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An Overview ofBronchopulmonary DysplasiaThe Good, The Bad and The Ugly       Roy Maynard, M.D.         May 31, 2011       ...
Abby - day 3               2 of 63
Abby - 4 months                  3 of 63
Abby - 1½ years                  4 of 63
Overview• Epidemiology• Pathophysiology• “Old” vs. “New” BPD• Clinical and laboratory• Management• Outcome• Conclusions   ...
Historical Perspectives of BPD  • Non-existent disease until advent of mechanical    ventilation for ill newborns in the 6...
Definition of BPD• Bancalari defined BPD as need for ventilation,  oxygen requirement at 28 days and abnormal CXR.• Shenna...
Definition• ATS proposed correct term is bronchopulmonary  dysplasia (BPD) so as to differentiate BPD from  other causes o...
Epidemiology• Less than 30 weeks gestation (usually 28 weeks or  less).• Birth weight <1250 grams.• Males > females.• The ...
Adjusted CLD at 36 Weeks: 2002                Vermont Oxford                                 Children’s-Minneapolis       ...
5 Stages of Lung Development http://www.cincinnatichildrens.org/research/div/pulmonary-biology/morphogenesis.htm. Accessed...
Pathophysiology• Old BPD pre-surfactant era• New BPD post-surfactant era   •Genetic predisposition (polygenic ?)   •Oxygen...
Pathophysiologyhttp://www.nature.com/jp/journal/v26/n1s/thumbs/7211476f1th.jpg. Accessed on June 7, 2010.                 ...
Stages of “Old” BPD4 stages:   •Acute lung injury   •Oxidative bronchiolitis   •Proliferative bronchiolitis   •Obliterativ...
Histopathology of “Old” BPD  • Altered inflation pattern of atelectasis and    overinflation  • Severe airway epithelial l...
Pathophysiologyhttp://www.cheo.on.ca/en/bpdtellme. Accessed on June 7, 2010.                                              ...
Pattern of Alternating Atelectasis and           Overinflation Old BPDT. Allen Merritt, MD., William H. Northway, Jr., MD....
Histopathology of “New” BPD • Decreased, large and simplified alveoli (alveolar   hypoplasia, decreased acinar complexity)...
Comparison of Normal Lungs and New BPDA. 5-month-old infant born at term.               B. Infant who has BPD, born at    ...
Clinical• Physical exam• Tachypnea• Tachycardia• Increased work of breathing• Retractions• Nasal flaring• Grunting• Crackl...
Laboratory Findings in Infants with BPD     • ABG        • Chronic respiratory acidosis with compensatory          metabol...
Progression of BPD- Patient 1116                             22 of 63
Progression of BPD- Patient 1116                             23 of 63
Progression of BPD- Patient 1116                             24 of 63
Lung Repair and Remodeling  • Severe BPD may be associated with abnormal    vascular remodeling       • Decreased angiogen...
Biochemistry• Alterations in growth factors predispose  developmental arrest of lung in the new BPD   •TGF-beta1        de...
Management• Diet   •Increased energy requirements   •Vitamin A   •120 kcal/kg/day   •NG or GT supplementation   •Swallowin...
Management• Medications   -- Diuretics        • Bronchodilators        • Albuterol/Levoalbuterol   -- Methlyxanthines     ...
Respiratory Pump Function• The ability of the pump to bring about normal gas  exchange is dependent on the balance between...
Optimum Medical Criteria for Home Management of ChronicRespiratory Failure in Infants with Bronchopulmonary Dysplasia     ...
Goals of Home Mechanical Ventilation •   Increase chest wall mobility and lung growth (wean steroids) •   Prevention of re...
Physiologic Changes Promoting Liberation from            Mechanical Ventilation   • Change in muscle fiber cell type compo...
Physiologic Changes Promoting Liberation from            Mechanical Ventilation     • Chest wall          • Increase ossif...
Weaning• No single ventilator mode or strategy has been  shown to be more effective than another in weaning  children from...
Weaning• SIMV and pressure support    •Wean down pressure support first 15-10-8• SIMV    •Wean rate to background (20) the...
Monitoring During the Weaning Process         • Weight gain and feeding habits         • Stamina for play time         • O...
Positive Outcome for BPD                         with Respiratory Failure                    Trach   HomeIncreasing Acuity...
BPDThe Good           38 of 63
Jackson- Day 1                 39 of 63
Progression of BPD- Patient 1115                             40 of 63
Progression of BPD- Patient 1115                             41 of 63
Jackson- 4 months                    42 of 63
Progression of BPD- Patient 1115                             43 of 63
Jackson- 4 months                    44 of 63
Jackson- 18 months                     45 of 63
Progression of BPD- Patient 1115                             46 of 63
Progression of BPD   The Bad                     47 of 63
Cystic Bronchopulmonary Dysplasia                             48 of 63
Cystic Bronchopulmonary Dysplasia                             49 of 63
Cystic Bronchopulmonary Dysplasia                             50 of 63
Cystic Bronchopulmonary Dysplasia                             51 of 63
Causes of Death• Progression of disease• Unexplained arrest• Disconnection• Hemorrhage• Inappropriate weaning• Unrelated i...
Negative Outcome for BPD                             with Respiratory Failure                                             ...
Severe BPDThe Ugly             54 of 63
Severe BPD Pre-Lung Transplant                           55 of 63
Chest X-Ray Post-Lung Transplant                            56 of 63
Chest X-Ray Post-Lung Transplant                            57 of 63
Long-Term Outcome• Severe BPD associated with poorer  neurodevelopmental outcome--males worse than  females• Increased ris...
24-Week Female -BPD/Clinical Asthma- Age 8                                     59 of 63
27-Week Male Twin- S/P Trach, Vent- Age 9                                    60 of 63
22 ½ Week Female- S/P Trach, Vent- Age 7                                    61 of 63
Conclusions• The “new” BPD represents an arrest in lung development.• Long-term pulmonary function is generally low-normal...
Q&AThank you for  attending!                63 of 63
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An Overview of Bronchopumonary Displasia (BPD)

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An Overview of Bronchopumonary Displasia (BPD)

  1. 1. An Overview ofBronchopulmonary DysplasiaThe Good, The Bad and The Ugly Roy Maynard, M.D. May 31, 2011 1 of 63
  2. 2. Abby - day 3 2 of 63
  3. 3. Abby - 4 months 3 of 63
  4. 4. Abby - 1½ years 4 of 63
  5. 5. Overview• Epidemiology• Pathophysiology• “Old” vs. “New” BPD• Clinical and laboratory• Management• Outcome• Conclusions 5 of 63
  6. 6. Historical Perspectives of BPD • Non-existent disease until advent of mechanical ventilation for ill newborns in the 60’s. • First description by Northway in 1967. • Initial report mostly in larger, more mature premature newborns treated with mechanical ventilation. 6 of 63
  7. 7. Definition of BPD• Bancalari defined BPD as need for ventilation, oxygen requirement at 28 days and abnormal CXR.• Shennan proposed need for supplemental oxygen at 36 weeks corrected gestational age.• Walsh (et al.) developed physiologic definition: 35–37 weeks, treated with mechanical ventilation, CPAP or supplemental oxygen needing 30% oxygen to keep sats 90%–96%. 7 of 63
  8. 8. Definition• ATS proposed correct term is bronchopulmonary dysplasia (BPD) so as to differentiate BPD from other causes of chronic respiratory disorders in infants.• Chronic lung disease of prematurity is another term commonly used. 8 of 63
  9. 9. Epidemiology• Less than 30 weeks gestation (usually 28 weeks or less).• Birth weight <1250 grams.• Males > females.• The lower the gestational age, the greater the risk.• Due to increased survival in VLBW babies, prevalence of BPD is increased.• Severity of the new BPD is less than the old BPD. 9 of 63
  10. 10. Adjusted CLD at 36 Weeks: 2002 Vermont Oxford Children’s-Minneapolis 80 70 69 60 64% Occurrence 50 44 40 30 32 30 28 20 22 18 10 11 8 0 501-750g 751-1000g 1001-1250g 1251-1500g 501-1500g 10 of 63
  11. 11. 5 Stages of Lung Development http://www.cincinnatichildrens.org/research/div/pulmonary-biology/morphogenesis.htm. Accessed on June 7, 2010. 11 of 63
  12. 12. Pathophysiology• Old BPD pre-surfactant era• New BPD post-surfactant era •Genetic predisposition (polygenic ?) •Oxygen toxicity •Pulmonary inflammation/chemical mediators •Barotrauma vs volutrauma •Infection/chorioamnionitis/preeclampsia •Stage of lung growth •Alveolar simplification 12 of 63
  13. 13. Pathophysiologyhttp://www.nature.com/jp/journal/v26/n1s/thumbs/7211476f1th.jpg. Accessed on June 7, 2010. 13 of 63
  14. 14. Stages of “Old” BPD4 stages: •Acute lung injury •Oxidative bronchiolitis •Proliferative bronchiolitis •Obliterative fibroproliferative bronchiolitis 14 of 63
  15. 15. Histopathology of “Old” BPD • Altered inflation pattern of atelectasis and overinflation • Severe airway epithelial lesions (hyperplasia, squamous metaplasia) • Airway smooth muscle hyperplasia • Prominent vascular hypertensive lesions • Decreased internal surface area and alveoli 15 of 63
  16. 16. Pathophysiologyhttp://www.cheo.on.ca/en/bpdtellme. Accessed on June 7, 2010. 16 of 63
  17. 17. Pattern of Alternating Atelectasis and Overinflation Old BPDT. Allen Merritt, MD., William H. Northway, Jr., MD., Bruce R. Boynton, MD. Contemporary Issues in Fetal andNeonatal Medicine. 4 Bronchopulmonary Dysplasia. Boston: Blackwell Scientific Publications; 1988:165. 17 of 63
  18. 18. Histopathology of “New” BPD • Decreased, large and simplified alveoli (alveolar hypoplasia, decreased acinar complexity) • Decreased, dysmorphic capillaries • Variable interstitial fibroproliferation • Less severe arterial/arteriolar vascular lesions • Negligible airway epithelial lesions • Variable airway smooth muscle hyperplasia 18 of 63
  19. 19. Comparison of Normal Lungs and New BPDA. 5-month-old infant born at term. B. Infant who has BPD, born at 28 weeks’ gestation, lung biopsy at 8 months.Jobe, A. NeoReviews Vol.7 No.10 2006 e531 2006. 19 of 63
  20. 20. Clinical• Physical exam• Tachypnea• Tachycardia• Increased work of breathing• Retractions• Nasal flaring• Grunting• Crackles 20 of 63
  21. 21. Laboratory Findings in Infants with BPD • ABG • Chronic respiratory acidosis with compensatory metabolic alkalosis, hypoxia • CXR • Normal-to-increased lung volumes • Bilateral interstitial changes of a variable nature • Pulmonary function • Decreased lung/respiratory compliance • Increased airway resistance/obstruction • Airway hyper responsiveness/asthma • Ventilation/perfusion abnormalities • Cardiac • Pulmonary hypertension • Silent ASD • Aorta-pulmonary collaterals 21 of 63
  22. 22. Progression of BPD- Patient 1116 22 of 63
  23. 23. Progression of BPD- Patient 1116 23 of 63
  24. 24. Progression of BPD- Patient 1116 24 of 63
  25. 25. Lung Repair and Remodeling • Severe BPD may be associated with abnormal vascular remodeling • Decreased angiogenesis • Vascular reactivity • Narrowing of blood vessel diameter • Pulmonary hypertension 25 of 63
  26. 26. Biochemistry• Alterations in growth factors predispose developmental arrest of lung in the new BPD •TGF-beta1 decreased •VEGF decreased •TGF-alpha decreased •PDGF decreased 26 of 63
  27. 27. Management• Diet •Increased energy requirements •Vitamin A •120 kcal/kg/day •NG or GT supplementation •Swallowing dysfunction (vocal cord paralysis following PDA ligation)• Immunizations •RSV (synagis, H1N1, influenza) 27 of 63
  28. 28. Management• Medications -- Diuretics • Bronchodilators • Albuterol/Levoalbuterol -- Methlyxanthines • Corticosteroids • Dexmethasone • Prednisolone • Budesonide• Oxygen 28 of 63
  29. 29. Respiratory Pump Function• The ability of the pump to bring about normal gas exchange is dependent on the balance between the mechanical load placed on the pump and the intrinsic function of the respiratory muscles.• Respiratory muscle fatigue is a state that develops when respiratory muscles are unable to maintain the targeted force output on repeated contractions and is reversible with rest. 29 of 63
  30. 30. Optimum Medical Criteria for Home Management of ChronicRespiratory Failure in Infants with Bronchopulmonary Dysplasia • Clinical •Positive trend on growth curve (weight) •Stamina for periods of play •Extended period of stability • Physiologic •30% or less oxygen •pCO2 less than 50 torr •IMV of 30 or less •Stable airway, mature fistula 30 of 63
  31. 31. Goals of Home Mechanical Ventilation • Increase chest wall mobility and lung growth (wean steroids) • Prevention of respiratory failure (neuromuscular disorders) • Prevent atelectasis/prevent overinflation • Provide respiratory muscle rest • Minimize iatrogenic lung injury • Prevent respiratory muscle atrophy • Liberation from mechanical ventilation • Safe environment • Family bonding and nurturing • Promote normal growth and development • Reduce costs 31 of 63
  32. 32. Physiologic Changes Promoting Liberation from Mechanical Ventilation • Change in muscle fiber cell type composition of the diaphragm (type II cells to type I cells ) • Lung growth • Increased compliance • Decreased airway resistance • Increased alveoli—increased surface area for gas exchange; improve V/Q mismatch, decrease oxygen needs and decrease pCO2 32 of 63
  33. 33. Physiologic Changes Promoting Liberation from Mechanical Ventilation • Chest wall • Increase ossification—more rigid, less compliant, stabilize lung volumes • Dynamic FRC—changes to static FRC • Increased stability of small and large airways • Increase respiratory muscle pump function 33 of 63
  34. 34. Weaning• No single ventilator mode or strategy has been shown to be more effective than another in weaning children from mechanical ventilation.• The most common strategy used is called sprinting or diaphragm training. The diaphragm is the most important respiratory muscle.• Dependent on underlying diagnosis.• Complicating factors may stall weaning. 34 of 63
  35. 35. Weaning• SIMV and pressure support •Wean down pressure support first 15-10-8• SIMV •Wean rate to background (20) then CPAP trials during the day or periods of decreased IMV, allow muscles to rest at night• Nocturnal ventilation •Increase nose filter trials or trach collar time during the day with gradual reduction in nocturnal IMV to CPAP• Trach collar and nose filter combination 24 hrs/day for 3 months, then airway evaluation by ENT for decannulation• Oxygen requirement is not a limiting factor 35 of 63
  36. 36. Monitoring During the Weaning Process • Weight gain and feeding habits • Stamina for play time • Oxygen needs • End-tidal CO2 • Developmental progress • Work of breathing • Pulmonary hypertension 36 of 63
  37. 37. Positive Outcome for BPD with Respiratory Failure Trach HomeIncreasing Acuity • • Off vent • • 4 8 12 16 20 24 Time (months) Decannulation 37 of 63
  38. 38. BPDThe Good 38 of 63
  39. 39. Jackson- Day 1 39 of 63
  40. 40. Progression of BPD- Patient 1115 40 of 63
  41. 41. Progression of BPD- Patient 1115 41 of 63
  42. 42. Jackson- 4 months 42 of 63
  43. 43. Progression of BPD- Patient 1115 43 of 63
  44. 44. Jackson- 4 months 44 of 63
  45. 45. Jackson- 18 months 45 of 63
  46. 46. Progression of BPD- Patient 1115 46 of 63
  47. 47. Progression of BPD The Bad 47 of 63
  48. 48. Cystic Bronchopulmonary Dysplasia 48 of 63
  49. 49. Cystic Bronchopulmonary Dysplasia 49 of 63
  50. 50. Cystic Bronchopulmonary Dysplasia 50 of 63
  51. 51. Cystic Bronchopulmonary Dysplasia 51 of 63
  52. 52. Causes of Death• Progression of disease• Unexplained arrest• Disconnection• Hemorrhage• Inappropriate weaning• Unrelated illness• Abdominal catastrophe/sepsis• Sudden airway compromise 52 of 63
  53. 53. Negative Outcome for BPD with Respiratory Failure • DeathIncreasing Acuity Trach • Lung Transplant • 4 8 12 16 20 24 Time (months) 53 of 63
  54. 54. Severe BPDThe Ugly 54 of 63
  55. 55. Severe BPD Pre-Lung Transplant 55 of 63
  56. 56. Chest X-Ray Post-Lung Transplant 56 of 63
  57. 57. Chest X-Ray Post-Lung Transplant 57 of 63
  58. 58. Long-Term Outcome• Severe BPD associated with poorer neurodevelopmental outcome--males worse than females• Increased risk for abnormal PFT but many not symptomatic (obstructive lung disease)• Increased risk for asthma• Increased incidence of abnormal chest CT abnormalities• Increased risk for COPD as adults? 58 of 63
  59. 59. 24-Week Female -BPD/Clinical Asthma- Age 8 59 of 63
  60. 60. 27-Week Male Twin- S/P Trach, Vent- Age 9 60 of 63
  61. 61. 22 ½ Week Female- S/P Trach, Vent- Age 7 61 of 63
  62. 62. Conclusions• The “new” BPD represents an arrest in lung development.• Long-term pulmonary function is generally low-normal or mildly abnormal for majority of the “new BPD.”• Post chronic respiratory failure patients are more likely to have obstructive pulmonary disease.• At risk for long-term respiratory morbidity, hospitalization, asthma and COPD; recent study suggests 2.4-fold increase risk for asthma.• Neurodevelopmental outcome correlates with severity of lung disease, clearly an increase in ADHD (increase 60%) and autism.• Uncommon disease among infants with birth weights >1500 grams. 62 of 63
  63. 63. Q&AThank you for attending! 63 of 63

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