Congenital Disorders resp

Question 1

Lesions of congenital origin derived from the primitive foregut

Answer 1

Bronchogenic Cysts

Question 2

Bronchogenic Cysts epidemiology

Answer 2

primary cysts of the Mediastinum

Question 3

Imaging studies of Bronchogenic Cysts

Answer 3

Uni-locular, non-calcified masses
Sphaerical or oval, with smooth outlines

Question 4

DD of bronchogenic cysts

Answer 4

Lung abscess, Tbc, infected bullas, neoplasms

Question 5

Macro features of bronchogenic cysts

Answer 5

Uni-locular cyst (cyst with a single sac)
Content: Clear fluid, haemorrhagic secretions or air

Question 6

Micro features of bronchogenic cysts

Answer 6

Cyst’s internal lining: Columnar ciliated epithelium
Cyst’s wall: Cartilage and bronchial mucous glands

Question 7

Clinical features of bronchogenic cysts

Answer 7

Cough - Fever - Pain - Dyspnoea
Tracheobronchial compression and lung infections
(in children; because of the soft tracheo-bronchial
tree)

Question 8

Complications of bronchogenic cysts

Answer 8

Infections (in cysts with bronchial communications)
Rapture of cyst into trachea, pleural or pericardial
cavity
Pneumothorax, accompanied by pleuritis

Question 9

CCAM

Answer 9

Congenital pulmonary airway malformation

Question 10

BPS

Answer 10

Broncho-pulmonary sequestration

Question 11

Pathogenesis of congenital cystic lesions of the lung

Answer 11

Abnormalities that occur during the branching and
proliferation of the bronchial structures
Abnormal proliferation of tissues, airway obstruction,
dysplasia and metaplasia of the normal tissue

Question 12

Made of peripheral and non-functioning lung tissue,
separated from the normal pulmonary structure

Answer 12

Broncho-pulmonary sequestration

Question 13

Localisation of broncho-pulmonary sequestration

Answer 13

Intralobar or extralobar

Question 14

Characterised by:
a. Chronic respiratory tract infections
b. Abnormally positioned internal organs
c. Infertility

Answer 14

Immotile cillia syndrome

Question 15

Genetics of immotile cillia syndrome

Answer 15

DNAI1 - DNAH5

Question 16

Signs & Symptoms of immotile cilia syndrome in babies

Answer 16

Breathing problems at births

Question 17

Signs & Symptoms of immotile cilia syndrome in early childhood

Answer 17

Frequent respiratory tract infections → Bronchiectasis

Question 18

Major diagnostic sign of immotile cilia syndrome

Answer 18

50% of patients have Situs inversus totalis
12% of patients: Heterotaxy syndrome or situs
ambiguus

Question 19

Why is infertility an adverse effect of immotile cilia syndrome?

Answer 19

Men -> flagella that moves the sperm is dysfunctional
Women -> cilia in the fallopian tubes are dysfunctional

Question 20

TX of immotile cilia syndrome

Answer 20

Tympanostomy tubes → Reduction of recurrent infections and conductive hearing loss

Question 21

Types of congenital diaphragmatic hernia

Answer 21

1. Postero-lateral Bochdalek-type (70%)
2. Anterior Morgagni-type (27%)
3. Central septum transversum-type (2-3%)