The mucopolysaccharidoses (MPS) are a group of inherited lysosomal storage disorders with clinical manifestations relevant to the orthopaedic surgeon. Our aim was to review the recent advances in their management and the implications for surgical practice.

The current literature about MPSs is summarised, emphasising orthopaedic complications and their management.

Recent advances in the diagnosis and management of MPSs include the recognition of slowly progressive, late presenting subtypes, developments in life-prolonging systemic treatment and potentially new indications for surgical treatment. The outcomes of surgery in these patients are not yet validated and some procedures have a high rate of complications which differ from those in patients who do not have a MPS.

The diagnosis of a MPS should be considered in adolescents or young adults with a previously unrecognised dysplasia of the hip. Surgeons treating patients with a MPS should report their experience and studies should include the assessment of function and quality of life to guide treatment.

Cite this article: Bone Joint J 2017;99-B:1132–9

Aims

In the United Kingdom, lower incidences of intraspinal abnormalities in patients with early onset idiopathic scoliosis have been observed than in studies in other countries. We aimed to determine the rates of these abnormalities in United Kingdom patients diagnosed with idiopathic scoliosis before the age of 11 years.

Aims

The eccentric glenosphere was principally introduced into reverse shoulder arthroplasty to reduce the incidence of scapular notching. There is only limited information about the influence of its design on deltoid power and joint reaction forces.

The aim of our study was to investigate how the diameter and eccentricity of the glenosphere affect the biomechanics of the deltoid and the resultant joint reaction forces.

Aims

The aim of this study was to analyse human muscle tissue before and after rotator cuff repair to look for evidence of regeneration, and to characterise the changes seen in the type of muscle fibre.

Aims

We wished to examine the effectiveness of tibial lengthening using a two ring Ilizarov frame in skeletally immature patients. This is a potentially biomechanically unstable construct which risks the loss of axial control.

Conventional growing rods are the most commonly used distraction-based devices in the treatment of progressive early-onset scoliosis. This technique requires repeated lengthenings with the patient anaesthetised in the operating theatre. We describe the outcomes and complications of using a non-invasive magnetically controlled growing rod (MCGR) in children with early-onset scoliosis. Lengthening is performed on an outpatient basis using an external remote control with the patient awake.

Between November 2009 and March 2011, 34 children with a mean age of eight years (5 to 12) underwent treatment. The mean length of follow-up was 15 months (12 to 18). In total, 22 children were treated with dual rod constructs and 12 with a single rod. The mean number of distractions per patient was 4.8 (3 to 6). The mean pre-operative Cobb angle was 69° (46° to 108°); this was corrected to a mean 47° (28° to 91°) post-operatively. The mean Cobb angle at final review was 41° (27° to 86°). The mean pre-operative distance from T1 to S1 was 304 mm (243 to 380) and increased to 335 mm (253 to 400) in the immediate post-operative period. At final review the mean distance from T1 to S1 had increased to 348 mm (260 to 420).

Two patients developed a superficial wound infection and a further two patients in the single rod group developed a loss of distraction. In the dual rod group, one patient had pull-out of a hook and one developed prominent metalwork. Two patients had a rod breakage; one patient in the single rod group and one patient in the dual rod group. Our early results show that the MCGR is safe and effective in the treatment of progressive early-onset scoliosis with the avoidance of repeated surgical lengthenings.

Cite this article: Bone Joint J 2013;95-B:75–80.

Instability after total knee replacement (TKR) accounts for 10% to 22% of revision procedures. All patients who present for evaluation of instability require a thorough history to be taken and physical examination, as well as appropriate imaging. Deep periprosthetic infection must be ruled out by laboratory testing and an aspiration of the knee must be carried out. The three main categories of instability include flexion instability, extension instability (symmetric and asymmetric), and genu recurvatum. Most recently, the aetiologies contributing to, and surgical manoeuvres required to correct, flexion instability have been elucidated. While implant design and patient-related factors may certainly contribute to the aetiology, surgical technique is also a significant factor in all forms of post-operative instability.

Cite this article: Bone Joint J 2014;96-B(11 Suppl A):112–4.

We describe our experience in the reduction of dislocation of the hip secondary to developmental dysplasia using ultrasound-guided gradual reduction using flexion and abduction continuous traction (FACT-R). During a period of 13 years we treated 208 Suzuki type B or C complete dislocations of the hip in 202 children with a mean age of four months (0 to 11). The mean follow-up was 9.1 years (five to 16). The rate of reduction was 99.0%. There were no recurrent dislocations, and the rate of avascular necrosis of the femoral head was 1.0%. The rate of secondary surgery for residual acetabular dysplasia was 19.2%, and this was significantly higher in those children in whom the initial treatment was delayed or if other previous treatments had failed (p = 0.00045). The duration of FACT-R was significantly longer in severe dislocations (p = 0.001) or if previous treatments had failed (p = 0.018).

This new method of treatment is effective and safe in these difficult cases and offers outcomes comparable to or better than those of standard methods.

Cite this article: Bone Joint J 2015;97-B:405–11.

Aims

Our aim was to compare kinematic with mechanical alignment in total knee arthroplasty (TKA).

Aims

The purpose of this study was to report the experience of dynamic intraligamentary stabilisation (DIS) using the Ligamys device for the treatment of acute ruptures of the anterior cruciate ligament (ACL).

Objectives

The purpose of this study was to develop an accurate, reliable and easily applicable method for determining the anatomical location of the joint line during revision knee arthroplasty.

Previous studies have identified clinical and demographic risk factors for recurrence in the treatment of idiopathic clubfoot (congenital talipes equinovarus). Evertor muscle activity is not usually considered amongst them. This study aimed to evaluate whether recurrence could be predicted by demographic, clinical and gait parameters. From a series of 103 children with clubfeet, 67 had completed a follow-up of two years: 41 male and 26 female, 38 with idiopathic and 29 with non-idiopathic deformities. The mean age was 3.2 years (2.1 to 6.3). Primary correction was obtained in all 38 children (100%) with an idiopathic deformity, and in 26 of 29 patients (90%) with a non-idiopathic deformity. Overall, 60 children (90%) complied with the abduction brace regime. At a mean follow-up of 31.4 months (24 to 62), recurrence was noted in six children (15.8%) in the idiopathic and 14 children (48.3%) in the non-idiopathic group. Significant correlation was found between poor evertor activity and recurrence in both groups. No statistically significant relationship was found between the rate of recurrence and the severity of the initial deformity, the age at the time of treatment, the number of casts required or the compliance with the brace.

After correction of idiopathic and non-idiopathic clubfoot using the Ponseti method, only poor evertor muscle activity was statistically associated with recurrence. The identification of risk factors for recurrent deformity allows clinicians to anticipate problems and advocate early additional treatment to improve muscle balance around the ankle.

Cite this article: Bone Joint J 2014;96-B:1264–8.

We report the results of vertebral column resection (VCR) for paediatric patients with spinal deformity. A total of 49 VCRs in paediatric patients from four university hospitals between 2005 and 2009 with a minimum two-year follow-up were retrospectively identified. After excluding single hemivertebral resections (n = 25) and VCRs performed for patients with myelomeningocele (n = 6), as well as spondylectomies performed for tumour (n = 4), there were 14 patients who had undergone full VCR at a mean age of 12.3 years (6.5 to 17.9). The aetiology was congenital scoliosis in five, neuromuscular scoliosis in three, congenital kyphosis in two, global kyphosis in two, adolescent idiopathic scoliosis in one and secondary scoliosis in one. A total of seven anteroposterior and seven posterolateral approaches were used.

The mean major curve deformity was 86° (67° to 120°) pre-operatively and 37° (17° to 80°) at the two-year follow-up; correction was a mean of 54% (18% to 86%) in the anteroposterior and 60% (41% to 70%) in the posterolateral group at the two-year follow-up (p = 0.53). The mean Scoliosis Research Society-24 total scores were 100 (92 to 108) for the anteroposterior and 102 (95 to 105) for the posterolateral group. There was one paraparesis in the anteroposterior group necessitating urgent re-decompression, with a full recovery.

Patients undergoing VCR are highly satisfied after a successful procedure.

Objectives

Sagittal alignment of the lumbosacral spine, and specifically pelvic incidence (PI), has been implicated in the development of spine pathology, but generally ignored with regards to diseases of the hip. We aimed to determine if increased PI is correlated with higher rates of hip osteoarthritis (HOA). The effect of PI on the development of knee osteoarthritis (KOA) was used as a negative control.

Children with congenital vertical talus (CVT) have been treated with extensive soft-tissue releases, with a high rate of complications. Recently, reverse Ponseti-type casting followed by percutaneous reduction and fixation has been described, with excellent results in separate cohorts of children with CVT, of either idiopathic or teratological aetiology. There are currently no studies that compare the outcome in these two types. We present a prospective cohort of 13 children (21 feet) with CVT of both idiopathic and teratological aetiology, in which this technique has been used. Clinical, radiological and parent-reported outcomes were obtained at a mean follow-up of 36 months (8 to 57). Six children (nine feet) had associated neuromuscular conditions or syndromes; the condition was idiopathic in seven children (12 feet).

Initial correction was achieved in all children, with significant improvement in all radiological parameters. Recurrence was seen in ten feet. Modification of the technique to include limited capsulotomy at the initial operation may reduce the risk of recurrence.

The reverse Ponseti-type technique is effective in the initial correction of CVT of both idiopathic and teratological aetiology. Recurrence is a problem in both these groups, with higher rates than first reported in the original paper. However, these rates are less than those reported after open surgical release.

Cite this article: Bone Joint J 2014;96-B:274–8.

The August 2015 Knee Roundup360 looks at: Two days as good as three in TKA; Bilateral TKA: minimising the risks; Tranexamic acid in knee arthroplasty: everyone should be using it, but how?; Initial follow-up for knee arthroplasty?; Navigation finds its niche?; Another take on navigation?; Multimodal care for early knee osteoarthritis; ACL graft fixation methods under the spotlight

We report patient-reported outcomes and complications associated with contemporary periacetabular osteotomy (PAO) surgery in treating symptomatic acetabular dysplasia and compare these outcomes with total hip arthroplasty (THA) in patients with similar demographic details. Two consecutive cohorts included patients between aged 18 to 40 years who had undergone either PAO (100 hips; 24 male, 76 female) or THA (55 hips; 18 male, 37 female). At a mean follow-up of 5.9 years (2 to 13), there was significant improvement in the modified Harris hip pain (p < 0.001, PAO and p < 0.001, THA), function (p < 0.001, PAO and p = 0.001, THA), and total scores (p < 0.001, PAO and p < 0.001, THA) within each cohort. There were no significant differences in the clinical outcome scores between the groups. Complication rates were low and similar in each cohort (p = 0.68). Similar to THA, contemporary PAO surgery is a clinically effective procedure that improves function and activity levels, provides pain relief and is associated with an acceptable complication rate.

Cite this article: Bone Joint J 2015;97-B:1322–7.

The aims of this study were to determine the functional impact and financial burden of isolated and recurrent dislocation after total hip arthroplasty (THA). Our secondary goal was to determine whether there was a difference between patients who were treated non-operatively and those who were treated operatively.

We retrospectively reviewed 71 patients who had suffered dislocation of a primary THA. Their mean age was 67 years (41 to 92) and the mean follow-up was 3.8 years (2.1 to 8.2).

Because patients with recurrent dislocation were three times more likely to undergo operative treatment (p < 0.0001), they ultimately had a significantly higher mean Harris Hip Score (HHS) (p = 0.0001), lower mean Western Ontario and McMaster Universities Arthritis Index (WOMAC) scores (p = 0.001) and a higher mean SF-12 score (p < 0.0001) than patients with a single dislocation. Likewise, those who underwent operative treatment had a higher mean HHS (p < 0.0001), lower mean WOMAC score (p < 0.0001) and a higher mean SF-12 score (p < 0.0001) than those who were treated non-operatively.

Recurrent dislocation and operative treatment increased costs by 300% (£11 456; p < 0.0001) and 40% (£5217; p < 0.0001), respectively.

The operative treatment of recurrent dislocation results in significantly better function than non-operative management. Moreover, the increase in costs for operative treatment is modest compared with that of non-operative measures.

Cite this article: Bone Joint J 2015; 97-B:1046–9.