Congenital pseudarthrosis of the tibia (CPT) is a rare but well recognised condition. Obtaining union of the pseudarthrosis in these children is often difficult and may require several surgical procedures. The treatment has changed significantly since the review by Hardinge in 1972, but controversies continue as to the best form of surgical treatment. This paper reviews these controversies.

Cite this article: Bone Joint J 2013;95-B:1027–34.

In a retrospective review of 302 clinically suspected cases of congenital syphilis, bone changes were found in 197. The skeletal manifestations were periostitis (102 cases), osteitis (20), and metaphyseal changes (71). Combinations of more than one lesion were found in 61. Pseudoparalysis was a presenting sign in 34 infants; 12 of these were found to have had pathological fractures and four had dactylitis. Complete radiological healing without residual changes was seen in all 59 cases that were recalled for review. The orthopaedic surgeon should consider the diagnosis of congenital syphilis when destructive lesions of bone are seen in an infant

A total of 38 relapsed congenital clubfeet (16 stiff, 22 partially correctable) underwent revision of soft-tissue surgery, with or without a bony procedure, and transfer of the tendon of tibialis anterior at a mean age of 4.8 years (2.0 to 10.1). The tendon was transferred to the third cuneiform in five cases, to the base of the third metatarsal in ten and to the base of the fourth in 23. The patients were reviewed at a mean follow-up of 24.8 years (10.8 to 35.6). A total of 11 feet were regarded as failures (one a tendon failure, five with a subtalar fusion due to over-correction, and five with a triple arthrodesis due to under-correction or relapse). In the remaining feet the clinical outcome was excellent or good in 20 and fair or poor in seven. The mean Laaveg-Ponseti score was 81.6 of 100 points (52 to 92). Stiffness was mild in four feet and moderate or severe in 23. Comparison between the post-operative and follow-up radiographs showed statistically significant variations of the talo-first metatarsal angle towards abduction. Variations of the talocalcaneal angles and of the overlap ratio were not significant. Extensive surgery for relapsed clubfoot has a high rate of poor long-term results. The addition of transfer of the tendon of tibialis anterior can restore balance and may provide some improvement of forefoot adduction. However, it has a considerable complication rate, including failure of transfer, over-correction, and weakening of dorsiflexion. The procedure should be reserved for those limited cases in which muscle imbalance is a causative or contributing factor

1. Three cases of congenital kyphosis at or near the thoraco-lumbar junction are reported. 2. The features of the deformity are discussed

Comparisons were made between 54 children with resolving congenital talipes equinovarus deformity and 81 children whose feet required surgical correction. There was a significant difference in the incidence of bilateral deformity, sex ratio and family history of congenital talipes in the two groups. The children with resolving deformity had feet which were indistinguishable from the normal side of unilaterally affected children in their calf muscle measurements at six weeks of age, and in the range of movement and radiographs at four years of age. Those requiring surgery had varying degrees of muscle reduction, and although often well corrected, had a reduced range of movement and altered joint anatomy at four years of age. This suggests that they are two distinct populations

1. The problem of instability of reduction in congenital dislocation and congenital subluxation of the hip has been studied and it has been concluded that the basic cause of this instability is the abnormal direction in which the entire acetabulum faces. 2. An operation, innominate osteotomy, has been designed to correct the abnormal direction of the entire acetabulum in children over the age of eighteen months. The principle of innominate osteotomy is redirection of the acetabulum so that the reduced dislocation or subluxation, which previously was stable only in a position of abduction and flexion, is rendered stable in the functional position of weight bearing. 3. The operative technique, and the pre-operative and post-operative management are described. 4. The indications for innominate osteotomy are outlined and the advantages of the operation are enumerated. 5. The early results of innominate osteotomy are very encouraging

We have reviewed the results in 37 patients with unilateral congenital short femur (Pappas classes III to IX), treated by different lengthening procedures. The increase in the length of the femur varied from 15.6% to 142%, excellent or good results being obtained in 32 patients (86%). There was an average of 1.9 complications per case, most being seen earlier in the series when the Wagner technique was used. With the Orthofix and the Ilizarov techniques, we used callus distraction in all cases. We found that the proximal diaphysis of the congenitally abnormal femur healed less well, and we now prefer to perform corticotomy and callus distraction of the distal metaphysis. The Ilizarov method gave the best results, offering the possibilities of the simultaneous use of a Hoffmann fixator across the hip and the treatment of knee dislocation and instability

Van Nes rotationplasty may be used for patients with congenital proximal focal femoral deficiency (PFFD). The lower limb is rotated to use the ankle and foot as a functional knee joint within a prosthesis. A small series of cases was investigated to determine the long-term outcome. At a mean of 21.5 years (11 to 45) after their rotationplasty, a total of 12 prosthetic patients completed the Short-Form (SF)-36, Faces Pain Scale-Revised, Harris hip score, Oswestry back pain score and Prosthetic Evaluation Questionnaires, as did 12 age- and gender-matched normal control participants. A physical examination and gait analysis, computerised dynamic posturography (CDP), and timed ‘Up & Go’ testing was also completed. Wilcoxon Signed rank test was used to compare each PFFD patient with a matched control participant with false discovery rate of 5%. There were no differences between the groups in overall health and well-being on the SF-36. Significant differences were seen in gait parameters in the PFFD group. Using CDP, the PFFD group had reduced symmetry in stance, and reduced end point and maximum excursions. Patients who had undergone Van Nes rotationplasty had a high level of function and quality of life at long-term follow-up, but presented with significant differences in gait and posture compared with the control group. Cite this article: Bone Joint J 2013;95-B:192–8

A long-term review of 131 children fitted with upper limb prostheses at the Ontario Crippled Children's Centre between 1965 and 1975 is reported. There were 116 children with congenital deficiencies and 15 who had had amputations. Follow-up ranged from 7 to 17 years. A total of 42 children had abandoned their prostheses, 37 of whom had congenital deformities and five were amputees. The level of deficiency was of fundamental importance in determining whether the prosthesis would be accepted; in the forearm, the longer the stump, the more likely it was that the child would discard the prosthesis. Overall, 50 per cent of children fitted over the age of two years abandoned their prostheses compared with only 22 per cent of patients who had been fitted before the age of two years. The highest drop-out rate was at the age of 13 years when the children became more conscious of their cosmetic appearance. Suggestions for reducing the high drop-out rate in the early teens are put forward

We report a prospective study of the feasibility of employing specially trained physiotherapists to screen neonates for congenital dislocation of the hip. During ten years 42,241 babies were screened, using clinical tests; 255 were diagnosed and treated by a Pavlik harness. In the same period 13 children presented late with congenital dislocation of the hip which had not been detected by the screening programme

The hips of twenty full-term African neonates have been examined in detail to determine any anatomical factors which might explain the difference in the incidence of congenital dislocation of the hip in the African and in the Caucasian. Measurements included the degree of anteversion of the femoral neck and the acetabulum and the diameter and depth of the acetabulum. The acetabulum tended to be deeper and to vary within a much narrower range than that reported for Caucasians, lending indirect support to the theoretical role of acetabular dysplasia in the aetiology of congenital dislocation of the hip. Measurements of the anteversion of the acetabulum and femoral neck were similar to those given for Caucasians

The term "congenital scoliosis" contributes little to our understanding of aetiology, for "congenital" simply means "to be born with" and is applicable to deformities present at birth whether these are genetically determined or acquired in utero. The presentation of monozygotic twins, one of whom has congenital scoliosis (vertebral anomalies) while the other is normal, provides a rare opportunity to study the cause of this deformity. Three pairs of monozygotic twins, previously unreported, are presented with a review of the previous literature. These cases add weight to the argument that congenital scoliosis may be acquired in utero rather than being genetically determined

Thirteen patients with congenital hypoplasia of the carpal scaphoid and associated abnormalities have been described and the significance of the condition discussed

We reviewed the serial radiographs of 54 hips in 47 children treated by closed reduction for congenital dislocation of the hip and followed to at least 14 years of age, to determine the causes of acetabular dysplasia. We excluded hips with femoral head deformity or residual subluxation and compared the results with those from a control series of unaffected hips of patients with unilateral CDH. Acetabular development after the age of 11 or 12 years was significantly worse in Severin group III than in Severin group I hips on the affected side or Severin group III in unaffected control hips. One of the causes of acetabular dysplasia at maturity was found to be impairment of acetabular development after 11 or 12 years of age. This may be attributable to impaired secondary ossification in the acetabular rim. Our findings emphasise the importance of continuing the follow-up of patients treated for congenital dislocation of the hip until full skeletal maturity

We report on a radiographic screening programme at four months of age for infants who were clinically normal at neonatal examination but were considered to be 'at risk' for congenital dislocation of the hip because of their family history, breech presentation, or a persistent click. From a total population of 13,662 live births over a two-year period, 357 (2.6%) infants at risk were identified. Of these 46 had abnormal radiographs (six subluxations, 40 acetabular dysplasia). In 12 infants treatment resulted in a normal hip; 34 required no treatment but were followed up until their radiographs were normal and walking had begun. Of the 311 infants with normal radiographs, 256 (82%) were examined after 15 months of age; none had any detectable abnormality. We suggest that radiography of the hip at four months is a valuable adjunct to neonatal screening for infants at increased risk of congenital dislocation of the hip

Operative correction of congenital vertical talus in nine feet in six patients is described. The operation consists of the comprehensive lengthening of the tendons in the foot and full peritalar release without excision of the navicular; it is performed through a dorsal transverse incision

We reviewed 33 patients (35 hips) after open reduction of congenital dislocation of the hip using Ludloff’s medial approach. The mean age at the time of operation was 14 months (5 to 29) and at the time of final follow-up 20.1 years (15 to 24) giving a mean duration of follow-up of 19.4 years (14 to 23). We evaluated the radiological results by the Severin classification and the extent of avascular necrosis using the criteria of Kalamchi. At the latest review 16 hips (45.7%) were in Severin groups I or II (acceptable results) and 19 (54.3%) in Severin groups III, IV or V (unacceptable results). There was evidence of avascular necrosis in 15 hips (42.9%). The radiological results began to become worse at about the age of ten years when type-II avascular changes presented. We conclude that Ludloff’s medial approach for open reduction is unsatisfactory for the treatment of congenital dislocation since about half of our patients required additional operations

1. A case of congenital indifference to pain in a boy aged fifteen years is described. The boy's sister was similarly affected. 2. Clinical features and treatment are described, and the features are compared with those in previously reported cases. 3. Causation is discussed in the light of the negative findings at necropsy on the patient's sister

A review of seventy-one children with sacral anomalies is presented. The aetiology is discussed and a classification of sacral anomalies is suggested, with three groups of patients: agenetic, dysgenetic and dysraphic. The clinical presentation of each group is discussed and the high incidence of congenital visceral and skeletal abnormalities is indicated in the dysgenetic group. The need for constant urological assessment is emphasised, particularly in the agenetic and dysraphic children

Seventy patients with 91 congenital short femora are classified. Deformities resulting maternal Thalidomide treatment are compared with those where Thalidomide was not involved and genetic and epidemiological factors investigated in 50 patients. No essential anatomical difference was found between the two groups of femora but the whole complex of abnormalities differed: the Thalidomide group showed femur-tibia-radius anomalies while the non-Thalidomide garoup had femur-fibula-ulna anomalies, indicating either different aetiological factors or different timing of the insult to the foetus. Some differences between congenital coxa vara and congenital short femur associated with coxa vara are mentioned. Simple hypoplasia of the femur may possibly have a multifactorial genetic background since it is associated with other minor abnormalities of the limbs in these families, whereas environmental factors only are associated with the more severe femoral defects