Recent reports have suggested an increase in the number of anterior cruciate ligament (ACL) injuries in children, although their true incidence is unknown. The prognosis of the ACL-deficient knee in young active individuals is poor because of secondary meniscal tears, persistent instability and early-onset osteoarthritis. The aim of surgical reconstruction is to provide stability while avoiding physeal injury. Techniques of reconstruction include transphyseal, extraphyseal or partial physeal sparing procedures. In this paper we review the management of ACL tears in skeletally immature patients. . Cite this article: Bone Joint J 2013;95-B:1562–9

Congenital pseudarthrosis of the tibia (CPT) is a rare but well recognised condition. Obtaining union of the pseudarthrosis in these children is often difficult and may require several surgical procedures. The treatment has changed significantly since the review by Hardinge in 1972, but controversies continue as to the best form of surgical treatment. This paper reviews these controversies.

Cite this article: Bone Joint J 2013;95-B:1027–34.

Aims. Management of displaced paediatric supracondylar elbow fractures remains widely debated and actual practice is unclear. This national trainee collaboration aimed to evaluate surgical and postoperative management of these injuries across the UK. Methods. This study was led by the South West Orthopaedic Research Division (SWORD) and performed by the Supra Man Collaborative. Displaced paediatric supracondylar elbow fractures undergoing surgery between 1 January 2019 and 31 December 2019 were retrospectively identified and their anonymized data were collected via Research Electronic Data Capture (REDCap). Results. A total of 972 patients were identified across 41 hospitals. Mean age at injury was 6.3 years (1 to 15), 504 were male (52%), 583 involved the left side (60%), and 538 were Gartland type 3 fractures (55%). Median time from injury to theatre was 16 hours (interquartile range (IQR) 6.6 to 22), 300 patients (31%) underwent surgery on the day of injury, and 91 (9%) underwent surgery between 10:00 pm and 8:00 am. Overall, 910 patients (94%) had Kirschner (K)-wire) fixation and these were left percutaneous in 869 (95%), while 62 patients (6%) had manipulation under anaesthetic (MUA) and casting. Crossed K-wire configuration was used as fixation in 544 cases (59.5%). Overall, 208 of the fixation cases (61%) performed or supervised by a paediatric orthopaedic consultant underwent lateral-only fixation, whereas 153 (27%) of the fixation cases performed or supervised by a non-paediatric orthopaedic consultant used lateral-only fixation. In total, 129 percutaneous wires (16%) were removed in theatre. Of the 341 percutaneous wire fixations performed or supervised by a paediatric orthopaedic consultant, 11 (3%) underwent wire removal in theatre, whereas 118 (22%) of the 528 percutaneous wire fixation cases performed or supervised by a non-paediatric orthopaedic consultant underwent wire removal in theatre. Four MUA patients (6%) and seven K-wire fixation patients (0.8%) required revision surgery within 30 days for displacement. Conclusion. The treatment of supracondylar elbow fractures in children varies across the UK. Patient cases where a paediatric orthopaedic consultant was involved had an increased tendency for lateral only K-wire fixation and for wire removal in clinic. Low rates of displacement requiring revision surgery were identified in all fixation configurations. Cite this article: Bone Joint J 2023;105-B(1):82–87

Aims. A national screening programme has existed in the UK for the diagnosis of developmental dysplasia of the hip (DDH) since 1969. However, every aspect of screening and treatment remains controversial. Screening programmes throughout the world vary enormously, and in the UK there is significant variation in screening practice and treatment pathways. We report the results of an attempt by the British Society for Children’s Orthopaedic Surgery (BSCOS) to identify a nationwide consensus for the management of DDH in order to unify treatment and suggest an approach for screening. Methods. A Delphi consensus study was performed among the membership of BSCOS. Statements were generated by a steering group regarding aspects of the management of DDH in children aged under three months, namely screening and surveillance (15 questions), the technique of ultrasound scanning (eight questions), the initiation of treatment (19 questions), care during treatment with a splint (ten questions), and on quality, governance, and research (eight questions). A two-round Delphi process was used and a consensus document was produced at the final meeting of the steering group. Results. A total of 60 statements were graded by 128 clinicians in the first round and 132 in the second round. Consensus was reached on 30 out of 60 statements in the first round and an additional 12 in the seond. This was summarized in a consensus statement and distilled into a flowchart to guide clinical practice. Conclusion. We identified agreement in an area of medicine that has a long history of controversy and varied practice. None of the areas of consensus are based on high-quality evidence. This document is thus a framework to guide clinical practice and on which high-quality clinical trials can be developed. Cite this article: Bone Joint J 2023;105-B(2):209–214

Aims. The aim of this study was to gain a consensus for best practice of the assessment and management of children with idiopathic toe walking (ITW) in order to provide a benchmark for practitioners and guide the best consistent care. Methods. An established Delphi approach with predetermined steps and degree of agreement based on a standardized protocol was used to determine consensus. The steering group members and Delphi survey participants included members from the British Society of Children’s Orthopaedic Surgery (BSCOS) and the Association of Paediatric Chartered Physiotherapists (APCP). The statements included definition, assessment, treatment indications, nonoperative and operative interventions, and outcomes. Descriptive statistics were used for analysis of the Delphi survey results. The AGREE checklist was followed for reporting the results. Results. A total of 227 participants (54% APCP and 46% BSCOS members) completed the first round, and 222 participants (98%) completed the second round. Out of 54 proposed statements included in the first round Delphi, 17 reached ‘consensus in’, no statements reached ‘consensus out’, and 37 reached ‘no consensus’. These 37 statements were then discussed, reworded, amalgamated, or deleted before the second round Delphi of 29 statements. A total of 12 statements reached ‘consensus in’, four ‘consensus out’, and 13 ‘no consensus’. In the final consensus meeting, 13 statements were voted upon. Five were accepted, resulting in a total of 31 approved statements. Conclusion. In the aspects of practice where sufficient evidence is not available, a consensus statement can provide a strong body of opinion that acts as a benchmark for excellence in clinical care. This statement can assist clinicians managing children with ITW to ensure consistent and reliable practice, and reduce geographical variability in practice and outcomes. It will enable those treating ITW to share the published consensus document with both carers and patient groups. Cite this article: Bone Joint J 2024;106-B(10):1190–1196

Aims. The aim of this study was to determine the consensus best practice approach for the investigation and management of children (aged 0 to 15 years) in the UK with musculoskeletal infection (including septic arthritis, osteomyelitis, pyomyositis, tenosynovitis, fasciitis, and discitis). This consensus can then be used to ensure consistent, safe care for children in UK hospitals and those elsewhere with similar healthcare systems. Methods. A Delphi approach was used to determine consensus in three core aspects of care: 1) assessment, investigation, and diagnosis; 2) treatment; and 3) service, pathways, and networks. A steering group of paediatric orthopaedic surgeons created statements which were then evaluated through a two-round Delphi survey sent to all members of the British Society for Children’s Orthopaedic Surgery (BSCOS). Statements were only included (‘consensus in’) in the final agreed consensus if at least 75% of respondents scored the statement as critical for inclusion. Statements were discarded (‘consensus out’) if at least 75% of respondents scored them as not important for inclusion. Reporting these results followed the Appraisal Guidelines for Research and Evaluation. Results. A total of 133 children’s orthopaedic surgeons completed the first survey, and 109 the second. Out of 43 proposed statements included in the initial Delphi, 32 reached ‘consensus in’, 0 ‘consensus out’, and 11 ‘no consensus’. These 11 statements were then reworded, amalgamated, or deleted before the second Delphi round of eight statements. All eight were accepted as ‘consensus in’, resulting in a total of 40 approved statements. Conclusion. In the many aspects of medicine where relevant evidence is not available for clinicians to base their practice, a Delphi consensus can provide a strong body of opinion that acts as a benchmark for good quality clinical care. We would recommend clinicians managing children with musculoskeletal infection follow the guidance in the consensus statements in this article, to ensure care in all medical settings is consistent and safe. Cite this article: Bone Joint J 2023;105-B(7):815–820

Aims. Open tibial fractures are limb-threatening injuries. While limb loss is rare in children, deep infection and nonunion rates of up to 15% and 8% are reported, respectively. We manage these injuries in a similar manner to those in adults, with a combined orthoplastic approach, often involving the use of vascularised free flaps. We report the orthopaedic and plastic surgical outcomes of a consecutive series of patients over a five-year period, which includes the largest cohort of free flaps for trauma in children to date. Methods. Data were extracted from medical records and databases for patients with an open tibial fracture aged < 16 years who presented between 1 May 2014 and 30 April 2019. Patients who were transferred from elsewhere were excluded, yielding 44 open fractures in 43 patients, with a minimum follow-up of one year. Management was reviewed from the time of injury to discharge. Primary outcome measures were the rate of deep infection, time to union, and the Modified Enneking score. Results. The mean age of the patients was 9.9 years (2.8 to 15.8), and 28 were male (64%). A total of 30 fractures (68%) involved a motor vehicle collision, and 34 (77%) were classified as Gustilo Anderson (GA) grade 3B. There were 17 (50%) GA grade 3B fractures, which were treated with a definitive hexapod fixator, and 33 fractures (75%) were treated with a free flap, of which 30 (91%) were scapular/parascapular or anterolateral thigh (ALT) flaps. All fractures united at a median of 12.3 weeks (interquartile range (IQR) 9.6 to 18.1), with increasing age being significantly associated with a longer time to union (p = 0.005). There were no deep infections, one superficial wound infection, and the use of 20 fixators (20%) was associated with a pin site infection. The median Enneking score was 90% (IQR 87.5% to 95%). Three patients had a bony complication requiring further surgery. There were no flap failures, and eight patients underwent further plastic surgery. Conclusion. The timely and comprehensive orthoplastic care of open tibial fractures in this series of patiemts aged < 16 years resulted in 100% union and 0% deep infection, with excellent patient-reported functional outcomes. Cite this article: Bone Joint J 2021;103-B(6):1160–1167

Aims. This study aims to define a set of core outcomes (COS) to allow consistent reporting in order to compare results and assist in treatment decisions for idiopathic clubfoot. Methods. A list of outcomes will be obtained in a three-stage process from the literature and from key stakeholders (patients, parents, surgeons, and healthcare professionals). Important outcomes for patients and parents will be collected from a group of children with idiopathic clubfoot and their parents through questionnaires and interviews. The outcomes identified during this process will be combined with the list of outcomes previously obtained from a systematic review, with each outcome assigned to one of the five core areas defined by the Outcome Measures Recommended for use in Randomized Clinical Trials (OMERACT). This stage will be followed by a two round Delphi survey aimed at key stakeholders in the management of idiopathic clubfoot. The final outcomes list obtained will then be discussed in a consensus meeting of representative key stakeholders. Conclusion. The inconsistency in outcomes reporting in studies investigating idiopathic clubfoot has made it difficult to define the success rate of treatments and to compare findings between studies. The development of a COS seeks to define a minimum standard set of outcomes to collect in all future clinical trials for this condition, to facilitate comparisons between studies and to aid decisions in treatment. Cite this article: Bone Jt Open 2021;2(4):255–260

Aims

Children with spinal dysraphism can develop various musculoskeletal deformities, necessitating a range of orthopaedic interventions, causing significant morbidity, and making considerable demands on resources. This systematic review aimed to identify what outcome measures have been reported in the literature for children with spinal dysraphism who undergo orthopaedic interventions involving the lower limbs.

Aims

The aim of this study is to evaluate the surgical treatment with the best healing rate for patients with proximal femoral unicameral bone cysts (UBCs) after initial surgery, and to determine which procedure has the lowest adverse event burden during follow-up.

Aims. Osteonecrosis (ON) can cause considerable morbidity in young people who undergo treatment for acute lymphoblastic leukaemia (ALL). The aims of this study were to determine the operations undertaken for ON in this population in the UK, along with the timing of these operations and any sequential procedures that are used in different joints. We also explored the outcomes of those patients treated by core decompression (CD), and compared this with conservative management, in both the pre- or post-collapse stages of ON. Methods. UK treatment centres were contacted to obtain details regarding surgical interventions and long-term outcomes for patients who were treated for ALL and who developed ON in UKALL 2003 (the national leukaemia study which recruited patients aged 1 to 24 years at diagnosis of ALL between 2003 and 2011). Imaging of patients with ON affecting the femoral head was requested and was used to score all lesions, with subsequent imaging used to determine the final grade. Kaplan-Meier failure time plots were used to compare the use of CD with non surgical management. Results. Detailed information was received for 85 patients who had developed ON during the course of their ALL treatment. A total of 206 joints were affected by ON. Of all joints affected by ON, 21% required arthroplasty, and 43% of all hips affected went on to be replaced. CD was performed in 30% of hips affected by ON. The majority of the hips were grade 4 or 5 at initial diagnosis of ON. There was no significant difference in time to joint collapse between those joints in which CD was performed, compared with no joint-preserving surgical intervention. Conclusion. There is a high incidence of surgery in young people who have received treatment for ALL and who have developed ON. Our results suggest that CD of the femoral head in this group of patients does not delay or improve the rates of femoral head survival. Cite this article: Bone Joint J 2021;103-B(3):589–596

Aims. The aim of this study was to compare the surgical and quality-of-life outcomes of children with skeletal dysplasia to those in children with idiopathic early-onset scoliosis (EOS) undergoing growth-friendly management. Patients and Methods. A retrospective review of two prospective multicentre EOS databases identified 33 children with skeletal dysplasia and EOS (major curve ≥ 30°) who were treated with growth-friendly instrumentation at younger than ten years of age, had a minimum two years of postoperative follow-up, and had undergone three or more lengthening procedures. From the same registries, 33 matched controls with idiopathic EOS were identified. A total of 20 children in both groups were treated with growing rods and 13 children were treated with vertical expandable prosthetic titanium rib (VEPTR) instrumentation. Results. Mean preoperative major curves were 76° (34° to 115°) in the skeletal dysplasia group and 75° (51° to 113°) in the idiopathic group (p = 0.55), which were corrected at final follow-up to 49° (13° to 113°) and 46° (12° to 112°; p = 0.68), respectively. T1-S1 height increased by a mean of 36 mm (0 to 105) in the skeletal dysplasia group and 38 mm (7 to 104) in the idiopathic group at the index surgery (p = 0.40), and by 21 mm (1 to 68) and 46 mm (7 to 157), respectively, during the distraction period (p = 0.0085). The skeletal dysplasia group had significantly worse scores in the physical function, daily living, financial impact, and parent satisfaction preoperatively, as well as on financial impact and child satisfaction at final follow-up, than the idiopathic group (all p < 0.05). The domains of the 24-Item Early-Onset Scoliosis Questionnaire (EOSQ24) remained at the same level from preoperative to final follow-up in the skeletal dysplasia group (all p > 0.10). Conclusion. Children with skeletal dysplasia gained significantly less spinal growth during growth-friendly management of their EOS and their health-related quality of life was significantly lower both preoperatively and at final follow-up than in children with idiopathic EOS. Cite this article: Bone Joint J 2019;101-B:1563–1569

Aims. Following the introduction of national standards in 2009, most major paediatric trauma is now triaged to specialist units offering combined orthopaedic and plastic surgical expertise. We investigated the management of open tibia fractures at a paediatric trauma centre, primarily reporting the risk of infection and rate of union. Patients and Methods. A retrospective review was performed on 61 children who between 2007 and 2015 presented with an open tibia fracture. Their mean age was nine years (2 to 16) and the median follow-up was ten months (interquartile range 5 to 18). Management involved IV antibiotics, early debridement and combined treatment of the skeletal and soft-tissue injuries in line with standards proposed by the British Orthopaedic Association. Results. There were 36 diaphyseal fractures and 25 distal tibial fractures. Of the distal fractures, eight involved the physis. Motor vehicle collisions accounted for two thirds of the injuries and 38 patients (62%) arrived outside of normal working hours. The initial method of stabilisation comprised: casting in nine cases (15%); elastic nailing in 19 (31%); Kirschner (K)-wiring in 13 (21%); intramedullary nailing in one (2%); open reduction and plate fixation in four (7%); and external fixation in 15 (25%). Wound management comprised: primary wound closure in 24 (39%), delayed primary closure in 11 (18%), split skin graft (SSG) in eight (13%), local flap with SSG in 17 (28%) and a free flap in one. A total of 43 fractures (70%) were Gustilo-Anderson grade III. There were four superficial (6.6%) and three (4.9%) deep infections. Two deep infections occurred following open reduction and plate fixation and the third after K-wire fixation of a distal fracture. No patient who underwent primary wound closure developed an infection. All the fractures united, although nine patients required revision of a mono-lateral to circular frame for delayed union (two) or for altered alignment or length (seven). The mean time to union was two weeks longer in diaphyseal fractures than in distal fractures (13 weeks versus 10.8 weeks, p = 0.016). Children aged > 12 years had a significantly longer time to union than those aged < 12 years (16.3 weeks versus 11.4 weeks, p = 0.045). The length of stay in hospital for patients with a Gustilo-Anderson grade IIIB fracture was twice as long as for less severe injuries. . Conclusion. Fractures in children heal better than those in adults. Based on our experience of deep infection we discourage the use of internal fixation with a plate for open tibial fractures in children. We advocate aggressive initial wound debridement in theatre with early definitive combined orthopaedic and plastic surgery in order to obtain skeletal stabilisation and soft-tissue cover. Cite this article: Bone Joint J 2017;99-B:544–53

The management of children’s fractures has evolved as a result of better health education, changes in lifestyle, improved implant technology and the changing expectations of society. This review focuses on the changes seen in paediatric fractures, including epidemiology, the increasing problems of obesity, the mechanisms of injury, non-accidental injuries and litigation. We also examine the changes in the management of fractures at three specific sites: the supracondylar humerus, femoral shaft and forearm. There has been an increasing trend towards surgical stabilisation of these fractures. The reasons for this are multifactorial, including societal expectations of a perfect result and reduced hospital stay. Reduced hospital stay is beneficial to the social, educational and psychological needs of the child and beneficial to society as a whole, due to reduced costs. Cite this article: Bone Joint J 2015; 97-B:442–8

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 aim of our survey was to study the current practice to manage DDH in UK by the members of the British Society for Children's Orthopaedic Surgery. An online questionnaire link to ask about the management of DDH was emailed to 204 members of the British Society for Children's Orthopaedic Surgery. The response rate was 39%. 73% respondents have a local screening programme, 19% screen only high risk children and 8% had no screening programme. Pavlik harness was used by 87% respondents for Graf Type 2, 96% for Graf type 3 and 90% for Graf type 4. 14% respondents will only observe for Graf Type 2. 36% respondents will follow up children every week, 45% every 2 weeks, 3% every 3 weeks, 9% every 4 weeks, 4% every 6 weeks and 3% will decide the follow up according to severity of DDH and treatment.1.3% respondents will follow up these patients for 6 months, 13% for 12 months, 10.5% each for 24 months, 36 months, 48 months and 50% until skeletal maturity. After the failure of initial splintage, 7% respondents will consider surgery immediately, 13.5% at 3 months, 36.5% at 6 months, 4% at 9 months, 28% at 12 months, 5.4% according to HIP-OP Trial and 5.6% according to the situation. There was no consensus about the treatment of DDH. 73% respondents have a local screening programme. The most common splintage method used was Pavlik harness. 45% respondents will follow up children every 2 weeks following the start of treatment. 50% respondents will follow up these patients until skeletal maturity. 36% respondents will consider surgery at 6 months following the failure of splintage. This survey highlights the fact that the management of DDH is an art based on the scientific evidence, parent's choice and personal expertise

This study aims to evaluate the development of deformity in patients with hypophosphataemic rickets and the evolution of the orthopaedic management thereof. Fifty-four patients had undergone treatment for hypophosphataemic rickets at our institution since 1995. Clinical records for all patients were obtained. Forty-one patients had long leg radiographs available that were analysed using Traumacad™ software. Statistical analysis was performed using SPSS 23 (SPSS Inc., Chicago, Illinois, USA). Of the 41 patients, 18 (43%) had no radiographic deformity. 20 have undergone bilateral lower limb surgery for persistent deformity (Mechanical Axis ≥ Zone 2). A further 3 patients are awaiting surgery. Six patients (12 limbs, 14 segments) had osteotomies and internal fixation as primary intervention: only one limb developed recurrent deformity. There were no major complications. Fourteen patients (28 limbs) had 8-plates (Orthofix, Verona) applied. In 5 limbs correction is on-going. Neutral alignment (central Zone 1) was achieved in 14/20 (70%) patients. Two patients required osteotomy and external fixation for resistant deformity. The mean rate of angular correction following 8-plate application was 0.3 and 0.7 degrees/month for the tibia and femur respectively. The mean age at 8-plate insertion was 10.25y (5–15y). Patients with more than 3 years of growth remaining responded significantly better than older patients (Fisher Exact Test, p=0.024). Guided growth was more successful in correcting valgus deformity than varus deformity (Fisher Exact Test, p=0.04). In the younger patients, diaphyseal deformity corrected as the mechanical axis improved at the rate of 0.2 and 0.7 degrees /month for the tibial and femoral shafts. Serum phosphate and alkaline phosphatase levels did not affect response to surgery or complication rate. Guided growth by means of 8-plates is a successful in addressing deformity in hypophosphataemic rickets. Surgery is best performed in patients with more than 3 years of growth remaining

The aim was to assess contemporary management of slipped capital femoral epiphysis (SCFE) by surveying members of the British Society of Children's Orthopaedic Surgery (BSCOS). A questionnaire with 5 case vignettes was used. Two questions examined the timing of surgery for an acute unstable SCFE in a child presenting at 6 hours and at 48 hours after start of symptoms. Two further questions explored the preferred method of fixation in mild and severe stable SCFE. The final question examined the management of the contralateral normal hip. Responses were entered into an Excel spreadsheet and the data w analysed using a chi-squared test. The response rate was 56% (110/196). 88.2% (97/110) responded that if a child presented with an acute unstable SCFE within 6 hours, they would treat it within 24 hours of presentation, compared with 40.9% (45/110) for one presenting 48 hours after the onset of symptoms (P<0.0001). 52.6% (58/110) of surveyed BSCOS members would offer surgery for an unstable SCFE between 1 and 7 days after onset of symptoms. Single screw fixation in situ was advocated by 96.4% (106/110) and 70.9% (78/110) while corrective osteotomy was preferred by 1.8% (2/110) and 26.4% (29/110) of respondents for the mild and the severe stable slips respectively (P<0.0001). Surgeons preferring osteotomy are more likely to perform an intracapsular technique. Prophylactic fixation of the contralateral normal hip was performed by 27.3% (30/110) of participants. There are significant differences in opinions between BSCOS members as to the optimal management of SCFE in children. This reflects the variable recommendations and quality in the current scientific literature. Further research is therefore required to determine best practice and enable consensus to be reached

Purpose. This study compares outcomes in patients with complete congenital fibula absence, associated with severe lower limb deformity, treated with an amputation protocol to those using an extension prosthesis. Method. 32 patients were identified. 9 patients (2M: 7F, median age at presentation of 22 yrs) utilized an extension prosthesis. 23 patients (16M: 7F, median age at presentation of 10 months) underwent 25 amputations during childhood: only two underwent tibial kyphus correction to facilitate prosthetic wear. Mobility was assessed using the SIGAM and K scores. Quality of life was assessed using the PedsQL inventory questionnaire; pain by a verbal severity score. Patients undergoing amputation were further subdivided by age, below and above 2 yrs at the time of surgery. Results. 19 Syme and one Boyd amputation in 19 patients were performed early (mean age 15 months). 4 Syme and one trans-tibial amputation in 4 patients took place in older children (mean age 6.6 years). K Scores were significantly higher (mean 4 versus 2) and pain scores lower in the amputation group allowing high impact activity compared to community ambulation with an extension prosthesis. The SIGAM and PedsQL scores were all better in the amputation group, but not significantly so. There was no significant difference in the scores based on the time of amputation. Conclusion. Complete fibula absence can present with significant lower limb deformity. Parental counselling regarding management is paramount in achieving the optimum functional outcome. Childhood amputation for severe limb length inequality and foot deformity in congenital fibula absence offers excellent short term functional outcome with prosthetic support. The tibial kyphus deformity does not need routine correction and facilitates prosthetic suspension. Accommodative extension prosthesis does offer reasonable long term function but outcome scores are lower

Aim. To assess the efficacy of combined medical and surgical management in obtaining normal lower limb mechanical alignment in a patient cohort with genotypically similar hypophosphataemic rickets. Methods. A notes and radiograph audit was performed of all patients attending our institution with hypophosphataemic rickets: a subset with PHEX gene anomalies was studied further. Lower limb radiographs were assessed at two points during childhood and note made of treatment start, compliance; indication, timing and result of surgery. Standing leg alignment radiographs were measured at skeletal maturity or at latest review. Results. 35 patients (16 females, 18 skeletally mature) were identified. 10 commenced treatment at <12m. 11 patients (5 female) underwent 24 surgical procedures (13 for varus deformities). Surgery was bilateral in 10/11 patients. 5/14 osteotomies were performed after skeletal maturity. Malalignment was common: with NSA (neck-shaft angle) abnormalities in 20%, abnormal angles at distal femur and proximal tibia in 58% and 60% respectively and ankle abnormalities in 24%, prior to surgery. Surgical management led to normal mechanical alignment at skeletal maturity. At latest review, no patient had a leg length difference. 40% of non-operated, skeletally immature limbs have significant malalignment despite medical therapy. There was no statistical relationship between treatment onset and need for surgical correction. The relationship between non-compliance with medical treatment and surgical intervention was confounded by the changing emphasis on preferred method of surgical correction from osteotomy to guided growth. Conclusion. Even when medical treatment is commenced promptly and adhered to, significant lower limb malalignment can occur requiring surgical correction. Guided growth principles allow early deformity correction. Significance. Lower limb malalignment should not be considered a failure of medical treatment but more a consequence of the disease process. Earlier surgical intervention may encourage a more normal pattern of growth