2. Congenital Pulmonary Malformations (CPM) - Cox

• emphysema will cause a mass effect causing collapse of the lungs and mediastinal shift
• development of pulmonary hypertension is due to vessel remodeling
• What disease?

  • complete absence of the lung or bronchial tree
  • due to absence of primordial progenitor 


  pulmonary agenesis
• Pulmonary agenesis can development pulmonary hypertension due to vessel remodeling
• What disease?

  • developmental arrest, distal pulmonary system beyond primitive form
  • absence of mature lung tissue 


  Pulmonary aplasia
• Pulmonary aplasia vs. agenesis

  • Aplasia - abnormal growth bronchus
  • Agenesis - no bronchial bud
• Fetus with pulmonary aplasia presents with respiratory distress

  • reduce lung tissue --> reduce gas exchange
• What disease?

  • incomplete development of lung & components
  • lung is under developed - size and weight reduced 


  pulmonary hypoplasia
• Pulmonary hypoplasia is due to?

  mechanical conditions restricting lung growth

  • oligohydramnios
  • thoracic cage abnormalities
  • diaphragmatic hernia
• Pulmonary aplasia can lead to early onset respiratory distress

  • lung is incapable of gas exchange
• Potter's sequence describes the typical physical appearance caused by pressure in utero due to oligohydramnios
• How can Prolonged oligohydramnios cause pulmonary hypoplasia?

  1. low amniotic fluid
  2. thoracic compression
  3. restricted lung growth
• What disease?

  • diaphragmatic defect causing abdominal contents to spill into chest cavity 


  congenital diaphragmatic hernia (CDH)
• Congenital diaphragmatic hernia can lead to what?

  1. exerts mass effects
  2. pulmonary hypoplasia
  3. respiratory distress
• Congenital diaphragmatic hernia (CHD) is due to?

  pleuroperitoneal canals fusion failure during 8 week gestation
• What type of hernia is the most common fetal congenital diaphragmatic hernia?

  Bochdalek hernia
• What type of hernia?

  • herniation small & large bowel
  • intraabdominal solid organs into thoracic cavity 


  Bochdalek hernia

  
• Bochdalek hernia (CDH) complications is due to pulmonary hypoplasia that can cause respiratory distress

  • reduced gas exchange area causing vascular remodeling
  • leading to pulmonary hypertension
• Bochdalek hernia (CDH) also associated with congenital cardiac defect - how?

  • mass effect can compromise left ventricle development causing hypoplastic LV
• Morgagni vs Bochdalek

  • frequency - morgangi
  • severity - morgangi
• Morgagni hernias (CDH) can present with?

  • early onset respiratory distress
  • causing frequent pulmonary infection
  • mass effect can compromise LV development
• What disease?

  • abnormal budding tracheobronchial tree due to central or peripheral pulmonary cyst
  • subtype of bronchopulmonary foregut malformation 


  bronchogenic cysts
• bronchogenic cysts can cause complications such as?

  1. mass effect
  2. airway compressed
  3. respiratory distress or emphysema
  4. lung collapse
• What disease?

  • also called accessory lung
  • bronchopulmonary mass without normal bronchial communication
  • normal/anomalous vascular supply - abnormal from systemic arterial supply - from aortic branch 


  Pulmonary sequestration
• Pulmonary sequestration is due to abnormal vascular supply

  • affect venous drainage
  • resulting in blood shunting
• Intralobar sequestration(ILS)

  • mass without normal bronchial communication
  • share common pleural lining with rest of the lung
  • venous drainage via pulmonary venous system
• Extralobar sequestration (ELS)

  • include thoracic and subdiaphragmatic types
  • covered by own pleural
  • vascular supply from aorta
  • drainage is systemic or pulmonary
• Extralobar vs. Intralobar sequestration

  • Extralobar - own pleural
  • intralobar - share with rest of the lung
• What disease?

  • focal interruption/obliteration bronchus
  • distal lung tissue normal but hyperinflated (air trapping) - pore of Kohn or canals of Lambert permit inflation (one way valve) 


  bronchial atresia
• Why does bronchial atresia cause hyperinflated lung?

  • accumulation of mucus can obstruct the lung
• What disease?

  • synonymous with congenital lobar emphysema
  • progressive overinflation of one or more lobes of neonate lung
  • air trapped during expiratory phase - expand like balloon 


  Congenital lobar overinflation (hyperinflation)
• Which lung is mostly affected in Congenital lobar overinflation (hyperinflation)?

  upper lobe
• When is Congenital lobar overinflation (hyperinflation) dx?

  neonatal period within first 6 month
• Congenital lobar overinflation (hyperinflation) is caused by

  • abnormal development of bronchial cartilage
  • absence cartilaginous ring
• Congenital lobar overinflation (hyperinflation) - absence of cartilage will lead to

  • lack of structural support leading to airway collapse during expiration
  • expiratory obstruction causing air trapping distally
  • leading to hyperinflated lung
• Congenital lobar overinflation (hyperinflation) can do what to nearby tissue?

  • compress nearby tissue causing respiratory distress and death
• What disease?

  • defect in development of terminal bronchioles
  • considered part of bronchopulmonary forgut malformation spectrum

  Congenital pulmonary airway malformation (CPAM)
• Congenital pulmonary airway malformation (CPAM) can be due to

  • Bronchoalveolar development failure
  • hamartomatous cyst proliferation
• Congenital pulmonary airway malformation (CPAM) due to hamartomatous cyst proliferation

  • usually benign
  • collection of disorganized cells that normally constitute lung and bronchi
  • there is no bronchial cartilage or gland
• Congenital lobar overinflation (hyperinflation) can symptoms such as

  • respiratory distress in infants
  • recurrent infection in older children