Congenital Limb Congenital Limb Length DiscrepancyLength Discrepancy

By By Dr Frazand Ali Dr Frazand Ali

PG-Trainee Orthopedics PG-Trainee Orthopedics LGH Lahore LGH Lahore

Limb Length Discrepancy Limb Length Discrepancy

Difference between the Difference between the length of upper and length of upper and lower arm / upper and lower arm / upper and lower leg is called “Limb lower leg is called “Limb Length Discrepancy” Length Discrepancy” (LLD)(LLD)

Causes Causes

CongenitalCongenital : : Femoral deficiency Femoral deficiency Coxa vara Coxa vara Fibular hemimelia Fibular hemimelia Tibial hemimelia Tibial hemimelia Psuedarthrosis of tibiaPsuedarthrosis of tibia NeurofibromatosisNeurofibromatosisTrauma Trauma Over riding fracture Over riding fracture Epiphyseal injuries Epiphyseal injuries

leading to shorteningleading to shortening

Infections Infections PoliomyelitisPoliomyelitis Septic arthritis Septic arthritis Osteomylitis leading to Osteomylitis leading to

growth plate damage growth plate damage Neurological Neurological Cerebral palsyCerebral palsyTumour Tumour Multiple exostosis Multiple exostosis Inflamatory Inflamatory Rheumatoid arthritis Rheumatoid arthritis

Signs and Symptoms Signs and Symptoms

Limping gait Limping gait Unappealing shoe lift Unappealing shoe lift Low back ache Low back ache Compensatory scoliosis Compensatory scoliosis Degenerative arthrosis of lumbar and Degenerative arthrosis of lumbar and

sacral region sacral region

Compensation and Compensation and ToleranceTolerance

Different Patients respond differently Different Patients respond differently to LLD depending upon age, height to LLD depending upon age, height and body weight e.t.c.and body weight e.t.c.

children tolerate discrepancies better children tolerate discrepancies better than adults because of their inherent than adults because of their inherent flexibility flexibility

a 6-foot-tall patient tolerates a 2-cm a 6-foot-tall patient tolerates a 2-cm discrepancy with little or no trouble, discrepancy with little or no trouble, whereas a 5-foot-tall patient would be whereas a 5-foot-tall patient would be less tolerant of the same discrepancy.less tolerant of the same discrepancy.

Clinical Examination Clinical Examination

Limb shortening Limb shortening

The relative knee heights are measured The relative knee heights are measured with the hips and knees flexed. with the hips and knees flexed.

Femoral length Tibial lengthFemoral length Tibial length

Neuromuscular:( muscle wastage on Neuromuscular:( muscle wastage on effected side and contractures ) effected side and contractures )

Joint examination is necessary Joint examination is necessary

Analyzing GaitAnalyzing Gait

The patient's gait is The patient's gait is evaluated for the evaluated for the compensatory compensatory mechanisms i.e.mechanisms i.e.

Pelvic tilting Pelvic tilting Long knee flexionLong knee flexion Pelvic internal Pelvic internal

rotation (less rotation (less frequently) frequently)

Equines footEquines foot

Radiologic Radiologic AssessmentAssessment

History and clinical History and clinical examination may not examination may not always allow for an accurate always allow for an accurate diagnosis diagnosis

scanogram, scanogram, aids in making aids in making a complete diagnosis a complete diagnosis

In growing children, a In growing children, a scanogram should be scanogram should be accompanied by a bone age accompanied by a bone age film of the wrist to correlate film of the wrist to correlate skeletal maturityskeletal maturity

Tibial hemimelia Tibial hemimelia

First described by Otto in 1941First described by Otto in 1941

congenital longitudinal deficiency of the tibia, congenital longitudinal deficiency of the tibia, congenital dysplasia of the tibia, paraxial tibial congenital dysplasia of the tibia, paraxial tibial hemimelia, tibial dysplasia, and congenital hemimelia, tibial dysplasia, and congenital deficiency or absence of the tibiadeficiency or absence of the tibia..

IncidenceIncidence : : 1 in 1 million live births1 in 1 million live births

Causes Causes

Exact cause is unknown, Exact cause is unknown, Sweet and LaneSweet and Lane described a described a

murine model for tibial hemimelia in murine model for tibial hemimelia in which the dominant mutation resides which the dominant mutation resides on the X chromosome.on the X chromosome.

ClassificationClassification Jones, Barnes, and Lloyd-Roberts Jones, Barnes, and Lloyd-Roberts

classification which is based on classification which is based on the early roentgenographic the early roentgenographic presentation; presentation;

In type 1AIn type 1A deformity there is a deformity there is a complete roentgenographic complete roentgenographic absence of the tibia and a absence of the tibia and a hypoplastic distal femoral hypoplastic distal femoral epiphysisepiphysis

In type 1BIn type 1B deformity there also deformity there also is no roentgenographic evidence is no roentgenographic evidence of a tibia, but the distal femoral of a tibia, but the distal femoral epiphysis appears more normal in epiphysis appears more normal in size and shape.size and shape.

In type 2In type 2 deformity a proximal deformity a proximal tibia of varying size is present at tibia of varying size is present at birth. The fibula usually is normal birth. The fibula usually is normal in size, but the head is proximally in size, but the head is proximally dislocateddislocated

Type 3Type 3 deformity, in which the deformity, in which the proximal tibia is not proximal tibia is not roentgenographically visible, is roentgenographically visible, is rare. The distal femoral epiphysis rare. The distal femoral epiphysis usually iswell formed, but the usually iswell formed, but the upper end of the fibula is upper end of the fibula is proximally dislocatedproximally dislocated

Type 4Type 4 (rare) deformity, the tibia (rare) deformity, the tibia is shortened and there is a is shortened and there is a proximal migration of the fibula proximal migration of the fibula with distal tibial fibular diastasiswith distal tibial fibular diastasis

TreatmentTreatment Goal of treatment is a Goal of treatment is a

functional limb equal in functional limb equal in length to the normal limblength to the normal limb

Type 1AType 1A deformities are deformities are most frequently treated with most frequently treated with knee disarticulation and knee disarticulation and sometime reconstruction.sometime reconstruction.

Brown described Brown described reconstruction of type 1A reconstruction of type 1A tibial hemimelia by tibial hemimelia by surgically transferred of surgically transferred of fibula into the intercondylar fibula into the intercondylar notch to create a tibianotch to create a tibia

Success of Brown procedure Success of Brown procedure depends upon presence of depends upon presence of quadriceps mechanism and quadriceps mechanism and absence of knee absence of knee contracturescontractures

In type 1B and type 2In type 1B and type 2 deformities a functional deformities a functional knee joint exists, and knee joint exists, and knee disarticulation is knee disarticulation is not required if the not required if the quadriceps mechanism quadriceps mechanism intactintact

A proximal tibiofibular A proximal tibiofibular synostosis combined synostosis combined with a Syme amputation with a Syme amputation or distal reconstruction or distal reconstruction is the treatment of is the treatment of choicechoice

Type 3Type 3 patients function patients function well as below-knee well as below-knee amputeesamputees

Type 4Type 4 deficiencies, deficiencies, treatment must be treatment must be individualized. Syme individualized. Syme amputation provides amputation provides excellent functionexcellent function

Most patients can be Most patients can be treatedwith combinations treatedwith combinations of distal tibiofibular of distal tibiofibular synostosis and distal fibular synostosis and distal fibular epiphysiodesis.epiphysiodesis.

Equinovarus deformities of Equinovarus deformities of the foot, if present, require the foot, if present, require soft tissue releases.soft tissue releases.

Fibular Hemimelia Fibular Hemimelia

Also known asAlso known as– congenital absence of the fibula,congenital absence of the fibula,– congenital deficiency of the fibula, congenital deficiency of the fibula, – paraxial fibular hemimelia, paraxial fibular hemimelia, – aplasiaaplasia– hypoplasia of the fibula.hypoplasia of the fibula.

CauseCause ------------unknown ------------unknown

Presenting ComplaintsPresenting Complaints

leg-length discrepancy with leg-length discrepancy with equinovalgus deformity of the footequinovalgus deformity of the foot

flexion contracture of the kneeflexion contracture of the knee femoral shorteningfemoral shortening instability of the knee and ankleinstability of the knee and ankle a stiff hindfoot with absent lateral a stiff hindfoot with absent lateral

raysrays

ClassificationClassification

Achterman and KalamchiAchterman and Kalamchi

– type 1 deformity (hypoplasia type 1 deformity (hypoplasia of the fibula) of the fibula)

– type 2 deformity (complete type 2 deformity (complete absence of the fibula).absence of the fibula).

Type 1 deformities are Type 1 deformities are further subdivided into further subdivided into types 1A and 1B.types 1A and 1B.

type 1Atype 1A, the proximal , the proximal fibular epiphysis is distal to fibular epiphysis is distal to the proximal tibial the proximal tibial epiphysis and the distal epiphysis and the distal fibular epiphysis is fibular epiphysis is proximal to the talar dome. proximal to the talar dome. type 1Btype 1B, the deficiency of , the deficiency of the fibula is more severe, the fibula is more severe, with 30% to 50% of the with 30% to 50% of the length missing and no length missing and no distal support for the ankle distal support for the ankle jointjoint

TreatmentTreatment

AimsAims– Equalize the limb length Equalize the limb length – Correction of foot deformityCorrection of foot deformity– Shoe lift and epiphysiodesis of normal Shoe lift and epiphysiodesis of normal

legleg– Syme amputation and prosthetic Syme amputation and prosthetic

rehabilitation when limb length rehabilitation when limb length discrepancy is predicted more then 12-discrepancy is predicted more then 12-15 cm and foot is deformed.15 cm and foot is deformed.

Proximal Femoral Focal Proximal Femoral Focal DeficiencyDeficiency

PFFD consists of a partial skeletal defect in PFFD consists of a partial skeletal defect in the proximal femur with a variably the proximal femur with a variably unstable hip joint, shortening, and unstable hip joint, shortening, and associated other anomalies.e.gassociated other anomalies.e.g– Fibular hemimelia and agenesis of the cruciate Fibular hemimelia and agenesis of the cruciate

ligaments of the kneeligaments of the knee– clubfoot,clubfoot,– congenital heart anomalies, congenital heart anomalies, – spinal dysplasia,spinal dysplasia,– facial dysplasiasfacial dysplasias

ClassificationClassification

Aitken's four-part Aitken's four-part classification schemeclassification scheme– A,B,C,DA,B,C,D

– Class AClass A there is a there is a normal acetabulum normal acetabulum and femoral head with and femoral head with shortening of the shortening of the femur and absence of femur and absence of the femoral neck on the femoral neck on early roentgenogramsearly roentgenograms

Class BClass B there is no there is no bony connection bony connection between the between the proximal femur proximal femur and the femoral and the femoral head, and a head, and a pseudarthrosis is pseudarthrosis is presentpresent

Class CClass C there is there is further degradation further degradation in the formation of in the formation of the hip, the hip, characterized by a characterized by a dysplastic dysplastic acetabulum, acetabulum, absent femoral absent femoral head, and short head, and short femurfemur

Class DClass D the the acetabulum, acetabulum, femoral head, and femoral head, and proximal femur are proximal femur are totally absent totally absent unlike in class C, unlike in class C, there is no ossified there is no ossified tuft capping the tuft capping the proximal femur.proximal femur.

..

Kalamchi et alKalamchi et al developed a simplified developed a simplified classification scheme for congenital deficiency classification scheme for congenital deficiency of the femur that included five groups:of the femur that included five groups:

group I,group I, short femur and intact hip joint; short femur and intact hip joint; group IIgroup II, short femur and coxa vara of the hip, short femur and coxa vara of the hip group IIIgroup III, short femur but well-developed , short femur but well-developed

acetabulum and femoral head; acetabulum and femoral head; group IVgroup IV, absent hip joint and dysplastic , absent hip joint and dysplastic

femoral segment femoral segment group V,group V, total absence of the femur. total absence of the femur.

Nine Pappas Classification of Nine Pappas Classification of Congenital Abnormalities of the Congenital Abnormalities of the

FemurFemur Class IClass I Femur absent

Ischiopubic bone structures Underdeveloped and deficient Lack of Acetabular development

Class II(Aitken D)Class II(Aitken D) Femoral head absent Ischiopubic bone structures delayed in ossification

Class III(Aitken B)Class III(Aitken B) No osseous connection between femoral shaft and head Femoral head ossification delayed Acetabulum may be absent

Class IV(Aitken A)Class IV(Aitken A) Femoral head and shaft joined by irregular calcification in Fibrocartilaginous matrix

Class V (AitkenA)Class V (AitkenA) Femur Incompletely ossified, hypoplastic, and irregular midshaft of femur abnormal

Class VIClass VI Distal femur short, irregular, and hypoplastic irregular distal femoral diaphysis

Class VIIClass VII Coxa vara hypoplastic femur proximal femoral diaphysis irregular with thickened cortex lateral

Class VIIIClass VIII Coxa valga hypoplastic femur femoral head and neck smaller proximal femoral physis horizontal abnormality

Class IXClass IX Hypoplastic femur

TreatmentTreatment

Many methods of limb equalization Many methods of limb equalization are available for treating LLD are available for treating LLD

a) Surgical a) Surgical

b) Nonsurgical)b) Nonsurgical)

Nonsurgical TreatmentNonsurgical Treatment

A 1-cm lift can fit A 1-cm lift can fit comfortably inside of comfortably inside of the shoe;.the shoe;.

Lifts placed on the Lifts placed on the sole of the shoe sole of the shoe function well up to function well up to approximately 3 cm. approximately 3 cm.

Beyond this, the shoe Beyond this, the shoe becomes heavy and becomes heavy and awkward awkward

Surgical ShorteningSurgical Shortening

Accomplished in one of two ways: Accomplished in one of two ways: (1) the physeal growth center can be (1) the physeal growth center can be

retarded or arrested prematurely by retarded or arrested prematurely by epiphysiodesis (growth plate arrest), epiphysiodesis (growth plate arrest), or or

(2) the long bone can be shortened (2) the long bone can be shortened by resecting a segment of the bone.by resecting a segment of the bone.

EpiphysiodesisEpiphysiodesis In 1933, Phemister (9) In 1933, Phemister (9)

described described epiphysiodesis as a epiphysiodesis as a technique to equalize technique to equalize discrepancies of 2 cm discrepancies of 2 cm to 5 cm; to 5 cm;

In recent years, In recent years, percutaneous percutaneous epiphsiodesis using epiphsiodesis using transphyseal screw transphyseal screw (PETS)techniques (PETS)techniques have replaced have replaced Phemister's method Phemister's method

EpiphysiodesisEpiphysiodesis

Bone Shortening Bone Shortening (Resection)(Resection)

Reserved for patientsReserved for patients not candidates for limb not candidates for limb

lengthening, lengthening, do not wish to undergo do not wish to undergo

lengtheninglengthening are skeletally too mature are skeletally too mature

for epiphysiodesis for epiphysiodesis In femurIn femur 5-6 cm 5-6 cm

shortening can be done shortening can be done without seriously effecting without seriously effecting soft tissues.soft tissues.

Tibial shortening osteotomyTibial shortening osteotomy

In tibia 2-3cm shortening can be perform

Surgical LengtheningSurgical Lengthening

Increasing bone length has been attempted by a Increasing bone length has been attempted by a variety of methods, including variety of methods, including

creating arteriovenous shunts, creating arteriovenous shunts, implanting foreign material under the implanting foreign material under the

epiphysis, epiphysis, stripping the adjacent periosteum, stripping the adjacent periosteum, ganglionectomy, ganglionectomy, mechanical distraction. mechanical distraction.

Of these, only mechanical distraction is a Of these, only mechanical distraction is a practical method of limb lengtheningpractical method of limb lengthening

Mechanical Bone Mechanical Bone LengtheningLengthening

Mechanical bone lengthening Mechanical bone lengthening was first reported by Codivilla was first reported by Codivilla in 1905. in 1905.

The lengthening site was held The lengthening site was held in place with plaster and a in place with plaster and a fraction pin fraction pin

In recent years, technical In recent years, technical advances in limb lengthening advances in limb lengthening have focused on the have focused on the development of external development of external fixators that allow for weight fixators that allow for weight bearing and maintenance of bearing and maintenance of joint function during gradual joint function during gradual bone and soft-tissue bone and soft-tissue regeneration. regeneration.

In the past 15 In the past 15 years, the primary years, the primary advancement in advancement in limb lengthening limb lengthening has been the has been the method described method described by Ilizarov, whose by Ilizarov, whose biologic principle of biologic principle of distraction distraction osteogenesis has osteogenesis has revolutionized limb revolutionized limb lengthening. lengthening.

Rotational osteotomy Rotational osteotomy

Van-nes described Van-nes described below knee below knee rotational 180 rotational 180 degree osteotomydegree osteotomy

Precautions Precautions

If shortening is of 12-14 cm lengthening If shortening is of 12-14 cm lengthening can be perform in stages that is first at 4-5 can be perform in stages that is first at 4-5 years and second 8-9 years ageyears and second 8-9 years age

Should done before contracture developedShould done before contracture developed Not recommended before 5 years of age Not recommended before 5 years of age

due to small bone sizedue to small bone size Before treatment Features including Before treatment Features including

scoliosis, pelvic obliquity, contractures, scoliosis, pelvic obliquity, contractures, dysplasias, and angular deformities must dysplasias, and angular deformities must be identified be identified

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