Management of bronchopulmonary dysplasia
- Ann R Stark, MD
Ann R Stark, MD
- Professor of Pediatrics
- Vanderbilt University School of Medicine
- Eric C Eichenwald, MD
Eric C Eichenwald, MD
- Professor of Pediatrics
- Perelman School of Medicine, University of Pennsylvania
Bronchopulmonary dysplasia (BPD), also known as neonatal chronic lung disease (CLD), is an important cause of respiratory morbidity in preterm newborns. Day-to-day care is mostly directed towards improving symptoms, with many common interventions having little impact on long-term outcome. Most patients with BPD gradually improve as healing occurs and lung growth continues, but the time required for improvement varies widely. Management is also directed at minimizing further injury, providing an optimal environment to support growth and recovery, and detecting complications associated with BPD.
The management of BPD is reviewed here. Pathogenesis and clinical features, prognosis, and potential strategies to prevent BPD are discussed separately. (See "Pathogenesis and clinical features of bronchopulmonary dysplasia" and "Outcome of infants with bronchopulmonary dysplasia" and "Prevention of bronchopulmonary dysplasia".)
Respiratory care is supportive and should minimize additional injury.
Mechanical ventilation — In patients with established bronchopulmonary dysplasia (BPD) who require mechanical ventilation, small tidal volumes are preferable to avoid additional mechanical injury. However, airway dilation and dead space to tidal volume ratio increase with chronic mechanical ventilation in extremely low birth weight (ELBW) infants , and infants with severe BPD may require higher tidal volumes to maintain effective ventilation. In particular, infants with bronchomalacia may require prolonged use, as well as higher levels of positive end-expiratory pressure (PEEP) to splint open large airways. In general, based on our clinical experience, maintaining a PEEP of 5 to 7 cm H2O minimizes atelectasis, and counters the development of pulmonary edema in infants with BPD. A slightly prolonged inspiratory duration of 0.4 to 0.5 seconds sometimes is needed to promote uniform lung inflation in patients who develop uneven airway obstruction. (See "Pathogenesis and clinical features of bronchopulmonary dysplasia", section on 'Mechanical ventilation'.)
Ongoing assessment of ventilator-dependent infants includes continuous pulse oximetry to monitor oxygenation, and intermittent blood gas sampling to monitor pH and PaCO2. The frequency of monitoring depends on the clinical condition of the patient. PaCO2 levels between 55 and 65 mmHg are tolerated as long as pH remains in the normal range (7.3 to 7.4). In patients with severe disease, PaCO2 values up to 70 mmHg may be tolerated on occasion to avoid further escalation of ventilator support.
- Keszler M, Nassabeh-Montazami S, Abubakar K. Evolution of tidal volume requirement during the first 3 weeks of life in infants <800 g ventilated with Volume Guarantee. Arch Dis Child Fetal Neonatal Ed 2009; 94:F279.
- Murthy K, Savani RC, Lagatta JM, et al. Predicting death or tracheostomy placement in infants with severe bronchopulmonary dysplasia. J Perinatol 2014; 34:543.
- DeMauro SB, D'Agostino JA, Bann C, et al. Developmental outcomes of very preterm infants with tracheostomies. J Pediatr 2014; 164:1303.
- Halliday HL, Dumpit FM, Brady JP. Effects of inspired oxygen on echocardiographic assessment of pulmonary vascular resistance and myocardial contractility in bronchopulmonary dysplasia. Pediatrics 1980; 65:536.
- Abman SH, Wolfe RR, Accurso FJ, et al. Pulmonary vascular response to oxygen in infants with severe bronchopulmonary dysplasia. Pediatrics 1985; 75:80.
- Tay-Uyboco JS, Kwiatkowski K, Cates DB, et al. Hypoxic airway constriction in infants of very low birth weight recovering from moderate to severe bronchopulmonary dysplasia. J Pediatr 1989; 115:456.
- Teague WG, Pian MS, Heldt GP, Tooley WH. An acute reduction in the fraction of inspired oxygen increases airway constriction in infants with chronic lung disease. Am Rev Respir Dis 1988; 137:861.
- Allen J, Zwerdling R, Ehrenkranz R, et al. Statement on the care of the child with chronic lung disease of infancy and childhood. Am J Respir Crit Care Med 2003; 168:356.
- Brown ER, Stark A, Sosenko I, et al. Bronchopulmonary dysplasia: possible relationship to pulmonary edema. J Pediatr 1978; 92:982.
- Bell EF, Acarregui MJ. Restricted versus liberal water intake for preventing morbidity and mortality in preterm infants. Cochrane Database Syst Rev 2014; :CD000503.
- Barrington KJ, Fortin-Pellerin E, Pennaforte T. Fluid restriction for treatment of preterm infants with chronic lung disease. Cochrane Database Syst Rev 2017; 2:CD005389.
- Puangco MA, Schanler RJ. Clinical experience in enteral nutrition support for premature infants with bronchopulmonary dysplasia. J Perinatol 2000; 20:87.
- Stewart AL, Brion LP. Routine use of diuretics in very-low birth-weight infants in the absence of supporting evidence. J Perinatol 2011; 31:633.
- Stewart A, Brion LP, Ambrosio-Perez I. Diuretics acting on the distal renal tubule for preterm infants with (or developing) chronic lung disease. Cochrane Database Syst Rev 2011; :CD001817.
- Stewart A, Brion LP. Intravenous or enteral loop diuretics for preterm infants with (or developing) chronic lung disease. Cochrane Database Syst Rev 2011; :CD001453.
- Pope JC 4th, Trusler LA, Klein AM, et al. The natural history of nephrocalcinosis in premature infants treated with loop diuretics. J Urol 1996; 156:709.
- Hufnagle KG, Khan SN, Penn D, et al. Renal calcifications: a complication of long-term furosemide therapy in preterm infants. Pediatrics 1982; 70:360.
- Hoffman DJ, Gerdes JS, Abbasi S. Pulmonary function and electrolyte balance following spironolactone treatment in preterm infants with chronic lung disease: a double-blind, placebo-controlled, randomized trial. J Perinatol 2000; 20:41.
- Wilkie RA, Bryan MH. Effect of bronchodilators on airway resistance in ventilator-dependent neonates with chronic lung disease. J Pediatr 1987; 111:278.
- Sosulski R, Abbasi S, Bhutani VK, Fox WW. Physiologic effects of terbutaline on pulmonary function of infants with bronchopulmonary dysplasia. Pediatr Pulmonol 1986; 2:269.
- Denjean A, Paris-Llado J, Zupan V, et al. Inhaled salbutamol and beclomethasone for preventing broncho-pulmonary dysplasia: a randomised double-blind study. Eur J Pediatr 1998; 157:926.
- National Asthma Education and Prevention Program: Expert panel report III: Guidelines for the diagnosis and management of asthma. Bethesda, MD: National Heart, Lung, and Blood Institute, 2007. (NIH publication no. 08-4051). www.nhlbi.nih.gov/guidelines/asthma/asthgdln.htm (Accessed on February 24, 2010).
- Doull IJ, Mok Q, Tasker RC. Tracheobronchomalacia in preterm infants with chronic lung disease. Arch Dis Child Fetal Neonatal Ed 1997; 76:F203.
- Committee on Fetus and Newborn. Postnatal corticosteroids to treat or prevent chronic lung disease in preterm infants. Pediatrics 2002; 109:330.
- Mirza H, Ziegler J, Ford S, et al. Pulmonary hypertension in preterm infants: prevalence and association with bronchopulmonary dysplasia. J Pediatr 2014; 165:909.
- Farrow KN, Steinhorn RH. Pulmonary hypertension in premature infants. Sharpening the tools of detection. Am J Respir Crit Care Med 2015; 191:12.
- Alagappan A, Malloy MH. Systemic hypertension in very low-birth weight infants with bronchopulmonary dysplasia: incidence and risk factors. Am J Perinatol 1998; 15:3.
- Anderson AH, Warady BA, Daily DK, et al. Systemic hypertension in infants with severe bronchopulmonary dysplasia: associated clinical factors. Am J Perinatol 1993; 10:190.
- Abman SH, Warady BA, Lum GM, Koops BL. Systemic hypertension in infants with bronchopulmonary dysplasia. J Pediatr 1984; 104:928.
- Tomashefski JF Jr, Oppermann HC, Vawter GF, Reid LM. Bronchopulmonary dysplasia: a morphometric study with emphasis on the pulmonary vasculature. Pediatr Pathol 1984; 2:469.
- Abman SH, Groothius JR. Pathophysiology and treatment of bronchopulmonary dysplasia. Current issues. Pediatr Clin North Am 1994; 41:277.
- Abman SH, Schaffer MS, Wiggins J, et al. Pulmonary vascular extraction of circulating norepinephrine in infants with bronchopulmonary dysplasia. Pediatr Pulmonol 1987; 3:386.
- Brownlee JR, Beekman RH, Rosenthal A. Acute hemodynamic effects of nifedipine in infants with bronchopulmonary dysplasia and pulmonary hypertension. Pediatr Res 1988; 24:186.
- Abman SH. Monitoring cardiovascular function in infants with chronic lung disease of prematurity. Arch Dis Child Fetal Neonatal Ed 2002; 87:F15.
- Malnick G, Pickoff AS, Ferrer PL, et al. Normal pulmonary vascular resistance and left ventricular hypertrophy in young infants with bronchopulmonary dysplasia: an echocardiographic and pathologic study. Pediatrics 1980; 66:589.
- Grover TR, Brozanski BS, Barry J, et al. High surgical burden for infants with severe chronic lung disease (sCLD). J Pediatr Surg 2014; 49:1202.
- Shepherd EG, Knupp AM, Welty SE, et al. An interdisciplinary bronchopulmonary dysplasia program is associated with improved neurodevelopmental outcomes and fewer rehospitalizations. J Perinatol 2012; 32:33.
- Balfour-Lynn IM, Field DJ, Gringras P, et al. BTS guidelines for home oxygen in children. Thorax 2009; 64 Suppl 2:ii1.
- RESPIRATORY SUPPORT
- Mechanical ventilation
- - Prolonged ventilation and tracheostomy
- FLUID RESTRICTION
- Our approach
- ACUTE EXACERBATIONS
- CHRONIC COMPLICATIONS
- Pulmonary artery hypertension
- Systemic blood pressure
- Vision and hearing
- Left ventricular hypertrophy
- COMPREHENSIVE MANAGEMENT APPROACH
- In-hospital care
- - Growth
- - Respiratory care
- - Surgery
- - Detection of complications
- - Planning
- - Care
- Pulmonary care
- Prevention of RSV infection
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS