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Bone & Joint Research
Vol. 3, Issue 1 | Pages 1 - 6
1 Jan 2014
Yamada K Mihara H Fujii H Hachiya M

Objectives. There are several reports clarifying successful results following open reduction using Ludloff’s medial approach for congenital (CDH) or developmental dislocation of the hip (DDH). This study aimed to reveal the long-term post-operative course until the period of hip-joint maturity after the conventional surgical treatments. Methods. A long-term follow-up beyond the age of hip-joint maturity was performed for 115 hips in 103 patients who underwent open reduction using Ludloff’s medial approach in our hospital. The mean age at surgery was 8.5 months (2 to 26) and the mean follow-up was 20.3 years (15 to 28). The radiological condition at full growth of the hip joint was evaluated by Severin’s classification. Results. All 115 hips successfully attained reduction after surgery; however, 74 hips (64.3%) required corrective surgery at a mean age of 2.6 years (one to six). According to Severin’s classification, 69 hips (60.0%) were classified as group I or II, which were considered to represent acceptable results. A total of 39 hips (33.9%) were group III and the remaining seven hips (6.1%) group IV. As to re-operation, 20 of 21 patients who underwent surgical reduction after 12 months of age required additional corrective surgeries during the growth period as the hip joint tended to subluxate gradually. Conclusion. Open reduction using Ludloff’s medial approach accomplished successful joint reduction for persistent CDH or DDH, but this surgical treatment was only appropriate before the ambulating stage. Cite this article: Bone Joint Res 2014;3:1–6


Orthopaedic Proceedings
Vol. 106-B, Issue SUPP_18 | Pages 35 - 35
14 Nov 2024
Bulut H Abasova F Basaran T Balaban P
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Introduction. Congenital scoliosis is a prevalent congenital spinal deformity, more frequently encountered than congenital lordosis or kyphosis. The prevailing belief is that most instances of congenital scoliosis are not hereditary but rather stem from issues in fetal spine development occurring between the 5th and 8th weeks of pregnancy. However, it has been linked to several genes in current literature. Our goal was to explore potential pathways through an exhaustive bioinformatics analysis of genes related to congenital scoliosis. Method. The literature from the 1970s to February 2024 was surveyed for genes associated with CS, and 63 genes were found to be associated with AIS out of 1743 results. These genes were analyzed using DAVID Bioinformatics. Result. Our pathway analysis has unveiled several significant associations with congenital scoliosis. Notably, “Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate” (P-Value:8.8E-3, Fold Enrichment: 20.6), “Central carbon metabolism in cancer” (P-Value:1.3E-3, Fold Enrichment: 10.3), and “Lysine degradation” (P-Value: 9.0E-3, Fold Enrichment: 9.1) emerge as statistically significant pathways. Additionally, “Endocrine resistance” (P-Value:4.4E-3, Fold Enrichment:7.4) and”EGFR tyrosine kinase inhibitor resistance” (P-Value: 1.7E-2, Fold Enrichment:7.3) pathways are noteworthy. These findings suggest a potential involvement of these pathways in the biological processes underlying congenital scoliosis. Furthermore, “Signaling pathways regulating pluripotency of stem cells” (P-Value:4.0E-4, Fold Enrichment:7.1), “Notch signaling pathway” (P-Value:6.7E-2, Fold Enrichment: 7.0), and “TGF-beta signaling pathway” (P-Value:6.2E-3, Fold Enrichment: 6.7) exhibit a less pronounced yet intriguing association that may warrant further investigation. Conclusion. In conclusion, our comprehensive analysis of the genetic etiology of congenital scoliosis has revealed significant associations with various pathways, shedding light on potential underlying biological mechanisms. While further research is needed to fully understand these associations and their implications, our findings provide a valuable starting point for future investigations into the management and treatment of congenital scoliosis


Orthopaedic Proceedings
Vol. 105-B, Issue SUPP_18 | Pages 2 - 2
1 Dec 2023
Basheer S Kwaees T Tang C Ali F Haslam P Nicolaou N
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Objectives. Congenital cruciate ligament deficiency is a rare condition that may occur in isolation or in association with longitudinal limb deficiencies such as fibular hemimelia or proximal femoral focal deficiency. Often anomalies of the menisci and their attachments can be very abnormal and impact on surgical management by standard techniques. Arthroscopic surgical knee reconstruction is undertaken to improve symptomatic instability and/or to stabilise and protect the knee for future planned limb lengthening surgery. The aim of this study is to evaluate the arthroscopic findings of patients undergoing surgery for congenital cruciate ligament deficiency, and specifically to determine the frequency and types of meniscal anatomical variations seen in these cases. Methods. Patients undergoing surgery for congenital cruciate ligament deficiency were identified from a prospectively collated database. Diagnosis was confirmed through review of the clinical notes and imaging. Operative notes and 4K saved arthroscopic images and video recordings for these cases were reviewed. Results. Over a six-year period (July 2017 – September 2023), 42 patients underwent surgery for congenital ligament deficiency and tibiofemoral instability (45 surgical episodes). Median age of patients at time of surgery was 10 years (range 4 – 17 years). The most frequent diagnosis was congenital longitudinal limb deficiency syndromes in 27 cases, with the most frequent being fibular hemimelia. Isolated congenital ligament deficiency without any other associated extra-articular manifestations occurred in 11 cases. Absence of meniscal root attachments or hypertrophy of meniscofemoral ligaments acting as ‘pseudo-cruciates’ were seen in over 25% of patients. In isolated ACL deficiency these were injured causing onset of instability symptoms and pain following trauma. Often these abnormal structures required addressing to allow surgical reconstruction. Conclusions. Our findings demonstrate that there are often meniscal variations seen in association with congenital absence or hypoplasia of the cruciate ligaments. In these patients hypertrophied meniscofemoral ligaments may act as cruciate-like structures and play a role in providing a degree of sagittal plane stability to the knee. However, when the knee becomes unstable to the point that cruciate ligament reconstruction is indicated, these meniscal variants may often require stabilisation using complex meniscal root repair techniques or variations to standard cruciate ligament reconstruction techniques to accommodate the variant anatomy


Orthopaedic Proceedings
Vol. 88-B, Issue SUPP_II | Pages 231 - 231
1 May 2006
Tsirikos A McMaster M
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Background: Congenital anomalies of the chest wall and Sprengel’s shoulder are often associated with congenital deformities of the spine. It has been suggested that extensive rib fusions on the concavity of a congenital scoliosis may adversely affect progression of the spine deformity, thoracic function and growth of the lungs, which can lead to a thoracic insufficiency syndrome. Methods: This is a retrospective study of the medical records and spine radiographs of 620 consecutive patients with congenital spine deformities. The rib anomalies were classified into simple and complex and the presence of a Sprengel’s shoulder was recorded. Subjects: There were 497 patients (80%) with scoliosis, 88 patients (14%) with kyphoscoliosis, and 35 patients with kyphosis (6%). The mean rate of scoliosis deterioration without treatment, age and curve size at surgery was compared for the different types of vertebral abnormalities in patients with and without rib anomalies. Results: Rib anomalies occurred in 124 patients. The rib anomalies were simple in 97 patients (78%) and complex in 27 (22%). The most common simple rib anomaly (70 patients; 72%) was a fusion of two or three ribs. The most common complex rib anomaly (20 patients; 74%) was a fusion of multiple ribs associated with a large chest wall defect. Rib anomalies were most commonly associated with a congenital scoliosis (116 patients; 93.6%), and much less frequently with a congenital kyphoscoliosis or kyphosis (8 patients). In those patients with a scoliosis, the rib anomalies were simple in 91 patients (78.5%) and complex in 25 patients (21.5%). Eighty-eight of the 124 patients (76%) with rib abnormalities had a scoliosis due to a unilateral unsegmented bar with or without contra-lateral hemivertebrae at the same level, and 8 patients had mixed unclassifiable vertebral anomalies. In contrast, only 17 patients with a scoliosis and rib anomalies had hemivertebrae alone. The rib anomalies were most frequently associated with a thoracic or thoracolumbar scoliosis (107 patients; 92.2%) and occurred on the concavity in 81 patients (70%), convexity in 27 patients (23%), and were bilateral in 8 patients. Sprengel’s deformity occurred in 45 patients; 43 patients with congenital scoliosis (8.6%), and 2 with kyphoscoliosis or kyphosis. A Sprengel’s shoulder occurred most frequently in association with a thoracic scoliosis due to a unilateral failure of vertebral segmentation (28 patients). The elevated shoulder was on the concavity of scoliosis in 26 patients and on the convexity in 16 patients. We compared the mean rate of curve progression without treatment, the age and curve size at spine surgery for the different types of spine deformities in patients with and without rib anomalies either simple or complex and found no significant difference (p> 0.05); the only statistical difference was that the mean age at surgery was higher for patients with a unilateral unsegmented bar without rib anomalies (p=0.005). Conclusion: Congenital rib anomalies and Sprengel’s shoulder occur most commonly on the concavity of a thoracic or thoracolumbar congenital scoliosis due to a unilateral failure of vertebral segmentation. However, these anomalies do not have an adverse effect on curve size or rate of progression


Orthopaedic Proceedings
Vol. 105-B, Issue SUPP_2 | Pages 44 - 44
10 Feb 2023
Kollias C Neville E Vladusic S McLachlan L
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Specific brace-fitting complications in idiopathic congenital talipes equinovarus (CTEV) have been rarely described in published series, and usually focus on non-compliance. Our primary aim was to compare the rate of persistent pressure sores in patients fitted with Markell boots and Mitchell boots. Our additional aims were to describe the frequency of other brace fitting complications and identify age trends in these complications. A retrospective analysis of medical files of 247 idiopathic CTEV patients born between 01/01/2010 - 01/01/2021 was performed. Data was collected using a REDCap database. Pressure sores of sufficient severity for clinician to recommend time out of brace occurred in 22.9% of Mitchell boot and 12.6% of Markell boot patients (X. 2. =6.9, p=0.009). The overall rate of bracing complications was 51.4%. 33.2% of parents admitted to bracing non-compliance and 31.2% of patients required re-casting during the bracing period for relapse. For patients with a minimum follow-up of age 6 years, 44.2% required tibialis anterior tendon transfer. Parents admitting to non-compliance were significantly more likely to have a child who required tibialis anterior tendon transfer (X. 2. =5.71, p=0.017). Overall rate of capsular release (posteromedial release or posterior release) was 2.0%. Neither medium nor longterm results of Ponseti treatment in the Australian and New Zealand clubfoot have been published. Globally, few publications describe specific bracing complications in clubfoot, despite this being a notable challenge for clinicians and families. Recurrent pressure sores is a persistent complication with the Mitchell boots for patients in our center. In our population of Australian clubfoot patients, tibialis anterior tendon transfer for relapse is common, consistent with the upper limit of tibialis anterior tendon transfer rates reported globally


Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_IV | Pages 568 - 568
1 Oct 2010
Tsiridis E Dickson R Dimopoulos K Gatzoulis M Papaphylactou M Polyzois I Rankine J Rubens M
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There is believed to be a correlation between congenital idiopathic scoliosis and congenital heart disease (CHD). Clinical and cardiological data was recorded for 3538 adolescents suffering from CHD. Data collected included the type of scoliosis; the direction of the curve; the Cobb angle; the number of curves and the presence or not of previous corrective cardiac surgery. Over 30% of the study group were found to suffer from scoliosis and a positive correlation with specific syndromes was also identified. The mean age of the patients was 34.0 +/− 14.0 years. The maximum Cobb angle was 107 degrees while the median was 7.6 degreees. Scoliosis was present in 37/188 (19.7%) was Eisenmenger syndrome (with R-L shunt) and 60/158= 38% with complex cardiac anatomy. There were also 20/103= 19.4% patients with univentricular (Fontan) circulation. Scoliosis was not necessarily related to previous corrective cardiac surgery, contrary to the current assumption in the literature. The hypothesis of common genetic pathway defects expressed both in cardiovascular and musculoskeletal organogenesis was raised and the TGF-beta pathway involvement is speculated


The Bone & Joint Journal
Vol. 104-B, Issue 6 | Pages 758 - 764
1 Jun 2022
Gelfer Y Davis N Blanco J Buckingham R Trees A Mavrotas J Tennant S Theologis T

Aims. The aim of this study was to gain an agreement on the management of idiopathic congenital talipes equinovarus (CTEV) up to walking age in order to provide a benchmark for practitioners and guide consistent, high-quality care for children with CTEV. Methods. The consensus process followed an established Delphi approach with a predetermined degree of agreement. The process included the following steps: establishing a steering group; steering group meetings, generating statements, and checking them against the literature; a two-round Delphi survey; and final consensus meeting. The steering group members and Delphi survey participants were all British Society of Children’s Orthopaedic Surgery (BSCOS) members. Descriptive statistics were used for analysis of the Delphi survey results. The Appraisal of Guidelines for Research & Evaluation checklist was followed for reporting of the results. Results. The BSCOS-selected steering group, the steering group meetings, the Delphi survey, and the final consensus meeting all followed the pre-agreed protocol. A total of 153/243 members voted in round 1 Delphi (63%) and 132 voted in round 2 (86%). Out of 61 statements presented to round 1 Delphi, 43 reached ‘consensus in’, no statements reached ‘consensus out’, and 18 reached ‘no consensus’. Four statements were deleted and one new statement added following suggestions from round 1. Out of 15 statements presented to round 2, 12 reached ‘consensus in’, no statements reached ‘consensus out’, and three reached ‘no consensus’ and were discussed and included following the final consensus meeting. Two statements were combined for simplicity. The final consensus document includes 57 statements allocated into six successive stages. Conclusion. We have produced a consensus document for the treatment of idiopathic CTEV up to walking age. This will provide a benchmark for standard of care in the UK and will help to reduce geographical variability in treatment and outcomes. Appropriate dissemination and implementation will be key to its success. Cite this article: Bone Joint J 2022;104-B(6):758–764


Bone & Joint Open
Vol. 3, Issue 1 | Pages 98 - 106
27 Jan 2022
Gelfer Y Leo DG Russell A Bridgens A Perry DC Eastwood DM

Aims. To identify the minimum set of outcomes that should be collected in clinical practice and reported in research related to the care of children with idiopathic congenital talipes equinovarus (CTEV). Methods. A list of outcome measurement tools (OMTs) was obtained from the literature through a systematic review. Further outcomes were collected from patients and families through a questionnaire and interview process. The combined list, as well as the appropriate follow-up timepoint, was rated for importance in a two-round Delphi process that included an international group of orthopaedic surgeons, physiotherapists, nurse practitioners, patients, and families. Outcomes that reached no consensus during the Delphi process were further discussed and scored for inclusion/exclusion in a final consensus meeting involving international stakeholder representatives of practitioners, families, and patient charities. Results. In total, 39 OMTs were included from the systematic review. Two additional OMTs were identified from the interviews and questionnaires, and four were added after round one Delphi. Overall, 22 OMTs reached ‘consensus in’ during the Delphi and two reached ‘consensus out’; 21 OMTs reached ‘no consensus’ and were included in the final consensus meeting. In all, 21 participants attended the consensus meeting, including a wide diversity of clubfoot practitioners, parent/patient representative, and an independent chair. A total of 21 outcomes were discussed and voted upon; six were voted ‘in’ and 15 were voted ‘out’. The final COS document includes nine OMTs and two existing outcome scores with a total of 31 outcome parameters to be collected after a minimum follow-up of five years. It incorporates static and dynamic clinical findings, patient-reported outcome measures, and a definition of CTEV relapse. Conclusion. We have defined a minimum set of outcomes to draw comparisons between centres and studies in the treatment of CTEV. With the use of these outcomes, we hope to allow more meaningful research and a better clinical management of CTEV. Cite this article: Bone Jt Open 2022;3(1):98–106


Orthopaedic Proceedings
Vol. 104-B, Issue SUPP_11 | Pages 45 - 45
1 Nov 2022
Chaudhary I Sagade B Jagani N Chaudhary M
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Abstract. Congenital posteromedial bowing of tibia (CPMBT) progresses with decreasing deformity and increasing shortening. Lengthening in CPMBT has not been studied extensively. Our series compares duration and complications of lengthening in younger vs older children. Methods. 28 tibial lengthenings (23 patients) by a single surgeon, divided into two equal groups of 14 segments: group-A ≤ 5 years, Group-B > 5 years. Lengthening was done in all with external fixators. We measured preoperative (bo) and postoperative (po) deformities, initial limb length discrepancy (LLD), LLD at maturity (LLDm), % LLD, amount of lengthening (AmtL) and %L, external fixator duration (EFD) and external fixator index (EFI). We graded complications by Lascombes' criteria, results by ASAMI Bone score. Results. Mean age was 8.8 ± 7.1 years. Follow-up was 7.9 years. Group-A had significantly greater preoperative deformities. LLD was similar in both. Expected LLD at maturity (LLDM) using the multiplier method was greater than previously reported (group-A: 4.4 – 9.5 cm; group-B: 2.5 – 9.7 cm).%L was 24% in group-A and 15.7% in group-B (p=0.002). EFD and EFI were lesser in group-A than group-B. Lascombes' triple contract was fulfilled in 11/14 lengthenings in group-A vs. 3/14 in group-B. ASAMI bone score was good and excellent in both groups. Conclusions. In our large series of CPMBT lenghtenings, we found younger children presenting with large deformities and LLDm could be safely lengthened with lesser EFD and complications than older children


Orthopaedic Proceedings
Vol. 104-B, Issue SUPP_5 | Pages 46 - 46
1 Apr 2022
Sagade B Chaudhary M Jagani N Chaudhary I
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Introduction. Congenital posteromedial bowing of tibia (CPMBT) is characterized by a decreasing deformity and an increasing limb shortening. Our series compares the duration and complications of lengthening in younger vs older children. Materials and Methods. We studied 28 tibial lengthenings in 23 patients, divided into two equal groups of 14 segments: group-A ≤5 years (preschool) and Group-B >5 years. We measured preoperative (bo) and postoperative (po) sagittal, coronal, and oblique plane deformities, limb length discrepancy (LLD), amount of lengthening (AmtL), percentage lengthening (%L), external fixator duration (EFD) and external fixator index (EFI). Complications were graded by Lascombes’ criteria, results by ASAMI Bone score. Results. Mean age= 8.8 ± 7.1 years; mean follow-up= 7.9 years. Group-A had significantly greater bo-sagittal, coronal, and oblique plane deformities. LLD (3.4 cm in group-A vs. 4.1 cm in group-B) was similar in both. LLD at maturity (LLD. M. ) by multiplier method ranged 4.4–9.5 cm in group-A and 2.5–9.7 cm in group-B. Though AmtL (3.5 cm and 4.1 cm) was similar in both, %L was 24% in group-A and 15.7% in group-B (p=0.002). EFD (116.6 days) and EFI (33.7 days/cm) were lesser in group-A vs group-B (200.3 days, p=0.001; 50.2 days/cm, p=0.01). Lascombes’ criteria were fulfilled in 11/14 lengthenings in group-A vs. 3/14 in group-B. ASAMI bone score was good and excellent in both groups (p=0.44). Conclusions. Younger children with large deformities and LLD. M. could be safely lengthened with lesser external fixator duration and complications than in older children


Orthopaedic Proceedings
Vol. 101-B, Issue SUPP_3 | Pages 7 - 7
1 Apr 2019
Sakale H Agrawal AC
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Incidence of Congenital talipes equino varus [CTEV] is 1 to 2 per 1000 birth, Out of all cases 20% cases are Non-idiopathic. The management of non-idiopathic CTEV, however, continues to be challenging due to Rigidity, Poor skin condition, Bony changes, Vascularity and Associated congenital abnormalities. In recent literature, short term results of Ponseti method for correction of non-idiopathic CTEV have been encouraging. As Ponseti method decreases the severity of deformity and hence decreases the need for extensive surgery. The aim of current study is to evaluate the results of Ponseti method in Non-idiopathic CTEV. Total 7 children below the age of one year with Non idiopathic clubfoot presented to us in the duration of 2013 to 2015 who were treated by us. The cases included are Streeters Dysplasia with congenital constriction rings 3, Arthrogryposis multiplex congenita with Developmental dysplasia of hip 2, Arthrogryposis multiple congenita spina Bifida 1, Pierre Robinson Syndrome with Ichthiosis 1. Initially all the patients treated with Ponseti casting technique and scoring was done using modified pirani scoring. At an average we could correct the foot from Pirani 7 to 2.5 with a relapse in 4 patients. 2 patients were treated again by Ponseti's method with success while treatment was discontinued in 2 feet. We recommended Ponseti's technique in Syndromic clubfoot as an non-surgical initial modality with good results given. The final outcome may depend more on the underlying condition than the CTEV


Orthopaedic Proceedings
Vol. 85-B, Issue SUPP_III | Pages 198 - 198
1 Mar 2003
Elsebaie H Basu P Noordeen M
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Background: The incidence of intra-spinal abnormality in congenital scoliosis is high. McMaster et al found an 18% incidence of myelographic abnormality in a series of 251 patients. Our objective was to report the MRI findings in a large series of patients with congenital scoliosis. Method: The notes, X-rays and MRI of 126 congenital scoliosis patients were reviewed to note the vertebral abnormality, curve progression, MRI findings and the presence of non-spinal congenital abnormality. These findings were then correlated to detect any association between them. Result: Forty-six patients (37%) had intra-spinal abnormalities detected on MRI. Sixty-six patients had failure of formation, 10 had failure of segmentation, 34 had mixed vertebral anomaly and 16 had congenital kyphosis or dislocation. MRI abnormality was significantly higher among patients with mixed anomaly (41%), congenital kyphosis (57%) and segmentation anomaly (40%) than those with failure of formation (29%). Presence of MRI abnormality did not correlate with curve progression or the presence of congenital abnormality affecting other organs. Conclusion: Intra-spinal abnormality in congenital scoliosis occurred in 37% cases. The incidence of such abnormality is higher in patients with congenital kyphosis, failure of segmentation and mixed vertebral anomalies


Orthopaedic Proceedings
Vol. 106-B, Issue SUPP_2 | Pages 113 - 113
2 Jan 2024
García-Rey E Gómez-Barrena E
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Pelvic bone defect in patients with severe congenital dysplasia of the hip (CDH) lead to abnormalities in lumbar spine and lower limb alignment that can determine total hip arthroplasty (THA) patients' outcome. These variables may be different in uni- or bilateral CDH. We compared the clinical outcome and the spinopelvic and lower limb radiological changes over time in patients undergoing THA due to uni- or bilateral CHD at a minimum follow-up of five years. Sixty-four patients (77 hips) undergoing THA due to severe CDH between 2006 and 2015 were analyzed: Group 1 consisted of 51 patients with unilateral CDH, and group 2, 113 patients (26 hips) with bilateral CDH. There were 32 females in group 1 and 18 in group 2 (p=0.6). The mean age was 41.6 years in group 1 and 53.6 in group 2 (p<0.001). We compared the hip, spine and knee clinical outcomes. The radiological analysis included the postoperative hip reconstruction, and the evolution of the coronal and sagittal spinopelvic parameters assessing the pelvic obliquity (PO) and the sacro-femoro-pubic (SFP) angles, and the knee mechanical axis evaluating the tibio-femoral angle (TFA). At latest follow-up, the mean Harris Hip Score was 88.6 in group 1 and 90.7 in group 2 (p=0.025). Postoperative leg length discrepancy of more than 5 mm was more frequent in group 1 (p=0.028). Postoperative lumbar back pain was reported in 23.4% of the cases and knee pain in 20.8%, however, there were no differences between groups. One supracondylar femoral osteotomy and one total knee arthroplasty were required. The radiological reconstruction of the hip was similar in both groups. The PO angle improved more in group 1 (p=0.01) from the preoperative to 6-weeks postoperative and was constant at 5 years. The SFP angle improved in both groups but there were no differences between groups (p=0.5). 30 patients in group 1 showed a TFA less than 10º and 17 in group 2 (p=0.7). Although the clinical outcome was better in terms of hip function in patients with bilateral CDH than those with unilateral CDH, the improvement in low back and knee pain was similar. Patients with unilateral dysplasia showed a better correction of the PO after THA. All spinopelvic and knee alignment parameters were corrected and maintained over time in most cases five years after THA


Orthopaedic Proceedings
Vol. 102-B, Issue SUPP_11 | Pages 58 - 58
1 Dec 2020
Ranson J Nuttall G Paton R
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Aims & Background. Congenital Talipes Equinovarus (CTEV) is the most common congenital musculoskeletal birth defect affecting 1 in 1000 births per annum. We have compared our surgical results to the British Society of Children's Orthopaedics (BSCOS) published guidelines. Methods. Between, 2006–16, patients who were referred for treatment of pathological CTEV were audited. Data from a combination of Clinical Portal, Orthotic Patient Administration System and Surgical Elogbook were assessed. In addition, the degree of deformity was classified by the Harrold & Walker method at the time of diagnosis (senior author). Most of this information was recorded prospectively and analysed retrospectively. Ponseti technique was the method of treatment. Results. 96 patients assessed (133 feet). There were 78 males and 18 females, 37 patients were affected bilaterally and 11 had associated syndromes. There were 23 Harrold & Walker (H&W) 1, 28 H&W 2 and 82 H&W 3 classification feet. Average time period in Ponseti boots and bars was 14.4 months (95% CI 12.9–15.9), average time in all types of bracing of was 17.1 months (95% CI 14.8–14.8). Number and rate of surgeries performed were as follows: 77 Tendoachilles release (63.1%), 19 Tibialis Anterior Transfer (5.6%), 15 Radical Release (12.3%), revision 25 Surgery (20.5%) & 5 Abductor Hallucis Release (4.1%). Conclusion. The audit confirms that the unit meets most of the current BSCOS guidelines. All surgical procedures apart from radical release surgery fall within accepted limits. This may be due, in part, to the syndromal cases. We do however demonstrate a significantly reduced average time period in bracing compared to that recommended by BSCOS. There are multiple reasons for this discrepancy including non-compliance and poor splint tolerance (child refusing to use). We feel this work demonstrates a reduced period in bracing can be achieved whilst maintaining standards of treatment


Orthopaedic Proceedings
Vol. 96-B, Issue SUPP_19 | Pages 16 - 16
1 Dec 2014
Olivier A Kayani B Briggs T
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Purpose. Congenital insensitivity to pain is a rare autosomal recessive condition that leads to varying degrees of sensory and autonomic neuropathy. The aim of the study was to explore the common orthopaedic presentations of congenital insensitivity to pain and provide guidance on their treatment and complications. Methods. This study presents the results of fifteen patients with congenital insensitivity to pain, which were referred and treated at our supra-regional referral centre. Intradermal histamine tests and quantitative sweat tests were performed on all fifteen patients. Results. The average age of presentation was 13.2 years (range, two to 28 years). Eight patients presented with Charcot arthropathies and joint dislocations, which involved the foot/ankle (n=4), knee (n=2) or spine (n=2). Four patients presented with fractures or avascular necrosis affecting the tarsal bones and three patients presented with recurrent infections of the lower limbs. Patient education regarding appropriate shoe ware, pedal hygiene, periods of non-weight bearing, spinal bracing and close follow-up within the multi-disciplinary team was the mainstay of treatment. Patients with infection underwent cultures, aspiration, magnetic resonance imaging and treatment with antibiotics. Only one patient required joint washout for septic arthritis of the ankle. Conclusions. Congenital insensitivity to pain presents with both acute and chronic pathology affecting the weight-bearing joints. Treatment should be aimed at off-loading the pressure on these joints and preventing the development of further complications. Surgical intervention should be reserved for patients not responding to conservative treatment or those requiring debridement/washout for extensive infection


Orthopaedic Proceedings
Vol. 85-B, Issue SUPP_III | Pages 198 - 198
1 Mar 2003
Elsebaie H Basu P Noordeen M
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Background: Surgical treatments described for congenital spinal deformity are i) convex growth arrest, ii) posterior or combined anterior and posterior fusion and iii) hemivertebrectomy. Posterior instrumentation is used as an adjunct to fusion, whenever possible. Anterior instrumentation for correction of congenital scoliosis has not been described. A preliminary report of the use of anterior instrumentation following hemi-vertebrectomy for correction of congenital spinal deformity is reported. Method: 15 patients with congenital scoliosis and 5 patients with congenital kyphosis underwent hemiverte-brectomy and anterior instrumentation with fusion for single-stage correction of deformity . The average age of the patients at the time of surgery was 31 months and at last follow-up 59 months. All patients had pre-operative MRI. Twelve patients had normal and 8 had abnormal MRI. The average operating time was 135 minutes and average blood loss was 462 ml. Implants used were downsize Synergy, Orion Colorado and AO Cervifix. Average sagittal Cobb angle for the scoliosis patients was 45.5° pre-operatively and 16.8° post-operatively. Average coronal Cobb angle in patients with congenital kyphosis was 61° pre-operatively and 21° postoperatively. At an average follow-up of 17 months, the correction is well maintained in all except one. This patient developed pseudarthrosis at 19 months post-operatively. This was treated with posterior instrumented fusion. There were no cases of neurologic compromise or deep wound infection. Conclusion: Because of the young age at which hemiver-tebrectomy is performed in congenital scoliosis patients, instrumentation is difficult. Posterior instrumentation has been well described in literature. Our early experience with anterior instrumentation after hemivertebrectomy shows promising results with very good correction of the deformity and no increase in complication rate


Orthopaedic Proceedings
Vol. 95-B, Issue SUPP_29 | Pages 38 - 38
1 Aug 2013
Moolman J Robertson A
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Purpose of study:. Congenital hallux varus is a rare condition presenting with medial deviation of the big toe. It consists of 2 variants: classical congenital hallux varus caused by an abnormal metatarsal-phalangeal articulation, and a more recently described variant due to a “bracket physis” of the first metatarsal. Our aim was to perform an audit of the spectrum of presentation of congenital hallux varus with its management and complications in our unit over a five year period. Description of methods:. A retrospective review of congenital hallux varus treated by a single surgeon was performed. Clinical notes, photographs and x-rays were reviewed. Two surgical methods of treatment were used as directed by clinical and radiological findings. Summary of results:. Four patients with congenital hallux varus were identified. Three had bilateral involvement, i.e. seven feet were treated. The ages of the patients ranged from 1 to 9 years, with 2 boys and 2 girls receiving treatment. In two patients (4 feet) the deformity was associated with pre-axial polydactyly. One patient had associated hand deformities. Only one of our four patients had a “bracket physis” and was treated with a metatarsal osteotomy. The other three patients (6 feet) were treated by soft tissue realignment using the Farmer procedure. One patient who had bilateral Farmer procedures subsequently developed a bunion over the 1st metatarso-phalangeal joint of one foot due to uncovering of the metatarsal head. No complications or recurrences were recorded in the other three patients on follow up visits. Conclusion:. Careful clinical and X-ray analysis is important to determine the type of congenital hallux varus and which method of surgical treatment is appropriate. Long term follow up is required to identify subsequent deformities


Orthopaedic Proceedings
Vol. 100-B, Issue SUPP_14 | Pages 90 - 90
1 Nov 2018
Tully R McQuail P McCormack D
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Congenital talipes equinovarus (CTEV), also known as club foot or talipes is a common congenital disorder. Parents are using the Internet more and more as a source of information about health care. Unfortunately, the quality of health care information on the Internet varies. This study looked at information available to parents using two instruments for judging the equality of information on the internet. The top five search engines were searched on Google. Three of these were also included in the top 50 sites in Ireland so these 3 sites were used. The phrases CTEV and club foot were searched from all 3 platforms. Websites were then scrutinized using the HON code and the DISCERN tool. 54 organic sites were found for the 3 search engines using the key word club foot. For the key word CTEV 55 matches were returned for the three search engines. 4 websites displayed the HON code. Using the discern tool CTEV websites had a mean score of 60 with a standard deviation of 17. While club foot had a mean score of 56.8 with a standard deviation of 13. Max score 80. Large volumes of information are available to parents on the Internet. Often parents find comfort in sharing experiences and feel empowered by learning about their children's illnesses. However, information provided on the interned can also be ambiguous and disingenuous. Practitioners should be aware of a number of key websites that parents can be directed towards


Orthopaedic Proceedings
Vol. 96-B, Issue SUPP_18 | Pages 9 - 9
1 Dec 2014
Aranganathan S Carpenter C Thomas D Hemmadi S O'Doherty D
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Complex congenital foot deformities pose a challenge to the surgeon due to poor results after extensive surgery. We report the clinical outcomes of children with complex congenital foot deformities treated with UMEX® (Universal mini-external fixator System) frames. This is a prospective review of our experience in patients treated in this way, from 2004 to 2011. The indications for treatment included resistant/recurrent Congenital Talipes Equino Varus (CTEV), cavo-varus deformity secondary to Charcot-Marie-Tooth disease, arthrogryposis, fibular hemimelia and other congenital abnormalities. A total of 32 children (35 feet) have been treated, out of which 22 were male and 10 were female patients. Age at surgery ranged from 3 to 15 years (median age – 7 years). Three patients underwent bilateral procedures; the reminder (29 patients) underwent unilateral foot operations. Twenty-eight patients had undergone previous surgery including soft-tissue and/or bony corrective procedures. The frames were removed at an average of 69 days after application, and the patients spent a further 6 weeks in a walking cast. Good functional outcomes were noted in 26 patients in the first postoperative year and in 19 patients in the fifth postoperative year. Further operations were needed in 10 patients. Complications occurred in 10 patients, predominantly pin-site infections and 1 case of bony overgrowth at pin-site and 1 of proximal tibio-fibular diastasis. This is a simple fixator to use with a short learning curve. In groups of patients with complex congenital abnormalities, we achieved good functional outcome with low-complication rates


Orthopaedic Proceedings
Vol. 91-B, Issue SUPP_II | Pages 365 - 365
1 May 2009
Mihelarakis J Markeas N Volonakis E Valentis E
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Aim: This study was carried out in order to clarify the causes that are mainly responsible for the necessity of reoperation after the initial correction of the deformity in congenital talipes equinovarus. The cases, which had been treated surgically with the same method and recurred later, were studied retrospectively in order to be ascertained epidemiological data related to the disease, to be isolated operative findings related to its pathology and to be estimated the surgical results based on clinical and radiological criteria. Material-Methods: During the 15-year-period from 1990 to 2004, 123 infants (196 feet) with congenital talipes equinovarus have been treated operatively. There were 88 males and 35 females. Seventy three patients (59.3%) had the deformity bilaterally, 20 patients in right foot and 30 in left. Family history was positive in 5 infants. Other congenital anomalies coexisted in 12 infants (9.7%). Preoperative application of successive plasters was started into the first week for 93 infants (75.6%) and its duration was 3 months for 83.7% of cases. All the patients have been operated on with posteromedial approach, extensive ligament division and generous release of soft tissues during the first year of age. Two thirds of cases (67.4%) were treated surgically into the first 6 months of age. Results: Anatomical variations were revealed during the operation in 14 feet (7.1% of the cases). The clinical results as well as the radiological signs into the first 6 postoperative months were satisfactory, but a reoperation was necessary in 21 feet (in 14 infants) for correction of part of the initial deformity into the following 2–5 years. The clinical criteria were related to the manner of standing and walking, the range of motion of the foot joints and block test. The radiological criteria were related to anteroposterior and lateral talocalcaneal angles and the angle between the longitudinal axis of the talus and that of the first metatarsal in the anteroposterior view as well as the position of the calcaneus in the lateral view. The causes that led to recurrence were related to imperfect correction with the plasters, to incomplete release of soft tissues during the initial operation and to some likely predisposing congenital and environmental factors. Conclusions: The prevention of recurrence of the initial deformity, in the operative correction of congenital talipes equinovarus, is mainly related to the attentive pre-operative application of plasters, the careful lege artis surgical technique and the early diagnosis and treatment of the predisposing factors