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

1. Twenty patients with congenital short tendo calcaneus are described. 2. All were treated by tendon lengthening and followed up for one and a half to seven years

Seventy patients with 91 congenital short femora are classified. Deformities resulting maternal Thalidomide treatment are compared with those where Thalidomide was not involved and genetic and epidemiological factors investigated in 50 patients. No essential anatomical difference was found between the two groups of femora but the whole complex of abnormalities differed: the Thalidomide group showed femur-tibia-radius anomalies while the non-Thalidomide garoup had femur-fibula-ulna anomalies, indicating either different aetiological factors or different timing of the insult to the foetus. Some differences between congenital coxa vara and congenital short femur associated with coxa vara are mentioned. Simple hypoplasia of the femur may possibly have a multifactorial genetic background since it is associated with other minor abnormalities of the limbs in these families, whereas environmental factors only are associated with the more severe femoral defects

We have reviewed the results in 37 patients with unilateral congenital short femur (Pappas classes III to IX), treated by different lengthening procedures. The increase in the length of the femur varied from 15.6% to 142%, excellent or good results being obtained in 32 patients (86%). There was an average of 1.9 complications per case, most being seen earlier in the series when the Wagner technique was used. With the Orthofix and the Ilizarov techniques, we used callus distraction in all cases. We found that the proximal diaphysis of the congenitally abnormal femur healed less well, and we now prefer to perform corticotomy and callus distraction of the distal metaphysis. The Ilizarov method gave the best results, offering the possibilities of the simultaneous use of a Hoffmann fixator across the hip and the treatment of knee dislocation and instability

Forty-three patients with unilateral congenital short tibia with partial or complete absence of the fibula are reviewed. The factors influencing the degree of leg shortening at maturity are considered. Serial radiographic measurements of leg length in fourteen patients covering an average observation period of 9-3 years support the hypothesis that the relative difference in growth between the two limbs remains remarkably constant. By estimating the percentage difference between the normal and abnormal leg lengths on the first measurable radiograph it is therefore possible to predict the likely shortening at maturity. This method of prediction allows the surgeon to make the decision to proceed to Syme's amputation or to the use of an extension prosthesis at about one year of age when the child starts to walk

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

1. Nineteen patients with congenital shortening of the femur without associated coxa vara have been examined and discussed.

2. The diagnosis is made on finding a short, bulky thigh, held in lateral rotation. The radiographs commonly show no abnormality apart from shortening, but delay in ossification of the head of the femur, with lateral bowing and cortical sclerosis of the shaft, are occasionally present. The overall shortening of the limb seldom exceeds three inches.

3. The place of various surgical procedures to control limb length is briefly discussed.

We describe 11 children with fibular hypoplasia and three- or four-ray feet, two bilaterally. This deformity is a less severe form of the better known congenital short tibia with absent or dysplastic fibula. If the leg-length discrepancy is minor, no treatment or a simple orthosis are used. Epiphysiodesis or leg lengthening by callotasis is appropriate for mild to moderate discrepancy. If the foot is non-functional, or the projected leg-length discrepancy is too great, early amputation and a prosthesis are advised

We performed intraoperative arthrography of the knee in 12 children with congenital short femur, Blount’s disease or Ollier’s disease in whom the Ilizarov technique was used for correction of deformity, leg lengthening or both. In each case, arthrography revealed a joint surface considerably different from that assumed from plain radiographs, and resulted in a change in the placement of our reference wires before application of the frame. This gave significant improvement in the mechanical axis obtained at the time of removal of the frame. The technique is safe, cheap and easy to perform. It is a useful adjunct to the application of the Ilizarov frame when used for complex lengthening and correction of deformity in the leg

Aims

Distraction osteogenesis with intramedullary lengthening devices has undergone rapid development in the past decade with implant enhancement. In this first single-centre matched-pair analysis we focus on the comparison of treatment with the PRECICE and STRYDE intramedullary lengthening devices and aim to clarify any clinical and radiological differences.

We performed one-stage lengthening using intercalary autogenous bone graft in 34 metatarsals and seven proximal phalanges in 21 patients with congenitally short metatarsals. At operation, in order to decrease the tension in the surrounding soft tissues, we gradually distracted the osteotomies of the affected bones for 20 to 30 minutes. The patients, all women, were followed up for a mean period of 2.1 years (1 to 6.5). The average gain in length for the 34 metatarsal procedures was 14 mm (6 to 21), equivalent to an increase of 32% (11 to 51), and for the seven proximal phalangeal lengthenings 8 mm (5 to 11), an increase of 54% (47 to 65). There was no evidence of neurovascular impairment. The technique of gradual distraction during operation is simple and effective. It overcomes the disadvantages of one-stage lengthening such as a small gain in length and neurovascular damage

Axial forces were measured during limb lengthening in a series of ten patients with varying pathologies in order to assess the mechanical characteristics of the distracted tissues and the levels of axial force to which soft tissues are subjected during leg lengthening. The pattern of force was found to vary according to the underlying pathology. For post-traumatic shortening in adults both the peak and the resting forces rose steadily during lengthening reaching maximum forces of the order of 300 N. Patients with congenitally short limbs developed very high peak forces (in some cases over 1000 N) and also showed large amounts of force relaxation (typically 400 to 500 N). When very high levels of force were recorded, there was a higher complication rate. In particular, there was a high instance of angular deformity. This occurred because the loads encountered resulted in failure of some of the external fixation frames

Three amputated legs with tibial dysplasia were studied by radiography, arteriography and anatomical dissection. The radiographic appearances were the same as the Type 1b tibial dysplasia described by Jones, Barnes and Lloyd-Roberts (1978) in that the tibiae were absent but the lower femoral epiphyses were normal. However, our anatomical findings differed from those of Jones et al. since no bony or cartilaginous anlage of the proximal tibia was found in any of the three legs. The pattern of vascular anomaly was identical in the three legs and similar to the findings of Hootnick et al. (1980) in congenital short fibula. Congenital fusion of the subtalar joint was a constant finding. These results support the hypothesis that the arterial and skeletal systems are vulnerable to a teratogenic insult in the fifth week of embryonic life. The bony and arterial anomalies should be borne in mind by the surgeon attempting reconstructive surgery for this condition

Aims

The Precice intramedullary limb-lengthening system has demonstrated significant benefits over external fixation lengthening methods, leading to a paradigm shift in limb lengthening. This study compares outcomes following antegrade and retrograde femoral lengthening in both adolescent and adult patients.

Aims

Computer hexapod assisted orthopaedic surgery (CHAOS), is a method to achieve the intra-operative correction of long bone deformities using a hexapod external fixator before definitive internal fixation with minimally invasive stabilisation techniques.

The aims of this study were to determine the reliability of this method in a consecutive case series of patients undergoing femoral deformity correction, with a minimum six-month follow-up, to assess the complications and to define the ideal group of patients for whom this treatment is appropriate.

This retrospective study compared post-operative epidural analgesia (E), continuous peripheral nerve blocks (CPNB) and morphine infusion (M) in 68 children undergoing limb reconstruction with circular frames. The data collected included episodes of severe pain, post-operative duration of analgesia, requirement for top-up analgesia, number of osteotomies, side effects and complications. There was a significant difference between the number of episodes of severe pain in patients receiving a morphine infusion and those receiving epidurals or CPNB (M vs E, p < 0.0001; M vs CPNB, p = 0.018). The CPNB group was associated with the lowest incidence of episodes of severe pain and top-up analgesia. Epidural analgesia was associated with significantly more nausea and vomiting than morphine infusion (p = 0.053) and CPNB (p = 0.023). It also had a significantly higher incidence of motor blockade than CPNB (p < 0.01). We found that the most effective method of post-operative analgesia for children undergoing lower limb reconstruction was sciatic nerve catheterisation and continuous infusion.

Van Nes rotationplasty may be used for patients with congenital proximal focal femoral deficiency (PFFD). The lower limb is rotated to use the ankle and foot as a functional knee joint within a prosthesis. A small series of cases was investigated to determine the long-term outcome. At a mean of 21.5 years (11 to 45) after their rotationplasty, a total of 12 prosthetic patients completed the Short-Form (SF)-36, Faces Pain Scale-Revised, Harris hip score, Oswestry back pain score and Prosthetic Evaluation Questionnaires, as did 12 age- and gender-matched normal control participants. A physical examination and gait analysis, computerised dynamic posturography (CDP), and timed ‘Up & Go’ testing was also completed. Wilcoxon Signed rank test was used to compare each PFFD patient with a matched control participant with false discovery rate of 5%.

There were no differences between the groups in overall health and well-being on the SF-36. Significant differences were seen in gait parameters in the PFFD group. Using CDP, the PFFD group had reduced symmetry in stance, and reduced end point and maximum excursions.

Patients who had undergone Van Nes rotationplasty had a high level of function and quality of life at long-term follow-up, but presented with significant differences in gait and posture compared with the control group.

Cite this article: Bone Joint J 2013;95-B:192–8.

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.

We report the results of intramedullary leg lengthening conducted between 2002 and 2009 using the Intramedullary Skeletal Kinetic Distractor in 69 unilateral lengthenings involving 58 femora and 11 tibiae. We identified difficulties that occurred during the treatment and assessed whether they were specifically due to the implant or independent of it. Paley’s classification for evaluating problems, obstacles and complications with external fixators was adopted, and implant-specific difficulties were continuously noted. There were seven failures requiring premature removal of the device, in four due to nail breakage and three for other reasons, and five unsuccessful outcomes after completion of the lengthening. In all, 116 difficulties were noted in 45 patients, with only 24 having problem-free courses. In addition to the difficulties arising from the use of external fixators, there were almost the same number again of implant-specific difficulties.

Nevertheless, successful femoral lengthening was achieved in 52 of the 58 patients (90%). However, successful tibial lengthening was only achieved in five of 11 patients (45%).

We carried out a retrospective study to assess the clinical results of lengthening the fourth metatarsal in brachymetatarsia in 153 feet of 106 patients (100 female, six males) using three different surgical techniques. In one group lengthening was performed by one-stage intercalary bone grafting secured by an intramedullary Kirschner-wire (45 feet, 35 patients). In the second group lengthening was obtained gradually using a mini-external fixator after performing an osteotomy with a saw (59 feet, 39 patients) and in the third group lengthening was achieved in a gradual manner using a mini-external fixator after undertaking an osteotomy using osteotome through pre-drilled holes (49 feet, 32 patients). The mean age of the patients was 26.3 years (13 to 48). Pre-operatively, the fourth ray of the bone-graft group was longer than that of other two groups (p < 0.000). The clinical outcome was compared in the three groups. The mean follow-up was 22 months (7 to 55).

At final follow-up, the mean lengthening in the bone-graft group was 13.9 mm (3.5 to 23.0, 27.1%) which was less than that obtained in the saw group with a mean of 17.8 mm (7.0 to 33.0, 29.9%) and in the pre-drilled osteotome group with a mean of 16.8 mm (6.5 to 28.0, 29.4%, p = 0.001). However, the mean time required for retention of the fixation in the bone-graft group was the shortest of the three groups. Patients were dissatisfied with the result for five feet (11.1%) in the bone-graft group, eight (13.6%) in the saw group and none in the pre-drilled osteotomy group (p < 0.000). The saw group included eight feet with failure of bone formation after surgery. Additional operations were performed in 20 feet because of stiffness (n = 7, all groups), failure of bone formation (n = 4, saw group), skin maceration (n = 4, bone-graft group), malunion (n = 4, bone-graft and saw groups) and breakage of the external fixator (n = 1, saw group).

We conclude that the gradual lengthening by distraction osteogenesis after osteotomy using an osteotome produces the most reliable results for the treatment of fourth brachymetatarsia.