Recent clinical studies have suggested that a neurological lesion may be a cause of adolescent idiopathic scoliosis and animal experiments have implicated the posterior column pathway. We have tried to determine if differences in neurological response could be detected and measured clinically, and have compared the threshold of detection of vibratory sensation in 20 girls with adolescent idiopathic scoliosis with that in 20 clinically normal age-matched controls. A highly significant reduction of the threshold of detection of vibration was seen in the scoliotic group compared to the controls (p less than 0.001). Curve magnitude did not correlate with this threshold for either the upper (r = 0.172) or lower extremity (r = 0.126). Significant asymmetry between right- and left-sided thresholds to vibration was demonstrated in the scoliotic group. Our study supports the concept that an aberration in the function of the posterior column pathway of the cord may be of primary importance in the aetiology of idiopathic scoliosis. A clinically practical test to measure this function is presented

Aims

To identify a suite of the key physical, emotional, and social outcomes to be employed in clinical practice and research concerning Perthes' disease in children.

Seat-belt fractures of the lumbar spine in adolescents and adults are well recognised but there are few reports of these injuries in young children. We reviewed all seat-belt injuries in skeletally immature patients (Risser 0), seen at a tertiary referral centre between 1974 and 1991. There were ten cases, eight girls and two boys, with an average age of 7.5 years (3 to 13). Four distinct patterns of injury were observed, most commonly at the L2 to L4 level. Paraplegia, which is thought to be uncommon, occurred in three of our ten cases. Four children had intra-abdominal injuries requiring laparotomy. There was a delay in diagnosis either of the spinal or of the intra-abdominal injury in five cases, although all had contusion of the abdominal wall, the 'seat-belt sign'. Treatment of the fractures was conservative, by bed rest and then hyperextension casts. The incidence of this potentially devastating injury can be reduced by the optimal use of restraints, but there is often a delay in diagnosis. Our classification system may aid in the early detection and evaluation of this injury

At The Hospital for Sick Children in Toronto, 21 undescended scapulae were corrected by the Woodward procedure in 20 patients over the past 18 years. The average age at operation was six years six months. The average follow-up period after operation was 8 years 9 months. Fifteen patients were girls and five were boys. All the patients had other associated abnormalities. Only one patient had a concomitant clavicular osteotomy. The average increase in glenohumeral abduction was 37 degrees. The average scapular lowering was two centimetres. Excellent or good cosmetic results were obtained in 80 per cent of patients. Ninety-three per cent of patients expressed satisfaction with the operative results. Complications were few. The Woodward procedure is a safe and relatively straightforward surgical means to effect a good cosmetic and functional improvement in a patient with a congenital undescended scapula. We do not recommend clavicular osteotomy as a routine, but it may be added to gain more correction with less risk of neurovascular compression

A girl aged ten, of Cape Coloured stock, with typical features of parastremmatic dwarfism has been investigated. The clinical manifestations included disproportionate dwarfism, limb deformity, a short stiff neck, and marked thoracic kyphosis. The radiographic changes were dramatic, the skeleton having a "flocky" appearance due to patches of radiolucency in an irregular lattice of sclerosis. The metaphyses and epiphyses of the long bones were grossly expanded, and the vertebrae were flattened and distorted. The clinical and radiographic features of five other previously reported individuals with parastremmatic dwarfism were very similar to those of our patient. The differential diagnosis of this condition includes metatrophic dwarfism, diatrophic dwarfism and the spondylo-epiphysial dysplasias. However, the unique "flocky" radiographic appearance of the bones permits diagnostic precision. There is some evidence to indicate that parastremmatic dwarfism might be transmitted as an autosomal dominant, although this is by no means certain. The fact that our patient had seven normal siblings and unaffected parents would be compatible woth autosomal recessive inheritance

Since 1987, 22 children with myelomeningocele have been fitted with reciprocating orthoses. The level of the spinal lesions ranged from T10 to L4 and 13 had associated spinal deformities. Twelve of the patients currently use a Reciprocating Gait Orthosis, seven use a Hip Guidance Orthosis or Parawalker, one has progressed to a Knee Ankle Foot Orthosis, one has died and one has been lost to follow-up. The reciprocating orthoses are worn for a mean of 3.5 hours per day (1 to 6.5); daily usage by girls is almost twice that by boys. The mean daily usage by community walkers is 4.2 hours (13 children) as against 2.8 hours by household ambulators (8 children). Active hip flexion is not essential and fixed-flexion contractures up to 35 degrees can be accommodated. The average breakdown rate is 0.45 per year with an average of 1.5 adjustments each year. The average annual cost of a reciprocating orthosis is Aus$750 (375 pounds, US$570); this includes fabrication, adjustments and repairs

Between 1988 and 1995, we studied 91 club feet from a series of 120 recalcitrant feet in 86 patients requiring surgical treatment. There were 48 boys and 20 girls. The mean age at operation was 8.9 months. Surgery consisted of an initial plantar medial release followed two weeks later by a posterolateral release. This strategy was adopted specifically to address the problems of wound healing associated with single-stage surgery and to ascertain the rate of relapse after a two-stage procedure. Immobilisation in plaster was used for three months followed by night splintage. The feet were classified preoperatively and prospectively into four grades according to the system suggested by Dimeglio et al. Grade-1 feet were postural and did not require surgery. All wounds were closed primarily. One superficial wound infection occurred in a grade-4 foot and there were no cases of wound breakdown. The rate of relapse was 20.4% in grade-3 and 65.4% in grade-4 feet. Two-stage surgery for the treatment of club foot seems to be effective in the reduction of wound problems but does not appear to give significantly better results in terms of relapse when performed for more severe deformities

We reviewed retrospectively 22 patients (23 limb segments) with fibular hemimelia treated by amputation or limb lengthening to evaluate these methods of treatment. There were 12 boys and 10 girls, all with associated anomalies in the lower limbs. Twelve patients (13 limb segments) had early amputation and prosthetic fitting and ten had tibial lengthening using the Ilizarov technique. At the latest follow-up, the twelve patients who had amputation were functioning well and had few complications. The ten patients who had lengthening had suffered numerous complications, and all had needed either further corrective surgery or to wear braces or shoe-raises. Two of the ten lengthened limbs required late amputation for poor function or cosmesis. There were fewer hospital admissions, clinic visits, and periods of absence from school in the amputation group. Our findings suggest that amputation is a more effective method of management than limb-lengthening in severe fibular hemimelia. The Ilizarov method is an attractive alternative for selected patients, but its exact role is not yet established. One problem is that families often have unrealistic expectations of the surgical and prosthetic technology available and may refuse amputation when this has been recommended

There is a close link between the embryological development of the musculoskeletal system and all other main organ systems. We report a prospective series of 202 patients with congenital vertebral abnormalities and document the associated abnormalities in other systems. There were 100 boys and 102 girls. In 153 there were 460 associated abnormalities, a mean of 2.27 abnormalities for each patient. Intravenous pyelography was carried out on 173 patients (85.6%) and ultrasonography on the remaining 29 (14.4%). Patients with genitourinary anomalies were more likely to have musculoskeletal (p = 0.002), gastrointestinal (p = 0.02) and cardiac abnormalities (p = 0.008) than those without genitourinary involvement. A total of 54 (26.7%) had at least one genitourinary abnormality, the most frequent being unilateral renal agenesis. There was urinary obstruction in six (3%). There was no association between genitourinary abnormality and the place of birth, parental age, birth order, level of spinal curvature, or the number, type and side of spinal anomaly. There was, however, a statistically significant association (p = 0.04) between costal and genitourinary abnormalities. The incidence of genitourinary abnormalities (26.7%) was similar to that of previously reported series. The diagnosis of a congenital vertebral abnormality should alert the clinician to a wide spectrum of possible associated anomalies most of which are of clinical importance

From 1948 to 1980, 93 children and adolescents had fusion in situ for severe spondylolisthesis with a slip of 50% or more, at a mean age of 14.8 years. Of these, 52 girls and 35 boys were reviewed after a mean follow-up of 13.8 years. The mean pre-operative slip was 76% and pain frequency correlated with the severity of the lumbosacral kyphosis but not with that of the slip. Posterior fusion was used in 54, posterolateral in 30 and anterior fusion in three patients. There were no major complications but 16 had re-operations for non-union or root symptoms. At follow-up there were three non-unions. After operation, 19 patients had 10% or more progression of the slip, but 10 showed correction by more than 10% as a result of remodelling. The lumbosacral kyphosis had increased by more than 10 degrees in 45%. Postoperative progression of the slip and of lumbosacral kyphosis was significantly more if the posterior element had been removed. At follow-up 77 patients were subjectively improved, four were unchanged, and one was worse. These results did not correlate with either the degree of the slip, or the angle of lumbosacral kyphosis. Fusion in situ is safe and gives good long-term clinical results. Secondary changes are associated with increased lumbosacral kyphosis, so reduction of this should be considered in severe cases

1. The incidence of osteogenic sarcoma, chondrosarcoma and Ewing's sarcoma in relation to age, sex and site is analysed in a study of 832 malignant primary bone tumours diagnosed in Sweden in 1958-68. The results are compared with those in other series. 2. The adolescent incidence peak for osteogenic sarcoma is caused by tumours localised to the long bones of the lower limb. The peak incidence occurs at a mean age of twelve years for girls and sixteen years for boys and is associated with the maximum growth velocity for the adolescent growth spurt. 3. Ewing's sarcoma, showing no sex difference with regard to its incidence peak, seems not to be associated with bone growth. 4. In the adult, the incidence of osteogenic sarcoma parallels that of chondrosarcoma, thus showing a successive increase with increasing age. In Sweden, where Paget's disease is uncommon, the incidence of osteogenic sarcoma over the age of thirty is only one-third of that during adolescence. 5. In osteogenic sarcoma and chondrosarcoma but not in Ewing's sarcoma, the characteristic predominance of males over females is valid only for localisations to the long bones of the lower limb, the pelvis and the spinal column and not for other sites. Internal factors such as age, sex, bone growth and maturation and also weight-bearing seems to be of importance in modifying the response of the tissue to a causative external factor, like a common virus

Aims

The direct posterior approach with subperiosteal dissection of the paraspinal muscles from the vertebrae is considered to be the standard approach for the surgical treatment of adolescent idiopathic scoliosis (AIS). We investigated whether or not a minimally-invasive surgery (MIS) technique could offer improved results.

Since this paper was submitted for publication three additional reports have appeared, two of which represent typical examples of the syndrome. Husson and Parkman (1961) reported the case of a female infant dying at the age of four months with chondroectodermal dysplasia, obliteration of the upper labiogingival sulcus, and congenital heart disease. The heart lesions included anomalous pulmonary venous return, single atrium and a persistent left superior vena cava. There was no known consanguinity. In reviewing the cardiac lesions described in chondroectodermal dysplasia they include another case with anomalous pulmonary venous return described by Darling, Rothney and Craig (1957), which also showed chondroectodermal dysplasia and polydactyly, though details of the extracardiac malformations are not given. Nabrady (1961) described a four-year-old Hungarian girl with ectodermal defects involving the teeth and nails but not the hair, polydactyly, distal shortening of the extremities producing dwarfing, and typical radiological appearances of the long bones. There was presumptive evidence of a septal defect of the heart. There was no consanguinity, but the mother was considered to show a "forme fruste" of the condition. A necropsy report by Meitner (1961) of a newly born premature infant with multiple congenital malformations of organs of ectodermal, mesodermal and endodermal origin is of interest because these malformations included extreme polydactyly of hands and feet, absence of nails, chondrodysplasia, and trilocular heart. In many respects, however, the case is atypical of chondroectodermal dysplasia

A programme for early mobilisation using a temporary prosthesis was evaluated in 17 children who had had an amputation above the knee for sarcomata. The temporary prosthesis had a performed adjustable polypropylene quadrilateral socket which was able to accommodate changes in the size of the stump during the first few months after amputation. The adjustable sockets were assembled onto wooden knee-shank-foot units or onto modular components covered with foam. The wooden units were better for routine use as more adjustment was possible between the socket and the knee and because they were more durable in active children. Prosthetic fitting usually took one hour and was carried out 10 days after the amputation to coincide with the start of the chemotherapy programme. The prosthesis was cosmetically acceptable, easy to use and provided a simple and economical way of rehabilitating the amputees and restoring their morale. After two to three months a new prosthesis with a laminated socket suspended by a waistband was supplied. The skin tolerated the closer fit of this socket and the small fluctuations in the size of the stump that occurred with each course of chemotherapy were easily accommodated by varying the thickness of the stump sock. A self-suspending laminated socket was provided after completion of the chemotherapy. The permanent sockets were assembled onto wooden components but the girls usually preferred the modular system covered with foam. The chemotherapy and rehabilitation programmes were successfully co-ordinated so that the children spent as little time as possible away from their normal activities

We assessed the medium-term outcome of three methods of isolated calf lengthening in cerebral palsy by clinical examination, observational gait analysis and, where appropriate, instrumented gait analysis. The procedures used were percutaneous lengthening of tendo Achillis, open Z-lengthening of tendo Achillis and lengthening of the gastrosoleus aponeurosis (Baker’s procedure). We reviewed 195 procedures in 134 children; 45 had hemiplegia, 65 diplegia and 24 quadriplegia. We established the incidence of calcaneus and recurrent equinus and identified ‘at-risk’ groups for each. At follow-up, 42% had satisfactory calf length, 22% had recurrent equinus and 36% calcaneus. The incidence of calcaneus in girls at follow-up was significantly higher (p = 0.002) while boys had an increased rate of recurrent equinus (p = 0.012). Children with diplegia who had surgery when aged eight years or younger had a 44% risk of calcaneus, while those over eight years had a 19% risk (p = 0.046). Percutaneous lengthening of tendo Achillis in diplegia was the least predictable, only 38% having a satisfactory outcome compared with 50% in the other procedures. The incidence of recurrent equinus in hemiplegic patients was 38%. Only 4% developed calcaneus. The type of surgery did not influence the outcome in patients with hemiplegia or quadriplegia. Severity of involvement, female gender, age at operation of less than eight years and percutaneous lengthening of tendo Achillis were ‘risk factors’ for calcaneus. Hemiplegia, male gender, and an aponeurosis muscle lengthening increased the risk of recurrent equinus

Between June 1988 and December 1997, we treated 332 babies with 546 dysplastic hips in a Pavlik harness for primary developmental dysplasia of the hip as detected by the selective screening programme in Southampton. Each was managed by a strict protocol including ultrasonic monitoring of treatment in the harness. The group was prospectively studied during a mean period of 6.5 ± 2.7 years with follow-up of 89.9%. The acetabular index (AI) and centre-edge angle of Wiberg (CEA) were measured on annual radiographs to determine the development of the hip after treatment and were compared with published normal values. The harness failed to reduce 18 hips in 16 patients (15.2% of dislocations, 3.3% of DDH). These required surgical treatment. The development of those hips which were successfully treated in the harness showed no significant difference from the normal values of the AI for the left hips of girls after 18 months of age. Of those dysplastic hips which were successfully reduced in the harness, 2.4% showed persistent significant late dysplasia (CEA < 20°) and 0.2% persistent severe late dysplasia (CEA < 15°). All could be identified by an abnormal CEA (< 20°) at five years of age, and many from the progression of the AI by 18 months. Dysplasia was considered to be sufficient to require innominate osteotomy in five (0.9%). Avascular necrosis was noted in 1% of hips treated in the harness. We conclude that, using our protocol, successful initial treatment of DDH with the Pavlik harness appears to restore the natural development of the hip to normal. We suggest that regular radiological surveillance up to five years of age is a safe and effective practice

Aims

Anterior cruciate ligament (ACL) surgery in children and the adolescent population has increased steadily over recent years. We used a national database to look at trends in ACL reconstruction and rates of serious complications, growth disturbance, and revision surgery, over 20 years.

We studied 29 girls and one boy with adolescent idiopathic scoliosis who were at Risser grade 0 at the time of posterior spinal fusion and were followed until maturity (mean 7.8 years). We used serial radiographs to measure the ratio of disc to vertebral height in the fused segments and to detect differential anterior spinal growth and assess its effect on scoliosis, vertebral rotation, kyphosis, and rib-vertebral-angle difference (RVAD). From one year after surgery to the latest review, the percentage anterior disc height decreased by nearly one-half and the percentage posterior disc height by nearly one-third in the fused segments (p < 0.001). There was a 4 degree increase in mean Cobb angle (p < 0.001), 11 patients (37%) having an increase of between 6 degrees and 10 degrees. There was a significant increase in mean apical rotation by 2 degrees (p = 0.003), and four patients (13%) had an increase of between 6 degrees and 16 degrees. There was little change in kyphosis. There was an increase in mean RVAD by 4 degrees (p = 0.003), seven patients (23%) showing a reduction by 1 degree to 7 degrees, and 11 (37%) increases of between 6 degrees and 16 degrees. Spinal growth occurs after posterior fusion in adolescents who are skeletally immature, as a result of continued anterior vertebral growth. There is some progression of scoliosis, vertebral rotation, and RVAD, but little change in kyphosis. The increase in deformity is not enough to warrant the use of combined anterior and posterior fusion. The findings are relevant to the management of progressive curves, the timing and extent of surgery, and the prognosis for progression of deformity in this group of patients

Aims

Intraoperative 3D navigation (ION) allows high accuracy to be achieved in spinal surgery, but poor workflow has prevented its widespread uptake. The technical demands on ION when used in patients with adolescent idiopathic scoliosis (AIS) are higher than for other more established indications. Lean principles have been applied to industry and to health care with good effects. While ensuring optimal accuracy of instrumentation and safety, the implementation of ION and its associated productivity was evaluated in this study for AIS surgery in order to enhance the workflow of this technique. The aim was to optimize the use of ION by the application of lean principles in AIS surgery.

We have reviewed, retrospectively, all children with a lower limb deformity who underwent an acute correction and lengthening with a monolateral fixator between 1987 and 1996. The patients were all under the age of 19 years and had a minimum follow-up of eight months after removal of the fixator. A total of 41 children had 57 corrections and lengthening. Their mean age was 11.3 years (3.2 to 18.7) and there were 23 girls and 18 boys. The mean maximum correction in any one plane was 23° (7 to 45). In 41 bony segments (either femur or tibia) a uniplanar correction was made while various combinations were carried out in 16. The site of the osteotomy was predominantly diaphyseal, at a mean of 47% (17% to 73%) of the total bone length and the mean length gained was 6.4 cm (1.0 to 17.0). Univariate analysis identified a moderately strong relationship between the bone healing index (BHI), length gained, maximum correction and grade-II to grade-III complications. For logistic regression analysis the patients were binary coded into two groups; those with a good outcome (BHI ≤ 45 days/cm) and those with a poor outcome (BHI > 45 days/cm). Various factors which may influence the outcome were then analysed by calculating odds ratios with 95% confidence intervals. This analysis suggested a dose response between increasing angular correction and poor BHI which only reached statistical significance for corrections of larger magnitude. Longer lengthenings were associated with a better BHI while age and the actual bone lengthened had little effect. Those patients with a maximum angulatory correction of less than 30° in any one plane had an acceptable consolidation time with few major complications. The technique is suitable for femoral deformity and shortening, but should be used with care in the tibia since the risk of a compartment syndrome or neurapraxia is much greater