Ultrastructural aspects of nerves, bones, and vessels in hereditary sensory neuropathy.

This study concerns two children, from the same otherwise normal family, presenting progressive spontaneous amputations of the limbs, suggesting peripheral hereditary sensory neuropathy. Samples from the distal nerves and vessels and the distal tibial epiphyses were examined ultrastructurally. The nerves showed signs of degeneration, and a typical bone necrosis, without osteoclasts, was observed in the 2 cm margin of the samples. Simultaneous modifications of the vessels suggest a neurally initiated vascular reflex resulting in bone destruction. Theories that might explain the observations are discussed.

Legg-Calvé-Perthes disease. Histochemical and ultrastructural observations of the epiphyseal cartilage and physis.

UNLABELLED: Biopsy specimens of the lateral aspect of the femoral head and neck were obtained from five children with Legg-Calvé-Perthes disease and were studied using histochemistry and electron microscopy. Beneath the normal articular cartilage there was a thick zone of hyaline (epiphyseal) cartilage containing sharply demarcated areas of hypercellular and fibrillated cartilage with prominent blood vessels. The fibrillated cartilage was strongly positive to alcian blue, weakly positive to periodic acid-Schiff, and positive to aniline blue. The interterritorial matrix in the hypercellular areas was weakly positive to both alcian blue and periodic acid-Schiff. Ultrastructural examination of these areas revealed many irregularly oriented large collagen fibrils and variable amounts of proteoglycan granules. These results suggest that in the fibrillar areas there are: (1) a high proteoglycan content, (2) a decrease in structural glycoproteins, and (3) a different size of collagen fibrils from that of normal epiphyseal cartilage. The hypercellular areas had a decrease in proteoglycans, glycoproteins, and collagen. The lateral physeal margin was often irregular, with a marked reduction of collagen and proteoglycan granules, and contained numerous large lipid inclusions. CLINICAL RELEVANCE: The abnormal areas in the epiphyseal cartilage of patients with Legg-Calvé-Perthes disease have different histochemical and structural properties from normal cartilage and from fibrocartilage. This suggests that the disease could be a localized expression of a generalized, transient disorder of epiphyseal cartilage that is responsible for delayed skeletal maturation. The cartilage lesions are similar to those seen in the vertebral plates in patients with juvenile kyphosis. Whether the epiphyseal cartilage abnormalities are primary or are secondary to ischemia remains uncertain; however, it appears that the collapse and necrosis of the femoral head could result from the breakdown and disorganization of the matrix of the epiphyseal cartilage, followed by abnormal ossification.

Neonatal surgery in clubfoot.

Neonatal surgery for clubfoot is conducted according to the following rules: One must work as close to the lesions as possible. It is important to avoid going beyond abnormal tissues. Dissection should be precise and economical. Opening of Lisfranc's joint or sectioning of the medial ligament must be avoided. One must operate with maximal accuracy. All areas of fibrosis must be excised, but care must be taken not to enter the interarticular space. If a bone deformity is present, one must accept an incomplete correction. One must act as soon as possible. This is the inverse of classic management in which orthopedic surgery was postponed. We prefer early surgery followed by intensive postoperative rehabilitation.

Chronic recurrent multifocal osteomyelitis (C.R.M.O.). A case report and review of literature.

The case of an 8-year-old boy presenting two episodes of osteomyelitis of the left and right tibial diaphysis is reported. The recurrent course, the negative cultures and the symmetrical aspect of the lesions suggest the diagnosis of recurrent multifocal osteomyelitis. The location of the lesions at the middle of the diaphysis and the histological finding of predominant bone necrosis are unusual.

[Scoliotic malformations with or without associated occult spinal dysraphia. A study in 82 children. Current radiological techniques, indications and findings]. / Scolioses malformatives, avec ou sans dysraphie spinale occulte. A propos de 82 observations de l'enfant. Techniques radiologiques actuelles, séméiologie et indications.

The possible association of spinal dysraphia with scoliotic malformations was studied in 82 children. Scoliotic malformations are not always the result of dysraphic malformations (diastematomyelia, hydromyelia, intrathecal lipoma, medullary cone ectopia). Recognition of dysraphic scoliotic malformations from among other types of scoliotic malformations can be accomplished before myelography by detecting vertebral malformations on standard films and, if necessary, by computed tomography. Segmental vertebral malformations, affecting vertebral bodies only, do not appear to arise from malformations of nerves. On the contrary, however, dysraphic lesions are always associated with vertebral neural malformations. Of currently employed techniques, only myelography with metrizamide, followed by computed tomography, gives the maximum amount of data concerning the lesions. Even the association of these two techniques does not demonstrate the presence of certain intrathecal fibrous lesions, due to lack of resolution.

[Is treatment of C.D.H. by progressive reduction by traction still advisable? (author's transl)]. / Que reste-t-il de l'extension continue dans le traitement de la luxation congénitale de hanche.

The authors analyse the results that they have obtained in the treatment of C.D.H. either by conservative or surgical methods. The prevention of osteochondritis remains the main worry. However traction leads to long-lasting immobilization and sometimes it is not sufficient. The authors think that osteochondritis may be related to hyper-pressure on the femoral head or to lesions of the vascular pedicle and that in some cases shortening of the femur or tenotomies may allow a decrease of the plaster cast period. The indications on capculotomy, rotation osteotomy, salter osteotomy are discussed. It is concluded that the periods of traction and plaster cast immobilization should be lessened as much as possible.

[Technics and strategy in the treatment of severe paralytic scoliosis]. / Techniques et stratégie dans le traitement de la scoliose paralytique grave.

Sixty-three paralytic scolioses with severe disability have been treated between 1973 and 1982. Fifty-two were due to poliomyelitis. The average angular deformity was 95.8 degrees and was related to delay in treatment and rapid progress of the curve. All cases were operated on. Spine fusion was performed either by the anterior approach (Dwyer or V.D.S.) or by a posterior approach (Harrington or Harrington - Luque). The pre-operative management was based on Cotrel or Halo traction. There were several complications - three non-unions, three neurological impairments, and nine cases of sepsis. It was concluded that the pre-operative management should be as short as possible, that the spine fusions should be done systematically and that any paralytic scoliosis increasing by more than 10 degrees a year between the ages of 8 and 10 years should be operated on early. This type of treatment should lower the incidence of severe disability and allow good functional and educational rehabilitation. The degree of diminished growth of the spine was moderate.

[Surgical treatment of dislocated hips after 5 years of age]. / Le traitement chirurgical de la hanche luxée après l'âge de 5 ans.

Fifty dislocated hips after the age of 5 were treated between 1975 and 1980. The surgical procedures performed combined tenotomy of the iliopsoas and adductor muscles, shortening of the femur, capsulorraphy and a pelvic osteotomy usually for re-orientation (Salter procedure) and rarely for widening of the acetabulum (Chiari procedure). It is thought that tenotomies and femoral shortening were more efficient than pre-operative traction which produced osteoporosis. However post-operative traction was used. In some cases plaster cast immobilisation was not used. The advantages of this technique were an acceleration of the surgical programme, better articular stability and early mobilisation. Osteochondritis and post operative stiffness were rare. The average range of movement included 90 degrees of flexion and lumbar balance was the most significantly improved factor. This type of surgical procedure aims to slow the development of coxarthrosis but it cannot diminish the risk. The limit of age for such treatment appears to be 10 years. After that, the indications need to be assessed carefully.

[Orthopedic reduction of scoliosis by Maguelone's technic. Indications and first results]. / Réduction orthopédique des scolioses par la technique de "Maguelone". Indications et premiers résultats

The authors describe an original technique for rapid closed reduction of scoliosis (Maguelone technique). It is based on mild distraction in suspension associated with derotation without pressure points. It is painless and efficient. In 50 cases it was followed by a conservative treatment using plaster casts. In 15 other cases it was the first stage of a surgical treatment of the deformity.

[Epiphyseal vascularization during growth. Study preliminary to transplantation]. / Vascularisation épiphysaire pendant la croissance. Etude préliminaire à la transplantation.

A study was conducted of 50 cadavers in children and 25 immature dogs to discover more precisely the vascularization of the lower end of the femur and the iliac crest. It was concluded that it is possible to transplant these epiphyses with anastomosis of three pedicles in the same line. It was also concluded that the vascularisation differs before and after the formation of the bony epiphyseal nucleus. The cartilaginous canals which preclude the vascularisation of hyaline cartilage, the formation of the bony nucleus and the nutrition of the germinal layers of the growth plate are described.