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.

Aim: We present a review on RSD in children and emphasise the role of multidisciplinary team approach in the management of 13 children. Method: 3 boys and 10 girls with a mean age of 13 years and 4 months were reviewed before and after treatment. They were assessed for pain (visual analogue score) and function. A team-care approach was utilised and co-ordinated by the Orthopaedic Surgeon. Other members included a physiotherapist, clinical psychologist and the pain care team. Results: All the patients received physical therapy (land and hydrotherapy). 5 patients received guanethidine blocks and 10 required psychological support. The time to diagnosis ranged from 2 to 11 months. All the patients improved over a period of 2.5–7 months. Conclusion: Early diagnosis with aggressive multidisciplinary goal oriented team approach should form the basis of management in children with RSD.

We have treated seven children with relapsed infantile Blount’s disease by elevation of the hemiplateau using the Ilizarov frame.

Three boys and four girls with a mean age of 10.5 years were reviewed at a mean of 29 months after surgery. All had improved considerably and were pleased with the results. The improvements in radiological measurements were statistically significant (p < 0.001). Three-dimensional CT reconstruction was useful for planning surgery. There were no major complications. The advantages of this technique are that in addition to elevation of the hemiplateau, rotational deformities and limb-length discrepancies may be addressed.

Limb lengthening and limb reconstruction using the Ilizarov system is a recognized treatment for children with congenital and acquired lower limb discrepancy and/or deformity. It is a complicated, costly, time consuming and challenging procedure for the multidisci-plinary team, the child and their family. Traditional outcome measures for this group of children tend to focus on X-ray appearances, lengthening indices and problems, obstacles and complications occurring during the treatment phase. At the present time there are unanswered questions as to their functional status as adults. The literature does not appear to have addressed this question as yet. For families considering this difficult treatment option, the potential function for their child as an adult may be valuable in the decision making process The aim of this study was to discover the physical, occupational and psychosocial function of a group of young adults who underwent Ilizarov procedures as children at Great Ormond Street Children’s Hospital.

Forty young adults, aged 18 – 27 years (mean age 19.9 years), who had Ilizarov procedures between 1992 and 2000, were sent questionnaires. Items included in the questionnaire were taken from the Toronto Extremity Salvage Score (TESS) to assess function and the Pediatric Orthopaedic Society of North America (POSNA) Adolescent Musculoskeletal Functional Health Questionnaire to assess psychosocial and occupational domains. Questions were also included to gather demographic information.

A total of 27 responses were received from 14 males and 13 females. Twenty four patients had Ilizarov procedures for limb lengthening or lengthening with deformity correction. Deformity correction only was carried out in three patients. Mean time since treatment was 5.6 years (range 9 – 2 years). A total of 24 tibial frames, 6 femoral, 4 whole leg and 2 foot frames were applied. Four patients had had repeat Ilizarov procedures. Six patients had had previous lengthenings using uniaxial fixators.

Functional ability indicators were high but activities such as kneeling, walking up and down slopes or hills, walking long distances and running were significant problems reported by more than half. Assistive devices (crutches, shoe raises, AFO, knee brace) were needed full time by five, with crutches, sticks and wheelchair used occasionally by three others.

Seven adults chose not to partake in sporting activity, with a further three finding it extremely hard and two impossible. Swimming was the most popular activity. Activity related pain was uncommon but pain in the limb requiring occasional analgesia was reported. All but one respondent worked full time or were students. Most occupations were office or shop based. Three men were manual workers. Time off work for problems related to their limb problem was minimal. Socialising with friends and family was high with only one respondent expressing extreme difficulty. Four men and five women identified scarring from the Ilizarov treatment as a cause of concern. Ten women and eight men raised body image issues. Twenty six adults said they would recommend Ilizarov treatment to others, if asked. Conclusions: We view this data as important to provide information for use when counselling It weill also help prospective patients and families of expectation for function in adult life.

We review the results of a modified quadricepsplasty in five children who developed stiffness of the knee after femoral lengthening for congenital short femur using an Ilizarov external fixator which spanned the knee.

All had a full range of movement of the knee before lengthening was undertaken. Unifocal lengthening was carried out in the distal metaphysiodiaphyseal region of the distal femur with a mean gain of 6.5 cm. The mean percentage lengthening was 24%.

At the end of one year after removal of the Ilizarov frame and despite intensive physiotherapy all patients had stiffness. Physiotherapy was continued after the quadricepsplasty and, at the latest follow-up (mean 27 months), the mean active flexion was 102° (80 to 130). The gain in movement ranged from 50° to 100°. One patient had a superficial wound infection which settled after a course of oral antibiotics. None developed an increased extension lag after surgery and all were very satisfied with the results. Quadricepsplasty is a useful procedure for stiffness of the knee after femoral lengthening which has not responded to physiotherapy.

Reflex sympathetic dystrophy is a syndrome characterised by pain and hyperaesthesia associated with swelling, vasomotor instability and dystrophic changes of the skin. It is rare in children, can occur without any previous history of significant trauma and may be recurrent and migratory.

We reported 13 new cases of RSD in children and emphasised the role of a multidisciplinary team approach in management. A review of the literature was included.

13 children (3 boys and 10 girls) with reflex sympathetic dystrophy were presented. They were aged between 8 and 17 years. Mean age at onset was 13 years 4 months. All of them had RSD involving the lower limbs. Thermography was performed in 10 cases. The average time to correct diagnosis was 4 months. Five ankles, 4 knees and 5 hips were involved (14 joints in 13 cases). Psychological assessments revealed abnormalities in all cases. Pain (visual analogue score) and function were assessed before and after treatment.

The most common therapy in children is progressive mobilisation supported by analgesic drugs, psychological and physical therapy. We individualised the therapy for each child. A team-care approach with the physiotherapist, psychologist and pain-care team co-ordinated by the Orthopaedic Consultant was the essence of our management. All children received physical therapy including a wide variety of non-standarised approaches involving analgesics and hydrotherapy. 5 patients received guanithidine blocks. Individual therapy was monitored with set achievable goals and weekly assessment of progression of mobility and joint motion.

Time from the first RSD episode to resolution averaged 6 months in our series [it was mean 10 weeks in the non-adolescent cases (8 cases) and 7 months in the adolescent one (5 cases)]. The pain and function scores improved remarkably in all patients.

RSD in children is not a widely recognised condition. There is often a delay in diagnosis in view of the rarity of the condition as well as the fact that specific diagnostic modalities are not readily available in all centres. Psychological factors should not be underestimated. Early diagnosis with an aggressive, multidisciplinary, monitored, ‘goal-oriented’ team approach should be the basis of management in these cases.

We present the results of treatment for relapsed infantile Blounts disease using a technique of hemiplateau elevation with the Ilizarov frame.

7 patients with a mean age of 10 years 6 months were reviewed at 30 months following hemiplateau elevation with/without ipsilateral tibial lengthening. Preoperatively clinical photographs, long leg standing radiographs and 3D computed tomography images were acquired. The patients were evaluated clinically (presence of knee pain, range of knee motion, knee stability and leg length discrepancy) and radiologically. Schoeneckers objective assessment was undertaken. The results were analysed statistically.

Clinically all the patients improved significantly. No patient had knee pain and the range of knee motion was from 0° to more than 100° of knee flexion. The radiological results and Schoeneckers grading are depicted below.

The improvement in radiological measurements were statistically significant. Pin site infection was present in all but settled with antibiotics.

The results of hemiplateau elevation for relapsed infantile Blounts disease are encouraging.