Management of Fibro-adipose Vascular Anomalies (FAVA) in Paediatric Practice.

Fibro-adipose vascular anomaly (FAVA) is a discrete type of vascular anomaly. We describe our experience managing FAVA at a tertiary level paediatric hospital and offer a treatment algorithm. METHODS: A retrospective review of 27 patients with proven FAVA was undertaken. All patients had undergone MRI and USS evaluation. Patient demographics, presenting concerns, treatment methods, and outcomes were recorded and evaluation with the paediatric outcomes data collection instrument (PODCI) completed a minimum of 12 months after definitive treatment. RESULTS: Mean age at presentation was 8.9 years (range: 9 m-17.4 y) and mean post-treatment follow-up was 7.4 y (range: 2 y-11.6 y). Twenty of 27 lesions affected the lower limb. Severe neurogenic-type pain was present in 23 cases and contractures across joints in 11 cases. Sclerotherapy with sodium tetradecyl sulphate was used in 11 cases, with no improvement in symptoms. Cryoablation provided pain relief in 3/4 cases, but contracture subsequently increased in one patient and pain recurred in another.Fourteen cases underwent surgery (four surgical excisions alone, 10 in combination with other procedures). Three patients required four further surgical procedures that include one amputation for intractable pain and poor function.PODCI evaluations suggest overall good function, with surgical management and interventional radiology that provide comparable results. Surgery did correct deformity. CONCLUSION: If conservative measures or cryoablation fail to achieve symptomatic control, surgical excision should be considered, combined with adjunctive procedures, to correct contractures and balance muscle forces.Relief of pain may compensate for the loss of muscle mass and overall improves function. Multidisciplinary team working is essential.

Non-operative versus operative management of open fractures in the paediatric population: A systematic review and meta-analysis of the adverse outcomes.

Open fractures are at greater risk of infection and delayed bone healing. Guidelines to reduce these risks exist for adult open fracture management but not for paediatric open fractures, where there is considerable practice variability. This systematic review evaluates the quality of the evidence and clinical outcomes for paediatric open fractures treated non-operatively versus operatively. A PROSPERO-registered, PRISMA-compliant systematic review and meta-analysis. Databases searched were MEDLINE, Embase and Web of Science (WoS) up to March 2019. Clinical studies reporting adverse outcomes of both non-operative and operative management of paediatric open fractures were included. Data extracted included demographics, treatment methods and outcomes. The primary outcome was infection (osteomyelitis and/or wound infection). Secondary outcomes were abnormalities of bone healing (delayed union, malunion and nonunion) and re-fracture. The ROBINS-I tool was used to assess risk of bias. Seventeen studies reporting 1093 open fractures were included. Non-operatively managed injuries had a lower risk of osteomyelitis (RR 0.33 [95%CI 0.12-0.86]), wound infection (RR 0.47 [95%CI 0.22-0.97]) and nonunion (RR 0.27 [95%CI 0.09-0.80]). Gustilo-Anderson (GA) III injuries had the highest incidence of osteomyelitis (10.7%) with no difference in outcome between operative and non-operative groups (RR 0.67 [95%CI 0.22-2.03]). Tibial GA III fractures were associated with a lower risk of osteomyelitis than femoral fractures: adverse effects were seen in the operative group. All studies were retrospective, observational and at high risk of bias. The quality of the evidence relating to paediatric open fractures is low, and findings are limited by significant methodological flaws in the literature. GA I and II fractures were commonly managed non-operatively and associated with a lower infection rate. Operative intervention was more frequent in GA III fractures, where the risk of infection was highest. Operative management of GA III fractures was not associated with a lower infection risk compared to non-operative management. Robust prospective multi-centre studies are needed to explore further the most effective management of paediatric open fractures and to inform guideline development.

Congenital talipes equinovarus: a systematic review of relapse as a primary outcome of the Ponseti method.

AIMS: The Ponseti method is the benchmark treatment for the correction of clubfoot. The primary rate of correction is very high, but outcome further down the treatment pathway is less predictable. Several methods of assessing severity at presentation have been reported. Classification later in the course of treatment is more challenging. This systematic review considers the outcome of the Ponseti method in terms of relapse and determines how clubfoot is assessed at presentation, correction, and relapse. PATIENTS AND METHODS: A prospectively registered systematic review was carried out according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Studies that reported idiopathic clubfoot treated by the Ponseti method between 1 January 2012 and 31 May 2017 were included. The data extracted included demographics, Ponseti methodology, assessment methods, and rates of relapse and surgery. RESULTS: A total of 84 studies were included (7335 patients, 10 535 clubfeet). The relapse rate varied between 1.9% and 45%. The rates of relapse and major surgery (1.4% to 53.3%) and minor surgery (0.6% to 48.8%) both increased with follow-up time. There was high variability in the assessment methods used across timepoints; only 57% of the studies defined relapse. Pirani scoring was the method most often used. CONCLUSION: Recurrence and further surgical intervention in idiopathic clubfoot increases with the duration of follow-up. The corrected and the relapsed foot are poorly defined, which contributes to variability in outcome. The results suggest that a consensus for a definition of relapse is needed. Cite this article: Bone Joint J 2019;101-B:639-645.

Universal or selective ultrasound screening for developmental dysplasia of the hip? A discussion of the key issues.

PURPOSE: To summarize recent developments and provide recommendations as to whether universal or selective programmes are advisable. METHODS: A literature review was performed and preference given to studies with higher levels of evidence. All programmes reviewed included clinical screening. RESULTS: Recent studies underline the need for high quality screening programmes to promote the early detection of developmental dysplasia of the hip (DDH). A small number of cases may be missed clinically but with universal ultrasound screening programmes the late presentation rates appear to be virtually zero. Contemporary studies show treatment rates with universal screening programmes which are now lower than those with selective ultrasound. There is little agreement over the criteria used for selective programmes. Alternative outcome measures, such as the first operation rate or the percentage undergoing major (open) surgery are both lowest with universal ultrasound screening programmes. Furthermore, a significant reduction in the rate of surgery for DDH later in life was seen after the introduction of universal ultrasound screening, whereas the defined criteria for selective screening may not detect the majority of patients who require late surgery. Abduction bracing with modern orthoses is associated with a zero rate of avascular necrosis (AVN), whereas closed reduction techniques have an overall risk of 10%. CONCLUSION: On clinical grounds, if future studies confirm that hip abduction in flexible orthoses is not associated with AVN, it may be time for a paradigm shift of screening for DDH towards a universal ultrasound protocol. The costs associated both with each type of screening programme and with the management of late presenting cases are also important but may be secondary to clinical benefit.

Posteromedial bowing of the tibia: a benign condition or a case for limb reconstruction?

PURPOSE: To review the initial deformity and subsequent remodelling in posteromedial bowing of the tibia and the outcome of limb reconstruction in this condition. PATIENTS AND METHODS: In all, 38 patients with posteromedial bowing of the tibia presenting between 2000 and 2016 were identified. Mean follow-up from presentation was 78 months. A total of 17 patients underwent lengthening and deformity correction surgery, whilst three further patients are awaiting lengthening and deformity correction procedures. RESULTS: The greatest correction of deformity occurred in the first year of life, but after the age of four years, remodelling was limited. The absolute leg-length discrepancy (LLD) increased throughout growth with a mean 14.3% discrepancy in tibial length. In the lengthening group, mean length gained per episode was 45 mm (35 to 60). Mean duration in frame was 192 days, with a mean healing index of 42.4 days/cm. Significantly higher rates of recurrence in LLD were seen in those undergoing lengthening under the age of ten years (p = 0.046). Four contralateral epiphysiodeses were also performed. CONCLUSION: Posteromedial bowing of the tibia improves spontaneously during the first years of life, but in 20/38 (53%) patients, limb reconstruction was indicated for significant residual deformity and/or worsening LLD. For larger discrepancies and persistent deformity, limb reconstruction with a hexapod external fixator should be considered as part of the treatment options. LEVEL OF EVIDENCE: Level IV (Case series).

Does orthopaedic surgery improve quality of life and function in patients with mucopolysaccharidoses?

PURPOSE: Mucopolysaccharidoses (MPS) are a group of rare lysosomal storage disorders associated with involvement of multiple organs along with a generalised skeletal dysplasia. Both haematopoetic stem cell transplant and enzyme replacement therapy have improved the outlook for patients while surgery remains high-risk and there is little information on clinical or functional outcome to justify many of the surgical procedures performed. This paper aims to summarise the orthopaedic surgical procedures in MPS patients for which quality of life (QoL) and functional data are available and to describe additional QoL and functional measurement tools of relevance to the assessment of orthopaedic outcomes in MPS. METHODS: We reviewed the available literature to look for reported outcomes of orthopaedic surgery to lower and upper limbs and the spine. In addition, we describe the general and MPS-specific health measures that might be of relevance to the orthopaedic surgeon. RESULTS: There is some evidence in the literature that orthopaedic surgery may improve QoL and function in some specific aspects of the MPS condition (in relation to genu valgum, carpal tunnel syndrome and trigger digits); however, the literature is sparse and consists of level 4/5 studies only. Further studies of these conditions should include QoL and functional assessment in order to confirm or refute these reports. In other areas (spine and hip), outcomes are judged largely on radiographic appearances with little clinical correlation and short follow-up; however, one long-term study of function following hip dysplasia surgery suggests poor outcomes. Anaesthetic morbidity/mortality is not insignificant in these complex patients with multi-organ involvement. Careful assessment is required, particularly when there is neurological involvement. CONCLUSIONS: Orthopaedic surgeons involved with MPS patients should be encouraged to use and report measures of QoL and function with respect to musculoskeletal manifestations and response to surgery, recognising that such assessments in these complex and challenging patients may require a multidisciplinary approach.

The orthopaedic management of lower limb deformity in hypophosphataemic rickets.

BACKGROUND: Many patients with X-linked hypophosphataemic rickets (X-LHPR) demonstrate significant lower limb deformity despite optimal medical management. This study evaluates the use of guided growth by means of hemi-epiphysiodesis to address coronal plane deformity in the skeletally immature child. METHODS: Since 2005, 24 patients with X-LHPR have been referred to our orthopaedic unit for evaluation. All patients had standardised long leg radiographs that were analysed sequentially before and after surgery if any was performed. The rate of correction of deformity was calculated based on peri-articular angles and diaphyseal deformity angles measured at regular intervals using Traumacad software. Clinical records were reviewed to obtain relevant clinical and demographic details. Statistical analysis was performed using SPSS 23 (SPSS Inc., Chicago, IL, USA). RESULTS: The indication for surgical intervention was a mechanical axis progressing through Zone 2 or in Zone 3 despite one year of optimised medical treatment. The 15 patients underwent 16 episodes of guided growth (30 limbs, 38 segments) at a mean age of 10.3 years. In four limbs, surgery has only taken place recently; and in three limbs, correction is ongoing. Neutral mechanical axis was restored in 16/23 (70%) limbs: six improved and one limb (one segment) required osteotomy for residual deformity. The mean rate of angular correction per month was 0.3° for the proximal tibia and 0.7° for the distal femur. Patients with ≥ 3 years of growth remaining responded significantly better than older patients (p = 0.004). Guided growth was more successful in correcting valgus than varus deformity (p = 0.007). In younger patients, diaphyseal deformity corrected at a rate of 0.2° and 0.6° per month for the tibia and the femur, respectively. There has been one case of recurrent deformity. Patients with corrected coronal plane alignment did not complain of significant residual torsional malalignment. Serum phosphate and alkaline phosphatase levels did not affect response to surgery. CONCLUSIONS: Guided growth is a successful, minimally invasive method of addressing coronal plane deformity in X-LHPR. If coronal plane deformity is corrected early in patients with good metabolic control, osteotomy can be avoided.

The mucopolysaccharidoses: advances in medical care lead to challenges in orthopaedic surgical care.

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.

A protocol for the use of closed reduction in children with developmental dysplasia of the hip incorporating open psoas and adductor releases and a short-leg cast: Mid-term outcomes in 113 hips.

AIMS: Our aim was to assess the effectiveness of a protocol involving a standardised closed reduction for the treatment of children with developmental dysplasia of the hip (DDH) in maintaining reduction and to report the mid-term results. METHODS: A total of 133 hips in 120 children aged less than two years who underwent closed reduction, with a minimum follow-up of five years or until subsequent surgery, were included in the study. The protocol defines the criteria for an acceptable reduction and the indications for a concomitant soft-tissue release. All children were immobilised in a short- leg cast for three months. Arthrograms were undertaken at the time of closed reduction and six weeks later. Follow-up radiographs were taken at six months and one, two and five years later and at the latest follow-up. The Tönnis grade, acetabular index, Severin grade and signs of osteonecrosis were recorded. RESULTS: A total of 67 hips (51%) were Tönnis grade 3/4 hips. By 12 months, 20 reductions (15%) had not been maintained, and these required open reduction. In all, 55% of these were Severin 1; the others were Severin 2, due to minor acetabular dysplasia. Of the 113 successful closed reductions, 98 hips (87%) were Severin 1. Surgery for residual DDH was offered for ten hips. Osteonecrosis was seen in 32 hips (29%) but was transient in 28. In total, two children (1.5%) had severe osteonecrosis. Bilateral dislocations were significantly more likely to fail and most Tönnis 4 hips failed. CONCLUSION: Closed reduction, with concomitant adductor and psoas release when required and the use of a short leg plaster of Paris cast for three months, can produce good mid-term results in children with DDH aged less than two years. This protocol is not recommended for Tönnis 4 hips. Cite this article: Bone Joint J 2016;98-B:1548-53.

Limb length discrepancy in cutis marmorata telangiectatica congenita: an audit of assessment and management in a multidisciplinary setting.

BACKGROUND: Cutis marmorata telangiectatica congenita (CMTC) is a vascular malformation, diagnosed based on cutaneous manifestations. It is associated with limb length discrepancy (LLD) and asymmetry, but the exact extent of this and its relationship to the site of the cutaneous manifestations have not been delineated. OBJECTIVES: To review the orthopaedic problems associated with CMTC, concentrating on the assessment and management of the LLD. METHODS: This study is a retrospective analysis of orthopaedic, dermatological and vascular data that were collected prospectively at our tertiary referral centre. We identified 80 patients with an initial diagnosis of CMTC; 57/69 patients with a confirmed diagnosis had lower-limb involvement. RESULTS: An LLD was identified in 51% of cases. The discrepancy was significant (defined as ≥ 2 cm) in nine patients and was confirmed using standing leg-length radiographs. Of these patients, three had epiphysiodesis to correct the discrepancy, and surgery is planned in five others. CONCLUSIONS: Limb length discrepancy and asymmetry are common in CMTC; however, this is below the significant threshold in most cases. It is therefore recommended that any discrepancy be initially monitored clinically. This should be followed by standing leg-length radiographs at the age of 10 years (girls) or 12 years (boys), or if the LLD is ≥ 2 cm. If this is confirmed radiologically, orthopaedic referral is advised to consider surgical intervention such as epiphysiodesis.

Open reduction for developmental dysplasia of the hip: failures of screening or failures of treatment?

INTRODUCTION: The aim of this study was to define the clinical indications and demographic characteristics of patients under-going open reduction for developmental dysplasia of the hip (DDH), and determine the proportion due to preventable failures of contemporary clinical screening and early management. METHODS: Case notes were reviewed of consecutive primary open reductions performed for non-teratologic hip dislocation at the Great Ormond Street Hospital for Children over a five-year period. Forty-eight patients (64 hips) were suitable for inclusion. A telephone survey confirmed selective hip ultrasonography screening protocols were employed in all maternity hospitals in our referral base. RESULTS: There were no cases of open reduction for unilateral DDH following Pavlik treatment commenced by six weeks of age, highlighting the importance of early detection and treatment. Eleven cases (23%) may have been avoided by appropriate implementation of existing selective ultrasonography screening protocols. Thirty-four cases (71%) presented after four months of age, suggesting open reduction is associated with late diagnosis rather than failure of primary management. None of these patients had neonatal hip ultrasonography and only 12% (4 patients) had a risk factor that should have triggered a scan. CONCLUSIONS: Compared with published results, the contemporary screening practices in our referral base are failing to eliminate late presenting DDH and the need for open surgical reduction. Changes in strategy and implementation are required to significantly improve screening efficacy.

Guided growth: recent advances in a deep-rooted concept.

Guiding growth by harnessing the ability of growing bone to undergo plastic deformation is one of the oldest orthopaedic principles. Correction of deformity remains a major part of the workload for paediatric orthopaedic surgeons and recently, along with developments in limb reconstruction and computer-directed frame correction, there has been renewed interest in surgical methods of physeal manipulation or 'guided growth'. Manipulating natural bone growth to correct a deformity is appealing, as it allows gradual correction by non- or minimally invasive methods. This paper reviews the techniques employed for guided growth in current orthopaedic practice, including the basic science and recent advances underlying mechanical physeal manipulation of both healthy and pathological physes.

Delaying treatment of supracondylar fractures in children: has the pendulum swung too far?

The aim of this retrospective multicentre study was to report the continued occurrence of compartment syndrome secondary to paediatric supracondylar humeral fractures in the period 1995 to 2005. The inclusion criteria were children with a closed, low-energy supracondylar fracture with no associated fractures or vascular compromise, who subsequently developed compartment syndrome. There were 11 patients (seven girls and four boys) identified from eight hospitals in three countries. Ten patients with severe elbow swelling documented at presentation had a mean delay before surgery of 22 hours (6 to 64). One patient without severe swelling documented at presentation suffered arterial entrapment following reduction, with a subsequent compartment syndrome requiring fasciotomy 25 hours after the index procedure. This series is noteworthy, as all patients had low-energy injuries and presented with an intact radial pulse. Significant swelling at presentation and delay in fracture reduction may be important warning signs for the development of a compartment syndrome in children with supracondylar fractures of the humerus.

Early results of a physiotherapist-delivered Ponseti service for the management of idiopathic congenital talipes equinovarus foot deformity.

We studied 24 children (40 feet) to demonstrate that a physiotherapist-delivered Ponseti service is as successful as a medically-led programme in obtaining correction of an idiopathic congenital talipes equinovarus deformity. The median Pirani score at the start of treatment was 5.5 (mean 4.75; 2 to 6). A Pirani score of > or =5 predicted the need for tenotomy (p < 0.01). Of the 40 feet studied, 39 (97.5%) achieved correction of deformity. The remaining foot required surgical correction. A total of 25 (62.5%) of the feet underwent an Achilles tenotomy, which was performed by a surgeon in the physiotherapy clinic. There was full compliance with the foot abduction orthoses in 36 (90%) feet. Continuity of care was assured, as one practitioner was responsible for all patient contact. This was rated highly by the patient satisfaction survey. We believe that the Ponseti technique is suitable for use by non-medical personnel, but a holistic approach and good continuity of care are essential to the success of the programme.

Osteofibrous dysplasia of the tibia. Is there a need for a radical surgical approach?

Osteofibrous dysplasia is an unusual developmental condition of childhood, which almost exclusively affects the tibia. It is thought to follow a slowly progressive course and to stabilise after skeletal maturity. The possible link with adamantinoma is controversial and some authors believe that they are part of one histological process. We retrospectively reviewed 16 patients who were diagnosed as having osteofibrous dysplasia initially or on the final histological examination. Their management was diverse, depending on the severity of symptoms and the extent of the lesion. Definitive (extraperiosteal) surgery was localised "shark-bite" excision for small lesions in five patients. Extensive lesions were treated by segmental excision and fibular autograft in six patients, external fixation and bone transport in four and proximal tibial replacement in one. One patient who had a fibular autograft required further excision and bone transport for recurrence. Six initially underwent curettage and all had recurrence. There were no recurrences after localised extraperiosteal excision or bone transport. There were three confirmed cases of adamantinoma. The relevant literature is reviewed. We recommend extraperiosteal excision in all cases of osteofibrous dysplasia, with segmental excision and reconstruction in more extensive lesions.

Clinical outcome of nerve injuries associated with supracondylar fractures of the humerus in children: the experience of a specialist referral centre.

Between 1998 and 2002, 37 neuropathies in 32 patients with a displaced supracondylar fracture of the humerus who were referred to a nerve injury unit were identified. There were 19 boys and 13 girls with a mean age of 7.9 years (3.6 to 11.3). A retrospective review of these injuries was performed. The ulnar nerve was injured in 19, the median nerve in ten and the radial nerve in eight cases. Fourteen neuropathies were noted at the initial presentation and 23 were diagnosed after treatment of the fracture. After referral, exploration of the nerve was planned for 13 patients. Surgery was later cancelled in three because of clinical recovery. Six patients underwent neurolysis alone. Excision of neuroma and nerve grafting were performed in four. At follow-up, 26 patients had an excellent, five a good and one a fair outcome.

Pediatric fractures and dislocations of the hip and pelvis.

Recognition of an unstable pelvic fracture or a significant hip injury in children is important. Clinical assessment plays a valuable role as does the judicious use of imaging modalities in determining the most effective form of treatment, but the routine use of the standard AP pelvic radiograph is questioned. The concept of age and skeletal maturity has been re-evaluated, allowing the appropriate identification of cases that would benefit from an aggressive operative approach. A dual-tier approach to the treatment of pediatric pelvic trauma is suggested with an appreciation that there is no substantial evidence base for the surgical treatment of most injuries. Displaced femoral neck fractures and injuries to the hip joint that damage the articular or physeal cartilages require careful assessment and prompt and careful reduction and stabilization.