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CHAPTER 58: Restrictive Disease of the Respiratory System 513

• Ong TH, Eng P. Massive hemoptysis requiring intensive care.
Intensive Care Med. 2003;29:317-320. • If mechanical ventilation is deemed appropriate, the use of low
tidal volumes and high respiratory rates during mechanical venti-
• Revel MP, Fournier LS, Hennebicque AS, et al. Can CT replace lation likely minimize ventilator-induced lung injury.
bronchoscopy in the detection of the site and cause of bleeding in • Idiopathic pulmonary fibrosis is typically refractory to pharmaco-
patients with large or massive hemoptysis? AJR Am J Roentgenol.
2002;179:1217-1224. therapy.
• Sakr L, Dutau H. Massive hemoptysis: an update on the role • Lung transplantation is a viable option in selected patients with
of bronchoscopy in diagnosis and management. Respiration. end-stage fibrosis.
2010;80:38-58.
• Shigemura N, Wan IY, Yu SC, et al. Multidisciplinary management Thoracic cage deformity and pulmonary fibrosis both result in a restric-
of life-threatening massive hemoptysis: a 10-year experience. Ann
Thorac Surg. 2009;87:849-853. tive limitation to breathing. Although relatively rare in the context of
pulmonary intensive care, these disorders present unique challenges that
• Swanson KL, Johnson CM, Prakash UB, McKusick MA, Andrews complicate ICU management. In this chapter, we describe the patho-
JC, Stanson AW. Bronchial artery embolization: experience with physiologic derangements in cardiopulmonary function associated with
54 patients. Chest. 2002;121:789-795. these disorders and how they affect management during acute illness.
• Valipour A, Kreuzer A, Koller H, Koessler W, Burghuber OC. A primary goal of this chapter is to offer a strategy for cardiovascular
Bronchoscopy-guided topical hemostatic tamponade therapy management and mechanical ventilation that minimizes the risk of
for the management of life-threatening hemoptysis. Chest. 2005; ventilator-induced complications and maximizes the chance for early,
127:2113-2118. successful extubation. Many of these recommendations are grounded
more on general precepts than on disease/disorder-specific evidence.
PATIENTS WITH THORACIC CAGE DEFORMITY

REFERENCES Although a number of disorders can deform and restrict the move-
ment of the respiratory system (Table 58-1), kyphoscoliosis (KS) is the
Complete references available online at www.mhprofessional.com/hall prototypical cause of severe thoracic deformity. Kyphoscoliosis is
the combination of kyphosis (posterior deformity of the spine) and
scoliosis (lateral deformity of the spine). It is far more common than
isolated cases of kyphosis or scoliosis, placing over 200,000 people in
Restrictive Disease the United States at risk of developing respiratory failure. Most cases are
1
CHAPTER idiopathic and begin in childhood. Other cases result from congenital
2
58 of the Respiratory System defects, connective tissue disease, poliomyelitis, thoracoplasty, syringo-
myelia, vertebral and spinal cord tumors, and tuberculosis.
Benjamin David Singer The pathophysiologic consequences of KS correlate with the degree
Thomas Corbridge of spinal curvature, but there is considerable variability. Patients with
1-3
4-6
Lawrence D. H. Wood severe deformity can lead long and relatively symptom-free lives, while
patients with lesser degrees of curvature may develop ventilatory failure
and cor pulmonale at a young age. The reason for this variability is not
7
KEY POINTS always clear. However, sleep-disordered breathing underlies clinical
deterioration in some patients. 8,9
• Scoliotic curves greater than 100° may cause dyspnea; curves The combination of a moderate kyphotic deformity and a moderate
greater than 120° are associated with alveolar hypoventilation and scoliotic deformity is functionally equivalent to a severe deformity of
cor pulmonale. either alone. Of the two, however, scoliosis produces greater physiologic
3
• Biphasic positive airway pressure may be effective in patients with derangements. In KS, scoliotic curves less than 70° (Fig. 58-1) rarely
acute hypercapnic respiratory failure. cause problems, while angles greater than 70° increase the risk of respira-
1,2
• Low tidal volumes and high respiratory rates likely minimize tory failure. The earlier in life this angle is achieved, the greater the risk
the risk of barotrauma during mechanical ventilation; however, of eventually developing respiratory failure, as curvature angles increase
gradual institution of anti-atelectasis measures may improve gas by an average of 15° over 20 years from an initial angle of 70°. 10-12 Angles
exchange and static compliance. greater than 100° can cause dyspnea; angles ≥120° can result in alveolar
• Nocturnal hypoxemia is common and may contribute to cardio- hypoventilation and cor pulmonale. 1,7
In order to decrease respiratory work, patients with severe deformity
vascular deterioration; routine polysomnography is recommended. and low respiratory system compliance take rapid and shallow breaths.
• Strategies for management of patients with chronic ventilatory failure
include daytime intermittent positive pressure ventilation, nocturnal
noninvasive ventilation, and ventilation through tracheostomy. TABLE 58-1 Selected Diseases of the Chest Wall
• Acute deterioration in respiratory status can occur from disease Pectus excavatum
progression, upper and lower respiratory tract infections, conges-
tive heart failure, failure to clear secretions, atelectasis, aspiration, Pectus carinatum
and pulmonary embolism. Poland syndrome
• Most patients with chest wall deformity survive their first episode Kyphoscoliosis
of acute respiratory failure. Thoracoplasty
• Patients with idiopathic pulmonary fibrosis admitted to the ICU Fibrothorax
with acute respiratory failure have an extremely poor prognosis.
Chest wall tumors

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