A case of Larsen syndrome with severe cervical malformations.

The authors report Larsen Syndrome in a male newborn with severe cervical spine malformations: segmentation abnormalities of the cervical spine and atlanto-axial dislocation. The severity of the cervical malformations occurring more often in the autosomal recessive form is emphasized.

[A case of exostosis of the base of the acetabulum in a child with multiple exostoses]. / Un cas d'exostose du fond du cotyle chez un enfant présentant une maladie exostosante multiple.

The authors report a case of an exostosis of the base of the acetabulum seen at the age of four months in a routine radiograph of the pelvis in a child suffering from osteochondromatosis. The growth in size of this exostosis led to subluxation of the hip at the age of three years. Surgical removal resulted in reduction of the subluxation and restoration of joint stability three years after operation. An intra-acetabular site for an exostosis has rarely been described.

[Chronic instability of the proximal tibio-fibular articulation: hemi-long biceps ligamentoplasty by the Weinert and Giachino technique. Apropos of 3 cases]. / Instabilité chronique de l'articulation tibio-fibulaire proximale: ligamento-plastie à l'hémi-long biceps selon Weinert et Giachino. A propos de 3 cas.

PURPOSE OF THE STUDY: Recurrent dislocations of the superior tibiofibular joint are rare. We report three cases of Weinert and Giachino ligament reconstruction. MATERIALS: From 1989 to 1994, two soccer players and one young girl (10 years old) presented recurrent dislocation of the superior TFJ. The mechanism was a direct trauma, and the type of dislocation was antero-lateral. These patients were free of tibial fracture, major ligament lesion of the knee, and neurologic symptoms (peroneal nerve injury). Pain was over the fibular head, mechanical and increased by jumping. A lateral "cracking" was present. Examination showed an antero-posterior mobility of the fibular head. X-ray showed horizontal proximal tibiofibular joint. M.R.I. eliminated a lateral meniscal pathology and collateral ligament injury. METHODS: The biceps tendon was split longitudinally for approximately seven centimeters. The posterior one half was transected proximally and mobilized. Secondly, the free end of the graft was passed posterior to anterior through a tibial tunnel. Thirdly, the tendon was sutured to the anterior tibial periosteum under tension with the fibular head reduced. RESULTS: Follow up at one year revealed that all the patients were active, free of dysfunction and laxity, with no further episodes of instability and no loss of ankle mobility. DISCUSSION: The diversity of treatments reported for this rare dislocation led us to believe that none proved its superiority. Resection of the proximal end of the fibula involves extensive dissection and creates a risk for the peroneal nerve vacuum and a lateral laxity. Arthrodesis of the superior tibiofibular joint without fibular osteotomy leads ankle pain, instability and a loss of ankle mobility. This procedure may also be complicated by screw loosening or breakage or by stress fracture of the fibula at the screw site. Arthrodesis of the superior tibiofibular joint with fibular osteotomy avoids these risks, but is possible for pain in the diaphyseal resection during the first 6 post operative months. Weinert and Giachino procedure, using a portion of the biceps tendon to reconstruct the superior tibiofibular ligament successfully stabilizes the superior tibiofibular joint, preserves all physiological function of the fibula and avoids resection or arthrodesis complications. CONCLUSION: Based on our experience with these three patients and on literature review, ligament reconstruction for recurrent dislocation of the superior tibiofibular joint gives stability and indolence, and avoids complications of resection and arthrodesis.

[Percutaneous in situ pin fixation in superior femoral epiphysiolysis]. / Fixation in situ par embrochage percutané dans l'épiphysiolyse fémorale supérieure.

PURPOSE: The purpose of this study was double. We evaluated long term results in 30 patients who had in situ pinning fixation. We tried to assess the advantage of pinning versus screw fixation. MATERIAL AND METHODS: We reviewed retrospectively 30 cases treated for SCFE by in situ pinning fixation. Between august 1985 and may 1995, 33 hips were treated (3 bilateral cases). The age averaged 10 years and 3 months. Twenty-one cases were chronic (64 per cent), 8 cases were acute forms (21 per cent) and 4 acute cases revealed progressive cases (14 per cent). There were 19 boys and 11 girls. The osteosynthesis was made with 3 pins (except in 4 cases with only 2 pins). RESULTS: The follow-up ranged from 20 to 50 months (average 35 months). The upper femoral growth plate was closed at an average of 16 months after surgery (6 months to 21 months). Three prophylactic controlateral fixations were performed before our protocola was established. No pin penetration was noted. No chondrolysis or avascular necrosis was noted. Complications were: 2 superficial hematomas with spontaneous resorption, 1 lateral pin migration without consequence on fixation, 1 break-pin in soft tissue without clinical consequence. The main complication was, in one case, a secondary slipping of the epiphysis after premature pin removal. Hip function was normal except in 2 cases with loss of motion in internal rotation. DISCUSSION: Stiffness of the screw is the most important advantage. A single screw seems to be enough. Removal of the screw is difficult, sometimes impossible. CONCLUSION: Percutaneous in situ pinning in SCFE is recommended. It is an easy surgical technique with an absence of complication such as chondrolysis or avascular necrosis in our experience. It is also the easiest method for hardware removal.

[Stiff painful spine in an 11-year-old girl with SAPHO syndrome]. / Rachis raide et douloureux chez une enfant de 11 ans et syndrome SAPHO.

Compression of the cervical spinal cord developed six months after the first signs of a stiff and painful spine in an 11-year-old girl. The clinical course, the association with other skeletal localization, particularly the sacroiliac joint and the roof of the acetabulum and the pathology findings of nonspecific inflammation led to the diagnosis of chronic recurrent multifocal osteomyelitis that can be included in a SAPHO syndrome (synovitis-acne-pustulosis- hyperostosis-osteitis). We report the specific findings in our case and review the literature to define the nosology framework of this rather uncommon condition. To our knowledge, this is the first report of spinal cord injury in a child related to SAPHO syndrome.

[Skin lesions and joint involvement in purpura fulminans (author's transl)]. / Séquelles cutanées et ostéoarticulaires majeures du purpura fulminans. Quatre observations.

Until recently, purpura fulminans had a very prognosis. It can now be treated and the patient may survive but extensive skin necrosis may lead to septic arthritis. The authors have observed 4 cases of this disease. 3 had to suffer amputation of a lower limb. They describe the type of care recommended in order to limit the extent of the necrosis.

[Initial degeneration of the articular cartilage in the rat]. / Dégénérescence initiale du cartilage articulaire chez le rat.

The authors have investigated the process of degeneration of articular cartilage in its early stages using an experimental model which has made it possible to show, for the first time, the effects of a mechanical parameter other than excessive pressure--that is excessive friction. The two healthy hips in twenty anaesthetised young rats were used for the study. One hip was kept immobile and served as a control. The other was subjected to physiological movements of flexion and extension continuously and without rest for periods varying from twelve hours to seven days. The articular cartilage of the two femoral heads was examined by light microscopy, transmission electron microscopy and scanning electron microscopy. A new fact was demonstrated. Cartilage wear is also associated with the simple movement of two joint surfaces over one another without any muscle contraction being involved. In the genesis of cartilage wear, excessive friction alone seems to be as important as excessive pressure.

[The treatment of upper femoral unicameral bone cysts in children by Ender's nailing technique]. / Indication inhabituelle de l'enclouage de Ender: le kyste osseux fémoral de l'enfant.

The authors have operated on 2 children aged 7 and 8 years who had developed a large unicameral bone cyst in the trochanteric and upper femoral diaphysial region. Ender's nails were inserted into the bone above the lower femoral epiphysial plate. Healing occurred rapidly.

[Posttraumatic nonunion of the clavicle in a child: case report]. / Pseudarthrose d'une fracture de clavicule chez l'enfant.

A thirteen-year-old child underwent surgery for posttraumatic nonunion of the clavicle. This complication, which is uncommon in adults and exceptional in children, occurred after three fractures of the left clavicle. The diagnosis can be retained after ruling out congenital nonunion of the clavicle. Surgery can be proposed if functional impairment persists for at least one year. For children, the preferred technique is osteosynthesis with centromedullary pinning and bone graft. While surveillance may not be necessary for a unique fracture of the clavicle, prolonged surveillance appears to be necessary in patients who sustain repeated fractures of the clavicle.

[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.

Weinert and Giachino ligament arthroplasty for the surgical treatment of chronic superior tibiofibular joint instability.

Recurrent dislocations of the superior tibiofibular joint are rare. The majority of these patients are physically active, young adults. Different surgical techniques have been described for the management of this pathology: resection of the proximal aspect of the fibula, arthrodesis of the superior tibiofibular joint with or without fibular osteotomy. Because of the problems reported with these techniques, we opted for Weinert and Giachino ligament reconstruction. From 1989 to 1994, three patients were treated and reviewed in our practice: all, soccer players on the local team, 20, 23, and 25 years old, with superior tibiofibular pain and tumefaction, without neurological symptoms, but with anteroposterior mobility of the fibular head. The average duration of the symptoms before operation was 9 months, and the average follow-up was 15 months. At the follow-up, pain and anteroposterior mobility were gone. Soccer playing was resumed at 7 months without recurrence but with low frequency and stress. In our opinion, the Weinert and Giachino ligament reconstruction, using a portion of the biceps tendon to reconstruct the superior tibiofibular ligament, appears an effective approach to restore indolence and stability to the superior tibiofibular joint and to avoid complications of resection and arthrodesis.

[Essential deformans osteolysis. Report of a case with progressive and generalized evolution (author's transl)]. / Ostéolyse progressive généralisée déformante. A propos d'une observation.

The authors report a clinical and radiographic course of a 16 years old caucasian girl with a progressive deformation of her skeleton. The primary lesions occur in knees and wrists. This disease was at first considered as a rheumatoid arthritis, then as a dysplasia epiphysialis multiplex, and now as essential deformans osteolysis (with carpal lysis, shortening of the forearm bones, dislocation of the elbows, disparition of the humeral and femoral heads, contracture of hips and knees, posterior tarsal lysis, and kypho-scoliosis). The clinical (particularly ophtalmologic), biological (including inflammatory, phospho-calcic and nephrologic evaluation with mucopolysaccharidosis urinary excretion) and histological (skin, muscle and bone) check-up were normal. They review the different classification established on lesion topography, on association or not with a nephropathy, finally on an dominant or recessive autosomal inheritance. Then the authors think that their case is similar to the ones of Winchester and Hollister. They discuss the etiopathogenic factors, and do not consider their case as a new mucopolysaccharidosis, but rather as a generalized disease of the collagen of bones.

[Serious pelvic traumatisms in children]. / Traumatismes pelviens graves de l'enfant]

The rareness of pelvic fractures in children is due to the resilience of bony tissue and to the thickness of the periosteum at this age. Therefore, such fractures are most often seen after violent traumatisms, which explains their frequent association with visceral lesions that determine the gravity and dominate the prognosis. The authors have presented 9 observations of fractures of the pelvis, with or without pelvis dislocation, which were the most often associated with urological lesions either acute (urethral rupture, bladder rupture) neither chronic (hydronephrosis) but sometimes with vasculo-nervous, gynecological or others lesions. The different therapeutic techniques and their indications in the treatment of such fractures are similar to those used in the adult. Excepting those cases of polytraumatisms, where mortality remains elevated, the treatment of such fractures is most often satisfactory in the short term, but requires a thorough and prolonged monitoring in children presenting associated urological and gynecological lesions in order to evaluate with precision the long term prognosis.

[Surgical management of pancreatic pseudocysts in children (author's transl)]. / Modalités du traitement chirurgical des pseudokystes pancréatiques de l'enfant.

The authors report eight new cases of pancreatic pseudocyst in children. Three principal operative procedures are generally performed with variable surgical indications. So, they emphasize the need of a precise pre and intra-operative evaluation of the pseudocyst. Thus, the internal drainage-cystogastrostomy or Roux-en-Y cystojejunostomy-appears to be the method of choice; however, external drainage and distal pancreatectomy have to be performed in some particular instances.