Congenital dislocation of the hip: optimal screening strategies in 2014.

A prospective multi-centre nationwide study of patients with congenital dislocation of the hip (CDH) diagnosed after 3 months of age was conducted with support from the French Society for Paediatric Orthopaedics (Société Française d'Orthopédie Pédiatrique [SoFOP]), French Organisation for Outpatient Paediatrics (Association Française de Pédiatrie Ambulatoire [AFPA]), and French-Speaking Society for Paediatric and Pre-Natal Imaging (Société Francophone d'Imagerie Pédiatrique et Prénatale [SFIPP]). The results showed inadequacies in clinical screening for CDH that were patent when assessed quantitatively and probably also present qualitatively. These findings indicate a need for a communication and educational campaign aimed at highlighting good clinical practice guidelines in the field of CDH screening. The usefulness of routine ultrasound screening has not been established. The findings from this study have been used by the authors and French National Health Authority (Haute Autorité de Santé [HAS]) to develop recommendations about CDH screening. There is an urgent need for a prospective randomised multi-centre nationwide study, which should involve primary-care physicians.

Unpredictability of hip behavior in Dyggve-Melchior-Clausen syndrome: a mid-term assessment of siblings.

Dyggve-Melchior-Clausen syndrome is a rare spondylo-epiphyseal disease, which almost constantly leads to both bilateral hip degeneration and dislocation. Few authors have reported to date the surgical management of this orthopaedic disorder. We present two new cases affecting siblings. One brother was treated by unilateral triple pelvic osteotomy combined with varus osteotomy of the proximal femur; the other was treated by bilateral Pemberton osteotomies with varus osteotomy of the proximal femur. At a respective 5-year and 3-year follow-up delay, both cases had evolved towards progressive subluxation recurrence along with severe hip degeneration. Based on both our experience and literature review, it seems that one should avoid operating these hips unless pain renders surgery mandatory. Total hip arthroplasty seems the only reliable surgical solution at the adult age and paediatric surgeons should keep in mind that previous femoral osteotomies will make it more challenging for adult orthopaedic surgeons to implant on a remodeled anatomy.

Congenital high scapula (Sprengel's deformity): four cases.

We report a series of four cases of congenital high scapula (or Sprengel's deformity) diagnosed and followed in our establishment. The main feature of this very rare congenital malformation of the pectoral girdle is an abnormally high, more or less dysmorphic scapula. A congenitally high scapula is often discovered in young children, when consequences for aesthetics, and sometimes functional difficulties, are brought to light. When surgical treatment is envisaged, imaging is recommended to diagnose a supernumerary structure, ossified (an omovertebral bone) or not (a fibrous and/or cartilaginous connection), extending from the scapula to the cervical spine. This needs to be resected. A CT scan is essential for detecting an omovertebral bone and the vertebral bone abnormalities that are often associated with it. MRI and ultrasound are very useful for assessing any fibrous and/or cartilaginous components. An ultrasound examination has the undeniable advantage of being quick and easy in these young children.

Progressive anterior vertebral fusion: a report of three cases.

We report a series of three cases of progressive anterior vertebral fusion diagnosed and monitored in our establishment. This very rare condition was discovered in young children while exploring a spinal deformity. With X-rays of the thoracolumbar spine it was possible to make a positive diagnosis and ensure follow-up. The radiographic semeiotics are characteristic and combine anterior pinching of the disc, well-delimited erosion of the anterior vertebral corners and anterior then posterior intervertebral ankylosis. MRI is very useful for assessing the extent of the intervertebral ankylosis and the condition of the residual discs, without irradiation. The condition develops over several months or years. Conservative treatment is usually sufficient.

Meningococcal purpura fulminans in children: I. Initial orthopedic management.

BACKGROUND: Purpura fulminans is a rare and extremely severe infection, mostly due to Neisseria meningitidis frequently causing early orthopedic lesions. Few studies have reported on the initial surgical management of acute purpura fulminans. The aim of this study is to look at the predictive factors in orthopedic outcome in light of the initial surgical management in children surviving initial resuscitation. METHODS: Nineteen patients referred to our institution between 1987 and 2005 were taken care of at the very beginning of the purpura fulminans. All cases were retrospectively reviewed so as to collect information on the total skin necrosis, vascular insufficiency, gangrene, and total duration of vasopressive treatment. RESULTS: All patients had multiorgan failure; only one never developed any skin necrosis or ischemia. Eighteen patients lost tissue, leading to 22 skin grafts, including two total skin grafts. There was only one graft failure. Thirteen patients were concerned by an amputation, representing, in total, 54 fingers, 36 toes, two transmetatarsal, and ten transtibial below-knee amputations, with a mean delay of 4 weeks after onset of the disease. Necrosis seems to affect mainly the lower limbs, but there is no predictive factor that impacted on the orthopedic outcome. We did not perform any fasciotomy or compartment pressure measurement to avoid non-perfusion worsening; nonetheless, our outcome in this series is comparable to existing series in the literature. V.A.C.(®) therapy could be promising regarding the management of skin necrosis in this particular context. While suffering from general multiorgan failure, great care should be observed not to miss any additional osseous or articular infection, as some patients also develop local osteitis and osteomyelitis that are often not diagnosed. CONCLUSIONS: We do not advocate very early surgery during the acute phase of purpura fulminans, as it does not change the orthopedic outcome in these children. By performing amputations and skin coverage some time after the acute phase, we obtained similar results to those found in the literature.

Meningococcal purpura fulminans in children. II: Late orthopedic sequelae management.

BACKGROUND: Purpura fulminans is a rare and extremely severe infection, mostly due to Neisseria meningitidis. Nineteen patients were followed up immediately after the initial multivisceral failure in order to diagnose late-onset orthopedic sequelae. We report our experience with these 19 patients, in light of our medical follow-up protocol and surgical management. MATERIALS AND METHODS: Nineteen patients were referred for acute purpura fulminans between 1987 and 2005 to our institution and followed up prospectively until the present. We collected information on all diagnosed orthopedic sequelae, all surgical procedures performed, and the actual orthopedic outcome. RESULTS: Fourteen patients developed at least one orthopedic sequel after a mean of 2 years delay, with a mean of 8.65 years follow-up (range 3-22 years). The most common presentation was lower limb physeal growth plate arrest in eight patients involving 18 growth plates, leading to five limb length discrepancies and 12 significant knee and/or ankle deviations. Patients were treated by completing epiphysiodesis in addition to limb lengthening and/or reaxation osteotomies, except for two patients, in which epiphysiolysis was performed. All outcomes are, to date, satisfactory, with both knee and ankle axes within the physiological range. Among the seven patients who underwent below-knee amputation, six needed stump revision because of skin conflict (4) or prosthetics misadaptation due to upper tibial varus (2). Regarding the upper limb, three patients presented with four cicatricial scar bands, one located on a ring finger, two at the first commissure, and one at the wrist (all were successfully treated by enlargement Z-plasties). Two patients developed hip avascular necrosis. CONCLUSION: It is important for children diagnosed with meningococcal purpura fulminans to be followed up closely starting from the very beginning by a pediatric orthopedic surgeon. It ensures that late-onset orthopedic sequelae will be diagnosed early. In accordance to the literature, this study highlights the high rate of lower limb epiphysiodesis, above all other types of sequelae. This study reports a possible link between purpura fulminans and avascular necrosis of the hip.

[Results of surgical treatment in simple syndactily using a commissural dorsal flap. About 54 procedures]. / Résultats du traitement chirurgical des syndactylies simples par lambeau commissural dorsal. A propos de 54 commissuroplasties.

OBJECTIVE: The authors evaluate their results of congenital syndactyly correction using a classic commisural dorsal flap technique and full thickness skin grafts to cover the lateral and medial aspect of the fingers. MATERIAL AND METHOD: It is a retrospective study on a homogeneous series of 38 children (54 simple syndactylies) operated over a 10-year period. Twenty-two children were included with a mean follow-up of five years and 10 months. RESULTS: This technique is reliable, reproducible, with a low rate of complications (only four complications requiring reoperation). Long-term outcomes are satisfactory in 92% of cases. CONCLUSION: This is an effective technique, restoring the natural shape of the web space. Procedures without skin-graft are less time-consuming in terms of operational time and cosmetically acceptable, but still need to prove their worth.

[Electrophysical surveillance in limb lengthening in 25 children and adolescents]. / Intérêt de la surveillance électrophysiologique des allongements de membre par fixateur externe: à propos de 25 enfants et adolescents.

OBJECTIVE: To evaluate neurological tolerance during limb lengthening in children and adolescents. MATERIAL AND METHODS: Retrospective study of 25 children and adolescents [15 girls, 10 boys; mean age 11.3 years, range 5-18 years] undergoing limb lengthening. Limbs involved were the femur [11 cases], tibia [10 cases], radius [3 cases] and humerus [1 case], and lengthening was 1 mm/day, adjusted depending on clinically and electrophysically observed alterations during weekly surveillance. RESULTS: Lengthening in 8 cases was accompanied by electrophysiological deterioration and in 1 by clinical alteration without electrophysiological anomaly. Eight of 25 lengthenings had to be slowed and 1 discontinued. DISCUSSION: This work confirms poor neurological tolerance with limb lengthening. It shows the interest of clinical and electrophysiological monitoring in preventing severe neurological accidents with such lengthening.

[Dyggve-Melchior-Clausen syndrome: differential diagnosis of mucopolysaccharidosis type IV or Morquio disease]. / Le syndrome de Dyggve-Melchior-Clausen: diagnostic différentiel de la mucopolysaccharidose de type IV ou maladie de Morquio.

UNLABELLED: Dyggve-Melchior-Clausen syndrome (DMCS) is an autosomal recessive skeletal dysplasia. Clinical and radiological similarities with Morquio's syndrome can initially lead wrongly to this diagnosis. CASE REPORT: A nine-year-old boy had mental retardation and progressive postnatal dwarfism. Platyspondyly and dysplastic epiphyses and metaphyses resembled those of Morquio's disease; however, clinical and radiological data led to the diagnosis of DMCS. CONCLUSION: Clinical and paraclinical features allowing the differentiation of Morquio's syndrome and DMCS are discussed. Initial clinical presentation may be similar, but the intellectual prognosis is different.

Follow-up study of severe slipped capital femoral epiphysis treated with Dunn's osteotomy.

This study evaluated long-term clinical and radiologic results of Dunn's osteotomy carried out in 50 consecutive cases for severe slipped capital femoral epiphysis. The results of all the patients were analyzed with 4 years 6 months of follow-up. The clinical results were very good and good in 90% of the patients and were fair or poor in 10% of the patients. Seven patients had an important complication (total necrosis or chondrolysis), and the clinical evolution was studied. Dunn's procedure seems to be adequate to correct severe slips.