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The hip Developmental dysplasia of the hip (DDH) عادل الهنداوي0د It was previously known as congenital dislocation of the hip (CDH), now it is called DDH because it comprises a spectrum of disorders ranging from acetabular dysplasia without dislocation to instability ( dislocation or subluxation), the unstable hip could be reduced but it is dislocatable or it is dislocated which is either reducible or irreducible. The dislocation can result from shallow acetabulum (acetabular dysplasia) or the dysplasia can result from the absence of the femoral head from the acetabulum (dislocated femoral head). Incidence; at birth the incidence is 10/1000 but after 3 wk it reduce to 1/1000 (most of them become stable), it affect female more than male (7:1), the left hip more than right (3:1) & the condition is bilateral (1 in every 5 cases). Etiology; 1. Genetic factor: the DDH tend to run in families & in some population. 2. Hormonal factor: high level of maternal estrogen, progesterone & relaxin in the last few weeks of pregnancy will cross the placenta & may aggravate the ligammentous laxity in newborn infant, therefore the condition is rare in premature who born before the hormone reach their peak. 3. Intrauterine position: breach presentation increases the incidence. 4. Postnatal factor: the condition is more common is societies who swaddle the babies with the legs together than in population who carry their babies on the back with the legs widely abducted. Pathology: At the birth: the capsule is stretched but no changes in the head & the acetabulum. During the infancy: many of the changes will occur secondary to abnormal position of the femoral head, these include (1) the femoral head dislocated posterolateral to the acetabulum (2) the acetabulum is shallow (3) the capsule is stretched (4) the ligamentum teres become long & hypertrophied (5) the acetabular labrum turned inward & called limbus . After weight bearing all the changes will increase & the femoral head will press on the ilium above the acetabulum to form false socket & the capsule will be squeezed between the iliopsoase & the acetabulum taking the shape of hourglass, the surrounding muscle with time become short. Clinical features; (1) In neonatal period 2 tests are useful in the diagnosis of DDH: Ortolani’s test: normally when flexing both hips to 90 the hips can be abducted to 90, if the hips are dislocated the abduction is limited, in Ortolani’s test we try to reduce the dislocated hip by pressing on the greater trochanter( with the hip flexed & abducted) if the hip reduced it is reducible if not it is irreducible. Barlow’s test: it is the reverse of Ortolani’s test, it is performed by flexing the hip to 90 & adducting it then try to push the head out the acetabulum by pressing with the thumb in the groin, if the head dislocated then it is dislocatable. (2) Late features: the mother may notice asymmetry in skin creases of the proximal thigh (more prominent on the dislocated side), or there is difficulty In applying napkin, on examination there is asymmetrical skin crease, the leg is short & externally rotated, there may be wide perineal gap (in case of bilateral dislocation), there is limited hip abduction & the child walk with limp (unilateral DDH) or waddling gait (in bilateral DDH). X-ray; during the first 6 months the femoral head & the acetabulum are largely cartilaginous & the x-ray is not useful therefore the best way for the diagnosis is the ultrasound. After 6 months there are several radiological lines can be used (1) Shenton’s line ; normally a line drown along the inferior border of the femoral neck should be continuous with the line on the inferior border of the superior pubic ramus, if it is broken then the hip is dislocated or sublaxed, (2) Perkin’s line; it is a vertical line along the outer edge of the acetabulum, the femoral head should be medial to this line, (3)Hilgenreiner’s line; it is a horizontal line that pass through the center of the triradiate cartilage, the femoral head should be below this line,(4) Von Rosen’s line; with the hip abducted to 45 the femoral shaft should point to the acetabulum. . Treatment; it depends on the age of the child; the aim is to obtain & maintain concentric reduction of the femoral head within the acetabulum: From birth-6 months; in the first 3 weeks it need observation as 90% of the DDH will become stable, at the age of 3 weeks; if the hip is stable then no treatment is needed, if the hip is reduced but is dislocatable it need abduction splint, if the hip is dislocated but it is reducible then reduction & abduction splint. The abduction splint (Pavlik harness or Von Rosen) should be continuing until the x-ray show good development of the acetabulum. From the age of 6-18 months; the dislocation can be reduced by closed reduction using skin traction on vertical frame (Gallow’s traction) with gradually increasing abduction for 3weeks until reduction is achieved then apply hip spica followed by splint. If closed reduction failed then open reduction followed by hip spica then splint. From 18 month- 4 yrs; closed reduction is difficult at this age group therefore the treatment of choice is open reduction with derotation osteotomy of the femur & if the acetabulum is shallow then acetabular osteotomy ( Salter’s osteotomy), then hip spica. Above the age of 4 yrs; if bilateral usually no treatment is needed because failure of surgery on one side will convert it from symmetrical to asymmetrical, if it is unilateral the do open reduction until the age of 8 yrs Adult; persistent dislocation in the adult will cause backache & difficulty in the walking usually at the age of 30-40 yrs, if so it can be treated by total hip arthroplasty. Acquired dislocation of the hip Dislocation occurring after the age of 1 is due to one of three causes; 1. Pyogenic arthritis: infection of the hip joint in infant may lead to destruction of the femoral head which is largely cartilaginous with distension of the joint with the pus with subsequent dislocation of the joint. 2. Muscle imbalance: unbalanced paralysis in childhood usually after cerebral palsy, myelomeningiocele, and poliomyelitis will cause weakness of abductor muscles with subsequent dislocation of the hip. 3. Posttraumatic dislocation. Femoral anteversion (In-toe gait) It is a condition in which the child walk with toe directed inward & may trip over his foot during running. Causes; it depend on the age of the child: Below the age of 3 yrs, it may results from forefoot adduction or tibial torsion. Above the age of 3 yrs, it result from excessive anteversion of femoral neck causes the hip to be internally rotated. Clinical features; the gait is clumsy, the child sit on the floor in television position (W- position) & on standing the patellae are turned inwards (squinting patellae). CT scan; to measure the degree of anteversion by taking CT scan across the hip & the knee then measuring the angle between the femoral neck & the transverse axis across the femoral condyles. Treatment; spontaneous correction is usual, if the condition persist after the age of 8 yrs the surgical correction by femoral derotation osteotomy. Coxa vara Normally the neck-shaft angle at birth is 160 & in adult between 125-135, in coxa vara the angle is less than 120. The disorder is either congenital or acquired: Congenital coxa vara; it occur in infancy & early childhood & result from a defect in endochondral ossification of the medial part of the femoral neck, weight bearing the neck bent into varus. Clinical features; it rarely cause hip pain, the leg is short, the thigh bowed & the child walk with Trendelenburg gait because failure of abductor mechanism, bilateral cases because they are symmetrical usually not seen until later when they develop osteoarthritis of the hip. X-ray; the Physeal line is vertical, the femoral neck is short, there may be a small triangle fragment in the medial aspect of the femoral neck. Treatment; this is by subtrochanteric valgus osteotomy, it is indicated if deformity is unilateral, painful or progressive. Acquired coxa vara; this can occur at any age & it result from: A. Bone softening disorders: like rickets, osteomalacia, osteoporosis, Paget’s disease, infection, fibrous dysplasia… B. Fractures like malunion of the subtrochanteric #, non union of femoral neck # or slipped capital femoral epiphysis. Treatment; by treating the underlying cause & correcting the deformity by valgus subtrochanteric osteotomy. Irritable hip It is transient synovitis of the hip, characterized by transient hip pain & restriction of movement in an otherwise healthy child, Clinical features; it affect boys more than girls (2:1), the usual age is 6-12 yrs, the patient presented with painful limp, examination reveals that hip movements are restriction& painful. The condition last 1-2 weeks & resolve spontaneously. Investigations; blood investigations & x-ray are normal but U/S reveals mild joint effusion. Treatment; mild cases require restriction of activity, for severe cases bed rest& skin traction, anti-inflammatory drugs like aspirin. Perthes’ disease It is a painful disorder of the child hood characterized by avascular necrosis of the bony nucleus of the femoral head. Pathogenesis; at the age of 4 months the femoral head receive its blood supply from 3 sources (1) metaphyseal vessels which penetrate the growth plate & supply the head (2) lateral epiphyseal vessels running in the retinacula (3) small vessels in the ligamentum teres. The metaphyseal blood supply decrease gradually until they disappear by the age of 4 yrs, the small blood supply in the ligamentum teres increase by the age of 7 yrs, therefore between 4 &7 yrs the blood supply to the head depend mainly on the lateral epiphyseal vessels, any increase in the intracapsular pressure (from joint effusion) will block these vessels leading to ischaemia. Pathology; the condition take 2-4 yrs to complete passing through 3 stages: 1. Ischemic stage, the femoral head ossific nucleus dead but the overlying cartilage continues to grow so the x-ray shows increase joint space. 2. Revascularization stage, new bone will be formed over the dead bone so the x-ray will show increase bone density, some of the dead bone will be resorbed & replaced by fibrous tissue revealed by decrease density on x- ray, the combined picture of increase & decrease density of the epiphysis will give the x-ray appearance of fragmentation, the metaphysis become wider & porotic 3. Remodeling stage, if the repair process is rapid the femoral head will restore its normal shape, but if the repair process is slow the head will collapse & the growth will distorted, the femoral head will become mushroom or oval shape & become elongated & displaced laterally from the acetabulum. Clinical features; the boys are affected more than girls(4:1), the age of presentation 4-8 yrs, the patient complain from painful limping for weeks which may recur intermittently. On examination the child look well, there may be muscle wasting, the hip is irritable & all movements are painful & restricted especially abduction. X-ray; (1) early stage there is widening of the joint space & asymmetry of bony nucleus ,(2) later on there is increase of bone density & fragmentation,(3) still later there is flattening of the femoral head, lateral displacement of the head, the metaphysis become wide & rarefied. (4) Sagging rope sign, it is a sclerotic line curving across the femoral neck (Sshape line) represent the distal margin of metaphyseal resorption. Prognosis; it depends on several factors: 1. Age, if the child is < 6 yrs the prognosis is good, the older the child the worse the prognosis. 2. Sex, it is more common in male if the female affected the prognosis is worse. 3. Degree of head involvement, the greater the amount the worse the prognosis. Head at risk; these are radiological signs which predict to worse outcome, these signs when present indicate that the head is no longer concentric with the acetabulum & its normal development will be affected, these signs are: Progressive uncovering of the epiphysis. Calcification in the cartilage lateral to the ossific nucleus. Radiolucent area in the lateral part of the bony epiphysis (Gage’s sign). Severe metaphyseal resorption & distortion. Treatment; there are 2 modalities of treatment: A. Symptomatic treatment; by bed rest with skin traction until the pain subside usually within 3 weeks, then resume normal activities but avoid sport with checking x-ray every month. B. Containment treatment; it means keeping the femoral head contained within the acetabulum during healing to restore its normal shape, the containment can be achieved by one of two methods: Keeping the hip widely abducted by plaster splint or removable brace for at least 1 yr. Surgery, by either subtrochanteric varus osteotomy or innominate osteotomy of the pelvis. If the hip is irritable the initial treatment is symptomatic, the definitive treatment will depend on the age of the child & the severity of the disease: 1. Children <6 yrs the treatment is symptomatic. 2. Children 6-8 yrs, if head involvement is mild then symptomatic treatment, if it is severe then containment. 3. Children >9 yrs, require containment treatment by operation. Slipped capital femoral epiphysis (epiphysiolysis) It is displacement of the proximal femoral epiphysis (head) through the hypertrophic zone of the growth plate it occur during the puberty (14-16 yrs), affect boys > girls (3:1), the left hip affected more than right & the condition is bilateral in 20% of the cases. Etiology; 1. Hormonal disturbances: like hypothyroidism or hypogonadism as the condition occur more in sexually immature, fatty adolescent (due to deficiency of sex hormones which are necessary for fusion of growth plate) or occur in tall adolescent (due to excessive secretion of growth hormone which lead to increase in the length of the patient), in both conditions there is increase weight of the patient causing excessive stress across the growth plate. 2. Trauma: which can be major trauma leading to acute slipping (30% of the cases), or repeated mild trauma lead to slow progressive slipping (70% of the cases). Pathology; The slipping occur through the hypertrophic zone of the epiphysis, the femoral head remain in the acetabulum, the femur roll into external rotation & the femoral neck displaced anteriorly threatening the anterior retinacular vessels of the femoral head which to avascular necrosis of the femoral head. The slipping may lead to premature fusion of between the epiphysis & the metaphysis. Clinical features; In 30% the condition occur as acute slip & in 70% it is chronic progressive slip, sometime it is acute on chronic, the patient is a child around puberty & either fatty sexually immature or thin tall. The patient complains from groin pain which may radiate to the knee with limping (these symptoms may recur with exercise). On examination the limb is short & externally rotated with limitation of movement. X-ray; On AP view: a line drawn along the upper border of femoral neck should pass through the femoral head; if not this mean that the head is slipped.(Trethowan’s sign) On lateral view: the angle between the growth plate line & the line that pass through the centre of the femoral neck should be 90, if it is less than 90 this means that the head is slipped. Complications; 1. Avascular necrosis of the femoral head from forceful manipulation or damage to the retinacular vessels during surgery. 2. Chondrolysis of the articular cartilage 3. Deformities like coxa vara (neck-shaft angle less than 20) result from premature fusion of the growth plate. 4. Slipping at other side. 5. Secondary osteoarthritis of the hip. Treatment; is by surgical stabilization of the slip, depending on the degree of the slip: Minor slip; when the slip less than 1/3 of the width of the femoral head, this require fixation in situ (without reduction) by 2-3 screws that pass through the femoral neck into the epiphysis. Moderate slip; when the slip between 1/3-2/3 of the width of the femoral head, in this we accept the deformity & do fixation in situ, then after 2 yrs if the deformity is severe we do corrective osteotomy below the neck. Severe slip; when the slip is more than 2/3 of the width of the femoral head, this can be treated by open reduction & internal fixation or by fixation in situ followed by corrective osteotomy. Pyogenic arthritis of the hip It is usually seen in children less than 2 yrs, the causative microorganism is staphylococcus. The infection starts either as an arthritis or as an osteomyelitis of the proximal femur with secondary arthritis. Clinical features; the child look ill & in pain, the limb is held still, on examination the point of tenderness is over the hip & all movements are restricted. Investigations; X-ray: early on it show soft tissue swelling with displacement of femoral head. U/S: shows joint effusion. Aspiration: reveals pus. Complications; if the infection not treated early, the femoral head & neck will destroy with development of pathological dislocation of the hip. Treatment; drainage the hip by arthrotomy, antibiotics & resting the hip by traction or abduction splint. Osteonecrosis (avascular necrosis of the femoral head) Femoral head is the commonest site of osteonecrosis because its peculiar blood making it vulnerable to ischaemia from arterial cut-off, venous stasis, intravenous stasis, intraosseous sinusoidal compression or combination of these factors. Causes; it is either (1) posttraumatic following # of the femoral neck or hip dislocation the main cause here interruption of arterial blood supply, or (2) non-traumatic Osteonecrosis this is either idiopathic or it is secondary to some other conditions like steroids, alcohol intake, infiltrative disorders(Gaucher’s disease), sickle cell disease, caisson disease, SLE. Pathology (staging) the condition is divided into 4 stages: Stage I; there is little or no pain, the x-ray look normal but the MRI will show the changes. The definitive diagnosis is by bone biopsy. Stage II; the patient complain from hip pain & limitation of movement, the xray will show increase sclerosis within the femoral head (sign of repair process) the femoral head contour is preserved (no collapse of the head) Stage III; there is more advanced stage in which there is structural destruction of the femoral head with loss of the normal contour of the head. Stage IV; there is collapse of the articular surfaces with secondary osteoarthritis. Clinical features; usually the patient is about 20-40 yrs, present with pain, limping &, the condition is bilateral in 50% of the cases. On examination there is limping, +ve Trendelenburg sign, the thigh muscles are wasted, the limb is short because of the collapse of the femoral head, restriction of all hip movements especially abduction & external rotation. There is tendency of the hip to roll into external rotation during passive flexion because the tries to get the damaged part away from stress. X-ray; during the early stages it is normal, the first signs appear only 6-9 months after the occurrence of bone death. First there is increased density of the femoral head (sclerosis) resulting from reactive new bone formation from the surrounding live bone, then destructive changes appears in the necrotic segment as a thin subchondral fracture line called (crescent sign), then there is flattening & collapsed of the femoral head & finally osteoarthritis of the femoral head. MRI; the MRI will shows the changes in the bone marrow long before the xray, there is a band of altered signal intensity running through the femoral head. Treatment; it depends on the stage of the disease; the underlying causative factors should be treated if possible: Stage I & II: analgesia, avoid weight bearing on the affected side by using crutches & by osseous decompression to relief venous stasis & intraosseous hypertension which will relieve the pain & improve blood supply to the femoral head. Stage III: this can be treated by realignment osteotomy to bring the affected area of the head away from point of maximum stress. Stage IV: this can be treated by arthrodesis or arthroplasty (partial or total)