Open synovectomy of the ankle joint in young haemophiliacs: mid-term to long- term results of a single-centre series of 32 procedures.

INTRODUCTION: In haemophiliacs, recurrent hemarthrosis and chronic synovitis lead to chronic arthropathy. Synovectomy is indicated when medical treatment fails. Few studies report the results of open synovectomy of the ankle in haemophiliacs with a small number of procedures and also a limited follow-up. AIM: The aim of this paper is to report the long-term results of open surgical synovectomy of the tibio-talar joint. METHODS: Thirty-two open synovectomies were performed in 21 young haemophiliacs in the same haemophilia center using an antero-lateral and postero-medial approaches. The median follow-up was 15.4 years. Clinical (Petrini scores) and radiological evaluations (Pettersson scores) were made preoperatively and at each multidisciplinary follow-up visit. Wilcoxon and Spearman's tests were used for the statistical analysis. RESULTS: Preoperative median Petrini score was 6 (range 3-12), and improved at 2 and 5 years follow-up (P = 0.0003 and P = 0.0001 respectively). At 10 and 15 years follow-ups, median score remained below preoperative score (median 3.5, range 0-11). Ten ankles had a follow-up of more than 20 years. Preoperative median Petterson score presented a slight but continuous worsening in the first 2 and 5 years of follow-ups (P = 0.02, P = 0.003), but not correlation between clinical and radiological results was observed. CONCLUSION: Our long-term results support that clinical scores are improved even if radiological scores progress. Open synovectomy retards the progression of the arthropathy, but not stops it. Bleeding and pain are controlled and even if recurrence of bleedings is frequent, it is less severe, less painful and requiring less factors replacement.

Spontaneous obturator internus muscle haematoma: a new unpublished cause of ilioipelvic pain in haemophilia.

Obturator muscles haematoma are rarely reported. The most often reported cases are primary pyomyositis or posttraumatic haematomas occurring during pelvic fractures. We firstly report herein two cases of spontaneous obturator internus haematoma (OIH) in two haemophiliacs with inhibitor. Clinical data and imaging of two patients treated in our clinic are reported here according to previously defined criteria of OIH in posttraumatic situation. Both patients were children suffering from severe and moderate haemophilia A, respectively, with an inhibitor at the time of the event. The clinical feature was marked by an iliopelvic pain letting discussing hip haemarthrosis, appendicitis or iliopsoas haematoma. For both patients ultrasonography (US) failed to provide the diagnosis. Careful and repeated clinical examinations eventually lead to suspect obturator haematoma which was confirmed by abdominopelvic computed tomography (CT) and magnetic resonance imaging (MRI). Respectively, high dose of FVIII or rFVIIa regimen allowed a rapid control of the muscular bleeding in the low and high responder inhibitor patients. Spontaneous OIH may be added to the differential diagnosis of iliopelvic pain in severe forms of haemophilia. US still often performed at first in such case remains unhelpful; abdominopelvic CT or MRI should be performed to discriminate among different diagnoses, including OIH which stays probably undiagnosed.

Surgical treatment of a 180° thoracolumbar fixed kyphosis in a young achondroplastic patient: a one-stage "in situ" combined fusion and spinal cord translocation.

An achondroplastic patient with a thoracolumbar kyphosis was first seen at the age of 16 at our institution. His only concern at that time was the aesthetic implication of his deformity. His physical examination was normal except for loss of the neurologic reflexes in the lower limbs. The radiographs showed a fixed 180° thoracolumbar kyphosis with correct frontal and sagittal balances. No spinal cord anomaly was found on MRI. Two years later, he developed a progressive neurogenic claudication of the lower limbs. He was still neurologically intact at rest. The MRI showed an abnormal central spinal cord signal in front of the apex of the kyphosis associated with the narrow congenital spinal canal. In regards to this progressive neurological worsening, a surgical treatment was decided. We decided to perform a front and back arthrodesis combined with a spinal cord decompression without reduction of the deformity. A five-level hemilaminotomy was performed with a posterior approach at the kyphosis deformity. The spinal cord was individualised onto 10 cm and the left nerve roots were isolated. A decancellation osteotomy of the three apex vertebrae and a disc excision were performed. The posterior aspect of the vertebral body was then translated forward 2 cm and in association with the spinal cord. Two nerve roots were severed laterally to approach the anterior part of the kyphosis and a peroneal strut graft was inlayed anterolaterally. A complementary anterior and a right posterolateral fusion was made with cancellous bone. The patient was immobilised in a cast for 3 months relayed by a thoracolumbosacral orthosis for 6 months. At 3 years follow-up, the neurogenic claudication had disappeared. No worsening of the kyphosis was observed. His only complaint is violent electric shock in the lower limbs with any external sudden pressure on the spinal cord in the area uncovered by bone.

Anterior and lateral overcoverage after triple pelvic osteotomy in childhood for developmental dislocation of the hip with acetabular dysplasia: Frequency, features, and medium-term clinical impact.

BACKGROUND: Triple pelvic osteotomy (TPO) is a treatment option in children and adolescents with residual acetabular dysplasia after developmental dislocation of the hip (DDH). However, TPO to redirect the acetabulum is often blamed for anterior and lateral overcoverage of the femoral head. The main objectives of this study were to assess the potential clinical impact, frequency, and radiological features of acetabular overcorrection. Evidence of post-operative remodelling and associations linking younger age at surgery and/or dysplasia severity to the existence and magnitude of overcorrection were sought. HYPOTHESIS: Acetabular overcorrection has little or no clinical impact. PATIENTS AND METHODS: TPO was performed on 41 hips in 31 patients at a mean age of 6.3 years (range, 3.0-15.2 years). Mean follow-up was 13.8 years (range, 5.4-28.7 years) and mean age at last re-evaluation was 22.1 years (range, 13-39 years). Clinical outcomes were assessed based on the Harris Hip Score (HHS) and Postel-Merle d'Aubigné (PMA) score. Radiographs were used to look for a cross-over sign (CO+) and to measure the vertical-centre edge (VCE) and vertical-centre anterior (VCA) angles and the acetabular index (AI). Overcorrection was defined as AI≤0° and/or VCE≥35° and/or VCA≥40° and/or CO+. RESULTS: The HHS and PMA score values were good or excellent for 39 (94%) hips. One or more parameters indicated overcorrection of 33 (80.5%) hips. No significant differences were found between the overcorrected hips and the hips with normal parameters. DISCUSSION: TPO effectively corrects residual acetabular dysplasia. Overcorrection is common in all three planes but has little clinical impact in young adults. The high frequency of overcorrection mandates a careful pre- and intra-operative evaluation of acetabular version. LEVEL OF EVIDENCE: IV, retrospective study.

[Fibrous dysplasia of the proximal femur in children and teenagers: surgical results in 22 cases]. / La dysplasie fibreuse de l'extrémité proximale du fémur chez l'enfant et l'adolescent: résultats du traitement chirurgical dans 22 cas.

PURPOSE OF THE STUDY: Fibrous dysplasia is a rare benign bone tumor which occurs preferentially in the proximal femur. In children, there is a risk of repeated fractures and coxa vara deformity, particularly in the polyostotic form. The most common orthopedic problem is fibrous dysplasia of the proximal femur which generally requires surgical treatment. The purpose of this study was to analyze clinical and radiological outcome after surgical treatment. MATERIAL AND METHODS: All children who underwent surgical treatment for fibrous dysplasia of the proximal femur between 1979 and 2001 were reviewed retrospectively. The study cohort included 22 children (11 boys and 11 girls). Eight patients had a monostotic form and 14 a polyostotic form of the disease. For the monostotic forms, the type of treatment depended on the size of the tumor and its localization but curettage was used in all cases. For the polyostotic forms, treatment consisted in valgus osteotomy with "humeralization" in the event of associated coxa vara in combination with internal fixation, generally with a centromedullary nail. RESULTS: In the monostotic forms, the clinical outcome was considered good in all cases. Nearly total involution of the tumor was noted in 75% of patients. In the polyostotic forms, osteotomy with "humeralization" and centromedullary nailing provided stable correction of the deformation. Outcome was less satisfactory because of fractures and deformities. DISCUSSION: In light of our results and those reported in the literature, the prognosis of the monostotic form can be considered good after surgical treatment. For the polyostotic form, preventive fixation is necessary. Osteotomy with "humeralization" appears to correct the deformity and prevent coxa vara in certain cases. For the more severe forms, medical treatment with biphosphonates may be a useful complement to the surgical treatment.

Anterior screw-plate fixation in adolescent idiopathic scoliosis: 15-year outcomes.

BACKGROUND: Few published data are available on long-term outcomes of anterior spinal fusion for adolescent idiopathic scoliosis (AIS). The objective of this single-centre retrospective study was to assess clinical and radiological outcomes of one-stage anterior spinal fusion achieved using precontoured titanium anterior screw-plates. HYPOTHESIS: Our hypothesis was that anterior instrumentation produced both good functional outcomes and good correction in the coronal and sagittal planes. MATERIAL AND METHODS: This procedure was performed in 111 patients between 1975 and 1993. Among them, those who underwent a comprehensive evaluation at least 15 years later were included. The SRS-30 questionnaire and Oswestry Disability Index (ODI) were used to assess functional outcomes. Radiographic outcomes were evaluated on antero-posterior and lateral full-spine radiographs obtained pre-operatively, post-operatively, and at last follow-up. RESULTS: The study included 35 patients, who were re-evaluated after a mean of 21 years (15-31 years). Mean pre-operative Cobb's angle was 44°, mean age at surgery was 14.7 years, mean SRS-30 score was 3.65/5, and mean ODI was 14.9%. At last follow-up, mean Cobb's angle was 14.7° and 25 patients exhibited coronal misalignment with a mean deviation of 12mm. In the sagittal plane, the mean sagittal vertical axis (SVA) measured using the C7 plumb line was -28mm, with 8mm of anterior translation compared to the post-operative value (36mm). The functional outcome assessed using the SRS-30 score correlated significantly with pelvic tilt and anterior SVA translation. CONCLUSION: Anterior spinal fusion produces good long-term functional outcomes in AIS. Correction is both satisfactory and sustained. Anterior SVA translation over time may be associated with better functional outcomes. LEVEL OF EVIDENCE: IV (retrospective study).

[Avulsion fracture of the tibial tubercle in adolescents: 22 cases and review of the literature]. / Fracture-avulsion de la tubérosité tibiale antérieure chez l'adolescent: à propos de 22 cas et revue de la littérature.

PURPOSE OF THE STUDY: Fracture of the anterior tibial tubercle is exceptional in adolescents. The purpose of this work was to better understand the circumstances of such fractures, identify potential complications, and assess outcome of different therapeutic options. MATERIAL AND METHOD: This was a retrospective analysis of a consecutive series of 22 children with avulsion fracture of the anterior tibial tubercle. The circumstances of the fracture were noted. The Ogden classification was used to establish the radiological type. Associated lesions were also noted as were any complications. Two types of treatment were given: orthopedic or surgical (several types). The functional outcome was assessed at last follow-up. RESULTS: Mean age at fracture of the anterior tibial tubercle was 13 years (range 12-16) in 14 boys and 7 girls. It occurred after a jump or reception after a jump in 22 of the children. Five patients presented symptomatic homo- or contralateral anterior tibial apophysitis before the accident. The fracture was not displaced (type IA) in ten patients and was treated orthopedically. In twelve patients, the fracture was displaced (type IB, II, or III) and required osteosynthesis. Immobilization was maintained for four weeks on average (range 3-7). Associated lesions were observed in half of the twelve displaced fractures, with five cases of patellar ligament avulsion and three cases of meniscal injury. There were no complications with type IA fractures. For the twelve displaced fractures, complications were observed in seven children: hematoma (n = 2), infrapatellar hypoesthesia (n = 2), complete rupture of the patellar ligament 38 months after surgery (n = 1), recurvatum with leg length discrepancy (n = 1), and stiff knee (n = 1). Mean follow-up was two years (range 9 months-8 years). The functional outcome was excellent in all patients with a non-displaced fracture and in seven of the twelve patients who underwent surgical treatment. DISCUSSION: Non-displaced fracture of the anterior tibial tubercle I children (type IA) should be considered as a separate entity responding very well to orthopedic treatment. The other cases of displaced fracture generally require surgery which enables an assessment of often associated lesions. The prognosis of displaced fractures of the anterior tibial tubercle is more sever due to the associated lesions and potential complications. We describe the second case of recurvatum and leg length discrepancy following fracture of the anterior tibial tubercle. CONCLUSION: Displaced fracture of the anterior tibial tubercle in adolescents is often associated with soft tissue injury (patellar ligament, menisci). Surgery is indispensable and provides good functional results.

Sternal cleft: case report and review of a series of nine patients.

Cleft sternum is a rare malformation due to partial or total failure of sternal fusion at an early stage of embryonic development. Sternal clefts can be classified as superior, inferior, or complete. Here we report on a 2-year-old boy with inferior sternal cleft and complex cardiac malformation. We review a series of 9 children with sternal clefts, referred to us over a 10-year period. Hypothetical mechanisms for this developmental anomaly are discussed.

Upper limb malformations in DiGeorge syndrome.

We report on upper limb anomalies in two children with a complete DiGeorge sequence: conotruncal defects, hypocalcemia, thymic aplasia, and facial anomalies. One child had preaxial polydactyly, and the other had club hands with hypoplastic first metacarpal. In both patients, molecular analysis documented a 22q11 deletion. To our knowledge, limb anomalies have rarely been reported in DiGeorge syndrome, and they illustrate the variable clinical expression of chromosome 22q11 deletions.

Osteochondritis dissecans of the humeral capitellum in children.

We studied 13 cases of osteochondritis dissecans of capitellum humeri in 12 children, 11 boys and 1 girl, aged between 10 and 15 years. We distinguished this affection from Panner's disease which affects young children and resembles Legg-Perthes-Calvé disease of the hip. Operative treatment was performed in 7 out of 13 elbows for removal of loose bodies or excision of osteochondritis in situ with cartilage damage. In other cases, functional treatment was carried out. At long-term follow-up, ranging from 2 to 13 years, clinical examination demonstrated satisfactory results in 9 cases; in 3 cases limitation of movement was related to fracture of the radial head or to delay in operative treatment for too long. Roentgenographically, changes related to growth disturbance were constantly observed; they involved the radial head, the olecranon, the trochlea and the proximal end of the ulna.

Desepiphysiodesis--elimination of partial premature epiphyseal closure. Experience of 17 cases.

Between 1975 and 1990, 17 growth plates have been operated on by epiphyseal bridge resection. The children were from 4 years and 10 months to 13 years and 10 months old. The etiology of partial closure was traumatic (10 times), caused by therapeutic mistakes (3 times), septic osteomyelitis (1 case), purpura fulminans (1 case), unknown (2 cases). There was always length discrepancy or deformity of bone. The regions that have been subjected to treatment were distal femur, proximal tibia, distal tibia, distal radius. Evaluation of the bone bridge was made by tomoscintigraphies and recently by MR imaging and computed tomoscintigraphy. The bone bridge size was from 2.5% to 60% of the growth plate surface; surgical technique consists of resection of bone bridge connecting epiphysis and metaphysis which is replaced by methyl metacrylate. In 16 cases simultaneous corrective osteotomy was performed. Results are poor, there were only two good results and 8 failures; seven results were medium. The failures can all be explained by mistakes in technique or indication, except one. Indications are post-traumatic narrow bridges in young children. It would be useful to know the vitality of the residual growth plate.

Evolution of scoliosis in six children treated with growth hormone.

During a period of 6 years to 11 years, the authors have been following six girls with scoliosis and treated with growth hormone (GH) for a growth insufficiency. The treatment with GH started after the discovery of the scoliosis for five patients. Three curve progressions have been observed, but always in the puberty period. Only one progression was noticed at the beginning of the GH treatment, but it was relieved with bracing. The results of this study do not permit one to conclude that a relation exists between GH treatment and scoliotic progression. This treatment is nevertheless not devoid of side effects, and a rigorous supervision is necessary.

Obstetric dislocation of the thoracic spine: case report and review of the literature.

A fracture dislocation of the upper thoracic spine with spinal cord injury is reported in a neonate. This rare injury is associated with attendant predisposing obstetric circumstances (breech transverse presentations, large baby size) that can alert clinicians of potential problems and aid in the diagnosis of neonatal hypotonia and paralysis.

[Solitary infantile myofibromatosis of bone]. / Myofibromatose infantile osseuse isolée.

We report one case of Solitary infantile Myofibromatosis of Bone in a 14-year old girl. Radiologic features of the tibial lesion were consistent with the diagnosis of a benign bone tumor. The final diagnosis was made on a total resection of the tumor. Histologically, the tumor consisted of nodules, hyalinized or cellular, with spindle-shaped cells resembling fibroblasts or smooth-muscle cells. There were numerous vascular spaces in close contact with these nodules. The tumor cells were immunoreactive with anti-alpha-smooth muscle actin antibodies. These data were consistent with the diagnosis of Myofibromatosis. The histologic features were similar to those of Infantile Myofibromatosis of other sites such as skin and soft tissue. The case reported is rare and not yet published, due to the age and sex of the patient, and the location of the tumor.

[Pelvic osteotomies: Salter, triple osteotomy, Chiari. Philosophy, technic, choice, indications]. / Ostéotomies pelviennes: Salter, triple ostéotomie, Chiari. Philosophie, technique, choix, indications.

Pelvic osteotomies were proposed in order to preserve as much as possible of the acetabular cartilage. The aims were twofold: centering of the head of the femur in the acetabulum, to improve its stability; correction of the delay in growth of the acetabulum: there is a potential correction, allowing for the femoral growth, or a passive correction by widening a deep acetabulum. Two types of osteotomy are proposed: reorientation osteotomies (Salter, triple osteotomy) which have both a mechanical and biological effect and widening osteotomies (Chiari), which are in fact extracapsular arthroplasties. All the techniques of reorientation osteotomy are very similar and achieve a reorientation of the acetabulum on the femoral head by varization, retroversion and, when possible, medialization. The Chiari osteotomy, which is technically difficult, should be performed according to the very precise rules described by Chiari.

[Pavlik's harness in congenital hip dislocation before the first birthday]. / Le harnais de Pavlik dans la luxation congénitale de hanche avant un an.

Three hundred cases of congenital dislocation of the hip have been treated with the Pavlik harness since 1979. The reduction is obtained by progressive flexion of the thighs. The femoral head penetrates the acetabulum from bottom to top. Placing the harness and managing the treatment must be very carefully done. The explanation given to the family is essential to success. Complications are rare. Arthrography followed by reduction in traction should be considered in case of failure. The Pavlik harness is indicated under the age of 9 months.

[Treatment of the dislocated hip in older children]. / Traitement de la hanche luxée chez le grand enfant.

Between 1976 and 1987, 85 cases of CDH occurring after walking age were followed in the department. Eighteen children aged 5 years or older were reviewed. In 11 children the dislocation was unilateral and in 7 bilateral; out of these 25 hips, 20 were high luxations and 5 occurred at a lower level. Two children, 6 and 13 years old, had a bilateral dislocation. Except for 2 children of 5 years of age who were treated conservatively, surgical treatment was indicated in the other 18 cases usually after tenotomy and derotation. In 13 hips, a surgical repositioning was performed, associated with femoral osteotomies, either shortening osteotomies (7) or derotation osteotomies (3). Pelvic osteotomies were indicated either immediately or later in 15 hips. A triple osteotomy was usually performed (11); a Salter (2) or a Chiari (2) has been rarely used. After a follow-up of 5 years, evaluation according to clinical and radiological criteria showed 14 good and 7 bad results. The latter 7 cases had either a dislocation or an eccentration. Reviewing these series, the authors describe the problems in the treatment of the dislocated hip in the older child: the indications, the therapeutic strategy for those dislocations and the decision for treatment of the bilateral high dislocation in the older child.

[Hemivertebra. -- classification, natural history and prognosis (author's transl)]. / Les hémi-vertèbres. Classification, historie naturelle et éléments de pronostic.

The authors have studied a series of seventy patients with hemivertebra. Classification should be based on two criteria--firstly, that the hemivertebra can be fused or separate and secondly, vertebrae above and below the hemivertebra may or may not show transitional abnormality. The natural history may take one of three courses: 1--Deformity may be severe from the beginning in cases of separate hemivertebra associated with transitional abnormalites. 2--The deformity may be stable until the age of nine or ten years, after which a severe scoliosis or kypho-scoliosis may develop. 3--The deformity may be stable throughout the whole of the growing period. The prognosis should be based on the existence of transitional abnormalities, the characteristics of the hemisvertebra (separate or fused), the sex of the patient, assoicated congenital defects, the rotation of the hemivertebra, and the level of the deformity. Treatment should be surgical in cases with early progression during the first few years of life by excision of the hemisvertebra associated with correction using Harrington rods. At the thoracic level, this surgical procedure is dangerous and spine fusion is more appropriate. The second period for surgical treatment is at puberty, either by spine fusion or correction and fusion. At this age, excision of the hemivertebra may still be indicated in the lumbar spine.

[Fractures of the talus in children. Apropos of 23 cases]. / Les fractures de l'astragale de l'enfant. A propos de 23 cas.

The authors have reviewed 23 fractures of the talus in children. They propose a classification based on the radiological maturity of the bone and the specific type of fracture. Group I: Children younger than 6 years (6 cases). The fracture line separates the bone into two parts. The diagnosis was difficult, with displacement being underestimated in some cases. Associated lesions in the same limb were present in 5 cases. Five fractures of the neck of the talus were treated conservatively with excellent or fair results. One comminuted fracture was treated surgically with a poor result. In all cases treatment should aim to maintain a good tibio-tarsal joint. Group II: Children older than 6 years (17 cases). Eight cases were osteochondral fractures, two were partial fractures and seven were complete fractures. Two of these developed subsequent avascular necrosis. The relationship between osteochondral fractures and osteochondritis is discussed.

[Congenital pseudarthrosis of the tibia and fibula in children. Results of the treatment of 18 cases with nails and bone grafts]. / Pseudarthroses congénitales du tibia et du péroné chez l'enfant. Résultats du traitement de 18 cas par enclouage et greffe.

Eighteen cases of congenital pseudarthrosis of the leg in 17 children were treated by nailing and tibio-fibular graft with a mean follow-up of six years (from one to twelve years). Six patients had reached the end of growth. Primary union was obtained in 17 cases in a mean time of 6.5 months (from 3.5 to 18 months), but one case developed a new pseudarthrosis at the level of an associated upper tibial osteotomy. Only one case required a supplementary graft. Walking was usually achieved without any apparatus after five-and-a-half months, the tibia being protected by a nail which was left in place until the end of growth. In two cases, a fracture produced by trauma occurred in the limb long after consolidation. These two fractures healed in the normal time. Inequality of limb length was present in all the cases, but its progress varied. On three occasions it was treated by femoral lengthening. Stiffness of the ankle and subtaloid joints resulted from nailing of the hindfoot in nine cases and improved very little after freeing of the joints. The use of telescopic rods avoids the need for further operations to change the rod during growth. We consider that this is a reliable method but that, in certain cases, the effects on growth and on the foot can be diminished by appropriate modifications of technique.