Aims: To review the hip subluxations or dislocations occurring during femoral lengthening in patients with congenital longitudinal lower limb deficiencies. Methods: Sixty-three patients with congenital longitudinal lower limb deficiencies underwent femoral lengthening using either De Bastiani, Villarubias or Ilizarov technique. Acetabular index, medial joint space, CE angle of Wiberg, acetabular angle of Sharp and neck-shaft angle were measured on anteroposterior radiographs of hip before, during and after lengthening. Acetabulum was considered dysplastic when the Sharp angle was more than 45 degrees. Hip was considered to be subluxed when the medial joint space increased during lengthening. Results: During femoral lengthening, eleven hips subluxed as measured by the increase in medial joint space and one hip dislocated. All these hips had a pre-operative acetabular index more than 25 degrees, CE angle less than 20 degrees and Sharp angle more than 45 degrees. The average neck-shaft angle was 75 degrees. Following subluxation, lengthening was stopped and the hips were reduced in hip spica after adductor and sartorius tenotomies. In one patient femoral shortening and acetabuloplasty had to be done to reduce the sub-luxation. No case of avascular necrosis or chondrolysis was noted. Conclusions: Hip subluxation during femoral lengthening of congenital longitudinal lower limb deficiencies tends to occur when there is associated ace-tabular dyplasia and femoral coxa vara. Careful preoperative assessment, if need be hip reconstruction prior to lengthening and close monitoring during lengthening is recommended.

Aims: To review the presentation, progression, treatment, and outcome of congenital posteromedial bow of tibia. Methods: Seventeen patients were studied using radiographs and medical records retrospectively. The time period was from 1989 to 2002. Data was collected with special reference to deformity correction and lengthening. Complications were analysed. Results: Eight of the patients were male and nine female; eleven of them had deformity correction and lengthening, whereas two are awaiting surgery, two underwent contralateral epiphysiodeses and one, periosteal stripping. Twelve were treated with the Ilizarov device, seven with LRS. The range of discrepancy pre – operatively was from 3 to 8.8 cm. Mean length gained was 3.7 cm with residual discrepancy within 0.6 cm. Complications noted were minor grades of infection and 3 patients required further corrective surgery. The bone-healing index was 62 days per cm. There was some decrease in ankle movement noted, this was unchanged or improved post-operatively. Conclusion: Successful simultaneous deformity correction and lengthening for this condition is possible. The Ilizarov frame provided more benefits in reducing complications. This is the largest series of lengthening and correction of this deformity published and the residual deformity and initial length discrepancy is greater than previously stated.

Aim: To assess the outcome of operative treatment of joint deformities using circular external fixators in arthrogryposis

Materials and Methods: 16 cases were identified in 9 children, who underwent application of Ilizarov external fixation from 1989 to 2000 at the Sheffield Children’s Hospital for progressive correction of knee and foot deformities. This treatment modality was combined with either a soft tissue release, soft tissue distraction or a bony correction. Clinical outcomes were assessed, and comparisons were made between the different treatment modalities. Results: Three fixed flexion deformities of the knee treated with progressive correction and soft tissue distraction all achieved initial correction, but recurred some time after removal of fixators. Out of five club-feet treated with an Ilizarov frame with progressive soft tissue distraction alone, three deformities recurred despite long term splinting. The remaining eight club-foot deformities were treated with a bony procedure combined with gradual correction in the circular frame, and all corrections were maintained at follow up. The average treatment time in the fixator was 17 weeks (12–50 weeks), and the average follow up time was 36 months. Complications included 4 pin track sepses, 1 osteitis requiring a sequestrectomy, 1 transient neurapraxia and 1 fracture following removal of the fixator.

Conclusion: The treatment of joint deformities in arthrogryposis remains challenging and difficult, and complications do occur. Combining the Ilizarov device with a bony procedure seems to have superior results and less recurrence of deformities than pure progressive soft tissue correction.

Aims: To test the hypothesis that there is a trend to over correction in talipes patients who demonstrate signs of generalised joint laxity.

Patients and Methods: 45 patients with an average age of 6.9yrs(3–16) were examined for generalised joint laxity using the Biro score. This gave 65 feet (20 bilateral) for clinical assessment using the podoscope and graded based on Tachdijans flat foot score.

Results: The results were assessed and the patients divided into 2 groups depending on whether or not they had joint laxity. This left 19 patients with 26 feet in the non-lax group and 26 patients with 39 feet in the lax group. The 2 groups were then compared to see if there was a difference in flat foot grade. In the non-lax group 2 patients showed evidence of over-correction whereas in the lax group 18 patients(25 feet)were over corrected to some extent. Using the fisher’s exact test there was a significant difference between the 2 groups with a trend towards over correction in those with generalised joint laxity (p=. 002).

Conclusion: Based on the findings of this study there is a correlation between generalised joint laxity and over correction in congenital talipes equinovarus.

Objective: To review the existing classifications in characterizing the pathological morphology of congenital lower limb deficiencies and their usefulness in planning limb reconstruction.

Methods: Ninety-five patients undergoing limb reconstruction were classified using existing classifications. Predominantly femoral deficiencies were classified using Aitken,Amstutz,Hamanishi,Gillespie andTorode,Fixsen and Lloyd-Roberts, Kalamchi, and Pappas systems and fibular deficiencies were classified using Coventry and Johnston, Achterman and Kalamchi, and Birch systems.

Results: All patients with predominantly femoral deficiencies also had associated shortening of ipsilateral tibia and fibula. Similarly, most patients with predominantly fibular deficiencies also had some associated shortening ipsilateral femur. Acetabular dysplasia, knee instability due to cruciate insufficiency and lateral femoral condylar hypoplasia were found in both femoral and fibular deficiencies. None of the existing classification systems were able to represent the complete pathologic morphology in any given patient. Due consideration of alignment, joint stability and length discrepancy of affected limb as a whole at the planning stage of reconstruction could not be ascertained using these classification systems. Instead, it was useful to characterize the morphology of the involved limb using the following method:

Acetabulum: Dysplastic/ Non-dysplastic (AC index, Sharp’s angle, CE angle)

Conclusion: Congenital longitudinal lower limb deficiency is a spectrum of disorder involving the entire lower limb. Existing classifications do not represent the complete morphology of the entire involved lower limb and therefore a systematic method of characterizing the morphology of the lower limb is more useful in planning limb reconstruction.

Objective: To review the hip subluxations or dislocations occurring during femoral lengthening in patients with congenital longitudinal lower limb deficiencies.

Methods: Sixty-three patients with congenital longitudinal lower limb deficiencies underwent femoral lengthening using either De Bastiani, Villarubias or Ilizarov technique. Acetabular index, medial joint space, CE angle of Wiberg, acetabular angle of Sharp and neck-shaft angle were measured on anteroposterior radiographs of hip before, during and after lengthening. The Acetabulum was considered dysplastic when the Sharp angle was more than 45 degrees. Hip was considered to be subluxed when the medial joint space increased during lengthening.

Results: During femoral lengthening, eleven hips sub-luxed as measured by the increase in medial joint space and one hip dislocated. All these hips had a preoperative acetabular index more than 25 degrees, CE angle less than 20 degrees and Sharp angle more than 45 degrees. The average neck-shaft angle was 75 degrees. Following subluxation, lengthening was stopped and the hips were reduced in hip spica after adductor and sartorius tenotomies. In one patient femoral shortening and acetabulo-plasty had to be done to reduce the subluxation. No case of avascular necrosis was noted.

Conclusion: Hip subluxation during femoral lengthening of congenital longitudinal lower limb deficiencies tends to occur when the acetabular index is more than 25 degrees, Sharp angle is more than 45 degrees, CE angle is less than 20 degrees and when there is associated femoral coxa vara. Careful preoperative assessment is required, and if need be hip reconstruction prior to lengthening. Close monitoring during lengthening is recommended.

We reviewed the outcome of 30 patients treated with an Ilizarov frame for resistant clubfoot deformity. Each patient was assessed using objective and subjective outcome measures. We used clinical examination, X-ray analysis, pedobarography and gait analysis and the Activities Scale for Kids questionnaire, developed and validated by The Hospital for Sick Children, Toronto, Canada.

The average questionnaire score was 83. This suggested a good subjective outcome when compared to the average score of 38 achieved by children with untreated clubfoot. Patients were into 2 groups using this score. Patients scoring over 75 were considered to have a good outcome and those scoring less than 75 were considered to have a bad outcome. The objective results were then compared.

We found no difference between the 2 groups using clinical examination and X-ray. Pedobarography showed lower pressures in the bad subjective group, in particular virtually no pressure was generated under the heel when walking.

The pressure distribution also showed the bad group to have the pressure balance towards the front of the foot over the 5^th metatarsal head.

Gait analysis showed differences. The bad group had increased pelvic obliquity and increased pelvic movement suggesting an inefficient gait, increased hip abduction in swing, hyperextension of the knee on loading and decreased dorsiflexion of the ankle in swing when compared to the good group.

Our conclusions were that subjectively this group of patients did well after surgical treatment using an Ilizarov frame.

Clinical examination can show significant intra- and inter-observer error and X-ray is unreliable in children whose feet are congenitally deformed. Pedobarography and gait analysis seem to correlate better with subjective outcome. We know that a good foot is a functional foot and it may be that functional assessment is a more appropriate means of assessing results of treatment in these patients.

Increased incidence of complications has been reported when lengthening limbs with underlying bone disorders such as dysplasias and metabolic bone diseases. There is a paucity of literature on limb lengthening in Osteogenesis Imperfecta (OI), probably due to the concern that the bone containing abnormal collagen may not tolerate the external fixators for a long term and there may not be adequate regenerate formation from this abnormal bone.

We performed limb lengthening and deformity correction of nine lower limb long bones in six children with OI. Four children were type I and two were type IV OI as per Sillence classification. The mean age was 14.7 years. All six children had lengthening for femoral shortening and three of them also had lengthening for tibial shortening on the same side. Angular deformities were corrected during lengthening. Five limb segments were treated using a monolateral external fixator and four limb segments were treated using an Ilizarov external fixator. In three children, previously inserted femoral intramedullary nails were left in situ during the course of femoral lengthening. The average lengthening achieved was 6.26 cm. Limb length discrepancies were corrected to within 1.5 cm of the length of the contralateral limb in five children. In one child with fixed pelvic obliquity and spinal scoliosis, functional leg length was achieved. The mean healing index was 33.25 days/cm of lengthening. Among the complications significant ones included, one deep infection, one fracture through the midshaft of the femur, and development of anterior angulation deformity after the removal of the fixator in one tibia. Abnormal bone of OI tolerated the external fixator throughout the period of lengthening without any incidence of migration of wires and screws through the soft bone when distraction forces were applied. The regenerate bone formed within the time that is normally expected in limb lengthening procedures performed for other conditions. We conclude that despite abnormal bone characteristics, limb reconstruction to correct limb length discrepancy and angular deformity can be done safely in children with OI.

The aim was to study the use of limb reconstruction techniques in the management of Ollier’s Dysplasia over a period of 25 years.

This was a retrospective review of case records and radiographs of patients who had lower limb reconstruction for deformity and limb length discrepancy. There were a total of 9 patients of whom 7 had reached maturity and four of these were still under follow up.

The major aims of surgery were to correct lower limb length discrepancy and deformity. A total of twenty segments were operated upon. These were 11 femurs and 9 tibiae. In some segments repeated surgery was required. 41 index and 54 secondary procedures were necessary giving an average of 10.5 procedures per patient. The most common problems were difficulty in fixation in abnormal bone, premature consolidation reflecting the rich osteogenic potential and growth related recurrence of deformities and discrepancy. The mean length gained was 13.8 cms per patient. Healing of regenerate occurred with radiologically normal appearance even in chondro-dysplastic areas. All patients who had completed treatment had a satisfactory mechanical axis and the mean length discrepancy was 1.7 cms.

Patients with Ollier’s dysplasia appear to respond well to limb reconstructive surgery. It is possible to correct severe limb length discrepancies and angular deformities. Surgeons should be aware of the possibility of premature healing and should consider faster lengthening rates of up to 1.5 mms per day. Distraction should begin early by day 5 or less. Immature patients should be warned about the possibility of recurrence of deformity and possible need for repeated surgery.