Publications by authors named "Fixsen J"

Major congenital limb deficiencies are rare and the experience of most orthopaedic surgeons of their management will be small. The suggestion of the establishment of special limb deficiency clinics seems a sensible way of collecting the necessary expertise together in one place in order to advise patient and parents on the long-term management, throughout life, of their problems. Advances in imaging have led to prenatal diagnosis, which produces very significant problems in counselling parents before their child is born.

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This paper presents a review of the literature, describes the principal author's (B.R.) personal experience and provides the results of the European Paediatric Orthopaedic Society (EPOS) multicenter study.

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This study was designed to analyze the different therapeutic methods used by European Paediatric Orthopaedic Society members from 13 countries for congenital pseudarthrosis of tibia. The treatment data of 340 patients who underwent 1287 procedures for this condition were analyzed. The essential findings were that the method of choice needed to approach the biological problem with the aims of: (1) resecting the pseudarthrosis to provide stability, the basic requirement for bony consolidation; (2) correcting length discrepancy and axial deformity; (3) achieving fusion; and (4) solving the additional problems around the main deformity such as alignment, leg length discrepancy and ankle valgus.

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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.

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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.

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Sjögren-Larsson syndrome is a rare autosomal recessive neurocutaneous disorder in which the combination of spasticity, ichthyosis, and mental retardation commonly result in patients being wheelchair bound. We reviewed a cohort of children with Sjögren-Larsson syndrome who were successfully managed with early physiotherapy and later soft-tissue surgery. The favourable outcome seen in these patients should encourage orthopaedic intervention in suitable cases.

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Congenital convex pes valgus.

J Bone Joint Surg Br

March 1999

Congenital convex pes valgus (congenital vertical talus) is a rare condition. We reviewed ten feet in seven patients who had had surgical correction. All had been operated on by the senior author (JF) and the same surgical technique was used throughout, incorporating transfer of the tibialis anterior to the neck of the talus.

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We report two cases of children with multiple hereditary osteochondromatosis (MHO) in whom painful restriction of hip movement developed due to intraacetabular osteochondromata. Excision of the lesions relieved pain and restored joint movement after 14 and 3 months' follow-up, respectively. Long-term follow-up of these patients is essential.

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Subluxation of the hip presenting for the first time in a child over the age of four years is rare. We report ten cases treated over nearly 11 years by the senior author (JAF). We describe the surgical procedures and the results, at maturity, of nine of the ten patients.

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We describe the management of significant knee deformity in 44 knees of 22 patients suffering from classical arthrogryposis multiplex congenita (amyoplasia congenita). Follow-up ranged from a minimum of 18 months to 19 years 3 months, with an average 7 years 8 months. Thirteen patients showed fixed flexion of the knees at birth and 9 showed fixed extension.

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Four case histories are used to illustrate orthopaedic complications of vascular access in neonates. The two main pathologies, extravasation and thrombosis, are discussed and the management of each outlined. Mechanisms of avoidance of such complications are described and advocated.

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Seven patients with osteogenesis imperfecta who have undergone humeral rodding were reviewed. Satisfactory functional results were obtained in six of seven patients. We discuss the indications for surgery in our unit, the complications, and the results in comparison with those of other centres.

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This paper reviews the technique of callotasis used to correct limb length discrepancy due to congenital causes. Many more lower than upper limb lengthening procedures have been reported. Despite the low complication rate reported by the originators of the technique, patients undergoing limb lengthening because of congenital problems are at significant risk of pin tract sepsis, joint contracture, nerve palsies, angular deformities and fracture through the regenerate bone.

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We report a postal survey of 59 families of children with osteogenesis imperfecta. From the 51 replies we collected data on developmental milestones and walking ability and related them to the Sillence and the Shapiro classifications of osteogenesis imperfecta. Twenty-four of the patients had been treated by intramedullary rodding.

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Background: We reviewed 281 lower limb lengthenings in 240 patients treated at three centers for congenital, post-traumatic, or postinfective limb length discrepancy (LLD) in the period 1984 to 1992.

Study Design: A retrospective review was done of hospital charts and radiographs of patients who had completed lengthening at least 12 months prior to the beginning of the study. The variables studied included patients' age at operation, the bone segment lengthened, whether a corticotomy or an osteotomy had been done, the amount of lengthening planned and achieved, the level(s) of corticotomy or osteotomy, the external fixator used, and the difficulties encountered.

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Children with fibular hypoplasia and three-or four-ray feet have a forme fruste of congenital short tibia with absent or dysplastic fibula. We outline the criteria for management of this condition. Although fibular deficiency can be considered benign, two thirds of our patients underwent operation.

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Seven patients (average age, 9.2 years; range, 7.6-10.

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Carpal tunnel syndrome is common in adults but rare in children. It is known to occur in association with the mucopolysaccharidoses and other systemic conditions in childhood. We describe three children with a mild variant of mucopolysaccharidosis II (Hunter's syndrome) in whom disabling median nerve compression was treated by standard decompression.

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The abnormalities of the knee associated with congenital short femur (CSF) and proximal femoral focal deficiency (PFFD), have been recognized for many years. The emergence of new and more effective lengthening techniques that can correct major congenital leg length discrepancies has made the abnormalities of the knee much more relevant. Some investigators have reported a high incidence of knee subluxation and dislocation during lengthening procedures in patients with CSF, attributing the incidence to knee anomalies.

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We studied 67 patients with 78 affected femurs to determine the natural history of proximal femoral focal deficiency (PFFD) with respect to hip instability and to establish guidelines for management. Using the classification systems of Aitken and Fixsen and Lloyd-Roberts as applied to a radiograph taken at age 12-15 months, we could accurately predict development of hip instability, defined as formation of a pseudoarthrosis or complete failure of hip development. The pseudoarthrosis occurred either at the cervical or subtrochanteric level of the femur.

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