Bronchopulmonary Dysplasia

Overview

Bronchopulmonary dysplasia (BPD) is a form of chronic respiratory disease manifested by the persistent need for supplemental oxygen (fraction of inspired oxygen >21%) for more than 28 days after birth for infants born at <32 weeks gestation. The lung disease is characteristically heterogeneous, encompassing both large and small airways with abnormalities in both the lung parenchyma and pulmonary vasculature. Typically diagnosed prior to discharge from a neonatal intensive care unit, the medical home primary care clinician needs to be aware of how to provide ongoing follow-up care for babies and children affected by BPD.

Other Names

Arrest of Lung Development
Chronic Lung Disease of Prematurity or Infancy
Evolving Chronic Lung Disease
Neonatal Chronic Lung Disease
Respiratory Insufficiency

ICD-10

P27.1, Bronchopulmonary dysplasia originating in the perinatal period
Z99.81, Dependence on supplemental oxygen
90772, Injection of palivizumab
90378, Palivizumab (Synagis) serum for intramuscular use

Prognosis

While many children with BPD outgrow their symptoms and have no long-term respiratory problems, there is increasing evidence that BPD may be a life-long problem for some children. Children with BPD are at increased risk of long-term respiratory complications such as reactive airways disease, chronic obstructive pulmonary disease, and lung function decline, especially those with a history of the most severe forms of BPD. [Collaco: 2018]
Long-term outcomes of BPD remain difficult to characterize as studies of adult populations represent survivors of outdated care. Studies of formerly preterm young adults with BPD show obstructive pulmonary defects compared to controls without BPD. [Vom: 2014]
Common respiratory infections can lead to severe morbidity and potential mortality in children with BPD. In the 1st year of life, 49% of infants with BPD will require rehospitalization. [Smith: 2004] BPD increases susceptibility to infection, which persists into childhood. BPD severity is correlated with long-term respiratory morbidity and neurodevelopmental impairments, including cerebral palsy, with severe BPD requiring prolonged positive pressure support predicting the greatest risk of poor neurodevelopmental outcome. [Trittmann: 2013]

Pearls & Alerts

Chronic lung disease can occur in term-infants
BPD is seen in children born prematurely; however, chronic lung disease can also occur in term infants who experience neonatal lung injury (i.e. congenital lung malformations, congenital diaphragmatic hernia, meconium aspiration syndrome, etc.). The mechanisms of chronic lung disease in term infants likely differs from those that cause BPD in preterm infants. Newer definitions tend to limit BPD to babies born at <32 weeks gestation (Jensen, 2019; Higgins, 2018, [Abman: 2017]). More people are using the term chronic lung disease for patients born at 32 weeks gestation or more.
Risks during first 2 years
Children with BPD related to prematurity are at increased risk of emergency room visits and hospitalizations related to respiratory infections during the first 2 years of life. [McGrath-Morrow: 2019]
Lung function testing is necessary
All children with BPD should have lung function testing, typically at age 4 or 5 years.

Risk Factors

Risk factors for BPD development include low gestational age at birth, low birth weight, maternal chorioamnionitis, sepsis, necrotizing enterocolitis requiring surgery, patent ductus arteriosus, neonatal lung injury (e.g., pneumothorax, pulmonary interstitial emphysema, atelectasis), fetal growth restriction, and placental insufficiency. [Abman: 2017]

Diagnosis

Diagnostic Criteria

BPD is a form of chronic respiratory disease manifested by the persistent need for supplemental oxygen (fraction of inspired oxygen >21%) for more than 28 days after birth. [Jobe: 2001] Diagnosis of BPD is currently based entirely on clinical course rather than exam, lab results, or imaging findings. However, chest radiographs from the neonatal intensive care unit (NICU) stay of infants with BPD may show:
  • Central airway abnormalities (tracheobronchomalacia or narrowing/stenosis)
  • Structural remodeling, hyperreactivity, and bronchoconstriction of the small airways
  • Decreased alveolarization and vascular remodeling in the distal airspaces and pulmonary vasculature

Clinical Classification

Classification of BPD has been evolving due to changes in care received in the NICU. Generally, BPD severity is categorized as mild, moderate, or severe.
  • For infants born <32 weeks gestation, BPD severity grading of mild, moderate or severe is determined at post-menstrual age of 36 weeks based on level of supplemental O2 need (FiO2 at room air, <30% or >30%) and/or need for positive pressure support (high-flow nasal cannula, CPAP, or positive pressure ventilation).
  • For infants born at greater than 32 weeks gestation, BPD severity is determined similarly based on date of discharge or by 56 days of life, whichever comes first. [Jobe: 2001]
More severe BPD correlates with greater likelihood of respiratory and neurocognitive morbidities. [Thébaud: 2019] Similarly, prolonged length of NICU stay has been associated with severity of neurocognitive morbidity (Bauer: 2020).

Physical Examination

Respiratory

Infants and children with BPD may present with tachypnea, mild subcostal retractions, or increased anterior posterior diameter (barrel chest). Infants may have sporadic cough or signs of reflux (e.g., back-arching, fussiness). Wheezing, heavy breathing, or noisy breathing may be heard if there is associated laryngomalacia or tracheomalacia or intercurrent illness. In older children, there may be abnormal chest wall development with bilateral concavity of the lower chest wall from chronic subcostal retractions.
When establishing care, inquire about “baseline” retractions. At future visits, monitor for changes in the work of breathing compared to baseline.

Skin

Reactions to adhesives can occur where stickers are used to anchor nasal cannula. Counsel the family to use skin protectant and adhesive remover.

Treatment & Management

Goal of Management

Because children with BPD related to prematurity are at increased risk of emergency room visits and hospitalizations related for respiratory infections in the first 2 years of life, as well as increased risk of developing chronic obstructive pulmonary disease, the goals of management in the medical home include:
  • Optimizing nutrition and growth
  • Providing adequate outpatient oxygen therapy and weaning when able
  • Preventing complications through targeted immunizations and avoidance of respiratory pathogens and pollutants
  • Monitoring for and treating comorbid conditions
  • Providing family support and answering questions
  • Coordinating interdisciplinary care with specialists such as pediatric pulmonologists

Management by System

Respiratory

  • Provide, or refer to Pulmonology, for serial monitoring of lung function (spirometry) to assess for the development of obstructive (e.g., asthma, tracheobronchomalacia, etc.) and/or restrictive lung disease. The chronic lung disease of children with BPD often shows airflow obstruction and intermittent pulmonary exacerbations can be frequent in the first 2 years of life. Long-term lung function of children and adults with a history of BPD has, on average, lower lung function (FEV1) compared to children and adult controls born at term. All children with BPD should have complete pulmonary function testing, typically starting at age 4 or 5 years. [McGrath-Morrow: 2019]
  • Provide home oxygen therapy for children with chronic hypoxemia and wean as tolerated. Home pulse oximetry studies while sleeping or serial clinic evaluations of oxygen saturations can be used to monitor progress and to wean off oxygen therapy used at home and during car seat use.
  • If there is persistent nocturnal hypoxemia or signs and symptoms of sleep-disordered breathing (e.g., snoring, restless sleep), refer for a polysomnography/sleep study to assess for sleep-disordered breathing (e.g., obstructive and/or central sleep apnea).
  • Refer to ENT or Pulmonology to perform airway endoscopy to assess for possible upper and lower airway abnormalities such as suspected tracheobronchomalacia and to develop a co-management plan. Management of abnormalities may require additional investigation (e.g., modified barium swallow study), medications, initiation of non-invasive positive pressure (CPAP), or surgical interventions.
  • Obtain echocardiogram to screen for pulmonary hypertension every 4-6 months for children with moderate to severe BPD who are at the highest risk for developing pulmonary hypertension. This is typically in the first 1-2 years of life. Risk factors include poor growth and history of pulmonary hypertension in the NICU.

Nutrition and Growth

  • Monitor carefully and ensure adequate nutrition, including calories and protein. Linear growth is particularly important for lung growth. See the Portal's Premature Infant Follow-Up for more information on feeding and nutrition in this population.

Infectious Disease

  • Provide routine childhood immunization including influenza annually and pneumococcal vaccinations.
  • Administer palivizumab (Synagis) to eligible children <24 months of age to prevent serious lung disease from RSV infection. [American: 2014] For a summary of recommendations, see RSV Recommendations (AAP).
  • Administer expanded pneumonia vaccine, PPSV-23, at ≥2 years of age, at least 8 weeks after completing PCV-13. PPSV-23 is recommended for all children with chronic lung disease (BPD is considered a CLD of prematurity/infancy) in children ≥2 years of age. A second dose of PPSV-23 is recommended 5 years after the first dose after age 2 years to targeted populations for children who have anatomic or functional asplenia, including SCD, HIV infection, or other immunocompromising condition. [Nuorti: 2010]

Comorbid Conditions

Comorbidities that may occur in infants with BPD include gastroesophageal reflux, dysphagia, aspiration, sleep-disordered breathing (including obstructive and central sleep apnea), atelectasis, pulmonary hypertension, developmental delays, and cerebral palsy. [Gou: 2018] Studies are underway to better understand the rates of comorbidities.

Testing

Imaging

After discharge from the NICU, there is generally no need for repeated imaging of children with known BPD if they improve as expected and wean off oxygen. For those patients that do not improve as expected, the following testing may be considered.
  • Consider chest radiographs to differentiate complications such as aspiration pneumonitis, reactive airways disease, chronic atelectasis, or bacterial pneumonia.
  • In severe cases, pediatric pulmonologists might utilize bronchoscopy or chest CT to evaluate for complications such as tracheobronchomalacia, and parenchymal or cystic disease.
  • In cases where airway abnormalities are suspected (presence of stridor, noisy breathing, poor sleep, etc.) refer to Pediatric Pulmonology to perform airway endoscopy to assess upper and lower airway abnormalities (e.g., tracheobronchomalacia, bronchiectasis, interstitial lung disease).
  • Obtain echocardiogram to screen for pulmonary hypertension every 4-6 months for children with BPD at high risk of developing pulmonary hypertension, typically in the first 1-2 years of life. Children with severe BPD, or moderate BPD with poor growth, are at highest risk of developing pulmonary hypertension. Children with a history of BPD who show poor growth or inability to wean off supplemental oxygen (chronic hypoxemia) should undergo echocardiogram screening for pulmonary hypertension.
  • A chest CT with angiogram may be used to assess for vascular anomalies such as pulmonary vein stenosis or pulmonary hypertension.

Labs

None needed routinely. Consider a capillary or venous blood gas if the infant is ill-appearing and in respiratory distress.

Pulse Oximetry

Many infants with BPD have chronic hypoxemia and will need to wean off oxygen as an outpatient. Pulse oximetry is recommended in the home for oxygen therapy monitoring. Monitoring oxygen while feeding and sleeping can provide insight into what the infant or child’s ongoing requirement is because O2 levels tend to drop during feeding and sleeping. For weaning off supplemental oxygen used in car seats, guidelines include oxygen saturation not falling <90% for >10 seconds or heart rate < 80 bpm for >10 seconds on room air, for at least 90 minutes or the duration of the car ride (whichever is longer). Nocturnal home oximetry studies are helpful to determine readiness to wean off supplemental O2 while sleeping. Home oxygen therapy is recommended for children with BPD complicated by chronic hypoxemia. Chronic hypoxemia is defined as either: 1) greater or equal to 5% of recording time spent with SpO2 93% or less during a continuous recording, or 2) at least 3 separate findings of an SpO2 less than or equal to 93% measured intermittently. [Hayes: 2019]

Referrals

BPD is a complex disease that affects multiple systems and therefore severe BPD usually requires a multi-disciplinary approach. Children with BPD should be referred to a pediatric pulmonologist (or BPD specialist), nutritionist, feeding therapist, gastroenterologist, cardiologist, sleep medicine pulmonologist, or ENT specialist for further evaluation and intervention as indicated.

Pediatric Pulmonology

Refer if child still requires supplemental oxygen 2 months after discharge from NICU (or around CGA 2 months), has chronic respiratory symptoms, or difficult to manage reactive airways disease or asthma. All children with severe BPD or moderate BPD with poor growth should be seen by Pulmonology outpatient. Lung function testing should be performed for children with BPD, typically starting at age 4-5 years. Refer to a sleep medicine pulmonologist if there are signs and symptoms of sleep-disordered breathing (e.g., apneas, snoring, pauses in sleep, or restless sleep) or persistent nocturnal hypoxemia Pediatric Pulmonology (see NW providers [0]).

Nutrition Assessment Services

Refer all children with poor growth. Furthermore, keep in mind that good growth, particularly linear growth, is associated with resolution of symptoms, therefore nutrition assessment should be considered in all patients with severe BPD. Nutrition Assessment Services (see NW providers [0])

Feeding Therapist

Refer children with signs/symptoms of dysphagia or aspiration. Swallow Disorder/Dysphagia Clinics (see NW providers [0])

Pediatric Gastroenterology

Refer for faltering growth, failure to thrive, feeding difficulties, severe GER, constipation, or consideration of G-tube placement. Pediatric Gastroenterology (see NW providers [1])

Pediatric Cardiology

Refer if there are shunt lesions (e.g., PDA, ASD, VSD), history of pulmonary hypertension, abnormal echocardiogram, or EKGs. Pediatric Cardiology (see NW providers [1])

Pediatric Otolaryngology

Refer for signs and symptoms of upper airway obstruction, such as noisy breathing, stridor, or concerns for anatomy airway abnormalities (e.g., moderate to severe laryngomalacia, laryngeal cleft, tracheomalacia, vocal cord paralysis or paresis, enlarged tonsils or adenoids). Pediatric Otolaryngology (see NW providers [1])

Resources

Information & Support

For Parents and Patients

Bronchopulmonary Dysplasia (CHEST Foundation)
Symptoms, diagnosis and treatment information for patients and families about bronchopulmonary dysplasia (BPD).

Practice Guidelines

Abman SH, Collaco JM, Shepherd EG, Keszler M, Cuevas-Guaman M, Welty SE, Truog WE, McGrath-Morrow SA, Moore PE, Rhein LM, Kirpalani H, Zhang H, Gratny LL, Lynch SK, Curtiss J, Stonestreet BS, McKinney RL, Dysart KC, Gien J, Baker CD, Donohue PK, Austin E, Fike C, Nelin LD.
Interdisciplinary Care of Children with Severe Bronchopulmonary Dysplasia.
J Pediatr. 2017;181:12-28.e1. PubMed abstract / Full Text
Recommendations from the “BPD Collaborative, a group of clinicians from interdisciplinary care programs for infants with sBPD at several major medical centers, including neonatologists, pulmonologists, critical care physicians, gastroenterologists, nurse specialists, and others, to address controversies and promote research to enhance the care of children with sBPD. The review emphasizes an interdisciplinary approach throughout the NICU and outpatient courses.

American Academy of Pediatrics Committee on Infectious Diseases; American Academy of Pediatrics Bronchiolitis Guidelines Committee.
Updated guidance for palivizumab prophylaxis among infants and young children at increased risk of hospitalization for respiratory syncytial virus infection.
Pediatrics. 2014;134(2):e620-38. PubMed abstract

Patient Education

What is Bronchopulmonary Dysplasia? (American Thoracic Society) (PDF Document 310 KB)
A printable, colorful, 2-page, patient handout on BPD. Main points covered are: Know your baby’s symptoms and what problems to look for; Give your baby oxygen and other treatments as directed; Make sure your baby is getting good nutrition to grow; Take actions to help your baby avoid respiratory infections; Avoid tobacco smoke and other air pollution.

Tools

Effective FiO2 Conversion for Infants on Nasal Cannula
A calculator to approximate effective inspired oxygen; Benaron DA & Benitz WE. Maximizing the stability of oxygen delivered via nasal cannula. Arch Pediatr Adolesc Med. 1994 Mar;148(3):294-300.

Services for Patients & Families Nationwide (NW)

For services not listed above, browse our Services categories or search our database.

* number of provider listings may vary by how states categorize services, whether providers are listed by organization or individual, how services are organized in the state, and other factors; Nationwide (NW) providers are generally limited to web-based services, provider locator services, and organizations that serve children from across the nation.

Helpful Articles

Abman SH, Collaco JM, Shepherd EG, Keszler M, Cuevas-Guaman M, Welty SE, Truog WE, McGrath-Morrow SA, Moore PE, Rhein LM, Kirpalani H, Zhang H, Gratny LL, Lynch SK, Curtiss J, Stonestreet BS, McKinney RL, Dysart KC, Gien J, Baker CD, Donohue PK, Austin E, Fike C, Nelin LD.
Interdisciplinary Care of Children with Severe Bronchopulmonary Dysplasia.
J Pediatr. 2017;181:12-28.e1. PubMed abstract / Full Text
Recommendations from the “BPD Collaborative, a group of clinicians from interdisciplinary care programs for infants with sBPD at several major medical centers, including neonatologists, pulmonologists, critical care physicians, gastroenterologists, nurse specialists, and others, to address controversies and promote research to enhance the care of children with sBPD. The review emphasizes an interdisciplinary approach throughout the NICU and outpatient courses.

Duijts L, van Meel ER, Moschino L, Baraldi E, Barnhoorn M, Bramer WM, Bolton CE, Boyd J, Buchvald F, Del Cerro MJ, Colin AA, Ersu R, Greenough A, Gremmen C, Halvorsen T, Kamphuis J, Kotecha S, Rooney-Otero K, Schulzke S, Wilson A, Rigau D, Morgan RL, Tonia T, Roehr CC, Pijnenburg MW.
European Respiratory Society guideline on long-term management of children with bronchopulmonary dysplasia.
Eur Respir J. 2020;55(1). PubMed abstract
This European practice guideline provides recommendations for monitoring and treating children with bronchopulmonary dysplasia (BPD) who have been discharged from the hospital or were >36 weeks of postmenstrual age.

Davidson LM, Berkelhamer SK.
Bronchopulmonary Dysplasia: Chronic Lung Disease of Infancy and Long-Term Pulmonary Outcomes.
J Clin Med. 2017;6(1). PubMed abstract / Full Text
This article discusses increased pulmonary morbidities seen in children and adults with a history of bronchopulmonary dysplasia (BPD). Combined with environmental and infectious exposures, these conditions related to BPD may result in significant long-term pulmonary sequalae and represent a growing burden on health systems.

Authors & Reviewers

Initial publication: August 2020
Current Authors and Reviewers:
Author: Khanh Lai, MD
Contributing Author: Jennifer Goldman-Luthy, MD, MRP, FAAP
Reviewers: Peer review pending
Leif D. Nelin, MD

Page Bibliography

Abman SH, Collaco JM, Shepherd EG, Keszler M, Cuevas-Guaman M, Welty SE, Truog WE, McGrath-Morrow SA, Moore PE, Rhein LM, Kirpalani H, Zhang H, Gratny LL, Lynch SK, Curtiss J, Stonestreet BS, McKinney RL, Dysart KC, Gien J, Baker CD, Donohue PK, Austin E, Fike C, Nelin LD.
Interdisciplinary Care of Children with Severe Bronchopulmonary Dysplasia.
J Pediatr. 2017;181:12-28.e1. PubMed abstract / Full Text
Recommendations from the “BPD Collaborative, a group of clinicians from interdisciplinary care programs for infants with sBPD at several major medical centers, including neonatologists, pulmonologists, critical care physicians, gastroenterologists, nurse specialists, and others, to address controversies and promote research to enhance the care of children with sBPD. The review emphasizes an interdisciplinary approach throughout the NICU and outpatient courses.

American Academy of Pediatrics Committee on Infectious Diseases; American Academy of Pediatrics Bronchiolitis Guidelines Committee.
Updated guidance for palivizumab prophylaxis among infants and young children at increased risk of hospitalization for respiratory syncytial virus infection.
Pediatrics. 2014;134(2):e620-38. PubMed abstract

Collaco JM, McGrath-Morrow SA.
Respiratory Phenotypes for Preterm Infants, Children, and Adults: Bronchopulmonary Dysplasia and More.
Ann Am Thorac Soc. 2018;15(5):530-538. PubMed abstract
This review describes respiratory conditions that individuals born prematurely may experience throughout their lifespan including alveolar, airway, and vascular phenotypes associated with bronchopulmonary dysplasia, as well as non-bronchopulmonary dysplasia respiratory phenotypes such as airway malacia, obstructive sleep apnea, and control of breathing issues. The review illustrates what is known about the potential for progression and/or lack of resolution of these respiratory phenotypes in childhood and adult life.

Gou X, Yang L, Pan L, Xiao D.
Association between bronchopulmonary dysplasia and cerebral palsy in children: a meta-analysis.
BMJ Open. 2018;8(9):e020735. PubMed abstract / Full Text
This study suggests that bronchopulmonary dysplasia (BPD) is a risk factor for cerebral palsy (CP) in children.

Hayes D Jr, Wilson KC, Krivchenia K, Hawkins SMM, Balfour-Lynn IM, Gozal D, Panitch HB, Splaingard ML, Rhein LM, Kurland G, Abman SH, Hoffman TM, Carroll CL, Cataletto ME, Tumin D, Oren E, Martin RJ, Baker J, Porta GR, Kaley D, Gettys A, Deterding RR.
Home Oxygen Therapy for Children. An Official American Thoracic Society Clinical Practice Guideline.
Am J Respir Crit Care Med. 2019;199(3):e5-e23. PubMed abstract / Full Text

Jobe AH, Bancalari E.
Bronchopulmonary dysplasia.
Am J Respir Crit Care Med. 2001;163(7):1723-9. PubMed abstract
This article summarizes a workshop on Bronchopulmonary Dysplasia for the National Institute of Child Health and Human Development, the National Heart, Lung and Blood Institute, and the Office of Rare Diseases held in June, 2000. The workshop reviewed the definition of BPD and lung injury in very preterm infants, identified gaps in knowledge about lung development and the best indicators of outcome for infants with BPD, and determined priorities for future research.

McGrath-Morrow SA, Collaco JM.
Bronchopulmonary dysplasia: what are its links to COPD?.
Ther Adv Respir Dis. 2019;13:1753466619892492. PubMed abstract / Full Text
This review discusses the epidemiology and manifestations of BPD and its long-term consequences throughout childhood and into adulthood. Available evidence suggests that disrupted lung development, genetic susceptibility and subsequent environment and infectious events that occur in prenatal and postnatal life likely increase the predisposition of children with BPD to develop early onset chronic obstructive pulmonary disease (COPD).

Nuorti JP, Whitney CG.
Prevention of Pneumococcal Disease Among Infants and Children --- Use of 13-Valent Pneumococcal Conjugate Vaccine and 23-Valent Pneumococcal Polysaccharide Vaccine: Recommendations of the Advisory Committee on Immunization Practices (ACIP).
Division of Bacterial Diseases, National Center for Immunization and Respiratory Diseases; (2010) https://www.cdc.gov/mmwr/preview/mmwrhtml/rr5911a1.htm.

Smith VC, Zupancic JA, McCormick MC, Croen LA, Greene J, Escobar GJ, Richardson DK.
Rehospitalization in the first year of life among infants with bronchopulmonary dysplasia.
J Pediatr. 2004;144(6):799-803. PubMed abstract
This study describe rates and identify risk factors for rehospitalization during the first year of life among infants with bronchopulmonary dysplasia (BPD).

Thébaud B, Goss KN, Laughon M, Whitsett JA, Abman SH, Steinhorn RH, Aschner JL, Davis PG, McGrath-Morrow SA, Soll RF, Jobe AH.
Bronchopulmonary dysplasia.
Nat Rev Dis Primers. 2019;5(1):78. PubMed abstract / Full Text

Trittmann JK, Nelin LD, Klebanoff MA.
Bronchopulmonary dysplasia and neurodevelopmental outcome in extremely preterm neonates.
Eur J Pediatr. 2013;172(9):1173-80. PubMed abstract / Full Text
This study of infants born at <27 weeks between 2004-2010 found no increased risk of neurodevelopmental impairment at 18 months for those discharged from the NICU with supplemental oxygen compared to those discharged on room air.

Vom Hove M, Prenzel F, Uhlig HH, Robel-Tillig E.
Pulmonary outcome in former preterm, very low birth weight children with bronchopulmonary dysplasia: a case-control follow-up at school age.
J Pediatr. 2014;164(1):40-45.e4. PubMed abstract
This study found that preterm-born children with a history of BPD are significantly more likely to have lung function abnormalities, such as airway obstruction and respiratory symptoms, at school age compared with preterm-born children without BPD in the era of surfactant use.