Five patients with classical Ehlers-Danlos syndrome developed severe spinal deformities. Two were shown to have type-VI collagen abnormalities. Three had a double structural scoliosis of the thoracic and lumbar regions, one had a single thoracic scoliosis and one had a thoracic kyphosis. The curves first developed before the age of four years, and were not controlled by bracing. Major corrective surgery with posterior fusion was performed at a mean age of 11 years 8 months. Excessive blood loss could be controlled and although wound haematoma and dehiscence were common, they did not provide major problems. The spinal fusions healed satisfactorily.
Adolescent idiopathic scoliosis in 152 patients was treated by Luque L-rod instrumentation and early mobilisation without external support. This series was compared with a matched group of 156 patients treated by Harrington instrumentation and immobilised in an underarm jacket for nine months. All the operations in both groups were performed by one surgeon and the patients were followed prospectively for more than two years. Correction of the scoliosis in the frontal plane was similar in both groups. However, the normal sagittal contour was better maintained with Luque rods, especially in the thoracolumbar and lumbar regions, and provided less loss of correction than with Harrington rods. Neither method significantly derotated the scoliosis. All the patients with Luque instrumentation developed a solid fusion despite breakage of the sublaminar wires at one or two levels in 4.9%. There were no major neurological complications with either type of instrumentation.
Twenty-three patients with severe paralytic thoracolumbar scoliosis due to a myelomeningocele were treated by a two-stage procedure. Before operation the mean scoliosis was 98 degrees: after the first-stage procedure, an anterior spinal fusion and correction with Dwyer instrumentation, this was reduced to a mean of 45 degrees. Approximately two weeks later a posterior spinal fusion with Harrington instrumentation was performed, further reducing the scoliosis to a mean of 29 degrees. The pelvic obliquity also was reduced from a mean of 32 degrees to 6 degrees. Although such management carries risks (one patient died of cardiorespiratory failure after the first stage and one patient was made worse), 21 of the 23 patients had improved posture and function.
We studied 104 patients with a total of 154 hemivertebrae which had produced scoliotic curves. Of the hemivertebrae 65% were of a fully segmented (non-incarcerated) type, 22% were semi-segmented and 12% were incarcerated. We found that the degree of scoliosis produced depended on four factors: first, the type of the hemivertebra; secondly, its site; thirdly, the number of hemivertebrae and their relationship to each other; and finally, the age of the patient. Semi-segmented and incarcerated hemivertebrae usually do not require treatment. Fully segmented non-incarcerated hemivertebrae may require prophylactic treatment to prevent significant deformity.
Fourteen patients with ankylosing spondylitis had an extension osteotomy for severe flexion deformity of the spine. The Smith-Petersen technique was modified by using a compression device which allows a slow, finely controlled closure of the osteotomy, and provides rigid internal fixation. There were no serious neurological complications. All the patients were able to see straight ahead after operation, and all had solid fusion at nine months, having maintained good correction.
Seven patients treated in infancy by a lumbar theco-peritoneal shunt for idiopathic communicating hydrocephalus presented later in childhood after developing a characteristic orthopaedic syndrome. This included a severe, rigid and progressive lumbar hyperlordosis, severe bilateral restriction of straight leg raising and abnormalities of stance and gait. Four of the patients, who had severe hyperlordotic curves of over 90 degrees, required operations to correct their extreme deformity. The recommended method of correction is a three-stage procedure: first, anterior wedge resection osteotomies at several levels in the lumbar spine, then a period of "90-90" femoral traction, and finally a posterior fusion and stabilisation using Harrington instrumentation. The results were good, with few complications.
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.
The operative and anaesthesic technique for 44 patients undergoing posterior spinal fusion with Harrington rod instrumentation for idiopathic scoliosis is described. There were two groups of 21 and 23 patients, matched for diagnosis and status before operation. The management of both groups was similar but in one group anaesthesia with induced hypotension was employed, using a mixture of sodium nitroprusside and trimetaphan. The mean blood loss at operation and after operation in this group was significantly lower than in the other group, with a consequent reduction in the transfusion requirement. No adverse sequelae were observed. All patients showed a drop in haemoglobin concentration after operation, despite clinically adequate blood transfusion.
Twenty-four children with infantile or juvenile idiopathic scoliosis had their spines corrected and solidly fused posteriorly before the age of eleven years. The growth of the fusion area was then accurately measured for a mean of 4.5 years during the adolescent growth spurt. During this period all longitudinal growth in the posterior elements ceased. The vertebral bodies continued to grow anteriorly, but the thick posterior fusion prevented the development of a lordosis. Initially the anterior growth was accommodated by narrowing of the intervertebral disc spaces, but eventually the vertebral bodies bulged laterally towards the convexity and pivoted on the posterior fusion, giving rise to loss of correction, increasing vertebral rotation and recurrence of the rib hump.
The factors during and after operation which influence the development of a solid and stable posterior spinal fusion have been evaluated in 406 patients with scoliosis. The patients were managed in three different ways and all pseudarthroses were accurately detected by exploring the spines six months after the attempted fusion. The incidence of pseudarthroses was significantly lowered from 25 per cent in Group I to 3.8 per cent in Group III by the application of Harrington instrumentation and the use of large amounts of autogenous iliac bone grafts in addition to an interfacetal fusion. Early mobilisation 7 to 10 days after operation and a return to normal activities in a well-moulded underarm plaster jacket did not have a detrimental effect on the development of the fusion or the early maintenance of correction. Those spines with supplementary bone grafts stabilised more rapidly and had better maintenance of correction with only minimal loss after removal of all external support at 10 months.
Scintigraphy using technetium-labelled methylene diphosphonate was performed on 110 scoliotic patients six months after an attempted fusion and the findings compared with those at exploration to detect the possible sites of pseudarthroses. The majority of patients (65 per cent) had a uniform uptake of isotope over the fused area and all but one had a solid fusion. A second group (35 per cent) had a more patchy uptake and eight of the nine patients with pseudarthroses were in this group. Pseudarthroses were detected as localised areas of increased uptake but there were also a number of false positives and scans that were difficult to interpret due to continuing new bone formation in immature fusions. In those scans performed after one year the pseudarthroses which had been missed were seen more clearly in contrast to the diminished generalised activity in the fused area.
Twenty-seven children with progressive infantile idiopathic scoliosis have been reviewed after long-term management. Twenty-two children had single thoracic curves which were diagnosed at an early age and treated in a modified Milwaukee brace until the age of ten years when the spine was corrected and fused. The mean correction after operation was 40 per cent of the initial degree of curvature seen in early childhood before treatment. Solid spinal fusion led to a further moderate loss in correction due to bending of the fusion mass before the spine became stable several years later. Five children had double structural curves and were treated only in a brace. This provided less satisfactory control of these curves but because of the minimal cosmetic deformity, extensive spinal fusion was avoided.
The clinical, radiological and pathological features of hallux rigidus affecting nine toes (in seven patients) are described. Characteristic chondral and osteochondral lesions are seen to occur at a specific site on the metatarsal head, and account for the limitation of dorsiflexion but relatively unrestricted plantarflexion typical of hallux rigidus. Radiologically these lesions are often missed because they are mainly cartilaginous and are later obscured by secondary degenerative changes. Histological evidence indicates a traumatic aetiology and a mechanism of injury is suggested.
A series of 246 patients with scoliosis and attempted fusion had exploration performed six months later in order to detect and treat any pseudarthrosis at an early stage and so prevent subsequent loss of correction. Bilateral or unilateral pseudarthroses occurred in 25 per cent and were of three types--definite, hairline and doubtful. Single unilateral pseudarthroses accounted for 6 per cent and were of little if any clinical significance. The hairline pseudarthroses could not be seen radiologically and were easily missed at exploration. In general the pseudarthroses were least common in the more rigid parts of the spine and in curves which by nature of their aetiology or long duration had become most rigid. Neither the initial severity of the curve nor the degree of correction obtained before the initial attempted fusion had any apparent effect on the incidence. Follow-up for an average of four years has shown that a pseudarthrosis is of little significance with regard to the ultimate result provided it is recognised early and repaired.
Hindfoot (subtalar) movement and disability after fracture of the tibial shaft have been studied. One hundred patients with soundly healed fractures of the tibial shaft treated by immobilisation in a long leg plaster cast were examined at least one year after the fracture had healed. Subtalar movement was accurately measured by a special technique and the patients were questioned specifically about any disability associated with their hindfoot. Subtalar movement was limited to some degree in 72% of patients. Forty-three per cent of patients complained of symptoms related to their subtalar joint and 12% found these symptoms troublesome.
1. One hundred and twenty-six metacarpo-phalangeal joints affected by rheumatoid arthritis were studied macroscopically at either synovectomy or arthroplasty. 2. The sites and extent of the initial erosion corresponded with the sites and size of the synovial pouches. 3. The areas of cartilage degeneration were related to the degree of flexion, ulnar deviation and subluxation of the proximal phalanx on the metacarpal head. 4. The routine radiographic findings were not a true reflection of all the pathological changes within the joint. 5. It is suggested that by encouraging joint movement and preventing deformity the degree of cartilage degeneration may be diminished.
1. Opinion is divided as to the value of synovectomy of the knee in juvenile rheumatoid arthritis. 2. Between 1964 and 1970 thirty synovectomies of the knee were performed in twenty-seven children, who have now been kept under review for one to eight years. Three have required a second operation. 3. The operative findings at various stages are presented and discussed, with special reference to the effect on growth of the limb. 4. Synovectomy is a useful procedure which should be reserved for cases where effusion persists despite nine months of conservative treatment and performed before signs of bone erosion appear.
