Statement on the Care of the Child with Chronic Lung Disease of Infancy and Childhood
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American Thoracic Society Documents Statement on the Care of the Child with Chronic Lung Disease of Infancy and Childhood This Official Statement of the American Thoracic Society Was Approved by the ATS Board of Directors, December 2002 CONTENTS EXECUTIVE SUMMARY Executive Summary Chronic lung disease of infancy (CLDI) represents the final com- I. Introduction, Definitions, and Epidemiology .......356 mon pathway of a heterogeneous group of pulmonary disorders A. Introduction .............................356 that start in the neonatal period. Often the inciting factor is bron- B. Definitions of Bronchopulmonary Dysplasia . 357 chopulmonary dysplasia (BPD), a chronic condition that usually C. Is the Prevalence of Chronic Lung Disease evolves after premature birth and respiratory distress syndrome of Infancy Changing? ......................357 due to surfactant deficiency. Myriad other conditions can also D. Differential Diagnosis ......................358 cause airway and parenchymal inflammation that leads to chronic airflow obstruction, increased work of breathing, and airway hyp- II. Pathophysiology and Pathogenesis ...............358 erreactivity. Usually the inciting factors are not only the underlying A. Respiratory System........................358 disorders, but also the effects of the supportive treatment, includ- B. Cardiovascular System .....................363 ing mechanical ventilation, barotrauma, and oxygen toxicity. C. Feeding, Nutrition, and Gastrointestinal System . 363 These aggressive interventions for serious neonatal and infant lung D. Renal System ............................364 diseases are often responsible for much of the chronic pulmonary E. Neurologic System and Development ..........365 F. Ophthalmology ...........................366 abnormalities that follow. There has been an evolution of the G. Chronic Lung Disease of Infancy as a etiologies of CLDI as well; most current CLDI is seen in infants Multisystem Disease .......................366 born increasingly prematurely, and represents a disorder of intra- uterine inflammation and premature extrauterine lung develop- III. Evaluation and Diagnostic Studies ...............366 ment characterized by alveolar simplification. This is in contrast A. Respiratory .............................366 to the early descriptions of BPD, in which postnatal inflammation B. Cardiologic ..............................369 and fibrosis due to barotrauma and oxygen toxicity played more C. Nutritional ..............................370 of a role. D. Renal ..................................370 These early lung disorders have far-reaching consequences that E. Neurodevelopmental ......................371 extend into childhood and beyond. In addition, they are often F. Ophthalmologic . .................371 accompanied by precipitating and complicating conditions that are not relegated to the respiratory system; CLDI is truly a IV. Treatment .................................371 multisystem disorder. This statement reviews more recent ad- A. Transitioning the Infant with Chronic Lung vances in our understanding of the pathophysiology of CLDI, not Disease from Hospital to Home . .............371 B. Specific Interventions ......................374 only in the respiratory system but also in the multiple organ 1. Bronchodilators ........................374 systems involved in these children. The current approaches to 2. Antiinflammatory Drugs ..................375 diagnostic evaluation of CLDI and its complications are reviewed, 3. Oxygen Therapy .......................376 and specific interventions based on understanding pathophysio- 4. Airway Problems and Tracheostomy Care . 378 logic mechanisms are discussed. Throughout, an interdisciplinary 5. Long-Term Ventilator Care ...............379 approach to the care of these children is emphasized. Finally, 6. Diuretics, Afterload Reducers, and Other future directions for clinical research leading to better understand- Cardiac Pharmacology . .................379 ing and more effective prevention and treatment of CLDI are 7. Nutrition .............................379 suggested. 8. Development Intervention . ..............381 9. Ophthalmology ........................381 I. INTRODUCTION, DEFINITIONS, AND EPIDEMIOLOGY 10. Well-Child Care ........................382 11. Ethical Issues . .................382 A. Introduction Chronic lung disease of infancy (CLDI) is a heterogeneous group V. Conclusions and Clinical Research Questions ......383 of respiratory diseases of infancy that usually evolves from an acute respiratory disorder experienced by a newborn infant. CLDI most commonly occurs in infants with birth weights less than 1,500 g, and especially in those with birth weights less than 1,000 g and who are treated for respiratory distress syndrome (RDS). However, any disorder that produces an acute lung injury and/or Members of the ad hoc Statement Committee have disclosed any direct commercial requires treatment with positive-pressure mechanical ventilation associations (financial relationships or legal obligations) related to the preparation and high concentrations of inspired oxygen during the initial weeks of this statement. This information is kept on file at the ATS headquarters. of life predisposes the infant to the development of CLDI. There- Am J Respir Crit Care Med Vol 168. pp 356–396, 2003 DOI: 10.1164/rccm.168.3.356 fore, in addition to RDS, conditions that have resulted in CLDI Internet address: www.atsjournals.org include pneumonia/sepsis, meconium aspiration pneumonia, pul- American Thoracic Society Documents 357 treatment of the child with CLDI are based on review of the more recent literature and the experience of the authors. These are not clinical practice guidelines per se, which are best based on formal evaluation of large, randomized, placebo-controlled, blinded clinical trials, often including metaanalyses. Although such trials, some of which are described in this statement, are available in the neonatology literature, they deal primarily with prenatal and postnatal prevention of BPD. Unfortunately, such large clinical trials dealing with the care of established CLDI and CLD in later childhood have not been performed. Until this is the case, care for these children will have to be based on critical evaluation of the literature and experience. B. Definitions of Bronchopulmonary Dysplasia A working definition of BPD is necessary, because it is from BPD that the majority of cases of CLDI arise. Since BPD was Figure 1. Proposed nosology of chronic lung disease of infancy. The first described by Northway and coworkers in 1967 (4), there term bronchopulmonary dysplasia (BPD) best describes chronic lung has been considerable debate about the clinical and functional disease subsequent to oxygen and/or ventilator therapy for respiratory characteristics (and the age at which to determine these charac- distress syndrome (RDS) in preterm newborns. Some full-term newborns teristics) that should be used in its definition. In 1979, Bancalari can have BPD subsequent to mechanical ventilation for other neonatal and coworkers (5) proposed three basic criteria to define BPD: respiratory conditions. Chronic lung disease of prematurity (CLDP) is (1) supplemental oxygen requirement at 28 days of postnatal sometimes used interchangeably with BPD, but this term is best reserved life, (2) persistent abnormalities of the chest radiograph, and for other chronic lung diseases of the preterm infant that can arise after (3) tachypnea in the presence of rales or retractions. In 1989, the an initial period without an oxygen or ventilatory requirement. All these Maternal and Child Health Bureau (6) proposed the following disorders are types of chronic lung disease of infancy (CLDI), which can evolve after infancy into CLD of childhood and adolescence. FT ϭ full- diagnostic criteria for BPD: (1) positive-pressure ventilation dur- term; p¯RDS ϭ post–respiratory distress syndrome of prematurity. ing the first 2 weeks of life for a minimum of 3 days, (2) clinical signs of respiratory compromise persisting longer than 28 days of age, (3) requirement for supplemental oxygen for more than 28 days to maintain a PaO2 above 50 mm Hg, and (4) chest monary hypoplasia, persistent pulmonary hypertension, apnea, radiograph with findings characteristic of BPD. tracheoesophageal fistula, congenital diaphragmatic hernia, con- The use of the above-defined criteria has been questioned genital heart disease, and congenital neuromuscular disorders (7–10). With advances in treatment, many infants who still re- (1, 2). quire oxygen at the age of 28 days either do not require prolonged The terminologies used to describe chronic lung disease aris- mechanical ventilation during the newborn period or do not ing from neonatal insults are confusing. The terms “bronchopul- have the characteristic radiographic changes of BPD. It has thus monary dysplasia” (BPD) and “chronic lung disease of prematu- been suggested that simple oxygen requirement at 28 days in rity” (CLDP) are sometimes used interchangeably to describe infants with birth weights of 1,500 g or less be used as a criterion chronic respiratory disease following treatment for RDS in pre- to define BPD (7, 9). term infants. A National Institutes of Health (NIH, Bethesda, Shennan and coworkers (8) disputed the definition of BPD MD) workshop report suggested that the term “bronchopulmo- based on oxygen need beyond 28 days of age. They reasoned nary dysplasia” be retained in preference to “chronic lung dis- that most BPD observed presently