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