Results of talectomy for inveterate or recurrent clubfoot.

INTRODUCTION: The aim of the present study was to define indications for talectomy in congenital paralytic, dystrophic or idiopathic, inveterate or recurrent, clubfoot. HYPOTHESIS: Talectomy is a valid option for paralytic, dystrophic or idiopathic, inveterate or recurrent, clubfoot. PATIENTS AND METHODS: A single-center retrospective series comprised 52 clubfeet in 31 patients. Etiology was paralytic in 34 feet (65%) (17 arthrogryposes, 10 myelomeningoceles, 4 encephalopathies, 3 peripheral neuropathies), dystrophic in 6 (12%) and idiopathic in 12 (23%). In 27 feet, there was history of surgery (52%). Mean age at talectomy was 4.7 years. In 45 feet (87%), there were associated procedures (soft-tissue release, tendon surgery, calcaneal or lateral arch osteotomy, tibiocalcaneal fusion) and talectomy was isolated in 7 feet (13%). Mean follow-up was 9 years. Final assessment was based on the modified Ghanem and Seringe classification (G&S) and the Ankle-Hindfoot Scale (AHS). RESULTS: All feet required at least one complementary procedure, either in the same step or as revision. Revision surgery was performed in 17 cases (33%), including all 7 feet with isolated talectomy (7 calcaneal tendon lengthenings, 10 mid- or hind-foot osteotomies, 6 tibiocalcaneal fusions, one calcaneocuboid fusion, and 2 progressive corrections by external fixator). Finally, 33 feet (63%) had good G&S results, 44 (85%) were pain-free, and 40 (77%) were plantigrade. DISCUSSION: Talectomy for paralytic or dystrophic inveterate or recurrent clubfoot provided satisfactory medium-term results. Associated to other procedures, it achieves a pain-free plantigrade foot in most cases. Tibiocalcaneal fusion has an analgesic effect. Talectomy may, however, not be indicated in idiopathic clubfoot, given the patients' high functional demand and the existence of alternative treatments. LEVEL OF EVIDENCE: IV, retrospective series.

Long-term results of ankle arthrodesis in children and adolescents with haemophilia.

INTRODUCTION: Ankle arthrodesis in patients with haemophilia is a joint-non-preserving treatment option often resulting in substantial pain relief and termination of haemarthrosis by osseous consolidating of the joint. However, limited data are available regarding ankle arthrodesis in young patients. AIM: The aim of this study was to evaluate the long-term clinical and radiographic results with a minimum follow-up of ten years in children with haemophilia who underwent ankle arthrodesis. METHODS: We retrospectively reviewed the results of ankle fusions performed in young haemophiliacs in our department between 1980 and 2006. The Méary procedure was used for arthrodesis, performed on patients with closed growth plates. Only one patient had no fixation, due to being only six years old. The modified American Orthopaedic Foot & Ankle Society (AOFAS) hindfoot score was calculated at last follow-up, and standard radiographic evaluations were performed. RESULTS: A total of 22 ankles were analysed from 17 patients. The mean follow-up was 19.7 ± 8 years. The mean age of the patients was 15.5 (6-23) years at the time of index surgery. There were no intra-operative or peri-operative complications related to ankle arthrodesis. The mean modified AOFAS score at last follow-up was 83 ± 10.5. Radiographic assessment demonstrated solid osseous fusion at the arthrodesis site, with no axis deformities. Two patients developed secondary subtalar arthrosis, treated by subtalar arthrodesis six years after initial ankle arthrodesis in one case. CONCLUSION: In our study, tibiotalar arthrodesis in young patients with haemophilia resulted in good long-term functional outcome with a low surgery-related complication rate.

Ischiovertebral dysplasia: a retrospective analysis of 30 consecutive cases pointing out the specifics and risks of the spine management.

STUDY DESIGN: A review of clinical publications, current knowledge, and recent developments regarding the etiology of ischiovertebral dysplasia was combined with a clinical review of the condition. OBJECTIVE: To acquaint orthopedic spine surgeons with identification patterns of ischiovertebral dysplasia in order to provide them with guidelines about spine management and which complications to expect. SUMMARY OF BACKGROUND DATA: Ischiovertebral dysplasia is a rare skeletal dysplasia that may appear in a sporadic fashion or be inherited with an autosomal dominant inheritance pattern. It is defined by the association of an ischiopubic ramus hypoplasia and a vertebral dysplasia. It leads to a specific spine deformity whose management and complications should be clarified. METHODS: Thirty consecutive patients from 0 to 31 years of age with ischiovertebral dysplasia were included from 5 centers specialized in congenital spinal deformities. Frontal and sagittal Cobb angles before treatment, natural history of the curves, therapeutic options, and their complications were systematically analyzed. RESULTS: All the patients had a vertebral dysplasia and 28 of them developed a spinal deformity. This deformity was an extremely severe thoracic kyphoscoliosis in 25 cases. The other deformities were a thoracolumbar scoliosis in 1 case and a thoracolumbar kyphosis in 2 cases. The management of the thoracic kyphoscoliosis was always challenging and complications included death by respiratory failure (3 cases) and neurological impairment (9 cases). CONCLUSION: Recognizing the occurrence of ischioverterbral dysplasia is very important to allow for dedicated treatment. The authors advocate preoperative distraction and circumferential fusion to prevent progression of the curve and to avoid the potentially fatal sequelae associated with this disorder. LEVEL OF EVIDENCE: 4.

Coxa vara in chondrodysplasia: prognosis study of 35 hips in 19 children.

BACKGROUND: To better understand anatomical and functional outcomes of coxa vara in chondrodysplasia according to the initial presenting hip morphology, disease type, and impact of surgery. METHODS: Clinical and radiographic records of 19 children (35 hips) diagnosed with coxa vara and with osteochondrodysplasia were reviewed. We classified the hip radiographic findings into 2 groups: (a) group I, coxa vara with a fragmented and/or nonossified head; and (b) group II, coxa vara with a regular femoral head. Surgical indications in coxa vara included decreased range of hip motion (usually diminished abduction, extension, and internal rotation), coxa vara with progression documented on regular follow-up hip radiographs, and/or severe coxa vara with a Hilgenreiner epiphyseal angle (HEA) of 60 degrees or more. Follow-up was until the completion of growth and, for some patients, into early adulthood. Mean follow-up was 8 years. RESULTS: Twenty-five hips were operated on in 13 patients. In 23 hips, the procedure was a valgus osteotomy fixed by pins and wire. A pelvic extension osteotomy without valgus osteotomy was performed in one patient (2 hips). Coxa vara recurred in 7 hips. In 4 of these hips, repeat surgery with hypervalgus osteotomy was indicated to stop epiphyseal slipping (3 hips) or to improve the arc of motion (1 hip). Functional outcomes were poor in coxa vara associated with poor epiphyseal development (nonossified or fragmented epiphysis) as seen in spondyloepiphyseal dysplasia congenita, spondyloepimetaphyseal dysplasia, Kniest disease, and multiple epiphyseal dysplasia. Coxa vara with physeal instability as observed in spondylometaphyseal dysplasia resulted in deformity recurrence postoperatively during growth. In contrast, outcome was better in cases of coxa vara with nonphyseal/nonepiphyseal involvement, that is, good femoral head morphology, stable physis, and good articular cartilage, as seen in cases of metaphyseal dysplasia and cleidocranial dysplasia. CONCLUSIONS: Coxa vara with physeal and epiphyseal involvement and severe impairment of the articular cartilage has a poor prognosis even after reconstructive surgery. In coxa vara with an abnormal physis, there were numerous postsurgical recurrences of the deformity during growth if the physis was not stabilized at the time of valgus osteotomy. In these cases, we should delay osteotomy until an HEA greater than 60 degrees. Coxa vara in which only the metaphysis of the femoral neck is involved, the deformity is milder and often requires no treatment. Indications for surgery in this group are increasing coxa vara, Trendelenburg gait, or an HEA greater than 60 degrees.

Correction of flexible thoracic scoliosis below 65 degrees--a radiological comparison of anterior versus posterior segmental instrumentation applied to similar curves.

UNLABELLED: Direct comparison of the correction of scoliosis achieved by different surgical methods is usually limited by the heterogeneity of the patients analyzed (their age, curve pattern, curve magnitude, etc.). The hypothesis is that an analysis of comparable scoliotic curves treated by different implant systems could detect subtle differences in outcome. The objective of this study was therefore: (1) to measure the 3D radiological parameters of scoliotic deformity and to quantify their postoperative changes, and (2) to compare the radiographic results achieved with one anterior and one posterior instrumentation methods applied to similar curves but representing different mechanisms of correction. MATERIAL AND METHODS: The clinical notes and radiographs of 46 patients operated on for adolescent idiopathic scoliosis were reviewed. The inclusion criteria consisted of: a single thoracic curve, right convex, a frontal Cobb angle minimum of 45 degrees and a maximum of 65 degrees , flexibility on a lateral bending test of more than 30%, and a Risser test value of between 1 and 4. The operative procedures were: Cotrel-Dubousset instrumentation (CDI) for 25 patients (the CD group) and correction by anterior instrumentation (Pouliquen plate) for 21 patients (the ANT group). Preoperative and postoperative long cassette standing antero-posterior and lateral radiographs were examined. The frontal and sagittal thoracic Cobb angle, apical vertebra transposition (AVT), apical vertebra rotation (AVR), lowest instrumented vertebra (LIV) tilt, C7 vertebra shift and rib cage shift (RCS) were all compared. A computed reconstruction was produced with Rachis-91 software. Vertebral axial rotation angle was evaluated throughout the spine. RESULTS: Postoperative assessment revealed a mean correction of the frontal Cobb angle of 37.0 degrees for the CD group and 41.0 degrees for the ANT group. The AVT operative correction was 45.8 and 42.7 mm, respectively, and AVR correction was 1.8 and 12.6 degrees , respectively. The postoperative change of the sagittal Th4-Th12 Cobb angle was not significant for any method but it was significant (P=0.05) for the CD group if the curves were divided preoperatively into hypokyphotic and normokyphotic subgroups and then analyzed separately. Computed assessment demonstrated a correction of segmental axial rotation of more than 50% in the main thoracic curve in the ANT group, significantly more than that in the CD group (P<0.001). CONCLUSIONS: Anterior instrumentation provided better correction of the vertebral axial rotation and of the rib hump. CD instrumentation was more powerful in translation and more specifically addressed the sagittal plane: the postoperative thoracic kyphosis angle increased in the hypokyphotic curves and slightly decreased in the normokyphotic curves.

Surgical treatment of high-grade lumbosacral spondylolisthesis in childhood, adolescent and young adult by the "double-plate" technique: a past experience.

Between 1979 and 1996, 40 patients with high-grade lumbosacral spondylolisthesis were treated in our institution using a newly designed osteosynthesis device. The mean age was 13 years and 6 months, and the mean follow-up was 18 years. Combined posterior decompression and anterior reduction, instrumentation and fusion of the slippage were performed in all cases. The technique includes reduction of the slippage by means of an anteriorly placed plate that engages two screws, previously placed during the posterior approach, going through the S1 vertebra. Progressive compression applied on the plate by the screws achieves reduction. Complete fusion was obtained in all 40 patients. Twelve patients presented a postoperative radiculopathy, from which only ten recovered completely. There were six L4-L5 annulus lesions, responsible for instability, produced by the plate. We report five late infections. Thirty-five of the forty patients were asymptomatic at the latest follow-up. The double compressive plate technique proved to be effective in obtaining lumbosacral fusion and optimal slippage reduction. However, the high rates of neurological and infectious complications preclude recommendation of this technique in its present form.

Delta1-pyrroline-5-carboxylate synthase deficiency: neurodegeneration, cataracts and connective tissue manifestations combined with hyperammonaemia and reduced ornithine, citrulline, arginine and proline.

UNLABELLED: Delta1-pyrroline-5-carboxylate synthase (P5CS) catalyses the reduction of glutamate to Delta1-pyrroline-5-carboxylate, a critical step in the biosynthesis of proline, ornithine and arginine. Recently, we reported a newly recognised inborn error due to deficiency of P5CS in two sibs, one presenting at birth with hypotonia, dysmorphic signs, pes planus and clonic seizures. Both developed progressive neurodegeneration and peripheral neuropathy, joint laxity, skin hyperelasticity and bilateral subcapsular cataracts. Their metabolic phenotype includes mild hyperammonaemia, hypo-ornithinaemia, hypocitrullinaemia, hypo-argininaemia and hypoprolinaemia. Incorporation of 3H-proline into protein was deficient in fibroblasts incubated with 3H-glutamate. Both patients are homozygous for the missense mutation R84Q in P5CS. Here, we describe the clinical phenotype of the sibs in detail and show that a relative deficiency of urea cycle intermediates (ornithine, citrulline and arginine) during fasting periods results in a paradoxical hyperammonaemia. Furthermore, we show the results of ornithine loading tests and indirect enzyme studies corroborating the biological significance of the defect in P5CS in vivo. CONCLUSION: The metabolic phenotype of Delta1-pyrroline-5-carboxylate synthase deficiency is easily missed. The combination of low levels of ornithine, citrulline, arginine and proline plus a tendency to hyperammonaemia or one of the above together with a clinical phenotype of neurodegeneration with peripheral neuropathy and/or cataracts and connective tissue manifestations should suggest this disorder. Early recognition would allow a therapeutic trial with citrulline and proline.

Long-term outcome in Desbuquois dysplasia: a follow-up in four adult patients.

Desbuquois dysplasia is a rare chondrodysplasia characterized by short stature, joint laxity, and specific radiographic findings. We report the natural history of four patients (three boys and one girl) with Desbuquois dysplasia ages 16-22 years. The mean height in adulthood was 114 cm (-8.5 SD) with progressive deceleration of the growth curve from birth (-4 SD) to adulthood. Obesity was noted consistently and facial abnormalities were still present but less obvious than in childhood. Three of four patients had mental retardation of varying degree. Hyperlaxity was persistent but limited motion of various joints was also noted. Orthopedic complications included coxa vara or valga (3/4), scoliosis (3/4), marked lordosis (3/4), and ambulatory difficulties (3/4). Surgical treatment was necessary for all four patients, involving large joints, spine and hands. Other complications included acute open-angle glaucoma secondary to a congenital malformation of the angle in one case. In addition to consistent radiological findings, elevated greater trochanter, generalized osteoporosis especially of the spine, scoliosis and/or lordosis, wide metaphyses, flat epiphyses, and coxa vara or valga were part of the natural history of the disorder. Our study emphasizes the care of older patients with Desbuquois dysplasia.

Buruli's ulcer: three cases diagnosed and treated in France.

The authors report three cases of skin ulcer, called Buruli's ulcer. This is a tropical disease, caused by Mycobacterium ulcerans. The diagnosis is often belated, because the infection is rare in Europe. Diagnosis is based on the clinical aspects, and can be confirmed by a biopsy and a molecular study. A culture is needed to test antibiotics. Treatment is always surgical, including a wide excision of all lesions, and specific oral antibiotics for 6 months.