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

Type IIb, so called mobiled pseudoarthrosis according to Paley classification, is characterized by congenital pseudoarthrosis of proximal femur with an isolated small and stiff femoral head. We are unable to create a moveable hip joint but appropriate length of the affected extremity can be reached by gradual lengthening. In previous classification it is known as Type Aitken C or Pappas III. Type IIIa, with diaphysial deficiency of femur, corresponds to Type D according to Aitken or to Type I and II according to Pappas. In Type IIIa, the knee joint is developed and functional with the ROM more than 45 degrees. In Type IIIb the knee joint is more or less stiff and functionally unuseable. These three groups present the most severe congenital short femur deformities, but their occurrence is fortunately very seldom – less than 1 in 300 thousand live births. Among 41 cases of congenital short femur Pappas I–IV which were collected during 30 years from the Czech population of 10 million – Pappas I was seen in one case, Pappas II in five cases, Pappas III in 16 cases and Pappas IV in 19 cases. From the 16 cases of Pappas III deformity was found in three of them – stiffness of isolated femoral head was found and these three patients were added to this group. Method of Treatment: In Type IIb we use complex treatment consisting of six consecutive steps:. Distraction of the distal part of femur up to acetabular level. Connection between head and diaphysis. First femoral lengthening. Lengthening of the tibia. Contralateral epiphysiodesis around the knee. Plastic surgery. Lengthening between 15 and 39 cm was reached. In Type IIIa, ilio-femoral fusion (knee-for-hip procedure) was performed in five cases. The functional results are excellent. There was no need for Syme amputation or rotationplasty. The prerequisite is at least 60 degrees arc of motion in the knee joint. Severe restricted ROM in the knee joint may lead to pseudoarthrosis. In Type IIIb (2 cases), the residual fragment of distal femur with unfunctional knee joint was stabilized in socket formed after pelvic osteotomy in the level of original acetabulum. The removal of telescopic proximo-distal movement stabilized the supportive function of the extremity

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

Introduction: Calcaneal lengthening osteotomy as a treatment for severe flat foot was described by Evans (1975) and his indications were expanded by Mosca (1995). Materials and methods: We reviewed 28 feet operated on in 21 patients with neuromuscular processes (17) and with flat foot-valgus associated with congenital short Achilles tendon (11). The mean age was 12.2 (6–18) and mean follow-up was 7.3 years (4–11). In all cases the associated Achilles tendon was lengthened. We evaluated the subjective and objective clinical results (Kitaoka, 1994) and the radiological parameters. Results: Subjective clinical: excellent and good 89.28% (100% in neuromuscular, 72.7 in short Achilles tendon). Objective clinical: (preop 64.25, postop 90.06 at 3 years, 86.13 at 7 years). The worsening of outcomes was due to three fair or poor results in the short Achilles tendon group (one required triple bone fusion). All but one presented complete subtalar mobility. Normal alignment in all but one, with slight valgus; none in varus. Conclusions: This surgical procedure gave excellent long-term results: subjective, nearly 90% excellent; objective: radiological anatomical correction, subtalar and tarsal mobility maintained. Its outcome was comparatively worse in the short Achilles tendon group than in the neuromuscular cases

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.

Background: The occurrence of congenitally short metatarsals is associated with an abnormal gait and an aesthetically displeasing appearance. Similarly, short metacarpals result in severe cosmetic disfigurement, particularly in young female patients. Methods: We examined and performed bone lengthening surgery in 13 female and 2 male patients. Of these, procedures were conducted on 12 metatarsals of 8 patients, 4 metacarpals and 1 metatarsal of a single patient, 1 metacarpal and 1 metatarsal of a single patient and 7 metacarpals of 5 patients. The mean age of the patients who underwent metacarpal procedures was 14.5 (10–21) years while the mean age of those who underwent metatarsal procedures was 17.5 (10–25) years. The callotasis method was employed for these procedures and we used either a unilateral external fixator and/or a circular external fixator. Results: The mean healing index and increase in metacarpal length was 1.6 (1.1–2.3) months/cm and 17.6 (13–26) mm, respectively. The mean follow-up period for patients who underwent metacarpal lengthening was 57.5 (12–96) months. The mean healing index and increase in metatarsal length was 1.6 (1.0–2.0) months/cm and 24.3 (20–30) mm respectively. The mean follow-up period for patients who underwent metatarsal lengthening was 48.3 (12–72) months. The preoperative AOFAS (American Orthopaedic Foot and Ankle Society) scores were good in 5 and excellent in 9 cases. The functional scores of metatarso-phalangial (MTP) joint of lengthened metatarsals for the lesser toe were excellent in 12 and good in 2 cases based on the AOFAS scoring system. All patients who underwent metacarpal lengthening reported that they were satisfied and could conduct their daily activities with good functional and aesthetic results. Complications included 4 angulations, 1 subluxation and 1 non-union and were seen in 6 of the metatarsal lengthening cases that exceeded 40% (or > 20 mm) of the total length of the original bone. Interpretation: There are recommendations in the literature that allow for the avoidance of severe complications and for the shortening of the consolidation period. We conclude that the periosteum must be protected with percutaneus osteotomy and lengthening should be performed at a rate of 0.25 mm twice a day, should not exceeding 40% of the original bone length (or > 20 mm). If the anticipated lengthening exceeds these predefined values then we suggest that the procedure should be performed using a circular external fixator with temporary fixation of the MCP or the MTP joint and the inclusion of the proximal phalanx in the frame

Introduction: Lengthening for congenital femoral hypoplasia is associated with a significant refracture rate and problems in recovery of knee motion. We present a series of 7 patients where two techniques of lengthening were used and recovery of knee motion compared.

Methods: This is a retrospective study. The outcome of interest was recovery of knee motion. All children were diagnosed to have congenital femoral hypoplasia. In two patients (group A), conventional metaphyseal osteotomies of the femur and tibia, with ankle and knee bridging fixators were applied. In the remaining five (group B), a combination of soft tissue releases, patella ‘capture’, a modified external fixator configuration and early conversion to internal fixation was used. Osteotomies of tibia and femur were performed in the mid-shaft and lengthening progressed at 0.75 mm per day. At 4 months, before regenerate consolidation, both femur and tibia were plated using a submuscular technique. The limb was supported in a cast, which was replaced by a brace at 3 weeks and knee motion exercises started. Lengthening was kept to within 15% in both groups.

Results: Recovery in knee flexion to greater than 90 degrees was accomplished by 4 of the 5 Group B patients by 4 months. One patient failed to attend for physiotherapy and did not progress with knee motion recovery. One patient sustained a fracture proximal to the submuscular plate which needed revision surgery. This did not hinder progress with knee motion recovery. In comparison, patients in group A reached 90 degrees of flexion at 12 months with one patient not exceeding 85 degrees at final follow up and subsequently needing a quadricepsplasty.

Conclusion: Recovery of knee motion may be assisted by soft tissue releases in combination with early conversion to internal fixation.

Introduction. We present the first 12 consecutive patients, undergoing elective paediatric limb reconstruction with an external fixator, for the 12-month period October 2020-October 2021. This is a single surgeon series for a newly appointed Consultant with limited previous experience. Arrangements were made for mentoring by a senior surgeon recently retired from the NHS but still active in private practice. Materials and Methods. The average age of patients was 10.5years at the time of frame application (5—15 years). Four frames in three patients were for Blount's; two for sequelae of NF1; two for posteromedial tibial bow with shortening, two for fibula hemimelia; one congenital short femur, one for sequelae of neonatal sepsis and one for bone loss following tumour resection. Results. We present early outcomes and complications for this challenging cohort. Two frames were revision frames, and a further two had had previous frame treatment. Where shortening was a primary component of the deformity (six cases) the mean lengthening required was 5cm (4–6cm). Conclusions. The benefits of this arrangement include enabling newly appointed Consultants to take on complex cases from the start of appointment, dealing with enormous waiting pressures and minimising complications. The BOA reported this year that 25% of the over 45 consultant workforce intend to retire within three years. In this context, a renewed focus is needed on succession planning, proleptic appointments, and novel schemes to retain experienced surgeons within complex NHS practice: we present a successful example of this strategy

Purposes:. See if permanent damage of the growth plate after physeal distraction is the rule and. Identify factors with influence on the viability of the physis after physeal distraction. Introduction: Surgeons have always been concerned about the fate of the growth plate after physeal distraction and for that reason this technique has usually been considered only in patients nearing maturity. Previous experimental work has shown that the velocity of distraction has an influence on the viability of the growth plate at follow-up (recommended rate: 0.5 mm/day). Clinically, it has also been our observation that the condition of the physis prior to distraction is another important factor related to physeal function in the long term. Patients and methods: Since 1987 we have used low velocity physeal distraction in 43 bone segments of which 37 cases have been followed-up at least for 24 months and this has been the group included in this study. The indications were lengthening (14), angular deformity correction (19) and resection of benign bone tumours (4). Most patients (24) were older than 10 y.o. and 22 of them were followed-up until maturity. We have retrospectively reviewed these patients looking at the radiological morphology and function of the distracted growth plate at follow-up. Results: Out of the 24 children older than 10 y.o., twenty showed a premature complete physeal closure. We looked with interest at the 13 cases younger than 10 y.o. since the repercussions of iatrogenic physeal damage would obviously be bigger in this age group. Five out of the 13 showed premature closure and in the remnant eight an open growth plate was observed at follow-up. All patients with open and/or functioning physes after distraction had no local injuries in the growth plate prior to distraction (4 congenital short femora and 4 normal physes). On the contrary, four out of the five cases with prematurely closed physes, had a local physeal damage prior to distraction (3 bony bridges and one non-union), and the remnant was a congenitally short femur. Growth after distraction was difficult to assess in the congenitally short femora but it has been very satisfactory in the 4 cases of previously normal physes (2 benign tumours and 2 femoral shortenings due to hip disorders). In three cases of congenital short femur in pre-teenagers we were able to repeat distraction twice through the same physis, since it had remained open after the first distraction. Conclusions: Physeal premature closure often follows physeal distraction, but not always. The condition of the physis prior to lengthening is an important factor with influence on its viability after distraction

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

Forearm lengthening in children is controversial. Paley (1990) and Peterson (1994) advocate aggressive treatment of the deformity for cosmetic and functional reasons. Scoenecker (1997) has shown that mature patients are comfortable with their appearance and functional deficit. We reviewed 8 forearm lengthenings performed in 8 children in the 14 year period from 1991 to 2004. Five patients had ulnar shortening (osteochondromata = 4, growth arrest due to trauma = 1). Of the three patients with radial shortening, one was due to a congenital short radius and two following growth arrest (post trauma and meningococcal septicemia). The shortening resulted in a cosmetically unacceptable ulnar or radial tilt with absent radial or ulnar deviation of the wrist and decreased supination and/or pronation. One patient with a proximal ulnar osteochondroma had a dislocation of the radial head with cubitus varus. Excision of the osteochondroma was done 6 months prior to lengthening. Lengthening was accomplished with two Ilizarov rings and a distal corticotomy for radial and proximal for ulnar shortening. Reduction of the dislocated radial head was achieved with an olive wire. Associated procedures were: hemiepiphyseal stapling of the distal radius for an increased radial articular angle in 3 patients with osteochondroma, and corrective osteotomy of the distal radius in 1 patient with growth arrest. The average lengthening obtained was 23 mm (range 13–40 mm) with an average lengthening index of 1.45 months per cm. At an average follow-up of six years (range 2–15 years; 7 to maturity) all patients were satisfied with the cosmetic improvement and had full radial and ulnar deviation. Except for two patients the supination/pronation was improved. We concluded the forearm lengthening is warranted for cosmetic and functional reasons