We report four patients with a form of Ehlers-Danlos syndrome associated with soft-tissue contractures from birth and skin hyperalgesia. In early infancy, these cases were thought to be forms of arthrogryposis multiplex congenita, Larsen's syndrome or Marfan's syndrome. The characteristic triad of signs of Ehlers-Danlos disease gradually appeared from four to six years of age, allowing us to establish the correct diagnosis. We discuss the differential diagnosis of these connective-tissue disorders and the problems of the orthopaedic treatment of the associated joint deformities. Ehlers-Danlos syndrome is a heterogeneous group of metabolic diseases of collagen and our cases constitute a group which is distinct from the conventional types

We investigated the pathogenesis of soft-tissue contracture in club foot, using immunohistochemistry to study 41 biopsy specimens and 12 normal deltoid ligaments from cadavers. Five biopsy specimens were studied by electron microscopy (EM) to determine the presence of myofibroblasts. All 41 specimens of club foot stained positively for vimentin as against only one of the 12 control specimens. By contrast, there was no difference in staining for desmin or α-smooth muscle actin. EM showed some variability in the appearance of ligamentous cells. Most contained bundles of microfilaments in the cytoplasm and many had abundant pinocytotic vesicles, but no basal lamina or plasmalemmal attachment plaques. Cells of the medial ligamentous tissue in patients with club foot contain vimentin and others have myofibroblastic characteristics. Both features may contribute to recurrence after soft-tissue release

1. Six patients are reported in whom ischaemic muscle contractures followed prolonged external pressure. 2. Systemic hypoxaemia may be a predisposing factor in patients with overdoses of central nervous system depressants. 3. If treatment is to be successful, early diagnosis is required, and the most important factor is an awareness of the condition. 4. The treatment of the late case is discussed

Ischaemia is a rare complication of injury to a limb but must be excluded in every case. Two distinct types occur: Type I, where a proximal arterial injury gives rise to ischaemia distally; and Type II, where a direct injury gives rise to ischaemia at the site of the injury. Whatever the nature of the insult, an ischaemic contracture only develops as a result of swelling of the soft tissues where these soft tissues are contained in un unyielding osteofascial compartment. This secondary ischaemia can only be relieved by a timely fasciotomy. The diagnosis of ischaemia in an injured limb and the indications to operate on it can usually be made on clinical grounds alone

A method of treatment of Volkmann's ischaemic contracture is described which retains wrist movement and restores reasonably good function to the hand in suitable cases

Our aims were to describe the distribution of α-smooth muscle actin (SMA)-containing cells in Dupuytren’s tissue in vivo and to determine the effects of selected agents in regulating the expression of SMA in Dupuytren’s cells in vitro. In selected hypercellular zones of Dupuytren’s nodules up to 40% of the cells contained SMA, as shown by immunohistochemistry. A lower percentage (20%) of SMA-containing cells was found in regions of lower cellularity. A notable finding was that treatment in vitro of Dupuytren’s cells with platelet-derived growth factor significantly reduced the content of SMA. Cells from the same patients showed a significant increase in expression of SMA in response to treatment with transforming growth factor, which confirmed recent findings. In addition, interferon-γ, which has been previously used as a treatment for Dupuytren’s disease in a clinical study, had no reproducible effect on the expression of this actin isoform. Our findings are of significance for the conservative management of contractures

We treated 22 children (28 limbs) with diplegic cerebral palsy who were able to walk by the Baumann procedure for correction of fixed contracture of the gastrosoleus as part of multilevel single-stage surgery to improve gait. The function of the ankle was assessed by clinical examination and gait analysis before and at two years (2.1 to 4.0) after operation. At follow-up the ankle showed an increase in dorsiflexion at initial contact, in single stance and in the swing phase. There was an increase in dorsiflexion at initial push-off without a decrease in the range of movement of the ankle, and a significant improvement in the maximum flexor moment in the ankle in the second half of single stance. There was also a change from abnormal generation of energy in mid-stance to the normal pattern of energy absorption. Positive work during push-off was significantly increased. Lengthening of the gastrocnemius fascia by the Baumann procedure improved the function of the ankle significantly, and did not result in weakening of the triceps surae. We discuss the anatomical and mechanical merits of the procedure

The fine structure of palmar fascia from patients with Dupuytren's contracture (DC) was compared with that from patients with carpal tunnel syndrome (CTS). In contrast to previous assumptions, the ultrastructure of fibroblasts both in vivo and in vitro from DC and CTS appeared identical, indicating that myofibroblasts are not specific to DC. The major differences between DC and CTS were: 1) a sixfold and fortyfold increase in fibroblast density in cord and nodular areas of DC compared with CTS; 2) a more disorganised pattern of collagen fibrils in DC; and 3) markedly narrowed microvessels surrounded by thickened, laminated basal laminae and proliferating fibroblasts in DC compared with CTS. To account for these morphological changes a hypothesis is presented which proposes that oxygen-free radicals cause pericytic necrosis and fibroblastic proliferation. This hypothesis provides a potential avenue for therapy of DC and other fibrotic conditions

1. A case of Volkmann's ischaemic contracture, in which function was greatly improved by a muscle slide operation, is described. 2. The possibility of regeneration of muscle after ischaemia is reviewed in the light of recent research. 3. Injection experiments suggest that the forearm flexors, like the gastroenemius,are supplied by end arteries

Thirty-two contractures of the quadriceps induced by injection in 17 children have been reviewed. The average age at presentation was 3 years 4 months. All the children had suffered a severe illness within the first few weeks of life and all had received intramuscular injections into the thigh. Three methods of treatment were used: conservative, distal quadricepsplasty, and proximal release. At follow-up none of the patients treated by proximal release had an extension lag, which was present in 70% of those treated by distal quadricepsplasty. Apart from one case with slight improvement in the range of knee flexion, conservative treatment, at best, could only prevent further loss. The clinical presentation and management is described, and the advantages of proximal release are discussed

1. Previous reports of the role of the quadriceps in producing limitation of movement or dislocation of the patella have tended to implicate one particular part of the complex. Experience gained in thirty-five quadricepsplasties in children has shown that all parts of the quadriceps (except vastus medialis) and the ilio-tibial band also may play a part and that each of these must be attended to if a satisfactory result is to be obtained.

2. A simple technique of operation is described and illustrated.

3. Since this paper was prepared, a patient showing features of particular interest has been seen and treated.

1 . A case of plantar Dupuytren's disease in a woman aged twenty-three is described.

2. A review of the literature has shown that the histology and rapid recurrence have led to an erroneous diagnosis of fibrosarcoma and treatment by amputation on several occasions.

1. Two patients are reported with progressive fibrosis of the vastus intermedius muscle causing limitation of flexion of the knee. The first patient, a girl of ten years who was one of binovular twins, noticed difficulty in walking at the age of five, the disability increasing with age. The other girl, aged three, was one of uniovular twins and displayed the abnormality as soon as she started walking.

2. The nature of the condition remains obscure.

3. Surgery is effective, and simple division of the affected muscle segment is recommended. A good range of passive flexion is obtained at once; the active range can be obtained thereafter by physiotherapy. Because this requires the patient's cooperation it is suggested that the best age for surgery is about five or six years.