's arthropathy of the spine must be considered in paraplegic and tetraplegic patients with spinal deformity with bone destruction and vertebral dislocation in the absence of an infection or neoplastic disease. The treatment of a Charcot's spine is circumferential fusion and osteosynthesis. Monitoring by clinical and imaging examination must be continued, because multifocal vertebral lesions can occur in cases of extensive proprioceptive deficit.

Purpose: Frequency of hip impairment, with sub-luxation or dislocation, during evolution of neuromuscular diseases depends on intensity and spreading of palsy. At the end of growth or at the beginning of adult life, secondary arthritis can induce pain and lack of mobility. The importance of the chondropathy and irreducible lack of congruence may doom to failure a conservative articular or periarticular surgery. Total hip arthroplasty is an alternative, but the risks of dislocation, ectopic ossifications and infection make often refute this indication. We report our experience of total arthroplasty for paralytic hip, about 18 cases., Materials and Methods: We reviewed retrospectively 14 consecutive patients with neuromuscular disease, who had 18 total arthroplasties of paralytic hips. The origin of neuromuscular disease was cerebral for 7 patients (6 cerebral palsy: 4 spastic or athetosic tetraplegias, 1 spastic diplegia, 1 hemiplegia; 1 head trauma), 6 medullar disorders (1 Friedreich disease, 2 acute anterior poliomyelitis, 1 vascular injury, 1 malformative spine with sacral agenesis and 1 cervical spine trauma) and 1 muscular affection (Steinert disease). Mean age of the patient was 40 year old (19 to 64). Mean follow up was 5 years. Intensity and diffusion of weakness were variables, compatible with gait with or without help for 11 patients, and for 3 patients with sitting posture and transfer. The coxopathy, with pain stiffness and vicious attitudes, induced the loss of gait or sitting posture and transfer. The goal of the arthroplasty was the restitution of the initial function. 11 hip had previous surgery, with infection in 2 cases. Arthritis was secondary to hip palsy in 14 cases (4 dislocations, 6 subdislocations, 3 complications of surgery of paralytic hip dislocation in childhood, 1 nervous arthropathy), and independent of palsy in 4 cases (1 femoral head avascular necrosis ans 3 primary arthritis). The prosthesis were LFA Charnley Kerboull in all cases except 1. We used transtrochanteric approach., Results: Mean follow-up is 5.6 years. Functional initial goal has been obtain in all cases. Ectopic ossifications occurred in 3 cases, without functional consequence. One acetabular loosening occurred after 13 years and has been reoperated on. There was no polyethylene wear. We noticed 1 mechanical and 2 chemical femoral loosening. Prosthetic dislocation occurred in 4 cases, always during the 4 post-operative months, without recurrence after this critical period. There were no infection., Discussion: If gait is possible, there is no satisfactory alternative to total hip arthroplasty. In absence of gait, total hip arthroplasty gives also the best functional results. Girdlestone procedure is not indicated because it will induce the loss of transfer and side effects as ascension of proximal femur with recurrence of adduction bringing out pain and sometimes scabs., Conclusion: Total arthroplasty of paralytic hip induced restitution of initial function for all patients and an acceptable rate of complication after strict selection of patients and indications, specific operative technique and rehabilitation for each patient. This results encourage us to carry on with this therapeutic orientation.

Idiopathic scoliosis starts in childhood, still increases all along adult life, progressively deteriorates especially in lumbar area, and lately results into functional problems in elderly patients. More recently described an osteoarthritic lumbar variety of scoliosis starts "de novo" in the fifties, leading as well to surgical management, mainly in its kyphoscoliotic variety. Surgical correction procedures such as performed in young patients were progressively adjusted to elderly patients, with consideration to special conditions, such as age, general health and bone quality. Besides, orthopedic management and bracing should not be disregarded in older patients.

Idiopathic scoliosis arises at any age in childhood. Its increasing is usually progressive, following growth, with a peak at puberty. Well codified determining factors for severity are: age at beginning, angulation and bone maturation. Functional and consequential effects remain anyway limited. Many scoliosis increase in adult life, giving way to deterioration, prevailing at the lumbar level, with osteo-arthritic changes and typical patterns such as dislocation at the junctional areas. Functional repercussion may thus arise, mainly pain and, in severe thoracic scoliosis impairment of the pulmonary function. A special form of degenerative scoliosis may appear over 45 or 50 years of age with progressive backache and/or radicular pain.

The authors have observed a number of cases of ectopic ossification in patients in a rehabilitation centre for paraplegia. They review the clinical features of the condition, its frequency and its localization. The time of onset and the spontaneous evolution of the condition was found to be variable. Operations were performed on 35 joints - 16 hips, 13 knees and 6 elbows. The results are described. The authors emphasize the value of serial scintigrams before surgery to assess the maturation of ossification. Operations based on the results of such investigations appear to have been followed by a lower rate of recurrence.