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

We investigated the pathogenesis of soft-tissue contracture in club foot, using immunohistochemistry to study 41 biopsy specimens and 12 normal deltoid ligaments from cadavers. Five biopsy specimens were studied by electron microscopy (EM) to determine the presence of myofibroblasts. All 41 specimens of club foot stained positively for vimentin as against only one of the 12 control specimens. By contrast, there was no difference in staining for desmin or α-smooth muscle actin. EM showed some variability in the appearance of ligamentous cells. Most contained bundles of microfilaments in the cytoplasm and many had abundant pinocytotic vesicles, but no basal lamina or plasmalemmal attachment plaques. Cells of the medial ligamentous tissue in patients with club foot contain vimentin and others have myofibroblastic characteristics. Both features may contribute to recurrence after soft-tissue release

We reviewed the long-term radiological outcome, complications and revision operations in 19 children with quadriplegic cerebral palsy and hip dysplasia who underwent combined peri-iliac osteotomy and femoral varus derotation osteotomy. They had a mean age of 7.5 years (1.6 to 10.9) and comprised 22 hip dislocations and subluxations. We also studied the outcome for the contralateral hip. At a mean follow-up of 11.7 years (10 to 15.1) the Melbourne cerebral palsy (CP) hip classification was grade 2 in 16 hips, grade 3 in five, and grade 5 in one. There were five complications seen in four hips (21%, four patients), including one dislocation, one subluxation, one coxa vara with adduction deformity, one subtrochanteric fracture and one infection. A recurrent soft-tissue contracture occurred in five hips and ten required revision surgery. In pre-adolescent children with quadriplegic cerebral palsy good long-term outcomes can be achieved after reconstruction of the hip; regular follow-up is required. Cite this article: Bone Joint J 2013;95-B:259–65

When performing total hip replacement (THR) in high dislocated hips, the presence of soft-tissue contractures means that most surgeons prefer to use a femoral shortening osteotomy in order to avoid the risk of neurovascular damage. However, this technique will sacrifice femoral length and reduce the extent of any leg-length equalisation. We report our experience of 74 THRs performed between 2000 and 2008 in 65 patients with a high dislocated hip without a femoral shortening osteotomy. The mean age of the patients was 55 years (46 to 72) and the mean follow-up was 42 months (12 to 78). All implants were cementless except for one resurfacing hip implant. We attempted to place the acetabular component in the anatomical position in each hip. The mean Harris hip score improved from 53 points (34 to 74) pre-operatively to 86 points (78 to 95) at final follow-up. The mean radiologically determined leg lengthening was 42 mm (30 to 66), and the mean leg-length discrepancy decreased from 36 mm (5 to 56) pre-operatively to 8.5 mm (0 to 18) postoperatively. Although there were four (5%) post-operative femoral nerve palsies, three had fully resolved by six months after the operation. No loosening of the implant was observed, and no dislocations or infections were encountered. Total hip replacement without a femoral shortening osteotomy proved to be a safe and effective surgical treatment for high dislocated hips

The potential harm to the growth plate following reconstruction of the anterior cruciate ligament in skeletally-immature patients is well documented, but we are not aware of literature on the subject of the fate of the graft itself. We have reviewed five adolescent males who underwent reconstruction of the ligament with four-strand hamstring grafts using MR images taken at a mean of 34.6 months (18 to 58) from the time of operation. The changes in dimension of the graft were measured and compared with those taken at the original operation. No growth arrest was seen on radiological or clinical measurement of leg-length discrepancy, nor was there any soft-tissue contracture. All the patients regained their pre-injury level of activity, including elite-level sport in three. The patients grew by a mean of 17.3 cm (14 to 24). The diameter of the grafts did not change despite large increases in length (mean 42%; 33% to 57%). Most of the gain in length was on the femoral side. Large changes in the length of the grafts were seen. There is a considerable increase in the size of the graft, so some neogenesis must occur; the graft must grow

Residual muscle weakness in obstetric brachial plexus palsy results in soft-tissue contractures which limit the functional range of movement and lead to progressive glenoid dysplasia and joint instability. We describe the results of surgical treatment in 98 patients (mean age 2.5 years, 0.5 to 9.0) for the correction of active abduction of the shoulder. The patients underwent transfer of the latissimus dorsi and teres major muscles, release of contractures of subscapularis pectoralis major and minor, and axillary nerve decompression and neurolysis (the modified Quad procedure). The transferred muscles were sutured to the teres minor muscle, not to a point of bony insertion. The mean pre-operative active abduction was 45° (20° to 90°). At a mean follow-up of 4.8 years (2.0 to 8.7), the mean active abduction was 162° (100° to 180°) while 77 (78.6%) of the patients had active abduction of 160° or more. No decline in abduction was noted among the 29 patients (29.6%) followed up for six years or more. This procedure involving release of the contracted internal rotators of the shoulder combined with decompression and neurolysis of the axillary nerve greatly improves active abduction in young patients with muscle imbalance secondary to obstetric brachial plexus palsy

At our institution surgical correction of symptomatic flat foot deformities in children has been guided by a paradigm in which radiographs and pedobarography are used in the assessment of outcome following treatment. Retrospective review of children with symptomatic flat feet who had undergone surgical correction was performed to assess the outcome and establish the relationship between the static alignment and the dynamic loading of the foot. A total of 17 children (21 feet) were assessed before and after correction of soft-tissue contractures and lateral column lengthening, using standardised radiological and pedobarographic techniques for which normative data were available. We found significantly improved static segmental alignment of the foot, significantly improved mediolateral dimension foot loading, and worsened fore-aft foot loading, following surgical treatment. Only four significant associations were found between radiological measures of static segmental alignment and dynamic loading of the foot. Weakness of the plantar flexors of the ankle was a common post-operative finding. Surgeons should be judicious in the magnitude of lengthening of the plantar flexors that is undertaken and use techniques that minimise subsequent weakening of this muscle group. Cite this article: Bone Joint J 2013;95-B:706–13

1. Three families with the characteristic changes of the nail-patella syndrome are reported. In addition to the well established bone and nail defects they had soft-tissue contractures, web formation around the elbows and muscular hypoplasias. The literature on soft-tissue changes is reviewed and their importance emphasised. 2. Asymmetrical joint development is considered to be a characteristic feature of the syndrome. 3. The incidence of renal disease in our families is recorded. 4. The literature on the basic defect is reviewed. The likelihood of a primary alteration in connective tissue chemistry is considered

Total hip replacement for adults with unreduced congenital dislocation presents a difficult problem because soft-tissue contractures usually prevent sitting at the normal anatomical level. Extensive soft-tissue division or a high-level acetabulum leads to reduced function and poor fixation of the components. We describe a new technique for hip replacement in such cases. The shortened abductors and flexors are released proximally and excision of the upper third of the ilium allows them to be repaired without tension, while providing bone graft to reconstruct the acetabular roof. We report 12 such replacements in 10 patients with good results and few early complications

Experience in the management of fourteen children with melorheostosis has been reviewed. The principal and presenting clinical features were unilateral soft-tissue contractures associated with inequality of limb length. In contrast to the disease in adults, pain occurred infrequently and was never intense. The average interval between the discovery of the clinical features and the correct diagnosis was six years. The distinctive radiographic feature in the child was an endosteal pattern of hyperostosis marked by streakiness of the long bones and spotting of the small. This differs from the usual subperiosteal or extracortical pattern of hyperostosis seen in adults. The surgical treatment of the contractures proved difficult and recurrence of the deformity was the rule. Distal ischaemia occurred when the chronically contracted and flexed joint was rapidly extended

We reviewed two similar groups of patients with medial osteoarthritis of the knee treated by unicompartmental arthroplasty. The group receiving an Oxford meniscal-bearing implant, with no medial release, showed significantly better mechanical alignment than that receiving a fixed-bearing implant. Under-correction, with its ominous mechanical implications, was much more common with the fixed-bearing design. Over-correction was rare and was seen in both designs about equally. Degenerative stenosis of the intercondylar notch was common and appeared to put the anterior cruciate ligament at risk of rupture, especially after correction of the varus deformity. We consider that postoperative leg alignment and soft-tissue balance after unicompartmental knee replacement are determined more by the implant design and the surgical technique than by any variation in soft-tissue contracture. Release of the medial collateral ligament is not necessary for realignment, but a generous notchplasty is often needed to allow normal anterior cruciate ligament function

We have compared the density of nerve fibres in the synovium in club foot with that of specimens obtained from the synovium of the hip at operations for developmental dysplasia. The study focused on the sensory neuropeptides substance P; calcitonin gene-related peptide; protein gene product 9.5, a general marker for mature peripheral nerve fibres; and growth associated protein 43, a neuronal marker for new or regenerating nerve fibres. In order to establish whether there might be any inherent difference we analysed the density of calcitonin gene-related peptide-positive nerve fibres in the hip and ankle joints in young rats. Semi-quantitative analysis showed a significant reduction in the number of sensory and mature nerve fibres in the synovium in club foot compared with the control hips. Calcitonin gene-related peptide (CGRP) positive fibres were reduced by 28%, substance P-positive fibres by 36% and protein gene product 9.5-positive fibres by 52% in club foot. The growth associated protein 43-positive fibres also seemed to be less in six samples of club foot. No difference in the density of CGRP-positive nerve fibres was observed in the synovium between ankle and hip joints in rats. The lack of sensory input may be responsible for the fibrosis and soft-tissue contractures associated with idiopathic club foot

Orthopaedic surgeons are currently faced with an overwhelming number of choices surrounding total knee arthroplasty (TKA), not only with the latest technologies and prostheses, but also fundamental decisions on alignment philosophies. From ‘mechanical’ to ‘adjusted mechanical’ to ‘restricted kinematic’ to ‘unrestricted kinematic’ — and how constitutional alignment relates to these — there is potential for ambiguity when thinking about and discussing such concepts. This annotation summarizes the various alignment strategies currently employed in TKA. It provides a clear framework and consistent language that will assist surgeons to compare confidently and contrast the concepts, while also discussing the latest opinions about alignment in TKA. Finally, it provides suggestions for applying consistent nomenclature to future research, especially as we explore the implications of 3D alignment patterns on patient outcomes.

Cite this article: Bone Joint J 2023;105-B(2):102–108.

Aims

The aim of this study was to evaluate whether achieving medial joint opening, as measured by the change in the joint line convergence angle (∆JLCA), is a better predictor of clinical outcomes after high tibial osteotomy (HTO) compared with the mechanical axis deviation, and to find individualized targets for the redistribution of load that reflect bony alignment, joint laxity, and surgical technique.

Aims

Giant cell tumour of bone (GCTB) is a locally aggressive lesion that is difficult to treat as salvaging the joint can be associated with a high rate of local recurrence (LR). We evaluated the risk factors for tumour relapse after treatment of a GCTB of the limbs.

Aims

The aim of this study was to develop and internally validate a prognostic nomogram to predict the probability of gaining a functional range of motion (ROM ≥ 120°) after open arthrolysis of the elbow in patients with post-traumatic stiffness of the elbow.

Aims

In our unit, we adopt a two-stage surgical reconstruction approach using internal fixation for the management of infected Charcot foot deformity. We evaluate our experience with this functional limb salvage method.

Aims

The patient-acceptable symptom state (PASS) is a level of wellbeing, which is measured by the patient. The aim of this study was to determine if the proportion of patients who achieved an acceptable level of function (PASS) after medial unicompartmental knee arthroplasty (UKA) was different based on the status of the anterior cruciate ligament (ACL) at the time of surgery.

Aims

To describe and analyze the mid-term functional outcomes of a large series of patients who underwent the Hoffer procedure for brachial plexus birth palsy (BPBP).

Aims

The aim of this study was to analyze the results of reverse shoulder arthroplasty (RSA) in patients with type 1 sequelae of a fracture of the proximal humerus in association with rotator cuff deficiency or severe stiffness of the shoulder.