Congenital scoliosis: a frontal plane evaluation of 251 operated patients 14 years old or older at follow-up.

INTRODUCTION: Congenital scoliosis, carrying an incidence between 0.5 and 1 per 1000 births, raise the problem of their evolutive potential. HYPOTHESIS: Some predictive factors for the evolution of scoliotic curvature due to congenital vertebral malformation (CVM) can be found. MATERIAL AND METHODS: This was a retrospective multicenter study of 251 patients, at least 14 years old when evaluated at end of follow-up, with CVM and spinal deformity predominating in the frontal plane. RESULTS: 38.8% of patients showed associated neurologic, visceral or orthopedic abnormalities. CVM was single in 60.6%, double in 20.3%, triple in 6.4% and multiple in 12.7% of cases. 34.1% of CVMs were thoracic. Congenital scoliosis curvature was single in 88.8% of patients, double in 10% and triple in 1.2%. Mean curvature angle was 31.7° at diagnosis (range, 0-105°) and 41.3° preoperatively (range, 10-105°). Sixty-one patients showed associated kyphosis. Mean change in postoperative curvature angle over follow-up was 1.6° (range, -20° to 38°) in the 73 patients managed by arthrodesis, -0.4° (-24° to 30°) in the 64 managed by epiphysiodesis, and 0.4° (-18° to 35°) in the 49 managed by hemivertebral (HV) resection. Results were found to correlate significantly with age at surgery for patients managed by epiphysiodesis, but not for those managed by HV resection or arthrodesis. DISCUSSION: More than 30% of congenital scolioses involve associated intraspinal abnormality. All CVM patients should therefore undergo medullary and spinal MRI to assess the CVM in all three planes, and the medullary canal and its content. The evolution of scoliotic curvature induced by CVM is hard to predict. Several factors are to be taken into account: CVM type, number and location, and patient age. Curvature progression may be slow or very fast. It accelerates during the peak of puberty, stabilizing with bone maturity. Surgery is mandatory in evolutive scoliosis. Four procedures may be recommended, according to type of CVM and especially to patient age: arthrodesis, convex epiphysiodesis, HV resection or rib distraction. Surgery seeks to correct the spinal deformity induced by the CVM and prevent compensatory curvature and neurologic complications, while conserving sagittal and frontal spinal balance and sparing as many levels as possible. In case of HV involvement, the procedure of choice is CVM resection, which provides 87.5% good results in this indication; the procedure is relatively safe, conservative of spinal levels, and without age limit. LEVEL OF EVIDENCE: Level IV. Retrospective study.

Applications in spinal imbalance.

The pelvis may be seen as a single vertebra, between the spine and the femurs. The anatomy of this pelvic vertebra has changed with the evolution of species, notably with the transition to bipedalism, with the consequent appearance of lumbar lordosis. The lumbosacral angle, almost non-existent in other mammals, is at its greatest in humans. Pelvic and spinal radiological parameters reflect the sagittal balance of the spine in bipedal humanity. Applications in the management of spinal imbalance are numerous. Arthrogenic or degenerative kyphosis is the stereotypic example of spinal aging. Postoperative flat back following spine surgery is hard to prevent. Scoliosis surgery in adults should now take greater account of the patient's individual sagittal balance, by analyzing the pelvic and spinal parameters. The extent of arthrodeses performed during adolescence to manage idiopathic scoliosis may also induce problems of balance in adulthood if these elements are not taken into account.

Applications in hip pathology.

Everyday clinical practice frequently leads us to suspect a close relationship between the lumbar spine and the hip-joints. Sagittal balance fundamentally expresses a postural strategy mobilizing the dynamic structure of the lumbar-pelvic-femoral complex in an authentic balance by which obligatory coupled movements transmit stresses in a single structure, the spine, to the two-part structure of the lower limbs, and vice-versa. Flexion contracture is a frequent hip pathology, but congenital dislocation and ankylosis of the hip have the greatest impact on the spine, due to excessive mechanical strain and/or spinal malalignment, which is initially supple but becomes fixed. Clinical analysis, backed up if necessary by infiltration tests and imaging, guides indications for surgical management. These considerations suggest a general attitude that considers not just the hip itself, for which the patient is consulting, but the lumbar-pelvic-femoral complex as a whole (and also the knee) before undertaking total hip replacement. Femoro-acetabular impingement is a recently described pathology associating morphological hip-joint abnormality and labral and joint cartilage lesions, leading to early osteoarthritis of the hip. Abnormal spinal or pelvic parameters have not been found associated with femoro-acetabular impingement. Congenital pelvic tilt is a benign and often overlooked pathology in children. Supra- and infra-pelvic pelvic tilt in childhood palsy raises the difficult strategic issue of how to get these children in their wheelchair with a well-balanced spine over a straight pelvis and frontally and sagittally balanced hips.

[Epidemiological survey of wrist fractures in children]. / Les fractures du poignet de l'enfant: étude épidémiologique.

PURPOSE OF THE STUDY: The incidence of wrist fractures is increasing in children. We undertook an epidemiological survey in western France to determine characteristic features. MATERIAL AND METHODS: A prospective multicentric study was conducted over one year in five university hospital centers in western France. The survey included 839 wrist fractures in children aged 6 to 16 years. The following criteria were recorded: age and gender, date and energy of the trauma, side and description of the fracture. Displacement was measured on the AP and lateral radiographs. RESULTS: The boys were older than the girls at the time of the wrist fractures, generally during the summer season (May to October). The left wrist was involved in 55% of the fractures; 83% were metaphyseal fractures. Fractures with epiphyseal detachment were more frequent in adolescents and occurred after high-energy trauma. Metaphyseal fractures were more common in girls than in boys. Displacement was greater in older children, particularly in case of epiphyseal detachment. DISCUSSION: We did not observe the male predominance often reported in our country. The girls probably practice sports as much as boys.

[Angiomatoid fibrous histiocytoma: a rare pathologic entity]. / Histiocytome fibreux angiomatoïde: une entité rare.

Angiomatoid fibrous histiocytoma is an unusual tumor, affecting primarily young adults who develop local disease with favorable prognosis. This contrasts with the aggressive natural history of malignant fibrohistiocytoma. We report case of a 9-year-old girl who presented with a tumor mass of soft tissues with an unusual deep location, thereby with non distinctive clinical features. Surgical treatment was performed.

EXT 1 gene mutation induces chondrocyte cytoskeletal abnormalities and defective collagen expression in the exostoses.

Hereditary multiple exostoses (HME), an autosomal skeletal disorder characterized by cartilage-capped excrescences, has been ascribed to mutations in EXT 1 and EXT 2, two tumor suppressor-related genes encoding glycosyltransferases involved in the heparan sulfate proteoglycan (HSPG) biosynthesis. Taking advantage of the availability of three different exostoses from a patient with HME harboring a premature termination codon in the EXT 1 gene, morphological, immunologic, and biochemical analyses of the samples were carried out. The cartilaginous exostosis, when compared with control cartilage, exhibited alterations in the distribution and morphology of chondrocytes with abundant bundles of actin filaments indicative of cytoskeletal defects. Chondrocytes in the exostosis were surrounded by an extracellular matrix containing abnormally high amounts of collagen type X. The unexpected presence of collagen type I unevenly distributed in the cartilage matrix further suggested that some of the hypertrophic chondrocytes detected in the cartilaginous caps of the exostoses underwent accelerated differentiation. The two mineralized exostoses presented lamellar bone arrangement undergoing intense remodeling as evidenced by the presence of numerous reversal lines. The increased electrophoretic mobility of chondroitin sulfate and dermatan sulfate proteoglycans (PGs) extracted from the two bony exostoses was ascribed to an absence of the decorin core protein. Altogether, these data indicate that EXT mutations might induce a defective endochondral ossification process in exostoses by altering actin distribution and chondrocyte differentiation and by promoting primary calcification through decorin removal.

[Thromboembolic complications in surgery of the spine in children and adolescents]. / Complications thromboemboliques de la chirurgie du rachis chez l'enfant et l'adolescent.

A retrospective analysis by 34 surgical teams from 1981 to 1990 revealed 15 cases of deep vein thrombosis of the lower limb and one pulmonary embolism in children aged 16 or less. The various prophylactic methods used by the different teams led us to undertake a prospective study the aim of which was to determine the risk of deep vein thrombosis and pulmonary embolism in teenager rachis surgery. The purpose of our study was to recognize congenital or acquired factors predisposing to thrombosis and to detect without delay any deep vein thrombosis by ultrasound examination and doppler study. The preliminary results were obtained in 19 teenagers.

[Prolonged mechanical nasal ventilation. Apropos of 27 case of myopathy]. / Ventilation mécanique prolongée par voie nasale. A propos de 27 cas de myopathie.

We report the results of a retrospective study of a group of 27 patients with a myopathy who were ventilated at home using a nasal mask over a period of 5 years. Twelve patients were ventilated in a preventive fashion before any orthopaedic surgical intervention and 15 out of necessity because of respiratory failure and/or hypercapnia. There was a statistically significant improvement in the PaO2 while the PaCO2 remained stable. The vital capacity (CV) was unaltered. Side effects were relatively frequent but did not lead to this method of ventilation being stopped. One patient died from a very advanced cardio-myopathy after having stopped his own assisted ventilation. Another patient died at home of bronchial congestion. One patient had a tracheotomy after 3 years of ventilation. The treatment was judged overall as positive amongst the 19 patients who responded to a questionnaire anonymously. We are able to confirm the efficacy of this mode of ventilation by the nasal route as much therapeutically as prophylactically, which is against the recently reported results in a multi-centre study.

[Circumscribed spinal myositis ossificans of infections origin]. / Myosite ossifiante circonscrite rachidienne d'origine infectieuse.

A case of paravertebral, non-traumatic myositis ossificans in a 4.5 year-old boy is reported. Infectious spondylitis and osteosarcoma were considered before the diagnosis was established on the basis of histological examinations of a biopsy performed during excision. A group G beta hemolytic streptococcus was isolated from the biopsy, suggesting an infectious etiology which has rarely been reported. The child appears to be completely cured one year after excision.

[Chronic pain of the knee in children and adolescents: diagnosis and therapeutic management]. / Les douleurs chroniques du genou chez l'enfant et l'adolescent: dignostic et conduite thérapeutique.

Chronic pain of the knee in children and adolescents may result from multiple etiologies, mainly related to the pathology of the extensor apparatus. The diagnosis is mostly based on a good interrogatory, a careful and systematic physical examination and standard X-rays. Complementary investigations such as arthrography, arthroscopy and CT scan are only needed in a limited number of cases. The pathology of the hip is part of the differential diagnosis.

[Operations for lengthening the upper limbs in children and adolescents]. / Les opérations d'allongement du membre supérieur chez l'enfant et l'adolescent.

We report 16 cases of lengthening of the upper limb: 4 humerus, 12 ulna from 1976 to 1987. Humerus lengthening are progressive with Wagner apparatus. They gave lengthening from 7.5 to 12 cm. There were no important complication. Ulna lengthening were done in patients with multiple hereditary osteochondromes and hereditary multiple exostoses, to correct the deformities of the forearm, and prevent dislocation of the proximal radial head. Actually progressive lengthenings replace immediate ones with radial osteotomy.

[Cartilaginous tumors in children]. / Tumeurs cartilogineuses de l'enfant.

For the last 15 years, 44 cartilaginous bone tumors had been treated in children aging from 2 to 15 years. 80% were chondromas, either isolated (27), or multiples in enchondromata (9). Treatment varied from simple biopsy to extensive excision with cancellous bone graft. Two of these patients experienced postoperative recurrence but have been cured by another surgical excision. One chondromyxoid fibroma of the big toe and 2 chondroblastomas of the femur had been treated by excision with no recurrence. Three young boys (mean age 5 years) who had a metachondromatosis with chondromas of the extremities had been treated with one recurrence. Two children had a chondrosarcoma and both of them died from lung metastasis. Surgical treatment is indicated for all these tumors and is discussed with our results for each of them.

[Pyogenic sacroiliac arthritis in children. Apropos of 6 cases]. / Les arthrites sacro-iliaques à pyogènes de l'enfant. A propos de 6 cas.

Pyogenic arthritis of the sacro-iliac joint is uncommon in children; 6 cases were observed over 4 years. The diagnosis may be difficult and should include specific examination of the sacro-iliac joint. Posterior pain with a febrile illness needs radiological examination and a skeletal scintigraphy, which has always been positive in our experience. All the patients had a very good functional recovery after antibiotherapy and rest (bed or plaster).

[Extemporaneous lengthening of the femur in children and adolescents. Study of 25 cases]. / Allongement extemporané du fémur chez l'enfant et l'adolescent. Etude de 25 cas.

Twenty-five one-stage lengthenings of the femur have been made using a specific technique: an oblique osteotomy in the frontal plane with a mean length of 12 cm, with routine tenotomies and fascial divisions. The average lengthening was 37 mm, that is 9.4 per cent of the femoral length. Complications were uncommon: one infection, three non-unions and one fracture, none of which resulted in any loss of length or deleterious late effects. One-stage lengthening is ideally indicated in adolescents at the end of growth for inequalities of limb length of 40 to 50 mm mainly affecting the femur, whatever the cause.

[Aneurysmal bone cysts in children. Study of 28 cases]. / Le kyste anévrysmal des os chez l'enfant. Etude de 28 cas.

Twenty-eight aneurysmal bone cysts in children aged between 3 and 16 years, with a mean follow-up of five years, have been reviewed. The diagnosis in these uncommon lesions was straightforward in 22 cases, based on the clinical, radiological and macroscopic features. The histological characteristics confirmed the diagnosis. Apart from these typical lesions, there were some cases in which the diagnosis remained doubtful in spite of a review of the sections and a re-assessment of the radiological and clinical findings. For the surgeon, the diagnosis is primarily from a unicameral bone cyst, whose treatment is very different. For the pathologist, the diagnostic problem is not so much from benign dystrophies, which are often difficult to distinguish from them, but from rare lesions in children, such as telangiectatic sarcoma, which have a more serious prognosis. The high recurrence rate, in four cases out of eleven, after curettage led to a preference for more radical treatment whenever possible, such as excision or resection, which was performed in 14 cases and which limited the liability to recurrence. Aneurysmal bone cyst is a benign tumour whose severity depends on its site, especially in the spine, where there is a risk of neurological complications, on its size, which may render surgical removal difficult and on its proximity to the growth plate, which may result in disturbance to growth which not infrequently occurs.

[Van Nes rotation osteoplasty in congenital dysplasias and aplasias of the proximal femur in young children. A study of 6 cases]. / L'ostéoplastie de retournement de Van Nes, dans les hypoplasies et aplasies proximales congénitales du fémur chez l'enfant. Etude de 6 cas.

Six rotation-plasties, as described by Van Nes, have been carried out in children between 5 and 11 years of age, 5 of whom had congenital defects of the proximal femur, and the other femoral hypoplasia. The operation involved arthrodesis of the knee, accurate shortening of the leg so that the ankle would be at the same level as the opposite knee at the end of growth, and clockwise derotation of the leg through 180 degrees. The only complications encountered were 3 transient palsies of the peroneal nerve. Rerotation was only required if the operation was performed in early childhood, or if it had been carried out counterclockwise. The ideal age for operation seems to be between 8 and 10 years of age. The results are satisfactory. Function and gait are improved. The prosthesis may need to be long because of the pelvi-femoral instability associated with the basic deformity. The operation is indicated in some children with congenital abnormalities of the femur who would otherwise require a rigid brace or an amputation to accommodate a prosthesis.

[Braces for congenital leg length inequality in children]. / L'appareillage des inégalités de longueur des membres inférieurs d'origine congénitale chez l'enfant.

Despite recent advances in techniques of limb equalisation, some congenital anomalies cannot be corrected and shortening at the end of growth is severe. These children then need to be braced permanently. The role of the surgeon is then limited to satisfactory adaptation of the prosthesis which must be light, cosmetic and functional. The authors described different types of bracing according to the type of anomaly, its level, its permanent state and the existence of unilateral or bilateral involvement. Indications are given for surgery to obtain satisfactory fitting of the patient to the brace. In congenital femoral aplasia were realised either bracing similar to that of above the knee amputation after knee arthrodesis, or bracing after Van Nes procedure, the foot being rotated 180 degrees to allow the motion of the braces. The authors have observed 20 such cases.

[The surgical form of the hemolytic-uremic syndrome]. / Forme chirurgicale d'un syndrome hémolytique et urémique.

The hemolytic-uremic syndrome consist of micro-angiopathic hemolytic anemia, acute renal failure, and thrombocytopenia following a prodromal illness of gastroenteritis. The syndrome can present in dramatic fashion with severe abdominal pain and signs of peritonitis suggesting an acute surgical crisis. In some cases, laparotomy is necessary, and we report a case with colectomy.

Anatomical basis of the surgical approach to the kidney in children.

The authors studied the different surgical approaches to the upper urinary tract in children. These approaches are the posterolateral lumbar, posterior and anterolateral lumbar routes. Special emphasis should be given to the anterolateral extraperitoneal approach. This approach in children gives excellent exposure of the kidney and causes minimum disruption of the normal anatomy, thereby yielding very satisfactory functional and cosmetic results.