Advertisement for orthosearch.org.uk
Results 1 - 20 of 75
Results per page:
The Bone & Joint Journal
Vol. 105-B, Issue 3 | Pages 331 - 340
1 Mar 2023
Vogt B Toporowski G Gosheger G Laufer A Frommer A Kleine-Koenig M Roedl R Antfang C

Aims. Temporary hemiepiphysiodesis (HED) is applied to children and adolescents to correct angular deformities (ADs) in long bones through guided growth. Traditional Blount staples or two-hole plates are mainly used for this indication. Despite precise surgical techniques and attentive postoperative follow-up, implant-associated complications are frequently described. To address these pitfalls, a flexible staple was developed to combine the advantages of the established implants. This study provides the first results of guided growth using the new implant and compares these with the established two-hole plates and Blount staples. Methods. Between January 2013 and December 2016, 138 patients (22 children, 116 adolescents) with genu valgum or genu varum were treated with 285 flexible staples. The minimum follow-up was 24 months. These results were compared with 98 patients treated with 205 two-hole plates and 92 patients treated with 535 Blount staples. In long-standing anteroposterior radiographs, mechanical axis deviations (MADs) were measured before and during treatment to analyze treatment efficiency. The evaluation of the new flexible staple was performed according to the idea, development, evaluation, assessment, long-term (IDEAL) study framework (Stage 2a). Results. Overall, 79% (109/138) of patients treated with flexible staples achieved sufficient deformity correction. The median treatment duration was 16 months (interquartile range (IQR) 8 to 21). The flexible staples achieved a median MAD correction of 1.2 mm/month/HED site (IQR 0.6 to 2.0) in valgus deformities and 0.6 mm/month/HED site (IQR 0.2 to 1.5) in varus deformities. Wound infections occurred in 1%, haematomas and joint effusions in 4%, and implant-associated complications in 1% of patients treated with flexible staples. Valgus AD were corrected faster using flexible staples than two-hole plates and Blount staples. Furthermore, the median MAD after treatment was lower in varus and valgus AD, fewer implant-associated complications were detected, and reduced implantation times were recorded using flexible staples. Conclusion. The flexible staple seems to be a viable option for guided growth, showing comparable or possibly better results regarding correction speed and reducing implant-associated complications. Further comparative studies are required to substantiate these findings. Cite this article: Bone Joint J 2023;105-B(3):331–340


Bone & Joint Open
Vol. 3, Issue 2 | Pages 158 - 164
17 Feb 2022
Buddhdev P Vallim F Slattery D Balakumar J

Aims. Slipped upper femoral epiphysis (SUFE) has well documented biochemical and mechanical risk factors. Femoral and acetabular morphologies seem to be equally important. Acetabular retroversion has a low prevalence in asymptomatic adults. Hips with dysplasia, osteoarthritis, and Perthes’ disease, however, have higher rates, ranging from 18% to 48%. The aim of our study was to assess the prevalence of acetabular retroversion in patients presenting with SUFE using both validated radiological signs and tomographical measurements. Methods. A retrospective review of all SUFE surgical cases presenting to the Royal Children’s Hospital, Melbourne, Australia, from 2012 to 2019 were evaluated. Preoperative plain radiographs were assessed for slip angle, validated radiological signs of retroversion, and standardized postoperative CT scans were used to assess cranial and mid-acetabular version. Results. In all, 116 SUFEs presented in 107 patients who underwent surgical intervention; 47 (52%) were male, with a mean age of 12.7 years (7.5 to 16.6). Complete radiological data was available for 91 patients (99 hips) with adequate axial CT imaging of both hips. Overall, 82 patients (82%) underwent pinning in situ (PIS), with subcapital realignment surgery (SRS) performed in 17 patients (18%) (slip angles > 75°). Contralateral prophylactic PIS was performed in 72 patients (87%). On the slip side, 62 patients (68%) had one or more radiological sign of retroversion. Tomographical acetabular retroversion was more pronounced cranially than caudally of the acetabulum on both the affected side and the contralateral side (p < 0.001) as expected in the normal population. Increasing severity of the slip was found to be directly proportional to the degree of reduction in cranial and central acetabular version (p < 0.05) in the SUFE hips. Conclusion. Acetabular retroversion is more prevalent in patients with SUFE than previously reported, and have been shown be correlated to the severity of the slip presentation. The presence of radiological signs of acetabular retroversion could be used to justify prophylactic contralateral pinning. Cite this article: Bone Jt Open 2022;3(2):158–164


The Bone & Joint Journal
Vol. 106-B, Issue 7 | Pages 751 - 758
1 Jul 2024
Yaxier N Zhang Y Song J Ning B

Aims

Given the possible radiation damage and inaccuracy of radiological investigations, particularly in children, ultrasound and superb microvascular imaging (SMI) may offer alternative methods of evaluating new bone formation when limb lengthening is undertaken in paediatric patients. The aim of this study was to assess the use of ultrasound combined with SMI in monitoring new bone formation during limb lengthening in children.

Methods

In this retrospective cohort study, ultrasound and radiograph examinations were performed every two weeks in 30 paediatric patients undergoing limb lengthening. Ultrasound was used to monitor new bone formation. The number of vertical vessels and the blood flow resistance index were compared with those from plain radiographs.


Bone & Joint Open
Vol. 5, Issue 7 | Pages 581 - 591
12 Jul 2024
Wang W Xiong Z Huang D Li Y Huang Y Guo Y Andreacchio A Canavese F Chen S

Aims

To investigate the risk factors for unsuccessful radial head reduction (RHR) in children with chronic Monteggia fractures (CMFs) treated surgically.

Methods

A total of 209 children (mean age 6.84 years (SD 2.87)), who underwent surgical treatment for CMFs between March 2015 and March 2023 at six institutions, were retrospectively reviewed. Assessed risk factors included age, sex, laterality, dislocation direction and distance, preoperative proximal radial metaphysis width, time from injury to surgery, reduction method, annular ligament reconstruction, radiocapitellar joint fixation, ulnar osteotomy, site of ulnar osteotomy, preoperative and postoperative ulnar angulation, ulnar fixation method, progressive ulnar distraction, and postoperative cast immobilization. Independent-samples t-test, chi-squared test, and logistic regression analysis were used to identify the risk factors associated with unsuccessful RHR.


The Bone & Joint Journal
Vol. 104-B, Issue 2 | Pages 302 - 308
1 Feb 2022
Dala-Ali B Donnan L Masterton G Briggs L Kauiers C O’Sullivan M Calder P Eastwood DM

Aims

Osteofibrous dysplasia (OFD) is a rare benign lesion predominantly affecting the tibia in children. Its potential link to adamantinoma has influenced management. This international case series reviews the presentation of OFD and management approaches to improve our understanding of OFD.

Methods

A retrospective review at three paediatric tertiary centres identified 101 cases of tibial OFD in 99 patients. The clinical records, radiological images, and histology were analyzed.


The Bone & Joint Journal
Vol. 103-B, Issue 8 | Pages 1428 - 1437
2 Aug 2021
Vogt B Roedl R Gosheger G Frommer A Laufer A Kleine-Koenig M Theil C Toporowski G

Aims

Temporary epiphysiodesis (ED) is commonly applied in children and adolescents to treat leg length discrepancies (LLDs) and tall stature. Traditional Blount staples or modern two-hole plates are used in clinical practice. However, they require accurate planning, precise surgical techniques, and attentive follow-up to achieve the desired outcome without complications. This study reports the results of ED using a novel rigid staple (RigidTack) incorporating safety, as well as technical and procedural success according to the idea, development, evaluation, assessment, long-term (IDEAL) study framework.

Methods

A cohort of 56 patients, including 45 unilateral EDs for LLD and 11 bilateral EDs for tall stature, were prospectively analyzed. ED was performed with 222 rigid staples with a mean follow-up of 24.4 months (8 to 49). Patients with a predicted LLD of ≥ 2 cm at skeletal maturity were included. Mean age at surgery was 12.1 years (8 to 14). Correction and complication rates including implant-associated problems, and secondary deformities as well as perioperative parameters, were recorded (IDEAL stage 2a). These results were compared to historical cohorts treated for correction of LLD with two-hole plates or Blount staples.


The Bone & Joint Journal
Vol. 103-B, Issue 11 | Pages 1736 - 1741
1 Nov 2021
Tolk JJ Eastwood DM Hashemi-Nejad A

Aims

Perthes’ disease (PD) often results in femoral head deformity and leg length discrepancy (LLD). Our objective was to analyze femoral morphology in PD patients at skeletal maturity to assess where the LLD originates, and evaluate the effect of contralateral epiphysiodesis for length equalization on proximal and subtrochanteric femoral lengths.

Methods

All patients treated for PD in our institution between January 2013 and June 2020 were reviewed retrospectively. Patients with unilateral PD, LLD of ≥ 5 mm, and long-leg standing radiographs at skeletal maturity were included. Total leg length, femoral and tibial length, articulotrochanteric distance (ATD), and subtrochanteric femoral length were compared between PD side and the unaffected side. Furthermore, we compared leg length measurements between patients who did and who did not have a contralateral epiphysiodesis.


Orthopaedic Proceedings
Vol. 97-B, Issue SUPP_9 | Pages 9 - 9
1 Aug 2015
Yeo A Richards C Eastwood D
Full Access

This study aimed to define the rates of lower limb angular correction using temporary hemiepiphysiodesis in differing skeletal pathologies. A retrospective review of 61 children (36M:25F) with angular deformities about the knee who underwent 8-plate hemiepiphysiodesis (mean age 10.8y) was undertaken. The children were divided into 9 groups based on their underlying pathology (lower limb hypoplasia, Blount's disease, skeletal dysplasia, rickets, metabolic disease, acquired growth disturbance, vascular malformation, steroid use and complex genetic disorders). Radiographic measurements of each limb segment was undertaken using the TraumaCad® digital templating software based on standing long-leg radiographs - mechanical lateral distal femoral angle (mLDFA) and mechanical medial proximal tibial angle (mMPTA). The rate of correction of each parameter was calculated as a function of the time lapse between the operation date and first radiographic evidence of full correction of the mechanical axis (zone 1). A total of 144 limb segments (80 distal femoral, 64 proximal tibial physes) were analysed. 62.5% of children had mechanical axes outside the knee joint at the time of operation; 63.2% achieved full correction. The rate of angular correction at the distal femur (mLDFA) was quickest in those with acquired growth disturbance (1.15°/month), complex genetic disorders (1.12°/month) and rickets (0.93°/month). It was slowest in those with vascular malformation (0.40°/month), lower extremity hypoplasia (0.44°/month) and metabolic disease (0.49°/month). At the proximal tibia, mMPTA correction was quickest in those with acquired growth disturbance (0.77°/month) and skeletal dysplasia (0.57°/month); whilst being slowest in those with metabolic disease (0.22°/month) and Blount's disease (0.29°/month). The rate of angular correction about the knee varies with the underlying pathology with correction rates varying up to 3-fold. This study demonstrated the differential rate of correction of angular deformities in children with different skeletal pathologies, which would help guide the timing of hemiepiphysiodesis


The Journal of Bone & Joint Surgery British Volume
Vol. 87-B, Issue 5 | Pages 710 - 715
1 May 2005
van Huyssteen AL Hastings CJ Olesak M Hoffman EB

We reviewed 34 knees in 24 children after a double-elevating osteotomy for late-presenting infantile Blount’s disease. The mean age of patients was 9.1 years (7 to 13.5). All knees were in Langenskiöld stages IV to VI. The operative technique corrected the depression of the medial joint line by an elevating osteotomy, and the remaining tibial varus and internal torsion by an osteotomy just below the apophysis. In the more recent patients (19 knees), a proximal lateral tibial epiphysiodesis was performed at the same time. The mean pre-operative angle of depression of the medial tibial plateau of 49° (40° to 60°) was corrected to a mean of 26° (20° to 30°), which was maintained at follow-up. The femoral deformity was too small to warrant femoral osteotomy in any of our patients. The mean pre-operative mechanical varus of 30.6° (14° to 66°) was corrected to 0° to 5° of mechanical valgus in 29 knees. In five knees, there was an undercorrection of 2° to 5° of mechanical varus. At follow-up a further eight knees, in which lateral epiphysiodesis was delayed beyond five months, developed recurrent tibial varus associated with fusion of the medial proximal tibial physis


The Bone & Joint Journal
Vol. 103-B, Issue 5 | Pages 991 - 998
1 May 2021
Lyu X Chen T Yang Z Fu G Feng C Zhang T Lu M

Aims

The objective of this study was to evaluate the clinical and radiological outcomes of patients younger than six months of age with developmental dysplasia of the hip (DDH) managed by either a Pavlik harness or Tübingen hip flexion splint.

Methods

Records of 251 consecutive infants with a mean age of 89 days (SD 47), diagnosed with DDH between January 2015 and December 2018, were retrospectively reviewed. Inclusion criteria for patients with DDH were: younger than 180 days at the time of diagnosis; ultrasound Graf classification of IIc or greater; treatment by Pavlik harness or Tübingen splint; and no prior treatment history. All patients underwent hip ultrasound every seven days during the first three weeks of treatment and subsequently every three to four weeks until completion of treatment. If no signs of improvement were found after three weeks, the Pavlik harness or Tübingen splint was discontinued. Statistical analysis was performed.


The Bone & Joint Journal
Vol. 103-B, Issue 3 | Pages 589 - 596
1 Mar 2021
Amin N Kraft J Fishlock A White A Holton C Kinsey S Feltbower R James B

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.


The Bone & Joint Journal
Vol. 102-B, Issue 10 | Pages 1412 - 1418
3 Oct 2020
Ballhause TM Stiel N Breyer S Stücker R Spiro AS

Aims

Eight-plates are used to correct varus-valgus deformity (VVD) or limb-length discrepancy (LLD) in children and adolescents. It was reported that these implants might create a bony deformity within the knee joint by change of the roof angle (RA) after epiphysiodesis of the proximal tibia following a radiological assessment limited to anteroposterior (AP) radiographs. The aim of this study was to analyze the RA, complemented with lateral knee radiographs, with focus on the tibial slope (TS) and the degree of deformity correction.

Methods

A retrospective, single-centre study was conducted. The treatment group (n = 64 knees in 44 patients) was subclassified according to the implant location in two groups: 1) medial hemiepiphysiodesis; and 2) lateral hemiepiphysiodesis. A third control group consisted of 25 untreated knees. The limb axes and RA were measured on long standing AP leg radiographs. Lateral radiographs of 40 knees were available for TS analysis. The mean age of the patients was 10.6 years (4 to 15) in the treatment group and 8.4 years (4 to 14) in the control group. Implants were removed after a mean 1.2 years (0.5 to 3).


Aims

The study was undertaken to compare the efficacy of Woodcast splints and plaster-of-Paris casts in maintaining correction following sequential manipulation of idiopathic clubfeet.

Methods

In this randomized prospective trial, 23 idiopathic clubfeet were immobilized with plaster-of-Paris casts and 23 clubfeet were immobilized with a splint made of Woodcast that encircled only two-thirds the circumference of the limb. The number of casts or splints needed to obtain full correction, the frequency of cast or splint-related complications, and the time taken for application and removal of the casts and splints were compared.


The Bone & Joint Journal
Vol. 102-B, Issue 10 | Pages 1405 - 1411
3 Oct 2020
Martynov I Klink T Slowik V Stich R Zimmermann P Engel C Lacher M Boehm R

Aims

This exploratory randomized controlled trial (RCT) aimed to determine the splint-related outcomes when using the novel biodegradable wood-composite splint (Woodcast) compared to standard synthetic fibreglass (Dynacast) for the immobilization of undisplaced upper limb fractures in children.

Methods

An exploratory RCT was performed at a tertiary paediatric referral hospital between 1 June 2018 and 30 September 2019. The intention-to-treat population consisted of 170 patients (mean age 8.42 years (SD 3.42); Woodcast (WCG), n = 84, 57 male (67.9%); Dynacast (DNG), n = 86, 58 male (67.4%)). Patients with undisplaced upper limb fractures were randomly assigned to WCG or DNG treatment groups. Primary outcome was the stress stability of the splint material, defined as absence of any deformations or fractures within the splint during study period. Secondary outcomes included patient satisfaction and medical staff opinion. Additionally, biomechanical and chemical analysis of the splint samples was carried out.


The Bone & Joint Journal
Vol. 95-B, Issue 4 | Pages 568 - 573
1 Apr 2013
Pichler K Herbert V Schmidt B Fischerauer EE Leithner A Weinberg A

Matrix metalloproteinases (MMPs), responsible for extracellular matrix remodelling and angiogenesis, might play a major role in the response of the growth plate to detrimental loads that lead to overuse injuries in young athletes. In order to test this hypothesis, human growth plate chondrocytes were subjected to mechanical forces equal to either physiological loads, near detrimental or detrimental loads for two hours. In addition, these cells were exposed to physiological loads for up to 24 hours. Changes in the expression of MMPs -2, -3 and -13 were investigated. We found that expression of MMPs in cultured human growth plate chondrocytes increases in a linear manner with increased duration and intensity of loading. We also showed for the first time that physiological loads have the same effect on growth plate chondrocytes over a long period of time as detrimental loads applied for a short period. These findings confirm the involvement of MMPs in overuse injuries in children. We suggest that training programmes for immature athletes should be reconsidered in order to avoid detrimental stresses and over-expression of MMPs in the growth plate, and especially to avoid physiological loads becoming detrimental. Cite this article: Bone Joint J 2013;95-B:568–73


The Bone & Joint Journal
Vol. 102-B, Issue 8 | Pages 1056 - 1061
1 Aug 2020
Gordon JE Anderson JT Schoenecker PL Dobbs MB Luhmann SJ Hoernschemeyer DG

Aims

Current American Academy of Orthopaedic Surgeons (AAOS) guidelines for treating femoral fractures in children aged two to six years recommend early spica casting although some individuals have recommended intramedullary stabilization in this age group. The purpose of this study was to compare the treatment and family burden of care of spica casting and flexible intramedullary nailing in this age group.

Methods

Patients aged two to six years old with acute, non-pathological femur fractures were prospectively enrolled at one of three tertiary children’s hospitals. Either early closed reduction with spica cast application or flexible intramedullary nailing was accomplished under general anaesthesia. The treatment method was selected after discussion of the options by the surgeon with the family. Data were prospectively collected on patient demographics, fracture characteristics, complications, pain medication, and union. The Impact on Family Scale was obtained at the six-week follow-up visit. In all, 75 patients were included in the study: 39 in the spica group and 36 in the nailing group. The mean age of the spica group was 2.71 (2.0 to 6.9) years and the mean age of the nailing group was 3.16 (2.0 to 6.9) years.


Bone & Joint Open
Vol. 1, Issue 7 | Pages 364 - 369
10 Jul 2020
Aarvold A Lohre R Chhina H Mulpuri K Cooper A

Aims

Though the pathogenesis of Legg-Calve-Perthes disease (LCPD) is unknown, repetitive microtrauma resulting in deformity has been postulated. The purpose of this study is to trial a novel upright MRI scanner, to determine whether any deformation occurs in femoral heads affected by LCPD with weightbearing.

Methods

Children affected by LCPD were recruited for analysis. Children received both standing weightbearing and supine scans in the MROpen upright MRI scanner, for coronal T1 GFE sequences, both hips in field of view. Parameters of femoral head height, width, and lateral extrusion of affected and unaffected hips were assessed by two independent raters, repeated at a one month interval. Inter- and intraclass correlation coefficients were determined. Standing and supine measurements were compared for each femoral head.


Orthopaedic Proceedings
Vol. 95-B, Issue SUPP_6 | Pages 11 - 11
1 Feb 2013
Scally M Van't Hoff W Bockenhauer D Eastwood D
Full Access

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


Orthopaedic Proceedings
Vol. 98-B, Issue SUPP_15 | Pages 6 - 6
1 Sep 2016
Horn A Wright J Eastwood D
Full Access

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


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXIV | Pages 11 - 11
1 May 2012
Siddiqui N Malaga-Shaw O Eastwood D
Full Access

Purpose. To define the orthopaedic problems associated with pseudoachondroplasia (PSACH) and their functional impact. Methods. We reviewed the medical records of 12 consecutive patients presenting to our unit. Radiographic analysis of deformity included assessment of mechanical axis and dysplasia at hip, knee and ankle measured by acetabular index (AI), Reimer's migration percentage (MP), neck-shaft angle, distal lateral femoral (aDFLA) and proximal (mMPTA) and distal tibial angles. The paediatric/adolescent PODCI questionnaires and the SF36 were used to assess quality of life issues. Results. 12 patients (9 female) were reviewed at median age 18yrs (range 12-43yrs). Most symptoms related to walking tolerance, joint discomfort and deformity: 9 patients had genu varum, 7 tibial torsion, 2 patella instability and 3 significant low back pain. All patients had hip dysplasia. 10 had medial displacement of the mechanical axis, with a mean mDFLA 105 deg (88 -128) and mMPTA 75deg (51-90). 2 patients have been treated only with growth hormone; 10 patients have undergone a total of 9 distal femoral, 19 proximal tibial and 2 supramalleolar osteotomies. 6 procedures were performed using an external fixator. 7 limb segments have been treated by guided growth and in all these cases alignment has improved. One patient has had bilateral hip arthroplasties (age 29), a second patient has had bilateral patellectomies. These 10 patients have undergone a mean 3.8 operative procedures on a mean 2.4 occasions. Patients scored less well than their peer groups in all domains of the PODCI assessment. All have maintained some independent mobility. Conclusions. PSACH is a severe skeletal dysplasia with deformity at all levels of the lower limb affecting patient satisfaction and quality of life. Knee deformities are those which most frequently require surgical intervention. Significance. The genetic defect in PSACH differs from that in achondroplasia, joint degeneration is more common and maintenance of limb alignment is essential