THORACIC Section 6: Congenital Flashcards
Unilateral abscence of a pectoral muscle (Syndrome)
Poland Syndrome
Congenital obliteration of a short segment of a lobar, segmental or subsegmental bronchus near its origin.
Bronchial atresia
Most common lung segment involved in Bronchial Atresia?
Apical-posterior segment of the left upper lobe.
Probable etiology in Bronchial Atresia?
Vascular insult in utero
Gradual worsening dyspnea over several years. There was no clear history of childhood infection.
Bronchial atresia
Describe the finding.
Give the diagnosis.
Bronchial atresia
There is a focal area of hypoattenuation involving much of the left upper lobe. There is reduced vascularity within this hyperlucent segment. Centrally, there is a bronchocele. No evidence of over expansion of the lobe.
Describe.
Diagnosis and pattern?
Bronchial Atresia
Tubular, low density right upper lobe structure branching away from the hilum without connection to the tracheobronchial tree. The surrounding lung is hyperlucent due to air trapping.
A large “nodule” (sometimes with a branching or “finger in the glove” morphology) nearby in the hilum — which represents mucoid impaction in the blind ended bronchus.
Primary differential for Bronchial Atreisa.
What differentiates it from a mucus plug?
Endobronchial tumor.
If HU is < 25 = mucoid impaction.
When you see Pulmonary AVM, you think about?
HHT (Hereditary Hemorrhagic Telangiectasa/ Osler Weber Rendu).
Pulmonary AVMs are commonly found in what lung segments?
lower lobes (more blood flow)
Congenital lung disease that can be a source of right to left shunt?
Pulmonary AVM
Afferent vessel size in Pulmonary AVM that warrants treatment?
> 3 mm
MOST COMMON congenital Venous anomaly of the chest?
Persistent left Superior Vena Cava
Persistent Left SVC usually drains where?
Coronary sinus
5% of persistent left SVC drains in?
Left atrium - causing R>L shunt
Congenital lung disease showing the classic “Unilateral lucent lung”
Swyer-James
Post infectious obliterative bronchiolitis after a viral infection in childhood
Swyer-James
Diagnosis? What can you see?
Swyer-James
The size of the affected lobe is smaller than a normal lobe ( it’s not hyper-expanded).
Congenital lung disease defined by fusion of the basilar segment of the lower lobe behind the heart.
Horseshoe Lung
What is it? What is the association?
Horseshoe lung
Scimitar syndome
partial anomalous pulmonary venous drainage of right lung, right lung hypoplasia, dextraposition of heart, and anomalous systemic arterial supply from aorta or one of its branches to the right lung.
