Imaging Pearls ❯ Chest ❯ Pediatric Pathology
|
-- OR -- |
|
- Congenital Lung Anomalies
• Normal vascularity
• Congenital lobar hyperinflation
• Congenital pulmonary airway malformation
• Bronchogenic cyst
• Bronchial atresia
• Parenchymal agenesis, hypoplasia
• Abnormal vascularity
• Scimitar syndrome
• Sequestration - Congenital Lobar Hyperinflation
• Formerly, lobar emphysema-misnomer
• Etiology-partial bronchial obstruction
• deficient bronchial cartilage
• intraluminal web, stenosis, malacia
• Onset: > 90% cases < 6 months
• Neonates : dyspnea, cyanosis, cough
• Older pts: wheezing or incidental - Congenital Pulmonary Airway Malformation
• Formerly, Congenital Cystic Airway Malformation (CCAM)
• 25% of congenital lung lesions
• Not true “cyst”, rather dilated bronchioles
• Normal arterial supply & venous drainage
• Communicates with bronchial tree - CPAM : Clinical
• Most symptomatic as neonates
• cyanosis, grunting, tachypnea
• 10% found in older children & adults
• Often presents as recurrent pneumonia - CPAM: Imaging
• Type 0 (1-3%) perinatal death (not imaged)
• Type 1 (60-70%) (ADULT lesion)
• one or more dominant cysts: 3-10 cm in size
• surrounding smaller cysts
• Type 2 (15-20%) cysts, 1-3 cm
• Type 3 (5-10%) microcysts (< 5mm) on cut sections (not imaged, high mortality)
• Type 4 (15%) cysts up to 10 cm - CPAM: Associated Lesions
• Malignant processes have been described in
• association with CPAM types and 4
• Type 1: bronchoalveolar carcinoma (BAC)
• Type 4: pleuropulmonary blastoma
• Benign process associated with types 2 and 3
• Type 2: bronchial atresia & sequestration’
• Type 3: pulmonary hypoplasia - Pleuropulmonary Blastoma
• Children < 3 years ( 1 or 2 cases in adults)
• Malignancy of lung/pleura
• sarcomatous and embryonal cells
• 3 types-1 (cystic), 2 (cystic/solid), 3 (solid)
• Increasing malignancy 1 to 3
• Survival 80-85% type 1; 40-50% type 3
- Cystic Adenomatoid Malformation:Facts
- AKA CAM
- Intralobar mass of disorganized pulmonary parenchyma that communicates with bronchial tree
- 25% of congenital lung disorders
- On CT and CXR can look like lung abscess or necrotizing pneumonia
- Equal frequency in all lobes - Differential Dx Includes
- Congenital
- lobar emphysema
- Diaphragmatic hernia
- Neurogenic cyst
- Sequestration
- Mediastinal teratoma
- Lung abscess