[Multiple pterygium syndrome in children. 7 cases]. / Le syndrome des pterygia multiples chez l'enfant. Etude de 7 cas.

Seven children have been observed and treated for a multiple pterygia syndrome. The characteristics of this affection which is rare and mostly transmitted as an autosomal recessive, are: a growth retardation, a facial dysmorphia, pterygia resulting in flexion deformities, and anomalies of hands, feet and external genitale. But the severity of the syndrome is linked with the spine malformation, whose fusions produce severe and precocious deformities. This syndrome must be differentiated from the popliteal pterygium syndrome, whose repercussions are only functional; from the arthrogryposis multiplex congenita, whose syndrome is already complete at birth; and from the false secondary pterygia (caudal spinal agenesis, campomelic syndrome, quadriceps hypoplasia). The multiple pterygia syndrome imposes a genetic investigation and a serious observation of the spine and of the evolutive flexion deformities.

[Scoliosis, spondylolysis and lumbosacral spondylolisthesis. A study of their association apropos of 82 cases in children and adolescents]. / Scoliose, spondylolyse et spondylolisthésis lombo-sacré: Etude de leur association chez l'enfant et l'adolescent à propos de 82 cas.

The authors have reviewed the charts of 82 patients who presented the association of a scoliosis and a spondylolisthesis. They insist upon the necessity to treat each abnormality for itself. 26 patients have been simply followed in the clinic, they required no treatment because of the modicity of the scoliosis and the spondylolisthesis. 23 patients were treated with orthosis because of the scoliosis progression. In this group the spondylolisthesis was not a major concern and remained stable. 15 patients had to be operated on because their scoliosis was threatening. The existence of a spondylolisthesis must not be a deterrent to the arthrodesis, and the orthotic treatment must not be carried on if inadequate. The risks to observe, below the spine fusion a progression of the slippage are extremely low (no case in our series). Although we tried to obtain a fusion of the lysis with an isthmic arthrodesis in two cases, we do not think that it represents a prerequisite to a spine fusion above the level of the lysis. 13 patients had a lumbosacral fusion for a great slippage spondylolisthesis. In this group the scoliosis had no relationship with the spondylolisthesis in four patients. But, in nine patients the scoliosis appeared to be directly related to the spondylolisthesis. The "Arthrodesis-reduction" of the spondylolisthesis enabled us to correct, at least partially, the scoliosis. At last 3 patients had a great slippage spondylolisthesis, and a threatening scoliosis. For these rare cases we propose the lumbosacral fusion with reduction of the spondylolisthesis and then, a few months later, an arthrodesis of the scoliosis as we have carried out twice successfully.

[Syringomyelia and scoliosis in children and adolescents. Apropos of 14 cases]. / Syringomyélie et scoliose chez l'enfant et l'adolescent. A propos de 14 cas.

The authors report their experience of 14 cases of scoliosis in children or young adults in association with syringomyelia. The neurological lesion was discovered at a variable time during the development of the scoliosis which may appear to be idiopathic for several years. There were 11 cases of syringomyelia or hydromyelia and three intramedullary tumours with cyst formation. The neurological lesion must be treated first and sufficiently early to avoid the development of muscle weakness and scoliosis. The scoliosis can be treated by normal without risk of additional complications.

[Surgical treatment of scoliosis of 100 degrees and greater in children and adolescents (neurological and myopathic scoliosis excluded). Apropos of a series of 66 cases]. / Traitement chirurgical des scolioses de 100 degr'es et plus chez l'enfant et l'adolescent (à l'exclusion des scolioses neurologiques et myopathiques). A propos d'une série de 66 cas.

In a retrospective study the authors had compared the results obtained in two series of identical scoliosis. All were examined after a two year minimal follow-up. Group A. There were 31 children operated on between 1966 and 1972. The average initial curve measured 111 degrees, and the average follow-up was eight years. In this group there was no preoperative preparation, and a Harrington rod was accompanied by cancellous grafting. The average correction was 34 degrees. Four paraplegias were seen, three of which recovered. Group B. There were 45 children operated on between 1973 and 1980. The average initial curve was 170 degrees and the average follow-up was 4.5 years. In this group, there was pre-operative use of a Halo cast routinely. In 14 children, Harrington rodding was supplemented by anterior fusion to correct associated kyphosis. The posterior Harrington rodding was completed by tibial grafting. The average correction was 47 degrees, without any neurological complications. It was noted that after one year, pulmonary function was not impaired to a greater extent after an anterior fusion than after a purely posterior fusion. Two-thirds of the cases gained considerable cosmetic benefit.

[Primary bone tumors of the spine in children. Apropos of 66 cases]. / Tumeurs osseuses primitives du rachis chez l'enfant. A propos de 66 cas.

Primary tumours of the spine in children are infrequent. The authors have studied 60 benign and 6 malignant tumours in 4 orthopaedic centres. These tumours have been diagnosed in 3 different ways - in the study of cord compression, as a finding in the course of a previously known disease, or as an isolated tumour. In most cases, the diagnosis could be made even before biopsy whose technique and indications are discussed. The treatment is based on the pathology but in all cases, it must avoid any loss of stability of the spine such as may occur after extensive resection.

[Primary synostosis of the tarsus in children. Apropos of 32 cases in 20 patients]. / Les synostoses essentielles du tarse chez l'enfant. A propos de 32 cas chez 20 patients.

The authors have studied 32 tarsal coalitions in 20 children. Twenty two were between the talus and the calcaneum, 4 were medial and 6 were rare varieties. The diagnosis is often missed because 40 p. 100 of the children with tarsal coalition had no complaints. The indirect radiological signs have been analysed and precise techniques described for detecting the coalition. Only 11 feet were operated on. On 4 occasions the coalition was simply resected. This always gave a good functional result and good mobility was obtained in the subtaloid joint in 2 cases. The other cases were treated by triple arthrodesis. The authors consider that an earlier diagnosis would lead to better prevention of secondary deformities. The best indication for simple resection is in coalition between the calcaneum and the navicular.

Osteoarticular abnormalities and orthopedic complications in children with chromosomal aberrations.

Chromosome abnormalities may result in a great number of osteo-articular anomalies. Little documentation concerning these anomalies is available. The main orthopedic problems encountered in 67 children with chromosomal abnormalities are reported here. Patients should be evaluated at regular intervals in order to detect osteoarticular involvement. Gradual deterioration results in disabling conditions which are difficult to manage. Orthopedic management should be mainly preventive. It's purpose is to delay or to avoid the onset of disabling complications.

[Surgical treatment of dorso lumbar scoliosis in a homocystinuric child (author's transl)]. / Une observation de scoliose dorso-lombaire grave opérée chez un enfant atteint d'homocystinurie.

The authors report an observation which is interesting in many points of view. 1. A homocystinuria was found in a 7 year old child. The diagnosis of this disease in the sister of the patient led to make a family schedule and permitted to connect to this homocystinuria the entirety of the pathological signs found in the patient, particularly a serious scoliosis. 2. Because of the rarity, the severity of the vital prognosis and the risk of thrombo-embolic complications of the homocystinuria any important surgical intervention was given up until the age of 13 years, but the aggravation of the scoliosis at this moment made an operation necessary. After an orthopaedic, dietetic and anti-aggregative preparation, a posterior vertebral arthrodesis was done without any incidence. 3. One year after this intervention, the orthopaedic as well as the medical evolution have been favourable, and there have been no accident to deplore, especially no thrombo-embolism.

[Limited osteochondritis complicating coxa valga in children (author's transl)]. / Ostéochondrite parcellaire sur coxa valga chez l'enfant. A propos de 8 observations.

Height cases of limited "osteochondritis" were observed in children showing some degree of coxa valga. The radiological appearances were different from those of Perthe's disease and resembled those of osteochondritis dissecans of the knee but with stability during their development. These "osteochondritis" were often bilateral; all the patients were male. In four cases some degree of platyspindyly was seen. It is thought that these hips are earlier and limited varieties of osteo-chondrodysplasia. The indications for varus osteotomy are discussed.

[Diastematomelia (author's transl)]. / La diastématomyelie (ou syndrome de la double chorde): etude de 21 cas.

21 cases have been observed and followed up for 10 years. All were children. Three types of anomaly were noted: Spinal curvature (mostly scoliosis), skin abnormalities in the mid line (hypertrichosis, angioma, skin dimples) and problems in the lower limbs (varus deformities of the feet, leg length discrepancy, muscular atrophy, neurological disorders). These signs, even if not all are present, must lead to a study of the vertebral bodies and to gas myelography whcih demonstrates a double spinal cord over a variable length from 2 to 14 segments. 15 children were treated surgically by mobilisation of the spinal cord and arthrodesis of the spine. 4 of them were made worse. In contrast, none of the 6 children treated conservately was made worse. The authors conclude that surgery should be reserved for recent spontaneous neurological deterioration and severe deformities of the spine.

[Spinal cord complications in the surgery of spinal deviations. Apropos of 24 cases]. / Les complications médullaires de la chirurgie des déviations rachidiennes. A propos de 24 observations.

The authors review 24 medullary complications which they encountered while treating scoliosis and kyphosis over a period of 8 years. They studied separatly the complications encountered in the pure kyphosis and those encountered in the scoliosis. 7 medullary complications occured while treating pure kyphosis, 5 Potts disease, 1 achondroplasia and 1 Morquio disease. All the kyphosis were of importance with a mean curve over 100 degrees. In 6 cases the medullary symptoms occurred during elongation prior any surgery. The authors distinguished between Pott disease where the symptoms occurred after minimal reductions and the chondrodysplasia where the symptoms occurred after important reductions. Thus the stiff Pott kyphosis where elongation results in medullary symptoms because of medullar compression before any correction, appears to be different from reductible kyphosis where the strong medullary tension is responsible for the symptoms. Two complications occurred after a vertebral osteotomy one immediatly and one after an elongation of a pelvic halo.

[Spine deformity in Marfan's syndrome in childhood (author's transl)]. / Les déviations rachidiennes de la maladie de Marfan chez l'enfant. Etude de 20 cas.

The authors have studied twenty cases of spine deformity in children suffering from Marfan's syndrome. They describe the main features of the disease and point out the difficulty of accurate diagnosis in children with minor involvement. The deformities are those of a dysplastic scoliosis as shown by the frequency of vertebral inversion on the lateral view: dorso-lumbar kyphosis, decreased lumbar lordosis. The treatment should be surgical because of the danger of a fatal outcome without treatment. The incidence of non-union is high and the authors recommend systematic revision of the fusion double curve scolioses with a moderate kyphosis. In cases of severe kyphosis, the addition of anterior grafting is recommended.

[Orthopedic problems in fibrous dysplasia in infant's bones. A report on 23 cases (author's transl)]. / Les problèmes orthopédiques de la dysplasie fibreuse des os chez l'enfant.

The authors report on 23 cases of fibrons in infants who had been treated surgically, and review the therapeutic problem which arise from this particular type of bone fragility. When located in the diaphysis they had been treated by screw fixation, isolated grafts being constantly unsuccessful. Various procedures had been used in 9 cases with localization in the proximal end of the femur, and they are reviewed critically.

Astragalectomy in children. Results, technics, and indications based on personal experience in 33 cases. / L'Astragalectomie chez l'enfant. Résultats, technique, indications d'aprés notre expérience de 33 cas

The authors have operated on 33 equinovarus feet, (14 patients) with various condition--myelomeningocele, arthrogryposis, club foot or dwarfism. The results were poor, only 11 being satisfactory. Some failures were due to incorrect tecunique. The authors describe the technique which they consider to be the best. This procedure appears to have its main indication in myelomeningocele and arthrogryposis when the feet are severely deformed and rigid.