Aims. To describe the clinical, radiological, and functional outcomes in patients with isolated congenital thoracolumbar kyphosis who were treated with three-column osteotomy by posterior-only approach. Methods. Hospital records of 27 patients with isolated congenital thoracolumbar kyphosis undergoing surgery at a single centre were retrospectively analyzed. All patients underwent deformity correction which involved a three-column osteotomy by single-stage posterior-only approach. Radiological parameters (local kyphosis angle (KA), thoracic kyphosis (TK), lumbar lordosis (LL), pelvic tilt (PT), sacral slope (SS), C7 sagittal vertical axis (C7 SVA), T1 slope, and pelvic incidence minus lumbar lordosis (PI-LL)), functional scores, and clinical details of complications were recorded. Results. The mean age of the study population was 13.9 years (SD 6.4). The apex of deformity was in thoracic, thoracolumbar, and lumbar spine in five, 14, and eight patients, respectively. The mean operating time was 178.4 minutes (SD 38.5) and the mean operative blood loss was 701.8 ml (SD 194.4). KA (preoperative mean 70.8° (SD 21.6°) vs final follow-up mean 24.7° (SD 18.9°); p < 0.001) and TK (preoperative mean -1.48° (SD 41.23°) vs final follow-up mean 24.28° (SD 17.29°); p = 0.005) underwent a significant change with surgery. Mean Scoliosis Research Society (SRS-22r) score improved after surgical correction (preoperative mean 3.24 (SD 0.37) vs final follow-up mean 4.28 (SD 0.47); p < 0.001) with maximum improvement in self-image and mental health domains. The overall complication rate was 26%, including two neurological and five non-neurological complications. Permanent neurological deficit was noted in one patient. Conclusion. Deformity correction employing three-column osteotomies by a single-stage posterior-only approach is safe and effective in treating isolated congenital thoracolumbar kyphosis. Cite this article: Bone Joint J 2021;103-B(7):1309–1316

Diastematomyelia is a rare congenital abnormality of the spinal cord. This paper summarises more than 30 years’ experience of treating this condition. Data were collected retrospectively on 138 patients with diastematomyelia (34 males, 104 females) who were treated at our hospital from May 1978 to April 2010. A total of 106 patients had double dural tubes (type 1 diastematomyelia), and 32 patients had single dural tubes (type 2 diastematomyelia). Radiographs, CT myelography, and MRI showed characteristic kyphoscoliosis, widening of the interpedicle distance, and bony, cartilaginous, and fibrous septum. The incidences of symptoms including characteristic changes of the dorsal skin, neurological disorders, and congenital spinal or foot deformity were significantly higher in type 1 than in type 2. Surgery is more effective for patients with type 1 diastematomyelia; patients without surgery showed no improvement

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

This paper reports a high incidence of minor congenital anomalies in boys and girls with Perthes' disease compared with that in a control population. There is a similarity of the incidence of minor anomalies in the children with Perthes' disease to that in babies with a single major congenital defect. Multiple major defects were more numerous and more severe than in the control children. It is speculated that there may be a congenital abnormality affecting skeletal development which in some way makes the hip susceptible to Perthes' disease at a later date

Aims. The aim of this study was to gain an agreement on the management of idiopathic congenital talipes equinovarus (CTEV) up to walking age in order to provide a benchmark for practitioners and guide consistent, high-quality care for children with CTEV. Methods. The consensus process followed an established Delphi approach with a predetermined degree of agreement. The process included the following steps: establishing a steering group; steering group meetings, generating statements, and checking them against the literature; a two-round Delphi survey; and final consensus meeting. The steering group members and Delphi survey participants were all British Society of Children’s Orthopaedic Surgery (BSCOS) members. Descriptive statistics were used for analysis of the Delphi survey results. The Appraisal of Guidelines for Research & Evaluation checklist was followed for reporting of the results. Results. The BSCOS-selected steering group, the steering group meetings, the Delphi survey, and the final consensus meeting all followed the pre-agreed protocol. A total of 153/243 members voted in round 1 Delphi (63%) and 132 voted in round 2 (86%). Out of 61 statements presented to round 1 Delphi, 43 reached ‘consensus in’, no statements reached ‘consensus out’, and 18 reached ‘no consensus’. Four statements were deleted and one new statement added following suggestions from round 1. Out of 15 statements presented to round 2, 12 reached ‘consensus in’, no statements reached ‘consensus out’, and three reached ‘no consensus’ and were discussed and included following the final consensus meeting. Two statements were combined for simplicity. The final consensus document includes 57 statements allocated into six successive stages. Conclusion. We have produced a consensus document for the treatment of idiopathic CTEV up to walking age. This will provide a benchmark for standard of care in the UK and will help to reduce geographical variability in treatment and outcomes. Appropriate dissemination and implementation will be key to its success. Cite this article: Bone Joint J 2022;104-B(6):758–764

Talipes equinovarus is one of the more common congenital abnormalities affecting the lower limb and can be challenging to manage. This review provides a comprehensive update on idiopathic congenital talipes equinovarus with emphasis on the initial treatment. Current management is moving away from operative towards a more conservative treatment using the Ponseti regime. The long-term results of surgical correction and the recent results of conservative treatment will be discussed

There were 47 patients with congenital muscular torticollis who underwent operative release. After a mean follow-up of 74 months (60 to 90), they were divided into two groups, one aged one to four years (group 1) and the other aged five to 16 years (group 2). The outcomes were assessed by evaluating the following parameters: deficits of lateral flexion and rotation, craniofacial asymmetry, surgical scarring, residual contracture, subjective evaluation and degree of head tilt. The craniofacial asymmetry, residual contracture, subjective evaluation and overall scores were similar in both groups. However, group 2 showed superior results to group 1 in terms of the deficits of movement, surgical scarring and degree of head tilt. It is recommended that operative treatment for congenital muscular torticollis is postponed until the patient can comply successfully with post-operative bracing and an exercise programme

Adult presentation of neglected congenital muscular torticollis is rare. We report 12 patients with this condition who underwent a modified Ferkel’s release comprising a bipolar release of sternocleidomastoid with Z-lengthening. They had a mean age of 24 years (17 to 31) and were followed up for a minimum of two years. Post-operatively a cervical collar was applied for three weeks with intermittent supervised active assisted exercises for six weeks. Outcome was assessed using a modified Lee score and a Cheng and Tang score. The mean pre-operative rotational deficit was 8.25° (0° to 15°) and mean lateral flexion deficit was 20.42° (15° to 30°), which improved after treatment to a mean of 1.67° (0° to 5°) and 7.0° (4° to 14°) after treatment, respectively. According to the modified Lee scoring system, six patients had excellent results, two had good results and four had fair results, and using the Cheng and Tang score, eight patients had excellent results and four had good results. Surgical management of adult patients with neglected congenital muscular torticollis using a modified Ferkel’s bipolar release gives excellent results. The range of neck movement and head tilt improved in all 12 patients and cosmesis improved in 11, despite the long-standing nature of the deformity

We studied the effect of trochanteric osteotomy in 192 total hip replacements in 140 patients with congenital hip disease. There was bony union in 158 hips (82%), fibrous union in 29 (15%) and nonunion in five (3%). The rate of union had a statistically significant relationship with the position of reattachment of the trochanter, which depended greatly on the pre-operative diagnosis. The pre-operative Trendelenburg gait substantially improved in all three disease types (dysplasia, low and high dislocation) and all four categories of reattachment position. A persistent Trendelenburg gait post-operatively was noticed mostly in patients with defective union (fibrous or nonunion). Acetabular and femoral loosening had a statistically significant relationship with defective union and the position of reattachment of the trochanter. These results suggest that the complications of trochanteric osteotomy in total hip replacement for patients with congenital hip disease are less important than the benefits of this surgical approach

A combined anterior and posterior surgical approach is generally recommended in the treatment of severe congenital kyphosis, despite the fact that the anterior vascular supply of the spine and viscera are at risk during exposure. The aim of this study was to determine whether the surgical treatment of severe congenital thoracolumbar kyphosis through a single posterior approach is feasible, safe and effective. We reviewed the records of ten patients with a mean age of 11.1 years (5.4 to 14.1) who underwent surgery either by pedicle subtraction osteotomy or by vertebral column resection with instrumented fusion through a single posterior approach. The mean kyphotic deformity improved from 59.9° (45° to 110°) pre-operatively to 17.5° (3° to 40°) at a mean follow-up of 47.0 months (29 to 85). Spinal cord monitoring was used in all patients and there were no complications during surgery. These promising results indicate the possible advantages of the described technique over the established procedures. We believe that surgery should be performed in case of documented progression and before structural secondary curves develop. Our current strategy after documented progression is to recommend surgery at the age of five years and when 90% of the diameter of the spinal canal has already developed. Cite this article: Bone Joint J 2013;95-B:1527–32

We report the case of a 24-year-old man with a congenital meniscoid articular disc of the triangular fibrocartilage complex with extensor carpi ulnaris tenosynovitis. His young age, the normal articular cartilage, the lack of degenerative changes at the margins of the defect and its bilateral occurrence made this diagnosis likely. A congenital defect of the articular disc of the triangular fibrocartilage complex should not be misinterpreted as a traumatic rupture and is usually asymptomatic

Aims

Morphological abnormalities are present in patients with developmental dysplasia of the hip (DDH). We studied and compared the pelvic anatomy and morphology between the affected hemipelvis with the unaffected side in patients with unilateral Crowe type IV DDH using 3D imaging and analysis.

1. One hundred cases of torticollis and 117 cases of sternomastoid tumour have been reviewed. 2. Congenital torticollis can be sub-divided into two groups: postural and muscular. 3. Congenital postural torticollis is present at birth; it is not associated with a sternomastoid tumour; it is transient in nature; and it does not require operation for its relief. 4. Congenital muscular torticollis is preceded by a sternomastoid tumour which is clinically evident in one-fifth of all cases. 5. The ischaemic theory of the causation of sternomastoid tumours is not supported by recent histological investigations. Some other cause, which probably is operative before birth, must be sought. 6. Four-fifths of all cases of sternomastoid tumours resolve spontaneously and leave no deformity. Excision of the tumour in infancy is therefore unjustifiable. 7. Open division of the muscle and of the cervical fascia in congenital muscular torticollis cures the deformity but leaves an unsightly scar. 8. Subcutaneous tenotomy can be relied upon to cure the deformity if post-operative treatment is carried out skilfully and assiduously over a prolonged period. 9. If complete correction is not gained at the time of subcutaneous tenotomy a better result can be assured by open division of the upper end of the muscle through an incision within the hair line

Difficulties posed in managing developmental dysplasia of the hip diagnosed late include a high-placed femoral head, contracted soft tissues and a dysplastic acetabulum. A combination of open reduction with femoral shortening of untreated congenital dislocations is a well-established practice. Femoral shortening prevents excessive pressure on the located femoral head which can cause avascular necrosis. Instability due to a coexisting dysplastic shallow acetabulum is common, and so a pelvic osteotomy is performed to achieve a stable and concentric hip reduction. We retrospectively reviewed 15 patients (18 hips) presenting with developmental dysplasia of the hip aged four years and above who were treated by a one-stage combined procedure performed by the senior author. The mean age at operation was five years and nine months (4 years to 11 years). The mean follow-up was six years ten months (2 years and 8 months to 8 years and 8 months). All patients were followed up clinically and radiologically in accordance with McKay’s criteria and the modified Severin classification. According to the McKay criteria, 12 hips were rated excellent and six were good. All but one had a full range of movement. Eight had a limb-length discrepancy of about 1 cm. All were Trendelenburg negative. The modified Severin classification demonstrated four hips of grade IA, six of grade IB, and eight of grade II. One patient had avascular necrosis and one an early subluxation requiring revision. One-stage correction of congenital dislocation of the hip in an older child is a safe and effective treatment with good results in the short to medium term

This paper reviews the current knowledge relating to the management of adult patients with congenital hip disease. Orthopaedic surgeons who treat these patients with a total hip replacement should be familiar with the arguments concerning its terminology, be able to recognise the different anatomical abnormalities and to undertake thorough pre-operative planning in order to replace the hip using an appropriate surgical technique and the correct implants and be able to anticipate the clinical outcome and the complications

Ninety-seven limbs, in eighty-one patients, with a diagnosis of congenital deficiency of the fibula have been reviewed. A classification was devised to distinguish the minimal hypoplasia of the fibula (Type I) from the well-known complete absence (Type II). Congenital anomalies of the femur were present in 76 per cent of patients with Type I deficiency and in 59 per cent with Type II. The shortening of the limb was by 13 per cent in Type I and by 19 per cent in Type II, and the percentage shortening was fairly constant during growth. A detailed description of the spectrum of other congenital anomalies was found to be characteristic: for example, the ball and socket formation of the ankle, tarsal coalition and anomalies of the foot. The treatment aimed simply to equalise leg length in Type I deficiency, while amputation of the foot and the fitting of a prosthesis were necessary in Type II to obtain satisfactory function

We have evaluated the results of total hip replacement in patients with congenital hip disease using 46 cemented all-polyethylene Charnley acetabular components implanted with the cotyloplasty technique in 34 patients (group A), and compared them with 47 metal-backed cementless acetabular components implanted without bone grafting in 33 patients (group B). Patients in group A were treated between 1988 and 1993 and those in group B between 1990 and 1995. The mean follow-up for group A was 16.6 years (12 to 18) and the mean follow-up for group B was 13.4 years (10 to 16). Revision for aseptic loosening was undertaken in 15 hips (32.6%) in group A and in four hips (8.5%) in group B. When liner exchange was included, a total of 13 hips were revised in group B (27.7%). The mean polyethylene wear was 0.11 mm/yr (0.002 to 0.43) and 0.107 mm/yr (0 to 0.62) for groups A and B, respectively. Polyethylene wear in group A was associated with linear osteolysis, and in group B with expansile osteolysis. In patients with congenital hip disease, when 80% cover of the implant can be obtained, a cementless acetabular component appears to be acceptable and provides durable fixation. However, because of the type of osteolysis arising with these devices, early exchange of a worn liner is recommended before extensive bone loss makes revision surgery more complicated

1. Congenital dislocation of the hip in identical twins is reported. 2. The heredity of congenital dislocation of hip is discussed. Studies in twins show that congenital dislocation of hip is probably a hereditary dysplasia of the acetabulum and upper end of the femur, and that external factors play a less important role

Aims. To our knowledge, no study has compared the long-term results of cemented and hybrid total hip arthroplasty (THA) in patients with osteoarthritis (OA) secondary to congenital hip disease (CHD). This is a demanding procedure that may require special techniques and implants. Our aim was to compare the long-term outcome of cemented low-friction arthroplasty (LFA) and hybrid THA performed by one surgeon. Patients and Methods. Between January 1989 and December 1997, 58 hips (44 patients; one man, 43 woman; mean age 56.6 years (25 to 77)) with OA secondary to CHD were treated with a cemented Charnley LFA (group A), and 55 hips (39 patients; two men, 37 women; mean age 49.1 years (27 to 70)) were treated with a hybrid THA (group B), by the senior author (GH). The clinical outcome and survivorship were compared. Results. At all timepoints, group A hips had slightly better survivorship than those in group B without a statistically significant difference, except for the 24-year survival of acetabular components with revision for aseptic loosening as the endpoint, which was slightly worse. The survivorship was only significantly better in group A compared with group B when considering reoperation for any indication as the endpoint, 15 years postoperatively (74% vs 52%, p = 0.018). Conclusion. We concluded that there was not a substantial difference at almost any time in the outcome of cemented Charnley LFAs compared with hybrid THAs when treating patients with OA of the hip secondary to CHD. We believe, however, that after improvements in the design of components used in hybrid THA, this could be the method of choice, as it is technically easier with a shorter operating time. Cite this article: Bone Joint J 2019;101-B:1050–1057

1. General joint laxity affecting more than three joints was found in 7 per cent of normal schoolchildren. Similar laxity was found in fourteen of a random series of forty-eight girls, and in nineteen of twenty-six boys, with non-familial congenital dislocation of the hip. Such laxity was also found in four of seven girls and five of seven boys with familial (first degree relative affected) congenital dislocation of the hip. 2. It is concluded that persistent generalised joint laxity, which is often familial, is an important predisposing factor to congenital dislocation of the hip in boys. It is less important in girls, except perhaps in familial cases, as in girls there is an alternative temporary hormonal cause of joint laxity