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KYPHOSCOLIOSIS

Epidemiology and Pathobiology


Kyphoscoliosis, which is a common spinal disorder, affects approximately 1
in 1000 individuals, and about 1 in 10,000 affected individuals has a severe
spinal deformity. Deformities include excessive spinal curvature in the
coronal (scoliosis) and sagittal (kyphosis) planes as well as rotation of the
spinal axis. Kyphoscoliosis can be idiopathic or can be secondary
(paralytic) and associated with neuromuscular diseases, such as muscular
dystrophy and polio.
Idiopathic kyphoscoliosis, in which there may be a familial
predominance, usually manifests in late childhood or early adolescence
and involves females more than males with a ratio of 4:1. Although a defect
in the chromatin-remodeling gene family (CHD7) has been associated with
idiopathic kyphoscoliosis, other genes have also been identified.
Kyphoscoliosis produces one of the most severe restrictive
impairments of all the chest wall diseases. Total lung capacity and vital
capacity may be reduced to as low as 30% of predicted values. This
restrictive pathology becomes most severe as the degree of spinal
angulation increases. The patients age, degree of spinal rotation, presence
of respiratory muscle weakness, and involvement of the thoracic vertebrae
are all factors that promote the restrictive process. Respiratory failure is a
common cause of morbidity and mortality in patients with kyphoscoliosis.
Clinical Manifestations
Individuals with mild to moderate kyphoscoliosis may have complaints of
back pain and have psychosocial problems as a result of their deformity.
Adolescents with mild idiopathic kyphoscoliosis usually have normal
exercise capacity, whereas those with moderate idiopathic kyphoscoliosis
have reduced exercise capacity with additional exercise limitation due to
deconditioning. With severe deformities, patients may experience dyspnea
with minimal exertion or at rest.
Severe kyphoscoliosis can be readily diagnosed on physical
examination. Typical findings are the dorsal hump, which is due to the

angulated ribs and shoulder asymmetry, as well as the hip tilt that is related
to the spinal rotation. In younger individuals with milder spinal deformities,
the initial changes may be subtle. The Adams forward bend test, in which
the examiner observes for thoracic or lumbar region asymmetry while the
patient bends forward at the waist until the spine becomes parallel to the
floor, can help detect minor deformities. With severe kyphoscoliosis, signs
of right heart failure may be present such as cyanosis, distended neck
veins, peripheral edema, and hepatomegaly.
Individuals with kyphoscoliosis are particularly prone to hypoventilation
during sleep, especially REM sleep. Because sleep-related abnormalities
and their effects on cardiorespiratory function are potentially treatable,
individuals with kyphoscoliosis should be evaluated for nocturnal
hypoventilation well in advance of the development of daytime
hypercapnia.
Diagnosis
Although spinal deformity is often readily apparent on physical examination,
the degree of spinal deformity should be assessed by calculation of the
angle of spinal curvature (the Cobb angle) from radiographs. This angle is
formed by the intersection of lines parallel to the top and bottom vertebrae
of the scoliotic or kyphotic curves (Fig. 99-1). Angles more than 100
degrees are usually associated with respiratory symptoms, and angles
more than 120 degrees with respiratory failure. Factors associated with
progression of the spinal deformity include inspiratory muscle weakness, a
large spinal curvature at the time of presentation, skeletal immaturity, and a
thoracic location of the curve apex. Individuals with inspiratory muscle
weakness and kyphoscoliosis are more prone to develop respiratory failure
than those with normal inspiratory muscle strength.






TREATMENT
Patients should be encouraged to remain physically active to minimize
peripheral muscle deconditioning. In addition, general supportive measures
including immunizations against influenza and pneumococci, smoking
cessation, maintenance of a normal body weight, and treatment of
respiratory infections in a timely fashion should be instituted. Patients with
severe kyphoscoliosis and Cobb angles of more than 100 degrees should
be monitored closely for respiratory complications. Respiratory failure may
be precipitated by respiratory infections or by medications that produce
central nervous system depression.
Nocturnal hypoventilation typically precedes findings of daytime hyper-
capnia and hypoxemia and should be treated with NPPV. Indications for

instituting NPPV include symptoms of nocturnal hypoventilation or signs of
cor pulmonale with either an elevated daytime PaCO2 or nocturnal oxygen
saturation of less than 89% for 5 consecutive minutes. Supplemental
oxygen will be needed if hypoxemia persists despite correction of
hypoventilation. NPPV can reduce the number and duration of
hospitalizations and improve gas exchange, daytime blood gases, quality of
life, and survival (see Table 99-2).
Surgical and nonsurgical (back-brace) treatments have been used in
skeletally immature patients with idiopathic kyphoscoliosis in an effort to
correct or prevent progression of the spinal deformity. Braces have been
used for growing children with Cobb angles between 25 and 40 degrees,
whereas surgery has been used for adolescents with a Cobb angle of more
than 45 degrees. The overall role of surgical management in restoring
function to that of nonscoliotic individuals and minimizing the possibility of
respiratory failure is not clear.
Prognosis
Idiopathic kyphoscoliosis has a better prognosis than kyphoscoliosis
secondary to neuromuscular diseases. In general, individuals with mild
idiopathic kyphoscoliosis have an overall benign course. Patients with
moderate or severe deformities are at higher risk of developing respiratory
complications.
In secondary kyphoscoliosis, factors such as an early age of onset,
rapid curve progression during growth, progression of scoliosis after
skeletal maturity, large curves at the time of presentation, and a thoracic
rather than a thoracolumbar or lumbar location of the curve apex are risk
factors for respiratory complications. Respiratory failure may occur in
individuals with mild or moderate kyphoscoliosis owing to concurrent
respiratory muscle dysfunction. Muscle strength should be evaluated in
individuals with respiratory failure and Cobb angles of less than 100
degrees. When cor pulmonale develops, the prognosis is poor, and death
may occur within 1 year without therapy.

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