Bronchopulmonary Dysplasia (BPD)
Summary
Bronchopulmonary Dysplasia (BPD), also known as Chronic Lung Disease of Prematurity (CLD), is a chronic lung condition affecting preterm infants, particularly those born at extremely low gestational ages (<28 weeks) or extremely low birth weights (<1000g). It is characterised by arrested lung development and persistent oxygen requirement beyond 28 days of life or at 36 weeks postmenstrual age (PMA). BPD is a consequence of the immature lung's response to injury from mechanical ventilation (volutrauma/barotrauma), oxygen toxicity, and inflammation, often on a background of infection (chorioamnionitis) and inadequate nutrition. Prevention strategies (antenatal steroids, surfactant, gentle ventilation, caffeine) have reduced severe BPD, but the condition remains a major cause of morbidity in survivors of extreme prematurity. Long-term management includes respiratory support, prevention of RSV infection (Palivizumab), nutrition optimisation, and follow-up for pulmonary hypertension and neurodevelopmental outcomes.
Key Facts
- Definition (NICHD): Need for supplemental oxygen for ≥28 days. Severity assessed at 36 weeks PMA.
- Risk Factors: Extreme prematurity (<28 weeks), Low birth weight, Mechanical ventilation, High FiO2, Chorioamnionitis, PDA, Sepsis.
- Pathophysiology: Arrested alveolar and vascular development. Inflammation. Fibrosis (in severe cases).
- Prevention: Antenatal Corticosteroids, Surfactant, Caffeine, Gentle Ventilation (CPAP, Volume-Targeting), Vitamin A.
- Management: Respiratory support (Home O2), Diuretics, Postnatal Corticosteroids (Carefully), Nutrition, RSV Prophylaxis.
- Prognosis: Many improve by age 2-3. Some have persistent airway disease. Risk of Pulmonary Hypertension.
Clinical Pearls
"Old BPD vs New BPD": "Old BPD" (Northway, 1967) described severe lung injury with fibrosis in larger preterm infants on high ventilator settings. "New BPD" (post-Surfactant era) is primarily arrest of lung development in extremely preterm infants, with fewer fibrotic changes.
"36 Weeks PMA is the Key Timepoint": Severity of BPD is assessed at 36 weeks corrected gestational age based on oxygen requirement.
"Caffeine Saves Lungs": Caffeine citrate reduces the risk of BPD (CAP Trial). It is a key preventive intervention for very preterm infants.
"RSV is the Enemy": Infants with BPD are at very high risk of severe RSV bronchiolitis. Palivizumab (Synagis) prophylaxis is critical during RSV season.
Why This Matters Clinically
BPD is one of the most common serious complications of prematurity, affecting long-term respiratory health, growth, and neurodevelopment. Prevention is key. Understanding the pathophysiology and evidence-based management (gentle ventilation, avoiding O2 toxicity, caffeine, nutrition) can reduce the burden of this disease.
Incidence
- Very Preterm (<32 weeks): ~25-40% develop BPD.
- Extremely Preterm (<28 weeks): ~50-80% develop BPD.
- VLBW (<1500g): ~20-30%.
- ELBW (<1000g): ~40-70%.
Risk Factors
| Factor | Mechanism |
|---|---|
| Gestational Age | Most important. Lower GA = Higher risk. |
| Birth Weight | ELBW (<1000g) highest risk. |
| Mechanical Ventilation | Volutrauma, Barotrauma. |
| Oxygen Exposure | Oxidative stress. Free radical damage. |
| Chorioamnionitis | Prenatal inflammation. |
| Postnatal Sepsis | Systemic inflammation. |
| Patent Ductus Arteriosus (PDA) | Pulmonary oedema. |
| Male Sex | Higher risk. |
| White Ethnicity | Higher risk than Black ethnicity (Controversial). |
| Postnatal Growth Failure | Poor nutrition impairs lung growth. |
The Developing Lung
| Stage | Gestational Age | Key Feature |
|---|---|---|
| Canalicular | 16-26 weeks | Formation of primitive alveolar ducts and surfactant-producing cells. |
| Saccular | 24-38 weeks | Air sacs (saccules) form. Thinning of air-blood barrier. |
| Alveolar | 36 weeks - 2 years | True alveoli form. Alveolar septation. |
Extremely preterm infants are born during the Canalicular/Early Saccular phase – before alveoli have formed.
"Old BPD" vs "New BPD"
| Feature | Old BPD (Northway 1967) | New BPD (Post-Surfactant Era) |
|---|---|---|
| Population | Larger preterm (30-34 weeks). | Extremely preterm (<28 weeks). |
| Primary Injury | Severe ventilator trauma + O2 toxicity. | Arrested lung development. |
| Pathology | Fibrosis, Airway smooth muscle hypertrophy. | Simplified alveoli (Fewer, larger). Dysmorphic vasculature. |
| X-ray | Cystic changes, Fibrotic stranding. | Hazy lungs, Hyperinflation. |
Pathogenic Mechanisms
| Mechanism | Effect |
|---|---|
| Inflammation | Chorioamnionitis, Sepsis, Ventilator-induced. Cytokine release. |
| Oxidative Stress | Immature antioxidant defences. Free radical damage from O2. |
| Volutrauma | Overdistension of immature alveoli. |
| Barotrauma | High pressures. Less common with modern ventilation. |
| Impaired Alveolarization | Fewer, larger alveoli. Reduced gas exchange surface. |
| Vascular Maldevelopment | Reduced pulmonary vascular bed. Risk of Pulmonary Hypertension. |
Acute Phase (NICU)
| Feature | Notes |
|---|---|
| Respiratory Distress | Tachypnoea, Retractions, Grunting (Initially from RDS). |
| Oxygen Requirement | Persistent need for supplemental O2. |
| Ventilator Dependence | Difficulty weaning from respiratory support. |
| Apneas / Bradycardias | Common in preterm. May worsen with BPD. |
Chronic Phase (Post-Discharge)
| Feature | Notes |
|---|---|
| Chronic Oxygen Requirement | May need Home Oxygen for months-years. |
| Tachypnoea at Rest | Increased work of breathing. |
| Poor Feeding / Growth Failure | High caloric expenditure. Feeding difficulties. |
| Recurrent Respiratory Infections | Increased susceptibility. RSV is particularly dangerous. |
| Wheezing | "Asthma-like" symptoms. May persist into childhood. |
| Exercise Intolerance | In older children/adolescents. |
NICHD 2001 Definition
The standard diagnostic criteria.
- Diagnosis: Supplemental oxygen requirement for ≥28 cumulative days.
- Severity Assessment: At 36 weeks PMA (or discharge if earlier) for infants <32 weeks GA. At 56 days postnatal age for infants ≥32 weeks GA.
Severity Grading (at 36 weeks PMA)
| Severity | Oxygen Requirement |
|---|---|
| Mild BPD | Breathing room air. |
| Moderate BPD | Oxygen <30% FiO2. |
| Severe BPD | Oxygen ≥30% FiO2 OR Positive Pressure Ventilation (PPV/CPAP). |
Investigations
| Investigation | Purpose |
|---|---|
| CXR | Hyperinflation, Hazy opacities, Cystic changes (Severe). |
| Oxygen Saturation Monitoring | Target SpO2 91-95%. |
| Echocardiogram | Screen for Pulmonary Hypertension. |
| Blood Gases | Assess oxygenation, Ventilation. |
| Growth Monitoring | Weight gain critical. |
Evidence-Based Prevention Strategies
| Intervention | Mechanism | Evidence |
|---|---|---|
| Antenatal Corticosteroids | Accelerates lung maturation. Increases surfactant. | Strong (Cochrane). NNT ~10 for BPD. |
| Surfactant Therapy | Reduces RDS severity. Allows gentler ventilation. | Strong. |
| Caffeine Citrate | Reduces apnoea. Allows earlier extubation. Anti-inflammatory. | Strong (CAP Trial). Reduces BPD/Death. |
| Gentle Ventilation / CPAP | Avoid volutrauma. Early CPAP instead of intubation. | Strong (SUPPORT, COIN trials). |
| Volume-Targeted Ventilation | Reduces volutrauma. | Moderate. |
| Judicious Oxygen Use | Avoid hyperoxia (Free radicals). Target SpO2 91-95%. | Strong (NeOProM Meta-analysis). |
| Vitamin A | Supports epithelial repair. | Moderate (Reduces BPD in ELBW). IM injections needed. |
| Avoiding Overhydration / PDA | Reduce pulmonary oedema. | Moderate. |
| Infection Control | Prevent sepsis/chorioamnionitis. | Indirect evidence. |
Principles
- Respiratory Support: Provide adequate oxygenation without toxicity.
- Nutrition: Optimise growth. High caloric intake.
- Prevent Complications: RSV prophylaxis. Screen for Pulmonary Hypertension.
- Developmental Care: Minimise NICU morbidity.
Respiratory Support
| Modality | Notes |
|---|---|
| Supplemental Oxygen | Target SpO2 91-95%. Avoid hyperoxia. |
| CPAP / High Flow | First-line non-invasive support. |
| Mechanical Ventilation | If needed: Volume-targeted, Gentle settings. |
| Home Oxygen | Many infants require O2 after discharge. Wean as lung growth allows. |
Pharmacological Management
| Drug | Dose / Route | Indication | Notes |
|---|---|---|---|
| Diuretics (Spironolactone + Chlorothiazide) | PO | Pulmonary oedema. Improve lung compliance. | Common. Monitor electrolytes. |
| Furosemide | PO / IV | Acute fluid overload. | Avoid long-term (Ototoxicity, Nephrocalcinosis). |
| Postnatal Corticosteroids (Dexamethasone) | IV | Severe BPD, Ventilator-dependent beyond 7 days. | DART Regimen: Low-dose, short course. Reduces BPD but risks neurodevelopmental harm at high doses. |
| Inhaled Corticosteroids (Budesonide) | Inhaled / via ETT | Evolving evidence. May reduce BPD (NEUROSIS trial). | Used variably. |
Nutrition
| Principle | Notes |
|---|---|
| High Caloric Intake | 120-150 kcal/kg/day. |
| Concentrated Feeds | May need fortified breast milk or concentrated formula. |
| Monitor Growth | Weekly weights. Head circumference. Length. |
| Avoid Fluid Overload | High-calorie, low-volume feeds. |
RSV Prophylaxis (Palivizumab – Synagis)
| Criteria for Palivizumab (UK Example) | Notes |
|---|---|
| Born <35 weeks AND <6 months at RSV season start | With additional risk factors (CLD, CHD, Immunodeficiency). |
| BPD with ongoing treatment (O2, Steroids, Diuretics) | Within last 6 months. |
| Dosing | 15 mg/kg IM monthly during RSV season (Nov-Mar in UK). |
Pulmonary Complications
| Complication | Notes |
|---|---|
| Pulmonary Hypertension | Develops in 10-25% of severe BPD. Screen with Echo. May need Sildenafil. |
| Recurrent Respiratory Infections | Especially RSV bronchiolitis. |
| Airways Disease | Wheezing, Asthma-like symptoms. Bronchial hyperreactivity. |
| Chronic Respiratory Symptoms | May persist into adolescence/adulthood. |
Non-Pulmonary Complications
| Complication | Notes |
|---|---|
| Neurodevelopmental Impairment | Increased risk (Cerebral palsy, Cognitive delay). Related to prematurity + BPD. |
| Growth Failure | Caloric deficit. Chronic illness effect. |
| Feeding Difficulties | Oral aversion. Gastro-oesophageal reflux. |
| Retinopathy of Prematurity (ROP) | Separate complication of prematurity. Oxygen-related. |
Prognosis
| Outcome | Notes |
|---|---|
| Mortality (Severe BPD) | ~10-30% in first year. Often from respiratory or PH complications. |
| Improvement with Age | Lung growth continues for years. Many improve by age 2-3. |
| Adult Outcomes | Abnormal PFTs common. Reduced exercise capacity. COPD-like phenotype. |
Prognostic Factors
| Factor | Association |
|---|---|
| Gestational Age | Lower GA = Worse prognosis. |
| BPD Severity | Severe BPD = Higher mortality, Worse neurodevelopment. |
| Pulmonary Hypertension | Significantly increases mortality and morbidity. |
| Postnatal Growth | Poor growth = Worse outcomes. Good growth = Better lung recovery. |
| Socioeconomic Status | Lower SES = Higher rehospitalisation, Worse outcomes. |
| Smoking Exposure | Second-hand smoke worsens respiratory outcomes. |
Adult Respiratory Outcomes (Long-Term Follow-Up Studies)
| Finding | Prevalence |
|---|---|
| Abnormal Spirometry | 50-70% of BPD survivors have airflow obstruction. |
| Reduced Exercise Capacity | 40-60%. |
| Asthma Symptoms | 30-40%. Often labelled "asthma" in childhood. |
| Emphysema / Air Trapping on CT | Common in severe BPD. |
| Accelerated Lung Function Decline | COPD-like trajectory possible. |
Key Exam Tips (For Exams)
| Topic | High-Yield Point |
|---|---|
| Definition | O2 for ≥28 days. Severity at 36 weeks PMA. |
| Prevention | Antenatal steroids, Surfactant, Caffeine, CPAP, SpO2 91-95%. |
| Caffeine | CAP Trial: Reduces BPD/Death. Start early. |
| Steroids | DART Trial: Low-dose Dexamethasone. Controversial due to neurodevelopmental risk. |
| Old vs New BPD | Old = Fibrosis. New = Arrested alveolar development. |
| RSV Prevention | Palivizumab (Synagis) monthly during RSV season. |
Post-Discharge Surveillance
| Clinic | Purpose |
|---|---|
| Neonatal Follow-Up | Growth, Development, Respiratory status. |
| Respiratory Paediatrics | BPD clinic. Wean O2. Manage airways disease. |
| Cardiology | If Pulmonary Hypertension. Echo follow-up. |
| Developmental Clinic | Neurodevelopmental assessment. Early intervention. |
| Ophthalmology | ROP screening (If applicable). |
| Dietitian | Optimise nutrition. |
Home Oxygen Weaning
| Principle | Notes |
|---|---|
| Criteria for Weaning | Stable SpO2 in room air during sleep, feeds, and activity. |
| Oximetry Studies | Often done prior to weaning. |
| Typical Timeframe | Months to 1-2 years. Varies. |
Key Guidelines
| Guideline | Organisation | Notes |
|---|---|---|
| AAP Policy on BPD | American Academy of Pediatrics | Prevention and Management. |
| BAPM Framework | British Association of Perinatal Medicine | Oxygen saturation targeting. |
| European Consensus | European Society of Paediatric Research | Comprehensive evidence review. |
Landmark Trials
| Trial | Finding |
|---|---|
| CAP Trial (2006) | Caffeine reduces BPD/Death and improves neurodevelopmental outcome. |
| SUPPORT / COIN Trials | Early CPAP vs Intubation. CPAP non-inferior, less lung injury. |
| DART (2007) | Low-dose Dexamethasone (0.89 mg/kg over 10 days) reduces BPD with acceptable safety. |
| NeOProM Meta-analysis | Lower O2 targets (91-95% vs 95-99%) reduce ROP and BPD but slightly increase mortality. |
Scenario 1:
- Stem: A 25-week gestation infant is now 36 weeks PMA and requires 28% FiO2. What is the diagnosis and severity?
- Answer: Bronchopulmonary Dysplasia (BPD). Severity: Moderate (Requires O2 <30% at 36 weeks PMA).
Scenario 2:
- Stem: What interventions prevent BPD in extremely preterm infants?
- Answer: Antenatal Corticosteroids, Surfactant, Caffeine Citrate, Early CPAP (Avoid intubation if possible), Volume-Targeted Ventilation, Judicious Oxygen Use (Target SpO2 91-95%), Vitamin A.
Scenario 3:
- Stem: Why is Caffeine important in BPD prevention?
- Answer: Caffeine Citrate (CAP Trial) reduces apnoea, allows earlier extubation, has anti-inflammatory effects, and significantly reduces the combined outcome of BPD or Death.
Scenario 4:
- Stem: A mother asks why her baby with BPD is being given monthly injections. Explain.
- Answer: Your baby is receiving Palivizumab (Synagis). It's an antibody that protects against RSV – a virus that causes bronchiolitis. Babies with BPD are at very high risk of severe RSV infection, so we give this monthly during winter to prevent it.
Scenario 5:
- Stem: What is the difference between "Old BPD" and "New BPD"?
- Answer: Old BPD (Northway 1967) was seen in larger preterm infants with severe ventilator-induced lung injury and fibrosis. New BPD affects extremely preterm infants (<28 weeks) and is characterised by arrested alveolar development (Simplified, larger alveoli) with less fibrosis, due to the use of Surfactant and gentler ventilation.
| Scenario | Urgency | Action |
|---|---|---|
| Preterm infant with evolving BPD | Routine | Neonatal team management. |
| Severe BPD, Ventilator dependent | NICU | Ongoing specialist care. |
| Home O2 infant with acute respiratory illness | Urgent/Emergency | A&E / Paediatric assessment. |
| Suspected Pulmonary Hypertension | Urgent | Echo. Paediatric Cardiology. |
| Poor growth despite feeds | Routine/Urgent | Dietitian. Consider NG/Gastrostomy. |
What is BPD?
Bronchopulmonary Dysplasia (BPD), also called Chronic Lung Disease of Prematurity, is a lung condition that affects babies born very early. Their lungs are not fully developed when they are born, and the treatments used to help them breathe (like oxygen and breathing machines) can sometimes cause damage or slow down lung growth.
Why did my baby get BPD?
Babies born very early (before 28 weeks) have immature lungs. The lungs need oxygen and sometimes a breathing machine to survive, but these can cause inflammation. Your baby's lungs are growing, but they need time and support to catch up.
What is the treatment?
- Oxygen: Some babies need extra oxygen at home until their lungs grow stronger.
- Medicines: Diuretics (water pills) can help with fluid in the lungs. Sometimes steroids are used.
- RSV Protection: A monthly injection during winter protects against a virus that can be very dangerous for babies with BPD.
- Good Nutrition: Calories help your baby grow, and growing helps the lungs heal.
Will my baby get better?
Most babies with BPD improve as they grow. The lungs continue to develop for the first few years of life. Some children have ongoing breathing problems like wheezing, but many do very well.
Key Counselling Points
- Lung Growth Takes Time: "Your baby's lungs will continue to grow and heal over the first 2-3 years."
- RSV is Dangerous: "Please keep your baby away from people with colds. The monthly injection protects against the most dangerous virus."
- Avoid Smoke: "Second-hand smoke makes lung problems much worse."
- Nutrition is Key: "Good feeding and weight gain help the lungs grow."
- Follow-Up is Crucial: "Regular check-ups help us wean oxygen and catch any problems early."
Discharge Criteria for Home Oxygen
When can infants go home on oxygen?
| Criterion | Requirement |
|---|---|
| Stable Clinical Status | No acute illness. Tolerating feeds. |
| Stable Oxygen Requirement | Consistent FiO2 for >8-72 hours. |
| Appropriate Weight Gain | Growing appropriately. |
| Parents Trained | Competent with O2 equipment, Feeding, Resuscitation. |
| Equipment in Place | O2 supply, Pulse oximeter, Emergency plan. |
| Follow-Up Arranged | Paediatric respiratory / Neonatal follow-up booked. |
| Palivizumab Started | If during RSV season. |
Avoiding Common Pitfalls
| Pitfall | Consequence | Prevention |
|---|---|---|
| Aggressive Ventilation | Volutrauma, Worse BPD. | Volume-targeted, Low pressures, Early CPAP. |
| High Oxygen Targets | Oxidative stress, ROP, BPD. | Target SpO2 91-95% (Not 95-99%). |
| Delayed Caffeine | More apnoeas, Delayed extubation. | Start Caffeine early (<24 hours in <30 weeks). |
| Fluid Overload | Pulmonary oedema, Worsens lung function. | Careful fluid management. Diuretics if needed. |
| Inadequate Nutrition | Impaired lung growth. | Aggressive caloric supplementation. |
| Not Screening for PH | Missed Pulmonary Hypertension. | Echo in severe BPD. |
Oxygen Saturation Targeting: The Debate
Summary of major trials.
| Trial | Target Ranges | Key Finding |
|---|---|---|
| SUPPORT | 85-89% vs 91-95% | Lower target reduced ROP but increased mortality. |
| BOOST II | 85-89% vs 91-95% | Similar findings. |
| COT | 85-89% vs 91-95% | Lower target associated with increased mortality. |
| NeOProM Meta-analysis | Lower vs Higher | Lower targets: Less ROP, Less BPD, BUT ~1% higher mortality. |
Current Practice: Most units target 91-95% (Balance between avoiding oxygen toxicity and preventing mortality).
Parents' Questions Answered
| Question | Answer |
|---|---|
| "Why does my baby need oxygen at home?" | Your baby's lungs are still developing. The oxygen supports them while they grow. |
| "How long will my baby be on oxygen?" | It varies – usually months to 1-2 years. We'll wean as the lungs mature. |
| "Is RSV really that dangerous?" | Yes. Babies with BPD can get very sick from RSV. The monthly injection protects against it. |
| "Will my baby have asthma?" | Some children with BPD have wheeze or asthma-like symptoms. Not all do. |
| "Can I take my baby outside?" | Yes, but avoid crowded places and sick people, especially during winter. |
Special Populations
| Population | Considerations |
|---|---|
| Infants Requiring Long-Term Ventilation | Tracheostomy may be needed. Home ventilation programs. |
| Infants with Pulmonary Hypertension | Sildenafil, Close Cardiology follow-up. Poor prognosis if severe. |
| Infants with Severe Growth Failure | May need NG / Gastrostomy feeding. Dietitian input. |
| Global Developmental Delay | Early intervention services. Developmental paediatrician. |
| Standard | Target |
|---|---|
| Antenatal steroids given where indicated | >0% |
| Caffeine started within 24 hours in <30 weeks | >5% |
| Palivizumab offered to eligible infants | 100% |
| Echo for Pulmonary Hypertension in severe BPD | 100% |
| Discharged infants on oxygen have follow-up plan | 100% |
- William Northway (1967): First described BPD in a landmark paper.
- Original Case Series: 32 infants with RDS who survived with O2 and mechanical ventilation.
- Findings: Severe lung injury: Fibrosis, Airway smooth muscle hypertrophy, Emphysematous changes.
- Terminology: Named "Bronchopulmonary Dysplasia" – dysplasia = abnormal growth.
- Modern Era: The condition we see today ("New BPD") is different – milder histology but affects more vulnerable, extremely preterm infants.
- Jobe AH, Bancalari E. Bronchopulmonary dysplasia. Am J Respir Crit Care Med. 2001. PMID: 11401865 (NICHD Definition)
- Schmidt B, et al. (CAP Trial). Caffeine for Apnea of Prematurity. N Engl J Med. 2006. PMID: 16707748
- Doyle LW, et al. (DART). Low-dose Dexamethasone facilitates extubation. Pediatrics. 2006. PMID: 16651286
- Northway WH, et al. Pulmonary disease following respirator therapy of hyaline-membrane disease. N Engl J Med. 1967. PMID: 6017773
Medical Disclaimer: MedVellum content is for educational purposes and clinical reference. If your baby has breathing difficulties, please consult your medical team.