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

Eight patients with neurofibromatosis presented with symptoms of cervical spine involvement over a period of 17 years, five of them within the second decade of life. The symptoms included neurological deficit in five, a neck mass in four, and deformity in three; only two complained of pain. Osteolysis of vertebral bodies with kyphosis of more than 90 degrees was the most common radiological feature. Posterior fusion failed in the one patient in whom it was performed. Good results were achieved by anterior fusion, alone, or combined with posterior fusion. Surgical complications included one death in a patient with a malignant neurofibroma, and one case of transient neurological deterioration

Aims

After the initial correction of congenital talipes equinovarus (CTEV) using the Ponseti method, a subsequent dynamic deformity is often managed by transfer of the tendon of tibialis anterior (TATT) to the lateral cuneiform. Many surgeons believe the lateral cuneiform should be ossified before surgery is undertaken. This study quantifies the ossification process of the lateral cuneiform in children with CTEV between one and three years of age.

Fifty consecutive comminuted fractures of the femoral shaft were treated by closed unlocked intramedullary nailing. Twelve unstable fractures also had cast-bracing. There were no cases of infection or non-union, and satisfactory results were achieved in 38 fractures (76%). More severe comminution led to a higher incidence of unsatisfactory results, but malrotation deformity was seen more often in less comminuted fractures and appeared to be due to poor operative reduction. Shortening in severe comminution was the main complication and was not controlled by supplementary cast-bracing. Closed unlocked intramedullary nailing is effective for lesser grades of comminution, but fractures with no cortical continuity at reduction should be treated with a locking nail

This paper describes the design, development and early surgical experience with a stereotactic device to allow closed retrieval and interchange of intramedullary rods in children with osteogenesis imperfecta. This relatively atraumatic procedure may allow more frequent rod interchange than with other techniques, lessening the likelihood of deformity and fracture in the unsupported skeleton when the bone has outgrown the intramedullary rod. The procedure was developed by design studies in vitro followed by intramedullary rodding of tibiae of New Zealand white rabbits. It has been used in children 12 times, in six tibiae and six femora: 11 rods have been successfully retrieved, with rod interchange in eight of these cases

Unilateral Colles' fractures in 187 patients over the age of 55 years were studied in a randomised prospective trial: 97 fractures were minimally displaced and were treated either conventionally or in a crepe bandage; 90 displaced Colles' fractures were reduced and of these 47 were treated conventionally while 43 were encouraged to mobilise the wrist in a cast which restricted extension. Early wrist movement hastened functional recovery and led to earlier resolution of wrist swelling. Discomfort was no greater than in patients who were treated conventionally. The bony deformity, which recurred irrespective of the method of treatment, was not adversely affected by early mobilisation

Ten patients with 13 ankylosed elbows after burns are described. Six elbows, fixed in nearly full extension, had almost total functional disability; the other seven had varying amounts of deformity. In five of the 13 elbows there was a continuous bony mass with loss of the joint space; these were treated by a modified excision arthroplasty which restored good movement and useful function, though there was some lateral laxity. Six elbows had a posterior bony block; this was excised, which restored a useful arc of movement. The literature on bony ankylosis after burns is reviewed and the management of these cases in a developing country is described

The changing incidence of idiopathic scoliosis in 672 patients who attended the Edinburgh Scoliosis Clinic between 1968 and 1982 inclusive is reported. Of these patients, 144 had infantile, 51 juvenile and 477 adolescent idiopathic scoliosis. Thirty-seven of the infantile curves progressed and 107 resolved. The relative frequency of both progressive and resolving infantile idiopathic scoliosis (given as a percentage of the total number of patients with idiopathic scoliosis) declined from 41.75 per cent in the four years from 1968 to 1971, to four per cent in the three years from 1980 to 1982. It is suggested that infantile idiopathic scoliosis is a preventable deformity and that the position in which the infant is laid may be a causative factor

Degenerative changes of the first carpometacarpal joint commonly cause pain, weakness and adduction deformity. Many patients respond to conservative treatment, but in resistant cases an abduction wedge osteotomy of the base of the first metacarpal has been found to relieve symptoms with less complications than other operations. Twenty-one patients with 23 osteotomies have been reviewed, with a follow-up from 2 to 17 years. All have had lasting relief from pain and consider that they have full function, with no stiffness or limited abduction. Osteotomy is indicated mainly for cases where the arthritis is confined to the carpometacarpal joint, but also relieves pain in cases of peritrapezial arthritis

The results of the use of Harrington rods in the treatment of spinal fractures were reviewed. It was found that with burst fractures where the anterior pillar was deficient there was a significant incidence of loss of reduction. Anterior bone supplementation is recommended in these fractures when major loss of height or angulation occurs. Several technical faults were detected which, in most cases, also led to a loss of reduction. Almost all patients with an anatomical reduction were free of pain. There was no correlation between the loss of reduction and the lapse of time before operation, the levels of instrumentation, the length of the fusion, the severity of the initial deformity, the degree of initial correction or the presence or absence of a neurological deficit

In children with obstetric brachial plexus palsy (OBPP) who develop an internal rotation deformity of the shoulder, release of subscapularis improves the range of external rotation of the shoulder and the strength of supination of the forearm. We studied the strength of supination in 35 healthy adult volunteers at 45° of both internal and external rotation. The mean and maximum torques were greater in external than internal rotation by 8.7% and 7.5%, respectively. This was highly significant (p < 0.0001). The increased strength of supination in external rotation is probably because the maximum power of biceps, particularly the long head, may be exerted in this position. In children the difference may be even greater due to anatomical differences causing the dramatic increases in the strength of supination after surgery for OBPP. In adults our findings suggest that the supination exercises which are undertaken after injury or surgery to the forearm or wrist should be performed in external rotation

Fifty-four adults with eighty hips affected by congenital disease which had not been treated have been reviewed. Fifty-nine per cent of forty-two dislocated hips had fair or poor grading scores. The incidence of osteoarthritis was markedly increased in the presence of a well-developed false acetabulum. Unilateral dislocation led to valgus deformity and degenerative changes in the ipsilateral knee in seven of twenty-two patients. Dislocation did not increase the incidence of symptomatic lumbar spondylosis. The height of the dislocated head on the ilium was not found to be related to the prognosis for the hip, the knee or the lumbar spine and did not correlate with the development of the false acetabulum. Frank congenital subluxation eventually led to osteoarthritis of the hip

1. The etiology and natural progress of lumbar kyphosis in children from three to twelve years of age with myelomeningocele are reviewed. 2. The indications for operation have included intractable or recurrent skin ulceration, inability to wear calipers for walking, inability to sit in a wheel-chair and inability to perform ileal conduit operations. 3. The technique of osteotomy-excision of lumbar vertebrae used in eighteen cases is described. 4. The results in fourteen children are described. The primary aims of operation have been achieved in all patients. 5. A comparison is made with the results of neonatal osteotomy-excision of the spine in the newborn. Recurrence of deformity, but at a much reduced rate, must be anticipated after either operation

A classification of a group of malformations of the extremities is given and an underlying common pattern is developed from a survey of 693 deformed limbs. It is characterised by a certain reduction tendency of the affected bones and the malformed extremity, an axis of malformation and an interdependence between the proximal and peripheral parts of the deformed limbs. The group, called dysmelia, has a common morphology, but morphologically identical types have been seen caused by thalidomide and as hereditary or sporadic cases. An attempt has been made not only to name and classify these deformities but also to clarify the underlying principles of their morphology. This will enable the teratologist to see the pattern of these malformations in comparison with that of normal development of the limbs and of experimentally induced limb defects

1. In this analysis of forty-three patients with syringomyelia, twenty-seven (63 per cent) had scoliosis. This association is probably due to the early involvement of the ventro-medial and dorso-medial nuclei of the spinal cord by expanding lesions. 2. The literature makes no reference to the treatment of scoliosis associated with syringomyelia. Two cases are presented of attempts to correct this scoliosis–one because of increasing deformity, the other for increasing backache. 3. Due to the presence of cystic lesions characteristic of syringomyelia, corrective operative treatment of scoliosis may present an added risk. 4. Because of the high incidence of scoliosis in patients with syringomyelia, any patient with scoliosis should be examined for evidence of neurological deficit

1. A study is presented of 286 extra-articular subtalar arthrodeses done during the years 1958 to 1965 on 258 patients with pes calcaneo-valgus, pes planovalgus, pes varus or flail foot. 2. Certain changes in the surgical technique originally presented by Grice have been made. We have applied the arthrodesis not only to valgus feet but also to varus feet, and we have described the details of the surgical technique as used on the varus foot. 3. In none of our cases was there failure of fusion or reabsorption of the graft. The unsatisfactory results (239 per cent) were caused by residual varus or valgus deformity of the foot

1. Arthrodesis of the hip is satisfactory provided a good range of knee flexion is preserved. 2. The hip is best arthrodesed in its deformed position, and the deformity corrected by a high femoral osteotomy. Knee range can readily be retained by treating the patient on traction for the first six weeks instead of using plaster. 3. Thirty-three arthrodeses were attempted without osteotomy. Only thirteen were satisfactory. Even our best method without osteotomy gave sound fusion in only seven out of ten cases. 4. In a series of twenty-three unselected cases in which osteotomy was performed in addition to other methods, fusion occurred in twenty-two