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Author: James McCarthy, MD, FAAOS, Associate Professor of Orthopedic Surgery, Temple University School of Medicine; Assistant Chief of Staff, Medical Director of Gait Laboratory, Shriners Hospital for Children of Philadelphia

James McCarthy is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Orthopaedic Surgeons, and American Orthopaedic Association

Editors: Dennis P Grogan, MD, Clinical Professor, Department of Orthopedic Surgery, University of South Florida College of Medicine; Chief of Staff, Department of Orthopedic Surgery, Shriners Hospital for Children of Tampa; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Thomas M DeBerardino, MD, Director, John A Feagin, Jr Sports Medicine Fellowship at West Point, Clinical Instructor in Surgery, Orthopedic Surgery Service, Keller Army Community Hospital at West Point; Dinesh Patel, MD, FACS, Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital; Carlos J Lavernia, MD, FAAOS, Adjunct Clinical Professor, Department of Orthopedic Surgery, University of Miami School of Medicine; Medical Director, Orthopedic Institute at Mercy Hospital

Author and Editor Disclosure

Synonyms and related keywords: bowing of the diaphysis of the tibia, posteromedial bowing, calcaneovalgus foot deformity, leg-length discrepancy, limb-length inequality

There are multiple etiologies for tibial bowing (see Etiology). Tibial bowing specifically refers to bowing of the diaphysis of the tibia with the apex of the deformity directed anterolaterally, anteromedially, or posteromedially. Each type of bowing tends to have a classic etiology.

Anterolateral bowing is associated with pseudarthrosis of the tibia and neurofibromatosis (see Neurofibromatosis). Anteromedial bowing is associated with fibular hemimelia (see Fibular Hemimelia). The focus of this article is on posteromedial tibial bowing.

Posteromedial bowing is a congenital bowing of the tibia (with the apex directed posteriorly and medially) and a calcaneovalgus foot deformity. Both of these deformities tend to resolve with little clinical disability; however, a leg-length inequality commonly develops that often requires treatment. If a significant leg-length inequality results, the patient will have an abnormal gait and may be at risk for increased back pain or deformity. Treatment options vary depending on the degree of limb-length inequality, age of the patient, expected height, and desires of the patient or family. Treatment options include slowing the growth of the longer limb or lengthening the shorter limb.

History of the Procedure

Limb-length equalization procedures have primarily been performed using 1 of 2 general approaches: slowing the growth of the longer limb with an epiphysiodesis or lengthening the shorter limb.

Phemister (1933) described his classic technique for epiphysiodesis. He removed a section of the epiphysis, rotated it 90°, and replaced the bone. Today, the most common technique is the percutaneous drill epiphysiodesis, performed with the aid of an image intensifier. This technique has been reported to result in physeal closure in 85-100% of patients with few complications.

The first published report of a limb-lengthening procedure in the English literature dates to 1904 in Italy (Codivilla). Newer techniques, such as the Ilizarov and Wagner techniques, have been performed for 50 years. The Ilizarov technique and variations thereof are the procedures most often used today. It is named after Gavril Abramovich Ilizarov, a Russian physician who used his technique to treat injured World War II veterans. Lengthening is usually performed using corticotomy and gradual distraction with a ring fixator and fine wires. This technique can result in an increase of 25% or more in bone length.

Problem

Posteromedial bowing is a congenital anomaly associated with a severe calcaneovalgus foot deformity. These deformities usually improve significantly with little or no treatment (see Images 1-3). Ultimately, limb-length inequality is usually of the greatest concern with tibial bowing.

Frequency

The true incidence is unknown, although this is a relatively infrequent disorder.

Etiology

Each type of tibial bowing tends to have its own etiology (see Introduction). Etiologies of tibial bowing include the following:

  • Posteromedial bowing

  • Anteromedial bowing (fibular hemimelia)

  • Anterolateral bowing (tibial pseudarthrosis)

  • Blount disease (infantile tibia vara)

  • Physiologic bowing

  • Rickets

  • Focal fibrous dysplasia

  • Trauma (ie, Cozen fracture)

  • Dysplasias

The developmental etiology of posterior medial bowing is unknown, but most authors believe it occurs secondary to abnormal fetal positioning, with the dorsiflexed foot plastered against the anterior aspect of the tibia. Primary abnormal embryologic development, such as limb bud or circulatory abnormalities and intrauterine fracture, has also been suggested as a possible developmental etiology.

Pathophysiology

The pathophysiology of the resultant limb-length inequality may be related to the bowing. The degree of initial tibial deformity (which largely resolves by age 8 y) has been shown to be ultimately associated with the severity of tibial shortening and resulting limb-length inequality. Animal models have demonstrated that unbalanced longitudinal pressures affect physeal growth. It is speculated that bowing results in unbalanced longitudinal pressures and, ultimately, in a decreased rate of growth. This theory is contradicted by the fact that tibial growth inhibition remains constant as the child grows even though the deformity improves. The rate of growth of the affected tibia would be expected to approach that of the unaffected leg as the bowing resolves; this is not the case. Additionally, in the few patients who underwent early realignment osteotomy, tibial growth was still inhibited, resulting in a subsequent limb-length inequality.

Clinical

The deformity is often obvious and is present at birth. The foot is usually dorsiflexed to such a degree that it makes contact with the anterior aspect of the distal tibia. The posterior bow of the tibia is less obvious but can be easily palpated. A dimple may be present in the skin posterior to the apex of the bow.

The Galeazzi test (see Image 4), typically used to assess hip dislocation, can also be used to assess any congenital disorder that results in a significant limb-length inequality. The examination is performed with the patient supine and the hips and knees flexed. The result is considered positive if knee height is asymmetrical. It is also helpful to assess whether the limb-length inequality is primarily from the femur or from the tibia and to assess limb length in someone with knee or hip flexion contractures.

Differential diagnoses include the other types of tibial bowing, such as anterolateral and anteromedial bowing. Intrauterine fracture or osteogenesis imperfecta may also result in tibial bowing. These are often easily differentiated based on physical examination findings in which the direction of the tibial bowing and the associated foot deformity are noted. Anteromedial bowing is often associated with congenital loss of the lateral rays of the foot and fibular deficiency. Anterolateral bowing is associated with a pseudarthrosis of the tibia that may be obvious radiographically at birth or may develop with growth. Approximately 50% of children with anterolateral bowing are eventually diagnosed with neurofibromatosis.



Initial treatment of the foot deformity includes stretching, serial casting, or splinting. The bowing deformity rapidly corrects. A 50% correction is usually seen by age 2 years, although a mild deformity often persists (see Image 3). The rationale for corrective tibial osteotomy is less clear, as a tibial osteotomy is rarely indicated; however, a significant deformity that interferes with development, especially if little or no correction is seen by age 2 years or a symptomatic and persistent deformity is seen in children older than 10 years, may be an indication for tibial osteotomy.

Performing a tibial osteotomy does not seem to decrease the need for later limb equalization. Most children with posteromedial bowing will require a limb equalization procedure. The type of procedure depends on the degree of projected limb-length inequality at skeletal maturity. Typically, limb-length inequality is 2-6 cm at skeletal maturity. Usually, an appropriately timed epiphysiodesis can restore limb-length equality, although a lengthening procedure may be indicated for more severe projected limb-length inequalities (>5 cm), especially in children of short stature.



Posteromedial bowing is defined by the apex of the tibial curve being directed posteriorly and medially. Bowing in other directions is usually associated with different disorders.



Understanding the nature of the deformity and establishing the correct diagnosis are very important. Tibial osteotomies in children with anterolateral deformities can be disastrous. This type of tibial deformity can be associated with persistent pseudarthrosis even without any surgical procedures, and performing an osteotomy may promote or instigate an early nonunion. If the patient has a metabolic etiology for their bowing, treat the metabolic disorder before considering surgical options. Posteromedial bowing typically self-resolves, leaving only the limb-length inequality to be addressed.



Lab Studies

  • No studies are necessary other than radiographic images unless concern exists regarding the diagnosis, in which case other studies (eg, a metabolic study to assess for rickets) may be ordered.

Imaging Studies

  • Initial evaluation
    • The initial evaluation should include an anteroposterior (AP) radiograph of the lower extremity with a ruler to measure limb length and to assess the deformity.
    • Hip dysplasia, although not normally seen in patients with this disorder, can be assessed at age 6-12 weeks.
    • Obtain a lateral radiograph of both tibiae.
    • Obtain radiographs of the foot if the foot fails to correct by age 4-6 weeks. This should include AP and lateral views of the foot and a plantarflexion lateral view of the foot to assess for a vertical talus.

  • Limb-length inequality at skeletal maturity must be assessed before any type of limb equalization procedure (epiphysiodesis, lengthening, or shortening) is performed.
    • Typically, an epiphysiodesis is required at about age 11 years in females and age 13 years in males, but this varies depending on the patient's skeletal age and the degree of the limb-length inequality.
    • Epiphysiodesis can be indicated as early as age 8 years. As mentioned previously, if the degree of limb inequality is large (>5 cm) and the patient is not expected to be tall, a lengthening may be considered.
    • Limb-length inequality at skeletal maturity is most reliably predicted from a series of at least 3 radiographs taken at least 6 months apart.

  • Various radiographic measures have been used to determine limb-length inequality.
    • The teleoroentgenogram is a single-exposure AP radiograph of the lower extremity with a ruler. This study is subject to a magnification error of 5-10% at the outer border of the film but has the advantage of showing coronal (angular) deformities and is not subject to movement errors.
    • Orthoradiography incorporates 3 separate exposures (hip, knee, and ankle) in an effort to avoid magnification errors.
    • Scanography uses a similar technique, but exposure size is reduced and all 3 exposures are on 1 film cassette (see Image 5).
    • Both orthoradiography and scanography are subject to movement errors, and angular deformities cannot be assessed.
    • All of the techniques are inaccurate if the patient has knee or hip flexion contractures or if the patient is simply flexing the knee or hip asymmetrically at the time of exposure. If the knee appears as a tunnel view, there is undoubtedly a significant degree of knee flexion. Lateral radiographs or separate (prone) radiographs of the femur and tibia with a radiopaque ruler can be obtained to assess limb length in patients with knee flexion contractures.
    • The use of computed tomography (CT) scanning to assess limb length has increased. CT scanning uses less radiation and is more accurate than conventional radiographic techniques in patients with knee or hip flexion contractures.
    • Ultrasound is now being used as well, primarily as a screening tool.

Diagnostic Procedures

  • Once the current limb-length inequality has been measured, a prediction of the ultimate limb-length inequality at skeletal maturity is needed to determine treatment. There are 3 methods typically used to do this: the arithmetic method, the growth-remaining curve, and the Moseley straight-line graph. A fourth method, known as the multiplier method, has also been recently described, in which an arithmetic formula is used to determine limb inequality at maturity.
    • The simplest method is the arithmetic method. This method assumes growth of the distal femur to be 1 cm per year, the growth of the proximal tibia to be 0.6 cm per year, and that boys reach skeletal maturity at age 16 years and girls at age 14 years.
    • The growth-remaining curve relates chronologic age to limb length to determine a child's growth percentile. Using this, the remaining growth of the tibia or femur can be determined graphically.
    • The advantage of the Moseley straight-line graph, which combines information from both the arithmetic method and the growth-remaining curve, is that several measurements (preferably at least 3, separated by 6 months) can be plotted on 1 graph. The Moseley straight-line graph relies on determination of the bone age as estimated from a left hand/wrist film.
    • When these 3 techniques (ie, arithmetic method, growth-remaining curve, Moseley straight-line graph) were evaluated, the accuracy rates of these 3 methods showed little significant difference.
    • The multiplier method simply takes the current limb-length inequality and multiplies it by a constant listed in a table by chronologic age. Timing of the epiphysiodesis can then be estimated by use of an arithmetic formula to determine limb-inequality at maturity. It is as precise as other methods for determining limb length at maturity and can accurately estimate the timing for epiphysiodesis.

Histologic Findings

The histology of posteromedial bowing is unknown, but animal studies performed to model angular deformities demonstrate increased trabecular bone formation in the area of the apex of the angular deformity, with no new cartilage cells, and subepiphyseal bone condensation with subsequent thinning of the epiphyseal plate.



Medical therapy

Currently, no medical therapies exist for limb-length inequality. Nonsurgical treatment includes stretching, serial casting, or splinting. This should be initiated at birth. If significant correction is not obtained by age 4-6 weeks, question the diagnosis; the possibility of a more serious foot deformity, such as a vertical talus, must be ruled out radiographically. After the foot has fully corrected, a splint can be made to maintain correction until age 12-24 months. Bracing of the bowing deformity has been suggested, but it is not currently believed to significantly alter the natural history.

Most children with posteromedial tibial bowing have a limb-length inequality averaging 3 cm, but this can vary from about 2 to 6 cm. Typically, a limb-length inequality of 2 cm or less is not a functional problem. Often, limb length can be equalized with a shoe lift. About two thirds of limb-length inequalities are corrected with a lift; up to 1 cm can be inserted in the shoe. Larger limb-length inequalities require the shoe to be built up. This is necessary for every shoe worn and limits the type of shoe that the patient can wear.

Limb-length inequalities of more than 5 cm are difficult to treat with a shoe lift. The shoe looks unsightly, and often the patient complains of instability with such a large lift. A foot-in-foot prosthesis can be used for larger limb-length inequalities. This is often a temporizing measure for very young children with significant limb-length inequalities. The prosthesis is bulky, and a fixed equinus contracture may result.

Surgical therapy

The type of surgical treatment depends on the degree of projected limb-length inequality at skeletal maturity. Epiphysiodesis is a reliable procedure that inhibits growth with few complications. This cannot be performed on patients who are skeletally mature, and the final limb-length inequality and the degree of growth inhibition must be predicted and are subject to error. In addition, epiphysiodesis effectively shortens the longer leg and is a procedure that is usually performed on the uninvolved side, both of which may be unappealing to the patient and family. Typically, predicted limb-length inequalities of 2-6 cm can be corrected with an appropriately timed epiphysiodesis.

Lengthening is usually performed with corticotomy and gradual distraction. This technique can result in an increase of 25% or more in bone length, but, typically, a lengthening of 15% (or about 6 cm) is recommended. The limits of lengthening depend on patient tolerance, bony consolidation, maintenance of joint range of motion, and stability of the joints above and below the lengthened limb.

Preoperative details

Predicted limb-length inequality at skeletal maturity must be carefully assessed, and the effect of the given procedure on future growth must be estimated. Estimated height is also important, especially with a large limb-length inequality, because this may determine if an epiphysiodesis or lengthening should be performed. Preoperative teaching is important, especially for lengthening procedures that can last several months and require a great deal of tolerance and cooperation from the patient and family. The bone is typically lengthened about 1 mm/day, after a 7-10 day latency period. The total time in the fixator is about 1 month per 1 cm (10 mm) of lengthening and includes both the time to lengthen and time for the bone to consolidate and become strong enough to bear weight.

Intraoperative details

Numerous fixation devices are available for lengthening, such as the ring fixator with fine wires, monolateral fixator with half pins, and the hybrid frame. The choice of fixation device depends on the desired goal. A monolateral device is easier to apply and is better tolerated by the patient. The disadvantages of monolateral fixation devices include the following: limitation of the degree of angular correction that can be obtained concurrently; the cantilever effect on the pins, which may result in angular deformity, especially when lengthening the femur in large patients; and adjustments are difficult to make without placing new pins. Monolateral fixators and circular fixators appear to have similar success rates, especially with more modest lengthenings of 20% or less.

Postoperative details

Postoperative care is minimal for patients who have an epiphysiodesis. Knee range of motion (ROM) should be monitored. Full extension and 90° of flexion should be obtained by 2 weeks after surgery. Weightbearing can begin immediately, and the patient can return to sports at 6-12 weeks.

Postoperative care for patients undergoing lengthening with an external fixator is quite demanding. Lengthening begins 5-7 days postoperatively and continues at 1 mm/d until the desired length is obtained. Careful assessment of the joints adjacent to the fixator is mandatory in order to assess for ROM and joint subluxation.

Follow-up

Continued monitoring of limb-length inequality is needed for patients undergoing an epiphysiodesis. An orthoroentgenogram or scanogram should be taken every 6 months until skeletal maturity. The expected goal is limb-length equality within 1 cm at skeletal maturity.

For patients undergoing limb lengthening, the fixator can sometimes be removed in an outpatient setting, but usually these devices are removed with the patient under sedation.



Epiphysiodesis has been reported to result in physeal closure in 85-100% of patients with few complications. In the author's review (McCarthy, 2003) of 44 patients who underwent proximal tibial epiphysiodesis, no complications occurred, although lack of growth inhibition, angular deformity, and knee stiffness can occur.

Numerous complications can occur when performing limb-lengthening procedures, even in experienced hands. Complication rates vary significantly among reported studies and seem to depend on the degree of lengthening, definition of complication, and the surgeon's experience. Complication rates from most series, including that of the authors (McCarthy, 2000; 2003), are about 1 per procedure, and many of these require operative treatment. Fortunately, the ultimate objective can usually still be obtained.

The most common complication is pin-site infection. Depending on how this complication is diagnosed, treated, and reported, it may occur in nearly every patient. Numerous pin-care protocols have been developed. Some authors are demonstrating good success with a shower regimen after the incisions have healed. This author uses this regimen in combination with standard cleaning of the pin sites and oral antibiotics if excessive discharge, redness, or swelling is present. Periosteal reaction occurs around the pin sites in most patients, and this may be an early indication of loosening.

Newer pins, coated with hydroxyapatite, have improved fixation to bone and may reduce the rate of infection and loosening during external fixation for distraction osteogenesis. Use of hydroxyapatite-coated pins should be considered in clinical situations requiring prolonged external fixation.

Knee ROM decreases uniformly in femoral lengthening by an average of 37°, but at follow-up, the mean loss in ROM is usually minimal.

Other, more ominous complications include fracture, osteomyelitis, and joint subluxation. The incidence of these more serious complications is about 25% with an experienced surgeon.

Less commonly considered effects of limb lengthening include muscle weakness, pain, and possible physeal inhibition. The last effect is extremely important if lengthening procedures are planned for younger patients with an open physis. Some reports, including this author's (McCarthy, 2003), found little difference in prelengthening and postlengthening growth velocities, indicating little effect of lengthening on the adjacent growth plates (with moderate lengthenings). Other reports (Sharma, 1996) have found growth inhibition, especially in the tibia and in children after extensive lengthening procedures (> 30%).

Unlike pain associated with conventional surgery, pain with lengthening seems to continue beyond the postoperative period and through the lengthening and consolidation phases, until the fixator is removed.

The use of somatosensory evoked potential (SEP) monitoring may be helpful in preventing neurologic injuries, especially of the peroneal nerve. The use of ultrasound, electrical stimulation, or both, while not routinely prescribed, may decrease the time to consolidation.



Although the angulation and foot deformity associated with posteromedial bowing improve dramatically, some deformity, including tibial torsion and muscle atrophy, often remains. This is usually not a significant disability.



Experimental methods of producing lengthening, such as cultured chondrocytes transfer, vascular surgery, and periosteal sleeve resection, are being studied.



Media file 1:  Anteroposterior radiograph of a 1-year-old child with posteromedial tibial bowing.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  X-RAY

Media file 2:  Lateral radiograph of a 1-year-old child with posteromedial tibial bowing.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  X-RAY

Media file 3:  Anteroposterior and lateral radiograph of a 9-year-old child with posteromedial tibial bowing. Note that the bowing has significantly improved compared to Images 1 and 2.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  X-RAY

Media file 4:  Posteromedial tibial bowing. The Galeazzi test. Note the difference in the height of the flexed knees.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Image

Media file 5:  Scanogram of a patient with posteromedial tibial bowing and a limb-length inequality.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Image

Media file 6:  A hydroxyapatite-coated Schanz pin used to secure external fixator devices to the bone.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Photo



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Tibial Bowing excerpt

Article Last Updated: Jul 19, 2007