Deformity of the forearm due to growth disturbance of the ulna occurs in a number of conditions such as ulnar deficiency, multiple exostoses, and neurofibromatosis. We report a previously unrecognised form, caused by focal cortical indentation. We have treated five children with this condition, three girls and two boys; the mean age at presentation was 5 years (2 to 8). The deformity was first recognised about the age of two years, and progressed gradually. The radiological findings were the same in all cases. The focal cortical indentation was seen at the distal end of the ulna with anteromedial bowing and dysplasia. The radial head was dislocated posterolaterally. In one patient the histological findings at the site of indentation were of a fold of tissue resembling periosteum, which interfered with enchondral ossification. Treatment by ulnar lengthening using an external fixator and osteotomy which corrected both the ulnar deformity and reduced the dislocated radial head in two cases gave the best results.
We have reviewed 65 bipolar arthroplasties of the hip in 55 patients with osteoarthritis secondary to dysplasia. The mean age at operation was 56 years (42 to 79) and the mean period of follow-up was 7.2 years (5.0 to 13.0). The average Merle d’Aubigné and Postel score before operation was 10.3 and at final follow-up 15.0. There were excellent or good results in 42 hips (65%). Migration of the outer head was observed in 50 hips (77%), most often in the group with a centre-edge angle of less than 0°, an acetabular head index of less than 60%, coverage of the outer head by the original acetabulum of less than +10° and after bone grafting of the acetabulum. This migration was progressive in 31 (62%) of the 50 hips, with massive osteolysis of the acetabulum in eight. The extent and progression of migration were closely associated with this osteolysis. Four hips required revision for extensive migration of the outer head with destruction of the acetabulum or severe acetabular osteolysis, and nine hips are awaiting revision for failure of the bipolar prosthetic head. The overall failure rate was 18%. We report major problems after bipolar hip arthroplasty for osteoarthritis secondary to hip dysplasia with progressive migration of the outer head and massive acetabular osteolysis at five years or more after operation.
We have reviewed the cervical spine radiographs of 180 patients with athetoid cerebral palsy and compared them with those of 417 control subjects. Disc degeneration occurred earlier and progressed more rapidly in the patients, with advanced disc degeneration in 51%, eight times the frequency in normal subjects. At the C3/4 and C4/5 levels, there was listhetic instability in 17% and 27% of the patients, respectively, again six and eight times more frequently than in the control subjects. Angular instability was seen, particularly at the C3/4, C4/5 and C5/6 levels. We found a significantly higher incidence of narrowing of the cervical canal in the patients, notably at the C4 and C5 levels, where the average was 14.4 mm in the patients and 16.4 mm in normal subjects. The combination of disc degeneration and listhetic instability with a narrow canal predisposes these patients to relatively rapid progression to a devastating neurological deficit.
Some radiological features of the lumbar spine of 84 patients with spastic diplegia were compared with 50 control subjects. The average age of the patients was 20.1 years (3 to 39). Spondylolysis of the fifth lumbar vertebra was found in 21%, four times more frequently than in normal subjects. No patient under nine years of age had spondylolysis and the frequency increased with age. The average angle of lumbar lordosis in spastic patients in the standing position was greater than in normal subjects, and increased with age. The patients had a decreased sacrofemoral angle which caused an increase in Ferguson's angle and explained the increased angle of lumbar lordosis.
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.