schwartz-jampel syndrome is a autosomal recessive disorder of 2 types. Femoral epiphyseal dysplasia is a part of this.
1 n 2 good prognosis.3 n 4 chances of mild to moderate arthritis5 developpainfull arthritis
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epiphysealavascular necrosis of the proximal femoral physis causes an alteration of the longitudinal growth of the femoral neck that allows for a relative “greater trochanteric overgrowth.” This results in a foreshortened femoral neck termed coxabrevis
HISTORY Jacques Calve France 1875 -1954 Arthur Legg USA 1874 - 1939 George Perthes Germany 1869 - 1927
DEFINITION A self limiting non-inflammatory condition, affecting the capital femoral epiphysis with stages of degeneration and regeneration, leading to restoration of the bone nucleus. Osteonecrosis of the proximal femoral epiphysis in a growing child caused by poorly understood non genetic factors
Epidemiology Usually ages 4 – 8 years As early as 2 years, as late as teens Boys:Girls 4 : 1 Bilateral 10 - 12% No evidence of inheritance ?? 10%
Pathogenesis Histological changes described by 1913 Secondary ossification center= covered by cartilage of 3 zones: Superficial Epiphyseal Thin cartilage zone Capillaries penetrate thin zone from below
Pathological stages Stage 1, ischaemia: -dead trabecular bone -collapsed trabecular bone -thickened articular cartilage -physeal disruption -cartilage extending from the physis into the metaphysis
Pathological stages Stage 2, resorption, fragmentation, re- vascularisation, and repair: -invasion of vascular granulation tissue -new bone forming on dead trabeculae -woven new bone formation Stage 3, re-ossification and resolution: -new bone, woven and lamellar Stage 4, re-modelling: -return to normal architecture
Presentation Often insidious onset of a limp Pain in groin, thigh, knee 17% relate trauma history Can have an acute onset
Physical Exam Decreased ROM, especially abduction and internal rotation Trendelenburg test often positive Adductor contracture (due to long standing spasm) Muscular atrophy of thigh/buttock/calf Limb length discrepency (due to head collapse)
Differential Diagnosis CAUSES OF AVASCULAR NECROSIS - sickle cell disease - thalassemia - other hemoglobinopathies - steroid medication - after traumatic hip disloction EPIPHYSEAL DYSLASIAS - multiple epiphyseal dysplasia - spondyloepiphyseal dysplasia
Imaging AP pelvis Frog leg lateral Key = view films sequentially over the course of disease
CLASSIFICATION The classifications for LCPD can be divided into - the one that defines the stage of the disease and - the ones used to prognosticate outcome.Waldenstrom’s radiographic classification defines 4 stages of LCPD during the active phase of the disease
CLASSIFICATION Three radiographic classification systems, namely - the Catterall, - Salter-Thompson, and - lateral pillar,have been developed as prognosticators of outcome that are to be applied at the stage of fragmentation.
Initial Stage Early radiographic signs: Failure of femoral ossific nucleus to grow "Waldenstroms sign" (increased joint space and apparent mild pseudosubluxation) a subchondral fracture may be seen in the early stages in the infracted area (crescent, Salters or Caffreys sign). Irregular physeal plate Blurry/ radiolucent metaphysis
Fragmentation Stage Bony epiphysis begins to fragment Areas of increased lucency and density osteolysis of the superolateral portion of the femoral head (Gage sign on x-ray) Evidence of repair aspects of disease
Salter-Thompson Classification Simplification of Catterall Based on status of lateral margin of capital femoral epiphysis Group A (Catterall I & II equivalent) Group B (Catterall III & IV equivalent)
Lateral Pillar Classification (Herring Classification) 3 groups: A) no lateral pillar involvment B) >50% lat height intact C) <50% lat height intact
Stulberg Classification Class 1 completely normal Class 2 Spherical head with enlargement, short neck, or steep acetabulum Class 3 Non-spherical head (ovoid, mushroom, or umbrella shaped) Class 4 Flat femoral head, flat acetabulum Class 5 Flat femoral head, round acetabulum
Mose Classification (1980) A radiolucent template, with concentric circles, is placed over the femoral head on AP and lateral radiographs. -If the femoral head spheroid does not deviate more that 1 mm from the template, the result is considered as good. -On the other hand, femoral heads deviating within 2 mm or more than 3 mm from the template are considered to be fair and poor, respectively.
Magnetic Resonance Imaging(MRI) with conventional sequencing is used - to assess the extent of femoral head infarction. - provide a good anatomic picture of the cartilaginous femoral head including flat or round shape, - the degree of extrusion of the femoral head, - the degree of superolateral displacement of the femoral head (subluxation), - The eversion of the labrum, and the extent of necrosis.
ARTHROGRAPHY may be used to evaluate possible methods of treatment. opportunity to evaluate coverage and mobility under direct vision during fluoroscopy helpful in assessing containability before any treatment is started. can help identify the best position for femoral head containment and demonstrate absence of hinge abduction prior to containment surgery.
Laredo arthrographic classificationidentifies 5 types of hip; Type 1 hips , are normal. Type 2 hips, the femoral head is still spherical but is larger than normal. Type 3 hips, the femoral head is ovoid in shape. Type 4 hips have a large and flattened femoral head, and the labrum loses its concavivity. Moreover, hinge abduction is present. Type 5 hips show a femoral head larger than normal and saddle shaped; the labrum is still elevated
Computed Tomography Allow early diagnosis of bone collapse, curvilinear zones of sclerosis, Identify intraosseous cysts in later stages of LCP disease. Moreover CT provides precise information about the anatomic relationship between femoral head and acetabulum. Allow study of the 3-dimensional nature of the deformity.
Bone Scintigraphy Precedes radiographic changes by an average of 3 months. Does not describe the extent of femoral head involvement, rather The scintigraphic patterns are associated with the revascularization versus recanalization process.
PROGNOSIS 60% of kids do well without Rx AGE is key prognostic factor: <6y = good outcome regardless of Rx 6-8y = not always good results with just containment >9y = containment option is questionable, poorer prognosis, significant residual defect
Poor Prognosis Sex Bone age Uncovering of Femur head Percentage involvement Adduction contracture Weight
Catterall’s Head at risk signsAlong with his classification system, Catterall also described head-at-risk signs associated with a poor outcome. Lateral subluxation of the head Whole of the head involved Calcification lateral to epiphysis Metaphyseal cysts Gage’s sign Horizontal physis
Management of Perthes Disease The primary long-term goal of treatment of Legg- Calve´ -Perthes disease is to try to prevent secondary degenerative arthritis of the hip in adult life by achieving the short-term goal. The primary short-term goal of treatment of Legg- Calve´ -Perthes disease is to try to ensure that when the disease is completely healed the femoral head is spherical, and minimally enlarged.
TIME FRAMES The treatment of Legg-Calve´ -Perthes disease needs to be divided into 3 distinct time frames: a.) Early in the course of the disease: from the onset of the disease to the early fragmentation stage b.) Late in the course of the disease: from the late fragmentation stage to full reossification of the femoral head (complete healing) c.) After complete healing: after the disease has healed and residual sequelae are present
TREATMENT EARLY IN THE COURSEOF THE DISEASE The goal of treatment early in the course of the disease is to retain the normal shape of the femoral head by:a.) Identifying patients at risk for a poor outcome as soon as possibleb.) Containing the femoral head as early as possible in patients at risk of a poor outcome
TREATMENT EARLY IN THE COURSEOF THE DISEASE Containment may be achieved by nonoperative or operative means and surgical options include femoral and /or pelvic surgery Containment may or may not be combined with weight relief In order for containment to be successful, it should be achieved before the late stage of fragmentation Containment should be maintained until the late reconstitution (reossification) stage
METHODS OF CONTAINMENT Bed rest and range of motion exercises Casts and Bracing: Removable abduction orthosis Pietrie casts Hips abducted and internally rotated
Containment by proximal femoralvarus osteotomy.
CONTAINMENT BY SALTEROSTEOTOMY The Salter osteotomy is a transverse osteotomy of the pelvis along a line from the sciatic notch to just above the anterior inferior iliac spine. The acetabulum is then rotated laterally and anteriorly using the pubic symphysis as a hinge.
CONTAINMENT BY SHELFPROCEDURE Shelf acetabuloplasty is a procedure where the margin of the acetabulum is extended to provide more coverage for the femoral head One advantage of the shelf acetabuloplasty is preservation of limb length without excessive trochanteric prominence. Another advantage is long-term improved coverage of the enlarged femoral head that develops following Perthes disease.
CONTAINMENT BY TRIPLE PELVICOSTEOTOMY The triple pelvic osteotomy combines - the transverse osteotomy of Salter with - complete osteotomies of the superior pubic ramus and ischium.This allows greater mobility of the acetabulum without interfering with growth of the triradiate cartilage.
TREATMENT EARLY IN THE COURSEOF THE DISEASEThe decision to consider containment treatment early in the course of the disease is primarily governed by the age of onset of the disease with patients divided into 4 age groups. - < 5 yrs - 5 to 8 yrs - 8 to 12 yrs - > 12 yrs
TREATMENT EARLY IN THE COURSEOF THE DISEASE Children less than 5 years of age at the onset of the disease: treatment is seldom needed regardless of severity of involvement of the femoral head. (However, if femoral head extrusion occurs treatment will be needed)
TREATMENT EARLY IN THE COURSEOF THE DISEASEChildren 5 years or older but less than 8 years of age:a. Early containment is indicated if it is possible to determine that more than half the femoral epiphysis is necrotic.b. Early determination cannot be made in most patients. These patients should be monitored with periodic (4-monthly) radiographs to detect early extrusion of the femoral head.
TREATMENT EARLY IN THE COURSEOF THE DISEASEc. Containment treatment should be considered as soon as extrusion of the femoral head is detected.d. Extrusion is determined by a break in the Shenton line.e. No containment is needed in this age group when extrusion does not occur
TREATMENT EARLY IN THE COURSEOF THE DISEASEChildren >8 years but < 12 years of age:a. Should be treated by containment as soon as the disease is diagnosed regardless of the extent of necrosis. Containment should be initiated before the fragmentation stage and before extrusion whenever possibleb. Alternative methods should be considered when the patient presents in the late stage of fragmentation
TREATMENT EARLY IN THE COURSEOF THE DISEASE Children 12 years of age or older at the onset of the disease:Containment should NOT be considered in these adolescents as it does not work.Treatment considerations should be similar to treatment of adults with osteonecrosis
TREATMENT LATE IN THE COURSEOF THE DISEASE The goal of treatment of Legg-Calve´ - Perthes late in the course of the disease is to attempt to minimize the extent of deformation of the femoral head that has already developed. The treatment in the late fragmentation stage may be remedial or salvage depending on - the deformity of the femoral head or - the presence of hinge abduction
TREATMENT LATE IN THE COURSEOF THE DISEASE In children who have hinge abduction the goal of treatment is -to correct hinge abduction and -facilitate some remodeling of the femoral head. Containment may be considered if the femoral head can be contained without hinge abduction. If hinge abduction is present. A valgus femoral osteotomy is a reliable choice to improve motion and reduce pain.
TREATMENT AFTER HEALING OFTHE DISEASE The goals of treatment of adolescents or young adults with healed Legg-Calve´ - Perthes disease and deformity of the femoral head is to - improve function, - relive pain, and - delay the onset of secondary degenerative arthritis The treatment approach depends on the specific cause of pain, dysfunction, or deformity
TREATMENT AFTER HEALING OFTHE DISEASE If the femoral head is spherical or ovoid and there is coxa brevis with a Trendelenburg gait, consider trochanteric advancement with or without lengthening the femoral neck If there is pain on account of femoro-acetabular impingement consider repairing the labral pathology and/or correcting impingement
TREATMENT AFTER HEALING OFTHE DISEASE A deficient acetabular roof may require- labral support or- pelvic osteotomy with or without realignment of the proximal femur Symptoms caused by osteochondritis dessicans can sometimes be relieved by removing the loose fragment
TREATMENT AFTER HEALING OFTHE DISEASE The role of reshaping a grossly deformed femoral head is uncertain although in a few selected cases of moderate deformity, it may be of benefit -Femoral head reduction osteotomy done for coxa megna.
TREATMENT AFTER HEALING OFTHE DISEASE When the articular surface is severely damaged salvage procedures such a total hip replacement should be considered
ROLE OF CORE DECOMPRESSION May be a useful adjunct to current methods of treatment. Younger children with early stage disease may benefit from multiple small drillings, Largecore decompression, debridement, and grafting for Perthes disease in older children with larger lesions and more advanced stages of disease.