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Lower Extremity Limb Length Discrepancy Etiologies & Lower Extremity Manifestations

Kelvin A. Barry, B.S.


Limb length discrepancy is a condition that affects the majority of the worlds population. Many people are completely unaware of differences in the length of their legs and even their arms. Limb length discrepancy is a condition that may be asymptomatic for years, decades, or even a lifetime. People who do have symptoms often times do not realize that their symptoms are attributed to a difference in the lengths of their legs. This article discusses various etiologies and podiatric manifestations of differences in the lengths of the lower extremities.

Introduction
Limb length discrepancy (LLD) affects many parts of the body from the neck down to the toes, with a majority of the problems occurring in the back and the feet. Assessment of lower extremity limb length discrepancies is a challenging task; evaluation of the discrepancy requires an understanding of the significance of the disparity, as well as the natural history of the disorder.(1) This condition often goes unnoticed by many healthcare professionals, and many of the symptoms are treated as some other cause for example, stress, trauma, nutritional defect or symptoms that result in back or leg pain. Approximately 85% of the worlds population exhibits LLD, and about 67% of them experience pain associated with differences in lower extremity lengths.(2) These discrepancies occur in two etiologic types congenital and acquired.(2) Congenital LLD is present from birth and is associated with many birth defects and deformities.(3) Acquired LLD result from developmental changes in bone and soft tissue, infectious processes, iatrogenic causes, or trauma. (1,3)
Fig. 1 Measurement of a boy for shortened left limb. In far left picture hip is lowered on short side. In picture to immediate left a block placed under short limb has returned hip to level

Classification of the types of deformities can be functional, structural, or mixed. Functional or positional length differences usually have equal osseous structures but some abnormal soft tissue involvement including soft tissue contractures or foot function aberrations.(4) Structural or true length differences involve the osseous system, and usually have trauma or congenital growth inequality as their etiology.(4) The combined or mixed type of LLD is the most commonly seen in the lower extremity and foot and is due to bony involvement with soft tissue compensatory involvement.(4)

Etiologic Factors
The most common cause of a limb length discrepancy is due to genetic anomalies. Trauma is another frequent cause of LLD.(4) As in assessing any problem, a very thorough history is warranted because determining the etiology helps determine whether the discrepancy will remain static or begin to progress in severity. Congenital Etiologies Congenital hemiatrophy with skeletal anomalies is a major congenital cause of short leg lengths.(5) These structural deformities tend to include aplasia or hypoplasia of long bones in the thighs and legs.(4,5) Dyschondroplasia, such as Olliers disease, is another structural congenital cause of LLD. Olliers disease is a non-hereditary
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defect that usually causes tumors in the bones of one extremity, affecting not only the long bones, but also those of the foot. The tumors affect the growth plates, which over time causes the limb length discrepancy.(6) Hemarthrosis due to hemophilia and Partial giantism with some vascular abnormalities can also cause limb differences due to epiphyseal growth stimulation.(4) One such vascular anomaly is Klippel-Trenaunay-Weber syndrome, which presents at birth or during early infancy or childhood.(5,7) Klippel-Trenaunay-Weber syndrome is characterized by a triad of port-wine stain, varicose veins and bony and soft tissue hypertrophy involving an extremity.(7) Congenital dislocated hip (CDH), coxa vara, and clubfoot are some joint-associated structural deformities that may also create limb length differences. In CDH, most dislocations occur after birth and are related to unstable or dislocatable hips, ligamentous laxity of the joint capsule, and intrauterine (breech) presentation.(8) Cutis marmorata telangiectatica congenita (CMTC), a rare benign condition usually present at birth, is characterized by areas of cutaneous marbling that may become more pronounced with low temperatures (Figure 2). This condition involves relative growth retardation of the affected leg.(9)

Infectious Etiologies Infectious processes that leave bony, structural defects or functional soft tissue defects can cause differences in the lengths of limbs. Epiphyseal plate destruction due to osteomyelitis of tibia or femur, tend to retard bone growth via premature epiphyseal closure.(4,5) Septic arthritis and tuberculosis (of hip, knee, and foot) tend to also cause premature epiphyseal closure.(4,5) Diaphyseal osteomyelitis of the femur or tibia is another condition that affects the epiphyseal plate. The only difference is this condition causes a growth stimulation of the epiphyses. There are other conditions in this category of epiphyseal growth stimulation such as septic arthritis, syphilis of femur or tibia, Brodies abscess, and metaphyseal tuberculosis of femur or tibia.(1,4,5) Brodies abscess is a chronic welldemarcated rim of sclerotic bone that surrounds a residual lesion and is considered a result of chronic osteomyelitis. Another disease which affects limb length is poliomyelitis. Poliomyelitis, an inflammatory process that affects the gray matter of the CNS, is primarily a neuromuscular condition, but since it is caused by infection from a small RNA enterovirus of the Picornaviridae group, it can be considered an infectious cause of LLD. Elephantitis as a result of long-standing obstructed lymphatic vessels is yet another potential cause of LLD. The obstruction is usually caused by acute or chronic soft tissue infections. (5) The obstructions can also come from soft tissue contractures or can be iatrogenic. Neoplastic Etiology Tumors can stimulate or retard bone growth, especially at growth plates. Osteochondromas, giant cell tumors, and nuerofibromatosis cause premature closures, therefore shortening affected bones. Tumors that stimulate growth because of increased blood supply include hemangiomas and fibrous dysplasia. Neurofibromatosis may be characterized by a definite pattern of progression of leg length discrepancy. The patients clinical appearance is
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Fig. 2 CMTC in a 2-year-old child, which most commonly presents on the leg and foot. Picture courtesy of Dermatlas Image Atlas Online. (www.dermatlas.org)

usually one of frank hypertrophy of the calf, generally manifested in the distal leg and ankle region. In a young child, the lower extremity often appears relatively normal; although, with closer examination, the lower extremity often appears to be slightly longer and bigger. However, with growth, and increase in hypertrophy of the bone and soft tissue, the deformity becomes more obvious.(10) Neurofibromatosis is often associated with fibular aplasia and scoliosis. Osteochondromas and giant cell tumors are easily explained. These simply cause damage to epiphyseal and metaphyseal growth plates, resulting in early closure of the growth plate. In addition, osteochondromas can cause additional destruction to the cortex. Hemangiomas tend to cause a hypertrophy of the affected limb.(10) The high blood supply to these areas is the main cause of this hypertrophy gigantism. Clinically, the appearance varies with the degree of involvement- involved limb generally looks much fatter than the normal limb. In the more severely involved limbs it can develop into a massive, grotesque clinical appearance despite corrective surgical procedures.(10) Examples of hemangiomas can be seen in patients with Marfuccis syndrome and Klippel-TrinnelWeber syndrome. Traumatic Etiology Trauma is a frequent cause of LLD.(4) Breech presentation at birth could produce a long bone fracture due to lower extremity manipulation. Premature closure of an epiphyseal plate will lead to lengthening of the non-injured limb. Fractures of the tibia or femur in children may result in increased bone lengtha hyperemic healing reaction results in stimulation of growth in the corresponding epiphyseal plate. Children under the age of 10 have the greatest growth stimulation in femoral fractures, producing an increase in bone length.(4) Fractures in long bones of children should be reduced in an overriding position to minimize the increased length. The types of injury to epiphyseal plate, as described by Salter and Harris, may determine whether or not a LLD or short leg syndrome will occur.(4) SalterHarris classification type 4 and type 5 injuries will cause limb length differences to occur because of
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direct damage to the epiphyseal plate Type 4 is responsible for early closure and also for angular deformity. Type 5 is a crushing of the epiphyseal plate. These deformities are strictly positional in nature. Another traumatic cause of LLD is severe burns that result in soft tissue contractures and bony damage. The precise mechanism causing growth retardation in severely burned lower limbs is not well understood. It is suggested that impairment of epiphyseal growth might result from prolonged ischemia of the epiphyseal plate. It has also been suggested that retardation of growth could result from at restrictive or strangling effect of thick scar about the distal tibial metaphyseal and ankle joint areas, with inhibition of epiphyseal growth.(11) Other Etiologies Some other etiologies of LLD include paralysis, immobilization of long duration by weight-relieving braces, damage to femoral or tibial epiphyseal plates due to radiation, and a host of idiopathic entities. Legg-Calve-Perthes disease, an epiphyseal osteonecrosis of the upper end of the femur, is a contributing condition to limb length differences. Slipped upper femoral epiphysis is another condition that clearly contributes to the differences in limb lengths. These conditions usually present in younger male patients complaining of groin or medial knee pain.

Lower Extremity Manifestations


A majority of the manifested problems from LLD tend to be localized to the back, particularly the vertebral column, hip, and the feet. Many of the related problems are mechanical or functional in nature. It is generally felt that associated problems include increased energy expenditure in gait, cosmetically disturbing gait, equinus deformity of the foot and ankle, scoliosis, lower back problems, and degenerative changes to the hip.(12) Gait and Trunk Compensations At least four distinct compensation mechanisms can emerge during the gait of an

individual with LLD; these include steppage, circumduction, vaulting, and hip-hiking. Sometimes individuals perform one or more of these compensatory activities to make walking more fluid and easy.(13) The typical gait of a patient with a limb length difference involves a stepping down onto the short limb and vaulting over the long. To compensate for the discrepancy during gait and to minimize vaulting, patients must either walk on the toes of the short leg or flex the knee of the long leg.(12,14) From this, there is increased vertical movement of the pelvis and center of gravity, requiring increased expenditure of energy and resulting in a less efficient gait. The cosmetic effect is a very important part of the problem as perceived by the patient, especially with adolescents, and is often a major factor in the treatment decision.(12) These differences in gait, and also stance, tend to apply forces on various other parts of the lower trunk. One would expect the back, hip, and knee to be affected. Much has been stated but little proven concerning the effects of LLD on the back. There is circumstantial evidence to suggest that LLD contribute to the development of degenerative arthritis of the hip.(12) Scoliosis during stance, caused by the pelvic obliquity that shows up when legs of different length are fully extended and the feet flat on the floor, is a constant finding. Studies of older populations have been done that disclosed an increased incidence of scoliosis in patients with LLD.(4,12) Although it is generally felt by orthopedists that patients with uncorrected LLD over a lifetime are prone to develop low back pain due to degenerative disease in the lumbosacral spine, this has not been proven. The pelvic obliquity that occurs during bipedal stance in LLD results in a tilting of the acetabulum, which reduces coverage of the femoral head on the long side. This reduces the weight-bearing area, increases pressure exerted on the joint surfaces, and produces a sharp gradient in pressure at the edge of the acetabulum.(4,12,14) Conversely, the obliquity produces an increased acetabular contact area on the short side. The Shorter leg will receive less pressure; the longer side receiving abductor tone. This may result in trochanteric bursitis.(4)
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Distal Compensations It has been hypothesized that compensation occurs along the kinematic chain of lower extremity in an attempt to equalize the limb length difference. It has been theorized that patients with LLD generally compensate by extending the short limb or flexing the long limb in standing rather than having unequal weight bearing.(3) Young children usually compensate by walking on toes (equinus positioning of ankle) of the short side. Older children and adults compensate by dipping the hip on the short side. (5) Along with the equinus positioning of the ankle, patient also present with genu recurvatum or hyperextension of the knee joint. Equinus also leads to gastrocsoleus muscle tightness, and in conjunction with supination of the foot (cavus foot type) can cause the patient to present with metatarsalgia, submetatarsal head lesions due to excess plantarflexion of the metatarsals, and the development of hammertoes and clawtoes as proximal phalanges respond by dorsiflexion. Pronation and supination are two common forms of foot compensation in limb length discrepancies. The long leg usually produces subtalar joint pronation; the calcaneal inclination angle is decreased which lowers the height of the talus. (Fig. 3, left) This produces a shortening of the effective functioning length of the longer limb. The short limb may increase its functioning length by supination of the subtalar joint. (Fig. 3, right) This combination of subtalar and midtarsal joint compensation requires adequate range of motion in the foot joints.(6)

Fig. 3 Diagram of pronation and supination of the foot with respects to the subtalar joint and calcaneus. Depiction of subtalar joint motion in the right foot.

Blustein and DAmico reported that the limb length difference results in a biomechanical compensation which, in turn, results in an increase in the degree of pronation on long side. Calcaneal eversion is an important component of subtalar joint pronation. Pronation causes plantarflexion and adduction of the talus, internally rotates the leg, everts the calcaneus, and decreases the calcaneal inclination, therefore shortening the limb. Calcaneal inversion is an important component of subtalar joint supination. Biomechanics of LLD cause the subtalar joint of the short limb to increase the amount of supination, thereby increasing calcaneal inclination, resulting in a lengthening of the short limb and rebalancing of the pelvis.(15) In a study by Langer (Langer Newsletter, 1983), several different things occurred in the foot during the gait cycle: increased stance phase time in the longer limb; shorter cadence on the longer side; increased and prolonged medial heel pressure on the compensatorily pronated long limb; a shorter contact phase on the long limb; a longer midstance on the long limb; and a longer propulsive phase on the pronating longer side.(4) One other theory about the manifestations of LLD in the foot suggests that in order to provide stability, the patient compensates by externally rotating the foot and limb on the short side, causing a majority of the weight bearing to fall medial to the axis of the subtalar joint. This results in excessive pronation of the foot and excessive medial strain on the lower extremity of the short limb.(15)

There is a need for more observations of patients gait and posture, especially in children, in order to help medical professionals make more accurate and earlier diagnosis of limb length problems, thus preventing symptoms in patients as they grow older. This can lead to earlier preventative or corrective measures that can, in the later future, prevent a persons symptoms from becoming worst. LLD is a complex podiatric diagnosis to work with. Treatments are best determined by proper classification. Early recognition of the etiologic factor(s) and aggressive treatment are the best weapons against limb length discrepancy.

References
1. Stanitski DF: Limb-length inequality: Assessment and Treatment Options. J Am Acad Orthop Surg, 7(3): 143-153, 1999. Kiper, D. Leg Length Discrepancies. Fitness Runner Holiday 1999. 6 September 2002 http://www.drkiper.com/articles/LegLength.html Leg Length Differences. Maryland Center for Limb Lengthening and Reconstruction. 6 September 2002 http://www.umm.edu/mcllr/mcllr_treat.html Vogel F Jr.: short-leg syndrome. Clin podiatry, 1(3): 581-599. Limb Length Inequality: Evaluation & Treatment. Orthopaedics. Connecticut Childrens Medical Center. 27 September 2002 http://www.ccmckids.org/departments/Orthopaedic s/orthoed10.htm Extended possibilities for patient with leg length discrepancy. Shriners Hospital Page. 6 September 2002 http://www.shrinershq.org/shc/houston/olliers101.html Lisko JH, Fish F: Klippel-Trenaunay-Weber Syndrome. E-Medicine. 19 March 2003 http://www.emedicine.com/derm/topic213.htm

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Summary
Limb length discrepancy affects a larger population than one would normally expect. A large percentage (approximately 85%) of the population has a difference in the lengths of their lower limbs. Many people are discovering, via the orthopedist or podiatrist that they have uneven limb lengths; and many of them have symptoms, such as back pain, which they never suspected were attributed to the difference in the lengths of their lower extremities.
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D'Alessandro, MP: Congenital Hip Dislocation (Infantile Hip Dislocation) (Congenital Dislocation of the Hip) (CD) (Developmental Dysplasia of the Hip) (DDH). 19 March 2003 http://www.vh.org/pediatric/provider/radiology/PA P/MSDiseases/CongDislocHip.html 9. Dutkowsky JP, Kasser JR, Kaplan LC: Leg Length discrepancy associated with vivid cutis marmorata. J Pediatric Orthop, 13(4):456-458, 1993 10. Pappas AM, Nehme AM: Leg length discrepancy associated with hypertrophy. Clin Orthop, 144: 198-211, 1979.

11. Salter RB, Harris WR: Injuries Involving the Epiphyseal Plate. J Bone Joint Surg Am, 45:587, 1963. 12. Frantz CH, Delgado S: Limb-length discrepancy after third-degree burns about the foot and ankle. Report of four cases. J Bone Joint Surg Am, 48(3): 443-450, 1966. 13. Moseley CF: Leg-length discrepancy. Pediatric Clin North Am, 33(6): 1385-1394, 1986. 14. Gurney B, Mermier C, Robergs R, Gibson A, Rivero D: effects of limb-length discrepancy on gait economy and lower-extremity muscle activity in older adults. J Bone Joint Surg Am, 83-A(6): 907-915, 2001. 15. Song KM, Halliday SE, Little DG: The effect of limb-length discrepancy on gait. J Bone Joint Surg Am, 79(11): 1690-1698, 1997. 16. Bloedel PK, Hauger B: The effects of limb length discrepancy on subtalar joint kinematics during running. J Orthop Sports Phys Ther, 22(2): 60-64, 1995.