PubMed HealthSearch

Biomedical subjects

J A Fixsen

Publications and source records attributed to J A Fixsen.

At least 19 recordsLinked to original sources

Management of forme fruste fibular hemimelia.

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. Leg length inequality may require epiphysiodesis, leg lengthening, or simply a shoe raise. Only very occasionally, when the foot is nonfunctional, is amputation through the ankle undertaken.

Adolescent

A review of 240 patients undergoing distraction osteogenesis for congenital post-traumatic or postinfective lower limb length discrepancy.

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. RESULTS: Limb length discrepancy was reduced within 2 cm (1.6 percent or lower) of the normal contralateral leg in 249 (89 percent) lengthenings in 208 patients. The average time spent with the fixator in situ was 186.4 days (range, 94 to 617 days), with an average healing index of 35.3 days per cm (range, 26 to 43 days per cm). Femoral osteotomies or corticotomies healed faster than tibial osteotomies or corticotomies. The healing index in post-traumatic and postinfective LLD was significantly lower than in congenital conditions. The younger patients showed a significantly decreased time to bone healing and fewer complications. CONCLUSIONS: A significantly greater number of difficulties were found in patients whose lengthening exceeded 18 percent of the original length of bone. Bifocal lengthenings healed significantly faster than single level ones. The three external fixators used were equally effective for lengthenings of less than 20 percent. The Ilizarov and the Monticelli-Spinelli circular fixators were associated with a significantly decreased number of complications when lengthenings above 20 percent were performed.

Adolescent

The prognosis for walking in osteogenesis imperfecta.

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. Both classifications helped to predict eventual walking ability. We found that independent sitting by the age of ten months was a predictor for the use of walking as the main means of mobility with 76% attaining this. Of the patients who did not achieve sitting by ten months, walking became the main means of mobility in only 18%. The developmental pattern of mobility was similar in the rodded and non-rodded patients.

Adolescent

Distraction osteogenesis in congenital limb length discrepancy: a review.

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. A minority of studies focus specifically on limb lengthening for congenital defects. Most series simply include some congenital patients, but it is difficult to retrieve the data, and to generalize from them. The amount of lengthening with an acceptable complication rate should not exceed 25%; of the initial bone length. Even using circular frames with small pins, practically all patients may be expected to develop at least one complication each, ranging from pin tract infection to the necessity of carrying out additional unplanned operative procedures either during or after the treatment period. The prevalence of major complications seems to be correlated with the complexity and the duration of the treatment. The functional outcome and the psychological problems associated with a lengthy procedure, which may require long periods of repeated hospitalization, have only rarely been studied. However, it appears that prolonged strength loss is frequent, and that significant psychological morbidity is experienced. Patients and their families should be counselled before and during the procedure on these lesser known aspects of callotasis lengthening.

Arm

Hunter's syndrome as a cause of childhood carpal tunnel syndrome: a report of three cases.

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.

Carpal Tunnel Syndrome

Natural history and treatment of instability of the hip in proximal femoral focal deficiency.

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. Cervical instability was difficult to treat operatively, but subtrochanteric instability fused spontaneously in 30% of cases and responded well to operative treatment when necessary. For unilateral failure of hip joint formation, various surgical procedures were used to assist prosthetic fitting, but bilateral cases were treated nonoperatively.

Adolescent

Knee in congenital short femur.

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. We assessed knee function in a group of 24 patients with CSF. Detailed evaluation of knee stability showed that not all knees were unstable; even when knees were unstable, the degree of instability was highly variable. No patient complained of knee pain or other knee symptoms.

Adolescent

Radiographic features of limb lengthening in children.

We report our experience in radiographic imaging in limb lengthening procedures using callotasis for limb lengthening discrepancy with the Orthofix dynamic monoaxial external fixator. Seventeen patients (average age at operation 10.8 years) completed 22 bone segments (7 femora, 11 tibiae, 4 ulnae) lengthening procedures. The average time for formation of good medullary bridging was over 6 months. The commonest radiographic abnormality was a periosteal reaction around the pin sites seen in 14 patients, while evidence of pin loosening was present in 10. All patients with pin loosening also showed periosteal reaction which pre-dated the loosening in 5 of 12 patients by an average of 47 days. Angulation was detected in 9 patients in the a.p. plane, and in 6 in the lateral plane. Six patients developed a ragged radiolucent region through the newly forming bone at an average of 95 days from the corticotomy but with uneventful healing.

Adolescent

Imaging in bone lengthening. A review.

Limb lengthening using callotasis needs careful preoperative planning and great care during the lengthening period. The most common method used to monitor limb lengthening is plain radiography, but the time of frame removal is still left to the judgment of the surgeon. This paper reviews some of the methods used experimentally and clinically to monitor the process, outlining their possible advantages and disadvantages for application in routine clinical practice. Considering availability, cost, and safety, an imaging protocol could involve preoperative assessment with computed tomography scan; immediate postoperative anteroposterior (AP) and lateral radiographs; weekly ultrasound scanning for the 1st 8 weeks; and monthly AP and lateral radiographs during the lengthening phase and if there is any clinical suspicion of complication during the consolidation phase. If available, dual energy bone densitometry can be performed every 2 to 4 weeks from 8 weeks, until removal of the fixator.

Absorptiometry, Photon

Distal fibulectomy for Ewing's sarcoma.

We treated five children with non-metastatic Ewing's sarcoma of the distal fibula by distal fibulectomy. At a mean of eight years after surgery they all had nearly normal levels of painfree function. Distal fibulectomy is a good alternative to the more radical amputations recommended in the past.

Adolescent

Revision of failed open reduction of congenital dislocation of the hip.

We report a review of 33 hips (32 patients) which had required repeat open reduction for congenital dislocation of the hip. They were followed up for a mean of 76 months (36 to 132). Factors predisposing to failure of the initial open reduction were simultaneous femoral or pelvic osteotomy, inadequate inferior capsular release, and inadequate capsulorrhaphy. Avascular necrosis had developed in more than half the hips, usually before the final open reduction. At review, 11 of the hips (one-third) were in Severin grade 3 or worse; five had significant symptoms and only ten were asymptomatic and radiographically normal. Once redisplacement has occurred after primary open reduction, attempts to reduce the head by closed means or by pelvic or femoral osteotomy are usually unsuccessful and a further open reduction is necessary.

Child, Preschool

Ultrasonographic appearance of regenerate bone in limb lengthening.

The appearance of regenerate bone during the process of limb lengthening using a mono-axial device was studied using radiographs and ultrasound scanning in nine patients. The corticotomy site initially appeared as a sonolucent area. Poorly organized echogenic foci were detectable 2 weeks after the distraction was begun. After 4 weeks, these areas became aligned. At 7-8 weeks, a clear impression of a new cortical margin was seen. The formation of a medullary canal started at this point, progressing to a radiographically evident canal. Ultrasound scanning can reduce considerably exposure to ionising radiation in patients undergoing limb lengthening. Accurate measurements are possible in the early stages, and ossification can be monitored. Axial deviation can be seen but not evaluated. The maturity of the regenerate bone still has to be assessed clinically.

Bone Lengthening

Lengthening of congenital limb length discrepancy using callotasis: early experience of the Hospital for Sick Children.

Seventeen patients were reviewed after callotasis lengthening for congenital limb length discrepancy. The average age at lengthening was 10.8 years. Length discrepancy before lengthening ranged from 4.5 cm to 12 cm for the leg, and 24 mm to 30 mm for the forearm. At completion of the lengthening process, all but one patient had their discrepancy corrected successfully. The difficulties encountered were classified into problems, obstacles and complications. All patients suffered from superficial infection, but in only one case did this become a complication, resulting in a residual femoral discrepancy of 2 cm. Of the other three patients who suffered a complication, one fractured through the newly formed bone. The fracture was treated conservatively. In two further femoral lengthenings, the fixator had to be exchanged under general anaesthesia because it had reached its maximum excursion. Callotasis appears to be a safe and reliable method for correcting congenital limb length discrepancy in children.

Adolescent