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Many neuromuscular disorders are dealt with in some detail throughout this text, and this chapter focuses on those that commonly require orthopedic attention as part of their management. Other medical and surgical issues related to the care of patients are dealt with in other chapters and are cross-referenced when appropriate.

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This congenital condition is discussed in Chapter 549. The incidence of myelomeningocele has decreased over the last 2 decades, in part due to the recommendation for prenatal folate supplementation preceding and in early pregnancy. The level of spina bifida is defined by the most caudal functioning nerve root—that is, patients with sacral level spina bifida (eg, S1 level) have functioning hip flexors, quadriceps, hamstrings, and anterior tibialis musculature, but have absent innervation of the foot intrinsic musculature and absent bowel and bladder innervation. Patients with thoracic spina bifida have no lower extremity motor or sensory function.

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Treatment of the infant with myelomeningocele begins with closure of the open sac and shunting of hydrocephalus in the early neonatal time period. Orthopedic treatment begins when the child is ready developmentally to begin weight-bearing. Patients with thoracic-level spina bifida can weight-bear in standing frames but may require foot surgery to correct deformities such as talipes equinovarus, or clubfoot. Additionally, young children on occasion require hip abduction contracture release to allow them to be positioned into braces.

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Upper-lumbar-level patients most often can ambulate as young children with use of hip–knee–ankle–foot orthoses (HKAFOs), but often require clubfoot release. Typically, these patients become nonambulatory in adolescence when the energy to walk with extensive bracing becomes prohibitive. Lower-lumbar-level patients have present quadriceps function and most often remain ambulatory throughout childhood. Deformity must be addressed surgically in patients with lower-lumbar- and sacral-level myelomeningocele to prevent skin breakdown and infection in the insensate foot.

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Spine deformity is common in patients with myelomeningocele. Scoliosis can result from congenital malformations of the vertebrae in young children but more commonly occurs in later childhood or adolescence, presenting as a long sweeping curve with pelvic obliquity. Scoliosis is more common in patients with proximal-level spinal lesions. Bracing may be useful in young children with flexible curves, but anterior and posterior spinal fusion with instrumentation is the treatment of choice for larger curves.

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Kyphosis may be present in very young children with thoracic-level myelomeningocele. Sharp angular kyphosis can interfere with the ability to sit in a wheelchair easily and may lead to chronic skin breakdown over the spine and resultant infection. Surgical treatment is technically challenging.1-6 See Chapter 536 for further discussion.

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Cerebral palsy (CP) is the most common neuromuscular disorder seen in children. It is defined as a nonprogressive (static) encephalopathy resulting from injury, ischemia, or infection of the brain in the perinatal period. Cerebral palsy is discussed in detail in Chapter 554.

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Cerebral palsy can be classified either by the movement disorder present, or by ...

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