Presentation Lld Farzand

Congenital Limb Length Discrepancy

By Dr Frazand Ali PG-Trainee Orthopedics LGH Lahore

Limb Length Discrepancy 

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

Causes Congenital :  Femoral deficiency  Coxa vara  Fibular hemimelia  Tibial hemimelia  Psuedarthrosis of tibia  Neurofibromatosis Trauma  Over riding fracture  Epiphyseal injuries leading to shortening

Infections  Poliomyelitis  Septic arthritis  Osteomylitis leading to growth plate damage Neurological  Cerebral palsy Tumour  Multiple exostosis Inflamatory  Rheumatoid arthritis

Signs and Symptoms  Limping

gait  Unappealing shoe lift  Low back ache  Compensatory scoliosis  Degenerative arthrosis of lumbar and sacral region

Compensation and Tolerance  Different

Patients respond differently to LLD depending upon age, height and body weight e.t.c.  children tolerate discrepancies better than adults because of their inherent flexibility  a 6-foot-tall patient tolerates a 2-cm discrepancy with little or no trouble, whereas a 5-foot-tall patient would be less tolerant of the same discrepancy.

Clinical Examination 

Limb shortening

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

Femoral length

Tibial length

 Neuromuscular:(

muscle wastage on effected side and contractures )

 Joint

examination is necessary

Analyzing Gait The patient's gait is evaluated for the compensatory mechanisms i.e.  Pelvic tilting  Long knee flexion  Pelvic internal rotation (less frequently)  Equines foot

Radiologic Assessment 

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History and clinical examination may not always allow for an accurate diagnosis scanogram, aids in making a complete diagnosis In growing children, a scanogram should be accompanied by a bone age film of the wrist to

Tibial hemimelia  First

described by Otto in 1941

 congenital

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

Causes  Exact

cause is unknown,  Sweet and Lane described a murine model for tibial hemimelia in which the dominant mutation resides on the X chromosome.

Classification 

Jones, Barnes, and Lloyd-Roberts classification which is based on the early roentgenographic presentation;

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

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

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

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

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

Treatment 

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Goal of treatment is a functional limb equal in length to the normal limb Type 1A deformities are most frequently treated with knee disarticulation and sometime reconstruction. Brown described reconstruction of type 1A tibial hemimelia by surgically transferred of fibula into the intercondylar notch to create a tibia Success of Brown procedure depends upon presence of quadriceps mechanism and absence of knee contractures

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In type 1B and type 2 deformities a functional knee joint exists, and knee disarticulation is not required if the quadriceps mechanism intact A proximal tibiofibular synostosis combined with a Syme amputation or distal reconstruction is the treatment of choice

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Type 3 patients function well as below-knee amputees Type 4 deficiencies, treatment must be individualized. Syme amputation provides excellent function Most patients can be treatedwith combinations of distal tibiofibular synostosis and distal fibular epiphysiodesis. Equinovarus deformities of the foot, if present, require soft tissue releases.

Fibular Hemimelia  Also

– – – – –

known as

congenital absence of the fibula, congenital deficiency of the fibula, paraxial fibular hemimelia, aplasia hypoplasia of the fibula.

Cause ------------unknown

Presenting Complaints  leg-length

discrepancy with equinovalgus deformity of the foot  flexion contracture of the knee  femoral shortening  instability of the knee and ankle  a stiff hindfoot with absent lateral rays

Classification 

Achterman and Kalamchi – type 1 deformity (hypoplasia of the fibula) – type 2 deformity (complete absence of the fibula).

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Type 1 deformities are further subdivided into types 1A and 1B. type 1A, the proximal fibular epiphysis is distal to the proximal tibial epiphysis and the distal fibular epiphysis is proximal to the talar dome. type 1B, the deficiency of the fibula is more severe, with 30% to 50% of the length missing and no distal support for the ankle joint

Treatment  Aims

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Equalize the limb length Correction of foot deformity Shoe lift and epiphysiodesis of normal leg – Syme amputation and prosthetic rehabilitation when limb length discrepancy is predicted more then 1215 cm and foot is deformed.

Proximal Femoral Focal Deficiency 

PFFD consists of a partial skeletal defect in the proximal femur with a variably unstable hip joint, shortening, and associated other anomalies.e.g – Fibular hemimelia and agenesis of the cruciate ligaments of the knee – clubfoot, – congenital heart anomalies, – spinal dysplasia, – facial dysplasias

Classification 

Aitken's four-part classification scheme – A,B,C,D – Class A there is a normal acetabulum and femoral head with shortening of the femur and absence of the femoral neck on early

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

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

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

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Kalamchi et al developed a simplified classification scheme for congenital deficiency of the femur that included five groups: group I, short femur and intact hip joint; group II, short femur and coxa vara of the hip group III, short femur but well-developed acetabulum and femoral head; group IV, absent hip joint and dysplastic femoral segment group V, total absence of the femur.

Nine Pappas Classification of Congenital Abnormalities of the Femur

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Class I Femur absent Ischiopubic bone structures Underdeveloped and deficient Lack of Acetabular development Class II(Aitken D) Femoral head absent Ischiopubic bone structures delayed in ossification Class III(Aitken B) No osseous connection between femoral shaft and head Femoral head ossification

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

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

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

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

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

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Class IX Hypoplastic femur

Treatment  Many

methods of limb equalization are available for treating LLD a) Surgical b) Nonsurgical)

Nonsurgical Treatment 

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A 1-cm lift can fit comfortably inside of the shoe;. Lifts placed on the sole of the shoe function well up to approximately 3 cm. Beyond this, the shoe becomes heavy and awkward

Surgical Shortening Accomplished in one of two ways:  (1) the physeal growth center can be retarded or arrested prematurely by epiphysiodesis (growth plate arrest), or  (2) the long bone can be shortened by resecting a segment of the bone.

Epiphysiodesis 

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In 1933, Phemister (9) described epiphysiodesis as a technique to equalize discrepancies of 2 cm to 5 cm; In recent years, percutaneous epiphsiodesis using transphyseal screw (PETS)techniques have replaced Phemister's method

Epiphysiodesis

Bone Shortening (Resection) Reserved for patients  not candidates for limb lengthening,  do not wish to undergo lengthening  are skeletally too mature for epiphysiodesis  In femur 5-6 cm shortening can be done without seriously effecting soft tissues.

Tibial shortening osteotomy

In tibia 2-3cm shortening can be perform

Surgical Lengthening Increasing bone length has been attempted by a variety of methods, including  creating arteriovenous shunts,  implanting foreign material under the epiphysis,  stripping the adjacent periosteum,  ganglionectomy,  mechanical distraction. Of these, only mechanical distraction is a practical method of limb lengthening

Mechanical Bone Lengthening 

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Mechanical bone lengthening was first reported by Codivilla in 1905. The lengthening site was held in place with plaster and a fraction pin In recent years, technical advances in limb lengthening have focused on the development of external fixators that allow for weight bearing and maintenance of joint function during gradual

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

Rotational osteotomy 

Van-nes described below knee rotational 180 degree osteotomy

Precautions 

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If shortening is of 12-14 cm lengthening can be perform in stages that is first at 4-5 years and second 8-9 years age Should done before contracture developed Not recommended before 5 years of age due to small bone size Before treatment Features including scoliosis, pelvic obliquity, contractures, dysplasias, and angular deformities must be identified

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