Orthopedic and Surgical Susan D. Apkon, MD,a​ Ben Alman, MD,​b David J. Birnkrant, MD,​c Robert Fitch, MD,d​ Robert Lark, MD, MS,d​ WilliamManagement Mackenzie, MD,​e Norbert Weidner, of MD,f​ Michael the Sussman, Patient MDg With Duchenne Muscular Dystrophy abstract Orthopedic care is an important aspect of the overall management of patients with Duchenne muscular dystrophy (DMD). In addition to progressive muscle weakness and loss of function, patients may develop joint , scoliosis, and osteoporosis, causing fractures; all of these necessitate intervention by a multidisciplinary team including an orthopedic surgeon as well as rehabilitation specialists such as physio- and occupational therapists. The causes of these musculoskeletal complications are multifactorial and are related to primary effects on the muscles from the disease itself, secondary effects from weak muscles, and the related side effects of treatments, such as glucocorticoid use that affect strength. The musculoskeletal manifestations of DMD change over time as the disease progresses, and therefore, musculoskeletal management needs change throughout the life span of an individual with DMD. In this review, we target pediatricians, neurologists, orthopedic surgeons, rehabilitation physicians, anesthesiologists, and other individuals involved in the management of patients with DMD by providing specific recommendations to guide clinical practice related to orthopedic issues and surgical management in this setting.

Duchenne muscular dystrophy (DMD) has common and predictable musculoskeletal manifestations. In addition to progressive muscle weakness and loss of function, patients may develop joint contractures, scoliosis, and fractures due to osteoporosis. The causes of these musculoskeletal complications are multifactorial and are related to primary effects on the aDepartment of Rehabilitation Medicine, Seattle Children’s Hospital, Seattle, Washington; bDepartment of Orthopaedic Surgery, Duke University Medical Center, Durham, North Carolina; cMetroHealth Medical Center, Case Western Reserve University, Cleveland, Ohio; dDuke University Health System, Department of , Durham, North Carolina; eNemours/ Alfred I Dupont Hospital for Children, Wilmington, Delaware; fCincinnati Children’s Hospital Medical Center, Cincinnati, Ohio; and gShriner’s Hospital for Children, Portland, Oregon

The guidelines or recommendations in this article are not American Academy of Pediatrics policy and publication herein does not imply endorsement.

Dr Apkon served as chairperson for the Duchenne Muscular Dystrophy Care Considerations Orthopedic and Surgical Management Working Group, as convened by the Centers for Disease Control and Prevention, and drafted the initial manuscript; Drs Alman, Birnkrant, Fitch, Lark, Mackenzie, Weidner, and Sussman served on the Duchenne Muscular Dystrophy Care Considerations Orthopedic and Surgical Management Working Group, as convened by the Centers for Disease Control and Prevention, and contributed to the development of corresponding recommendations; and all authors reviewed and revised the manuscript, approved the final manuscript as submitted, and agree to be accountable for all aspects of the work. DOI: https://​doi.​org/​10.​1542/​peds.​2018-​0333J Accepted for publication Jul 26, 2018 Address correspondence to Susan D. Apkon, MD, Department of Rehabilitation Medicine, Seattle Children’s Hospital, 4800 Sand Point Way NE, Seattle, WA 98105. E-mail: [email protected] PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). Copyright © 2018 by the American Academy of Pediatrics FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose. FUNDING: Partially supported by the Cooperative Agreement, NU38OT000167, funded by the Centers for Disease Control and Prevention. POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.

Downloaded from www.aappublications.org/news by guest on September 26, 2021 SUPPLEMENT ARTICLE PEDIATRICS Volume 142, number s2, October 2018:e20180333J muscles from the disease itself, In the 2018 DMD Care treatment recommendations secondary effects from weak Considerations, sponsored by because the evidence for and muscles, and the adverse skeletal the Centers for Disease3 Control against this treatment is limited. effects of glucocorticoids on bone and Prevention,​ authors For a comprehensive discussion of strength. Because musculoskeletal seek to reinforce the 2010 stretching and the use of orthoses 2 “ manifestations of DMD change over recommendations related to using to prevent contractures, refer to the time, management needs change orthoses and surgical management Rehabilitation Management of the ” throughout the life span. of joint contractures and scoliosis. Patient With Duchenne Muscular

However, in this revision, authors Dystrophy article8 as part of this Musculoskeletal management emphasize some of the changing Contracturesupplement .Management: Surgical of the patient with DMD aims to patterns observed over the past Intervention preserve motor function for as long several years in each of these areas. as possible, promote bone health, Although the incidence of scoliosis minimize the occurrence of joint has decreased dramatically in those ∼ Many of the studies on surgical contractures, and maintain a straight treated with glucocorticoids (from interventions to prevent limb spine. A progressive approach to 90% to 30%), the long-term impact contractures included in the monitoring and interventions is of glucocorticoid treatment on the 2010 Care Considerations were recommended, with the overall goal late development of scoliosis is performed before the acceptance of of both nonsurgical and surgical unknown; long-term4,5​ monitoring is glucocorticoid treatment. Thus, their interventions being focused on thereby required. ‍ Additionally, results should be interpreted with maximizing function and ensuring in the 2018 Care Considerations, caution. None of the older studies comfort. authors place a greater emphasis on show that lower extremity surgeries the identification and management improve ambulatory ability or An interdisciplinary team should be of a child with both long bone quality of life. Surgical intervention involved in assessing and treating and vertebral fractures as well in the early ambulatory stage is not musculoskeletal complications as identification of risk factors recommended. Surgery may be more of DMD. This team may include a for osteoporosis. More details on beneficial for children in the middle neurologist, an orthopedic surgeon, these issues are provided in the ambulatory stage, although even in physical and occupational therapists, Bone Health and Osteoporosis this group, the frequency with which a rehabilitation physician, and a Management article that is part of surgery is recommended is low social worker. A pulmonologist and 6,7​ this supplement. ‍ For an overview compared with past trends. a cardiologist should be included in of the 2018 Orthopedic Care the team when surgical interventions The goal for lower extremity surgery Considerations, see Fig 1. are recommended. Endocrinologists in the ambulatory stage is to improve or osteoporosis specialists often are SPECIFIC CORE RECOMMENDATIONS ambulation. The consensus in the responsible for bone health issues, 2018 Care Considerations is that a topic beyond the scope of this surgery on the foot and Achilles article except for a discussion on tendon alone may be sufficient to the management of acute fractures. , spine, and fracture improve gait in select patients who Focused musculoskeletal evaluations management are important facets of have significant ankle contracture but should begin at the time of diagnosis orthopedic treatment in DMD. Each good strength in the quadriceps and and continue at least twice per year. is discussed below, and the issues to hip extensors. Surgery on the hips and Experienced clinicians can monitor consider in the surgical management knees is not recommended. To control the musculoskeletal system using of patients with DMD are reviewed an equinovarus foot deformity, the history and physical examination, inAmbulatory Fig 2. Stage surgeon should consider a tenotomy timed motor performance tests, Contracture Management: Stretching of the flexor halluces longus, flexor observational or video recordings and Orthoses digitorum longus, and posterior tibial of gait, and diagnostic tests, such as tendon if varus is present, in addition spine radiographs for monitoring to Achilles tendon lengthening. for scoliosis and vertebral fracture The current recommendation for a Posterior tibial tendon transfer also detection. Beyond the1, 2​2010 DMD proactive approach to contracture can be considered when a varus foot Care Considerations,​ ‍ no existing management should continue to position is observed. These surgeries guidelines specifically address be followed. However, families should only be considered in patients musculoskeletal management in should be involved with how with severe contractures and strong patients with DMD. this is prioritized among other ambulation. Patients should begin Downloaded from www.aappublications.org/news by guest on September 26, 2021 PEDIATRICS Volume 142, number s2, October 2018 S83 FIGURE 1 Considerations for orthopedic and surgical care of patients with DMD, by stage of disease. (Reprinted with permission from Birnkrant DJ, Bushby K, Bann CM, et al. Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopedic management. Lancet Neurol. 2018;17[4]:347–361.)

walking in short leg casts on the first it is still important to conduct a The expert panel also emphasizes or second postoperative day and may clinical assessment at least annually. routine monitoring of the spine require ankle-foot orthoses long-term During the ambulatory stage, for vertebral fractures, starting at ’ for ambulation after the casts are visual assessment, including use of diagnosis or no later than the time Spineremoved Management at 4 weeks. the Adams forward bend test, is of glucocorticoid initiation and appropriate. If a curve is observed, then at regular intervals thereafter,7 radiographic assessment should as discussed in detail elsewhere. follow. Radiography also is valuable if Vertebral fractures can be associated Although the onset of scoliosis in visual inspection alone is inadequate, with significant postural kyphosis. A the ambulatory patient is unusual, such as in a child with obesity. postural kyphosis puts a compressive Downloaded from www.aappublications.org/news by guest on September 26, 2021 S84 APKON et al health specialist to determine the appropriateness of pharmacological treatment with bisphosphonates. More details on proactive monitoring and treating vertebral fractures can “ be found in the 2018 Care Guidelines, Part 2 and in the Bone Health and Osteoporosis Management of the ” Patient With Duchenne Muscular

Dystrophy article3,7​ found in this supplementFracture Management. ‍

Families should receive instruction on removing obstacles in the home setting, such as throw rugs and toys, to prevent falls. Special care should be used outdoors when navigating uneven surfaces. Patients using a wheelchair in the community should be reminded to use seat belts at all times because falls out of the wheelchair are a common cause of lower limb fractures.

Osteoporosis and subsequent fractures have been associated with the use of glucocorticoids in many conditions, including DMD. Although vertebral fractures have been a consistent finding in studies in which researchers look at the long-term impact of glucocorticoids–

on patients with DMD, the effect9 11 on limb fractures is not as clear. ‍ ‍ In 1 large study, however, researchers examined the rate of long in a sample of 143 patients FIGURE 2 and found that the fracture rate in Surgical considerations for patients with DMD. *Guidance applies to older teen-aged and adult those treated with glucocorticoids patients. (Reprinted with permission from Birnkrant DJ, Bushby K, Bann CM, et al. Diagnosis and was 2.6 times greater than in the management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and patients who had not received orthopedic management. Lancet Neurol. 2018;17[4]:347–361.) 4 steroids. During the ambulatory stage, a lower limb fracture requires aggressive management to maintain ambulation. Early mobilization can ” force on the upper lumbar vertebrae, Management of the Patient With be enhanced if internal or external which is usually the apex of the Duchenne Muscular Dystrophy 7 fixation is used12 instead of casting kyphosis. A history of back pain article found in this supplement or splinting. Consultation with at any stage should also prompt are to provide pain relief and to cardiology and pulmonary specialists spine radiographs. The goals of prevent worsening of existing and is required before placing the child treating vertebral fractures with new vertebral fractures. Spinal under anesthesia. Postoperative “ a bisphosphonate as discussed in orthoses are not recommended. The referral to a physical therapist the Bone Health and Osteoporosis patient should be referred to a bone facilitates a focused approach to Downloaded from www.aappublications.org/news by guest on September 26, 2021 PEDIATRICS Volume 142, number s2, October 2018 S85 resuming ambulation quickly and procedure may help him position his until skeletal maturity is achieved. safely. feet on the wheelchair or make shoes Orthopedic surgeons should be ° easier to wear. Use of daytime ankle- involved if the patient has a spinal Fat syndrome has been foot orthoses is needed after surgery curve of 20 or more. Spinal orthosis described in patients with acute 15 on the foot and ankle to prevent is not recommended. To maintain lower extremity fracture or trauma. the contractures from recurring. an erect position to improve function, Presenting symptoms include altered Surgical interventions for hip and young men should use seating mental status, respiratory distress, 13 knee contractures are not generally systems with lateral supports in their and . Immediate “ recommended, except in rare cases wheelchairs. For more details, see medical attention is required because to alleviate symptoms, because the Rehabilitation Management of of the high risk of morbidity and ” significant correction of these the Patient With Duchenne Muscular mortality. The neuromuscular team established contractures is unlikely Dystrophy article that is part of this should ensure that the medical 8 to be achieved. supplement. and orthopedic team treating the Spine Management child is aware of this condition. In a 2013 updated Cochrane Educating family members about fat collaborative report, authors embolism syndrome and the need to The widespread use of concluded that in the absence of seek emergency medical attention glucocorticoids has dramatically any randomized controlled trials if symptoms are observed is also decreased the development of to evaluate the effectiveness of

Earlyimportant. Nonambulatory Stage scoliosis and need for5 subsequent scoliosis surgery in young men surgical intervention. There is Contracture Management: Stretching, with DMD, no evidence-based 16 Standing, and Orthoses still much to be learned about the recommendation could be made. impact of glucocorticoids on the Despite the lack of randomized spine, including the duration of controlled trials, authors of the 2018 glucocorticoid treatment needed to Care Considerations noted that in The focus on contracture reduce the risk of a progressive curve nonrandomized controlled studies, management changes in the patient and whether this treatment merely a positive impact of posterior spine who is no longer ambulatory, with 5 delays the onset. In recent studies fusion on function, sitting balance a greater emphasis on the hips – in which researchers followed young and tolerance, pain, and quality of and knees at later stages because 16 18 men into their twenties, it was shown life has been shown. ‍ ‍ Posterior severe contractures of the hips that glucocorticoids protect against spinal instrumentation and fusion and knees can make wheelchair ° the development of scoliosis well are recommended in those whose positioning challenging and may 5 past skeletal maturity. spinal curve is >20 , are prepubertal, prevent supported standing. An and are not on glucocorticoids occupational therapist should Every clinical examination because progression of the curve is develop a home program to focus on should include a spine inspection, expected. Although patients taking upper extremity stretching because regardless of glucocorticoid use. In glucocorticoids may still develop a the elbow, pronator, wrist, and finger nonambulatory patients, orthopedic scoliosis, it is reasonable to wait until flexors can develop contractures surgeons and other experienced 14 progression is documented. Patients over time. Improvements in wrist clinicians should be able to use visual who do not develop scoliosis may extension, grip strength, and hand inspection, although body habitus develop kyphosis of the spine due to function have been demonstrated frequently makes this challenging. vertebral fractures that may become in those wearing resting night hand Clinicians with less experience may severe and symptomatic. Although splints. For a further discussion of wish to obtain a spine radiograph “ the fractures may cause pain, there is contracture management, refer to when a patient with DMD first no risk of neurologic impairment. the Rehabilitation Management of ” becomes nonambulatory. Once the Patient With Duchenne Muscular radiographs show a curve, the When surgery is done to correct ° Dystrophy article8 as part of this skeletal maturity of the patients scoliosis, it is recommended that supplementContracture .Management: Surgical will dictate further surveillance. those with a pelvic obliquity of >15 Interventions Patients with immature skeletons also have stabilization and fusion into should have a radiograph every 6 the pelvis. This helps patients with months, whereas at least once a year seating and positioning. Fusion to During the nonambulatory stage, is sufficient for skeletally mature the L-5 is sufficient for patients who surgical intervention of the foot patients. Puberty is delayed in many do not have a severe pelvic obliquity. and ankle is generally performed glucocorticoid-treated patients, so No evidence indicates an advantage only if the patient requests it. This closer surveillance must continue of screws over wires or hooks to Downloaded from www.aappublications.org/news by guest on September 26, 2021 S86 APKON et al Late Nonambulatory Stage ’ achieve segmental instrumentation Contracture Management: Stretching Consultation with the patient s and Orthoses pulmonologist and cardiologist is of the spine, but screws provide ’ superior fixation in the lower lumbar critically important to ensure that spine and should be considered if the patient s lung and heart function instrumentation will stop in the Despite long-term stretching and are robust enough to withstand this lumbar spine distally. With surgical orthotic use, adolescents and adults surgical intervention. Correction and ’ intervention of the spine, the aim is with DMD frequently have fixed stabilization of scoliosis is essential to reduce the patient s pain, improve contractures at the hips and knees to maintain comfort and function. as well as equinovarus contractures his ability to sit comfortably, 17and Although it was once thought that prevent further progression. at the ankles. These rarely progress the rate of respiratory decline would Fracture Management to the point that they interfere with slow with a spinal fusion, authors sitting, although they can make have contradicted this understanding positioning in a wheelchair more in recent studies. In some studies, a challenging and make lying flat slowing progression of respiratory– Anticipatory guidance during difficult, which may interfere with decline has been shown; in 17,others,19​ 21 no routine neuromuscular clinic sleep. Contractures also occur in difference has been shown. ‍ ‍‍ visits should continue as an the upper extremities. Although Referral to a seating specialist is important part of a fracture no studies have been published on recommended because a supportive prevention program even for contractures in children treated seating system will provide greater the nonambulatory child. Seat with glucocorticoids, it appears that Fractureupright support Management in the wheelchair. belt use at all times when in the the severity of contractures has wheelchair should be emphasized. substantially decreased in these Fractures also occur in the setting children. For a further discussion of Prevention of fractures should of transfers. Specialized training by stretching and the use of orthoses continue throughout the life span, physical therapists is recommended to manage contractures in the late “ with ongoing education during when a child becomes fully nonambulatory stage, refer to the every neuromuscular clinic visit. As wheelchair dependent to ensure Rehabilitation Management of the ” in earlier stages of DMD, fracture that all caregivers understand Patient With Duchenne Muscular reduction, stabilization, and pain how to transfer a child to and from Dystrophy article as part of this 8 control are the treatment goals for an various surfaces safely. Specialized supplementContracture .Management: Surgical acute fracture in the adolescent and equipment, such as a Hoyer lift or Interventions adult during the late nonambulatory overhead lift, should be prescribed stage. Operative stabilization is to assist with transfers and necessary for proximal femur minimize the risk of a child being Unless factors such as pain, fractures, whereas a cast or splint dropped accidently. positioning, or skin integrity are may be sufficient for distal femoral concerns, surgical interventions metaphyseal fractures, but surgical Although the goal of managing a involving the upper or lower stabilization may allow the patients lower limb fracture in an ambulatory extremities are not recommended to resume sitting and return to patient is to bear weight as soon duringSpine Management this late stage of DMD. school more quickly. When surgical as possible, a more conservative intervention is recommended, approach is appropriate for special attention by cardiology and nonambulatory patients. An Examination of the spine at every pulmonology is required. Wheelchair unstable fracture may require visit is now recommended through modifications or rental wheelchairs , but for bone the entire life span given the lack may be required when a cast is healing and pain control, splinting of information regarding late placed. alone may be enough. All children progression of scoliosis in the SPECIAL CONSIDERATIONS FOR need appropriate pain management, setting of long-term glucocorticoid PATIENTS UNDERGOING SURGERY but special monitoring may be use and longer life expectancy. necessary for children who have When progression is a concern Cardiac Management pulmonary and cardiac compromise. in patients with known scoliosis, Fat embolism syndrome should be yearly anteroposterior sitting spinal suspected if altered mental status radiographs are warranted. For those A cardiologist should be consulted and respiratory distress occurs with a progressive curve, posterior before all surgical procedures. The after a lower limb fracture or spinal fusion is recommended patient with DMD is at particular severe trauma. during the late nonambulatory stage. risk of cardiac compromise during Downloaded from www.aappublications.org/news by guest on September 26, 2021 PEDIATRICS Volume 142, number s2, October 2018 S87 ’ major procedures, such as scoliosis noninvasive ventilation, should be loss is not recommended surgery, secondary to blood loss avoided unless the patient s baseline because such techniques provide and fluid shifts. An echocardiogram FVC exceeds the thresholds indicated hemodynamic risk in the setting of and electrocardiogram should be above and blood CO2 levels are cardiomyopathy often associated performed close to the time of monitored to detect hypoventilation with the patient with DMD. Cell- surgery. For minor procedures caused by muscle weakness or saver technology, along with the use in patients with normal cardiac sedation. Incentive spirometry is of aminocaproic acid or tranexamic function, a cardiac evaluation not indicated owing to the potential acid, can be considered to help is suggested if the most recent lack of efficacy in patients with manage intraoperative blood loss. investigation was more than 1 respiratory muscle weakness and the Postoperative anticoagulation – year previously. Anesthesiologists availability of preferred alternatives, with heparin or aspirin is not should be aware that cardiac such as mechanical insufflation appropriate. Use of compression decompensation during surgery exsufflation. Most patients will be stockings or sequential compression Respiratoryis a risk for patients Management with DMD. able to be extubated within the first for prevention of deep-vein 24 hours but should remain under thrombosis may be indicated. close observation after extubation FUTURE DIRECTIONS The guidelines on pre- and to ensure adequate respiratory perioperative respiratory function. The emerging use of experimental management from the 2010 Care After careful consideration of the – Considerations document remain risks and benefits, patients with treatments and the anticipation of valid. Respiratory interventions significant respiratory muscle more FDA approved medications, are intended to provide adequate weakness may be eligible for such as eteplirsen, are expected respiratory support during induction surgery, albeit with increased to change the course of DMD of, maintenance of, and recovery risk, if these patients are highly for the ambulatory child. It is from procedural sedation or skilled preoperatively in the use of unknown how these treatments general anesthesia. In particular, noninvasive ventilation and assisted will affect the musculoskeletal they are designed to reduce the Anestheticcough. Agents system. However, if started early, risk of postprocedure endotracheal they may help prolong walking extubation failure, postoperative and subsequent development of contractures and bone health, atelectasis, and . These When young men with DMD given greater opportunities for goals can be achieved by giving are exposed to inhalational weight bearing and the potential patients with significant respiratory anesthetics or when they are ability to avoid glucocorticoids. muscle weakness noninvasively administered succinylcholine, the Cautious recommendations for assisted ventilation and assisted risk of developing anesthesia- surgical interventions are required cough after surgery. This can associated is ’ in light of the relatively unknown be determined by preoperative increased. This is trajectory of the child s course. In pulmonary function test results. associated with hyperkalemia but is future work, researchers should Preoperative training in and frequently confused with malignant 22 focus on musculoskeletal issues, postoperative use of manual hyperthermia,​ which is a separate including contractures, fractures, and assisted cough techniques entity. and scoliosis in the child who are necessary for older teen- Because of this increased risk, receives disease-modifying aged and adult patients whose total intravenous anesthesia is medications. With improvements baseline peak cough flow is <270 strongly recommended. in life expectancy, a focus on the L per minute or whose baseline Depolarizing muscle relaxants, natural history of musculoskeletal maximum expiratory pressure such as succinylcholine, are issues in the adult with DMD is is <60 cm water. Preoperative absolutely contraindicated owing needed, with an emphasis on training in and postoperative use of Bloodto the riskLoss of fatal reactions. scoliosis and bone strength. noninvasive ventilation are strongly recommended for patients with a ABBREVIATIONS baseline forced vital capacity (FVC) of Another major concern when <50% predicted and necessary with a providing anesthesia for spinal FVC of <30% predicted. DMD: Duchenne muscular fusion surgery is significant blood dystrophy Extubation to supplemental oxygen loss. The use of hypotensive FVC: forced vital capacity alone, without concomitant use of anesthetics to minimize blood Downloaded from www.aappublications.org/news by guest on September 26, 2021 S88 APKON et al REFERENCES the patient with Duchenne muscular evidence? J Paediatr Child Health. dystrophy. Pediatrics. 2018;142(suppl 2): 2014;50(10):E3 E9 1. Bushby K, Finkel R, Birnkrant DJ, et al; – e20180333E​ DMD Care Considerations Working 16. Cheuk DK, Wong V, Wraige E, Baxter Group. Diagnosis and management of 8. Case L, Apkon S, Eagle M, et al. P, Cole A. Surgery for scoliosis in Duchenne muscular dystrophy, part 1: Rehabilitation management of the Duchenne muscular dystrophy. diagnosis, and pharmacological and patient with Duchenne muscular Cochrane Database Syst Rev. psychosocial management. Lancet dystrophy. Pediatrics. 2018;142(suppl 2): 2015;(10):CD005375 e20180333D​ Neurol. 2010;9(1):77–93 17. Suk KS, Lee BH, Lee HM, et al. 2. Bushby K, Finkel R, Birnkrant DJ, et al; 9. Houde S, Filiatrault M, Fournier A, et al. Functional outcomes in Duchenne DMD Care Considerations Working Deflazacort use in Duchenne muscular muscular dystrophy scoliosis: Group. Diagnosis and management of dystrophy: an 8-year follow-up. Pediatr comparison of the differences Duchenne muscular dystrophy, part Neurol. 2008;38(3):200–206 between surgical and nonsurgical 2: implementation of multidisciplinary 10. James KA, Cunniff C, Apkon SD, et al. treatment. J Bone Joint Surg Am. care. Lancet Neurol. 2010;9(2):177–189 Risk factors for first fractures among 2014;96(5):409–415 3. Birnkrant DJ, Bushby K, Bann CM, et al; males with Duchenne or Becker 18. Takaso M, Nakazawa T, Imura T, et al. DMD Care Considerations Working muscular dystrophy. J Pediatr Orthop. Surgical management of severe Group. Diagnosis and management of 2015;35(6):640–644 scoliosis with high risk pulmonary Duchenne muscular dystrophy, part 11. Biggar WD, Politano L, Harris VA, et al. dysfunction in Duchenne muscular 2: respiratory, cardiac, bone health, Deflazacort in Duchenne muscular dystrophy: patient function, quality and orthopaedic management. Lancet dystrophy: a comparison of two of life and satisfaction. Int Orthop. Neurol. 2018;17(4):347–361 different protocols. Neuromuscul 2010;34(5):695–702 4. King WM, Ruttencutter R, Nagaraja HN, Disord. 2004;14(8–9):476–482 19. Alexander WM, Smith M, Freeman BJ, et al. Orthopedic outcomes of long- 12. Huber H, André G, Rumeau F, Sutherland LM, Kennedy JD, Cundy PJ. term daily corticosteroid treatment Journeau P, Haumont T, Lascombes The effect of posterior spinal fusion in Duchenne muscular dystrophy. P. Flexible intramedullary nailing for on respiratory function in Duchenne Neurology. 2007;68(19):1607–1613 distal femoral fractures in patients muscular dystrophy. Eur Spine J. 2013;22(2):411 416 5. Lebel DE, Corston JA, McAdam LC, with myopathies. J Child Orthop. – Biggar WD, Alman BA. Glucocorticoid 2012;6(2):119–123 20. Rober to R, Fritz A, Hagar Y, et al. treatment for the prevention of 13. McAdam LC, Rastogi A, Macleod K, The natural history of cardiac and scoliosis in children with Duchenne Douglas Biggar W. Fat Embolism pulmonary function decline in patients muscular dystrophy: long-term Syndrome following minor with duchenne muscular dystrophy. follow-up. J Bone Joint Surg Am. trauma in Duchenne muscular Spine. 2011;36(15):E1009–E1017 2013;95(12):1057–1061 dystrophy. Neuromuscul Disord. 21. Chua K, Tan CY, Chen Z, et al. Long-term 6. Weber D, Hadjiyannakis S, McMillan H, 2012;22(12):1035–1039 follow-up of pulmonary function and Nortiz G, Ward L. Obesity and endocrine 14. Weichbrodt J, Eriksson BM, Kroksmark scoliosis in patients with Duchenne’s management of the patient with AK. Evaluation of hand orthoses in muscular dystrophy and spinal Duchenne muscular dystrophy. Duchenne muscular dystrophy. muscular atrophy. J Pediatr Orthop. Pediatrics. 2018;142(suppl 2): Disabil Rehabil. 2017:1–9 2016;36(1):63–69 e20180333F​ 15. Har vey A, Baker L, Williams K. Non- 22. Hayes J, Veyckemans F, Bissonnette B. 7. Ward L, Hadjiyannakis S, McMillan surgical prevention and management Duchenne muscular dystrophy: an old H, Noritz G, Weber D. Bone health of scoliosis for children with Duchenne anesthesia problem revisited. Paediatr and osteoporosis management of muscular dystrophy: what is the Anaesth. 2008;18(2):100–106

Downloaded from www.aappublications.org/news by guest on September 26, 2021 PEDIATRICS Volume 142, number s2, October 2018 S89 Orthopedic and Surgical Management of the Patient With Duchenne Muscular Dystrophy Susan D. Apkon, Ben Alman, David J. Birnkrant, Robert Fitch, Robert Lark, William Mackenzie, Norbert Weidner and Michael Sussman Pediatrics 2018;142;S82 DOI: 10.1542/peds.2018-0333J

Updated Information & including high resolution figures, can be found at: Services http://pediatrics.aappublications.org/content/142/Supplement_2/S82 References This article cites 22 articles, 3 of which you can access for free at: http://pediatrics.aappublications.org/content/142/Supplement_2/S82# BIBL Permissions & Licensing Information about reproducing this article in parts (figures, tables) or in its entirety can be found online at: http://www.aappublications.org/site/misc/Permissions.xhtml Reprints Information about ordering reprints can be found online: http://www.aappublications.org/site/misc/reprints.xhtml

Downloaded from www.aappublications.org/news by guest on September 26, 2021 Orthopedic and Surgical Management of the Patient With Duchenne Muscular Dystrophy Susan D. Apkon, Ben Alman, David J. Birnkrant, Robert Fitch, Robert Lark, William Mackenzie, Norbert Weidner and Michael Sussman Pediatrics 2018;142;S82 DOI: 10.1542/peds.2018-0333J

The online version of this article, along with updated information and services, is located on the World Wide Web at: http://pediatrics.aappublications.org/content/142/Supplement_2/S82

Pediatrics is the official journal of the American Academy of Pediatrics. A monthly publication, it has been published continuously since 1948. Pediatrics is owned, published, and trademarked by the American Academy of Pediatrics, 345 Park Avenue, Itasca, Illinois, 60143. Copyright © 2018 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 1073-0397.

Downloaded from www.aappublications.org/news by guest on September 26, 2021