II. Definition

  1. Chronic Lung Disease in Infants and Children
  2. Occurs secondary to Hyaline Membrane Disease associated with Prematurity

III. Epidemiology

  1. Incidence increases with earlier Gestational ages
  2. Incidence increases with lower birth weights
    1. Incidence: Affects 50-80% of infants with birth weight <900 grams
    2. Incidence has dramatically decreased with the regular use of surfactant in Premature Infants

IV. Pathophysiology

  1. Chronic lung inflammation and scarring related to prolonged Mechanical Ventilation in Premature Infants
  2. Reults in lung remodeling

V. Risk Factors

  1. Very low birth weight
  2. Meconium Aspiration
  3. Congenital Heart Disease (e.g. Patent Ductus Arteriosus)
  4. Perinatal infections (e.g. TORCH Viruses)
  5. Persistent Pulmonary Hypertension
  6. Significant levels of Ventilatory support

VI. Signs

  1. Tachypnea
  2. Wheezing
  3. Increased work of breathing
  4. Harrison Groove
    1. Thoracic depression at the lower aspect of the chest (at diaphragm insertion)
    2. Results in pear-shaped chest appearance

VII. Diagnosis

  1. General BPD Criteria (all 3 required)
    1. Mechanical Ventilation in the neonatal period
    2. Continued oxygen needs (FIO2 >21%) at age 28 days
    3. Respiratory insufficiency
  2. Newborns born at <32 weeks gestation
    1. Mild
      1. Breathing room air at 36 weeks Postmenstrual age OR by time of discharge
    2. Moderate
      1. Oxygen requirements <30% FIO2 at 36 weeks Postmenstrual age OR by time of discharge
    3. Severe
      1. Requires >30% FIO2 or PPV/N-CPAP at 36 weeks Postmenstrual age OR by time of discharge
  3. Newborns born at >=32 weeks gestation
    1. Mild
      1. Breathing room air at 56 days Postnatal age OR by time of discharge
    2. Moderate
      1. Oxygen requirements <30% FIO2 at 56 days Postnatal age OR by time of discharge
    3. Severe
      1. Requires >30% FIO2 or PPV/N-CPAP at 56 days Postnatal age OR by time of discharge
  4. References
    1. Jobe (2001) Am J Respir Crit Care Med 163(7):1723-9 +PMID:11401896 [PubMed]

VIII. Imaging: Chest XRay

  1. Stage 1 (Days 1 to 3)
    1. Findings similar to respiratory distress syndrome of the newborn
  2. Stage 2 (Days 4 to 10)
    1. Radiopacity markedly increased
  3. Stage 3 (Days 10-20)
    1. Cystic pattern
  4. Stage 4 (Days >28)
    1. Hyperexpansion
    2. Cardiomegaly may be present
    3. Emphysematous areas

IX. Management: Exacerbations and general concerns

  1. Treat as Asthma Exacerbation initially
    1. See Pediatric Asthma
    2. Recognize that some obstruction may be fixed and unresponsive to further bronchodilation
      1. Trial Bronchodilators but do not over-use if poor response
    3. Supportive care may be mainstay of therapy (oxygen, IV hydration)
  2. Oxygen
    1. Keep Oxygen Saturation at 92% or higher
    2. Home oxygen may be required
  3. Inhaled agents
    1. Albuterol
    2. Ipratropium Bromide
    3. Inhaled Corticosteroids (e.g. Budesonide or Fluticasone)
  4. Adjunctive agents
    1. Diuretic indications
      1. Consider in sudden weight gain
    2. Antibiotics indications
      1. Immunodeficiency
      2. Recurrent respiratory infections
      3. Suspected Bacterial illness
  5. Car Seats may need adjustment (restraint may exacerbate symptoms)
    1. Require Car Seat test before NICU discharge
    2. Special prone or supine car safety device may be indicated

X. Management: Hospital admission indications

  1. Respiratory Rate >70-80 per minute (or significant increase from baseline)
  2. Hypoxia with increased oxygen requirement (or hypercarbia)
  3. Poor feeding secondary to respiratory condition
  4. Apnea
  5. New Pulmonary Infiltrates

XI. Complications

  1. Heart and lung changes
    1. Cor Pulmonale (or Pulmonary Hypertension)
    2. Right Ventricular Hypertrophy (and main pulmonary artery enlargement)
  2. Apparent Life-Threatening Events In Children (ALTE)
  3. Respiratory infections (e.g. RSV)
    1. Increased susceptibility
    2. Increased severity
  4. Reactive Airways
    1. Episodic respiratory distress and Wheezing
  5. Growth Delay
    1. Require higher Caloric Intakes due to increased work of breathing

XII. Associated Conditions: Exacerbating factors seen in very Premature Infants

  1. Subglottic Stenosis (from prolonged intubation)
  2. Laryngomalacia
  3. Tracheomalacia

XIII. Course

  1. Lung function often normalizes by teen years

XIV. References

  1. Claudius and Boyer in Majoewsky (2013) EM:Rap 13(2):10
  2. Fuchs and Yamamoto (2011) APLS, Jones and Bartlett, Burlington, p, 72-5
  3. Walsh (2006) Pediatrics 117: S52-6 [PubMed]

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Ontology: Bronchopulmonary Dysplasia (C0006287)

Definition (MSH) A chronic lung disease developed after OXYGEN INHALATION THERAPY or mechanical ventilation (VENTILATION, MECHANICAL) usually occurring in certain premature infants (INFANT, PREMATURE) or newborn infants with respiratory distress syndrome (RESPIRATORY DISTRESS SYNDROME, NEWBORN). Histologically, it is characterized by the unusual abnormalities of the bronchioles, such as METAPLASIA, decrease in alveolar number, and formation of CYSTS.
Definition (NCI) A non-neoplastic lung disorder which develops in premature neonates who have been treated with oxygen and mechanical ventilation.
Definition (CSP) chronic lung disease of infants, possibly related to oxygen toxicity or barotrauma, characterized by bronchiolar metaplasia and interstitial fibrosis.
Concepts Disease or Syndrome (T047)
MSH D001997
ICD10 P27.8
SnomedCT 67569000
English Bronchopulmonary Dysplasia, Dysplasia, Bronchopulmonary, bronchopulmonary dysplasia, BPD - Bronchopulmon dysplasia, Chron lung disease of prematur, Perinatal bronchopulm.dysplas., BRONCHOPULM DYSPLASIA, bronchopulmonary dysplasia of fetus or newborn, bronchopulmonary dysplasia of fetus or newborn (diagnosis), Bronchopulmonary Dysplasia [Disease/Finding], bronchopulmonary dysplasia (BPD), chronic lung disease of babies, BPD, Bronchopulmonary dysplasia of newborn, BPD - Bronchopulmonary dysplasia, Bronchopulmonary dysplasia, Chronic lung disease of prematurity, Perinatal bronchopulmonary dysplasia, Ventilator lung in newborn, Bronchopulmonary dysplasia of newborn (disorder), bronchopulmonary; dysplasia, dysplasia; bronchopulmonary, ventilator lung; newborn
Dutch chronische longziekte t.g.v. vroeggeboorte, bronchopulmonaal; dysplasie, dysplasie; bronchopulmonaal, ventilator lung; pasgeborene, bronchopulmonaire dysplasie, Bronchopulmonale dysplasie, Dysplasie, bronchopulmonale
French Maladie pulmonaire chronique de la prématurité, Dysplasie bronchopulmonaire, Dysplasie broncho-pulmonaire
German chronische Lungenerkrankung bei Fruehgeburt, bronchopulmonale Dysplasie, Bronchopulmonale Dysplasie
Italian Pneumopatia cronica del prematuro, Displasia broncopolmonare
Portuguese Doença crónica dos pulmões de prematuridade, Displasia broncopulmonar, Displasia Broncopulmonar
Spanish Enfermedad pulmonar crónica del prematuro, displasia broncopulmonar del recién nacido (trastorno), displasia broncopulmonar del recién nacido, displasia broncopulmonar, Displasia broncopulmonar, Displasia Broncopulmonar
Japanese 未熟児慢性肺疾患, 気管支肺異形成症, キカンシハイイケイセイショウ, ミジュクジマンセイハイシッカン
Swedish Bronkopulmonell dysplasi
Czech bronchopulmonální dysplazie, Bronchopulmonální dysplazie, Předčasná chronická plicní choroba
Finnish Bronkopulmonaalinen dysplasia
Polish BPD, Dysplazja oskrzelowo-płucna
Hungarian Bronchopulmonalis dysplasia, Éretlen tüdő chronikus betegsége
Norwegian Bronkopulmonal dysplasi