[Malignant bone tumours in children and adolescent].
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Adolescent↗
Biomedical subjects
G Bollini
Publications and source records attributed to G Bollini.
Relationship between formation of the femoral bicondylar angle and trochlear shape: independence of diaphyseal and epiphyseal growth.
During hominin evolution, an increase in the femoral bicondylar angle was the initial change that led to selection for protuberance of the lateral trochlear lip and the elliptical profile of the lateral condyle. No correlation is found during ontogeny between the degree of femoral obliquity and of the prominence of the lateral trochlear lip. Might there be a relationship with the elliptical profile of the lateral condyle? On intact femoral diaphyses of juvenile humans and great apes, we compared the anteroposterior length of the lateral and medial sides of the distal metaphysis. The two diaphyseal pillars remain equal during postnatal growth in great apes, while the growth of the lateral pillar far exceeds that of the medial pillar in humans. Increase in bicondylar angle is correlated with disproportionate anteroposterior lengthening of the lateral pillar. The increased anteroposterior length of the lateral side of the metaphysis would contribute to increasing the radius of the curvature of the lateral condyle, but not to the projection of the lateral trochlear lip. The similar neonatal and adult femoro-patellar joint shape in humans prompted an assessment of the similarity during growth of the entire neonatal and adult epiphyses. We showed that the entire epiphysis undergoes drastic changes in proportions during postnatal growth. Finally, we emphasize the need to distinguish the cartilaginous phenotype and the ossified phenotype of the distal femoral epiphysis (and of any epiphysis) during postnatal growth. This crucial distinction applies to most postcranial bones, for they almost all develop following the process of endochondral ossification.
Adaptation, Physiological↗
[Necrotizing cellulitis complicating varicella in two children given nonsteroidal anti-inflammatory drugs].
We report two cases of fasciitis with necrotizing hypodermitis of the foot and arm which complicated varicella in immunocompetent children given nonsteroidal anti-inflammatory drugs. The skin barrier and immune function are weakened by the varicella zoster virus. Exposure to nonsteroidal anti-inflammatory drugs further favors necrotizing cutaneous infections caused by group A beta hemolytic streptococci. MRI can confirm the presence of superficial aponevrosis necrosis defining necrotizing fasciitis but should not retard surgical management which is always indicated for necrotizing fasciitis and sometimes for necrotizing dermo-hypodermitis.
Anti-Inflammatory Agents, Non-Steroidal↗
Unusual phenotype with progressive vertebral fusion in a girl with an apparently balanced t(10;20)(p11;p13) translocation.
We report the case of a girl presenting with an unusual form of multiple joint fusion. Skeletal abnormalities consisted of radioulnar synostosis and vertebral fusions without any carpal, digital or tarsal involvement, and broad ribs and clavicles. Spinal X-rays were available from age 4 to 21, demonstrating that the spinal involvement was progressive and led to a complete anterior and lateral fusion of vertebrae. A complete sequencing of the NOGGIN gene failed to find any mutation. In addition, this girl was carrier of an apparently balanced reciprocal translocation t(10;20)(p11;p13). We investigated the role of the BMP2A gene as a potential candidate gene. Fluorescence in situ hybridization with YAC probes from chromosome 20 showed that the BMP2A gene was not disrupted by the translocation breakpoint.
Abnormalities, Multiple↗
[Radiological index as a predictive factor of kyphosis in spina bifida].
PURPOSE OF THE STUDY: The aim of this work was to validate the pertinence of a radiological index (P/A) used as a predictive factor for risk of kyphosis in children with spina bifida. P/A is defined as the ratio between P, the distance between the posterior vertebral wall and the remnant of the posterior arch, over A, the distance between the anterior wall and the posterior vertebral wall, measured on the lateral view of the spine in the most dysplastic vertebra before one year of age. MATERIAL AND METHODS: The files of 163 children were reviewed. The value of the P/A ratio was measured on lateral views of the spine obtained before the age of one year. The bone level on the anteroposterior film was also noted. The presence or absence of kyphosis was determined from the lateral views obtained at ten years of age. Paired variable analysis was used to compare the mean differences in P/A at one year and at ten years in 31 patients with a bone level of L1 or higher. The exact Fisher test was used to determine the difference in the kyphosis distributions in patients with a bone level of L1 or higher between those with P/A < or = 1/2 and the others. In addition, the specificity, sensitivity, and positive predictive value of a positive test (bone level L1 or higher and P/A < or = 1/2) for development of kyphosis were calculated. Results There was no statistically significant difference in the P/A mean value before the age of one year and after ten years. The distribution of patients with kyphosis was different between patients with a bone level of L1 or higher and P/A < /2 and the other patients (p < 0.01). Considering having a bone level of L1 or higher and a P/A < or = 1/2 as a prognostic test, the specificity was 100%, the sensitivity 87.56%, and the positive predictive value 100%. Many patients with spina bifida will develop kyphosis in the course of life. This spinal deformation raises many specific problems for patient management. Use of a radiological index as presented here allows early detection of patients at risk and can also predict which patients will remain free of deformation. This allows a selection of patients who can benefit most from more regular surveillance of the spinal static and avoid unnecessary radiographic examinations in others.
Adolescent↗
[Shoulder arthrodesis with vascularized fibular graft after tumor resection of the proximal humerus].
PURPOSE OF THE STUDY: Resection of malignant tumors of the proximal humerus often requires dissection of the rotator cuffs and the deltoid muscle. There is no consensus on the ideal method for shoulder reconstruction. We report the functional outcome in a homogeneous series of eight patients treated by arthrodesis using a vascularized free fibular flap. MATERIAL AND METHODS: Eight patients were included in this study. All had an aggressive tumor of the upper humerus. Tumor resection was associated with a rotator cuff and deltoid muscle resection in all patients. All patients then underwent shoulder arthrodesis using a free vascularized fibular flap fixed with a plate. Clinical and radiological evaluation was available for six patients at mean 28 months. The Musculoskeletal Tumor Society function score was used to assess overall function of the upper limb. The cosmetic outcome and radiographic bone healing as well as hypertrophy of the fibular graft were noted. RESULTS: Active abduction and active flexion were 82 degrees on average. All patients could bring their hand to the mouth. Circumduction was possible but limited in amplitude. The mean function score was 26.5/30 with an excellent functional outcome in all patients. The cosmetic outcome was considered poor by all patients. Radiographically, bone healing was achieved at last follow-up in all patients but there was one case of failed fusion between the fibular graft and the scapula which required secondary iliac grafting. Mean fibular graft hypertrophy was 32.8% at last follow-up. DISCUSSION AND CONCLUSION: Two reconstruction methods have been described for patients who require tumor resection of the upper humerus: reconstruction with preservation of glenohumeral joint function and shoulder arthrodesis. Many techniques have been described for each method. It is however difficult to compare the different series reported in the literature because rotator cuff and deltoid muscle resection was not systematically performed and reconstruction methods varied between patients. An analysis of the literature shows that preservation of motion of the scapular glenoid joint can give good functional results when the rotator cuff and deltoid muscle can be preserved. If they cannot, results favor shoulder arthrodesis which provides the patient with very satisfactory upper limb function. Use of a vascularized fibular flap has provided very good arthrodesis results. The patient must however be informed of the probable poor final cosmetic result.
Adolescent↗
[Results at skeletal maturity after double-approach hemivertebral resection].
PURPOSE OF THE STUDY: The natural history of congenital scoliosis or kypho-scoliosis resulting from a hemivertebra is well documented. The spinal deformation generally worsens in children with a free or semi-segmented hemivertebra situated in the thoraco-lumbar, lumbar or lumbosacral region. MATERIAL AND METHODS: From 1982 to 1997, fifteen pediatric patients with 15 hemivertebrae causing progressive scoliosis or kypho-scolisosis underwent hemivertebral resection via a double posterior and anterior approach associated with convex fusion. Mean age at surgery was 4.4 years. The fifteen patients were reviewed at bone maturity (Risser 4 or 5) to assess outcome. RESULTS: Genitourinary tract anomalies were associated in five of the 15 patients and intrathecal anomalies in two. Mean follow-up was 12.1 years. Segmental scoliosis was 30.2 degrees preoperatively, 12.3 degrees postoperatively and 8.9 degrees at last follow-up. The values for total scoliosis were 30.5 degrees, 12.3 degrees and 12.6 degrees respectively. This was a 70.5% improvement at last follow-up for segmental scoliosis and 58.7% for total scoliosis. The real trunk imbalance improved from 31% preoperatively to 9% at last follow-up. All these differences were significant. DISCUSSION: The main objective of surgical treatment for congenital scoliosis due to hemivertebra is to prevent the development of severe deformation which would necessitate a dangerous and difficult procedure to achieve correction. Resection of the hemivertebra via a double approach is the ideal method for early correction. The procedure not only corrects the spinal deformation but also prevents later deterioration. Correction should be performed as early as possible. The result is sustained over time if there is no other associated spinal anomaly. The procedure is safe and the technique well controlled in experimented hands. Patients need to wear a corset for only six months after resection of the hemivertebra.
Adolescent↗
[The newborn foot abnormalities].
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Foot↗
Leg amputation after intraosseous infusion in a 7-month-old infant: a case report.
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Amputation, Surgical↗
[Anatomic study of the anterior patellar groove in the fetal period].
PURPOSE OF THE STUDY: We performed a biometric analysis of the femoral trochlea in the fetus and compared our findings with those observed in adults in order to search for correlations with other biometric parameters of the femur. MATERIAL AND METHOD: Twenty-two fetuses (44 knees) conserved in formol and free of known orthopedic disease were studied. Fetal age ranged from 26 to 40 weeks. After anatomic dissection, digitalized images were used to obtain angle measurements with a dedicated software. Measurements made on the distal epiphyseal view were: anteroposterior dimension of the condyles, medial and lateral protrusion of the trochlear borders, difference in condyle height, length of the trochleal borders, alpha angle of the trochlear groove, trochlear slope. Measurements made on the AP femoral view were: femoral anteversion, length of the femoral neck, neck-shaft angle. Spearman's test was used to search for correlations. Results were compared with measurements obtained under the same conditions in a series of 32 adult knees published by Wanner. RESULTS: The trochlear alpha angle was 148 degrees (coefficient of variation 4%). The angle was greater than 150 degrees for 18 trochleae. The lateral border of the trochela was higher than the medial border in 37 of the 44 knees. There was no correlation with age and gender. Femoral anteversion was 27.01 degrees, with a high coefficient of variation (46%), and no correlation with the trochlear alpha angle. Comparison with measurements made on the adult knees revealed no significant difference. DISCUSSION: This is the first report of statistically significant biometric data of the fetal trochlea. The morphology of the lower femur observed during the third trimester of fetal live is the same as observed in adults. Morphological changes in the proximal femur occurring during growth do not appear to modify the morphology of the distal femur. The asymmetrical ingression of the patella into the trochlea, characteristic of modern man, is considered to result from bipedalism. Our study would suggest that the anatomic characteristics of the trochlea could have been integrated into the genoma during the course of evolution. This would be in favor of a genetic origin of trochlear dysplasia.
Adult↗
[Multifocal osteomyelitis as the first manifestation of chronic granulomatous disease].
Chronic granulomatous disease is a rare immune disease related to an anomaly in phagocytes NADPH oxidase. The characteristic clinical feature is early recurrent and sometimes serious infection. We report the case of a 22-month-old child who developed multifocal osteomyelitis, an unusual inaugural manifestation of chronic granulomatous disease. Septic chronic granulomatous disease is an uncommon differential diagnosis in children who develop recurrent infections. Diagnosis is established with specific blood tests: reduction of tetrazolium nitroblue, chemoluminescence test, molecular analysis. Therapeutic management must be undertaken as early as possible in order to preserve the long-term prognosis. No curative treatment is currently available. Aggressive treatment of each infectious focus with an adapted antibiotic regimen and in certain cases surgical debridement is required in addition to long-term antibiotic prophylaxis.
Consanguinity↗
[Biphosphonates in children: present and future].
Biphosphonates are synthetic analogs of the natural pyrophosphate molecule, introduced primarily for the treatment of Paget disease of bone. Their main mechanism of action consisting in an inhibition of osteoclastic activity is critical for treatment of disorders including an increased bone resorption. In childhood osteoporosis (especially osteogenesis imperfecta), hypercalcemia and heterotopic calcifications are the three main situations in which they have been successfully used, with however few follow-up and no controlled studies. The evolution of these compounds generating more potent products, given orally, with limited effect on bone mineralization should allow an extension of their use in pediatric patients. Multicentric studies are now necessary to specify their efficacy and guidelines for use in children.
Administration, Oral↗
[Treatment of the motor cerebral palsy child with botulinum toxin A: mode of action, injection places in management].
Many studies have demonstrated the effectiveness of botulinum toxin injections in order to decrease spasticity in cerebral palsy. However it is essential to carefully assess children to be treated, and the muscles which need to be injected. Injections of soleus in dynamic equinus without fixed contrature in very young children provide the best results, but other muscles may be injected such as upper limbs muscles. Injections have mainly a functional purpose, but sometimes they may contribute to prevent fixed contractures or pain during nursing.
Activities of Daily Living↗
[Total hip arthroplasty without cement in children and adolescents: 17 cases].
PURPOSE OF THE STUDY: Hip disease can produce major pain and functional disorders in children who should benefit from total hip arthroplasty. We report our experience with total hip prostheses implanted without cement in children. MATERIAL AND METHODS: We performed 17 total hip arthroplasties in 13 children who had various conditions, mainly chronic juvenile osteoarthritis and aseptic osteonecrosis secondary to sickle cell anemia. The acetabular inserts were not cemented. All the femoral stems were custom-made using computer-assisted preoperative planning based on standard x-rays and computed tomography findings. Outcome was assessed on the basis of patient satisfaction, pain, and function. Radiographically, we assessed stem implantation, stability and integration. Results were classed with the Harris score and also with the Steinbrocker classification in order to take into account the child's overall functional handicap. RESULTS: Mean follow-up was 36.4 months. There were three cases of superficial hematoma, one case of superficial sepsis and one acetabular loosening. All patients were satisfied. Unsatisfactory function, observed in 80% of the children preoperatively, was found in only 17% postoperatively. The Harris score improved from 23.8 preoperatively to 87.7 at last follow-up. There were no cases of stem loosening and integration was achieved in 85.3% of the cases proximally in the area with hydroxyapatite surfacing. DISCUSSION: The problem with these children is to determine when total hip arthroplasty should be proposed. We retain three important indications: uncontrollable chronic pain, normal school attendance impossible, no other possibility for conservative surgery. Several studies have reported only mediocre results with cemented stems. We opted for custom-made stems without cement for three reasons: preservation of bone stock, better adaptation to bone whose quality and morphology had been remodeled by the underlying condition and repeated osteotomies which also affect the gluteus medius, and finally, better chance of success for future revisions in these active young patients. CONCLUSION: The question on whether or not total hip arthroplasty should be performed early in these children to avoid osteotomies which could alter the longevity of a future prosthesis remains open. Custom-made stems inserted without cement have provided satisfactory results in our experience. To date, follow-up is too short to compare our results with those obtained by others using cemented stems.
Activities of Daily Living↗
[Progressive forearm lengthening in children: 14 cases].
PURPOSE OF THE STUDY: There are few indications for forearm lengthening in children. Several techniques have been proposed. We report our experience with progressive lengthening of the forearm in children using a unilateral axial external fixator and an improved technique consisting in initial insertion of an intramedullary guide wire. MATERIAL AND METHODS: Since 1990, we performed 14 forearm lengthenings in 9 children. Radial agenesia (5 forearms in 4 children), and hereditary multiple exostosis (3 forearms in 2 children) were the predominant causes. The ulna was involved in 9 cases and the radius in 5. Age at initiation of the lengthening procedure ranged from 4.5 to 14.8 years (mean 9.9). The lengthening technique consisted in a transverse subperiosteal osteotomy of the bone shaft then progressive distraction with a unilateral axial external fixator. When axial deviation had to be corrected, we used a subtraction osteotomy. In our last 10 cases, we inserted an intramedullary guide wire in the lengthened bone. The external fixation was left in place throughout the lengthening procedure and until complete bone healing. Serial radiographs were used to assess bone healing, the degree of lengthening achieved and any axial deviation at the end of lengthening. RESULTS: All 14 forearms were reviewed at a mean 50.6 months. Mean lengthening was 26.4 mm (range 10 - 52 mm). There were no nerve or vessel complications. In one case, reducible claw finger completely regressed after temporary interruption of the lengthening. There were 6 cases of late healing requiring a secondary bone graft. The healing index was 61.9 days per cm gained length. There were 3 cases with an axial deviation at the end of lengthening. DISCUSSION: Insertion of a guide wire in the bone being lengthened reduced the risk of late healing compared with lengthening procedures without a guide wire, avoiding axial deviation. In addition, this technique led to more rapid bone healing so the fixator could be removed earlier. We have found this method to be easier to perform on a normally axed segment. This would require an initial subtraction osteotomy for prior alignment. CONCLUSION: Forearm lengthening is a difficult procedure. Use of an intramedullary guide wire associated with an external fixation and an initial osteotomy for axial correction when needed and possibly stabilization of the wrist is an important contribution, particularly for malformed forearms.
Adolescent↗
Delayed cure of an omphalocele requiring abdominosternoplasty, right hepatectomy and partial splenectomy.
A large ventral hernia resulting from the primary treatment of an omphalocele according to the Gross technique was repaired at age 16. The girl presented with extra-abdominal development of the liver and the spleen along with hypotrophy of the abdominal and thoracic cavities. The operation included enlargement sternoplasty, liver and spleen-size reduction and prosthetic abdominal closure. The cosmetic and functional results are good and stable on 7-year follow-up. Surgical issues and blood transfusion policy are discussed. A multi-disciplinary pediatric surgical approach is advocated.
Female↗
Congenital pseudarthrosis of the tibia: history, etiology, classification, and epidemiologic data.
Congenital pseudarthrosis of the tibia is a rare disease with a variable history. The pseudarthrosis is usually not present at birth (and therefore is not truly congenital) but occurs during the first decade of life. Paget in 1891 was the first to describe a case. The etiology is unknown. Neurofibromatosis plays a role in approximately 50% of patients. In the others, hereditary and mechanical factors are debated. Various (morphologic) classification systems have been proposed (Andersen, Boyd, Crawford). Because the appearance changes during the course of the disease, all classification systems have limited value: the determining factor is the stage of the disease at which it was classified. Because of the rarity of the disease and the variability of its history, the European Pediatric Orthopaedic Society decided to carry out a multicenter study on this disease. This paper presents the epidemiologic data on the patients involved. Data have been gathered on 340 patients from 13 countries. Two hundred patients were male (58.8%), 140 were female (41.2%). The right side was affected in 165 patients (48.5%) and the left side in 172 (50.6%); 3 patients had bilateral disease. Symptoms of neurofibromatosis were present in 54.7%. Histologic examinations in 192 patients showed a nonspecific appearance in 45.3%; in 15.6% the ultrastructure resembled fibrous dysplasia, and in 39% there was histologic evidence of neurofibromatosis. Most of the lesions were initially localized in the middle or distal third of the tibia. In 29% the localization changed during the course of the disease.
Child↗
The fibula in congenital pseudoarthrosis of the tibia: the EPOS multicenter study. European Paediatric Orthopaedic Society (EPOS).
Fibular involvement in congenital pseudoarthrosis of the tibia (CPT) can be either a rare isolated pathology or is in association with the tibial changes. Out of 282 patients with CPT who had complete radiographic work-up, 62% (almost two thirds) demonstrated fibular pathology: 36% had true fibular pseudoarthrosis and the rest, i.e., 26% had fibular hypoplasia or dysplastic fibula. Neurofibromatosis was found in 62% of the patients with fibular pathology. The typical radiological features of tibial pseudoarthrosis are often missing in patients with fibular pathology. It is most probably because fibular changes precede the tibial involvement. In 250 patients with tibial surgical treatment, the highest rate of fusion occurred in patients with fibular involvement compared with those with normal fibula.
Child↗