ISSN:0271-6798    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Arthrogryposis is a sign associated with many specific conditions and syndromes. It is a term used to describe the presence of multiple joint contractures that are present at birth. It can be seen in isolation or in association with other congenital abnormalities as part of a syndrome with or without central nervous system involvement. The exact pathogenesis of arthrogryposis is unknown, but all involve fetal akinesia (decreased fetal movement) with subsequent joint contractures. In this article I describe the causes, genetic aspects, classification, and approach to diagnosis.

ISSN:0271-6798    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Fifty-two patients with arthrogryposis multiplex congenita were followed up for 1 to 36 years. There were six twin pregnancies, and delivery was complicated by breech position in 16 cases. In 19 cases the cause was atrophy of the alpha motoneurons of the spinal cord, detected by electromyography. Six patients did not achieve walking ability. Severe disability for other reasons was noted in two patients who had bilateral rigid extension contractures of the elbows and in six patients whose spinal deformities interfered with the balance of the trunk. Treatment of flexion contractures of the hips and knees seemed to be important in case it promotes the walking ability. Spinal deformities interfering with the balance of the trunk should be treated operatively. Restoration of elbow flexion was the main goal in operative treatment of the upper extremities. The intelligence of the patients was slightly above normal. The psychological analysis revealed significant diverging features compared with average population in testing situation. Socially the patients seemed to cope well.

ISSN:0271-6798    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

A cross-sectional study of 28 patients with multiple congenital contractures of miscellaneous origin is presented. We describe the clinical, genetic, and neurological diagnosis and the involvement of upper and lower extremities and spine. All treatments that patients received so far as well as functional outcome were studied. We compared these factors in children with anterior horn cell degeneration (AHCD) or amyoplasia with those of children with contractures of other origin. A correct genetical diagnosis of multiple congenital contracture is important because children with AHCD will need more extensive treatment than others, and their functional outcome seems to be worse.

ISSN:0271-6798    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

The involvement of the upper limb in arthrogryposis multiplex congenita for many patients means a far-reaching dependency on outside help. The extension contracture of the elbow joint especially makes it impossible to reach the mouth or to perform hygienic necessities. Therefore, the rehabilitation program includes an improvement of passive elbow flexion by capsulotomy or of active flexion by triceps transfer if possible, or both. In bilateral involvement, the optimal solution is to have one arm in flexion for reaching the head and mouth passively or even actively and one arm in extension for hygienic necessities. From 1973 to 1993 we performed 22 releases of the elbow contracture in 16 children. An additional triceps transfer was performed in five elbows. The overall results showed a marked increase of the range of motion and a functional improvement concerning the daily activities (in 17 children). In 5 children there was no gain but also no loss of functional capacities. In 3 of 5 children with an additional triceps transfer, an improvement of active flexion was attained. Pre- and postoperative physiotherapy is at least as important as the operative procedure itself.

ISSN:0271-6798    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

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. All were treated initially by physiotherapy and splintage, which was successful in all except 1 patient in the extended-knee group, whereas only 7 of 26 knees responded to physiotherapy and splintage alone in the flexed-knee group. Walking ability in the extended-knee group was high; 8 of 9 were community walkers with or without walking aids and orthoses and only one was a therapeutic walker. By contrast, in the flexed-knee group, despite posterior release surgery, which sometimes had to be repeated, only 6 of 13 patients were community walkers at follow-up, 2 were household walkers, 3 were therapeutic walkers, and 2 had stopped walking in adolescence and preferred to use a wheelchair full time. Long-term splintage is recommended but does not always prevent recurrence of deformity. Bony surgery was used only toward the end of growth or in one case when very severe deformity necessitated its use at an early age and it subsequently had to be repeated.Despite their severe handicap and multiple deformities, this group of children show a remarkable determination to walk with or without walking aids and orthoses:

ISSN:0271-6798    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

The purpose of this study was to investigate the efficacy of an external fixator (Ilizarov apparatus) for the treatment of severe joint contractures in patients with arthrogryposis multiplex congenita. Thirteen knees and 16 feet were treated in 13 patients at an average age of 11.9 years. The knee flexion contractures were corrected from 38.9opreoperatively to a mean of 6.5opostoperatively and to 17.3oat follow-up (34 months). In the foot deformities, the equinus position was corrected from 29.7oto 7.8oon average. This external fixator is an efficient tool for correction of deformities in arthrogryposis.

ISSN:0271-6798    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

We treated 34 femoral shaft fractures in 32 children with elastic intramedullary nailing using titanium rods. The 24 boys and 8 girls ranged from 6–17 years. All fractures united. The weight bearing was authorized at an average of 67 days and was complete at an average of 89 days. All children returned to school before the end of the second postoperative month. At an average of 2.5 years, no rotational malalignment was present. Neither anteroposterior nor frontal malalignment of more than 2° was noticed. A leg length discrepancy of more than 10 mm was present in 3 patients (8%). No injury of femoral head vascularization or of the proximal or distal growth plate was noticed. In our experience, this technique seems to be safe for the surgical treatment of the immature femoral shaft fractures. The elastic properties of titanium provide a very good stability of the montage, when the technique is correctly performed.

ISSN:0271-6798    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

We have reviewed 48 children's diaphyseal fractures of the femur and tibia managed with the Orthofix Dynamic Axial Fixator between 1987 and 1994. The indications for external fixation included open fractures, multiple injuries, failed conservative management, and unstable fracture configurations. All fractures healed without further surgical intervention, and the incidence of serious complications was low. Although pin track sepsis was common, compromise of the fixation as a result of this occurred in only one case. We conclude that use of the Orthofix for children's fractures is a safe and effective management option.

ISSN:0271-6798    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Twenty-four hip joints in 20 children with spastic cerebral palsy were assessed by standardised three-dimensional reconstructions from computed tomographic scans. All the hip joints showed a channel-like bony deformity of the acetabulum along which the femoral bead had slid out, indicating a unidirectional instability. The channel was oriented along the longitudinal axis of the body within a sector of 25°anteroposteriorly. In relation to the pelvis, the dislocation was directed more dorsally by 20°as a result of a flexion contracture of the hip. The size of the femoral head corresponded with the size of the acetabulum in every case, even in long-standing dislocations.

ISSN:0271-6798    

出版商:OVID    

年代:1997

数据来源: OVID