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Injuries in Physically Disabled Children Tanja Kraus, MD1 Georg Singer, MD2 Helmut Wegmann, MD2 Sebastian Tschauner, MD3 Martin Svehlik, MD, PhD1 Gerhard Steinwender, MD1 Erich Sorantin, MD, PhD3 1 Division of Paediatric and Adolescent Orthopaedics, Medical
University Graz 2 Department of Paediatric and Adolescent Surgery, Medical University Graz 3 Division of Paediatric and Adolescent Radiology, Medical University Graz, Graz, Austria
Address for correspondence Tanja Kraus, MD, Division of Paediatric and Adolescent Orthopaedics, Medical University Graz, Auenbruggerplatz 34, 8036 Graz, Austria (e-mail: [email protected]).
Abstract
Keywords
► ► ► ► ►
growing skeleton disabled osteoporosis metabolism injury
Disability is a condition or function that is judged to be significantly impairing relative to the usual standard of an individual or group. The spectrum of musculoskeletal disabilities in children is immense and varied. Musculoskeletal disabilities are congenital or acquired; they affect a child partially or generally and can occur as a permanent or transient disability. Although injuries still represent a major concern for children and adolescents worldwide, studies focusing on injuries in physically disabled children are lacking. To detect musculoskeletal injuries, radiographs are frequently required. In disabled children the radiographic findings can detect the skeletal injury but also can present special radiographic findings of the underlying disease. This review offers an overview of different musculoskeletal disabilities and their related injuries as well as characteristic findings on radiographs.
Injuries represent a major concern for children and adolescents worldwide. Injury rates vary considerably in their level of severity by age and sex,1 and most studies reporting injuries are limited either to a single type or to a specific cause of injury.2 Gallagher et al report the overall incidence of injured children and adolescents as 14.5% in a study among 87,000 patients in Massachusetts over a 1-year period.3 Although estimations suggest that 10% of children in the world experience some kind of disability, the number of injuries in disabled children has not been thoroughly researched.4,5 Disability is a condition or function judged to be significantly impairing relative to the usual standard of an individual or group. Disability includes a physical, sensory, cognitive, intellectual impairment, mental illness, and also various types of chronic diseases. Frequently there can be found combinations of these without clear distinctions. Musculoskeletal disabilities are congenital or acquired; they affect a child partially or generally and can occur as
Issue Theme Sports Injuries and Imaging in Children; Guest Editor, Erich Sorantin, MD, PhD
permanent or transient disability. ►Fig. 1 presents a possible classification with several examples. The injury risk in disabled children is influenced by decreased mobility and increased precautions. Children with disabilities tend to be weaker and more susceptible to early fatigue than their peers6–9 because they have a higher metabolic, cardiorespiratory, and mechanical expenditure of mobility. Moreover, for a given environmental exposure the risk is amplified due to their compromised adaptability to external hazards.10 It might be speculated that the limited activity of physically disabled children leads to a lower rate of injuries. However, it has to be taken into account that the use of medical devices (e.g., crutches, orthosis) often required in these patients might increase their risk of sustaining an injury. Younger children ( 4 per 1,000 live births.27–29 Cerebral palsy is a term for a group of permanent nonprogressive movement disorders that cause physical disability24 by a permanent and irreversible insult of the developing brain that manifests predominantly in motor system defects.30 The results are abnormal muscle tone with spasms and spasticity, abnormal reflexes, and abnormal motor development and coordination. Spasticity and co-spasticity across the joints as well as an impaired central motor drive hinder the normal function of the locomotor system. Joint and bone deformities due to muscle contractures are very common. Soft tissue findings consist of decreased muscle mass, decreased muscle volume, and loss of power while stiffness and the content of collagen in the muscles is increased.31 Energy consumption for activities of daily living is significantly higher in these patients compared with their peers.32 CP has different impacts on the patients’ lives. Some patients are able to walk and manage to cope with their everyday challenge alone. Others are confined to bed and depend on constant help due to serious mental and physical impairments. The level of impairment influences their physical activity.
Fig. 4 (a) Humeral fracture in a 13-year-old tetraspastic girl with cerebral palsy (CP). The child is physically and mentally handicapped and wheelchair bound. Due to fixed flexion contractures in the elbow joint and fixed abduction contracture in the shoulder joint, the upper extremities are particularly vulnerable to injuries. The child fractured her right humeral bone when it got stuck at the doorframe while the wheelchair was pushed by her mother. Note the low bone density that is already visible on the conventional radiograph. (b) The fracture was anatomically reduced. (c) Due to the patient’s underlying muscle contractures and high spasticity, postoperative dislocation and movement of one of the intramedullary nails occurred and had to be removed. As frequently seen in osteoporotic patients with CP, delayed fracture healing was observed. (d) Radiograph taken after 10 weeks of injury presents the delayed healing. Note that taking an X-ray in standard anteroposterior and lateral view in these children can be challenging due to a lack of cooperation.
Cerebral Palsy and Injuries The more a child is affected by CP, the lower his or her physical activity. The level of impairment corresponds to the physical activity. Low physical activity leads to low bone mineral density that with in turn increases the risk of fragility.33 CP patients with moderate to severe motor impairment (gross motor function classification system III, IV, and V) have a significantly higher risk of fractures.34 The management of fractures in CP children can be more complicated than in neurologically normal developing children. The spastic muscle tone in CP can cause an increase in shortening or malunion at the fracture side.35,36 Moreover, healing may be delayed in some cases (►Fig. 4).
Congenital Musculoskeletal Disabilities The exact incidence of congenital musculoskeletal abnormalities in children is difficult to determine not only because some of the abnormalities are not detectable at birth and therefore not reported at that time, but also because of the indefinite borderline between minor abnormalities and normal variations as these disabilities vary greatly in extent and severity.37 The incidence of congenital abnormalities detectSeminars in Musculoskeletal Radiology
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able at birth is 3%.37 If they are severe, they catch the attention of the observer immediately.
Congenital Partial Musculoskeletal Disabilities Adaption of pediatric-disabled patients to activities of daily living is influenced by the age when the impairment appears. Patients with congenital disabilities are used to their partial impairment from birth and they normally cope quite well. As noted earlier, when using medical aids the risk of injury is increased due to these devices. Special injury patterns as well as an increased injury risk are described for some congenital partial disabilities (e.g., spina bifida).
Spina Bifida Spina bifida is a developmental congenital disorder caused by the incomplete closure of the embryonic neural tube. Vertebrae overlying the spinal cord may be not fully formed, can remain unfused, or stay completely open. There is a risk of protrusion of the spinal cord in large openings of the bones.
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Spina Bifida and Injury The fracture risk in lower extremities is increased in children who are affected more severely from spina bifida and bound to a wheelchair due to immobilization and osteoporosis.38 Also the numbers of epiphysiolyses is increased, frequently affecting the distal femur or the proximal tibia.38 Fractures in children with spina bifida heal quickly. Excessive callus formation is seen in 30%38(►Fig. 5).
Dysmelia The spectrum of congenital deficiencies of the musculoskeletal system in children is immense and includes a wide variety of defects ranging from clinically minor anomalies to seriously disabling conditions.37 Dysmelia is defined as a group of malformations in which there is hypoplasia and partial or total aplasia of the tubular bones of the extremities, ranging from isolated peripheral hypoplasia to complete loss of the extremity. The condition is commonly known as limb deficiency or limb reduction defect. The prevalence rate for all types of limb deficiency is 0.69 per 1,000.39 ►Fig. 6 shows the radiograph of a newborn with right side congenital femoral deficiency. Characteristically the
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right femur is markedly shorter. The hip is flexed and held in abduction and external rotation. This deficiency can also imply lack of integrity, stability, and mobility of the hip and knee joints.
Dysmelia and Injury Injuries in these patients may not differ from their peers. Patients with congenital limb deficiencies adapt well to activities of daily living because they are used to their deficit from birth on. If the lower extremity is affected, medical devices are part of daily life beginning in early childhood. Fracture and soft tissue healing is not described as different.
Congenital General Musculoskeletal Disabilities The injury risk in children with congenital general musculoskeletal diseases is influenced by decreased mobility and increased precautions. These children tend to be weaker and more susceptible to early fatigue than their peers.6–9 Their higher metabolic, cardiorespiratory, and mechanical expenditure of mobility cause early fatigue and decreased performance in everyday activities. They are amplified to external hazards due to their compromised adaptability.10 It might therefore be speculated that their limited activity leads to a lower rate of injuries.
Short Stature Patients and Injuries Many conditions can cause short stature. Each case of short stature is special because of its effects on the bones and joints. The effects of gene abnormalities result in
Fig. 5 (a, b) Distal lower leg fracture in a 7-year-old boy with spina bifida due to a fall out of his wheelchair. (c, d) A huge callus formation is already seen 14 days after immobilization in the plaster of Paris. (e, f) At cast removal the callus formation became even more massive; unfortunately, the fracture healed in valgus deformation. Due to the permanent immobilization of this patient, the bones show signs of osteoporosis in these X-rays.
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According to its severity, spina bifida is categorized as spina bifida occulta or spina bifida aperta with or without myelomeningocele. The most common location of spina bifida is the lumbar and sacral area. The myelomeningocele is the most significant and common form that leads to disability in most affected individuals.
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Kraus et al. mal segments of the extremities are usually more severely involved than the distal portions. Thus the humerus and femur are disproportionately shorter compared with the radius and tibia.
Achondroplasia and Injury When bone fractures occur, healing does not seem to differ from their normal peers. However, radiographs show characteristic bone changes. An example is shown in ►Fig. 7.
Multiple Epiphyseal Dysplasia Multiple epiphyseal dysplasia, or Fairbanks disease, is a rare genetic disorder affecting the growing end of the long bones leading to short stature with short limbs. On X-rays small and irregular ossification centers can be found that are most apparent in the hips and knees. There are small capital femoral epiphyses that are hypoplastic. The acetabular roofs are poorly formed. Knees frequently show metaphyseal widening. In early adulthood, signs of osteoarthritis are already apparent (►Fig. 7).
Osteogenesis Imperfecta Fig. 6 (a) Radiograph of a 4-week-old girl with a congenital femoral deficiency (CFD). CFD shows a wide variability in the severity of femoral deficiency and deformity. The deficiency implies a lack of integrity, stability, and mobility of the hip and knee joints. Note the significantly shorter femur on the right side in this young girl. The hip is held in flexion, abduction, and external rotation. Frequently the femoral head shows retroversion. The proximal femoral growth plate is “distalized.” Its chondrocytes are immature. (b) Radiograph of a 12-year-old boy with a fibular hemimelia. For technical reasons the radiograph is stitched leading to two stripes (marked by asterisk). The patient has a leg length discrepancy (in the X-ray the right leg is supported with 2 cm) of the right shank leading to axial deviations and malrotation of the whole leg. The fibula is markedly thinner. Note the subsequent changes of the adjacent joints.
various deformities and specific degenerative changes. These are typically reflected in limb deformities and deformities of the axial skeleton. Most infants with short stature have to deal with thoracolumbar kyphosis, and their knees joints show signs of extreme laxity. Toddlers frequently have a variety of bowleg deformities. Abnormal morphology of the affected joints is a reason why there is further tension load and an increase of weightbearing forces on the particular body part. These individuals are additionally prone to secondary ligamentous laxity and associated problems.
Achondroplasia A common reason for short stature is achondroplasia, accounting for 70% of all cases. Rhizomelic short limbs and normal trunk height are characteristics of achondroplasia. The disease is the result of diminished proliferation of cartilage in the growth plate due to decreased enchondral bone formation. The radiographic hallmark of achondroplasia is symmetrical shortening of all long bones. The proxiSeminars in Musculoskeletal Radiology
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Osteogenesis imperfecta (OI) is the collective term for a heterogeneous group of connective tissue syndromes with an autosomal dominant inheritance. OI affects 1 in 5,000 to 10,000 children. According to Sillence, OI is subdivided into seven types based on radiologic and genetic characteristics showing clinically different severity.40 Mutation in genes responsible for the production of procollagen, the osteoblast produces an abnormal matrix that does not respond adequately to mechanical loadings.41 Major clinical characteristics include brittle bones with osteopenia that are prone to fracture throughout life, variable degrees of short stature, and progressive skeletal deformities. Radiographs in OI show thin and undertubulated gracile bones that can be of normal length or shortened, thickened, and deformed due to multiple fractures. Pathologic fractures tend to be oriented transversely within long bones. Shaft fractures and metaphyseal abnormalities with florid or exuberant callus formation can mimic an osteosarcoma. In more sever types, rib fractures are common and require exclusion before making a diagnosis of nonaccidental injury in a neonate.
Osteogenesis Imperfecta and Injuries Lower limb fractures are most frequent in patients with OI requiring long periods of immobilization that cause further bone loss42 (►Fig. 8). Bulloch et al reported an incidence of < 8% in their investigations of rib fractures in infancy.43 Ganesh et al reported three infants with intracranial hemorrhage with OI type I. The patients developed retinal hemorrhages and subdural hematomas after minor trauma.44 Tokoro reported an infant who had an acute subdural hematoma at birth and developed a progressive chronic subdural hematoma with local protrusion of the overlying skull.45 OI should also be taken into account as an important albeit rare differential diagnosis when dealing with an abused child.
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Fig. 7 (a) Pelvic view radiograph of a 6-week-old girl with achondroplasia. Characteristic findings are the flat acetabular roofs, small and greater sciatic notches, and short femurs with widened femoral metaphysis. (b, c) Intraoperative image intensifier pictures of the hip in a 16-year-old patient with epiphyseal dysplasia (b, anteroposterior view, c, lateral view). Images were taken at the beginning of an operation for deformity correction now showing, in contrast to the standard radiograph, the deformity precisely. Note the epiphyseal deformations of the proximal femur. The stature of the boy is short due to growth disturbances, and there are complex axial deformations and malrotations of his extremities. Early osteoarthritis is inevitable.
Bisphosphonates are currently indicated for the symptomatic treatment of children with OI.39,46 They decrease bone loss and slow bone turnover.41,47 Bone mineral density and physical activity increase while the fracture rate and chronic
pain decrease. Research also indicates an increased number of accidents in children with OI. Studies reporting exact numbers of injured OI children treated with bisphosphonates are lacking.
Fig. 8 (a, b) Radiographs of a 5-year-old girl with osteogenesis imperfecta (Sillence type IV). The transverse sclerotic lines represent individual treatment cycles with bisphosphonates and demonstrate continued linear bone growth. Intramedullary nails to protect the bone, which is prone to fracture, splint the long bones. (c, d) Distal femoral fracture in an adolescent with osteogenesis imperfecta (Sillence type III). The radiographs show a thin and undertubulated gracile femoral bone that is shortened, thickened, and deformed. There is a transverse supracondylar fracture with a florid callus formation. Note also the proximal femur that is severely deformed due to a previous fracture.
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Conclusion
18 Brenner RA, Taneja GS, Schroeder TJ, Trumble AC, Moyer PM, Louis
There are no special injury patterns due to the underlying impairment in children and adolescents. Injuries in impaired children depend on the underlying disease and its severity. Activity of the affected child is of crucial importance. A lower level of activity leads to osteoporosis and increases the risk of fractures. Radiographs are usually sufficient to detect both the bone injury and the underlying disease. Further imaging such as computed tomography and MRI are usually not needed, and its indication does not differ from that in typically developing peers.
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eastern Ohio trauma study: I. Magnitude of the problem. Am J Public Health 1983;73(7):746–751 Loder RT, Schultz W, Sabatino M. Fractures From trampolines: results from a national database, 2002 to 2011. J Pediatr Orthop 2014; March 28 (Epub ahead of print) Gallagher SS, Finison K, Guyer B, Goodenough S. The incidence of injuries among 87,000 Massachusetts children and adolescents: results of the 1980-81 Statewide Childhood Injury Prevention Program Surveillance System. Am J Public Health 1984;74(12): 1340–1347 World Health Organization. World Report on Disability. Geneva, Switzerland: WHO; 2011 UNICEF ECD Unit. Programming experiences in early child development. Available at: http://www.unicef.org/earlychildhood/files/programming%20experiences%20in%20early%20childhood.pdf Dykens EM, Rosner BA, Butterbaugh G. Exercise and sports in children and adolescents with developmental disabilities. Positive physical and psychosocial effects. Child Adolesc Psychiatr Clin N Am 1998;7(4):757–771, viii Campbell J, Ball J. Energetics of walking in cerebral palsy. Orthop Clin North Am 1978;9(2):374–377 Dichter JR, Levine SJ, Shelhamer JH. Approach to the immunocompromised host with pulmonary symptoms. Hematol Oncol Clin North Am 1993;7(4):887–912 Fernhall B, Unnithan VB. Physical activity, metabolic issues, and assessment. Phys Med Rehabil Clin N Am 2002;13(4):925–947 Rivara FP. Developmental and behavioral issues in childhood injury prevention. J Dev Behav Pediatr 1995;16(5):362–370 Xiang H, Leff M, Stallones L. Non-fatal injuries among adults with activity limitations and participation restrictions. Inj Prev 2005; 11(3):157–162 Sinclair SA, Xiang H. Injuries among US children with different types of disabilities. Am J Public Health 2008;98(8):1510–1516 Petridou E, Kedikoglou S, Andrie E, et al. Injuries among disabled children: a study from Greece. Inj Prev 2003;9(3):226–230 Sherrard J, Tonge BJ, Ozanne-Smith J. Injury risk in young people with intellectual disability. J Intellect Disabil Res 2002; 46(Pt 1):6–16 Rowe R, Maughan B, Goodman R. Childhood psychiatric disorder and unintentional injury: findings from a national cohort study. J Pediatr Psychol 2004;29(2):119–130 Soubhi H, Raina P, Kohen D. Neighborhood, family, and child predictors of childhood injury in Canada. Am J Health Behav 2004;28(5):397–409 Hawkins ER, Brice JH, Overby BA. Welcome to the world: findings from an emergency medical services pediatric injury prevention program. Pediatr Emerg Care 2007;23(11):790–795
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GM. Unintentional injuries among youth with developmental disabilities in the United States, 2006-2007. Int J Inj Contr Saf Promot 2013;20(3):259–265 Barnard AM, Nelson NG, Xiang H, McKenzie LB. Pediatric mobility aid-related injuries treated in US emergency departments from 1991 to 2008. Pediatrics 2010;125(6):1200–1207 Loder RT, Schwartz EM, Hensinger RN. Behavioral characteristics of children with Legg-Calvé-Perthes disease. J Pediatr Orthop 1993;13(5):598–601 Hailer YD, Montgomery S, Ekbom A, Nilsson O, Bahmanyar S. LeggCalvé-Perthes disease and the risk of injuries requiring hospitalization: a register study involving 2579 patients. Acta Orthop 2012; 83(6):572–576 Hailer YD, Haag AC, Nilsson O. Legg-Calvé-perthes disease: quality of life, physical activity, and behavior pattern. J Pediatr Orthop 2014;34(5):514–521 Misra M, Pacaud D, Petryk A, Collett-Solberg PF, Kappy M; Drug and Therapeutics Committee of the Lawson Wilkins Pediatric Endocrine Society. Vitamin D deficiency in children and its management: review of current knowledge and recommendations. Pediatrics 2008;122(2):398–417 Rosenbaum P, Paneth N, Leviton A, et al. A report: the definition and classification of cerebral palsy April 2006. Dev Med Child Neurol Suppl 2007;109:8–14 Chapman T, Sugar N, Done S, Marasigan J, Wambold N, Feldman K. Fractures in infants and toddlers with rickets. Pediatr Radiol 2010; 40(7):1184–1189 Agarwal A, Gulati D, Rath S, Walia M. Rickets: a cause of delayed walking in toddlers. Indian J Pediatr 2009;76(3): 269–272 Paneth N, Hong T, Korzeniewski S. The descriptive epidemiology of cerebral palsy. Clin Perinatol 2006;33(2):251–267 Surveillance of Cerebral Palsy in Europe. Prevalence and characteristics of children with cerebral palsy in Europe. Dev Med Child Neurol 2002;44(9):633–640 Winter S, Autry A, Boyle C, Yeargin-Allsopp M. Trends in the prevalence of cerebral palsy in a population-based study. Pediatrics 2002;110(6):1220–1225 Ca A. Neurodevelopmental Disabilities in Infancy and Childhood. Baltimore, MD: Paul H. Brookes; 2008 Barrett RS, Lichtwark GA. Gross muscle morphology and structure in spastic cerebral palsy: a systematic review. Dev Med Child Neurol 2010;52(9):794–804 Duffy CM, Hill AE, Cosgrove AP, Corry IS, Graham HK. Energy consumption in children with spina bifida and cerebral palsy: a comparative study. Dev Med Child Neurol 1996;38(3): 238–243 Fehlings D, Switzer L, Agarwal P, et al. Informing evidence-based clinical practice guidelines for children with cerebral palsy at risk of osteoporosis: a systematic review. Dev Med Child Neurol 2012; 54(2):106–116 Stevenson RD, Conaway M, Barrington JW, Cuthill SL, Worley G, Henderson RC. Fracture rate in children with cerebral palsy. Pediatr Rehabil 2006;9(4):396–403 McIvor WC, Samilson RL. Fractures in patients with cerebral palsy. J Bone Joint Surg Am 1966;48(5):858–866 Pritchett JW. Treated and untreated unstable hips in severe cerebral palsy. Dev Med Child Neurol 1990;32(1):3–6 Salter RB. Textbook of Disorders of the Musculoskeletal System. 3rd ed. Baltimore, MD: Lippincott Williams & Wilkins; 1998 Parsch K. Origin and treatment of fractures in spina bifida. Eur J Pediatr Surg 1991;1(5):298–305 Chen H. Dysmelia. In: Atlas of Genetic Diagnosis and Counseling. Totowa, NJ: Humana Press; 2006:312–322 Van Dijk FS, Sillence DO. Osteogenesis imperfecta: Clinical diagnosis, nomenclature and severity assessment. Am J Med Genet Part A 2014;164(6)A:1470–1481
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Injuries in Physically Disabled Children In: Glorieux F, Pettifor J, Juppner J, eds. Pediatric Bone: Biology and Diseases. San Diego, CA: Elsevier Science; 2003 42 Pizones J, Plotkin H, Parra-Garcia JI, et al. Bone healing in children with osteogenesis imperfecta treated with bisphosphonates. J Pediatr Orthop 2005;25(3):332–335 43 Bulloch B, Schubert CJ, Brophy PD, Johnson N, Reed MH, Shapiro RA. Cause and clinical characteristics of rib fractures in infants. Pediatrics 2000;105(4):E48 44 Ganesh A, Jenny C, Geyer J, Shouldice M, Levin AV. Retinal hemorrhages in type I osteogenesis imperfecta after minor trauma. Ophthalmology 2004;111(7):1428–1431
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K, Nakajima F, Yamataki A. Infantile chronic subdural hematoma with local protrusion of the skull in a case of osteogenesis imperfecta. Neurosurgery 1988;22(3): 595–598 46 Aström E, Söderhäll S. Beneficial effect of bisphosphonate during five years of treatment of severe osteogenesis imperfecta. Acta Paediatr 1998;87(1):64–68 47 Rauch F, Plotkin H, Travers R, Zeitlin L, Glorieux FH. Osteogenesis imperfecta types I, III, and IV: effect of pamidronate therapy on bone and mineral metabolism. J Clin Endocrinol Metab 2003;88(3): 986–992
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41 Plotkin H, Primorac D, Rowe D. Osteogenesis imperfecta.
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Injuries in Physically Disabled Children Tanja Kraus, MD1 Georg Singer, MD2 Helmut Wegmann, MD2 Sebastian Tschauner, MD3 Martin Svehlik, MD, PhD1 Gerhard Steinwender, MD1 Erich Sorantin, MD, PhD3 1 Division of Paediatric and Adolescent Orthopaedics, Medical
University Graz 2 Department of Paediatric and Adolescent Surgery, Medical University Graz 3 Division of Paediatric and Adolescent Radiology, Medical University Graz, Graz, Austria
Address for correspondence Tanja Kraus, MD, Division of Paediatric and Adolescent Orthopaedics, Medical University Graz, Auenbruggerplatz 34, 8036 Graz, Austria (e-mail: [email protected]).
Abstract
Keywords
► ► ► ► ►
growing skeleton disabled osteoporosis metabolism injury
Disability is a condition or function that is judged to be significantly impairing relative to the usual standard of an individual or group. The spectrum of musculoskeletal disabilities in children is immense and varied. Musculoskeletal disabilities are congenital or acquired; they affect a child partially or generally and can occur as a permanent or transient disability. Although injuries still represent a major concern for children and adolescents worldwide, studies focusing on injuries in physically disabled children are lacking. To detect musculoskeletal injuries, radiographs are frequently required. In disabled children the radiographic findings can detect the skeletal injury but also can present special radiographic findings of the underlying disease. This review offers an overview of different musculoskeletal disabilities and their related injuries as well as characteristic findings on radiographs.
Injuries represent a major concern for children and adolescents worldwide. Injury rates vary considerably in their level of severity by age and sex,1 and most studies reporting injuries are limited either to a single type or to a specific cause of injury.2 Gallagher et al report the overall incidence of injured children and adolescents as 14.5% in a study among 87,000 patients in Massachusetts over a 1-year period.3 Although estimations suggest that 10% of children in the world experience some kind of disability, the number of injuries in disabled children has not been thoroughly researched.4,5 Disability is a condition or function judged to be significantly impairing relative to the usual standard of an individual or group. Disability includes a physical, sensory, cognitive, intellectual impairment, mental illness, and also various types of chronic diseases. Frequently there can be found combinations of these without clear distinctions. Musculoskeletal disabilities are congenital or acquired; they affect a child partially or generally and can occur as
Issue Theme Sports Injuries and Imaging in Children; Guest Editor, Erich Sorantin, MD, PhD
permanent or transient disability. ►Fig. 1 presents a possible classification with several examples. The injury risk in disabled children is influenced by decreased mobility and increased precautions. Children with disabilities tend to be weaker and more susceptible to early fatigue than their peers6–9 because they have a higher metabolic, cardiorespiratory, and mechanical expenditure of mobility. Moreover, for a given environmental exposure the risk is amplified due to their compromised adaptability to external hazards.10 It might be speculated that the limited activity of physically disabled children leads to a lower rate of injuries. However, it has to be taken into account that the use of medical devices (e.g., crutches, orthosis) often required in these patients might increase their risk of sustaining an injury. Younger children ( 4 per 1,000 live births.27–29 Cerebral palsy is a term for a group of permanent nonprogressive movement disorders that cause physical disability24 by a permanent and irreversible insult of the developing brain that manifests predominantly in motor system defects.30 The results are abnormal muscle tone with spasms and spasticity, abnormal reflexes, and abnormal motor development and coordination. Spasticity and co-spasticity across the joints as well as an impaired central motor drive hinder the normal function of the locomotor system. Joint and bone deformities due to muscle contractures are very common. Soft tissue findings consist of decreased muscle mass, decreased muscle volume, and loss of power while stiffness and the content of collagen in the muscles is increased.31 Energy consumption for activities of daily living is significantly higher in these patients compared with their peers.32 CP has different impacts on the patients’ lives. Some patients are able to walk and manage to cope with their everyday challenge alone. Others are confined to bed and depend on constant help due to serious mental and physical impairments. The level of impairment influences their physical activity.
Fig. 4 (a) Humeral fracture in a 13-year-old tetraspastic girl with cerebral palsy (CP). The child is physically and mentally handicapped and wheelchair bound. Due to fixed flexion contractures in the elbow joint and fixed abduction contracture in the shoulder joint, the upper extremities are particularly vulnerable to injuries. The child fractured her right humeral bone when it got stuck at the doorframe while the wheelchair was pushed by her mother. Note the low bone density that is already visible on the conventional radiograph. (b) The fracture was anatomically reduced. (c) Due to the patient’s underlying muscle contractures and high spasticity, postoperative dislocation and movement of one of the intramedullary nails occurred and had to be removed. As frequently seen in osteoporotic patients with CP, delayed fracture healing was observed. (d) Radiograph taken after 10 weeks of injury presents the delayed healing. Note that taking an X-ray in standard anteroposterior and lateral view in these children can be challenging due to a lack of cooperation.
Cerebral Palsy and Injuries The more a child is affected by CP, the lower his or her physical activity. The level of impairment corresponds to the physical activity. Low physical activity leads to low bone mineral density that with in turn increases the risk of fragility.33 CP patients with moderate to severe motor impairment (gross motor function classification system III, IV, and V) have a significantly higher risk of fractures.34 The management of fractures in CP children can be more complicated than in neurologically normal developing children. The spastic muscle tone in CP can cause an increase in shortening or malunion at the fracture side.35,36 Moreover, healing may be delayed in some cases (►Fig. 4).
Congenital Musculoskeletal Disabilities The exact incidence of congenital musculoskeletal abnormalities in children is difficult to determine not only because some of the abnormalities are not detectable at birth and therefore not reported at that time, but also because of the indefinite borderline between minor abnormalities and normal variations as these disabilities vary greatly in extent and severity.37 The incidence of congenital abnormalities detectSeminars in Musculoskeletal Radiology
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able at birth is 3%.37 If they are severe, they catch the attention of the observer immediately.
Congenital Partial Musculoskeletal Disabilities Adaption of pediatric-disabled patients to activities of daily living is influenced by the age when the impairment appears. Patients with congenital disabilities are used to their partial impairment from birth and they normally cope quite well. As noted earlier, when using medical aids the risk of injury is increased due to these devices. Special injury patterns as well as an increased injury risk are described for some congenital partial disabilities (e.g., spina bifida).
Spina Bifida Spina bifida is a developmental congenital disorder caused by the incomplete closure of the embryonic neural tube. Vertebrae overlying the spinal cord may be not fully formed, can remain unfused, or stay completely open. There is a risk of protrusion of the spinal cord in large openings of the bones.
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Spina Bifida and Injury The fracture risk in lower extremities is increased in children who are affected more severely from spina bifida and bound to a wheelchair due to immobilization and osteoporosis.38 Also the numbers of epiphysiolyses is increased, frequently affecting the distal femur or the proximal tibia.38 Fractures in children with spina bifida heal quickly. Excessive callus formation is seen in 30%38(►Fig. 5).
Dysmelia The spectrum of congenital deficiencies of the musculoskeletal system in children is immense and includes a wide variety of defects ranging from clinically minor anomalies to seriously disabling conditions.37 Dysmelia is defined as a group of malformations in which there is hypoplasia and partial or total aplasia of the tubular bones of the extremities, ranging from isolated peripheral hypoplasia to complete loss of the extremity. The condition is commonly known as limb deficiency or limb reduction defect. The prevalence rate for all types of limb deficiency is 0.69 per 1,000.39 ►Fig. 6 shows the radiograph of a newborn with right side congenital femoral deficiency. Characteristically the
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right femur is markedly shorter. The hip is flexed and held in abduction and external rotation. This deficiency can also imply lack of integrity, stability, and mobility of the hip and knee joints.
Dysmelia and Injury Injuries in these patients may not differ from their peers. Patients with congenital limb deficiencies adapt well to activities of daily living because they are used to their deficit from birth on. If the lower extremity is affected, medical devices are part of daily life beginning in early childhood. Fracture and soft tissue healing is not described as different.
Congenital General Musculoskeletal Disabilities The injury risk in children with congenital general musculoskeletal diseases is influenced by decreased mobility and increased precautions. These children tend to be weaker and more susceptible to early fatigue than their peers.6–9 Their higher metabolic, cardiorespiratory, and mechanical expenditure of mobility cause early fatigue and decreased performance in everyday activities. They are amplified to external hazards due to their compromised adaptability.10 It might therefore be speculated that their limited activity leads to a lower rate of injuries.
Short Stature Patients and Injuries Many conditions can cause short stature. Each case of short stature is special because of its effects on the bones and joints. The effects of gene abnormalities result in
Fig. 5 (a, b) Distal lower leg fracture in a 7-year-old boy with spina bifida due to a fall out of his wheelchair. (c, d) A huge callus formation is already seen 14 days after immobilization in the plaster of Paris. (e, f) At cast removal the callus formation became even more massive; unfortunately, the fracture healed in valgus deformation. Due to the permanent immobilization of this patient, the bones show signs of osteoporosis in these X-rays.
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According to its severity, spina bifida is categorized as spina bifida occulta or spina bifida aperta with or without myelomeningocele. The most common location of spina bifida is the lumbar and sacral area. The myelomeningocele is the most significant and common form that leads to disability in most affected individuals.
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Achondroplasia and Injury When bone fractures occur, healing does not seem to differ from their normal peers. However, radiographs show characteristic bone changes. An example is shown in ►Fig. 7.
Multiple Epiphyseal Dysplasia Multiple epiphyseal dysplasia, or Fairbanks disease, is a rare genetic disorder affecting the growing end of the long bones leading to short stature with short limbs. On X-rays small and irregular ossification centers can be found that are most apparent in the hips and knees. There are small capital femoral epiphyses that are hypoplastic. The acetabular roofs are poorly formed. Knees frequently show metaphyseal widening. In early adulthood, signs of osteoarthritis are already apparent (►Fig. 7).
Osteogenesis Imperfecta Fig. 6 (a) Radiograph of a 4-week-old girl with a congenital femoral deficiency (CFD). CFD shows a wide variability in the severity of femoral deficiency and deformity. The deficiency implies a lack of integrity, stability, and mobility of the hip and knee joints. Note the significantly shorter femur on the right side in this young girl. The hip is held in flexion, abduction, and external rotation. Frequently the femoral head shows retroversion. The proximal femoral growth plate is “distalized.” Its chondrocytes are immature. (b) Radiograph of a 12-year-old boy with a fibular hemimelia. For technical reasons the radiograph is stitched leading to two stripes (marked by asterisk). The patient has a leg length discrepancy (in the X-ray the right leg is supported with 2 cm) of the right shank leading to axial deviations and malrotation of the whole leg. The fibula is markedly thinner. Note the subsequent changes of the adjacent joints.
various deformities and specific degenerative changes. These are typically reflected in limb deformities and deformities of the axial skeleton. Most infants with short stature have to deal with thoracolumbar kyphosis, and their knees joints show signs of extreme laxity. Toddlers frequently have a variety of bowleg deformities. Abnormal morphology of the affected joints is a reason why there is further tension load and an increase of weightbearing forces on the particular body part. These individuals are additionally prone to secondary ligamentous laxity and associated problems.
Achondroplasia A common reason for short stature is achondroplasia, accounting for 70% of all cases. Rhizomelic short limbs and normal trunk height are characteristics of achondroplasia. The disease is the result of diminished proliferation of cartilage in the growth plate due to decreased enchondral bone formation. The radiographic hallmark of achondroplasia is symmetrical shortening of all long bones. The proxiSeminars in Musculoskeletal Radiology
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Osteogenesis imperfecta (OI) is the collective term for a heterogeneous group of connective tissue syndromes with an autosomal dominant inheritance. OI affects 1 in 5,000 to 10,000 children. According to Sillence, OI is subdivided into seven types based on radiologic and genetic characteristics showing clinically different severity.40 Mutation in genes responsible for the production of procollagen, the osteoblast produces an abnormal matrix that does not respond adequately to mechanical loadings.41 Major clinical characteristics include brittle bones with osteopenia that are prone to fracture throughout life, variable degrees of short stature, and progressive skeletal deformities. Radiographs in OI show thin and undertubulated gracile bones that can be of normal length or shortened, thickened, and deformed due to multiple fractures. Pathologic fractures tend to be oriented transversely within long bones. Shaft fractures and metaphyseal abnormalities with florid or exuberant callus formation can mimic an osteosarcoma. In more sever types, rib fractures are common and require exclusion before making a diagnosis of nonaccidental injury in a neonate.
Osteogenesis Imperfecta and Injuries Lower limb fractures are most frequent in patients with OI requiring long periods of immobilization that cause further bone loss42 (►Fig. 8). Bulloch et al reported an incidence of < 8% in their investigations of rib fractures in infancy.43 Ganesh et al reported three infants with intracranial hemorrhage with OI type I. The patients developed retinal hemorrhages and subdural hematomas after minor trauma.44 Tokoro reported an infant who had an acute subdural hematoma at birth and developed a progressive chronic subdural hematoma with local protrusion of the overlying skull.45 OI should also be taken into account as an important albeit rare differential diagnosis when dealing with an abused child.
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Fig. 7 (a) Pelvic view radiograph of a 6-week-old girl with achondroplasia. Characteristic findings are the flat acetabular roofs, small and greater sciatic notches, and short femurs with widened femoral metaphysis. (b, c) Intraoperative image intensifier pictures of the hip in a 16-year-old patient with epiphyseal dysplasia (b, anteroposterior view, c, lateral view). Images were taken at the beginning of an operation for deformity correction now showing, in contrast to the standard radiograph, the deformity precisely. Note the epiphyseal deformations of the proximal femur. The stature of the boy is short due to growth disturbances, and there are complex axial deformations and malrotations of his extremities. Early osteoarthritis is inevitable.
Bisphosphonates are currently indicated for the symptomatic treatment of children with OI.39,46 They decrease bone loss and slow bone turnover.41,47 Bone mineral density and physical activity increase while the fracture rate and chronic
pain decrease. Research also indicates an increased number of accidents in children with OI. Studies reporting exact numbers of injured OI children treated with bisphosphonates are lacking.
Fig. 8 (a, b) Radiographs of a 5-year-old girl with osteogenesis imperfecta (Sillence type IV). The transverse sclerotic lines represent individual treatment cycles with bisphosphonates and demonstrate continued linear bone growth. Intramedullary nails to protect the bone, which is prone to fracture, splint the long bones. (c, d) Distal femoral fracture in an adolescent with osteogenesis imperfecta (Sillence type III). The radiographs show a thin and undertubulated gracile femoral bone that is shortened, thickened, and deformed. There is a transverse supracondylar fracture with a florid callus formation. Note also the proximal femur that is severely deformed due to a previous fracture.
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18 Brenner RA, Taneja GS, Schroeder TJ, Trumble AC, Moyer PM, Louis
There are no special injury patterns due to the underlying impairment in children and adolescents. Injuries in impaired children depend on the underlying disease and its severity. Activity of the affected child is of crucial importance. A lower level of activity leads to osteoporosis and increases the risk of fractures. Radiographs are usually sufficient to detect both the bone injury and the underlying disease. Further imaging such as computed tomography and MRI are usually not needed, and its indication does not differ from that in typically developing peers.
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41 Plotkin H, Primorac D, Rowe D. Osteogenesis imperfecta.
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