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

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

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

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

Aims. To describe the clinical, radiological, and functional outcomes in patients with isolated congenital thoracolumbar kyphosis who were treated with three-column osteotomy by posterior-only approach. Methods. Hospital records of 27 patients with isolated congenital thoracolumbar kyphosis undergoing surgery at a single centre were retrospectively analyzed. All patients underwent deformity correction which involved a three-column osteotomy by single-stage posterior-only approach. Radiological parameters (local kyphosis angle (KA), thoracic kyphosis (TK), lumbar lordosis (LL), pelvic tilt (PT), sacral slope (SS), C7 sagittal vertical axis (C7 SVA), T1 slope, and pelvic incidence minus lumbar lordosis (PI-LL)), functional scores, and clinical details of complications were recorded. Results. The mean age of the study population was 13.9 years (SD 6.4). The apex of deformity was in thoracic, thoracolumbar, and lumbar spine in five, 14, and eight patients, respectively. The mean operating time was 178.4 minutes (SD 38.5) and the mean operative blood loss was 701.8 ml (SD 194.4). KA (preoperative mean 70.8° (SD 21.6°) vs final follow-up mean 24.7° (SD 18.9°); p < 0.001) and TK (preoperative mean -1.48° (SD 41.23°) vs final follow-up mean 24.28° (SD 17.29°); p = 0.005) underwent a significant change with surgery. Mean Scoliosis Research Society (SRS-22r) score improved after surgical correction (preoperative mean 3.24 (SD 0.37) vs final follow-up mean 4.28 (SD 0.47); p < 0.001) with maximum improvement in self-image and mental health domains. The overall complication rate was 26%, including two neurological and five non-neurological complications. Permanent neurological deficit was noted in one patient. Conclusion. Deformity correction employing three-column osteotomies by a single-stage posterior-only approach is safe and effective in treating isolated congenital thoracolumbar kyphosis. Cite this article: Bone Joint J 2021;103-B(7):1309–1316

Diastematomyelia is a rare congenital abnormality of the spinal cord. This paper summarises more than 30 years’ experience of treating this condition. Data were collected retrospectively on 138 patients with diastematomyelia (34 males, 104 females) who were treated at our hospital from May 1978 to April 2010. A total of 106 patients had double dural tubes (type 1 diastematomyelia), and 32 patients had single dural tubes (type 2 diastematomyelia). Radiographs, CT myelography, and MRI showed characteristic kyphoscoliosis, widening of the interpedicle distance, and bony, cartilaginous, and fibrous septum. The incidences of symptoms including characteristic changes of the dorsal skin, neurological disorders, and congenital spinal or foot deformity were significantly higher in type 1 than in type 2. Surgery is more effective for patients with type 1 diastematomyelia; patients without surgery showed no improvement

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

There is a close link between the embryological development of the musculoskeletal system and all other main organ systems. We report a prospective series of 202 patients with congenital vertebral abnormalities and document the associated abnormalities in other systems. There were 100 boys and 102 girls. In 153 there were 460 associated abnormalities, a mean of 2.27 abnormalities for each patient. Intravenous pyelography was carried out on 173 patients (85.6%) and ultrasonography on the remaining 29 (14.4%). Patients with genitourinary anomalies were more likely to have musculoskeletal (p = 0.002), gastrointestinal (p = 0.02) and cardiac abnormalities (p = 0.008) than those without genitourinary involvement. A total of 54 (26.7%) had at least one genitourinary abnormality, the most frequent being unilateral renal agenesis. There was urinary obstruction in six (3%). There was no association between genitourinary abnormality and the place of birth, parental age, birth order, level of spinal curvature, or the number, type and side of spinal anomaly. There was, however, a statistically significant association (p = 0.04) between costal and genitourinary abnormalities. The incidence of genitourinary abnormalities (26.7%) was similar to that of previously reported series. The diagnosis of a congenital vertebral abnormality should alert the clinician to a wide spectrum of possible associated anomalies most of which are of clinical importance

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

This paper reports a high incidence of minor congenital anomalies in boys and girls with Perthes' disease compared with that in a control population. There is a similarity of the incidence of minor anomalies in the children with Perthes' disease to that in babies with a single major congenital defect. Multiple major defects were more numerous and more severe than in the control children. It is speculated that there may be a congenital abnormality affecting skeletal development which in some way makes the hip susceptible to Perthes' disease at a later date

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

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

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

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

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

Talipes equinovarus is one of the more common congenital abnormalities affecting the lower limb and can be challenging to manage. This review provides a comprehensive update on idiopathic congenital talipes equinovarus with emphasis on the initial treatment. Current management is moving away from operative towards a more conservative treatment using the Ponseti regime. The long-term results of surgical correction and the recent results of conservative treatment will be discussed

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

There were 47 patients with congenital muscular torticollis who underwent operative release. After a mean follow-up of 74 months (60 to 90), they were divided into two groups, one aged one to four years (group 1) and the other aged five to 16 years (group 2). The outcomes were assessed by evaluating the following parameters: deficits of lateral flexion and rotation, craniofacial asymmetry, surgical scarring, residual contracture, subjective evaluation and degree of head tilt. The craniofacial asymmetry, residual contracture, subjective evaluation and overall scores were similar in both groups. However, group 2 showed superior results to group 1 in terms of the deficits of movement, surgical scarring and degree of head tilt. It is recommended that operative treatment for congenital muscular torticollis is postponed until the patient can comply successfully with post-operative bracing and an exercise programme

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

Adult presentation of neglected congenital muscular torticollis is rare. We report 12 patients with this condition who underwent a modified Ferkel’s release comprising a bipolar release of sternocleidomastoid with Z-lengthening. They had a mean age of 24 years (17 to 31) and were followed up for a minimum of two years. Post-operatively a cervical collar was applied for three weeks with intermittent supervised active assisted exercises for six weeks. Outcome was assessed using a modified Lee score and a Cheng and Tang score. The mean pre-operative rotational deficit was 8.25° (0° to 15°) and mean lateral flexion deficit was 20.42° (15° to 30°), which improved after treatment to a mean of 1.67° (0° to 5°) and 7.0° (4° to 14°) after treatment, respectively. According to the modified Lee scoring system, six patients had excellent results, two had good results and four had fair results, and using the Cheng and Tang score, eight patients had excellent results and four had good results. Surgical management of adult patients with neglected congenital muscular torticollis using a modified Ferkel’s bipolar release gives excellent results. The range of neck movement and head tilt improved in all 12 patients and cosmesis improved in 11, despite the long-standing nature of the deformity