alterations of musculoskeletal function in children chapter 43 mosby items and derived items ©...

Alterations of Musculoskeletal Alterations of Musculoskeletal Function in ChildrenFunction in Children

Chapter 43Chapter 43

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc.Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc.

22Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc.Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc.

Musculoskeletal AlterationsMusculoskeletal Alterations OverviewOverview

CongenitalCongenital• ClubfootClubfoot

HereditaryHereditary• Muscular dystrophyMuscular dystrophy

AcquiredAcquired• Legg-Calvé-Perthes Legg-Calvé-Perthes

May be acute, chronic, or terminalMay be acute, chronic, or terminal


Bone FormationBone Formation Bone formation begins in two phases at about Bone formation begins in two phases at about

the eighth week of gestationthe eighth week of gestation Delivery of bone cell precursors to sites of bone Delivery of bone cell precursors to sites of bone

formationformation Aggregation of the bone cell precursors at primary Aggregation of the bone cell precursors at primary

centers of ossificationcenters of ossification


Bone FormationBone Formation Intramembranous formationIntramembranous formation

On or within the mesenchymeOn or within the mesenchyme Endochondral formationEndochondral formation

Cartilage anlageCartilage anlage PerichondriumPerichondrium Periosteal collarPeriosteal collar Secondary centers of ossificationSecondary centers of ossification


Bone FormationBone Formation


Bone GrowthBone Growth Until adult stature achieved, bone growth Until adult stature achieved, bone growth

occurs at the epiphyseal plate through occurs at the epiphyseal plate through endochondral ossificationendochondral ossification

Epiphyseal closureEpiphyseal closure Unites the metaphysis and the epiphysisUnites the metaphysis and the epiphysis Occurs earlier in females than males because of Occurs earlier in females than males because of

earlier puberty in femalesearlier puberty in females


Bone GrowthBone Growth Factors affecting bone growthFactors affecting bone growth

Growth hormone (secreted by pituitary)Growth hormone (secreted by pituitary) NutritionNutrition General healthGeneral health Many growth factors and regulators (fibroblast Many growth factors and regulators (fibroblast

growth factor)growth factor)


Skeletal DevelopmentSkeletal Development

In the newborn, the entire spine is concave In the newborn, the entire spine is concave anteriorly (kyphosed)anteriorly (kyphosed)

In the first 3 months of life, the cervical spine In the first 3 months of life, the cervical spine begins to arch (lordotic)begins to arch (lordotic)

Curve of lumbar spine develops with sittingCurve of lumbar spine develops with sitting Compared to adult, a newborn has a large head, Compared to adult, a newborn has a large head,

long spine, and short extremitieslong spine, and short extremities


Skeletal DevelopmentSkeletal Development Genu varum (peaks by 2½ years)Genu varum (peaks by 2½ years)

Occurs in all newborns due to intrauterine stressOccurs in all newborns due to intrauterine stress BowlegBowleg

Genu valgum (peaks by 5-6 years)Genu valgum (peaks by 5-6 years) Knock-kneesKnock-knees

Persistence past peak times is pathologicPersistence past peak times is pathologic


Muscle DevelopmentMuscle Development Between birth and maturity, muscle nuclei in Between birth and maturity, muscle nuclei in

the body increase 14 times in boys and 10 the body increase 14 times in boys and 10 times in girlstimes in girls

The composition and size of muscles vary The composition and size of muscles vary with agewith age


Congenital DefectsCongenital Defects SyndactylySyndactyly

Webbing of the fingersWebbing of the fingers Fusion of the soft tissues of the fingersFusion of the soft tissues of the fingers True syndactyly also includes fusion of the bones and True syndactyly also includes fusion of the bones and

nailsnails Vestigial tabsVestigial tabs

Extra digitExtra digit


Congenital DefectsCongenital Defects Anomalies on the medial or radial aspect of Anomalies on the medial or radial aspect of

the arm often associated with abnormalities the arm often associated with abnormalities of blood, heart, or kidneys. of blood, heart, or kidneys.

Lateral or ulnar-sided defects are less often Lateral or ulnar-sided defects are less often associated with systemic anomalies and are associated with systemic anomalies and are far more rarefar more rare


SyndactylySyndactyly


Congenital DefectsCongenital Defects Developmental dysplasia of the hipDevelopmental dysplasia of the hip

Formerly: congenital dislocation of the hipFormerly: congenital dislocation of the hip Abnormality of the proximal femur, acetabulum, or Abnormality of the proximal femur, acetabulum, or

bothboth Risk factorsRisk factors

• Female, metatarsus adductus, torticollis, Female, metatarsus adductus, torticollis, oligohydramnios, first pregnancy, and breech oligohydramnios, first pregnancy, and breech presentationpresentation

The hip can present as subluxated, dislocatable, The hip can present as subluxated, dislocatable, or dislocatedor dislocated


Congenital DefectsCongenital Defects Developmental dysplasia of the hipDevelopmental dysplasia of the hip

ManifestationsManifestations• Asymmetry of gluteal or thigh foldsAsymmetry of gluteal or thigh folds

• Limb length discrepancyLimb length discrepancy

• Limitation of hip abductionLimitation of hip abduction

• Positive Ortolani signPositive Ortolani sign

• Positive Barlow testPositive Barlow test

• Positive Trendelenburg gaitPositive Trendelenburg gait

• PainPain


Developmental Dysplasia of the HipDevelopmental Dysplasia of the Hip


Congenital DefectsCongenital Defects Deformities of the footDeformities of the foot

Metatarsus adductus (forefoot adduction)Metatarsus adductus (forefoot adduction)• Mild, moderate, or severe (degree of deformity and Mild, moderate, or severe (degree of deformity and

flexibility)flexibility)

Equinovarus deformity (clubfoot)Equinovarus deformity (clubfoot)• Positional equinovarusPositional equinovarus

• Idiopathic congenital equinovarusIdiopathic congenital equinovarus

• Tetratologic equinovarusTetratologic equinovarus

• Pes planus (flat foot)Pes planus (flat foot)


Congenital DefectsCongenital Defects TreatmentTreatment

BracesBraces Sequential castsSequential casts SurgerySurgery


Osteogenesis ImperfectaOsteogenesis Imperfecta

““Brittle bone disease”Brittle bone disease” Defect in collagen productionDefect in collagen production

Bone and vessel collagenBone and vessel collagen Sillence classificationSillence classification Results in osteoporosis, bowed and deformed Results in osteoporosis, bowed and deformed

limbs, short stature, spine curvature, and bluish limbs, short stature, spine curvature, and bluish sclerasclera

Can be evident before birth (in utero fractures)Can be evident before birth (in utero fractures)



SevereSevere Child may be stillborn or die soon after birth; Child may be stillborn or die soon after birth;

intrauterine fracturesintrauterine fractures MildMild

May not be diagnosed until child begins to walkMay not be diagnosed until child begins to walk May be mistaken for child abuseMay be mistaken for child abuse


RicketsRickets

Disorder causing mineralization failure, “soft” Disorder causing mineralization failure, “soft” bones, and skeletal deformitybones, and skeletal deformity

CausesCauses Insufficient vitamin DInsufficient vitamin D Insensitivity to vitamin DInsensitivity to vitamin D Renal wasting of vitamin DRenal wasting of vitamin D Inability to absorb calcium or vitamin D in the gutInability to absorb calcium or vitamin D in the gut


RicketsRickets


ScoliosisScoliosis Rotational curvature of the spineRotational curvature of the spine

NonstructuralNonstructural• Curvature is from a cause other than the spineCurvature is from a cause other than the spine

Structural Structural • Curvature associated with vertebral rotationCurvature associated with vertebral rotation

• Skeletal abnormalities, neuromuscular disease, trauma, Skeletal abnormalities, neuromuscular disease, trauma, extraspinal contractures, bone infections of the extraspinal contractures, bone infections of the vertebrae, metabolic bone disorders, joint disease, and vertebrae, metabolic bone disorders, joint disease, and tumorstumors


ScoliosisScoliosis


OsteomyelitisOsteomyelitis

Often associated with septic arthritis because Often associated with septic arthritis because infant’s bone has blood vessels that perforate infant’s bone has blood vessels that perforate the growth platethe growth plate

In children frequently begins as a blood abscess In children frequently begins as a blood abscess in the metaphysis of the bonein the metaphysis of the bone

In adolescents and adults may involve the In adolescents and adults may involve the vertebraevertebrae Back pain for several weeks may be only complaintBack pain for several weeks may be only complaint This age group is less often affected than younger This age group is less often affected than younger

populationspopulations


OsteomyelitisOsteomyelitis Much less common after the epiphyseal Much less common after the epiphyseal

plates are closed, except in the vertebral plates are closed, except in the vertebral bodybody Infection may develop in any part of a bone, and Infection may develop in any part of a bone, and

abscesses spread slowlyabscesses spread slowly Destruction of the cortex in a localized area may Destruction of the cortex in a localized area may

result in a pathologic fracture result in a pathologic fracture


OsteomyelitisOsteomyelitis Infection spreads under the periosteum and Infection spreads under the periosteum and

along the bone shaft or into the bone marrow along the bone shaft or into the bone marrow SequestraSequestra

• Sections of dead bone from periosteal separationSections of dead bone from periosteal separation

InvolucrumInvolucrum• Periosteal new bonePeriosteal new bone


OsteomyelitisOsteomyelitis


Juvenile Rheumatoid Arthritis Juvenile Rheumatoid Arthritis (JRA)(JRA)

Childhood form of rheumatoid arthritisChildhood form of rheumatoid arthritis The basic pathophysiology of JRA is the The basic pathophysiology of JRA is the

same as the adult formsame as the adult form One difference is the mode of onsetOne difference is the mode of onset

Arthritis in fewer than five jointsArthritis in fewer than five joints Arthritis in more than five jointsArthritis in more than five joints Systemic diseaseSystemic disease


Juvenile Rheumatoid Arthritis Juvenile Rheumatoid Arthritis (JRA)(JRA)

Differences in JRA and adult RADifferences in JRA and adult RA Large joints are affectedLarge joints are affected Subluxation, ankylosis of the cervical spineSubluxation, ankylosis of the cervical spine Joint pain is not as severeJoint pain is not as severe Positive antinuclear antibody testPositive antinuclear antibody test Chronic uveitisChronic uveitis Low detection of rheumatoid factorLow detection of rheumatoid factor Limited subcutaneous rheumatoid nodulesLimited subcutaneous rheumatoid nodules

• Common in heart, lungs, eyes, and other organsCommon in heart, lungs, eyes, and other organs


OsteochondrosisOsteochondrosis Avascular diseases of the boneAvascular diseases of the bone Legg-CalvLegg-Calvéé-Perthes disease-Perthes disease

Interrupted blood supply to the femoral headInterrupted blood supply to the femoral head Self-limiting diseaseSelf-limiting disease Deformation due to ischemia is permanentDeformation due to ischemia is permanent

Osgood-Schlatter diseaseOsgood-Schlatter disease Tendinitis of the anterior patellar tendon and Tendinitis of the anterior patellar tendon and

osteochondrosis of the tubercle of the tibiaosteochondrosis of the tubercle of the tibia


Legg-CalvLegg-Calvéé-Perthes Disease-Perthes Disease


Cerebral PalsyCerebral Palsy A static disorder of muscle tone and balance A static disorder of muscle tone and balance

caused by an ischemic insult to the braincaused by an ischemic insult to the brain Perinatal disorderPerinatal disorder Disease patternsDisease patterns

Hemiplegia, diplegia, quadriplegia Hemiplegia, diplegia, quadriplegia


Muscular DystrophiesMuscular Dystrophies Group of disorders that cause degeneration Group of disorders that cause degeneration

of skeletal muscle fibersof skeletal muscle fibers The muscular dystrophies cause progressive, The muscular dystrophies cause progressive,

symmetric weakness, and wasting of skeletal symmetric weakness, and wasting of skeletal muscle groupsmuscle groups


Duchenne Muscular DystrophyDuchenne Muscular Dystrophy Most common muscular dystrophyMost common muscular dystrophy X-linked recessive inheritanceX-linked recessive inheritance

Deletion of segment of DNA or single gene defect Deletion of segment of DNA or single gene defect on short arm of the X chromosomeon short arm of the X chromosome

Duchenne muscular dystrophy geneDuchenne muscular dystrophy gene Encodes for the dystrophin proteinEncodes for the dystrophin protein Dystrophin mediates the anchorage of the actin Dystrophin mediates the anchorage of the actin

cytoskeleton of the skeletal muscle fiber to the cytoskeleton of the skeletal muscle fiber to the basement membranebasement membrane


Duchenne Muscular DystrophyDuchenne Muscular Dystrophy Manifestations appear by 3 years of ageManifestations appear by 3 years of age

Slow motor developmentSlow motor development Progressive weaknessProgressive weakness Muscle wastingMuscle wasting Sitting and standing are delayedSitting and standing are delayed The child is clumsy, falls frequently, and has The child is clumsy, falls frequently, and has

difficulty climbing stairsdifficulty climbing stairs


Muscular DystrophyMuscular Dystrophy


Muscular DystrophiesMuscular Dystrophies Becker muscular dystrophyBecker muscular dystrophy Fascioscapulohumeral muscular dystrophyFascioscapulohumeral muscular dystrophy Scapuloperoneal muscular dystrophyScapuloperoneal muscular dystrophy Limb girdle muscular dystrophyLimb girdle muscular dystrophy


Bone and Muscle TumorsBone and Muscle Tumors Nonossifying fibromaNonossifying fibroma Simple bone cystsSimple bone cysts Aneurysmal bone cystsAneurysmal bone cysts Osteoid osteomaOsteoid osteoma Fibrous dysplasiaFibrous dysplasia OsteosarcomaOsteosarcoma Ewing sarcomaEwing sarcoma RhabdomyosarcomaRhabdomyosarcoma


Nonaccidental TraumaNonaccidental Trauma ““Corner” metaphyseal fracturesCorner” metaphyseal fractures

Long bone fractures caused by a twisting forceLong bone fractures caused by a twisting force Transverse tibial fractures are the most commonTransverse tibial fractures are the most common Associated with child abuse, but osteogenesis Associated with child abuse, but osteogenesis

imperfecta must be ruled outimperfecta must be ruled out


Nonaccidental TraumaNonaccidental Trauma

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