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Orthopaedic Proceedings
Vol. 95-B, Issue SUPP_3 | Pages 15 - 15
1 Jan 2013
Barron E Rambani R Sharma H
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The present study was conducted to evaluate the cost of physiotherapy both for inpatient and outpatient services. Significant physiotherapy resources are required to rehabilitate patients with an Ilizarov or Taylor Spatial Frame (TSF). Within Hull and East Yorkshire Hospitals NHS Trust Physiotherapy department the average number of outpatient treatment sessions per routine patient is 6. In comparison, the average number of treatment sessions required for a patient with an ilizarov (or TSF) is 24 for a trauma patient and 33 for a patient undergoing an elective procedure. Seventy three (73) patients received physiotherapy treatment with an Ilizarov frame or a Taylor spatial frame between April 2008 and April 2010. Physiotherapy input was recorded (in minutes) for the patients identified. This included treatment received as an inpatient as well as an outpatient (if the patient received their treatment within Hull and East Yorkshire NHS trust). Data collection was divided into either trauma or elective procedure for analytical purposes. The average cost of physiotherapy treatment to Hull and East Yorkshire Hospitals NHS Trust for an inpatient with an ilizarov frame is £121.82 per case (trauma) and £133.15 per case (elective). The average cost of physiotherapy treatment to Hull and East Yorkshire Hospitals NHS Trust for an outpatient for a trauma case was calculated as £404.65 and £521.41for an average elective case. This is in comparison to a routine patient costing the service £60.29 (when treated by a Band 7 physiotherapist). The present study gives valuable data for future business planning and assistance with the setting of local or national tariffs for the treatment of this patient group


Orthopaedic Proceedings
Vol. 98-B, Issue SUPP_15 | Pages 15 - 15
1 Sep 2016
Saville S Atherton S Ayodele O Walton R Bruce C
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We present a review of our Specialist Physiotherapy clinic for normal physiological variations of the lower limb (SPNV) clinics, demonstrating them to be clinically effective and cost effective. Children with normal variation of rotational profile and limb angulation present much anxiety to parents and primary care. Providing consultation: to eliminate significant pathology and reassure families, is an important service that a Paediatric Orthopaedic department provides. In our tertiary referral department we have a Specialist Physiotherapy led clinics into which primary care practitioners refer children with whom there are concerns about lower limb development variation. The (SPNV) Clinic was first set up by a Consultant and Senior Physiotherapist in 1999. The aim of the clinic was to reduce the waiting times for incoming referrals but ensuring they are seen in an appropriate environment by an experienced health care professional. Clinics are run by Senior Specialist Physiotherapists, alongside Consultant clinics who are available for advice and direction. This provides security for the physios, the Trust and the patient. Over 15 years there have been more than 4000 patient visits to this clinic. Over 80% were new patient visits. 70% of these visits were discharged in one or two reviews. 97.4% of new referrals were discharged without subsequent review by an orthopaedic surgeon. The most common conditions reviewed were Genu valgum (25%), Genu Varum (16%), intoeing (17%) and flexible flat feet (11%). The clinic has proven to be cost effective as well in drawing in up to £500,000 revenue into the trust in a single year. The department has been approached by other trusts to assist in the implementation of similar clinics. We present this review of the patients, as a template for supporting the work of Paediatric Orthopaedic Departments. This service has facilitated the streamlining of our Consultant Paediatric Orthopaedic clinics


The Bone & Joint Journal
Vol. 106-B, Issue 5 | Pages 501 - 507
1 May 2024
Galloway AM Keene DJ Anderson A Holton C Redmond AC Siddle HJ Richards S Perry DC

Aims

The aim of this study was to produce clinical consensus recommendations about the non-surgical treatment of children with Perthes’ disease. The recommendations are intended to support clinical practice in a condition for which there is no robust evidence to guide optimal care.

Methods

A two-round, modified Delphi study was conducted online. An advisory group of children’s orthopaedic specialists consisting of physiotherapists, surgeons, and clinical nurse specialists designed a survey. In the first round, participants also had the opportunity to suggest new statements. The survey included statements related to ‘Exercises’, ‘Physical activity’, ‘Education/information sharing’, ‘Input from other services’, and ‘Monitoring assessments’. The survey was shared with clinicians who regularly treat children with Perthes’ disease in the UK using clinically relevant specialist groups and social media. A predetermined threshold of ≥ 75% for consensus was used for recommendation, with a threshold of between 70% and 75% being considered as ‘points to consider’.


The Bone & Joint Journal
Vol. 95-B, Issue 2 | Pages 271 - 278
1 Feb 2013
Singh AK Roshan A Ram S

The Ponseti and French taping methods have reduced the incidence of major surgery in congenital idiopathic clubfoot but incur a significant burden of care, including heel-cord tenotomy. We developed a non-operative regime to reduce treatment intensity without affecting outcome. We treated 402 primary idiopathic clubfeet in patients aged < three months who presented between September 1991 and August 2008. Their Harrold and Walker grades were 6.0% mild, 25.6% moderate and 68.4% severe. All underwent a dynamic outpatient taping regime over five weeks based on Ponseti manipulation, modified Jones strapping and home exercises. Feet with residual equinus (six feet, 1.5%) or relapse within six months (83 feet, 20.9%) underwent one to three additional tapings. Correction was maintained with below-knee splints, exercises and shoes. The clinical outcome at three years of age (385 feet, 95.8% follow-up) showed that taping alone corrected 357 feet (92.7%, ‘good’). Late relapses or failure of taping required limited posterior release in 20 feet (5.2%, ‘fair’) or posteromedial release in eight feet (2.1%, ‘poor’). The long-term (> 10 years) outcomes in 44 feet (23.8% follow-up) were assessed by the Laaveg–Ponseti method as excellent (23 feet, 52.3%), good (17 feet, 38.6%), fair (three feet, 6.8%) or poor (one foot, 2.3%). These compare favourably with published long-term results of the Ponseti or French methods. This dynamic taping regime is a simple non-operative method that delivers improved medium-term and promising long-term results.

Cite this article: Bone Joint J 2013;95-B:271–8.


Bone & Joint Open
Vol. 1, Issue 12 | Pages 720 - 730
1 Dec 2020
Galloway AM van-Hille T Perry DC Holton C Mason L Richards S Siddle HJ Comer C

Aims. Perthes’ disease is a condition leading to necrosis of the femoral head. It is most common in children aged four to nine years, affecting around one per 1,200 children in the UK. Management typically includes non-surgical treatment options, such as physiotherapy with/without surgical intervention. However, there is significant variation in care with no consensus on the most effective treatment option. Methods. This systematic review aims to evaluate the effectiveness of non-surgical interventions for the treatment of Perthes’ disease. Comparative studies (experimental or observational) of any non-surgical intervention compared directly with any alternative intervention (surgical, non-surgical or no intervention) were identified from: Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE, the Cumulative Index to Nursing and Allied Health Literature (CINAHL), EMcare, Allied and Complementary Medicine Database (AMED), and the Physiotherapy Evidence Database (PEDro). Data were extracted on interventions compared and methodological quality. For post-intervention primary outcome of radiological scores (Stulberg and/or Mose), event rates for poor scores were calculated with significance values. Secondary outcomes included functional measures, such as range of movement, and patient-reported outcomes such as health-related quality of life. Results. In all, 15 studies (1,745 participants) were eligible for inclusion: eight prospective cohort studies, seven retrospective cohort studies, and no randomized controlled trials were identified. Non-surgical interventions largely focused on orthotic management (14/15 studies) and physical interventions such as muscle strengthening or stretching (5/15 studies). Most studies were of high/unknown risk of bias, and the range of patient outcomes was very limited, as was reporting of treatment protocols. Similar proportions of children achieving poor radiological outcomes were found for orthotic management and physical interventions, such as physiotherapy or weightbearing alteration, compared with surgical interventions or no intervention. Conclusion. Evidence from non-randomized studies found no robust evidence regarding the most effective non-surgical interventions for the treatment of children with Perthes’ disease. Future research, employing randomized trial designs, and reporting a wider range of patient outcomes is urgently needed to inform clinical practice. Cite this article: Bone Jt Open 2020;1-12:720–730


Bone & Joint Open
Vol. 1, Issue 11 | Pages 691 - 695
1 Nov 2020
Galloway AM Holton C Parnami V Wood M Craven J Green N Siddle HJ Richards S Comer C

Aims. Perthes’ disease is a condition which leads to necrosis of the femoral head. It is most commonly reported in children aged four to nine years, with recent statistics suggesting it affects around five per 100,000 children in the UK. Current treatment for the condition aims to maintain the best possible environment for the disease process to run its natural course. Management typically includes physiotherapy with or without surgical intervention. Physiotherapy intervention often will include strengthening/stretching programmes, exercise/activity advice, and, in some centres, will include intervention, such as hydrotherapy. There is significant variation in care with no consensus on which treatment option is best. The importance of work in this area has been demonstrated by the British Society for Children’s Orthopaedic Surgery through the James Lind Alliance’s prioritization of work to determine/identify surgical versus non-surgical management of Perthes’ disease. It was identified as the fourth-highest priority for paediatric lower limb surgery research in 2018. Methods. Five UK NHS centres, including those from the NEWS (North, East, West and South Yorkshire) orthopaedic group, contributed to this case review, with each entre providing clinical data from a minimum of five children. Information regarding both orthopaedic and physiotherapeutic management over a two-year post-diagnosis period was reviewed. Results. Data were extracted from the clinical records of 32 children diagnosed with Perthes’ disease; seven boys and 25 girls. The mean age of the children at diagnosis was 6.16 years (standard deviation (SD) 3.001). In all, 26 children were referred for physiotherapy. In the two-year period following diagnosis, children were seen a median of 7.5 times (interquartile range (IQR) 4.25 to 11) by an orthopaedic surgeon, and a median of 9.5 times (IQR 8 to 18.25) by a physiotherapist. One centre had operated on all of their children, while another had operated on none. Overall, 17 (53%) of the children were managed conservatively in the two-year follow-up period, and 15 (47%) of the children underwent surgery in the two-year follow-up period. Conclusion. The results of this case review demonstrate a variation of care provided to children in the UK with Perthes’ disease. Further national and international understanding of current care is required to underpin the rationale for different treatment options in children with Perthes’ disease. Cite this article: Bone Joint Open 2020;1-11:691–695


The Journal of Bone & Joint Surgery British Volume
Vol. 90-B, Issue 10 | Pages 1364 - 1371
1 Oct 2008
Wiig O Terjesen T Svenningsen S

This nationwide prospective study was designed to determine prognostic factors and evaluate the outcome of different treatments of Perthes’ disease. A total of 28 hospitals in Norway were instructed to report all new cases of Perthes’ disease over a period of five years and 425 patients were reported and followed for five years. Of these, 368 with unilateral disease were included in the present study. The hips were classified radiologically according to a modified two-group Catterall classification and the lateral pillar classification. A total of 358 patients (97%) attended the five-year follow-up, when a modified three-group Stulberg classification was used as a radiological outcome measure. For patients over six years of age at diagnosis and with more than 50% necrosis of the femoral head (152 patients), the surgeons at the different hospitals had chosen one of three methods of treatment: physiotherapy (55 patients), the Scottish Rite abduction orthosis (26), and proximal femoral varus osteotomy (71). Of these hips, 146 (96%) were available for the five-year follow-up. The strongest predictor of outcome was femoral head involvement of more or less than 50% (odds ratio (OR) = 7.76, 95% confidence interval (CI) 2.82 to 21.37), followed by age at diagnosis (OR = 0.98, 95% CI 0.92 to 0.99) and the lateral pillar classification (OR = 0.62, 95% CI 0.40 to 0.98). In children over six years at diagnosis with more than 50% of femoral head necrosis, proximal femoral varus osteotomy gave a significantly better outcome than orthosis (p = 0.001) or physiotherapy (p = 0.001). There was no significant difference between the physiotherapy and orthosis groups (p = 0.36), and we found no difference in outcome after any of the treatments in children under six years (p = 0.73). We recommend proximal femoral varus osteotomy in children aged six years and over at the time of diagnosis with hips having more than 50% femoral head necrosis. The abduction orthosis should be abandoned in Perthes’ disease


The Bone & Joint Journal
Vol. 105-B, Issue 4 | Pages 455 - 464
15 Mar 2023
de Joode SGCJ Meijer R Samijo S Heymans MJLF Chen N van Rhijn LW Schotanus MGM

Aims

Multiple secondary surgical procedures of the shoulder, such as soft-tissue releases, tendon transfers, and osteotomies, are described in brachial plexus birth palsy (BPBP) patients. The long-term functional outcomes of these procedures described in the literature are inconclusive. We aimed to analyze the literature looking for a consensus on treatment options.

Methods

A systematic literature search in healthcare databases (PubMed, Embase, the Cochrane library, CINAHL, and Web of Science) was performed from January 2000 to July 2020, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. The quality of the included studies was assessed with the Cochrane ROBINS-I risk of bias tool. Relevant trials studying BPBP with at least five years of follow-up and describing functional outcome were included.


The Bone & Joint Journal
Vol. 106-B, Issue 6 | Pages 623 - 630
1 Jun 2024
Perry DC Dritsaki M Achten J Appelbe D Knight R Widnall J Roland D Messahel S Costa ML Mason J

Aims

The aim of this trial was to assess the cost-effectiveness of a soft bandage and immediate discharge, compared with rigid immobilization, in children aged four to 15 years with a torus fracture of the distal radius.

Methods

A within-trial economic evaluation was conducted from the UK NHS and personal social services (PSS) perspective, as well as a broader societal point of view. Health resources and quality of life (the youth version of the EuroQol five-dimension questionnaire (EQ-5D-Y)) data were collected, as part of the Forearm Recovery in Children Evaluation (FORCE) multicentre randomized controlled trial over a six-week period, using trial case report forms and patient-completed questionnaires. Costs and health gains (quality-adjusted life years (QALYs)) were estimated for the two trial treatment groups. Regression was used to estimate the probability of the new treatment being cost-effective at a range of ‘willingness-to-pay’ thresholds, which reflect a range of costs per QALY at which governments are typically prepared to reimburse for treatment.


Bone & Joint Open
Vol. 4, Issue 11 | Pages 825 - 831
1 Nov 2023
Joseph PJS Khattak M Masudi ST Minta L Perry DC

Aims

Hip disease is common in children with cerebral palsy (CP) and can decrease quality of life and function. Surveillance programmes exist to improve outcomes by treating hip disease at an early stage using radiological surveillance. However, studies and surveillance programmes report different radiological outcomes, making it difficult to compare. We aimed to identify the most important radiological measurements and develop a core measurement set (CMS) for clinical practice, research, and surveillance programmes.

Methods

A systematic review identified a list of measurements previously used in studies reporting radiological hip outcomes in children with CP. These measurements informed a two-round Delphi study, conducted among orthopaedic surgeons and specialist physiotherapists. Participants rated each measurement on a nine-point Likert scale (‘not important’ to ‘critically important’). A consensus meeting was held to finalize the CMS.


Bone & Joint Open
Vol. 4, Issue 10 | Pages 735 - 741
2 Oct 2023
Galloway AM Pini S Holton C Perry DC Redmond A Siddle HJ Richards S

Aims

Perthes’ disease is an idiopathic avascular necrosis of the developing femoral head, often causing deformity that impairs physical function. Current treatments aim to optimize the joint reaction force across the hip by enhancing congruency between the acetabulum and femoral head. Despite a century of research, there is no consensus regarding the optimal treatment. The aim of this study was to describe the experiences of children, their families, and clinicians when considering the treatment of Perthes’ disease.

Methods

A qualitative study gathered information from children and their families affected by Perthes’ disease, along with treating clinicians. Interviews followed a coding framework, with the interview schedule informed by behavioural theory and patient and public involvement. Transcripts were analyzed using the framework method.


The Bone & Joint Journal
Vol. 104-B, Issue 9 | Pages 1081 - 1088
1 Sep 2022
Behman AL Bradley CS Maddock CL Sharma S Kelley SP

Aims

There is no consensus regarding optimum timing and frequency of ultrasound (US) for monitoring response to Pavlik harness (PH) treatment in developmental dysplasia of the hip (DDH). The purpose of our study was to determine if a limited-frequency hip US assessment had an adverse effect on treatment outcomes compared to traditional comprehensive US monitoring.

Methods

This study was a single-centre noninferiority randomized controlled trial. Infants aged under six months whose hips were reduced and centred in the harness at initiation of treatment (stable dysplastic or subluxable), or initially decentred (subluxated or dislocated) but reduced and centred within four weeks of PH treatment, were randomized to our current standard US monitoring protocol (every clinic visit) or to a limited-frequency US protocol (US only at end of treatment). Groups were compared based on α angle and femoral head coverage at the end of PH treatment, acetabular indices, and International Hip Dysplasia Institute (IHDI) grade on one-year follow-up radiographs.


The Bone & Joint Journal
Vol. 104-B, Issue 11 | Pages 1273 - 1278
1 Nov 2022
Chowdhury JMY Ahmadi M Prior CP Pease F Messner J Foster PAL

Aims

The aim of this retrospective cohort study was to assess and investigate the safety and efficacy of using a distal tibial osteotomy compared to proximal osteotomy for limb lengthening in children.

Methods

In this study, there were 59 consecutive tibial lengthening and deformity corrections in 57 children using a circular frame. All were performed or supervised by the senior author between January 2013 and June 2019. A total of 25 who underwent a distal tibial osteotomy were analyzed and compared to a group of 34 who had a standard proximal tibial osteotomy. For each patient, the primary diagnosis, time in frame, complications, and lengthening achieved were recorded. From these data, the frame index was calculated (days/cm) and analyzed.


Bone & Joint Open
Vol. 4, Issue 11 | Pages 865 - 872
15 Nov 2023
Hussain SA Russell A Cavanagh SE Bridgens A Gelfer Y

Aims

The Ponseti method is the gold standard treatment for congenital talipes equinovarus (CTEV), with the British Consensus Statement providing a benchmark for standard of care. Meeting these standards and providing expert care while maintaining geographical accessibility can pose a service delivery challenge. A novel ‘Hub and Spoke’ Shared Care model was initiated to deliver Ponseti treatment for CTEV, while addressing standard of care and resource allocation. The aim of this study was to assess feasibility and outcomes of the corrective phase of Ponseti service delivery using this model.

Methods

Patients with idiopathic CTEV were seen in their local hospitals (‘Spokes’) for initial diagnosis and casting, followed by referral to the tertiary hospital (‘Hub’) for tenotomy. Non-idiopathic CTEV was managed solely by the Hub. Primary and secondary outcomes were achieving primary correction, and complication rates resulting in early transfer to the Hub, respectively. Consecutive data were prospectively collected and compared between patients allocated to Hub or Spokes. Mann-Whitney U test, Wilcoxon signed-rank test, or chi-squared tests were used for analysis (alpha-priori = 0.05, two-tailed significance).


Bone & Joint Open
Vol. 5, Issue 1 | Pages 69 - 77
25 Jan 2024
Achten J Appelbe D Spoors L Peckham N Kandiyali R Mason J Ferguson D Wright J Wilson N Preston J Moscrop A Costa M Perry DC

Aims

The management of fractures of the medial epicondyle is one of the greatest controversies in paediatric fracture care, with uncertainty concerning the need for surgery. The British Society of Children’s Orthopaedic Surgery prioritized this as their most important research question in paediatric trauma. This is the protocol for a randomized controlled, multicentre, prospective superiority trial of operative fixation versus nonoperative treatment for displaced medial epicondyle fractures: the Surgery or Cast of the EpicoNdyle in Children’s Elbows (SCIENCE) trial.

Methods

Children aged seven to 15 years old inclusive, who have sustained a displaced fracture of the medial epicondyle, are eligible to take part. Baseline function using the Patient-Reported Outcomes Measurement Information System (PROMIS) upper limb score, pain measured using the Wong Baker FACES pain scale, and quality of life (QoL) assessed with the EuroQol five-dimension questionnaire for younger patients (EQ-5D-Y) will be collected. Each patient will be randomly allocated (1:1, stratified using a minimization algorithm by centre and initial elbow dislocation status (i.e. dislocated or not-dislocated at presentation to the emergency department)) to either a regimen of the operative fixation or non-surgical treatment.


The Journal of Bone & Joint Surgery British Volume
Vol. 85-B, Issue 2 | Pages 261 - 264
1 Mar 2003
Hosalkar HS Jones S Chowdhury M Hartley J Hill RA

We review the results of a modified quadricepsplasty in five children who developed stiffness of the knee after femoral lengthening for congenital short femur using an Ilizarov external fixator which spanned the knee. All had a full range of movement of the knee before lengthening was undertaken. Unifocal lengthening was carried out in the distal metaphysiodiaphyseal region of the distal femur with a mean gain of 6.5 cm. The mean percentage lengthening was 24%. At the end of one year after removal of the Ilizarov frame and despite intensive physiotherapy all patients had stiffness. Physiotherapy was continued after the quadricepsplasty and, at the latest follow-up (mean 27 months), the mean active flexion was 102° (80 to 130). The gain in movement ranged from 50° to 100°. One patient had a superficial wound infection which settled after a course of oral antibiotics. None developed an increased extension lag after surgery and all were very satisfied with the results. Quadricepsplasty is a useful procedure for stiffness of the knee after femoral lengthening which has not responded to physiotherapy


The Bone & Joint Journal
Vol. 104-B, Issue 4 | Pages 510 - 518
1 Apr 2022
Perry DC Arch B Appelbe D Francis P Craven J Monsell FP Williamson P Knight M

Aims

The aim of this study was to evaluate the epidemiology and treatment of Perthes’ disease of the hip.

Methods

This was an anonymized comprehensive cohort study of Perthes’ disease, with a nested consented cohort. A total of 143 of 144 hospitals treating children’s hip disease in the UK participated over an 18-month period. Cases were cross-checked using a secondary independent reporting network of trainee surgeons to minimize those missing. Clinician-reported outcomes were collected until two years. Patient-reported outcome measures (PROMs) were collected for a subset of participants.


Orthopaedic Proceedings
Vol. 95-B, Issue SUPP_24 | Pages 13 - 13
1 May 2013
Garala K Haque A Abraham A
Full Access

Aim. Iliopsoas tendinopathy occurs due to friction of the iliopsoas tendon on the iliopectineal bar or the hip capsule causing pain and snapping of the hip. In adolescent cases this condition is generally treated conservatively with physiotherapy, stretching and NSAIDs. Our aim was to study the outcome of non-operative management of iliopsoas tendinopathy. Method. A retrospective 4-year study identified adolescent patients with a clinical diagnosis of psoas tendinopathy managed non-operatively. These patients were followed up by postal questionnaire, which included functional assessment using the Non-Arthritic Hip Score (NAHS). A custom study questionnaire assessed current symptoms and the effects of physiotherapy. All patients not treated conservatively were removed from the study. Results. 36 patients were treated conservatively for psoas tendinopathy. 28 completed and returned the questionnaires to a satisfactory standard. Mean NAHS was 77.72 (CI: 69.91 to 85.54) at a mean follow up of 37 months (Range: 14 to 58 months; CI 31 to 43 months). Pain was the least disabling NAHS domain at final follow up with a significant improvement over the follow up period (p<0.001). 11 patients suffered from recurrence of symptoms (clicking, stiffness, decreased range of motion). 7 patients (25%) complained of significant pain following conservative management. A Numbers Needed to Treat analysis (NNT) was performed, comparing our data with that of surgically managed patients in previously published studies. This analysis suggests that for every 6 surgical releases, 5 would have recovered anyway with non-operative management. Conclusion. Non-operative management is successful in managing psoas tendinopathy in about 75% of adolescent patients. It is especially effective in males, and patients who are more active prior to development of symptoms


The Bone & Joint Journal
Vol. 103-B, Issue 12 | Pages 1815 - 1820
1 Dec 2021
Huhnstock S Wiig O Merckoll E Svenningsen S Terjesen T

Aims

The aim of this study was to assess the prognostic value of the modified three-group Stulberg classification, which is based on the sphericity of the femoral head, in patients with Perthes’ disease.

Methods

A total of 88 patients were followed from the time of diagnosis until a mean follow-up of 21 years. Anteroposterior pelvic and frog-leg lateral radiographs were obtained at diagnosis and at follow-up of one, five, and 21 years. At the five- and 21-year follow-up, the femoral heads were classified using a modified three-group Stulberg classification (round, ovoid, or flat femoral head). Further radiological endpoints at long-term follow-up were osteoarthritis (OA) of the hip and the requirement for total hip arthroplasty (THA).


The Journal of Bone & Joint Surgery British Volume
Vol. 79-B, Issue 4 | Pages 576 - 582
1 Jul 1997
Haddad FS Jones DHA Vellodi A Kane N Pitt MC

Children with a mucopolysaccharidosis or mucolipidosis suffer progressive disability of the hands, particularly in relation to dysfunction of the median nerve. This is an increasing problem because bone-marrow transplantation has dramatically improved survival without apparently changing the musculoskeletal manifestations. We have reviewed 48 children with these syndromes who required carpal tunnel decompression, recording symptoms, signs, radiological, electrophysiological and operative findings, histology and upper-limb function. In these children the carpal tunnel syndrome differs from that seen in adults. Symptoms are rare but signs such as decreased sweating, pulp atrophy, thenar wasting and manual clumsiness are much more common. At operation, the flexor retinaculum was thickened and a mass of white tenosynovium engulfed the flexor tendons. Most patients had some definite nerve constriction with a thickened epineurium. Functional improvement was seen after early decompression, with some benefit from simultaneous tendon release. Regular physiotherapy helped to maintain increased hand movement. We describe our assessment protocol, the physiotherapy and operative regime and the standard functional review which helps to maximise function in the hands and upper limbs of these children