Congenital cystic adenoid malformation IMAGING IS VERY ESSENTIAL
Bronchopulmonary foregut malformation:
- Arise from a supernumerary lung bud that develops below the normal lung bud.
- Location and communication with GIT depend on when in embryonic life the bud develops.
- Most malformations present clinically when they become infected (communication with GIT).
Overview of Bronchopulmonary Malformations:
60% basilar, left
80% left or below diaphragm
|Bronchogenic cyst||Mediastinum, 85%; lung, 15%|
|Congenital lobar emphysema||LUL, 40%; RML, 35%; RUL, 20%|
- Recurrent infection
- Lung abscess
- Hemoptysis during childhood.
- Non-functioning pulmonary tissue ànearly always posteromedial segments of lower lobes.
- Systemic arterial supply à anomalous arteries from the aorta (less common branch of the celiac artery)
- No connection to bronchial tree
Types of Pulmonary Sequestration:
- Large (>5 cm) mass near diaphragm
- Air-fluid levels if infected
- Surrounding pulmonary consolidation
- Sequestration may communicate with GIT.
- It Results from the abnormal budding of the tracheobronchial tree. Cysts contain respiratory epithelium.
- Mediastinum, 85% àposterior > middle > anterior mediastinum)
- Lung, 15%
|o Well-defined round mass in subcarinal / parahilar region
o Pulmonary cysts commonly located in medial 1/3 of lung
o Initially no communication with tracheobronchial tree
o Cysts are thin walled.
o Cysts can be fluid or air filled.
Congenital cystic adenoid malformation (CCAM):
- CCAM refers to a proliferation of polypoid glandular lung tissue without normal alveolar differentiation.
- Respiratory distress occurs during first days of life.
surgical resection (sarcomatous degeneration has been described).
(Stocker types 1 & 2)
|o single cyst or multiple cysts > 5 mm confined to one hemi-thorax.
o Better prognosis.
(Stocker type 3)
|o Homogeneous echogenic mass without discernible individual cysts.
o Closely resembles pulmonary sequestration or intrathoracic bowel from a diaphragmatic hernia.
o Less common.
|o Multiple cystic pulmonary lesions of variable size
o Air-fluid levels in cysts
o Variable thickness of cyst wall
Congenital lobar emphysema:
- Progressive overdistention of one or more pulmonary lobes but usually not the entire lung.
- 10% of patients have congenital heart disease àpatent ductus arteriosus [PDA] & ventricular septal defect [VSD].
- Idiopathicà 50%
- Obstruction of airway with valve mechanismà 50%:
- Bronchial cartilage deficiency or immaturity
- Web, stenosis
- Extrinsic compression
Types of Pulmonary Underdevelopment:
- Agenesis: Complete absence of one or both lungs (airways, alveoli, & vessels).
- Aplasia: Absence of lung except for a rudimentary bronchus that ends in a blind pouch.
- Hypoplasia: decrease in number and size of airways and alveoli; hypoplastic PA.
- Scimitar Syndrome (Hypogenetic Lung Syndrome, Pulmonary Venolobar Syndrome)
– A special form of a hypoplastic lung.
– The hypoplastic lung is àperfused from the aorta & drained by the IVC or portal vein.
– The anomalous vein has a resemblance to a Turkish scimitar (sword).
– Associations include:
1) Accessory diaphragm, diaphragmatic hernia
2) Bony abnormalitiesà hemivertebrae, rib notching, rib hypoplasia
3) CHD: atrial septal defect (ASD), VSD, PDA, tetralogy of Fallot
- Radiographic Features:
|o Small lung àmost commonly the right lung.
o Retrosternal soft tissue density à hypoplastic collapsed lung.
o Anomalous vein resembles a scimitar
o Systemic arterial supply from aorta
o Dextroposition of the heart àshift because of hypoplastic lung)
Congenital diaphragmatic hernia (CDH):
- 1 in 2000 to 3000 births.
- The mortality rate of isolated hernias is 60% (with postnatal surgery) and higher when other abnormalities are present.
- Respiratory distress occurs in the neonatal period.
- Associated abnormalities include:
Pulmonary hypoplasia (common)
Neural tube defects : spina bifida, encephalocele Anencephaly
|Bochdalek’s hernias:||Ø 90% of CDH à posterior:
o 75% are on the left, 25% on right
o Right-sided hernias are more difficult to detect because of similar echogenicity of liver & lung.
o Contents of hernia:
ü Stomach, 60%.
ü Colon, 55%.
ü Small intestine, 90%.
ü Spleen, 45%.
ü Liver, 50%.
ü Pancreas, 25%.
ü Kidney, 20%.
o Malrotation of herniated bowel is very common.
|Morgagni hernias:||Ø 10% of CDH à anterior:
o Most occur on right (heart prevents development on the left).
o Most common hernia contents: omentum, colon
o Accompanying anomalies common
|Eventration:||o Due to relative absence of muscle in dome of diaphragm
o Associated with:
ü Trisomies 13, 18, congenital CMV, rubella arthrogryposis multiplex, pulmonary hypoplasia
- Radiographic Features:
|o Hemi-diaphragm not visualized
o Multi-cystic mass in chest
o Mass effect
Kartagener syndrome (immotile cilia syndrome):
- Due to the deficiency of the dynein arms of cilia causing immotility of respiratory, auditory, & sperm cilia.
|o Complete thoracic & abdominal situs inversus
o Sinus hypoplasia & mucosal thickening
YOU CAN SEE REAL CASE IMAGES AT THIS LINK: