For decades, the classic indication for limb lengthening has been reserved for anisomelia that was expected to reach or exceed 5 cm at maturity. Epiphysiodesis was reserved for discrepancies in the 2–5 cm range. With the increasing sophistication of fixators, including rail, hexapod, and hybrid, complex deformities may be corrected simultaneously while moderate to extreme lengthening is achieved. More recently, iterations of telescoping intramedullary rods have further strengthened our armamentarium. Meanwhile, permanent epiphysiodesis techniques, both open and percutaneous, have yielded to more versatile and reversible tethering of one (angle) or both (length) sides of a physis. While the techniques of guided growth and callotasis seem to be diametrically opposed, they may be used in a tandem or complementary fashion, for the benefit of the patient. If treatment is undertaken during skeletal growth, one must be aware that issues remain regarding the accurate assessment of skeletal maturity and prediction of the ultimate outcome. Therefore, there is potential for over- or undercorrection. Reversible and serial guided growth now enable the surgeon to commence intervention at a comparatively young age, for the purpose of optimizing limb alignment and reducing the ultimate discrepancy. Frame application may be delayed or, in some cases, avoided altogether. With the limb properly aligned at the outset of lengthening, elective use of a telescoping intramedullary nail may now be favored over a frame accordingly.

Tibial hemimelia is a rare congenital lower limb deficiency presenting with a wide spectrum of associated congenital anomalies, deficiencies and duplications. Reconstructive options have been limited, and the gold standard for treatment has remained amputation with prosthetic fitting. There is now a better understanding of the genetics, etiology and pathoanatomy of tibial hemimelia. Armed with this knowledge, I present here a new classification to guide treatment and prognosis and then discuss new treatment strategies and techniques for limb reconstruction based on this new classification scheme.

Fibular hemimelia presents with foot deformity and leg length discrepancy. Previous classifications have focused on the degree of fibular deficiency rather than the type of foot deformity. Published methods of surgical reconstruction have often failed due to residual or recurrent foot deformity. The purpose of this report is to introduce new classification and reconstruction methods. The Paley SHORDT procedure is used to stabilize the ankle when there is a hypoplastic distal fibula with a dynamic valgus deformity. It involves shortening and realignment of the distal tibia relative to the fibula. In contrast, the Paley SUPERankle procedure is used when there is a fixed equinovalgus foot deformity. The SUPERankle uses a supramalleolar shortening-realignment osteotomy and/or subtalar osteotomies with anlage resection. Due to the bony instead of soft tissue correction of deformity, residual or recurrent deformity is prevented. Weakening of gastro-soleus and peroneal muscles is avoided by shortening of the tibia instead of tendon lengthening. The limitation of ankle motion is related to ankle dysplasia rather than surgery or lengthening. A plantigrade-stable foot and ankle leads to an excellent functional result comparable or better than a Syme’s amputation with prosthetic fitting. Serial lengthening procedures combined with the SHORDT or SUPERankle reconstruction lead to limb length equalization with a plantigrade, painless, functional foot.

Tibialis anterior tendon transfers (TATT) are commonly performed in young children following Ponseti casting for clubfeet. The classic TATT involves advancing the tendon through a hole drilled in the ossified cuneiform. The aim of this study was to determine if tendons transferred through unossified bones have untoward effects on subsequent bone development.

Twenty-five piglets underwent one of five surgical procedures. An 18-gauge needle was then used to place a tunnel through the bony or cartilaginous portion of the calcaneus (through direct visualization) and isolated slips of the flexor digitorum superficialis (FDS) were placed through the tunnels, as determined by surgical procedure. Radiographic and/or histologic evaluations of the calcaneal apophyses were then performed. A discrete (1–4) and dichotomous “Normal” or “Abnormal” scoring system was developed and its reliability assessed to grade the appearance of the calcanei. Calcaneal appearances following the surgical procedures were then compared with controls. The average load to failure of a subset of transferred tendons was then compared using an MTS machine.

The proposed apophyseal grading system (1–4) demonstrated an intraclass correlational coefficient (ICC) for consistency of 0.92 [95% confidence interval (CI) 0.88 < ICC < 0.95] and ICC for agreement of 0.91 (95% CI 0.86 < ICC < 0.95), indicating strong agreement and consistency. Similarly, Fleiss’ kappa for the 1–4 scoring system was found to be 0.67, indicating substantial agreement between reviewers. When the 1–4 system was translated into the dichotomous scheme “Normal” and “Abnormal”, the kappa value increased to 0.94, indicating strong agreement. Forty-six apophyses (13 control and 33 operative) were assessed using this scoring scheme. Apophyseal transfers were significantly more abnormal than controls (p < 0.0001), while no difference in abnormalities was found following tunnel placement alone (p = 1). Mechanical testing of the tendons transferred to bone or through the cartilaginous apophysis demonstrated no significant differences (p = 0.2).

Tendon transfers through unossified bones altered subsequent bone development.

While the long-term consequence of these structural changes is unknown, these findings suggest that tendon transfers through unossified bones should be avoided and alternative methods of tendon fixation explored.

The prognosis of limb length discrepancy is a major subject in paediatric orthopaedic surgery. The strategy depends on the prognosis and must be adapted to each patient. The residual growth of the lengthened segment often remains unknown, but is dependent on age, the percentage of lengthening and other factors. Using a large cohort of 150 children who had undergone bone lengthening procedures, we describe five patterns of post-intervention growth and identify factors that are favourable for normal residual growth. The criteria for bone lengthening which should maintain good residual growth are—bone age at lengthening should be before the pubertal growth spurt; the interval between two lengthening procedures should be over three years; the percentage of lengthening should be <30% of the initial segment; and no more than two lengthening procedures should be carried out during infancy.

The epidemiology and risk factors for developmental dysplasia of the hip (DDH) are still being refined. We investigated the local epidemiology of DDH in order to define incidence, identify risk factors, and refine our policy on selective ultrasound screening.

With a cohort study design, data were prospectively recorded on all live births in our region from January 1998 to December 2008. We compared data on babies treated for DDH with data for all other children. Crude odds ratios (ORs) were calculated to identify potential risk factors. Logistic regression was then used to control for interactions between variables.

There were 182 children born with DDH (with a total of 245 dysplastic hips) and 37,051 without. The incidence was 4.9 per 1000 live births. Female sex (adjusted OR 7.2, 95% confidence interval [CI] 4.6–11.2), breech presentation (adjusted OR 24.3, 95% CI 13.1–44.9), positive family history (adjusted OR 15.9, 95% CI 11.0–22.9) and first or second pregnancy (adjusted OR 1.8, 95% CI 1.5–2.3) were confirmed as risk factors (p < 0.001). In addition, there was an increased risk with vaginal delivery (adjusted OR 2.7, 1.6–4.5, p < 0.001) and post-maturity (OR 1.7, 1.2–2.4, p < 0.002).

One in 200 children born within our region requires treatment for DDH. Using both established and novel risk factors, we can potentially calculate an individual child’s risk. Our findings may contribute to the debate regarding selective versus universal ultrasound screening.

Prognostic Study: Level 1.

The aim of this study was to evaluate the effect of limb malformations on health-related quality of life (HRQL) and function of the extremities in middle-aged individuals with thalidomide embryopathy (TE). Between 1959 and 1962, approximately 150 children with multiple malformations were born in Sweden following the maternal intake of thalidomide during pregnancy, of whom 100 survived.

Thirty-one individuals with TE underwent evaluations of musculoskeletal manifestations by clinical examination. Validated questionnaires were used for the assessment of general HRQL [the 36-Item Short Form Health Survey (SF-36) and the EuroQ Five Dimensions health questionnaire (EQ-5D)]. The function of the upper and lower extremities was evaluated using specific questionnaires (Disabilities of the Arm, Shoulder and Hand scale and Rheumatoid and Arthritis Outcome Score, respectively). The lower limbs were evaluated by computed tomography. The median age of the study group was 46 years, and 42% were females. Twenty-five individuals had malformations of the hand, but 27 had a grip function. Five individuals had severe lower limb malformations. Individuals with at least one extremity with major malformation(s) that affected function (n = 15) were compared with those without (n = 16).

The physical HRQL for the entire study group [mean 40.6, 95% confidence interval (CI) 35.4–45.8], as evaluated by the Physical Composite Score (PCS) of the SF-36, was significantly lower than the national norm value (population-based norm) of 50.0, and the physical HRQL of the subgroup with major limb malformations (15/31) was even lower (mean 34.6, 95% CI 25.9–43.4). The mental aspects of HRQL, based on SF-36 and EQ-5D scores, were not affected in the entire study group or in the subgroups.

The physical quality of life was significantly lower in individuals with TE compared with the general national population, while the mental aspects were not affected.

IV.

Unlike external fixators, the use of solid intramedullary lengthening nails is restricted to defined anatomical preconditions, such as an adequate bone length. Furthermore, all deformity corrections except the lengthening procedure have to be implemented intraoperatively and cannot be adjusted postoperatively. Conversely, even complex deformity corrections can be performed using intramedullary devices after a thorough preoperative planning. For preparation of the intramedullary cavity as well as positioning of the lengthening nail according to the preoperative planning, reaming the medullary canal with rigid reamers which don’t follow the line of least resistance is inevitable. However, the application of solid lengthening nails might be limited, especially in children with ongoing epiphyseal growth, although a central perforation of the growth plate was shown to have no adverse effects on the growth potential. In cases with complex or multilevel deformities, an additional osteotomy and locking plate fixation could sometimes be a valuable solution in order to avoid external fixation. The low complication rate as well as the reduced compromising of soft tissues and periosteum render intramedullary lengthening nails the state-of-the-art procedure for limb lengthening in combination with deformity correction in patients who meet the anatomical preconditions.

Pin-tract infection (PTI) is the most commonly expected problem, or even an almost inevitable complication, when using external fixation. Left unteated, PTI will progress unavoidably, lead to mechanical pin loosening, and ultimately cause instability of the external fixator pin–bone construct. Thus, PTI remains a clinical challenge, specifically in cases of limb lengthening or deformity correction. Standardised pin site protocols which encompass an understanding of external fixator biomechanics and meticulous surgical technique during pin and wire insertion, postoperative pin site care and pin removal could limit the incidence of major infections and treatment failures. Here we discuss concepts regarding the epidemiology, physiopathology and microbiology of PTI in paediatric populations, as well as the clinical presentations, diagnosis, classification and treatment of these infections.

Distraction osteogenesis biologically resembles fracture healing with distinctive characteristics notably in the distraction phase of osteogenesis. In the latency phase of bone lengthening, like in the inflammatory phase of fracture repair, interleukines are released and act with growth factors released from platelets in the local haematoma, leading to attraction, proliferation and differentiation of mesenchymal stem cells into osteoblasts and other differentiated mesenchymal cells. These in turn produce matrix, collagen fibers and growth factors. A callus containing cells, collagen fibers, osteoid and cartilage matrix is formed. Provided stable fixation, distraction will trigger intramembranous bone formation. As distraction proceeds, the distraction gap develops five distinctive zones with unmineralized bone in the middle, remodelling bone peripherally, and mineralizing bone in between. During consolidation, the high concentration of anabolic growth factors in the regenerate diminishes with time as remodelling takes over to form mature cortical and cancellous bone. Systemic disease, congenital bone deficiencies, medications and substance abuse can influence the quality and quantity of regenerate bone, usually in a negative way. The regenerate bone can be manipulated when needed by using injection of mesenchymal stem cells and platelets, growth factors (BMP-2 and -7), and systemic medications (bisphosphonates and parathyroid hormone). Growth factors and systemic anabolic and antiresorptive drugs are prescribed on special indications, while distraction osteogenesis is not an authorized indication. To some extent, however, these compounds can be used off-label. Use in children presents special problems since growth factors and specific anabolic medications may involve a risk of inducing cancer.

In the last decades, limb lengthening has not been limited to the treatment of patients with dwarfism and deformities resulting from congenital anomalies, trauma, tumor and infections, but, has also been used for aesthetic reasons. Cosmetic lengthening by the Ilizarov method with circular external fixation has been applied to individuals with constitutional short stature who wish to be taller.

From January 1985 to December 2010, the medical records of 63 patients with constitutional short stature (36 M, 27F; 126 legs) who underwent cosmetic bilateral leg lengthening using a hybrid advanced fixator according to the Ilizarov method, were reviewed, retrospectively. The mean age was 24.8 years, while the mean preoperative height was 152.6 cm. Paley’s criteria were used to evaluate problems, obstacles, and complications from the time of surgery until 1 year after frame’s removal.

The mean lengthening achieved in all patients was 7.2 cm (range: 5–11 cm), with a mean duration of treatment of 9 months and 15 days (range: 7–18 months). The mean follow-up time was 6.14 years (range 1–10).

The cosmetic leg lengthening was helpful to all patients, improving their social capabilities and self-confidence. All patients considered their stature as normal and they reported satisfaction and gratification with important changes in their professional and personal life. Cosmetic leg lengthening may raise some ethical objections and for that reason patients should be well informed about all the risks and complications related to this type of surgery.

When we lengthen a bone in a child, the parents and the family circle are often obsessed by the amount a lengthening obtained. However, for the surgeon, lengthen a bone is quite pretty easy, but dealing with the joints above and below the lengthening area can be very challenging. Indeed, during the lengthening process, muscles and tendons will be progressively stretched, leading to potential joint contracture or even dislocation. The objective of the surgeon will be to avoid this situation. The first mean at disposal is the physiotherapy in order to help the joints to be more supple and to maintain their range of motion. The second mean is the soft tissue release before the surgery, during the lengthening process, or after the hardware removal when the capacities of physiotherapy are overdone. As a last resort, it can be helpful to bridge the joint to protect it during the lengthening.

Successful deformity correction depends on establishing the aetiology of the deformity. Clinical examination, additional laboratory tests and consultation with other experts may be needed to complete the workup. Imaging studies should include full-length standing X-rays in all relevant planes, and additional imaging modalities like computed tomography (CT) and magnetic resonance imaging (MRI) may add information on bone morphology and growth plates’ anatomy. Based on the data, analysis of the deformity and length differences is performed, followed by prediction of deformities at skeletal maturity. The patients need to be followed up on a regular basis and repeat analysis should be done to improve the accuracy of prediction for final limb length difference. Limb deformity and lengthening correction plans are drawn and updated during follow-up, to achieve straight and equal lower limbs at maturity. Timely surgical procedures are performed using appropriate techniques and the most modern technologies available. These principles are discussed and demonstrated with case examples.

Limb-length discrepancies and extremity deformities are among the most common non-traumatic orthopaedic conditions for which children are hospitalised. There is a need to develop new treatment options for lower-limb length discrepancy in order to ameliorate treatment outcomes, avoid or reduce rates of complication and provide early rehabilitation. The authors report on the basic principles, experimental and clinical data, advantages, problems and complications of a combined technique associating the Ilizarov method and flexible intramedullary nailing (FIN) in limb lengthening and deformity correction in children. They describe features of the use of hydroxyapatite-coated intramedullary nails in patients with certain metabolic bone disorders and in cases where bone consolidation has been compromised. The advantages of bone lengthening using a combined technique (circular fixator plus FIN) are a lower healing index, quicker distraction-consolidation, a reduced rate of septic and bone complications, the ability to correct deformities gradually and the increased stability of bone fragments during the external fixation period and after frame removal.

Amputations and fitting surgery have a long history in children with limb deficiencies. With the current developments in limb reconstruction and new techniques in prosthetics, the indications for amputation and fitting surgery might have shifted, but still have a very important role in creating high functional performance, optimal participation and quality of life. The purpose of this current concepts article is to give an overview of the indications, dilemmas and technical considerations in the decision-making for amputation and fitting surgery. A special part of this overview is dedicated to the indications, variations and outcomes in rotationplasties.

The article is based on the experience of a multidisciplinary reconstruction team for children with complex limb deficiencies, as well as research of the literature on the various aspects that cover this multidisciplinary topic.

For those children with a more severe limb deficiency, reconstruction is not always feasible for every patient. In those cases, amputation with prosthetic fitting can lead to a good result. Outcomes in quality of life and function do not significantly differ from the children that had reconstruction. For children with a postaxial deficiency with a femur that is too short for lengthening, and with a stable ankle and foot with good function, rotationplasty offers the best functional outcome. However, the decision-making between the different options will depend on different individual factors.

Amputations and rotationplasties combined with optimal prosthesis fitting in children with more severe limb deficiencies may lead to excellent short- and long-term results. An experienced multidisciplinary team for children with complex limb deficiencies should guide the patient and parents in the decision-making between the different options without or with prosthesis.

Instability of the knee is a common finding in patients with congenital limb deficiency. The instability can be attributed to soft tissue abnormalities, frontal, sagittal or rotational deformity of the lower limb and bony dysplasia of the patella or of the femoral condyles. In most of the cases, these pathomorphologic changes stay asymptomatic in daily activity. However, instability can appear during deformity correction and bone-lengthening procedures, leading to flexion contracture or subluxation of the knee.

A review of pediatric orthopaedic literature on different factors of knee instability, state-of-the-art treatment options in congenital limb deficiency and in cases of lengthening-related knee subluxation is presented and the authors’ preferred treatment methods are described.

Leg lengthening and deformity correction in patients with congenital limb deficiencies can be achieved with various techniques, such as guided growth, monolateral or circular external fixation and intramedullary lengthening nails. Radiographic assessment and clinical examination of the knee stability are obligatory to estimate the grade of instability prior to surgical procedures. Preparatory surgery, as well as preventive measures such as bracing, bridging of the knee and intensive physical therapy, can help to avoid subluxation during lengthening in unstable knees.

Adequate surgical techniques, preventive measures and early detection of signs of subluxation can lead to good functional results in patients with congenital limb deficiency.

In paediatric orthopaedics, deformities and discrepancies in length of bones are key problems that commonly need to be addressed in daily practice. An understanding of the physiology behind developing bones is crucial for planning treatment. Modulation of the growing bone can be performed in a number of ways. Here, we discuss the principles and mechanisms behind the techniques. Historically, the first procedures were destructive in their mechanism but reversible techniques were later developed with stapling of the growth plate being the gold standard treatment for decades. It has historically been used for both angular deformities and control of overall bone length. Today, tension band plating has partially overtaken stapling but this technique also carries a risk of complications. The diverging screws in these implants are probably mainly useful for hemiepiphysiodesis. We also discuss new minimally invasive techniques that may become important in future clinical practice.

The risk and consequences of an elbow or a wrist contracture are lower during a forearm lengthening than during a lower limb lengthening. This kind of complication can mostly be avoided by an active and intensive regimen of physiotherapy. However, there are some challenges to deal with in treating the disorder multiple exostoses and the radial club hand, including the lack of consensus on the best treatment for multiple exostoses. However, it is important to realize that the evolution of multiple exostoses can lead to a radial head dislocation which will damage the pronation and the supination range of motion. As this motion can be poor even without a radial head dislocation as a result of the radius being longer than the ulna, an interesting technique can be to lengthen the ulna to limit this phenomenon. In radial club hand, the main problem is the deviation of the hand requiring a centralization. The best treatment for centralization of the hand is to do a progressive correction with an external fixator. Thereafter, it is possible to lengthen the forearm, but this indication is mainly cosmetic in the unilateral radial club hand.