Alterations of Musculoskeletal Function in Children Chapter 43 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc.

2 Musculoskeletal Alterations  Overview  Congenital Clubfoot Clubfoot  Hereditary Muscular dystrophy Muscular dystrophy  Acquired Legg-Calvé-Perthes Legg-Calvé-Perthes  May be acute, chronic, or terminal

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 3 Bone Formation  Bone formation begins in two phases at about the eighth week of gestation  Delivery of bone cell precursors to sites of bone formation  Aggregation of the bone cell precursors at primary centers of ossification

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 4 Bone Formation  Intramembranous formation  On or within the mesenchyme  Endochondral formation  Cartilage anlage  Perichondrium  Periosteal collar  Secondary centers of ossification

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 5 Bone Formation

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 6 Bone Growth  Until adult stature achieved, bone growth occurs at the epiphyseal plate through endochondral ossification  Epiphyseal closure  Unites the metaphysis and the epiphysis  Occurs earlier in females than males because of earlier puberty in females

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 7 Bone Growth  Factors affecting bone growth  Growth hormone (secreted by pituitary)  Nutrition  General health  Many growth factors and regulators (fibroblast growth factor)

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 8 Skeletal Development  In the newborn, the entire spine is concave anteriorly (kyphosed)  In the first 3 months of life, the cervical spine begins to arch (lordotic)  Curve of lumbar spine develops with sitting  Compared to adult, a newborn has a large head, long spine, and short extremities

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 9 Skeletal Development  Genu varum (peaks by 2½ years)  Occurs in all newborns due to intrauterine stress  Bowleg  Genu valgum (peaks by 5-6 years)  Knock-knees  Persistence past peak times is pathologic

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 10 Muscle Development  Between birth and maturity, muscle nuclei in the body increase 14 times in boys and 10 times in girls  The composition and size of muscles vary with age

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 11 Congenital Defects  Syndactyly  Webbing of the fingers  Fusion of the soft tissues of the fingers  True syndactyly also includes fusion of the bones and nails  Vestigial tabs  Extra digit

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 12 Congenital Defects  Anomalies on the medial or radial aspect of the arm often associated with abnormalities of blood, heart, or kidneys.  Lateral or ulnar-sided defects are less often associated with systemic anomalies and are far more rare

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 13 Syndactyly

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 14 Congenital Defects  Developmental dysplasia of the hip  Formerly: congenital dislocation of the hip  Abnormality of the proximal femur, acetabulum, or both  Risk factors Female, metatarsus adductus, torticollis, oligohydramnios, first pregnancy, and breech presentation Female, metatarsus adductus, torticollis, oligohydramnios, first pregnancy, and breech presentation  The hip can present as subluxated, dislocatable, or dislocated

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 15 Congenital Defects  Developmental dysplasia of the hip  Manifestations Asymmetry of gluteal or thigh folds Asymmetry of gluteal or thigh folds Limb length discrepancy Limb length discrepancy Limitation of hip abduction Limitation of hip abduction Positive Ortolani sign Positive Ortolani sign Positive Barlow test Positive Barlow test Positive Trendelenburg gait Positive Trendelenburg gait Pain Pain

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 16 Developmental Dysplasia of the Hip

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 17 Congenital Defects  Deformities of the foot  Metatarsus adductus (forefoot adduction) Mild, moderate, or severe (degree of deformity and flexibility) Mild, moderate, or severe (degree of deformity and flexibility)  Equinovarus deformity (clubfoot) Positional equinovarus Positional equinovarus Idiopathic congenital equinovarus Idiopathic congenital equinovarus Tetratologic equinovarus Tetratologic equinovarus Pes planus (flat foot) Pes planus (flat foot)

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 18 Congenital Defects  Treatment  Braces  Sequential casts  Surgery

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 19 Osteogenesis Imperfecta  “Brittle bone disease”  Defect in collagen production  Bone and vessel collagen  Sillence classification  Results in osteoporosis, bowed and deformed limbs, short stature, spine curvature, and bluish sclera  Can be evident before birth (in utero fractures)

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 20 Osteogenesis Imperfecta  Severe  Child may be stillborn or die soon after birth; intrauterine fractures  Mild  May not be diagnosed until child begins to walk  May be mistaken for child abuse

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 21 Osteogenesis Imperfecta

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 22 Rickets  Disorder causing mineralization failure, “soft” bones, and skeletal deformity  Causes  Insufficient vitamin D  Insensitivity to vitamin D  Renal wasting of vitamin D  Inability to absorb calcium or vitamin D in the gut

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 23 Rickets

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 24 Scoliosis  Rotational curvature of the spine  Nonstructural Curvature is from a cause other than the spine Curvature is from a cause other than the spine  Structural Curvature associated with vertebral rotation Curvature associated with vertebral rotation Skeletal abnormalities, neuromuscular disease, trauma, extraspinal contractures, bone infections of the vertebrae, metabolic bone disorders, joint disease, and tumors Skeletal abnormalities, neuromuscular disease, trauma, extraspinal contractures, bone infections of the vertebrae, metabolic bone disorders, joint disease, and tumors

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 25 Scoliosis

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 26 Osteomyelitis  Often associated with septic arthritis because infant’s bone has blood vessels that perforate the growth plate  In children frequently begins as a blood abscess in the metaphysis of the bone  In adolescents and adults may involve the vertebrae  Back pain for several weeks may be only complaint  This age group is less often affected than younger populations

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 27 Osteomyelitis  Much less common after the epiphyseal plates are closed, except in the vertebral body  Infection may develop in any part of a bone, and abscesses spread slowly  Destruction of the cortex in a localized area may result in a pathologic fracture

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 28 Osteomyelitis  Infection spreads under the periosteum and along the bone shaft or into the bone marrow  Sequestra Sections of dead bone from periosteal separation Sections of dead bone from periosteal separation  Involucrum Periosteal new bone Periosteal new bone

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 29 Osteomyelitis

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 30 Juvenile Rheumatoid Arthritis (JRA)  Childhood form of rheumatoid arthritis  The basic pathophysiology of JRA is the same as the adult form  One difference is the mode of onset  Arthritis in fewer than five joints  Arthritis in more than five joints  Systemic disease

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 31 Juvenile Rheumatoid Arthritis (JRA)  Differences in JRA and adult RA  Large joints are affected  Subluxation, ankylosis of the cervical spine  Joint pain is not as severe  Positive antinuclear antibody test  Chronic uveitis  Low detection of rheumatoid factor  Limited subcutaneous rheumatoid nodules Common in heart, lungs, eyes, and other organs Common in heart, lungs, eyes, and other organs

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 32 Osteochondrosis  Avascular diseases of the bone  Legg-Calvé-Perthes disease  Interrupted blood supply to the femoral head  Self-limiting disease  Deformation due to ischemia is permanent  Osgood-Schlatter disease  Tendinitis of the anterior patellar tendon and osteochondrosis of the tubercle of the tibia

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 33 Legg-Calvé-Perthes Disease

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 34 Cerebral Palsy  A static disorder of muscle tone and balance caused by an ischemic insult to the brain  Perinatal disorder  Disease patterns  Hemiplegia, diplegia, quadriplegia

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 35 Muscular Dystrophies  Group of disorders that cause degeneration of skeletal muscle fibers  The muscular dystrophies cause progressive, symmetric weakness, and wasting of skeletal muscle groups

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 36 Duchenne Muscular Dystrophy  Most common muscular dystrophy  X-linked recessive inheritance  Deletion of segment of DNA or single gene defect on short arm of the X chromosome  Duchenne muscular dystrophy gene  Encodes for the dystrophin protein  Dystrophin mediates the anchorage of the actin cytoskeleton of the skeletal muscle fiber to the basement membrane

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 37 Duchenne Muscular Dystrophy  Manifestations appear by 3 years of age  Slow motor development  Progressive weakness  Muscle wasting  Sitting and standing are delayed  The child is clumsy, falls frequently, and has difficulty climbing stairs

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 38 Muscular Dystrophy

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 39 Muscular Dystrophies  Becker muscular dystrophy  Fascioscapulohumeral muscular dystrophy  Scapuloperoneal muscular dystrophy  Limb girdle muscular dystrophy

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 40 Bone and Muscle Tumors  Nonossifying fibroma  Simple bone cysts  Aneurysmal bone cysts  Osteoid osteoma  Fibrous dysplasia  Osteosarcoma  Ewing sarcoma  Rhabdomyosarcoma

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 41 Nonaccidental Trauma  “Corner” metaphyseal fractures  Long bone fractures caused by a twisting force  Transverse tibial fractures are the most common  Associated with child abuse, but osteogenesis imperfecta must be ruled out

Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 42 Nonaccidental Trauma