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

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

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

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

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

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

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

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

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

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

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

We studied the effect of trochanteric osteotomy in 192 total hip replacements in 140 patients with congenital hip disease. There was bony union in 158 hips (82%), fibrous union in 29 (15%) and nonunion in five (3%). The rate of union had a statistically significant relationship with the position of reattachment of the trochanter, which depended greatly on the pre-operative diagnosis. The pre-operative Trendelenburg gait substantially improved in all three disease types (dysplasia, low and high dislocation) and all four categories of reattachment position. A persistent Trendelenburg gait post-operatively was noticed mostly in patients with defective union (fibrous or nonunion). Acetabular and femoral loosening had a statistically significant relationship with defective union and the position of reattachment of the trochanter. These results suggest that the complications of trochanteric osteotomy in total hip replacement for patients with congenital hip disease are less important than the benefits of this surgical approach

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

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

A combined anterior and posterior surgical approach is generally recommended in the treatment of severe congenital kyphosis, despite the fact that the anterior vascular supply of the spine and viscera are at risk during exposure. The aim of this study was to determine whether the surgical treatment of severe congenital thoracolumbar kyphosis through a single posterior approach is feasible, safe and effective. We reviewed the records of ten patients with a mean age of 11.1 years (5.4 to 14.1) who underwent surgery either by pedicle subtraction osteotomy or by vertebral column resection with instrumented fusion through a single posterior approach. The mean kyphotic deformity improved from 59.9° (45° to 110°) pre-operatively to 17.5° (3° to 40°) at a mean follow-up of 47.0 months (29 to 85). Spinal cord monitoring was used in all patients and there were no complications during surgery. These promising results indicate the possible advantages of the described technique over the established procedures. We believe that surgery should be performed in case of documented progression and before structural secondary curves develop. Our current strategy after documented progression is to recommend surgery at the age of five years and when 90% of the diameter of the spinal canal has already developed. Cite this article: Bone Joint J 2013;95-B:1527–32

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

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

We report the case of a 24-year-old man with a congenital meniscoid articular disc of the triangular fibrocartilage complex with extensor carpi ulnaris tenosynovitis. His young age, the normal articular cartilage, the lack of degenerative changes at the margins of the defect and its bilateral occurrence made this diagnosis likely. A congenital defect of the articular disc of the triangular fibrocartilage complex should not be misinterpreted as a traumatic rupture and is usually asymptomatic

1. One hundred cases of torticollis and 117 cases of sternomastoid tumour have been reviewed. 2. Congenital torticollis can be sub-divided into two groups: postural and muscular. 3. Congenital postural torticollis is present at birth; it is not associated with a sternomastoid tumour; it is transient in nature; and it does not require operation for its relief. 4. Congenital muscular torticollis is preceded by a sternomastoid tumour which is clinically evident in one-fifth of all cases. 5. The ischaemic theory of the causation of sternomastoid tumours is not supported by recent histological investigations. Some other cause, which probably is operative before birth, must be sought. 6. Four-fifths of all cases of sternomastoid tumours resolve spontaneously and leave no deformity. Excision of the tumour in infancy is therefore unjustifiable. 7. Open division of the muscle and of the cervical fascia in congenital muscular torticollis cures the deformity but leaves an unsightly scar. 8. Subcutaneous tenotomy can be relied upon to cure the deformity if post-operative treatment is carried out skilfully and assiduously over a prolonged period. 9. If complete correction is not gained at the time of subcutaneous tenotomy a better result can be assured by open division of the upper end of the muscle through an incision within the hair line

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

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

Difficulties posed in managing developmental dysplasia of the hip diagnosed late include a high-placed femoral head, contracted soft tissues and a dysplastic acetabulum. A combination of open reduction with femoral shortening of untreated congenital dislocations is a well-established practice. Femoral shortening prevents excessive pressure on the located femoral head which can cause avascular necrosis. Instability due to a coexisting dysplastic shallow acetabulum is common, and so a pelvic osteotomy is performed to achieve a stable and concentric hip reduction. We retrospectively reviewed 15 patients (18 hips) presenting with developmental dysplasia of the hip aged four years and above who were treated by a one-stage combined procedure performed by the senior author. The mean age at operation was five years and nine months (4 years to 11 years). The mean follow-up was six years ten months (2 years and 8 months to 8 years and 8 months). All patients were followed up clinically and radiologically in accordance with McKay’s criteria and the modified Severin classification. According to the McKay criteria, 12 hips were rated excellent and six were good. All but one had a full range of movement. Eight had a limb-length discrepancy of about 1 cm. All were Trendelenburg negative. The modified Severin classification demonstrated four hips of grade IA, six of grade IB, and eight of grade II. One patient had avascular necrosis and one an early subluxation requiring revision. One-stage correction of congenital dislocation of the hip in an older child is a safe and effective treatment with good results in the short to medium term

This paper reviews the current knowledge relating to the management of adult patients with congenital hip disease. Orthopaedic surgeons who treat these patients with a total hip replacement should be familiar with the arguments concerning its terminology, be able to recognise the different anatomical abnormalities and to undertake thorough pre-operative planning in order to replace the hip using an appropriate surgical technique and the correct implants and be able to anticipate the clinical outcome and the complications

Purpose: Congenital idiopathic clubfoot is the most common musculoskeletal birth defect developing during the fetal period, but with no known etiology. MYH 2, 3, 7, and 8 are expressed embryonically or perinatally, the period during which congenital idiopathic clubfoot develops; are all components of Type II muscle, which is consistently decreased in clubfoot patients; and are associated with several muscle contracture syndromes that have associated clubfoot deformities. In this study, we hypothesized that mutations in embryonic and perinatal myosin genes could be associated with congenital idiopathic clubfoot. Methods: We screened the exons, splice sites, and predicted promoters of 24 bilateral congenital idiopathic clubfoot patients and 24 matched controls in MYH 1, 2, 3, and 8 via sequence-based analysis, and screened an additional 76 patients in each discovered SNP. Results: While many SNPs were found, none proved to be significantly associated with the phenotype of congenital idiopathic clubfoot. Also, no known mutations that cause distal arthrogryposis syndromes were found in the congenital idiopathic clubfoot patients. Conclusion: These findings demonstrate that congenital idiopathic clubfoot has a different pathophysiology than the clubfoot seen in distal arthrogryposis syndromes, and defects in myosin are most likely not directly responsible for the development of congenital clubfoot. Given the complexity of early myogenesis, many regulatory candidate genes remain that could cause defects in the hypaxial musculature that is invariably observed in congenital idiopathic clubfoot. Significance: This study further differentiates congenital idiopathic clubfoot as distinct from other complex genetic syndromes that can present with similar deformities, and thus facilitates further research to improve the clinical diagnosis and treatment of congenital idiopathic clubfoot

Study Design: Retrospective study with clinical and radiological evaluation of 15 patients with congenital kyphosis or kyphoscoliosis who underwent anterior instrumented spinal fusion for posterolateral or posterior hemivertebae (HV). Objective: To evaluate the safety and efficacy of early surgical anterior instrumented fusion with partial preservation of the HV in the treatment of progressive congenital kyphosis in children below the age of 3. We discuss the management of patients presenting with neurological compromise. Summary of background data: A variety of treatments have been described in the literature for the treatment of congenital kyphosis due to HV. We report the results of our technique. Materials and Methods: Between 1997 and 2005 we have treated 15 consecutive patients with progressive congenital kyphosis with anterior instrumented fusion and strut grafting. 13 patients had a single posterolateral HV and 2 patients a single posterior HV. Of the 15 patients in the study, 5 were girls and 10 boys. Mean age at surgery was 22 months (range 8–33). Mean follow-up period was 6.8 years. 13 HV were located in the thoracolumbar junction (T10-L2) and 2 in the thoracic spine. Results: The average operating time was procedure was 150 minutes (range, 130 to 210 minutes). The average blood loss 180 mL (range, 100 to 330 mL), equivalent to a mean external blood volume loss of 15% (range, 11 to 24%). Preoperative segmental Cobb angle averaging 34 º at last follow up. Compensatory coronal cranial and caudal curves corrected by 50%. The angle of segmental kyphosis averaged 39º (range, 20º to 80º) before surgery and 21 º (range, 11º to 40º) at last follow up. This represents a 43% of improvement of the segmental kyphosis, and a 64% of improvement of the segmental scoliosis at last followup. One case with initial kyphosis of 80 º continued to progress and required revision anterior and posterior surgery. There were no neurologic complications. Key points:. In progressive congenital kyphoisis, early diagnosis and aggressive surgical treatment are mandatory for a successful result. Early treatment minimizes the risks of surgery. Anatomical and physiological pitfalls in the treatment of congenital kyphosis are discussed. Anterior instrumented fusion of congenital kyphosis provides sagital and coronal correction in very young children with low risk of complications

Ninety-seven limbs, in eighty-one patients, with a diagnosis of congenital deficiency of the fibula have been reviewed. A classification was devised to distinguish the minimal hypoplasia of the fibula (Type I) from the well-known complete absence (Type II). Congenital anomalies of the femur were present in 76 per cent of patients with Type I deficiency and in 59 per cent with Type II. The shortening of the limb was by 13 per cent in Type I and by 19 per cent in Type II, and the percentage shortening was fairly constant during growth. A detailed description of the spectrum of other congenital anomalies was found to be characteristic: for example, the ball and socket formation of the ankle, tarsal coalition and anomalies of the foot. The treatment aimed simply to equalise leg length in Type I deficiency, while amputation of the foot and the fitting of a prosthesis were necessary in Type II to obtain satisfactory function

Aims: Pathologic studies in foetuses and stillborns with congenital clubfoot have shown atrophy of the musculature of the leg omolateral and incresased fibrous tissues within the muscles belly. Both the triceps surae and the tibialis posterior are mostly involved and their tendons thickened. Atrophy of the musculature of the leg has been described in various clinical studies on congenital clubfoot, but most of the authors believe that atrophy might be secondary to surgical treatment and prolonged immobilization in plaster cast and brace. In our study, we correlated the pathology of foetal leg muscle atrophy with leg muscle atrophy shown by patients with congenital clubfoot. Methods: We investigated the MRI aspects of leg muscles in untreated babies and in children and adults who had been treated soon after birth for unilateral congenital clubfooft. The MRI aspects of the leg muscle in treated patients were compared to those of untreated babies, and to the histopathologic findings of the same muscles in foetuses with congenital clubfoot. Results: The ratio between the muscles of the normal leg and the leg of the clubfoot side was almost the same as measured either on the histological sections of foetuses with congenital clubfoof or in patients before and after treatment, from birth to adulthood. Conclusions: Our study shows that in congenital club-foot leg muscle atrophy is a primitive pathologic finding rather than secondary to treatment

Untill recently, major reduction defects of the tibia were treated by amputation and prosthetic fitting. However, Wada et al (1) and Weber (2) recently reported impressive results of limb reconstruction in children with tibial aplasia. If an attempt is being made to reconstruct the leg and foot, a clear understanding of the nature of anomalies is necessary. A retrospective study of case records and radiographs of children with congenital anomalies of the tibia seen at our centre was undertaken to determine the patterns of associated anomalies in the leg and foot. In addition, five amputated specimens of the leg and foot from children with complete tibial aplasia were dissected. A wide spectrum of congenital anomalies of the tibia was seen and this included complete aplasia, partial aplasia, hypoplasia, dyplastic trapezoidal tibia and congenital bowing. Complete and partial aplasia was seen either with or without duplication of the formed skeletal elements. The patterns of duplication that were seen included fibular dimelia, pre-axial mirror polydactyly, duplication of the calcaneum, cuboid and lateral cuneiforms. Trapezoidal dysplastic tibia was associated with duplication of the talus and pre-axial mirror polydactyly. Dissection of the amputated specimens of complete tibial aplasia revealed aplasia of some muscles, aberratant tendinous structures, abnormal insertion of muscles and absence of the plantar arterial arch. An understanding the nature of these associated anomalies in children with tibial aplasia and dyplasia will help the surgeon to decide the strategies for reconstruction of the limb if that is the desired option. At the other end of the spectrum of congenital anomalies of the tibia is posteromedial bowing which was considered an innocuous condition that required little or no treatment. A review of 20 cases of posteromedial bowing demonstrated that there are number of problems related to the leg, ankle and foot that may require surgical intervention

Aims

Morphological abnormalities are present in patients with developmental dysplasia of the hip (DDH). We studied and compared the pelvic anatomy and morphology between the affected hemipelvis with the unaffected side in patients with unilateral Crowe type IV DDH using 3D imaging and analysis.

Aims. To our knowledge, no study has compared the long-term results of cemented and hybrid total hip arthroplasty (THA) in patients with osteoarthritis (OA) secondary to congenital hip disease (CHD). This is a demanding procedure that may require special techniques and implants. Our aim was to compare the long-term outcome of cemented low-friction arthroplasty (LFA) and hybrid THA performed by one surgeon. Patients and Methods. Between January 1989 and December 1997, 58 hips (44 patients; one man, 43 woman; mean age 56.6 years (25 to 77)) with OA secondary to CHD were treated with a cemented Charnley LFA (group A), and 55 hips (39 patients; two men, 37 women; mean age 49.1 years (27 to 70)) were treated with a hybrid THA (group B), by the senior author (GH). The clinical outcome and survivorship were compared. Results. At all timepoints, group A hips had slightly better survivorship than those in group B without a statistically significant difference, except for the 24-year survival of acetabular components with revision for aseptic loosening as the endpoint, which was slightly worse. The survivorship was only significantly better in group A compared with group B when considering reoperation for any indication as the endpoint, 15 years postoperatively (74% vs 52%, p = 0.018). Conclusion. We concluded that there was not a substantial difference at almost any time in the outcome of cemented Charnley LFAs compared with hybrid THAs when treating patients with OA of the hip secondary to CHD. We believe, however, that after improvements in the design of components used in hybrid THA, this could be the method of choice, as it is technically easier with a shorter operating time. Cite this article: Bone Joint J 2019;101-B:1050–1057

We have evaluated the results of total hip replacement in patients with congenital hip disease using 46 cemented all-polyethylene Charnley acetabular components implanted with the cotyloplasty technique in 34 patients (group A), and compared them with 47 metal-backed cementless acetabular components implanted without bone grafting in 33 patients (group B). Patients in group A were treated between 1988 and 1993 and those in group B between 1990 and 1995. The mean follow-up for group A was 16.6 years (12 to 18) and the mean follow-up for group B was 13.4 years (10 to 16). Revision for aseptic loosening was undertaken in 15 hips (32.6%) in group A and in four hips (8.5%) in group B. When liner exchange was included, a total of 13 hips were revised in group B (27.7%). The mean polyethylene wear was 0.11 mm/yr (0.002 to 0.43) and 0.107 mm/yr (0 to 0.62) for groups A and B, respectively. Polyethylene wear in group A was associated with linear osteolysis, and in group B with expansile osteolysis. In patients with congenital hip disease, when 80% cover of the implant can be obtained, a cementless acetabular component appears to be acceptable and provides durable fixation. However, because of the type of osteolysis arising with these devices, early exchange of a worn liner is recommended before extensive bone loss makes revision surgery more complicated

1. Congenital dislocation of the hip in identical twins is reported. 2. The heredity of congenital dislocation of hip is discussed. Studies in twins show that congenital dislocation of hip is probably a hereditary dysplasia of the acetabulum and upper end of the femur, and that external factors play a less important role

1. General joint laxity affecting more than three joints was found in 7 per cent of normal schoolchildren. Similar laxity was found in fourteen of a random series of forty-eight girls, and in nineteen of twenty-six boys, with non-familial congenital dislocation of the hip. Such laxity was also found in four of seven girls and five of seven boys with familial (first degree relative affected) congenital dislocation of the hip. 2. It is concluded that persistent generalised joint laxity, which is often familial, is an important predisposing factor to congenital dislocation of the hip in boys. It is less important in girls, except perhaps in familial cases, as in girls there is an alternative temporary hormonal cause of joint laxity

Congenital vertical talus is a rare deformity. Many different surgical procedures have been described, and there is debate about whether the correction should be done in one or two stages. We review the results of single stage surgical correction of congenital vertical talus. Between 1992 and 2000, five boys and seven girls were treated, ranging in age from eight months to two years. In six children both feet were involved, so there was a total of 18 feet. One child had spina bifida, four had arthrogryposis multiplex congenita and three had syndromes and chromosomal abnormalities. Four cases were idiopathic. Dorsolateral and medial incisions were used. Through the dorsolateral the sinus tarsus, calcaneocuboid and talonavicular joints were released and the extensors lengthened. Through the medial incision the navicula was reduced onto the talus, the tibialis posterior and talonavicular capsule were reefed and the tendo Achillis lengthened. The talonavicular and calcaneocuboid joints were pinned. The tibialis anterior was re-routed through the talar neck. Plasters were changed after two weeks and serial plasters were applied for four to six months. Follow-up ranged from one to seven years. Results were assessed clinically and radiologically, using the Adelaar 10 point scoring system. There were no wound complications or cases of avascular necrosis of the talus. Further surgery was required to correct cavus in two feet, to correct forefoot abduction in two, and to correct hindfoot valgus in one. Results were rated good in 12 feet and fair in six. Radiologically there was notable improvement in the anteroposterior and lateral talocalcaneal and tarso-first metatarsal angles. All patients were ambulant at last follow-up. In treating congenital vertical talus, good clinical and radiological results can be obtained with single stage correction of the hindfoot and midfoot deformities

Sixty-nine patients with congenital abnormality of the femur were reviewed. Their manifestation of femoral dysplasia ranged from an intact femur approximately 60 per cent of the length of the normal leg to a subtotal absence of the femur in which only the femoral condyles remained, often with a congenital fusion of the knee joint. Two groups were defined: Group I consisted of those with congenital hypoplastic femur in which the hip and knee could be made functional and where, in some patients at least, leg equalisation was possible; Group II consisted of those with true proximal focal femoral deficiency where the hip joint was never normal and the knee joint was always useless. The patients in each group were examined and evaluated with respect to clinical signs, surgical procedures performed, and prosthetic requirements and function. A protocol of treatment for both groups is suggested

Objective: To assess the clinical and radiological outcome of isolated hemivertebrae and multiple vertebral anomalies in the very young. Design: A cohort of patients with congenital scoliosis were identified on antenatal ultrasound and followed prospectively from twenty weeks in-utero (IU) for a mean of two years (range IU – five years). Subjects: Twenty fetuses with congenital hemivertebrae were identified from 16, 000 routine antenatal scans over a five-year period. Each was X-rayed at six weeks and investigated for renal and cardiac abnormalities. Outcome Measures: Clinical assessment included trunk balance, neurological abnormality and associated congenital abnormalities. Radiological assessment of curve magnitude and curve progression was performed using the Cobb method. The potential for progression (based on site, degree of incarceration, growth potential and presence of a congenital bar) was assessed. In all cases a clinical and radiological assessment was made at maximal follow-up. Results: One of the twenty fetuses aborted spontaneously at 23 weeks, three remain in-utero, leaving 16 live births. Thirteen of 20 had an isolated hemivertebra, and seven of twenty had multiple vertebral abnormalities. Two fetuses had renal agenesis, two had VATER’s syndrome and one had rib and abdominal wall abnormalities. The mean antenatal Cobb angle was 30°. IU curve progression was noted in three. Seven of the 16 live births required surgery based on curve magnitude, curve progression, truncal imbalance and the potential for progression. Surgery included anterior and posterior convex hemi-epiphyseodesis in two, and hemivertebra excision with fusion in five. The mean pre-operative Cobb angle was 36° (range 25–42) reducing to a mean of 21° (range 0–45) at maximal follow-up. Conclusions: Multiple vertebral abnormalities were more commonly associated with renal and cardiac abnormalities. If associated with the oligohyramnios sequence the fetus appeared to be at high risk. In general US detected isolated fetal hemivertebrae carry a good prognosis, nevertheless 38% of these cases at our institution underwent surgery within the first two years of life

1 . The magnitude of the problem of congenital anomalies becomes evident when one takes into consideration the fact that they cause the death of approximately one quarter of the human race either before or shortly after birth, and handicap an appreciable proportion of the survivors throughout their lives. Further, a significant percentage of infants judged to be normal at birth are found in later life to suffer from "disguised" anomalies of the skeleton and soft tissues. Though the study of genetic factors leading to congenital defects has attracted a great deal of attention during the last few decades, the importance of environmental causes of human malformations has received relatively less emphasis. The association of congenital anomalies such as cataract and cardiac septal defects with maternal intercurrent infection of rubella during the early months of pregnancy demonstrates clearly that changes in the germplasm cannot always be invoked as the cause of developmental abnormalities. Congenital malformations that are sometimes genetically determined, such as microphthalmos, cleft palate, and certain skeletal abnormalities, can be caused in the offspring not only by maternal nutritional deficiencies and x-radiation but also, at least in some animals, such as chickens, rats and rabbits, by the introduction of certain substances like insulin into the environment of the embryo during its development. 2. Since very little is known of the detailed histology of the early human embryo, the histological examination of cases of perverted growth is mainly limited to aborted foetuses which, unfortunately, tend to present varying degrees of post-mortem degeneration before accurate histological methods can be applied. It is exactly in this field that animal experiments can offer valuable help. According to Mall and other embryologists the pathological changes that take place in human foetuses and those obtained experimentally in animals are not merely "analogous or similar but identical.". 3. An attempt has been made to review, in some detail, the more important work which has been carried out on experimental teratogenesis, on the epidemiological implications of developmental arrests in humans, and on foetal abnormalities associated with maternal metabolic and hormonal disorders during pregnancy. 4. The technique employed for injection of insulin into the egg yolk has been described. Methods used for the estimation of blood sugar in chick embryos at various stages after injection of insulin and special histochemical techniques for localising polysaccharides in cartilage have been outlined. 5. A few salient experimental results have been tabulated, and some of the insulin-induced abnormalities have been illustrated. 6. The possible mechanism of action of insulin in the causation of the various developmental anomalies has been discussed. Broadly speaking, insulin seems to affect primarily the part or tissue which is in the most active stage of growth or differentiation at the time of the injection. Within the range of 0·05 to 6 units of insulin employed, the incidence, severity and distribution of the deformities appear to increase with the dose of the hormone. It has been observed that the hypoglycaemia caused by insulin injection is not counteracted till about the twelfth day of incubation, presumably because of excessive accumulation of glycogen in the yolk-sac membrane immediately after the injection, and because of lack of glycogen storage in the embryonic liver and the absence of active secretion in the endocrine glands concerned with the carbohydrate metabolism of the embryo. It has been suggested that this unchecked hypoglycaemia may deprive the mesenchyme, pre-cartilage and cartilage of glycogen and mucopolysaccharides (chondroiten-sulphuric acid complexes), depending on the time of injection and the dose of insulin, and thus not only give rise to a variety of single and multiple deformities in the cartilaginous skeleton but also interfere with the normal endochondral ossification, resulting in a generalised developmental disturbance of bone resembling osteogenesis imperfecta in the human. 7. Insulin-induced abnormalities can be prevented to a remarkable extent by injecting nicotinamide and riboflavin into eggs along with insulin. 8. The question of the practical application of the knowledge gained from experimental observations on insulin-induced developmental abnormalities in explaining the possible causation of congenital anomalies in humans by genetic and environmental teratogenic factors, has been discussed. It is suggested that the orderly progression from the mesenchymatous condensation to cartilage, and then through calcified cartilage to bone, may be disturbed by these teratogenic factors at critical phases during the development of the embryo, and a variety of single and multiple skeletal deformities may thus be induced. 9. A plea is made for routine pathological and radiological examination of aborted foetuses and stillborn infants more or less on the lines followed for experimentally induced deformities with a view to applying the knowledge gained from animal experiments to a better understanding of the etiology and pathology of human congenital anomalies. 10. As regards the possible prevention of these deformities, it is not always easy to offer sound eugenic advice in the cases of congenital malformations determined partly or completely by genetic factors, for two important reasons. First, it is often difficult to distinguish between genetically determined congenital anomalies and their phenocopies. Secondly, genetically determined developmental defects sometimes show surprisingly variable expressivity and penetrance. For the conditions in which both genetic and environmental factors are involved, the most profitable immediate line of attack would be on the environmental factors. A relatively simpler problem is presented by the malformations which are, for all practical purposes, entirely caused by environmental factors. Measures to prevent congenital anomalies caused by prenatal rubella, such as exposure of girls to the disease during childhood and protection of pregnant women during the early stages of pregnancy by immune serum, are under active consideration. 11 . Further energetic investigation of the causes of permaturity, stillbirths, monstrosities and congenital malformations is urgently needed, before embarking on a successful programme for prevention. "The day of successful prophylaxis is not yet, but it is much nearer than seemed possible a few years ago."

1. Eighty infants with congenital dysplasia of the hip, diagnosed before walking began, are reviewed. 2. The clinical and radiographic features of congenital dysplasia are described. 3. A dysplastic hip may either become normal spontaneously, or it may deteriorate to the state of true dislocation. 4. Treatment is discussed

Background: The treatment of congenital vertical talus has traditionally consisted of manipulation and application of casts followed by extensive soft-tissue releases. However, this treatment is often followed by severe stiffness of the foot and other complications. The purpose of this study was to evaluate a new method of manipulation and cast immobilization, based on principles used by Dobbs et al in patients with idiopathic congenital vertical talus, but applied in teratologic congenital vertical talus. Methods: Five consecutive cases of teratologic congenital vertical talus deformity were prospectively followed at a minimum of nine months post treatment with serial casting and limited surgery consisting of percutaneous Achilles tenotomy, fractional lengthening of the anterior tibial tendon, and percutaneous pin fixation of the talonavicular joint. The principles of manipulation and application of the plaster casts were similar to those used by Ponseti to correct a clubfoot deformity, but the forces were applied in the opposite direction. Clinical and radiographic assessments were carried out at the initial, immediate postoperative and the latest follow-up. Results: Initial correction was obtained both clinically and radiographically in all five feet. A mean of eight casts was required for correction. At the final evaluation, the mean ankle dorsiflexion was 20° and the mean plantar flexion was 31°. Radiographically, dorsal subluxation of the navicular recurred in one patient, but was functionally insignificant as the patient was a non-ambulator and required treatment for seating purposes only. At the time of the latest follow-up, there was a significant improvement in all of the measured radiographic parameters compared with the pretreatment values. Conclusions: Serial manipulation and cast immobilization followed by talonavicular pin fixation and percutaneous tenotomy of the Achilles tendon provides excellent results, in terms of the clinical appearance of the foot, and deformity correction, in patients with teratologic congenital vertical talus

1. The clinical and radiological features of thirty-two feet with congenital vertical talus are described and subdivided into groups determined by the presence or absence of associated abnormalities. 2. The differential diagnosis of congenital vertical talus from flat foot, talipes calcaneus and uncorrected club foot is discussed. 3. No benefit came from either non-operative treatment or tenotomy of the tibialis anterior

Malignant hyperthermia (MH) is a pharmacogenetic disorder, potentially lethal, due to the exposure to anesthetic drugs that triggers, a high increase of corporal temperature, progressive muscular stiffness, severe rabdomiolisis and death due to cardiac dysfunction. Many research works relate Malignant Hyperthermia to muscular illnesses or to the King Syndrome. Through this study we present the incidence of MH in patients with congenital vertebrae malformations. (CVM). The objective is to establish the incidence of the MH in patients who were operated on CVM and to alert about this association. 1029 patients with CVM were treated between 1972 and 2000. 390 with congenital vertebrae malformation were operated on. 3 patients (0.76%) (1 girl and 2 boys) developed MH while they underwent surgical treatment for the CVM. 1 patient presented an isolated congenital vertebrae malformation. 1 patient presented King Syndrome and the other presented Robert Syndrome. Only 1 elevated amount of preoperative CPK was found (the are no reports on the others). No muscular biopsy was done to test sensitivity. Two of them were biopsied for a post episode study. At the surgical moment, any patients reported personal or familiar antecedents of MH. No deaths were reported, although it is considered as a potentially lethal disorder. We found no reports in the literature in this subject. Most of the bibliographic data belonged to anesthesiologists or geneticists. Our approach as spine surgeons leaded us to the detailed analysis of this studies and the 0.76% (3 out of 390) incidence suggested us to have an alert attitude when facing patients with surgical MVC and take the necessary precautions

1. An unusual congenital anomaly of the cervical spine is described. This lesion caused a localised cervical kyphosis and resulted in the development of a mild tetraparesis. 2. The case reported is believed to be the first on record in the English literature of multiple posterior hemivertebrae in the cervical region. 3. The neck deformity was associated with an unusual combination of developmental anomalies–namely, brachyphalangy and bilateral congenital optic atrophy. 4. The importance of differentiating between congenital and acquired causes of kyphosis is emphasised. 5. The radiographic appearances of posterior hemivertebra are described, and the differential diagnosis is considered. 6. The development of the vertebral body, and the relationship between coronal cleft vertebra and posterior hemivertebra, are discussed. The possible role of a disturbance of vascular supply in pathogenesis is mentioned. 7. This report augments the growing literature on congenital skeletal anomalies occurring in combination with isolated congenital ocular defects

In five children, six forearms with a fixed pronation deformity secondary to congenital radioulnar synostosis were treated by a derotation osteotomy of the distal radius and the midshaft of the ulna. There were three boys and two girls with a mean age of 4.9 years (3.5 to 8.25) who were followed up for a mean of 29 months (18 to 43). The position of the forearm was improved from a mean pronation deformity of 68° (40° to 80°) to a pre-planned position of 10° of supination in all cases. Bony union was achieved by 6.3 weeks with no loss of correction. There was one major complication involving a distal radial osteotomy which required exploration for a possible compartment syndrome

Introduction: The knee joint in congenital longitudinal deformities of the lower extremity shows a large variety of pathological findings. Valgus deformity is found in most cases and is described as being juxta- articular. To describe the true anatomic pathology we performed a radiographic analysis of the knee joint in congenital longitudinal deformities. Patients and Method: Between 1985 and 2001 we treated 102 patients presenting with congenital longitudinal deformities. Inclusion criteria for this study were diagnoses of fibular hemimelia (FBH) and/or congenital femoral deficiency (CFD), an age between 5 and 16 years, unilateral affection and availability of long standing X-rays, whereas bilateral affection or previous operations on the lower extremities were defined as exclusion criteria. Twenty-four parameters were defined on the femur and tibia respectively and a nomenclature was created. The mean values including standard deviation were calculated and we statistically compared the parameters of the affected to those of the non-affected knee. Furthermore, MRI scans of the knee joint of 20 of these patients were evaluated. Results: Thirty- nine patients (19 female, 20 male) met the inclusion criteria. The average age at the time of evaluation was 8.87 years (3.1 SD). A combined deficiency of femur and tibia was found in 35 patients. The predominant diagnosis was CFD in 13, fibular hemime-lia in 13 and fibular aplasia in 9 cases. The anatomic lateral distal femoral angle (ALDFA) measured 75.4° (2.5 SD) on the affected, and 81.6° (1.6 SD) on the non-affected knee. The lateral distal femoral metaphyseal angle of the affected side and of the non-affected side showed no significant difference. The distal lateral femoral epiphyseal width (DLFEW) was decreased in the affected limb compared to the non affected limb, whereas the distal medial femoral epiphyseal width (DMFEW) of the affected and non-affected side showed only a minor difference. In the tibia we found no significant difference between the variables for the medial proximal tibial angle (MPTA) and for the medial proximal tibial metaphyseal angle (MPTMA) of the affected and the non-affected limb. A significant difference was found between the proximal lateral tibial epiphyseal width of the affected and the non-affected side. Analysis of the MRI scans revealed aplasia of the anterior cruciate ligament in 18 cases and aplasia of the posterior cruciate ligament in 8 of the 20 cases. The defect of ossification of the lateral tibial epiphysis as seen in plain X-rays is visible in the MRI scans as cartilage anlage. (Only the most important findings are summarized). Conclusions: In our patient population only four patients had FBH or CFD but 35 cases presented combined defects; we assume that the femur is affected to some extent in almost all cases of FBH. The hypoplasia was only found in the lateral aspects of femur and tibia and was primarily located within the femoral epiphysis. The metaphysis was not or only minimally affected in the evaluated longitudinal deficiencies. Awareness of sagittal instability, due to ACL and/or PCL aplasia, is necessary to avoid subluxation or dislocation when lengthening procedures are performed

Purpose. This study compares outcomes in patients with complete congenital fibula absence treated with an amputation protocol to those using an extension prosthesis. Introduction. Complete fibula absence presents with significant lower limb deformity. Parental counselling regarding management is paramount in achieving the optimum functional outcome. Amputation offers a single surgical event with minimal complications and potential excellent functional outcome. Method. 32 patients were identified. 9 patients (2M: 7F, median age at presentation of 22yrs) utilized an extension prosthesis. 23 patients (16M: 7F, median age at presentation of 10 months) underwent 25 amputations during childhood: only two underwent tibial kyphus correction. Mobility was assessed using SIGAM and K scores. Quality of life was assessed using the PedsQL inventory questionnaire; pain by a verbal severity score. Results. 19 Syme and one Boyd amputation in 19 patients were performed early (mean age 15 months). 4 Syme and one trans-tibial amputation in 4 patients took place in older children (mean age 6.6 years). K Scores were significantly higher (mean 4 versus 2) and pain scores lower in the amputation group allowing high impact activity compared to community ambulation with an extension prosthesis. The SIGAM and PedsQL scores were all better in the amputation group, but not significantly so. Conclusion. Childhood amputation for severe limb length inequality and foot deformity in congenital fibula absence offers excellent short term functional outcome with prosthetic support. The tibial kyphus does not need routine correction and facilitates prosthetic suspension. Accommodative extension prostheses offer reasonable long term function but outcome scores are lower

1. Four cases of true congenital vertical talus are described; in three of the four cases there were other major deformities of the skeleton. All were treated by open operation; the operation sacrificed part of the substance of the navicular bone, which was placed between the forepart of the calcaneus and the head of the talus. 2. The results five to ten years after operation show that stable reduction was maintained without any further treatment. They suggest, however, that more of the navicular bone could have been removed or that the whole navicular might be excised, at least in the more severe deformities. 3. Congenital vertical talus resembles club foot (equino-cavo-varus) in that difficulty in reduction and in maintenance of the reduction results from the tension in the medial pillar of the foot. Easing of the tension can result in recurrence of the dislocation or, alternatively, a reversal of the deformity

We describe a successful modified operative procedure at an average 19 months follow-up in 3 patients with congenital dislocation of the patella and compare its merits to the other procedures already reported in the literature. Congenital dislocation of the patella may be associated with other congenital conditions or syndromes i.e. Down’s syndrome, congenital vertical talus and cerebral palsy. Numerous operative techniques have been described in the literature which may be divided into 3 basic groups. A modification of the Langenskiold & Ritsila procedure is described. The original procedure describes an extensive lateral release with detachment and medial transfer of the patellar tendon through a curved incision. The tendon is routed through a fold of synovium medially and fixed distally to bone with sutures through drill holes in the proximal tibia. We found at surgery this synovium was too fragile to hold the transferred tendon and the use of drill holes unnecessary. The main alterations include a limited and straight anterior skin incision, a fashioning of a ‘buckle’ of the transferred distal patellar tendon to a distally based flap which avoids drill holes in the growing bone. This modification of the Langenskiold procedure was used successfully in 3 cases, including a revision of a failed Goldthwaite- Roux procedure in a mentally handicapped child. The results at average 19 months follow-up are successful. The early results confirm that the patella remains located and tracks normally. The valgus and flexion deformities have significantly improved. This modification of the Langenskiold & Ritsila procedure requires less dissection than other operations, with no bony surgery and a cosmetic scar. The Langen-skiold & Ritsila procedure has been successful and we feel that this modification simplifies and improves on the original technique

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

Introduction: Congenital kyphosis is an uncommon deformity caused by failure of the vertebral bodies to form and/or segment. It is treated surgically. Our purpose is to confirm whether the treatment protocols established years ago are still valid and to assess the benefits of improved implants. Materials and methods: Between 1985 and 2003, 24 patients underwent surgery in La Paz Hospital. They were classified into three groups: < 5 years (8 p), 6–12 (6 p) and > 13 (10 p). The minimum follow-up was 2 years (2–14). The procedures were posterior or circumferential spinal fusion, with or without instrumentation, and corrective osteotomy. Complications were evaluated radiographically. Results: In the first group posterior spinal fusion was performed in six patients and circumferential in two, with a correction rate of 55%. In the second group instrumented circumferential spinal fusion was performed in three cases and instrumented posterior in three (30% correction). In the third group eight of the ten patients underwent instrumented anteroposterior spinal fusion (osteotomy in five) and the correction rate was 45%. Complications: 3 pseudoarthrosis, 1 DVT, 2 infections and 3 failed implants. Conclusions: Congenital kyphosis can be controlled at any age, although early surgery is best. Gradual correction occurs after poster spinal fusion in children and instrumentation prevents revision of the fusion and prolonged immobilisation. Persons over the age of 5 with type I kyphosis usually require circumferential spinal fusion

A congenital, unilateral, fixed flexion deformity in a neonate was diagnosed as a congenital absence of the knee. A single cartilage mass, with fusion of the lower femoral and upper tibial ossification centres, was demonstrated by imaging studies. This condition has been reported in the literature only once before. Surgery on our patient, which was performed at the age of two years, consisted of separation of the fused cartilaginous anlage and gradual correction of the deformity using an Ilizarov frame

Introduction and purpose: The relationship between congenital heart disease and the increased prevalence of scoliosis is well known, although the same cannot be said about the etiology of scoliosis in these patients. Although thoracotomy is often associated to scoliosis, median sternotomy has so far not been identified as an etiological agent. he purpose of the study is to determine if patients with congenital heart disease who are subjected to a median sternotomy show a higher prevalence of spine deformities. Materials and methods: A retrospective review is made of patients operated on for congenital heart disease through median sternotomy before the age of 8, assessing the development of spine deformities. Simple chest radiographs of 128 patients were studied once they reached skeletal maturity and it was observed that they presented no spinal or costal deformities before surgery. Results: The prevalence of scoliosis was 34.3%; 16 of these patients (12.5%) had curves of more than 20° and 33 (25.8%) had thoracic kyphosis of less than 20°. Patients operated on before the age of 18 months had a significantly higher risk to develop scoliosis as compared with those treated later (odds ratio: 3.48; p=0.016). The development of scoliosis was not related with the type of cardiac malformation present. Conclusions: There is a high prevalence of scoliosis in patients subjected to a median sternotomy for a congenital heart pathology. The prevalence of scoliosis increases in patients operated on at younger ages

Introduction: Clubfoot – Congenital talipes equinovarus (CTEV) – is one of the most common congenital conditions requiring orthopaedic surgery. However little is known about the impact on health-related quality of life in these patients. A score on physical- and mental-health is used for this purpose. The aim of the present study was to compare health-related quality of life in CTEV to a background population. Materials and Methods: The Odense based Danish Twin Registry (DTR) is unique as it contains data on all 73,000 twin pairs born in Denmark over the last 130 years. All 46,418 twins born from 1931 through 1982 received an Omnibus questionnaire in the spring of 2002. The incidence of CTEV was self-reported. Included in the questionnaire were questions for The Medical Outcome Study Short Form-12 (SF-12). We calculated SF-12 Physical Component Summary scale (SF-12 PCS) and SF-12 Mental Component Summary scale (SF-12 MCS) using the SF Health Outcomes Scoring Software. 80 reported to have CTEV and the remaining 29,516 were used as controls. Results: 46,418 twins received and 34,944 (75%) returned the questionnaire. 34,485 (99% of the responders) answered the question ‘Were you born with club-foot?’ The sex distribution in these was 15,731 (46%) males and 18,754 (54%) females. The self-reported prevalence of CTEV was 0.0027 (95% confidence interval 0.0022–0.0034). 80 reported to have CTEV and the remaining 29,516 were used as controls. In the CTEV group SF-12 PCSmean was 50.18 (SD 11.19) vs 53.09 (SD 8.11) in the controls. p< 0.0007. In the CTEV group SF-12 MCSmean was 50.58 (SD 10.52) vs 51.78 (SD 8.47) in the controls. NS. Conclusion: The impact of congenital clubfoot on health-related quality of life was significant only on the physical scale. The clubfoot patients scoring lower than the controls. There was no difference in the mental scale between the two groups

Summary of Background Data: The craniofacial malformations described by Goldenhar can be associated with congenital anomalies of the vertebrae. This non-random association of abnormalities represents unilateral errors in the morphogenesis of the spine, as well as the first and second branchial arches. Purpose of the study: The aim of the present study was to determine the prevalence of Goldenhar related conditions in patients with congenital deformities of the spine and to describe the types of vertebral abnormalities and the necessity for treatment. Material-Methods: We performed a retrospective study of 668 consecutive patients with congenital deformities of the spine. The medical records and spinal radiographs were reviewed and patients with a Goldenhar associated condition were identified. The vertebral anomalies causing the spine deformity were detected on antero-posterior and lateral spine radiographs. The type and site of the craniofacial abnormalities, as well as other musculoskeletal deformities and systemic anomalies were recorded. Results: Fourteen patients had Goldenhar associated conditions (7 males and 7 females). A thoracic scoliosis was the most common type of deformity occurring in ten patients (71.5%). Eight of these patients had an isolated hemivertebra and the remaining two had a unilateral unsegmented bar with contralateral hemivertebra at the same level. There was only one patient with a lumbar scoliosis and this was due to a hemivertebra. The side of the vertebral anomaly correlated with that of the hemifacial microsomia in five of the eleven patients who had a scoliosis or kyphoscoliosis. A thoracolumbar kyphosis occurred in four patients; two had posterior hemivertebrae, one had wedge vertebrae, and the remaining patient had an anterior unsegmented bar. A thoracolum-bar kyphoscoliosis occurred in only one patient and was due to a posterolateral quadrant vertebra. Klippel-Feil syndrome occurred in six patients (42.8%). Eight patients (57%) underwent surgical treatment at a mean age of 9.8 years (range: 2.9–19). Four patients had a combined anterior-posterior spine arthrodesis. The remaining four patients had a posterior spinal arthrodesis. Conclusions. The prevalence of Goldenhar associated conditions in patients with congenital deformities of the spine was 2%. Failures of vertebral segmentation were the most frequent abnormality in the cervical spine, whereas failures of vertebral formation most commonly occurred in the thoracic or thoracolumbar spine

There is controversy whether congenital foot abnormalities are true risk factors for pathological dysplasia of the hip. Previous United Kingdom screening guidelines considered congenital talipes equinovarus (CTEV) to be a risk factor for hip dysplasia, but present guidelines do not. We assessed the potential relationship between pathological dysplasia of the hip and fixed idiopathic CTEV. We present a single-centre 21-year prospective longitudinal observational study. All fixed idiopathic CTEV cases were classified (Harrold and Walker Types 1 to 3) and the hips clinically and sonographically assessed. Sonographic Graf Type III, IV and radiological irreducible hip dislocation were considered to be pathological hip dysplasia. Over 21 years there were 139 children with 199 cases of fixed idiopathic CTEV feet. Sonographically, there were 259 normal hips, 18 Graf Type II hips, 1 Graf Type III hip and 0 Graf Type IV hip. There were no cases of radiological or sonographic irreducible hip dislocation. Fixed idiopathic CTEV should not be considered as a significant risk factor for pathological hip dysplasia. This conclusion is in keeping with the current newborn and infant physical examination guidelines in which the only risk factors routinely screened are family history and breech presentation. Our findings suggest CTEV should not be considered a significant risk factor in pathological dysplasia of the hip. Cite this article: Bone Joint J 2014;96-B:1553–5

Children with congenital vertical talus (CVT) have been treated with extensive soft-tissue releases, with a high rate of complications. Recently, reverse Ponseti-type casting followed by percutaneous reduction and fixation has been described, with excellent results in separate cohorts of children with CVT, of either idiopathic or teratological aetiology. There are currently no studies that compare the outcome in these two types. We present a prospective cohort of 13 children (21 feet) with CVT of both idiopathic and teratological aetiology, in which this technique has been used. Clinical, radiological and parent-reported outcomes were obtained at a mean follow-up of 36 months (8 to 57). Six children (nine feet) had associated neuromuscular conditions or syndromes; the condition was idiopathic in seven children (12 feet). Initial correction was achieved in all children, with significant improvement in all radiological parameters. Recurrence was seen in ten feet. Modification of the technique to include limited capsulotomy at the initial operation may reduce the risk of recurrence. The reverse Ponseti-type technique is effective in the initial correction of CVT of both idiopathic and teratological aetiology. Recurrence is a problem in both these groups, with higher rates than first reported in the original paper. However, these rates are less than those reported after open surgical release. Cite this article: Bone Joint J 2014;96-B:274–8

Structural defects of the posterior arch of the atlas are rare, and range from clefts of variable location and size to more extensive defects such as complete agenesis. These abnormalities are usually incidental radiological findings. We present a case of a fracture of the anterior arch of the atlas associated with a congenital abnormality of the posterior arch

The clinical and radiological features of 12 knees (10 patients) with congenital absence of the anterior cruciate ligament are presented. The high frequency of this condition in association with other more easily recognised congenital abnormalities of the knee is discussed. It is concluded that congenital absence of the anterior cruciate ligament is more common than generally suspected and is associated with other developmental abnormalities of both bone and soft tissue in the lower limb, particularly around the knee joint

We carried out three total knee replacements with proximal realignment in two patients with severe osteoarthritis of the knee and congenital dislocation of the patella. During the operation, the femur and the tibia were cut according to the recommendations of the manufacturer of the implant. The femoral component was placed in external rotation and the centre of the tibial component aligned in relation to the tibial tuberosity with regard to rotation and translation. After making the bone cuts, the iliotibial tract was detached from Gerdy’s tubercle, the popliteus tendon divided, and the biceps femoris tendon elongated by Z-plasty. After the trial implants were positioned, a proximal re-alignment procedure was performed. One knee had deep infection. There was one dislocated patella which was repositioned, and walking ability was improved in all knees. We believe that the rotational alignment of a prosthesis is as important as the soft-tissue surgery

1. Observations on ninety-four persons with congenital coxa vara living in a Turkish village named Gonyeli in Cyprus are presented. 2. Various clinical and laboratory studies showed, in addition to the congenital coxa vara of varying severity, marked short stature and bowing of the lower extremities in almost all affected individuals and relative iliac hypoplasia and protrusio acetabuli in some. 3. Pedigree analysis indicates that this hitherto undescribed condition is inherited as an autosomal dominant trait

Purpose of the study: Congenital dislocation of the patella is a very rare condition. The pathology is inconsistent and treatment modalities are unclear. The aim of the study was to show the results of operative treatment of congenital dislocation of the patella. Material and methods: 9 knee joints in 7 patients with congenital dislocation of the patella have been treated between 1989 and 1999. Additional diagnoses were Rubinstein-Taybe syndrome, Larsen syndrome, pteryg-ium syndrome and cerebral palsy in 1 patient each. The age at the time of surgery was 8.9 years on average (between 4.3 and 14.8 years). In 6 knees primary treatment was a medial shifting of the quadriceps muscle according to Stanisavljevic, in the other 3 a combination of lateral release according to Green, proximal quadriceps realignment according to Insall and either medial displacement of the tibial tuberosity according to Elmslie or a duplication of the patellar tendon according to Goldthwait had been done. Results: The follow-up time was 6.4 years on average (between 2 and 12.5 years). 4 of 9 patellae remained stable after 1 operation, 3 remained stable after 2 operations and 2 remained unstable. 4 of the 9 knees were symptomatic at the time of follow-up. All patients were able to walk and to run and all had full extension. Apart from recurrence there were no major complications. Conclusions: Permanent dislocation of the patella reduces the extension force of the quadriceps muscle significantly. Medial shifting of the quadriceps muscle according to Stanisavljevic gives the best chance to reduce the patella permanently. Postoperative taping, splinting and muscle-exercises are often necessary to get a stable situation

The early management of bilateral congenital popliteal webbing in a brother and sister is described. There was no familial history of webbing, but the maternal grandfather had a hare-lip. Although the sciatic nerve is so unfavourably placed in the web, correction of the flexion deformity can apparently be carried out safely after plastic repair of the skin by Z-plasty and excision of the fibrous web cord alone, without causing a traction paralysis. These cases appear to be unique in that the patients are siblings of different sex with identical congenital abnormalities of bilateral webbing, cleft palate and fistulae of the mucous membrane of the lower lip

The variability in measurement of angles in congenital scoliosis is not known, but it is postulated that it is larger than that in adolescent idiopathic scoliosis due to skeletal immaturity, incomplete ossification, and anomalous development of the end-vertebrae. To determine this variability, we selected 54 radiographs of adequate quality showing 67 scoliotic curves from children with congenital scoliosis. The end-vertebrae were preselected. Each curve was measured by the Cobb method on two separate occasions by six different observers, using the same goniometer and marker. The intraobserver variability was +/- 9.6 degrees and the interobserver variability +/- 11.8 degrees. If 'significant progression' is to be used as a criterion for surgical fusion in congenital scoliosis, there should be at least a 23 degrees increase, the entire range of the interobserver variability, in the curvature to ensure that the perceived increase is not due to variability in measurement

A five-day-old boy was referred with a soft-tissue mass in his right upper arm. Plain radiographs and ultrasound demonstrated a lesion extending from the axilla to the elbow on the posterolateral aspect of the humerus. Open biopsy confirmed the diagnosis of congenital haemangiopericytoma. After MRI and selective angiography, excision biopsy was carried out, but no adjuvant therapy was administered. At further examination, four years and ten months later, he was noted to have three small nodules at the site of the original tumour. Excision biopsy confirmed this to be a local recurrence, although the lesion was less cellular with no appreciable mitotic activity. Congenital haemangiopericytoma is a rare cause of a soft-tissue mass in children. Most tumours are benign, and recurrence is uncommon. The treatment is controversial, but most centres recommend the use of adjuvant chemotherapy, combined with complete excision. We recommend treatment with doxorubicin. Orthopaedic surgeons should be familiar with this tumour since 30% to 50% of cases occur in the limbs

We present a retrospective review of a single-surgeon series of 30 consecutive lengthenings in 27 patients with congenital short femur using the Ilizarov technique performed between 1994 and 2005. The mean increase in length was 5.8 cm/18.65% (3.3 to 10.4, 9.7% to 48.8%), with a mean time in the frame of 223 days (75 to 363). By changing from a distal to a proximal osteotomy for lengthening, the mean range of knee movement was significantly increased from 98.1° to 124.2° (p = 0.041) and there was a trend towards a reduced requirement for quadricepsplasty, although this was not statistically significant (p = 0.07). The overall incidence of regenerate deformation or fracture requiring open reduction and internal fixation was similar in the distal and proximal osteotomy groups (56.7% and 53.8%, respectively). However, in the proximal osteotomy group, pre-placement of a Rush nail reduced this rate from 100% without a nail to 0% with a nail (p < 0.001). When comparing a distal osteotomy with a proximal one over a Rush nail for lengthening, there was a significant decrease in fracture rate from 58.8% to 0% (p = 0.043). We recommend that in this group of patients lengthening of the femur with an Ilizarov construct be carried out through a proximal osteotomy over a Rush nail. Lengthening should also be limited to a maximum of 6 cm during one treatment, or 20% of the original length of the femur, in order to reduce the risk of complications

Purpose: Congenital insensitivity to Pain (CIP) is a rare peripheral neuropathy which may affect various sensory pathways and often affects the autonomic nervous system. Musculoskeletal manifestations include infections, fractures, growth disturbances, avascular necrosis, Charcot arthropathy, joint dislocations and heterotopic ossification. The purpose of the study was to review the orthopaedic problems in patients with Congenital Insensitivity to Pain and make treatment recommendations. Methods: Thirteen patients from eight families were examined and all charts and radiographs were reviewed. A quantitative sweat test was performed in five patients and an intradermal histamine test in ten. DNA was prepared in all patients and examined for specific mutations. Results: Three clinical presentations were found:. Type A – Five patients presented with multiple infections requiring many surgical procedures ranging from local debridement to below knee amputation. Type B – Three patients presented with fractures and growth disturbances of the lower limbs as well as avascular necrosis of the talus or femoral condyle. Two patients underwent corrective osteotomies due to deformities. Type C – Five patients presented with Charcot arthropathies, joint dislocations, fractures and infections. Four of them were mentally retarded. Patients underwent multiple surgical procedure to control infections. Attempts surgical stabilization of joints were unsuccessful. Mutations were found in four patients. Conclusions. Patient education, shoe ware and periods of non weight bearing are important in prevention and early treatment of decubitus ulcers. Differentiation between fractures and infections is difficult and should be based on aspiration and cultures in order to prevent unnecessary surgery. Established infections should be treated by wide surgical debridement. Deformities should be treated by corrective osteotomies and shortening should be treated with shoe lifts or epiphysiodesis. Joint dislocations should be treated non-operatively as attempts at surgical stabilization gave poor results

Purpose of the study: the Pavlik harness has been used for the treatment of congenital hip dislocation since it was designed by Arnold Pavlik in 1950. There remains however a certain debate concerning the best moment to start treatment and its duration. We advocate early use of the Pavlic harness for a short period. Material and methods: Forty-five hips (34 infants) were treated. The diagnosis of dislocation was clinical. The Barlow and Ortolani maneuvers were used to search for clinical instability classed as «positive dislocation test» or «negative test but presence of piston movement». Different classifications of positive tests were used to search for an association with increasing severity of hip instability. Static and dynamic ultrasound was then used to confirm the diagnosis of hip dislocation. A Pavlik harness was installed immediately after diagnosis of congenital hip dislocation, on the day of birth if possible, according to the precepts proposed by the inventor. Results: Among the 43 hips analyzed I the present series, reduction and stabilization was successfully achieved with the Pavlic harness in 40 used as early as possible for a short a period as possible. This 95.6% success rate (2 failures, 0 complications) was achieved within 3 o 8 weeks. Discussion: Our results are comparable with other series reporting early use of the Mubarak method. The duration of treatment was shorter with our therapeutic method. We did not attempt to treat the dysplasia, spontaneous regression was monitored radiographically. Conclusion: We consider congenital hip displasia to be a therapeutic emergency. Treatment should be undertaken as soon as the dislocating intrauterine constraints cease. Early use of the Pavlik harness on easily dislocated or dislocated reducible hips has given excellent results. The shorter treatment duration does not lead to any recurrence as long as clinical stability with formal radiographic confirmation at treatment end

1. Two cases of an unrecognised congenital defect of the humeral head are described and the cause is discussed. 2. Only six cases with similar radiographic appearances could be collected from the literature. In most of these cases other skeletal deformities were present, whereas in those now reported only the shoulder was affected. 3. Examination of radiographs suggests that the main deformity consists of lack of development of the capital epiphysis of the humerus. 4. Consideration of the cases, together with experimental data from the studies of Fell and Canti, suggests that the time in development at which the fault occurred was the presumptive joint stage, just when the articular rudiments had separated. 5. A "nociferous agent," acting only for a limited period, and only on certain tissues, is postulated. 6. It is suggested that the defects recorded should be recognised as a group of congenital deformities of the shoulder joint

We report the clinical outcomes of children with complex congenital foot deformities treated with UMEX® mini-external fixators. This is a prospective review of our experience in patients treated in this way, from 2004. The indications for treatment were resistant/recurrent Congenital Talus Equina Varus(CTEV), cavo-varus deformity secondary to Charcot-Marie-Tooth disease, arthrogryposis, fibular hemimelia and other congenital abnormalities. In addition, one patient underwent this treatment since he was allergic to casting material. A total of 32 children (35 feet) have been treated, with a male to female ratio of 22:10 respectively. The patient-age at operation ranged between 3 and 15 years (median age −7 years). Three patients underwent bilateral procedures and 29 patients underwent unilateral foot operations. Twenty-eight patients underwent previous soft-tissue and bony corrective operations with serial casting. The frames were removed at an average of 69 days after application with a further 6 weeks in a walking cast. Seventy-nine percent of the patients had good functional outcome. Further operations were needed in 10 patients. Complications occurred in 10 patients, including pin-site infections, bony overgrowth at pin-site and proximal tibio-fibular diastasis. In conclusion, this is a simple fixator to use with a small learning curve. In groups of patients with complex congenital abnormalities, we achieved good functional outcome with low-complication rates

Only two cases have been reported of congenital dislocation of the hip in infants born after extrauterine pregnancies. We report a further two and discuss the management and the variable outcome. These cases seem to confirm that congenital dislocation of the hip is associated with moulding forces rather than being a teratological abnormality

Introduction and Objectives: This rare disease (17 per million newborns) was described by Chatelaine in 1882 and is more prevalent in females compared to males (2.3:1). It is commonly accompanied by congenital or genetic malformations. Within the syndrome known as genu recurvatum, congenital knee dislocation (CKD) is the most extreme clinical form. In view of the scarcity of trials and reviews on the subject of CKD and results of therapy for the same, we determined to gather clinical case data from the hospital from 01–01-1964 to 01–01-2003 and evaluate therapeutic, orthopaedic, and surgical experience. Materials and Methods: Of 28,753 patients treated in the aforementioned period, 55 were treated for genu recurvatum. We selected 22 with unilateral or bilateral CKD. We individualised the cases of 14 patients with 20 knees as patients diagnosed and treated since birth by this centre, excluding those with multi-malformation genetic syndromes (Larsen’s syndrome, arthrogryposis, etc.). In terms of treatment, 13 knees were treated orthopaedically by means of successive manipulations and splinting until 90° of flexion was achieved. At that point, physical therapy was initiated. There were 7 other knees that received at least one surgical treatment when results of the previously-described orthopaedic procedures were unsatisfactory. The clinical outcome for the treatment method used in each case was assessed by means of evaluation of range of mobility of the knee joint, stability upon examination, residual deviation from the axes, and ability of the patient to walk at time of release. Results: Of the 65% of knees that were treated exclusively by orthopaedic methods, we observed ranges of flexion and stability such that splinting was not needed at an average of 55.2 days, with good to excellent clinical outcomes at an average of 42.4 days. Of the 35% of knees that required at least one surgical procedure, the procedure was performed between the ages of 30 and 176 days of life, with a mean of 90.8 days. As many as 75% of these knees required repeated surgical intervention during the treatment period, and good to excellent results were achieved in only 35% by 5 years of age. Discussion and Conclusions: Orthopaedic treatment continues to be the foundation of managing these patients, and plays a vital role even in cases were surgical intervention is chosen. The existence of other genetic or non-genetic developmental anomalies concurrent with CKD will determine the therapeutic strategy that is indicated and when such action should take place. We do not recommend surgical treatment except in cases of a continued lack of response to orthopaedic treatment

Five knees with congenital dislocation were explored. The cruciate ligaments were found to be absent or hypoplastic. The results obtained by reconstructing an anterior cruciate ligament appear to be good. On the basis of dissections of foetal knees we believe that the cruciate ligaments are the main structures preventing an anterior dislocation of the knee in early foetal life; this deviation from the adult pattern is due to the bony configuration of the foetal knee. We therefore postulate that the basic defect in congenital dislocation of the knee is an absence or hypoplasia of the cruciate ligaments

To evaluate the correction of complex congenital deformities of the lower limb by six axes deformity analyses and computer assisted correction using the Taylor TM Spatial Frame (TSF), from 1998 to 2000, the authors performed corrections of multiple congenital deformities in 24 lower limbs in 18 patients. There were 9 males and 9 females. There were a total of 29 bone segments, (8 femurs, 21 tibiae) in the 24 lower limbs that were corrected with application of the TSF. Our series included the following diagnoses and deformities: unknown skeletal dysplasia (2), achondroplasia (3), pseudoa-chondroplasia (1), multiple epiphyseal dysplasia (2), spondyloepiphyseal dysplasia (2), fibular hemimelia (3) tibia hemimelia (1), hypophosphatemic rickets (3), and posteromedial bowed tibia (1). The mean age of the patients was 15.4 years (range 0.5 to 35 years). The mean frame time until correction was 20.1 weeks (range 9 to 49 weeks). The mean follow up was 2.4 years (range 2 to 3.4 years). The apex of the deformity was directed posteromedial in 7, anterolateral in 6, medial in 5 and anteromedial in 5 patients. The mean coronal and sagittal plane deformities were 14.60 (range −230 to 400) and 70 (range, −400 to 280), respectively. The average magnitude of the deformity was 21.70 (range 90 to 470), and the plane of the deformity to the coronal plane was −23.30 (range −800 to 400). Eight patients had a mean lower extremity shortening of 12.3 mm (range 5 to 50 mm). One patient had 15° of internal rotation. With application of the TSF and the principles of distraction osteogenesis, we were able to reduce the coronal and sagittal plane deformities to 3.10 and 1.40 respectively. The overall mean magnitude of the deformity was decreased to 3.40. Shortening was corrected to an average of 3 mm. We experienced only 4 complications in the 24 limbs (16.7%). Complications in this patient group included one female patient with hypophosphatemic rickets who had residual deformity with significant lateral mechanical axis deviation due to inadequate translation. In addition, there were two superficial pin tract infections and one delayed union. Computer-assisted six axes deformity planning and TaylorTM Spatial Frame application effectively and safely correct complex congenital and developmental limb deformities and offer significant advantages over the well-established Ilizarov technique

Fifty-four adults with eighty hips affected by congenital disease which had not been treated have been reviewed. Fifty-nine per cent of forty-two dislocated hips had fair or poor grading scores. The incidence of osteoarthritis was markedly increased in the presence of a well-developed false acetabulum. Unilateral dislocation led to valgus deformity and degenerative changes in the ipsilateral knee in seven of twenty-two patients. Dislocation did not increase the incidence of symptomatic lumbar spondylosis. The height of the dislocated head on the ilium was not found to be related to the prognosis for the hip, the knee or the lumbar spine and did not correlate with the development of the false acetabulum. Frank congenital subluxation eventually led to osteoarthritis of the hip

1. Twenty-one cases of congenital dislocation of the hip were found on examination of 1,881 consecutive neonates on the first day of life, giving an incidence of eleven per 1,000 live births. 2. Insignificant high-pitched "clicks" were noted in 10 per cent of newborn children. 3. Conversion of half of the patients with hip dislocation to normal occurred during the first post-natal week. 4. Joint laxity was not a feature of the newborn with congenital dislocation of the hip. 5. Oestradiol, oestrone and oestriol were estimated in twenty-fourhour urine samples collected from sixteen patients with congenital dislocation of the hip and nineteen matched controls during the first six days of life. No significant differences in oestrogen output between the two groups were found. 6. The hypothesis that congenital dislocation of the hip is a result of an inborn error of oestrogen metabolism in the newborn is not supported

The purpose of this study is to present early results, common pitfalls and management in in cases of revision hip arthroplsty in patients with congenital disease of the hip. From 2001 to 2006, 36 consecutive cemented THAs with a history of congenital hip disease were revised due to aseptic loosening (31 cases), stem fracture (3 cases), septic loosening (2 case). There were thirty patients, all females, with a mean age at revision 61.7 years (range, 40 to 76). The revision was performed after a mean 15.4 years post primary operation (range, 9 to 26). In 7 cases the cup only, in 5 cases the stem only, and in 24 cases both components were revised. The mean follow-up was 43 months (range, 24 to 84). There were 3 intraoperative femoral fractures managed with long stem and circlage wires. Postoperatively, 5 hips were infected and sustained a 2 stage revision using a cement spacer. 3 hips were revised due to loosening. 28 cups and 28 stems remained intact for an average 45.2 months (range, 24 to 84). The probability of survival at 48 months was 76.3% (±9.7%) for the cups (12 components at risk) and 76.4% (±11.3%) for the stems (9 components at risk). Revision of a CDH arthroplasty is difficult and non predictable. Lack of acetabular bone stock and anatomical abnormalities of the femur lead to increased intra and postoperative complication rate

Malformation and hypoplasia of the clavicle can result in pain, impaired function, restricted shoulder movement, subjective feeling of instability and cosmetic deformity. There are no reports of clavicle lengthening by osteotomy and distraction osteogenesis (DO). This is a retrospective review of 5 patients (7 clavicles) who underwent clavicle lengthening by DO using a monolateral external fixator for clavicular hypoplasia. There were 3 males and 2 females with mean age 15 years (9 to 23) and mean follow-up 21 months (8 to 51). Preoperative diagnoses included Klippel-Feil syndrome, cleidocranial dysplasia with torticollis, congenital myopathy and Noonans syndrome and obstetric brachial plexus injury. Mean length gained was 31 mm (15 to 41) which represens an average of 24.7% of overall bone length. Mean time in fixator was 174 days (161 to 263) and mean external fixation index was 56 days/cm. Two patients required internal fixation following fixator removal to consolidate union and one required additional internal fixation for atrophic regenerate. Mean preoperative oxford shoulder score improved from 28.5 to 41 and all patients were extremely satisfied with their result. Two patients developed pin site infections. Clavicular lengthening by distraction osteogenesis for congenital clavicular hypoplasia is a previously unreported technique that enables gradual correction of deformity without risking brachial plexus traction injury following acute correction. It has the potential to improve shoulder pain, function, range of movement and cosmesis. Distraction ≥25% of overall bone length may require additional plate fixation to consolidate union

Congenital dislocation of the patella requires early surgical reduction for better walking. We studied the results of our combined soft tissue procedures performed on 6 knees in 5 children. The age at surgery ranged from 3 to 12 years with a mean of 5.3 years. The follow-up period ranged from 3 to 9 years with a mean of 5.1 years. Underlying diagnoses were fibular hemimelia in one knee, congenital dislocation of the knee which was reduced without surgery in two, and nail-patella syndrome in three. The surgical procedures involve lateral release to reduce the patella, resection and tightening of medial capsule and semitendinosus transfer to the patella. Quadriceps lengthening was also required in two knees. After lateral release, the semitendinosus tendon is detached at its insertion, and is pulled out at the musculotendinous junction. Then, the tendon is pulled down to the patella under the skin, and is passed through a drilled tunnel in the patella from superomedially to inferolaterally. Finally, the tendon is reflected and sutured to the anterior surface of the patella under sufficient tension at 20 degrees of knee flexion. The five knees operated on under 5 years of age were well reduced and well positioned in the femoral groove at follow-up. The one knee operated on at the age of 12 years showed subluxation. Episodes of giving way and abnormal gait disappeared after surgery in all the patients. Femoral groove depth increased after surgery. The improvement in the young infants was better than in older child. We found that the transferred semitendinosus tendon acts well to maintain good patellar position during knee flexion. We conclude that early surgical reduction is extremely important and it will stimulate proper development of femoral groove, and semitendinosus transfer combined with lateral release is effective to keep the patella in its groove during knee flexion

The purpose of this cohort study is to determine the incidence of all congenital vertebral anomalies detected antenatally through ultrasound. We also reported on the early mortality rate for this patient cohort, as well as the frequency and type of associated congenital anomalies. The East Midlands and South Yorkshire Anomalies Register consists of data on all voluntary reports of congenital anomalies, from an annual baseline birth rate of 67000 births. We analysed all registered congenital anomalies reported over a 10 year period. Between January 1997 and January 2007, 108 vertebral anomalies were reported, excluding spinal dysraphism (incidence 0.01%). 61 of these were detected antenatally (56%), 17 were detected postnatally (16%) and in 30 patients, the precise time of diagnosis was unclear (28%). At the time of analysis January 2007, 45 of 108 patients had died, either in utero or soon after delivery (42%). 12 fetuses remained in utero and 51 infants were alive. The mortality rate for antenatally diagnosed patients was 41% and the majority were electively terminated (72%). 2 fetuses electively terminated had vertebral anomalies in isolation. There is a relatively high incidence of elective termination of pregnancy as a result of antenatal anomaly ultrasound screening. We have evidence to suggest that foetuses with potentially minor congenital anomalies are being electively terminated at approximately 18 weeks gestation. A structured and timely spinal counselling should be offered once antenatal vertebral anomalies have been identified

Background: Congenital absence of the cruciate ligaments is a commonly associated pathology of the knee joint in congenital longitudinal deformities. We performed a radiological analysis and investigated gait patterns in patients with congenital absence of one or both cruciate ligaments. Patients and Methods: Thirty-four knee joints in thirty-one patients with congenital longitudinal deficiency of the lower limb were evaluated. The cruciate ligaments and associated abnormalities of the bony configuration were evaluated on magnetic resonance imaging and tunnel view radiographs. A radiological classification is proposed. Gait analysis was employed to determine kinematic, kinetic and electromyographic data in 24 of these patients and the results were compared to an age-matched control group. Results: We differentiated 3 main types of absence of the cruciate ligaments with typical associated changes in the femoral intercondylar notch (FIN) and the tibial eminence (TE). In type I (n=19) partial closure of the FIN and hypoplasia of the TE was observed in hypoplasia or absence of the ACL, in type II (n=7) these findings were aggravated by additional underlying hypoplasia of the PCL and in type III (n=8) absence of the FIN and a flat TE was observed in aplasia of both cruciate ligaments. The main findings in gait analysis were significantly increased flexion moment of the hip, increased flexion of the knee in midstance phase and reduced ankle power in comparison to the control group. Conclusion: The knee joint with aplastic cruciate ligaments shows typical radiological changes, thus, one will be able to distinguish between aplasia of the ACL only or both cruciate ligaments by observing plain tunnel view radiographs. Our obtained data of the gait analysis revealed specific gait patterns as adaption to underlying aplasia of the cruciate ligaments

A few points in this report deserve to be stressed. Indications–It is important that the orthopaedic surgeon should decide at a very early stage which of the two methods, closed or open, he must use. These do not exclude each other but are on the other hand complementary. Nowadays the dislocated hip can be reduced by open operation with a very good chance of lasting success. This should be carried out if a hip cannot easily be reduced otherwise, or if there is any doubt that closed reduction has been successful–and as early as possible, preferably before the age of three years. Technique–Ample exposure of the joint and removal of all obstacles to reduction are important. Reduction must be complete and stable but without stress, and there must be no interference with the articular bone and cartilage. After-care–Reduction, however perfect, is only the first step towards recovery. The hip must be observed carefully and the most suitable moments for mobilisation and for walking must be chosen; this calls for nice judgment. When it is clear that the roof of the acetabulum is not developing or that persistent valgus and anteversion may encourage subluxation, a secondary operation should be undertaken at once. Radiography is necessary about every three months for the first two years. Assessment of results–With a strict system of assessment, like McFarland's, we have observed 68·3 per cent favourable results in 171 hips treated by open reduction. It is obvious that the problem of congenital dislocation of the hip has not yet been completely solved. But if we review the progress of recent years we come to the encouraging conclusion that much has already been achieved, and that the efforts of the many orthopaedic surgeons who have dedicated themselves to the treatment of congenital dislocation of the hip have not gone unrewarded

1. Radiological criteria in the diagnosis of congenital dislocation of the head of the radius are suggested. 2. Some of the radiographic features described as characteristic of congenital dislocation of the head of the radius may be found also in post-traumatic dislocation of the radial head

1. A patient with ectopic bone in the upper arm associated with multiple congenital anomalies is reported. 2. The previous cases of congenital abnormalities in patients with ectopic bone formation have been indicated and the problem of etiology has been discussed

We present a systematic review of the results of the Ponseti method of management for congenital talipes equinovarus (CTEV). Our aims were to assess the method, the effects of modifications to the original method, and compare it with other similar methods of treatment. We found 308 relevant citations in the English literature up to 31 May 2010, of which 74 full-text articles met our inclusion criteria. Our results showed that the Ponseti method provides excellent results with an initial correction rate of around 90% in idiopathic feet. Non-compliance with bracing is the most common cause of relapse. The current best practice for the treatment of CTEV is the original Ponseti method, with minimal adjustments being hyperabduction of the foot in the final cast and the need for longer-term bracing up to four years. Larger comparative studies will be required if other methods are to be recommended

Congenital Hand Deformities are probably the most frequent cause of non-traumatic complaint in consultations on paediatric hand surgery. The incidence of occurrence is about 1 in 500 live births. Some of these abnormalities are minor and do not interfere with function. Many, however, have a significant functional and/or cosmetic deformity. Only a few congenital malformations have a regular single gene mode of transmission, most have an irregular and unpredictable pattern of inheritance; sometimes occurs as part of a malformation syndrome or skeletal dysplasia, or in a sporadic way. In fact, the cause for 40 to 50% of these anomalies is “unknown”. Themes like handling the patient and parents and the psychological effects of the anomaly, the general principles of treatment, timing of surgery and the principles of reconstructive surgery are discussed. The classification proposed by A.B. Swanson and revised with the assistance of the Congenital Anomalies Committee of the International Federation of Societies fo Surgery of the Hand will be used to propose the general guides of treatment. The most common anomalies will be approached in more detail and the option of treatment is discussed in base of experience of the author

Purpose: The prognosis of congenital hip displacement basically depends on the time of diagnosis and treatment. Recognising high-risk hips early remains a number one priority. We conducted a prospective study over a 20-year period from 1992 to 2002 to analyse the epidemiology of congenital hip displacement. Material and methods: The series included 1056 children with congenital hip disease (1491 hips). Epidemiological data, ultrasound and x-ray findings were recorded over 20 years. The objective was to identify risk factors and evaluate the impact of prevention measures. Results: The sex ratio showed female predominance, 6:1. The left hip was involved 1.8 times more often, with 41% bilateral involvement. Risk factors were major: family history (31%), breech presentation (25%), postural syndrome (12%); or minor: primiparity (54.4%), birth weight > 4 kg (9.2%). One or more major risk factors were found in 60.5% of the children and 30% had at least one minor risk factor. No risk factor was found in 40%. Screening efficacy improved with a rate of diagnosis before 4 months of 59% in 1983 and 96% in 2002. The number of hips discovered after the age of one year was 15% in 1983 and 6% in 2002. Discussion: The severity of the hip displacement is not influenced by risk factors nor bilateral involvement. Screening has enabled earlier diagnosis with a 37% increase in the rate of identification before the age of four months. This has been made possible by a systematic examination at birth and ultrasonography introduced in 1989. Conclusion: A regional map showing the paediatrician : maternity : general practitioner distributions is an important tool for screening campaigns. Despite adequate screening 40% of these children do not have any risk factor. Repeated examinations, communication and information exchange between healthcare professionals are the keys to success

1. Breech malposition and hormonal joint laxity produce atraumatic posterior dislocations in the hip joints of young rabbits. 2. Experimental studies were shown to cause the development of a limbus and other softtissue changes similar to those found in human congenital dislocations. 3. The development of femoral retroversion and anteversion in the presence of joint laxity is described. 4. The co-existence of breech malposition and hormonal joint laxity in utero, and their importance as prime factors in the etiology of congenital dislocation of the hip, are discussed

Purpose of the study: A population of 154 patients was studied to determine the advantages of continuous lengthening for congenital lower limb length discrepancy (LLLD). Material and methods: In a first series, we analyzed 80 progressive lengthenings using the standard rhythm (1 mm daily, 4 lenghtenings per day). In a second series (74 lengthenings) a high-frequency rhythm was used (1 mm daily, 60 lengthenings per day). Mean patient age was 10.3 years. Bone regeneration was not stimulated (either by extemporaneous compression or stable elastic centromedullary nailing) in this population. The automatic lengthening fixator was composed of the two standard pieces of the Ilizarov system and complementary blocks with self-propelled traction rods. Besides simplifying the lengthening procedure, these rods allowed high-frequency correction of associated deformities. Results: Femoral gain was 52 mm on average. For the tibia, the gain was 48 mm on average. For ordinary monosegmentary lengthenings, the healing index was 27.6 d/cm for the femur and 36.0 d/cm for the tibia. For multisegmentary lengthenings with the standard rhythm, the overall healing index was 20.3 d/cm. For patients with high-frequency lengthening, time to healing was shorter. The radiological findings showed the presence of significant bone regeneration which was never inhibited. For monosegmentary lengthenings, the healing index was 22.9 d/cm for the femur and 27.1 d/cm for the tibia. For the multisegmentary high-frequency lengthenings, the overall healing index was 14.7 d/cm. The difference between standard and high-frequency lengthening was significant. In the first series, motion of the adjacent joints was recovered within 12 to 18 months after removal of the fixator. The patients remained in the reclining position during the high-frequency lengthenings and very satisfactory results (complete recovery of joint motion) were obtained 12 months after removing the fixator. In addition, in the second series, there was no impact on the spontaneous growth of the lengthened segments. Discussion and conclusion: Congenital LLLD is generally more difficult to treat than acquired conditions (Damsin et al., Grill et al., Glorion Ch.). The rate of complications remains significant, particularly concerning healing complications and stiffness in the adjacent joints. Our clinical results prove that high-frequency lengthening provides optimal conditions for tissue regeneration. For children with congenital LLLD, continuous lengthening shortens the delay to healing and avoids stiffness in the adjacent joints

Congenital dislocation of the peroneal tendons is a rare and infrequently reported deformity of the foot in the neonate. Four cases of this deformity associated with a congenital calcaneovalgus deformity of the foot have been treated and followed to the resolution of both of the deformities. The calcaneovalgus foot proved more resistant to correction and required more prolonged and aggressive treatment than was usual when it was found as an isolated deformity. All four patients demonstrated other stigmata of intra-uterine malposition and oligohydramnios or both. Our anatomical studies suggested that the superior peroneal retinacular ligament was the critical stabilising structure for the peroneal tendons. A concept of the pathogenesis of this deformity is discussed and a proven regimen for its treatment presented

Purpose of the study: The natural history of congenital hip dysplasia with weight-bearing usually progresses towards degenerative joint disease. The anatomic type of the dislocation, whether treated or not, was well classified by Crowe who described four types. Material and methods: Since 1989, we have used an ABG hydroxyapatite (HA) coated prosthesis for the treatment of congenital hip dysplasia. The hemispheric acetabular implant is coated with hydroxyapatite and the femoral implant, which is inserted in an anatomic position with anteversion, antetorsion and anteflexion, has a HA-coated stem. Forty-three Crowe type 3 or 4 hips (high position) were treated with this technique:. implantation of the cup in the paleoacetabulum;. screwed autograft harvested from the femoral head to fill the bony defect;. implantation of an anatomic stem, without cement but with HA-coated shaft. Results: Cup implantation in the paleoacetabulum was achieved in all patients except two. A screwed autograft was inserted in 75% and remained stable over time for the larger grafts but tended to resorb for smaller grafts. For femoral anteverions, an ABG implant was used in 34 cases: 21 ABG1 stems, 11 ABF2 stems, and one ABG revision stem. The ABG stem enabled satisfactory anatomic restoration in 20 hips but with postoperative stiffness. For 14 hips, due to the important femoral anteversion related to the dysplasia, a reversed ABG-HA implant was used: eight left implants for right hips and six right implants for left hips. This «reversed» curvature gained 24° in the femoral anteversion plane. The outcome was excellent in these 14 cases, particularly with a clear improvement in postoperative external rotation. At close to 15 years follow-up we have had no case of femoral loosening, nor of femoral shaft osetolysis, with this type of implant. Discussion: Certain authors propose using a custom-made implant for sequelar congenital hip dysplasia, but we prefer the proposed technique which provides very satisfactory results and limits the need for custom-made material

Sixty patients with congenital deformities of the spine were operated upon in the past fifteen years using a two-stage procedure. In the fifty patients with scoliosis half of the deformities were due to hemivertebrae and half to unilateral bars. The average correction of the deformity was 47 per cent. Early neurological signs observed in two patients with a diastematomyelia resolved. Of the ten patients with kyphosis nine had neurological signs of impending paraplegia and one was completely paraplegic before operation; all improved markedly. Posterior spinal fusion alone in the rapidly progressing congenital deformity may not prevent further progression, particularly in those cases iwth unilateral bars. Anterior resection of the vertebral body with later posterior fusion with Harrington instrumentation is safe and effective

Beighton and Kozlowski, in 1980, first defined this disorder, in Afrikaners, the syndrome , evident at birth, is constantly manifested by dwarfism, ligamentous hyperlaxity, congenital scoliosis, and multiple dislocations(hip dislocation, radial head dislocation, scoliosis, spatulate thumbs, and generalized ligamentous hyperlaxity, children are of normal intelligence potential. We report A Tunisian family, in which the proband , and her parents family have the presumptive diagnosis of Beighton dysplasia(spondyloepimetaphyseal dysplasia), the proband manifested the full clinical criteria of the disorder, whereas the mother, and other family members are partially manifesting the disorder, but dysplastic hips is the common feature in most of the family members examined(from both paternal and maternal side), up to four family subjects are variably affected, ranging between congenital hip dislocation in two subjects and post adulthood dysplastic arthrosis. The striking clinical findings evolved from the study is the accompaniment of diverse skeletal abnormalities rather than the hip dysplasia, the mother is with adolescent type kyphosis, and two other family subjects are with short stature and scoliosis, fractures were encountered in three family subjects, this was secondary to osteoporosis, which in fact a general clinical feature in all the family subjects. The mode of inheritance of the disorder is compatible with autosomal recessive trait. The purpose of this study is to indicate the importance of the precise recognition of the underlying etiologies in children presented with congenital dysplastic hips, as a matter of fact this is our main strategy in the department