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.

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).

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.

Children ankle valgus deformity treatment using a transphyseal medial malleolar screw.

INTRODUCTION: Ankle valgus deformity is rare in children. It generally leads to difficulties wearing shoes, walking instability and mechanical pain. No medical treatment is effective and the only option is surgical correction of the deformity. Two main options are available: supramalleolar osteotomy and medial malleolar epiphysiodesis. We report our experience with epiphysiodesis using a transphyseal medial malleolar screw. PATIENTS AND METHODS: This is a retrospective study of all children followed for ankle valgus and treated by transphyseal medial malleolar screw epiphysiodesis in our department. The study included 10 cases of ankle valgus deformity in seven children (four with multiple extostoses, two type 1 neurofibromatosis, one Larsen's syndrome) who completed skeletal maturity. At surgery, median bone age was 12 years (10 to 13 years and 6 months) and the median tibiotalar angle was 17.5° (10° to 30°). RESULTS: At skeletal maturity, preoperative valgus was corrected in six patients (9/10 ankles). The median tibiotalar angle was 5° (0° to 25°). Valgus was not corrected in one patient (30° to 25°). No postoperative complications occurred. DISCUSSION: Epiphysiodesis by transphyseal medial malleolar screw is a simple, efficient and safe procedure to correct a significant or symptomatic ankle valgus deformity in children before skeletal maturity. LEVEL OF EVIDENCE: Level IV, retrospective study.

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.

Surgical synovectomy of the knee in young haemophiliacs: long-term results of a monocentric series of 23 patients.

BACKGROUND: In haemophiliacs, synovectomy is indicated for recurrent joint bleedings, despite medical treatment. METHOD: We report a series of 23 surgical synovectomies of the knee with a median follow-up of 8.8 years. The median age of patients at surgery was 13.5 years. Clinical and radiological evaluations were made according to the Petrini and the Pettersson scores, at 1 and 5 years after surgery, and at the last follow-up. Wilcoxon and Spearman's tests were used for the statistical analysis. RESULT: The Petrini score improved at 1 and 5 years (P < 0.001). Nine patients have 20 years of follow-up and a stable result. In more than half of the knees, no episode of recurrent bleeding occurred. The effect of surgery on the range of motion (ROM) was moderate and mobilisation under anaesthesia did not improve it significantly. There was a progressive worsening of the radiological score, but no correlation between clinical and radiological score was noticed (ρ = 0.08, P = 0.77). CONCLUSION: Complete synovectomy gives good long-term results in term of bleeding recurrence and overall function.

[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.

[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.

[Posterior spinal fusion for kypho-scoliosis associated with osteogenesis imperfecta: long-term results]. / Résultats des arthrodèses vertébrales postérieures dans les scolioses secondaires à une ostéogenèse imparfaite.

PURPOSE OF THE STUDY: A survey was conducted at the Necker-Enfants-Malades Hospital in Paris and the Roscoff Helio-Marin Center in Roscoff, France to document results of posterior spinal fusion for scoliosis associated with osteogenesis imperfecta. MATERIAL AND METHODS: The cases of 27 patients who underwent posterior spinal fusion and Harrington (n = 2) or CD (n = 23) instrumentation performed by one senior orthopedic surgeon were reviewed. Two patients had posterior arthrodesis without instrumentation and were left in halo-traction together with a plaster cast until bone healing was achieved. RESULTS: The series included eleven males and sixteen females. Mean age at fusion was 13 y 4 m. The average curvature before surgery was 77 degrees and the average correction was 37 degrees. Mean follow-up was six years. Minor complications due to halo pin infection or migration were observed in two patients and loss of correction with rod fracture in four. DISCUSSION: Because of the risk of physical and respiratory incapacity, we believe that spinal surgery and arthrodesis for spinal deformity should be considered after a preparation period by halo-traction with exceptional precautions to correct major curves and prevent complications that could occur in patients with osteogenesis imperfecta. Correction and stabilization of the scoliotic spine in osteogenesis imperecta has yet to be definitively described since we have noticed that the site of deformation is transferred to the sacroiliac region once spinal fusion is achieved.

[Aneurysmal bone cyst of the spine in children: a 9-year follow-up of 7 cases and review of the literature]. / Kyste anévrysmal du rachis chez l'enfant: sept cas avec un recul moyen de 9 ans et revue de la littérature.

PURPOSE OF THE STUDY: Aneurysmal cyst is an uncommon benign primitive bone tumor generally observed in young subjects, in a spinal localization in 10% of the cases. We report the clinical, radiological, and therapeutic aspects of seven cases of aneurysmal cyst of the spine. MATERIAL AND METHODS: This series included seven children, mean age 11.7 years (range 8-14) who were followed for 9 years on the average (range 2-24 years). Findings were compared with reports in the literature. RESULTS: Four cysts were located in the cervical spine (C1, C3, C4, C7), two in the thoracic spine (T4, T7) and one in the sacrum. Biopsy-resection was performed in six patients. The sacral cyst was treated by selective arterial embolization. Two complications were observed after surgical treatment. Bony filling of the lesion, evaluated by CT scan six months after surgery, was achieved in five cases, incomplete in two without recurrence of cystic cavities. DISCUSSION AND CONCLUSION: Aneurysmal bone cysts account for 15% of all primitive tumors of the spine. They are more frequent in the cervical and thoracic spine than in a lumbar or sacral localization. One vertebra is involved in most cases, generally the vertebral body and the posterior arch with an asymmetrical extension to one pedicle in some cases. Neurological complications of variable severity are frequent and can be improved by surgical removal of the tumor. CT scan and MRI are the best exams to analyze the spinal cyst, to show bony walls and liquid-liquid levels suggestive of aneurysmal bone cyst, to evaluate the tumor extension and to search for cord compression. When the CT scan and MRI show characteristic signs of aneurysmal bone cyst of the spine, we do not perform a biopsy systematically if complete removal of the tumor appears possible. Surgical removal with biopsy is the treatment of choice. Reconstruction and stabilization of the spine must be complete using bone graft with or without instrumentation. In case of medullary involvement, decompression must be performed rapidly with the most complete tumor removal as possible. Selective arterial embolization can be used in some localizations or as preparation to surgery. Radiotherapy is contraindicated in children because of the risk of radio-induced tumors.

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.

[Congenital pseudarthrosis of the clavicle: 25 childhood cases]. / Pseudarthrose congénitale de la clavicule.

PURPOSE OF THE STUDY: Congenital pseudarthrosis of the clavicle is rare, only 200 cases having been reported. Based on 25 personal cases and an overview of the literature, we try to explain the etiology of this condition and the different kinds of treatment. MATERIAL AND METHODS: A retrospective analysis was performed on twenty-five children (16 females, 9 males, mean age at the end of the follow up - 11.5 yrs) from three different centers. We assessed the outcome of surgical and nonsurgical procedures, based on pain, functional ability, cosmetic results, and x-ray examination. RESULTS: A family background was noted in three children. The lesion always involved the right side. Twenty patients presented a bump over the middle third of the clavicle, thirteen a foreshortened shoulder girdle, three complained of discomfort. In two cases, palpation of the clavicular area was painful. No neurovascular compressive syndrome was reported. None of the patients complained of a decrease in the range of motion or in the strength of the upper limb. X-rays showed a middle third defect. In five cases we found abnormal first ribs. Seventeen patients underwent surgery, at a mean age of 6 years and 4 months. The procedure always included excision of the pseudarthrosis at both ends and internal fixation with a wire or a plate. In only eight cases a bone graft was used. Healing was achieved in fourteen patients. Three patients needed a second surgical procedure. In these 3 cases we had not used bone grafting. All patients had a normal range of shoulder motion, except a twelve year old girl who complained of discomfort of the right upper limb. The cosmetic result was good in eleven cases, one surgical wound was noted as hypertrophic, and one developed a keloid. An asymmetry of the trunk was still noted in seven cases. The x-rays showed symmetric clavicles in ten cases. Eight patients were treated conservatively. All of them had a normal range of motion of the shoulder, six had a good cosmetic result and two cases a poor one. DISCUSSION: According to Alldred, the anomaly results from the failed coalescence of the two primary ossification centers of the clavicle. The overview of the literature and our findings (in one case) confirm that the cartilage which covers both ends of the bone is made of growth cartilage. However, the true mechanism of the nonunion is still unknown. The three familial cases of our work suggest a possible genetic transmission of the disease. The diagnosis is based on the following criteria: right side lesion, found in infancy, without previous fracture, increasing size with growth, without major functional consequences, without neurofibromatosis or cleidocranial dysostosis symptom. X-rays or histologic examination will confirm the diagnosis showing the usual findings described above. Complications of the pseudarthrosis of the clavicle are rare and late. Conservative management appears to give good results as seen with our eight patients. However surgical treatment ensures symmetrical shoulder girdles and good function with few complications. Therefore, we recommend performing an excision of the cartilaginous caps, followed by an iliac bone graft and an internal fixation with wire. Surgical management will be preferred in symptomatic patients, in the case of major or increasing deformity, or on parental request.

[Pott's disease paraplegia in children. Mechanics and therapeutic strategies. Six cases]. / Paraplégie d'origine pottique chez l'enfant. Mécanismes et stratégies thérapeutiques. A propos de 6 cas.

PURPOSE: We report a series of 6 Pott's disease paraplegias treated between 1982 and 1996. MATERIALS AND METHOD: Out of 15 children suffering from Pott's disease, 6 had paraplegia. Treatment consisted of anterior medullar decompression and anterior spine fusion with bone grafting. Two or three weeks later, posterior spine fusion was achieved systematically using a CD fixation device in 3 cases. RESULTS: Neurological signs completely disappeared in 5 children. Vertebral fusion was correct in all patients and kyphosis was less than 50 degrees. DISCUSSION: The posterior approach to the spine must be proscribed as a first step, except for spine dislocation. The anterior approach allowed us to drain the abscess, to correct the kyphosis, and to perform an anterior spine fusion. The posterior spine fusion was performed a few weeks later in order to avoid kyphosis aggravation. CONCLUSION: Prognosis of Pott's disease is good but at the present time, paraplegia remains too frequent. Adapted treatment must be performed without delay.

[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.

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.