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1

Review Sprengel Disorder

Clinical features

Undescended scapula often associated with winging, hypoplasia, and omovertebral connections (30% of cases; Fig. 3.1)

Most common congenital anomaly of the shoulder in children

Affected scapulae are usually small, relatively wide, and medially rotated.

Associated diseases:

Klippel-Feil syndrome (Sprengel deformity in one-third of cases)

Kidney disease

Scoliosis

Diastematomyelia

2

Review Congenital Pseudoarthrosis of the clavicle

Clinical features

Failure of union of medial and lateral ossification centers of right clavicle

Bilateral in less than 10%; left side if situs inversus

Manifests as an enlarging, painless, nontender mass

Causes

May be related to pulsations of the underlying subclavian artery

Radiographic findings

Anteroposterior (AP) view of the clavicle reveals rounded sclerotic bone at the pseudarthrosis site.

Treatment

Usually asymptomatic and does not require treatment

Surgery (open reduction and internal fixation [ORIF] with bone grafting) is indicated for unacceptable cosmetic deformities or significant functional symptoms (mobility of fragments and winging of scapula) at age 3–6 years.

Successful union is predictable (in contrast to congenital pseudarthrosis of tibia).

3

Review Poland Syndrome

Clinical features

Unilateral chest wall hypoplasia (sternocostal head of pectoralis major absent)

Hypoplasia of hand and forearm

Symbrachydactyly and shortening of middle fingers—simple syndactyly of ulnar digits, absence of shortening of middle digits

Examination findings

Chest deformities: chest wall hypoplasia, Sprengel deformity, scoliosis

Hand deformities

Absence or hypoplasia of metacarpals and phalanges

Carpal bone abnormalities

Absence of flexor and extensor tendons

Can be associated with radioulnar synostosis

Treatment: syndactyly release; caution should be taken about possible lack of soft tissue coverage requiring full-thickness skin graft

4

Review Apert Syndrome

Autosomal dominant due to mutation in FGFr2 gene

Characteristics

Bilateral complex syndactyly of hands and feet

Craniosynostosis—premature closure of cranial sutures—flattened skull with broad forehead

Ankylosis of interphalangeal joints (symphalangism)

Radioulnar synostosis

Glenoid hypoplasia

Decreased mental capabilities

Treatment

Surgical release of border digits done at 1 year of life

Digital reconstruction of middle digits to turn 3 digits into 2 digits done at 2 years old

5

Review Brachial Plexus Palsy

Clinical features

In 2 per 1000 births, an injury is still associated with stretching or contusion of the brachial plexus.

Typically manifests as internal rotation shoulder contracture and elbow and wrist flexion contractures

Progressive glenoid hypoplasia occurs in 70% of children with significant internal rotation contracture.

Hand function varies with level of brachial plexus deformity.

Three types commonly recognized (Table 3.1):

Erb-Duchenne (C5, C6)—best prognosis, most common

Klumpke (C8, T1)—poor prognosis

Total plexus palsy (C5–T1)—worst prognosis

Causes

Large size of neonate, shoulder dystocia, forceps delivery, breech position, prolonged labor

Radiographic studies

Investigations have focused on the position of the humeral head within the glenoid.

Posterior subluxation with erosion of the glenoid should be prevented.

Axillary lateral view of the shoulder should be obtained to evaluate position of humeral head.

If surgical reconstruction is planned, computed tomography (CT) scanning instead of magnetic resonance imaging (MRI) of the shoulder should be considered.

Treatment

Key to success of therapy consists of maintaining passive range of movement (ROM) and awaiting return of motor function (up to 18 months).

Parents should focus on passive elbow motion and shoulder elevation, abduction, and external rotation.

More than 90% of cases eventually resolve without intervention.

Lack of biceps function 6 months after injury and the presence of Horner syndrome carry a poor prognosis.

Options: early surgery to address nerve function, late surgery to address deformities

Microsurgical nerve grafting

Latissimus and teres major transfer to shoulder external rotators (L’Episcopo)

Tendon transfers for elbow flexion (Clark pectoral transfer and Steindler flexorplasty)

Pectoral and subscapularis release for internal rotation contracture and secondary glenoid hypoplasia (<5 years old)

Proximal humerus rotational osteotomy (>5 years old)

Release of the subscapularis tendon for internal rotation contracture, if performed by age 2 years, may result in improved active external rotation of the shoulder, with muscle transfer to assist in active external rotation.

6

Review rotational issues of the lower extremity

Introduction

In-toeing usually attributable to metatarsus adductus (in infants), internal tibial torsion (in toddlers), and femoral anteversion (in children <10 years)

Out-toeing typically a result of external rotation hip contracture (in infants) and external tibial torsion and external femoral torsion (in older children and adolescents)

All these problems may be a result of intrauterine positioning.

Deformities usually bilateral; clinician should be wary of asymmetric findings.

Evaluation should include measurements listed in Table 3.2 and illustrated in Fig. 3.2.

Metatarsus adductus

Clinical features

Forefoot adducted at tarsal-metatarsal joint

Lateral border of foot is convex instead of straight

Usually seen during first year of life

May be associated with hip dysplasia (10%–15% of cases)

Approximately 85% of cases resolve spontaneously.

Treatment

Nonoperative

Stretching exercises are used for feet that can be passively corrected to neutral position (heel bisector line aligns with second metatarsal).

Feet that cannot be passively corrected (rare situation) usually respond to serial casting, with mixed results.

Surgery

Lateral column shortening and medial column lengthening if patient older than 5 years old (mixed results)

Internal tibial torsion

Clinical features

Most common cause of in-toeing

Usually seen during second year of life and can be associated with metatarsus adductus and developmental dysplasia of the hip (DDH)

Internal rotation of tibia causes pigeon-toed gait.

Transmalleolar axis is internal.

Thigh-foot axis of −10 degrees

Treatment

Resolves spontaneously with growth

Operative correction is rarely necessary except in severe case, which is addressed with a supramalleolar osteotomy when child is between 7 and 10 years of age.

External tibial torsion

Clinical features

Cause of out-toeing; may cause disability and decrease physical performance

Can worsen with growth—normal increase in external torsion

Associated with increased femoral anteversion (miserable malalignment syndrome), early degenerative joint disease, neuromuscular conditions

Manifests as knee pain due to patellofemoral malalignment

Thigh-foot axis > 40

Treatment

Rest, rehabilitation

Supramalleolar osteotomy if child older than 8–10 years and external tibial torsion more than 40 degrees

Femoral anteversion

Clinical features

Internal rotation of femur; seen in 3- to 6-year-olds

Children with this problem classically sit with the legs in a W shape.

If associated with external tibial torsion, may lead to patellofemoral problems

Treatment

Disorder usually corrects spontaneously by age 10

Special shoes, therapy, and derotational braces have never been shown to improve rates of remodeling.

In older children with less than 10 degrees of external rotation, femoral derotational osteotomy (intertrochanteric is best) may be considered for cosmesis, although this is not a functional problem.

7

Review Leg Length Discrepancies

Many potential causes

Congenital disorders (e.g., hemihypertrophy, dysplasias, proximal femoral focal deficiency [PFFD], DDH)

Paralytic disorders (e.g., spasticity, polio)

Infection (disruption of physis)

Tumors

Trauma

Long-term problems associated with leg length discrepancy (LLD) include inefficient gait, equinus contractures of ankle, postural scoliosis and low back pain, possible hip osteoarthritis with uncovering of the femoral head of the long leg.

Discrepancy must be measured accurately (e.g., with blocks of set height under affected side; with scanography).

Lateral CT scanography—more accurate than conventional scanography if there are soft tissue contractures of hip, knee, or ankle

Can be tracked with Green-Anderson data, Moseley graph (with serial leg length radiographs or CT scanograms and with bone age determinations) or the Paley multiplier method (most accurate for congenital deformities)

A gross estimation of LLD can be made under the following assumption of growth per year up to age 16 in boys and age 14 in girls (Fig. 3.3):

Distal femur: ⅛ inch (9 mm) per year

Proximal tibia: ¼ inch (6 mm) per year

Proximal femur: ⅛ inch (3 mm) per year

More accurate results with Moseley graph

Treatment

In general, projected discrepancies at maturity of less than 2 cm are observed or treated with shoe lifts.

More than 50% of population have up to 2 cm of LLD and are asymptomatic.

Discrepancies of 2 to 5 cm

Epiphysiodesis of the long side

Shortening of the long side (ostectomy)

Lengthening of the short side

Discrepancies of more than 5 cm are generally treated with lengthening.

With use of standard techniques, lengthening of 1 mm a day is typical.

Ilizarov principles are followed, including metaphyseal corticotomy (preserving medullary canal and blood supply) followed by gradual distraction.

On rare occasions, physeal distraction (chondrodiastasis) can be considered.

This procedure must be performed in patients near skeletal maturity, because the physis almost always closes after this type of limb lengthening.

8

Quick review OCD

Clinical features

An intraarticular condition common in children 10 to 15 years of age that can affect many joints, especially the knee and elbow (capitellum)

Lesion thought to be secondary to trauma, ischemia, or abnormal epiphyseal ossification

Posterolateral portion of medial femoral condyle is most frequently involved

Symptoms include activity-related pain, localized tenderness, stiffness, and swelling, with or without mechanical symptoms.

Differential diagnosis includes anomalous ossification centers.

Radiographic studies

Tunnel (notch) view to evaluate condyles

MRI can determine whether there is synovial fluid behind the lesion (the worst prognosis for nonoperative healing).

Treatment

Nonoperative

Bracing and restricted weight bearing if the potential for growth remains significant (highest healing rates with open physes)

Operative

Surgical therapy is reserved for the adolescent with minimal growth left or a loose lesion.

Operative treatment includes drilling with multiple holes, fixation of large fragments, and bone grafting of large lesions.

Osteochondritis dissecans is commonly treated arthroscopically.

Poor prognosis is associated with lesions in the lateral femoral condyle and patella.

9

Quick review Discoid Meniscus

Clinical features

Abnormal development of the lateral meniscus leads to formation of a disc-shaped (or hypertrophic) meniscus rather than the normal crescent-shaped meniscus.

Symptoms include mechanical block and pain with catching and palpable click at knee.

Radiographic findings

Widening of the cartilage space on the affected side (up to 11 mm)

Squaring of condyles may be visible.

MRI yields three successive sagittal images with the meniscal body present.

Classification

Complete covering of tibial plateau

Incomplete covering of tibial plateau

Wrisberg variant—lacks posterior meniscotibial attachment; unstable

Treatment

If symptomatic and torn, the discoid meniscus can be arthroscopically débrided and then saucerized so it resembles a normal-appearing meniscus.

If not torn, it should be observed.

If detached (Wrisberg variant), meniscus should be repaired.

10

Quick review congenital dislocation of the knee

Clinical features

Spectrum of disease from rigid dislocation to mild contractures

Classic position is knee hyperextension

Often occurs in patients with myelodysplasia, arthrogryposis, Larsen syndrome

Structural components

Quadriceps contracture

Tight collateral ligaments and anterior subluxation of hamstring tendons

Associations

Developmental hip dysplasia (50% of patients have concomitant DDH)

Clubfoot

Metatarsus adductus

Treatment

Nonoperative

Reduction with manipulation and serial casting

Weekly casting

Knees should be reduced and cast before being treated with a Pavlik harness for DDH.

Operative

Performed if failure to achieve 30 degrees of knee flexion after 3 months of casting

Goal is to achieve 90 degrees of knee flexion.

Quadriceps lengthening (V-Y-plasty or Z-plasty)

Hamstring tendon transposition posteriorly

Collateral ligament mobilization

11

Review Tibial Bowing

 

ALways look

Probably mild

 

Posteromedial tibial bowing

Physiologic—thought to be due to intrauterine positioning. Posteromedial (PM) bowing is probably mild.

Usually of the middle and distal thirds of the tibia (Fig. 3.17)

Commonly associated with LLD, calcaneovalgus feet, and tight anterior structures

Spontaneous correction is the rule, but patient should be monitored to evaluate LLD.

Most common sequela of posteromedial bowing is an average LLD of 3 to 4 cm, which may necessitate an age-appropriate epiphysiodesis of the long limb.

Tibial osteotomies not indicated

Anteromedial tibial bowing

Commonly caused by fibular hemimelia (a congenital longitudinal deficiency of the fibula, which is the most common long-bone deficiency)

In addition to anteromedial bowing, fibular hemimelia is associated with:

Ankle instability due to ball-and-socket joint

Equinovalgus foot (with or without lateral rays)

Tarsal coalition

Femoral shortening (coxa vara, PFFD)

ACL insufficiency

Significant LLD often results from this disorder.

Classically, skin dimpling is seen over the tibia.

The fibular deficiency can be intercalary, which involves the whole bone (fibula is absent), or terminal.

Fibular hemimelia is linked to the sonic hedgehog gene.

Radiographic findings

Complete or partial absence of fibula, a ball-and-socket ankle joint (secondary to tarsal coalitions), and deficient lateral rays in the foot

Treatment

Varies from a simple shoe lift or bracing to Syme amputation

Decisions are based on the degree of foot deformity, the number of rays, and the extent of shortening of the limb.

Amputation is usually performed in the patient with a severely shortened limb or a stiff, nonfunctional foot, at about 10 months of age.

For less severe cases, reconstructive procedures, including lengthening, may be an alternative.

Such a procedure should include resection of the fibular remnant to avoid future foot problems.

Anterolateral tibial bowing

Clinical features

Congenital pseudarthrosis of the tibia is the most common cause of anterolateral bowing. Rarely is anterolateral (AL) bowing physiologic, so one should always look.

Often accompanied by neurofibromatosis

About 50% of patients with anterolateral tibial bowing have neurofibromatosis.

Only 10% of patients with neurofibromatosis have tibial bowing.

Treatment

Initial management/workup should include genetic consultation to check for possibility of neurofibromatosis.

Initial treatment includes a total-contact brace to protect the patient from fractures.

Intramedullary fixation with excision of hamartomatous tissue and autogenous bone grafting are options for nonhealing fractures.

A vascularized fibular graft or the Ilizarov method should also be considered if bracing fails.

Osteotomies to correct the anterolateral bowing are contraindicated.

Amputation (Syme) and prosthetic fitting are indicated after failure of two or three surgical attempts or as primary treatment.

Syme amputation is preferred to below-knee amputation in these patients because the soft tissue available at the heel pad is superior to that in the calf as a weight-bearing stump.

The soft tissue in the calf in these patients is often scarred and atrophic.

12

Tibial Hemimelia

Congenital longitudinal deficiency of the tibia

The only long-bone deficiency with a known inheritance pattern (autosomal dominant)

Much less common than fibular hemimelia and often associated with other bone abnormalities (especially a lobster-claw hand)

Also associated with insufficient extensor mechanism, clubfoot deformity

Clinically, the extremity is shortened and bowed anterolaterally with a prominent fibular head and an equinovarus foot, with the sole of the foot facing the perineum.

Treatment for severe deformity with total absence of the tibia is a knee disarticulation.

Fibular transposition (Brown) has been unsuccessful, especially when quadriceps function and the proximal tibia are absent.

When the proximal tibia and quadriceps functions are present, the fibula can be transposed to the residual tibia to create a functional below-knee amputation.

13

Review Genu Varum

Infantile:

Stage 1 or 2 bracing in less than 3 years old

Stage 2 if over age three and stage 3 then is a proximal tibia osteotomy

Stage 4-6 are complex and often require epiphysiolysis

 

Adolescent:

usually less severe, widening of the medial tibia physis

treat with proximal lateral tibia/fibula hemiphyiodesis

 

14

Review tibiofemoral angle and Drennan Metaphyseal-diaphyseal angle in tibia Vara

Comparison of tibiofemoral angle with the Drennan metaphyseal-diaphyseal angle in tibia vara. (A) Lines are drawn along the longitudinal axes of the tibia and femur; the angle between the lines is the tibiofemoral angle (32 degrees). (B) The metaphyseal-diaphyseal angle method is used to determine the metaphyseal-diaphyseal angle in the same extremity. A line is drawn perpendicular to the longitudinal axis of the tibia, and another is drawn through the two beaks of the metaphysis to determine the transverse axis of the tibial metaphysis. The metaphyseal-diaphyseal angle (20 degrees) is the angle bisected by the two lines.

15

Review Genu Varus Miller. book

Genu varum (bowed legs) normally evolves naturally to genu valgum (knock-knees) by age 2.5 years, with a gradual transition to physiologic valgus angulation by age 4 years (Fig. 3.14).

Physiologic genu varum (bowed legs)

Normal in children younger than 2 years

Radiographs in physiologic bowing typically show flaring of the tibia and femur in a symmetric manner.

Pathologic conditions that can cause genu varum include osteogenesis imperfecta, osteochondromas, trauma, various dysplasias, and (most commonly) Blount disease.

Infantile Blount disease (age 0–4 years)

Clinical features

Abnormal tibia vara

More common and usually affects both extremities

Classic presentation is in a child who is overweight and who begins walking before 1 year of age; disease is associated with internal tibial torsion.

Radiographic findings

Metaphyseal-diaphyseal angle abnormality and metaphyseal beaking

A Drennan metaphyseal-diaphyseal angle of more than 16 degrees is considered abnormal; the angle is formed between the metaphyseal beaks (demonstrated in Fig. 3.15).

Langenskiöld classification is based on degree of metaphyseal-epiphyseal changes (Fig. 3.16).

Treatment: based on age and correlated with stage of disease

Stage I or II: bracing in patients younger than 3 years

Better outcomes if unilateral or nonobese patients

Very difficult to ensure compliance

Stage II (if patient >3 years) and stage III: proximal osteotomy for tibia/fibula valgus angulation to overcorrect the deformity (because medial physeal growth abnormalities persist)

Stages IV to VI are complex, and multiple procedures may be required.

Epiphysiolysis is also needed for stages V and VI disease.

Adolescent Blount disease

Clinical features

Less severe than infantile forms and more often unilateral

Epiphysis appears relatively normal and does not have the beaking seen in infantile forms

Most characteristic radiographic finding is widening of the proximal medial physis

Thought to be from mechanical overload in genetically susceptible patients (obese, African American)

Treatment

Initial treatment is proximal tibial and fibular lateral hemiepiphysiodesis when growth remains.

Larger plates are usually required because of incidence of plate failure.

If residual deformity exists or physes are closed proximally, tibial and fibular osteotomy is performed.

When significant LLD is present, the Ilizarov technique allows for deformity correction and lengthening.

Genu valgum (knock-knees)

Clinical features

Up to 15 degrees at the knee is common in children 2 to 6 years of age.

Maximum valgus between ages 3 and 4

Cases within this physiologic range do not require treatment.

Differential diagnosis includes renal osteodystrophy (most common cause if condition is bilateral), tumors (e.g., osteochondromas), infections (may stimulate proximal asymmetric tibial growth), and trauma.

Treatment

Conservative treatment is ineffective in pathologic genu valgum.

Surgery at the site of the deformity should be considered in children older than 10 years with more than 10 cm between the medial malleoli or more than 15 degrees of valgus angulation.

Hemiepiphysiodesis (temporary or timed) of the medial side is effective for severe deformities if performed before the end of growth.

16

Treatment protocol for DDH

17

Ultrasound Evaluation of DDH

 

alpha above 60

femoral head bisected by illeum

Dynamic ultrasonography is useful for making the diagnosis in young children before ossification of the femoral head (which occurs at age 4–6 months) (Fig. 3.4).

Also useful for assessing reduction in a Pavlik harness and diagnosing acetabular dysplasia or capsular laxity

Success dependent on operator’s skill

On the coronal view, the normal α angle is greater than 60 degrees, and the femoral head is bisected by the line drawn down the ilium

18

Radiographic assessment of DDH

 

acetabular index less than 25 degrees

medial to perkins line

A and B) Drawings of the common radiographic measurements used to evaluate developmental dysplasia of the hip, anterior view. Note the delayed ossification, disruption of the Shenton line, and increased acetabular index on the left hip, which is dislocated.

19

DDH overview

Introduction

Represents abnormal development or dislocation of the hip secondary to capsular laxity and mechanical factors (e.g., intrauterine positioning)

Spectrum of disease

Dysplasia—shallow acetabulum

Subluxation

Dislocation

Teratologic—dislocated in utero and irreducible; associated with neuromuscular conditions and genetic abnormalities

Late dysplasia (adolescent and adult)

Risk factors

Breech positioning, positive family history (ligamentous laxity), female sex, and being a firstborn child (in that order)

Less intrauterine space accounts for increased incidence of DDH in firstborn children.

DDH is observed most often in the left hip (67% of cases), in girls (85%), in infants with a positive family history (≥20%), in the presence of increased maternal estrogens, and in breech births (30%–50%).

Also associated with postnatal positioning such as swaddling with the hips in extension

Clinical features

Associated with other problems related to intrauterine positioning, such as torticollis (20% of cases) and metatarsus adductus (10%); no association with clubfoot

If left untreated, muscles about the hip become contracted, and the acetabulum becomes more dysplastic and filled with fibrofatty tissue (pulvinar).

Potential obstructions to obtaining a concentric reduction in DDH:

Iliopsoas tendon

Pulvinar

Hypertrophied ligamentum teres

Contracted inferomedial hip capsule

Transverse acetabular ligament

Inverted labrum

The teratologic form is most severe and usually necessitates early surgery.

A pseudoacetabulum is present at or near birth.

Teratologic hip dislocations commonly manifest in association with syndromes such as arthrogryposis and Larsen syndrome.

Diagnosis

Physical examination

Early diagnosis possible with Ortolani test (elevation and abduction of femur relocates a dislocated hip) and Barlow test (adduction and depression of femur dislocates a dislocatable hip)

All children should undergo screening via physical examination.

Advanced screening is controversial, but screening ultrasound should be done for children with significant risk factors (breech position, family history).

Three phases are commonly recognized:

Dislocated (positive result of Ortolani test early; negative result of Ortolani test late, when femoral head cannot be reduced)

Dislocatable (positive result of Barlow test)

Subluxable (suggestive result of Barlow test)

Subsequent diagnosis is made with limitation of hip abduction in the affected hip as the laxity resolves and stiffness becomes more clinically evident.

Caution: abduction may be decreased symmetrically with bilateral dislocations.

Galeazzi sign—demonstrated by the clinical appearance of foreshortening of the femur on the affected side.

Performed with the patient’s feet held together and knees flexed (a congenitally short femur can also cause the Galeazzi sign)

Other clinical findings associated with DDH include asymmetric gluteal folds (less reliable) and Trendelenburg stance (in older children), increased lumbar lordosis, and pelvic obliquity.

Repeated examinations, especially in an infant, are important because a child’s irritability can prevent proper evaluation.

Radiography

Dynamic ultrasonography is useful for making the diagnosis in young children before ossification of the femoral head (which occurs at age 4–6 months) (Fig. 3.4).

Also useful for assessing reduction in a Pavlik harness and diagnosing acetabular dysplasia or capsular laxity

Success dependent on operator’s skill

On the coronal view, the normal α angle is greater than 60 degrees, and the femoral head is bisected by the line drawn down the ilium.

Radiographic studies and findings (Fig. 3.5)

Used in older children (after age 4 months)

Measurement of the acetabular index (normal <25 degrees)

Measurement of the Perkin line (normally the ossific nucleus of the femoral head is medial to this line)

Evaluation of the Shenton line useful

Later, delayed ossification of the femoral head on the affected side may be visible (femoral head ossification begins to show between 4 and 6 months).

Arthrography used to help judge closed reduction and possible blocks to reduction

Advanced imaging (CT, MRI) helpful after closed reduction to determine concentric reduction

Treatment (Fig. 3.6)

Based on achieving and maintaining early “concentric reduction” to prevent future degenerative joint disease. Specific therapy is determined by the child’s age.

Birth to 6 months

In hips that have normal examination findings but abnormal ultrasound findings, treatment recommendations are uncertain

Children should have close follow-up.

Repeat ultrasound at age 6 weeks, with treatment if continued signs of dysplasia

Pavlik harness

All Ortolani-positive (dislocated but reducible) hips should be treated with Pavlik harness.

Barlow-positive (reduced but dislocatable) hips may stabilize without treatment but should be watched closely; many writers advocate treating with Pavlik harness with observation.

If dislocated, reduction should be checked weekly with ultrasonography for 3 weeks.

Not reduced: transition to rigid abduction orthosis versus closed reduction, arthrography, and spica casting should be considered.

Reduced: continue harness until findings of examination and ultrasonography are normal.

6 to 18 months:

Hip arthrography, percutaneous adductor tenotomy, closed reduction, and spica casting

Postreduction CT or MRI used to confirm concentric reduction

If closed reduction fails: open reduction

18 months to 3 years:

Open reduction

3 to 8 years:

Osteotomy

Salter, Dega, Pemberton, or Staheli procedure

Older than 8 years:

Osteotomy

Growth plate open: triple (Steele), double pelvic (Southerland), Staheli procedure

Growth plate closed: Ganz and Chiari procedures

Total hip arthroplasty is performed when the child is an adult.

Specific treatment modalities

Pavlik harness

Designed to maintain reduction in infants (<6 months) in about 100 degrees of flexion and mild abduction (the “human position” [Salter position])

Confirm reduction by radiographs or ultrasound after placement of harness and brace adjustment.

Position of the hip should be within the safe zone of Ramsey (between maximum adduction before redislocation and excessive abduction, which increases risk of avascular necrosis [AVN]).

Impingement of the posterosuperior retinacular branch of the medial femoral circumflex artery has been implicated in osteonecrosis associated with DDH treated in an abduction orthosis.

Pavlik harness treatment is contraindicated in teratologic hip dislocations.

In a patient with a narrow safe zone (<40 degrees), adductor tenotomy should be considered.

Excessive flexion may result in transient femoral nerve palsy.

“Pavlik disease”—if attempts to reduce a hip do not succeed in 3 weeks, harness should be discontinued to prevent erosion of the pelvis superior to the acetabulum and subsequent difficulty with closed reduction and casting.

The Pavlik harness is usually worn 23 hours a day for at least 6 weeks after a reduction has been achieved and then an additional 6 to 8 weeks part time (nights and during naps).

Risk factors for Pavlik harness failure:

Patient older than 7 weeks at initiation of treatment

Bilateral dislocations

Absence of Ortolani sign

Closed and open reduction

Closed reduction

Performed for patients for whom Pavlik treatment fails and for patients 6 to 18 months of age

Performed using general anesthesia; procedure includes a physical examination, arthrography to assess reduction (look for thorn sign on arthrogram, indicating normal labral position), and hip spica casting with the legs flexed to at least 90 degrees and in the stable zone of abduction

CT or MRI often performed to confirm that hip is well reduced, especially in questionable cases

Open reduction

Reserved for children 6 to 18 months old in whom closed reduction fails, who have an obstructive limbus, or who have an unstable safe zone

Initial treatment for children 18 months and older.

Anterior approach, especially for patients older than 12 months (less risk to medial femoral circumflex artery)

Capsulorrhaphy, adductor tenotomy, and femoral shortening can be performed to take tension off the reduction, along with an acetabular procedure if severe dysplasia is present

Obstacles to reduction: transverse acetabular ligament, pulvinar, infolded labrum, inferior capsular restriction, hypertrophied ligamentum teres, and psoas tendon

Medial open reduction can be performed in children up to 12 months of age.

Less blood loss

Directly addresses obstacles to reduction

Does not provide access for a capsulorrhaphy

More often associated with osteonecrosis

Surgical risks

Osteonecrosis—the major risk associated with both open and closed reductions; caused by direct vascular injury or impingement versus disruption of circulation from osteotomies

Damage to medial femoral circumflex can occur with medial approach to hip; close association to psoas, which undergoes a tenotomy because it is a block to reduction.

Failure of open reduction is difficult to treat surgically because of the high rates of complication after revision surgery (osteonecrosis in 50% and pain and stiffness in 33% according to one study)

Diagnosis after age 8 (younger in patients with bilateral DDH) may contraindicate reduction because the acetabulum has little chance to remodel, although reduction may be indicated in conjunction with salvage procedures.

20

Pelvic Osteotomy Overview

 

21

Pelvic Osteotomy Overview

22

Congenital Coxa Vara

Quantification of the extent of radiographic deformity of the proximal femur in developmental coxa vara. (A) The neck–shaft angle is the angle between the axis of the femoral shaft and the axis of the femoral neck. (B) The head–shaft angle is the angle between the axis of the femoral shaft and an imaginary perpendicular line to the base of the capital femoral epiphysis. (C) The Hilgenreiner–epiphyseal angle is the angle between the Hilgenreiner line and an imaginary line parallel to the capital femoral physis.

23

LCP disease

Lateral pillar classification of Legg-Calvé-Perthes disease. Researchers derived the definition of normal pillars by noting the lines of demarcation between the central sequestrum and the remainder of the epiphysis on the anteroposterior radiograph. In group A, normal height of lateral pillar is maintained. In group B, more than 50% of height of lateral pillar is maintained. In group B/C (borderline), lateral pillar is 50% or less in height, but (1) it is very narrow (2 to 3 mm wide), (2) it has very little ossification, or (3) it has depressions in comparison with the central pillar. In group C, less than 50% of height of lateral pillar is maintained.

24

LCP pillar classification prognosis

25

SCFE

Slipped capital femoral epiphysis. (A) Acute SCFE with no remodeling present. (B) Chronic SCFE showing adaptive changes including callus in the junction of the metaphysis and epiphysis. (C) Acute-on-chronic changes with both sequelae of chronic SCFE (callus) and acute worsening displacement of the epiphysis. (D) Drawings of correct pin placement for guiding of percutaneous in situ screw fixation of SCFE; note that the starting point is anterior on the femoral neck to account for the posteriorly displaced epiphysis.

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Causitive organisms for musculoskeletal infections

27

Risk factors for special infections

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joints with intra-articular metaphysis

29

Common Organisms in Septic Arthritis by age

30

Cause of Fibrous dysplasia

A germ line defect in the GSA protein.