[Traumatic hand injuries in children]. / La main traumatique de l'enfant.

Hand injuries in children are frequent in pediatric emergencies. Epidemiology of hand injuries in children depends on age and social context. The management of these injuries requires experienced pediatrics in order to obtain a functional hand to preserve the potential growth and to reduce the risk of cosmetic sequelae. We will describe the specificities of hand trauma in children and their therapeutic principles.

Secondary mobilization of the first dorsal metacarpal artery flap for first web space reconstruction in a child: A case report.

The authors report the case of an iterative mobilization of a skin flap based on the first dorsal metacarpal artery. This flap was initially associated with a toe-to-hand transfer to provide adequate skin coverage in the reconstruction of a post-traumatic thumb defect in a 5-year-old child. More than 8years after initial surgery, this flap was mobilized again for recovery of the first web space opening, which was retracted. This case illustrates the possibility of remobilizing the first dorsal metacarpal artery flap to reduce donor site sequelae in children.

[Pericardial foreign body: an unusual cause of chest pain in children]. / Corps étranger péricardique: une cause inhabituelle de douleur thoracique chez l'enfant.

Penetrating thoracic trauma by a needle or pin is rarely described in children. Localization of the needle may sometimes be difficult. The needle can migrate from the entrance site into many organs with time and cause little initial morbidity. We describe the case of a 14-year-old male patient with a sewing needle accidentally inserted through the chest wall. The foreign body had migrated spontaneously to the pericardium. A computed tomography scan of the chest is needed to determine the location of the needle and show any complications. Pericardium foreign bodies are dangerous and need electrocardiography and cardiac ultrasound before treatment. Removal of the needle by thoracotomy or thoracoscopy is indicated.

Evidence of association between GDF5 polymorphisms and congenital dislocation of the hip in a Caucasian population.

OBJECTIVE: Congenital dislocation of the hip (CDH) is a multifactorial disease which involves genetic factors that are still unidentified. Recently, a functional polymorphism (rs143383) of the 5'-untranslated region of GDF5 (Growth/Differentiation Factor 5) - previously reported to be associated with osteoarthritis - has been associated with CDH in a Chinese population. The aim of our study was to determine whether GDF5, known to be involved in bone, joint and cartilage morphogenesis, is also associated with CDH in Caucasians. DESIGN: We genotyped three tagSNPs (rs224334, rs143384, rs143383) in 239 cases and 239 controls from western Brittany (France) where CDH is frequent, and tested the association using both single-locus and haplotype-based approaches. RESULTS: The most significant association was observed with rs143384. The T allele of this SNP was overrepresented in cases (65.9% vs 55.9%, P=0.002). Under a recessive model, carriers of the TT genotype had a 1.71-fold higher risk of developing CDH than carriers of the other genotypes (OR(TT vs CT+CC)=1.71, 95% CI: [1.18-2.48], P=0.005). At a nominal level, the association was also significant with rs143383 (OR(TT vs CT+CC)=1.52, 95% CI: [1.05-2.19], P=0.026). The haplotype carrying the susceptibility alleles of these SNPs was also more frequent in cases (65.9% vs 55.9%, OR=1.53, 95% CI: [1.18-1.98], P=0.002). CONCLUSION: This study reports, for the first time, the association between GDF5 polymorphisms and CDH in Caucasians, and points out another polymorphism of interest that requires further investigation. Reduction in GDF5 expression might lead to developmental deficiency of ligaments and capsule in hip joint, and therefore contribute to CDH pathogenesis.

Split cervical spinal cord malformation and vertebral dysgenesis.

We report a case of vertebral malformation associated with diplomyelia believed to be a type II split cord malformation. Cervicothoracic level cases are exceptional. This article reports the case of an 11-year-old boy with no neurological symptoms who had not undergone surgery. The diagnosis was made during pregnancy by prenatal screening with ultrasound and MRI. Several embryological theories have been offered to provide an explanation for this syndrome. Close follow-up is mandatory. Surgery must only be considered if neurological deterioration occurs.

Do HOXB9 and COL1A1 genes play a role in congenital dislocation of the hip? Study in a Caucasian population.

OBJECTIVE: Congenital dislocation of the hip (CDH), which is one of the most common congenital skeletal disorders, corresponds to an abnormal seating of the femoral head in the acetabulum. It is commonly admitted that CDH presents a genetic component. However, little is known about the genetic factors involved. This study aimed to determine the role of two potential candidate genes on chromosome 17 in CDH: HOXB9 (involved in limb embryonic development) and COL1A1 (involved in joint laxity). METHOD: We set up a case-control association study (239 cases and 239 controls) in western Brittany (France) where CDH is particularly frequent. The set of informative single nucleotide polymorphisms (SNPs) in each gene was selected using Tagger and genotyped using the SNaPshot method (n=2 and n=10, respectively). The association was tested both through single-locus and haplotype-based analyses, using SAS and Haploview softwares. In addition, we carried out the transmission disequilibrium test (TDT) with the same polymorphisms from a sample of 81 trios (i.e., 81 patients included in the case-control study and their both parents). RESULTS: The case-control study revealed no significant association between CDH and the tagSNPs selected in both HOXB9 and COL1A1. Moreover, the TDT did not reveal distortion in allelic and haplotype transmission of the studied markers. CONCLUSION: Our study did not support an association between HOXB9 and COL1A1 and CDH in our population. These negative findings were obtained by population- and family-based designs. Analysis of the genetic component of CDH should focus on other candidate genes.

[Osteochondritis dissecans of the femoral condyles: report of 892 cases]. / L'ostéochondrite disséquante des condyles fémoraux.

PURPOSE OF THE STUDY: Osteochondritis rarely involves the femoral condyles. Discovery in this localization raises several questions concerning the nature of the articular cartilage, the potential for spontaneous healing, and, in the event of a free fragment, the outcome after its loss or repair. MATERIAL AND METHODS: This multicentric study included 892 pediatric and adult cases, the cutoff between two series being defined by fusion of the inferior growth plate. We excluded medical or surgical osteochondritis, cases involving the patella, osteochondral fractures, juvenile polyosteochondrosis, adult osteonecrosis, and osteochondritis beginning after the age of 50 years. RESULTS: Mean age at diagnosis was 16.5 years. Mean age at treatment onset was 22 years. Pain was the predominant symptom. 80% of cases were unilateral and 70% involved the medial condyle. The anatomic lesions were different in adults, showing more advanced degradation. At diagnosis, Bedouelle stages Ia and IIb constituted 80% of the cases observed among children while in adults, 66% were Bedouelle stages IIb to IV. Outcome was very good for the majority of children with Hughston clinical stage 4 while half of the x-rays were Hughston stage 3 and 4. There were thus a large percentage of children with abnormal xrays whose disease history was not yet terminated. In the adult series, the percentages of Hughston 3 and 4 was about the same as clinically. The x-rays were rarely perfectly normal since half of the clinical stage 3 patients were noted in stage 4. An abnormal x-ray with a very good clinical presentation was observed in a very large proportion of patients. DISCUSSION: It is difficult to interpret the plain x-ray and identify patients with a potentially unfavorable prognosis. We defined three radiographic classes: defect, nodule and empty notch. The Bedouelle classification uses information from all available explorations, particularly MRI and arthroscopy. Numerous therapeutic methods are used. Interruption of sports activities is the first intention treatment for children. Data in the literature and the findings of this symposium do not demonstrate any beneficial effect of immobilization on healing compared with simple abstention from sports activities. Transchondral perforation is a simple operation with low morbidity. In 85% of cases, it was used for lesions with an intact joint cartilage considered stable in 96% of cases. Healing was achieved in six months for 48% if the growth plate had not fused. The fragment was fixed in 43% of the cases with a loose cartilage fragment. Outcome was fair but degraded with the state of the joint cartilage and thus the stability of the fragment. Fixation must stabilize the fragment but not prevent further consolidation via osteogenesis. This is why deep perforations are drilled beyond the ossified area and additional osteochondral grafts are used. The Wagner operation gives less satisfactory results than more complicated procedures. Removal of a sequestrum is a simple, minimally invasive procedure with an uneventful postoperative period, but in the long term it favors osteoarthritic degradation, especially when performed in adults. Mosaic grafts give good mid term results. Morbidity is low especially if the grafts are harvested above the notch. The question of chondrolysis around the grafts was beyond the scope of this study. Chondrocyte grafting is difficult to accomplish and is expensive. The mid term results are good for large lesions. Osteotomy is logical only in the event of early stage osteoarthritic degradation. DECISION ALGORITHM IN CHILDREN AND ADOLESCENTS: If the plain x-ray reveals a defect (class I), simple interruption of sports activities should be proposed. Two situations can then develop. First, in a certain number of patients, the pain disappears as the defective zone ossifies progressively. Complete cure is frequent before the age of 12 years. In the second situation, the knee remains painful and the x-ray does not change or worsens to a class II nodular formation. In this case an MRI must be obtained to determine whether the joint cartilage is normal. There are two possibilities. First, the osteochondral fragment is viable and most probably will become completely re-integrated, particularly if the lesion is far from the growth plate. Necrosis is the other possibility. Transchondral perforations are needed in this case. If on the contrary the cartilage is altered, there is little hope for spontaneous cure. Arthroscopy may be needed to complete the exploration. Fragments, especially if there is a large surface area, must be fixed. Perforations to favor revascularization are certainly useful here. In the last situation (class III), the fragment wobbles on a thin attachment or has already fallen into the joint space. This is the type of problem generally observed in adults. The decision algorithm in adults is the same as in children for the rare nodular aspects (class II). There could be a discussion between transcartilage perforation and fixation. If there are a large number of fragments, fixation may not be fully successful and the lesion might be considered class III. For class III lesions, three operations can be used: removal of the sequestrum, mosaic bone-cartilage grafts, or autologous chondrocyte grafts. At the same follow-up, mosaic grafts give better results than excision of sequestra. It may be useful to remove sequestra in a limited number of situations: if there is just a small area of osteochondritis, the lesion is old and partially healed, or the zone is non weight-bearing. For other lesions, we favor mosaic grafts. We still do not have enough follow-up to assess the long-term outcome with these mosaic grafts, but simple excision clearly favors osteoarthritic degradation. Can chondrocytes grafts be compared with mosaic grafts? Chondrocyte grafts have been used for very large lesions and have given results similar to mosaic grafts. It might also be possible to combine fixation of a loose fragment and a mosaic graft. LESSONS FROM THIS STUDY: 1) The prognosis of osteochondritis is better before than after fusion of the growth plate but the lesion does not always heal in children. 2) Presence of osteochondritis requires complementary anatomic and functional exploration to determine the stability and the vitality of the fragment. 3) Attention must be taken to perform transchondral perforations early enough, particularly in children. 4) Screw fixation is not always sufficient. The trophicity of the fragment and its blood supply must be improved. 5) Mosaic grafts are preferable to excision of the fragment. 6) Chondrocyte grafts will be more widely used in the future.

[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.

Clinical and electrophysiological phenotype of a homozygously duplicated Charcot-Marie-Tooth (type 1A) disease.

Type 1A of Charcot-Marie-Tooth disease (CMT1A) is associated with a microduplication of chromosome 17 (region 17p11.2) which contains PMP22, an important gene for peripheral nerve myelination. Patients carrying two duplications are expected to have a more severe phenotype, close to the Dejerine-Sottas syndrome. In this article, we report a family of 5 CMT1A patients in whom the unrelated father and mother carry a 17p11.2 duplication. The 2 daughters carry only one duplication (one given by the father, the other given by the mother), but the son carries two 17p11.2 duplications. Interestingly, the clinical phenotype of the son is more severe (scoliosis) compared to those of his sisters, but his motor nerve conduction velocities are in the range of a heterozygote CMT1A patient. The mechanisms leading to a more severe phenotype for CMT1A are discussed and may not be strictly related to lower nerve conduction velocities.

[Kirner's deformity. 4 case reports and review of the literature]. / La déformation de Kirner. A propos de 4 cas et revue de la littérature.

Kirner's disease is a rare deformity of the distal phalanx of the little finger, with a combination of camptodactyly and clinodactyly and clinodactyly. A review of the literature and analysis of our cases show that this anomaly appears to result from a unusual insertion of the deep flexor over the growth cartilage. No treatment is required in view of the absence of any clinical and functional symptoms.

[Anorectal traumas by impalement in children. Apropos of 3 cases]. / Les traumatismes ano-rectaux par empalement chez l'enfant. A propos de trois observations.

Traumatic impalement is an exceptional cause of anorectal lesions in the child. The clinical diagnosis is sometimes difficult and associated lesions raise the problem of the most appropriate exploratory examination. Based on our experience with three cases, we would emphasize the usefulness of hydrosoluble enema study prudently performed by the surgeon during the preoperative work-up. The precise localization of the lesions must be obtained with careful emergency exploration before repair. Treatment of the trauma includes lavage, drainage and sometimes colostomy. Antibiotics must always be prescribed. Early complications are dominated by infection and long-term sequellae, requiring a long follow-up.

[Flaps for covering extensive losses of cutaneous substance on the upper limb. Apropos of 15 cases]. / La couverture par lambeaux des grandes pertes de substance cutanée du membre supérieur. A propos de 15 cas.

The authors report on their recent experience concerning flap treatment of upper limb complex trauma, based on 15 cases. Proper wound care, and the use of healthy, well vascularised tissues for coverage is of paramount importance for good results, whose quality mostly depends on the severity of initial lesions. The results of this series are encouraging and appear to be a consequence of the great possibilities offered by the cutaneous or muscular flaps used.

[Characteristics of fractures of the distal part of the clavicle in children]. / Particularités des fractures des extrémités de la clavicule chez l'enfant.

Fractures of the clavicle are frequent in children and mostly affect the middle third. On the other hand, traumatic fractures of both medial and lateral thirds of the clavicle are rare. The lesions have special characteristics: difficulty in diagnosis for the medial third; risk of callus formation in the absence of surgical reduction of the lateral third. The authors report two observations of fractures of the medial and lateral thirds of the clavicle; they insist on the value of investigation by CT scan.

[Traumatic atlanto-axial dislocation in children: 7 cases]. / Luxation traumatique atloïdo-axoïdienne chez l'enfant: à propos de 7 cas.

Seven cases of atlantoaxial rotatory dislocation in children are reported. The CT scan permitted differentiation of the lesions in all the cases. Analysis of the results has led us to introduce two modifications for Fielding's classification: a distance between the atlas and odontoid of less than 5 mm is normal. sagittal dislocation must be included in the same classification. We propose the following therapeutic protocol: rotatory luxation without tears of the transverse ligament should receive orthopedic treatment, rotatory luxation with tears of the transverse ligament should have surgical treatment if orthopaedic treatment fails. sagittal luxation with or without rotatory fixation needs surgical treatment.

[Giant cell tumor of the tendon sheath of the long extensor muscle of the thumb in a 7-year-old child]. / Tumeur à cellules géantes de la gaine du tendon du long fléchisseur du pouce chez une enfant de 7 ans.

The authors report the case of a giant cell tumour of the flexor pollicis longus tendon sheath in a child. This benign tumour, usually observed in women between the ages of 30 to 50 years, has a highly controversial aetiopathogenesis; the existence of initial trauma is found in 50% of cases. The present case concerns a 7-year-old girl with swelling of the right thumb for two years. Surgical resection established the definitive diagnosis by histological examination of the specimen. The follow-up is currently two years, with no local recurrence.

[Epiphyseal fracture-avulsion of the distal extremity of the clavicle. Apropos of a case]. / La fracture-décollement épiphysaire de l'extrémité distale de la clavicule. A propos d'une observation.

Fracture of the clavicle is the commonest fracture in children. It is generally due to direct trauma to the shoulder. Orthopaedic usually ensures consolidation within three weeks. Fractures of the lateral third is uncommon and unusual. It corresponds to an epiphyseal avulsion of the distal extremity of the clavicle, equivalent to an acromioclavicular "pseudodislocation". The coracoclavicular ligaments remain intact. Surgical reduction of the clavicle into its periosteal sheath is necessary. The authors report a case of fracture of the lateral third of the clavicle in a 5 and a half year old child with spontaneous formation of a "neoclavicle".

[Sensory sequelae of injuries to the median nerve]. / Les séquelles sensitives des plaies du nerf médian.

Median nerve wounds are frequent because of the superficial course of this nerve, especially in the wrist. The sensory sequellae of such lesions, either complete or dissociated, represent a major handicap which may be associated to a motor impairment of the external thenar muscles. The curative procedures such as neurolysis and, above all, sutures or grafts, must be very widely used, even some time after the initial injury and in spite of motor reinnervation. The role of palliative surgery is therefore limited, although it is sometimes useful. There are many procedures, which may be summed up by two techniques: use of sensitive neurovascular skin flaps, the most frequent grafts, neurotization of the median nerve with a transfer of sensory rami from the radial nerve, a less-known technique. The treatment of the sensory sequellae of median nerve lesions is primarily based on a good initial management of the nerve lesions by a good-quality emergent microsurgical suture.