We reviewed 16 patients with spina bifida and unilateral dislocation of the hip at an average age of 17 years. Nine had a high neurological level (thoracic to L3) and seven a low lesion (L4 to sacral). We assessed the influence of unilateral dislocation of the hip on leg-length discrepancy, hip pain, hip stiffness and pressure sores of the ischial tuberosity. In non-walking patients with high-level lesions, unilateral dislocation gave little functional disability and did not appear to require reduction. In walking patients with low-level lesions, leg-length discrepancy led to a poor gait and functional problems which could be prevented by reduction of the dislocation. In all patients with low lesions, surgery was successful in maintaining reduction; in two of five patients with high lesions it was unsuccessful.

We studied the long-term results of the Miller operation at a mean age of 13 years in 22 patients (38 feet) with persistently symptomatic mobile flat feet associated with an isolated naviculocuneiform break. At a mean of 12 years (3 to 27) after surgery, 84% of the feet had a satisfactory clinical result. We conclude that the Miller operation is a useful procedure for adolescent patients with persistently symptomatic flat feet with an isolated break at the naviculocuneiform joint.

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.

In a consecutive series of 124 children with spina bifida we found that 220 (89%) of the 248 feet were deformed: 70 had a calcaneus deformity; 126 were in equinus; 16 were in valgus; 3 were in varus; and 5 had convex pes valgus. Operations were performed on 171 (78%) of the deformed feet. Spasticity of the muscles controlling the foot was detected in 36 (51%) of the 70 calcaneus feet and in 22 (17%) of the 126 equinus feet. The deformities were symmetrical in 94 children. There is a high incidence of foot deformity in patients with spina bifida who have no voluntary activity in the motors of the feet.

We studied 1061 children with myelomeningocele, reviewing 3184 pelvic radiographs from 802 patients. Hip dislocation had occurred by the age of 11 years in 28% of children with a thoracic neurosegmental level, 30% of those with an L1/2 level, 36% of L3, 22% of L4, 7% of L5 and only 1% of those with sacral levels. Hip dislocation was not inevitable even when there was maximal muscle imbalance about the hip. The average hip flexion contracture in children aged 9 to 11 years was significantly greater in those with thoracic (22 degrees) and L1/2 (33 degrees) levels than in those with L4 (9 degrees), L5 (5 degrees) or sacral (4 degrees) levels. Our findings indicate that muscle imbalance is not a significant factor in the production of flexion deformity or dislocation of the hip; both are commonly seen in the absence of imbalance. The restoration of muscle balance should no longer be considered to be the principal aim of the management of the hip in children with myelomeningocele.

We reviewed 20 patients with spina bifida who had had surgical management of tibial torsion. Eight had had bilateral procedures and 12 a unilateral procedure, giving a total of 28 limbs for analysis. We performed closed osteoclasis on seven limbs and tibial osteotomy on 21. In the closed osteoclasis group six limbs (85%) had a good result after an average follow-up of nine years (2 to 22). All limbs developed postoperative anteromedial bowing of the tibia which later remodelled. In the tibial osteotomy group 19 (90%) had a good result. The average follow-up was nine years (2 to 28). Complications occurred in seven limbs (33%). We recommend closed osteoclasis of the tibia for the young patient with spina bifida in whom walking is impeded by excessive internal tibial torsion, and supramalleolar tibial osteotomy in the older patient with excessive external tibial torsion and a planovalgus foot.

Benign paroxysmal torticollis is a self-limiting condition occurring during infancy. It resolves by the age of two to three years. Periodic episodes of torticollis may randomly alternate from side to side and be associated with other symptoms. The aetiology is unknown and no treatment is effective. It is relatively uncommon, and has not been previously reported in the orthopaedic literature, although initial referral may well be to an orthopaedic surgeon. We report four cases, and review the literature.

We studied the pathogenesis, incidence and consequences of avascular necrosis in 184 children treated for congenital dislocation of the hip. Of 210 hips, 99 (47%) had some evidence of avascular necrosis (total 81, partial 18). The incidence was not influenced by the age at reduction, the duration of traction or the use of adductor tenotomy. Patients treated by closed reduction without preliminary traction did not have a higher incidence of avascular necrosis. At long-term clinical and radiological review of 81 hips, early avascular necrosis significantly increased the chance of a poor outcome but did not predispose to acetabular dysplasia. If review includes minor forms of avascular necrosis, then this condition is common after closed reduction. Its presence is an important determinant of long-term radiological and clinical outcome.

We reviewed 12 patients with congenital hallux varus who had had operations on 20 feet to enable them to wear normal shoes and to improve the appearance. After an average follow-up of 12.7 years the results of soft tissue procedures were satisfactory in 12 of 17 feet. Arthrodesis of the first metatarsophalangeal joint, performed primarily in one foot and secondarily in two others was also satisfactory, but metatarsal osteotomy in two feet gave unsatisfactory results leading to local amputation. The unsatisfactory results were generally due to the appearance of shortness of the first metatarsal and rarely because of symptoms or recurrent deformity.

We report four children with sternomastoid contracture combined with torticolis secondary to congenital vertebral anomalies. Two had features of Klippel-Feil syndrome and a proximal release of the contracted sternomastoid produced good cosmetic correction initially. Progression of the deformity occurred subsequently without recurrence of sternomastoid contracture. One child had such mild deformity that it was merely observed. The fourth child was born with torticollis without sternomastoid tightness and a vertebral anomaly was later recognised. He slowly developed a sternomastoid contracture and his condition was considerably improved by sternomastoid release. This combination of causes of torticollis has not, as far as we know, been previously reported. The clinician should be aware of it and should also realise that radiographs of the very immature spine may not disclose the bony anomalies.

Over a 25-year period, 12 patients had from 2.5 to 5.1 cm operative shortening of the tibia and fibula for leg length discrepancy at between four and 18 years of age. All recovered normal function and there was minor cosmetic impairment in only two cases. The only vascular complication was temporary delay in return of the circulation to the foot after tourniquet removal in one patient. The procedure is valuable for discrepancy of tibial length in patients when they present too late for epiphyseal arrest, when there is doubt as to the appropriate timing for epiphyseal arrest, or when it is uncertain at an earlier stage whether there is need for surgical correction.

We describe three patients who developed gross calcaneus deformity following surgery for talipes equinovarus. One also had an associated valgus deformity and another had supination of the forefoot; all had intractable problems with footwear. Operation for transfer of the tibialis anterior to the heel, with correction of the associated deformities, was successful and improved both their gait and the shoe problems.

We reviewed 13 children with partial growth plate arrest who had been treated by epiphyseolysis. Eight were followed to skeletal maturity and five for at least four years. In three cases the affected limb was restored to normal and in five the operation was successful in improving angular deformity and leg length discrepancy such that further surgery was not necessary. In the five failures, angular deformity had progressed or limb length discrepancy had increased. There were no significant complications and the procedure did not prevent subsequent osteotomy or limb length equalisation. Epiphyseolysis was most effective for small bars and those affecting only the central area of the plate.

We investigated the reproducibility of the various radiological methods of assessment of hip dysplasia by making 474 assessments of hips and quantifying the inter-observer and intra-observer variation. There was a wide range of variability between the readings made by different observers and by one observer on two occasions. A measurement of acetabular index has to be given a range of +/- 6 degrees in order to be 95% confident of including the true measurement. We found the most helpful measurements to be the acetabular index, up to the age of eight years; the centre-edge angle, over the age of five years; and Smith's c/b ratio and neck-shaft angle. We feel, however, that the change in value over a series of radiographs in the same child is much more valuable. Single readings of all the radiological measurements investigated in this study were unreliable.

We have reviewed the serial radiographs of 63 hips in 53 children treated by closed reduction for congenital dislocation with a view to finding a radiological measurement which can predict subsequent acetabular development. All had been followed for more than seven years, and at latest review, 34 hips were dysplastic. Failure to obtain concentric reduction or its loss by migration of the femoral head within one year of reduction were the best predictors of persisting acetabular dysplasia and were best quantitated by the h/b ratio (Smith et al. 1968). The acetabular index at reduction or its decrease in the first year were not reliable predictors. Late treatment was less likely to lead to normal acetabular development, but avascular necrosis did not appear to have a significant influence. The average age at which the acetabulum stopped developing was five years, but ranged from 17 months to eight years. The failure of a dysplastic acetabulum to improve in each annual radiograph after closed reduction should lead to consideration of operation on the acetabulum.

The feet of 13 spina bifida patients who had undergone triple arthrodesis in adolescence were reviewed at an average of 10 years after operation. Fifteen of 18 feet were considered satisfactory (83%); of the remaining three, two had recurrent planovalgus deformities and one a painful pseudarthrosis. Three feet had required revision of the triple arthrodesis, and there was one postoperative infection. No patient had lost ambulatory status as a result of foot problems and eight of the 10 patients who previously needed calipers were able to discard them or to use lighter ones.

We describe three children with symptoms of damage to the attachment of an anomalous ossific centre of the lower fibular epiphysis. All three were aged 8 to 10 years at the time of the initial injury, had suffered recurrent ankle sprains and had well localised and consistent tenderness precisely at the site of the anomalous ossific centre. All their symptoms were relieved by excision of the ossicle with reconstitution of the fibular collateral ligament. Whilst a separate secondary centre of ossification at the lower fibula is present in 1% of healthy children between the ages of 6 and 12 years, the condition described is extremely uncommon. Excision of the fragment should be reserved for those patients with recalcitrant symptoms and with consistent tenderness precisely at the site of the accessory ossicle.

We describe a congenital deformity of the foot which is characterised by calcaneus at the ankle and valgus at the subtalar joint; spontaneous improvement does not occur and serial casting results in incomplete or impermanent correction of the deformities. Experience with five feet in four children indicates that release of the ligaments and tendons anterior and lateral to the ankle and lateral to the subtalar joint is the minimum surgery necessary; subtalar arthrodesis may be required in addition. The foot deformity described may occur as an isolated condition or in association with multiple congenital anomalies. The possibility of a neurological deficit should always be excluded.

Seven patients with Ewing's sarcoma of the pelvis were treated by chemotherapy followed by wide resection of the primary tumour. Although good function after operation is possible, survival in this series reflects the poor prognosis associated with the disease; two patients died, two are alive with local recurrence and metastases and three patients are alive with no evidence of disease.

One hundred and nine children with myelodysplasia were evaluated and classified according to the level and type of paralysis and its effect on functional ability. Thirty-one per cent of the patients were paralysed at the thoracic level, 26% at the upper lumbar level, 30% at the lower lumbar level and 13% at the sacral level. Fifty-four per cent of these patients demonstrated the classic flaccid paralysis in the lower limbs with normal upper limbs; 9% were flaccid in the lower limbs, but were spastic in the upper; 24% were spastic in the lower limbs; 13% were spastic in the upper and lower limbs. Patients with spastic lower limbs required more orthopaedic procedures, more days in hospital and in casts, and were less likely to walk than those with flaccid paralysis. Patients with spastic upper limbs were less likely to be independent in activities of daily living and were more likely to require special schools than patients with normal upper limbs. In addition to the spinal cord level of the lesion, the degree of spasticity is important in the evaluation, treatment and prognosis of myelodysplastic patients.

We present three cases of a previously undescribed condition characterised by unilateral tibia vara associated with an area of focal fibrocartilaginous dysplasia in the medial aspect of the proximal tibia. The three children affected were aged 9, 15 and 27 months respectively. Two required tibial osteotomy, but in one the deformity resolved without treatment. The pathogenesis of the focal lesion remains conjectural; the most likely explanation is that the mesenchymal anlage of the tibial metaphysis has, for unknown reasons, developed abnormally at the insertion of the pes anserinus.

Six boys with fragmentation of the proximal pole of the patella are reported; the condition was bilateral in one. Four of the six presented with symptoms of Osgood-Schlatter's disease or Larsen-Johansson disease of the same or of the contralateral knee, but they had no symptoms or signs relating to the proximal pole of the patella; one also had features suggesting minimal chondromalacia patellae. Two boys had no objective abnormality in either knee. It is suggested that the fragmentation may be a further form of traction osteochondritis of the attachments of the quadriceps mechanism. Attention is drawn to its characteristic radiographic appearance, its association with other forms of juvenile traction osteochondritis (which are commonly symptomatic), and to its occurrence in boys aged 10 or 11 years.

Of 47 patients with spina bifida who had had transplantation of the iliopsoas more than 10 years previously, 32 (68%) were community walkers, 3 were household walkers and 12 were non-walkers. Comparison with other published reports showed that, at the very least, the patients reviewed had not had their walking ability jeopardized by the inevitable loss of hip flexor power. Furthermore, all but three of the community walkers were able to climb and descend stairs. There was a high proportion of non-walkers in those patients whose operation had been performed in the first year of life and such early surgery is no longer recommended. We also found that the pre-operative assessment of muscle power had, in some patients, been inaccurate. Finally, we found that, at review, the power of the transferred muscles was poor, suggesting that transplantation is beneficial because it achieves permanent and major reduction in hip flexor power; this usually prevents recurrent hip flexion deformity and dislocation.

Sixty-two children were reviewed between 3 and 14 years (average 9.8 years) after flexor tenotomy for curly toes or hammer toes. No patients were aware of loss of flexor power in the toes. In only 5% of 188 toes was the operation unsuccessful. When the cause of failure was identifiable it proved to be that the scar crossed one or more flexor creases. None of the operated toes had an abnormally extended posture; only one toe was stiff and this resulted from tethering by a scar. It is concluded that open flexor tenotomy is an effective method for correcting curly toes and hammer toes in childhood. Pre-operative assessment must demonstrate that the resting length of the flexor tendons is unduly short, and that this shortening is the only cause of the deformity.

Patients with spina bifida cystica commonly have significant disability from a combination of valgus deformity of the ankle and subtalar joints with lateral tibial torsion and plano-abduction deformity of the foot. These deformities can be corrected by a single procedure which combines a supramalleolar tibial osteotomy with a lateral inlay triple fusion. This procedure was carried out on 20 feet in 15 patients and the results were reviewed after an average of three years (range 18 months to 7 years). In 75 per cent of feet the combination of deformities was fully corrected, ulcers and callosities were eliminated in 95 per cent, the use of calipers minimised in 95 per cent, and in all patients the problem of shoe-wrecking was reduced. Complications included recurrent valgus deformity, delayed union of the tibial osteotomy and failure of midtarsal fusion.

The accuracy of a previously described calculation for the timing of epiphysiodesis, in the management of leg length discrepancy, has been improved modifications in the way in which the calculation has been applied. The value of the method is confirmed by a study of 94 patients who had reached maturity; 94.1 per cent of these then had discrepancies within three-quarters of an inch of that calculated and 85.1 per cent within half an inch. Over-retardation of growth was more common than insufficient retardation and was partially compensated by calculating for a discrepancy at maturity. A further 34 patients were not available for assessment at maturity but were included in analyses of the conditions for which epiphysiodesis was performed and of early complications from the operation. Both early and late complications were minor and infrequent.

Subcutaneous rupture of the tendon of tibialis anterior immediately proximal to its insertion affects patients over the age of 45 years and is most common in the seventh decade. The symptoms at the time of rupture are milder than is the case with rupture of the calcaneal tendon and the early disability is slight. Thus, affected patients commonly present several weeks or months after rupture and at a stage when reattachment of the tendon to its normal site of insertion (the most satisfactory surgical management) is impossible. Although the disability is slight if repair is not performed, there should be no disability after surgical repair and this should be offered to those patients who lead an active life and who present in the first three months after rupture. The relevant literature is reviewed and experience with four further patients is recounted.

Seventy children who had suffered from Perthes' disease were reviewed clinically and radiologically three to eight years from the onset of the condition in order to determine retrospectively the most satisfactory method of assessing the prognosis and the correlation between the clinical and radiological result. In younger children the femoral head was more likely to be spherical at the conclusion of the pathological process but not necessarily of normal proportions nor normally covered by the acetabulum. The prognosis was significantly poorer for girls than for boys. Clinical factors were not an aid to prognosis in the individual cases, but overall there was a close correlation between the clinical and the radiological end-results. The most reliable radiological factors indicating the prognosis were the extent of uncovering of the femoral head, the Catterall grouping, the presence of calcification lateral to the outer limit of the acetabulum and lateral displacement of the femoral head, as measured by comparing the head to tear-drop distances on each side.

An operative technique for correction of the severe kyphosis that may develop in cases of myelomeningocele is described. It includes excision of the apical vertebrae, correction of the full length of the kyphotic segment and of rigid compensatory curves, and extensive fusion aided by internal fixation with longitudinal threaded wires. The technique evolved during the management of eleven children. Of these, in the first two the internal fixation employed was inadequate; two others died; and in the remainder good correction was maintained up to the time of review one to six years later.

A method of triple arthrodesis is described which involves inlay of the subtalar and midtarsal joints. It is applicable to the undeformed and valgus foot as is encountered in poliomyelitis, spasmodic flat foot, cerebral palsy and spina bifida. The operation was successful in controlling deformity and pain. The only significant complication was failure of fusion of the midtarsal joint which occurred in three of eighty-five feet (3-5%).

The hip problems in 116 children with myelomeningocele are discussed. The management described stresses the importance of selection of the type of operation; major surgery is appropriate only for those children who benefit significantly, and this generally means only those with strong quadriceps muscles on both sides. Some children with acetabular dysplasia gain immediate stability if acetabuloplasty is combined with other hip operations. Children who lack strong quadriceps muscles are best served by simpler procedures, such as tendon excision, designed to rid them of fixed deformity and to prevent recurrent deformity. The operations described are performed whenever possible under one anaesthetic and are combined with any other limb operation that may be necessary. It is suggested that the aim in management is not the treatment of paralytic dislocation of the hip but the correction of fixed deformity so that the child can stand with a stable posture. Subluxation and dislocation are treated incidentally to procedures designed to prevent or correct fixed deformity.

Two children with radial club hand and absence of the biceps muscle were treated by centralisation of the ulna into the carpus and triceps transfer. The two operations were performed only a short time apart so that the period between the procedures could be used to stretch the triceps and to enable the children to adapt to an altered position of the wrist and to mobility of the elbow at one step and following a single period of plaster immobilisation. It is very likely that function is better than it would have been had the condition remained untreated. Before operation the children had only a crude hook function of the hand against the forearm and could not bring the hand to the mouth. Even if function is not much improved, the improvement in appearance is considerable and is by itself sufficient to justify the procedures.