Introduction. Genu valgum is a common presentation in paediatric patients with congenital limb deformities. The aim of this study is to assess the outcome of guided growth surgery in paediatric patients referred via our physiotherapy pathway with isolated genu valgum and associated patellar instability. Materials & Methods. Patients were identified from our prospective patellar instability database. Inclusion criteria was acquired or congenital genu valgum associated with patellar instability in skeletally immature patients. The mechanical lateral-distal femoral angle was assessed on long leg alignment radiographs (mLDFA <85 degrees). Surgical treatment was the placement of a guided growth plate (PediPlate, OrthoPediatrics, USA) on the medial distal femoral physis (hemi-epiphysiodesis). KOOS-child scores were collected pre-operatively and post-operatively (minimum at 6 months). Results. Eleven patients (seven female) with mean age of 12(range 5–15) were identified. Five patients had congenital talipes equinovarus(CTEV), one fibular hemimelia, one di-George syndrome, one septic growth arrest and three had idiopathic genu valgum. Pre- and post-operative KOOS-child scores showed overall improvement: 58(range 36–68) to 88(65–99) and knee symptoms subscores: 64(43–71) to 96(68–100) p<0.01, t-test. Mean follow-up was 10 months (range 3–23). No subsequent dislocations/subluxations occurred during follow-up. Conclusions. Guided growth surgery is an effective way of treating symptomatic patellar instability in skeletally immature patients with genu valgum in the absence of other structural pathology. It was most common in our cohort in patients with unilateral CTEV. We would recommend to screen syndromic and congenital limb deformity patients for patellar instability symptoms in the presence of genu valgum

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

Aims

The aim of this retrospective cohort study was to assess and investigate the safety and efficacy of using a distal tibial osteotomy compared to proximal osteotomy for limb lengthening in children.

Introduction. Amputation or disarticulation is a reliable option for management of severe foot deformities and limb-length discrepancies, the surgical restoration of which are unpredictable or unfavourable. Of the various surgeries involving foot ablation, Syme's amputation is preferred for congenital deformities as it provides a growing, weight bearing stump with proprioception and cushioning. Materials and Methods. We reviewed data of all children who underwent Syme's amputation over the past 13 years at our institution. Surgical technique followed the same principles for Syme's but varied with surgeons. Results. Ten boys and ten girls, with an average age of 18 months and average follow up of 70 months were included in the study. The most common indication was fibular hemimelia. Wound complications were reported in three children, phantom pain in one, heel pad migration in two. None had wound dehiscence, flap necrosis, stump overgrowth, or calcaneal regrowth. None of this required surgical intervention. One child required an amputation at a higher-level secondary to a congenital malformation of nervous tissue in the affected leg. Prosthetic compatibility was 94.7 % and none used mobility aids. Six children participated in sports. Conclusions. Syme amputation is a safe and potentially advantageous procedure in children, with a low incidence of complications to offer patients with non-salvageable foot conditions. It offers good prosthetic use with minimal risk of complications and can offer patients a functional solution with only one surgical intervention throughout their childhood

Introduction

The purpose of our study was to retrospectively analyze our patients who were treated for FH and PFFD by means of guided growth temporary Hemiepiphysiodesis.

We sought to determine the effectiveness of the procedure, as well as its success rates, complications, and rebound phenomena.

Aims

Guided growth using eight-plates is commonly used for correction of angular limb deformities in growing children. The principle is of tethering at the physeal periphery while enabling growth in the rest of the physis. The method is also applied for epiphysiodesis to correct limb-length discrepancy (LLD). Concerns have been raised regarding the potential of this method to create an epiphyseal deformity. However, this has not been investigated. The purpose of this study was to detect and quantify the occurrence of deformities in the proximal tibial epiphysis following treatment with eight-plates.

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

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

Fibular Hemimelia is not just a fibular anomaly but there is entire limb involvement with varied expression in each segment. Factors which we have considered in treatment are the amount of fibula present, percentage of shortening, tibial and leg deformity and foot deformity. Residual or recurrent foot deformity is the prime reason for unsatisfactory results, so we have used Paley's classification which takes into consideration foot deformity. Our series is of 29 cases, Paley type I-7, Type II-6, Type III-16 and none of type IV. Tibial lengthening (+/−) bow correction was performed in 28 cases. Supramalleolar osteotomy was done in 4 cases. In foot, soft tissue release only was done in 6 cases and soft tissue release with osteotomy (subtalar or calcaneal) was done in 14 cases. Amputation was done in 2 cases. Age ranged from 11 months to 16 years. Mean follow up was 4.2 years. Mean lengthening was 3.5 cm. Desires lengthening was achieved in 21/29 cases and plantigrade foot was achieved in 16/29 cases. Complications faced were recurrence of foot deformity, knee valgus, knee fixed flexion deformity, knee subluxation and pin tract problems. Less than 3 rays and more than 25 cm of limb length discrepancy were poor prognostic factors. We had 7 excellent, 16 good and 6 poor results. To conclude, it is difficult to achieve the aim of plantigrade foot and limb length equality in all cases but radical surgery with foot correction and tibial lengthening can give good results

We report the results of a retrospective review of patients that underwent distal tibial deformity correction with transphyseal or supramalleolar osteotomy with or without tibial lengthening. The aims of the procedures performed were to obtain equal leg length, restore the alignment of the ankle joint and tibio—fibular relationship. Supramalleolar osteotomy enables deformity correction, can be combined with lengthening and is appropriate where the tibio—fibular relationship is normal. When the tibio—fibular relationship is abnormal, as is often the case with bone dysplasias, differential tibio—fibular lengthening can be performed. If physeal arrest has occurred, for example after sepsis, deformity correction can be achieved with a transphyseal osteotomy allowing correction and ensuring epiphysiodesis. When the fibular length is excessive, transphyseal osteotomy can be combined with a fibular shortening. Our review encompassed 12 patients over a period of 10 years with 5 having deformity after previous meningococcal septicaemia, 4 with fibular hemimelia, 2 with a history of previous trauma and 1 with deformity occurring after a compartment syndrome as a consequence of snake bite. Seven transphyseal osteotomies were performed in 5 patients (2 bilateral), 4 with deformity secondary to meningococcal septicaemia and 1 with deformity secondary to previous trauma. After 1 transphyseal osteotomy there was recurrent distal tibial deformity (14%) which occurred within 1 year requiring a later supramalleolar dome osteotomy. Of the other 6 transphyseal osteotomies all healed with no residual leg—length inequality or deformity. Seven patients underwent supramalleolar osteotomies with all healing and recurrent deformity occurring in 1 patient (14%). Future lengthening is required in 2 patients and 1 patient will undergo a subtalar joint arthrodesis for a painful valgus hindfoot. Distal tibial deformity correction is challenging but our results show that providing the stated principles are adhered to, successful management with an acceptable recurrent deformity rate is possible

This study evaluated the effect of limb lengthening on longitudinal growth in patients with achondroplasia. Growth of the lower extremity was assessed retrospectively by serial radiographs in 35 skeletally immature patients with achondroplasia who underwent bilateral limb lengthening (Group 1), and in 12 skeletally immature patients with achondroplasia who did not (Group 2). In Group 1, 23 patients underwent only tibial lengthening (Group 1a) and 12 patients underwent tibial and femoral lengthening sequentially (Group 1b).

The mean lengthening in the tibia was 9.2 cm (59.5%) in Group 1a, and 9.0 cm (58.2%) in the tibia and 10.2 cm (54.3%) in the femur in Group 1b. The mean follow-up was 9.3 years (8.6 to 10.3). The final mean total length of lower extremity in Group 1a was 526.6 mm (501.3 to 552.9) at the time of skeletal maturity and 610.1 mm (577.6 to 638.6) in Group 1b, compared with 457.0 mm (411.7 to 502.3) in Group 2. However, the mean actual length, representing the length solely grown from the physis without the length of distraction, showed that there was a significant disturbance of growth after limb lengthening. In Group 1a, a mean decrease of 22.4 mm (21.3 to 23.1) (4.9%) was observed in the actual limb length when compared with Group 2, and a greater mean decrease of 38.9 mm (37.2 to 40.8) (8.5%) was observed in Group 1b when compared with Group 2 at skeletal maturity.

In Group 1, the mean actual limb length was 16.5 mm (15.8 to 17.2) (3.6%) shorter in Group 1b when compared with Group 1a at the time of skeletal maturity. Premature physeal closure was seen mostly in the proximal tibia and the distal femur with relative preservation of proximal femur and distal tibia.

We suggest that significant disturbance of growth can occur after extensive limb lengthening in patients with achondroplasia, and therefore, this should be included in pre-operative counselling of these patients and their parents.

Most patients (95%) with fibular hemimelia have an absent anterior cruciate ligament (ACL). The purpose of this study was to assess the long-term outcome of such patients with respect to pain and knee function. We performed a retrospective review of patients with fibular hemimelia and associated ACL deficiency previously treated at our institution. Of a possible 66 patients, 23 were sent the Musculoskeletal Outcomes Data Evaluation and Management System (MODEMS) questionnaire and Lysholm knee score to complete. In all, 11 patients completed the MODEMS and nine completed the Lysholm score questionnaire. Their mean age was 37 years (27 to 57) at review. Five patients had undergone an ipsilateral Symes amputation. There was no significant difference in any subsections of the Short-Form 36 scores of our patients compared with age-matched controls. The mean Lysholm knee score was 90.2 (82 to 100). A slight limp was reported in six patients. No patients had episodes of locking of the knee or required a supportive device for walking. Four had occasional instability with sporting activities. . These results suggest that patients with fibular hemimelia and ACL deficiency can live active lives with a similar health status to age-matched controls

Purpose of the study. We describe a new technique of talar dome osteotomy in the treatment of fixed equinovalgus deformity of the foot in patients with Fibular Hemimelia and successfully applied it in two patients. Background. Fibular Hemimelia is a congenital absence or hypoplasia of fibula with associated fixed equinovalgus deformity of the foot. Treatment for this deformity ranges from corrective osteotomy of the tibia, calcaneum to Syme's amputation. Methods. The procedure of talar dome osteotomy is best applied to children before they start to walk. Through a Cincinnati approach, fibular anlage was excised and a talar dome osteotomy performed in the axial plane to correct the valgus deformity of the ankle. Additional procedures if required include corrective osteotomy of the distal tibia to correct remaining foot deformity after the initial correction, and tendo achillis lengthening. The corrected position is then maintained with a K-wire inserted through the calcaneum, osteotamised talus up into the distal tibia. K-wire was removed at 6 weeks and foot position thereafter maintained in an AFO orthrosis with the foot slightly inverted for next 2 years. Two patients diagnosed with fibular hemimelia (Coventry and Johnson type II) underwent correction of their fixed equino-valgus deformity with the above mentioned technique at the ages of 6 and 10 months respectively. AFO orthosis was used for two years and at 5 years of follow-up the deformity has remained corrected in both the ankles. Both these patients are due to undergo limb-lengthening procedures

In distal fibular resection without reconstruction, the stabilising effect of the lateral malleolus is lost. Thus, the ankle may collapse into valgus and may be unstable in varus. Here, we describe a child who underwent successful staged surgical correction of a severe neglected valgus deformity after excision of the distal fibula for a Ewing’s sarcoma.

Fibular hemimelia is associated with equinovalgus deformity of the ankle and hind foot and antero-medial bowing of tibia. A wedge shaped distal epiphysis of the tibia and tight posterolateral soft tissues play an important role in the pathogenesis of ankle valgus and lateral subluxation of foot. Tethering effect of fibular anlage may contribute to the deformities in the tibia and ankle. Lengthening procedures are associated with progression of these deformities. The purpose of this study is to determine whether Exner Osteotomy and Excision of Fibular anlage will correct the valgus deformity of the ankle and antero-medial bowing of tibia. A bending osteotomy through the distal tibial physis as described by Exner and excision of Fibular Anlage was performed in six limbs in five children (4 boys, 1 girl) with fibular hemimelia. Histology of Excised Fibular anlage was studied under light microscopy. The mean age at the time of surgery was twenty two months (range: 8 months to 5 years). The mean follow-up was two years and two months (13 months to 4 years and 8 months). Full Correction of ankle valgus and tibial bowing was achieved in three feet where, a cortical strut graft was used in the open wedge osteotomy. In two feet synthetic bone substitute was used. In these, tibial bowing corrected but slight ankle valgus remained. In one foot where synthetic bone substitute was used and the postoperative compliance with AFO was poor, bowing of tibia improved but ankle valgus recurred. Premature fusion of growth plate did not occur in any of the cases. Histology of fibular anlage showed replacement of bone tissue by mature collagen bundles surrounded by fibroconnective tissue. Exner Osteotomy and Excision of Fibular Anlage in Fibular Hemimelia corrects the ankle valgus and antero-medial bowing of tibia

Guiding growth by harnessing the ability of growing bone to undergo plastic deformation is one of the oldest orthopaedic principles. Correction of deformity remains a major part of the workload for paediatric orthopaedic surgeons and recently, along with developments in limb reconstruction and computer-directed frame correction, there has been renewed interest in surgical methods of physeal manipulation or ‘guided growth’. Manipulating natural bone growth to correct a deformity is appealing, as it allows gradual correction by non- or minimally invasive methods.

This paper reviews the techniques employed for guided growth in current orthopaedic practice, including the basic science and recent advances underlying mechanical physeal manipulation of both healthy and pathological physes.

Introduction: Fibular hemimelia (FH) is a congenital limb reduction deficiency characterised by partial or complete absence of the fibula and a spectrum of associated anomalies. For children with a major anticipated limb length discrepancy and severe foot deformity, management (amputation or limb reconstruction) is controversial. Materials and Methods: 8 children who are now adults (average age 28 years) underwent limb reconstruction as children in one of two UK centres for severe fibular hemimelia. All 8 participants were recalled to our institution for instrumented gait analysis. The SF-36 and lower limb domains of the Toronto Extremity Salvage Score (TESS) questionnaires were also administered. Results: Partcipants scored well for general health but had functional limitations reflected in lower TESS scores. Kinematic analysis revealed decreased sagittal knee motion and valgus knee alignment. Also ubiquitous were anterior pelvic tilt and obliquity with incomplete hip extension and reduced range of hip abduction. Kinetic analysis showed reduced peak plantar flexion moment with reduced push-off power and an internal hip adduction moment in late stance. These parameters are compared to control data for below knee amputees. Discussion and conclusions: Although the number of participants is small, this is the first study to use instrumented gait analysis for severe fibular hemimelia managed with limb reconstruction. The results add objective data to the debate over limb reconstruction or amputation in this group of children