This document discusses various congenital lung anomalies including pulmonary sequestration, bronchogenic cysts, congenital lobar emphysema, congenital cystic adenomatoid malformation, and other rare anomalies such as pulmonary lymphangiectasia and pulmonary hemangiomatosis. It provides definitions, descriptions, diagnostic approaches and treatment options for each condition.

1. Sequestration.
Bronchogenic cyst.
Cong. Lobar emphysema.
Cong. Cystic adenomatoid malformation.
Other rare anomalies.

2.  Def :- an abnormal segment of lung tissue that has no
communication with the tracheobronchial tree.
 Arterial supply:-
(1) thoracic aorta.
(2) abdominal aorta.
(3) intercostal artery
(4)other sources.
 blood supply usually enters the sequestration away from the
hilum, and most often at the base of lung.
 Venous return :-
(1) pulmonary veins .
(2) systemic venous system .

3. Types
(1) extralobar:- 25%
• separate from the remaining lung.
• with their own visceral pleura.
• They are round soft tissue masses .
• Common site :- above the dome of the diaphragm, with 90% found at
the base of the left lung.
• Other sites :-pericardium, diaphragm, below the diaphragm
retroperitoneally.
• Venous return :- 80% >>>>> azygos, hemiazygos.
20% >>>>>pulmonary veins .
• Associated with other anomalies (congenital diaphragmatic hernia
[CDH], congenital cystic adenomatoid malformation [CCAM],
pericardial cysts, cardiac defects, achalasia.

4. (2) Intralobar :- 75%.
 found in the right and left lower lobes mostly posterior segment of the
LLL.
 They are cystic abnormalities located within visceral pleural of lung
that communicate with the normal lung tissue through the pores of
Kohn
 venous drainage:- pulmonary veins.
 Other associated anomalies :- rare .

5. Diagnosis
 Symptoms :-
recurrent pulmonary infections and hemoptysis
 Investigations :-
mostly C.T chest
M.R.I rarely used
upper GI studies (if concern for enteric communication),

11. Treatment
 Surgical removal of segment or lobectomy .
 Early identification
 and control of the arterial supply is imperative to avoid bleeding
complications
 care should be taken to identify the venous return of the normal lung.
 Any communications with the GI tract should be identified and
divided

12. Aetiology and Incidence:
 They are the most common mediastinal cysts (60%) and are typically located
along the right paratracheal area.
 Resulted from abnormal budding of the trachea.
 Other sites :-
may be attached to the carina or lobar bronchus .
may be intraparenchymal below the diaphragm, or in the pericardium,
peristernal subcutaneous tissues, or skin .
Bronchial communication is rare.
Inner lining >>>>> ciliated pseudostratified respiratory epithelium with
goblet cells.
unilocular (2-10 cm) and may contain normal bronchial elements, such as
smooth muscle and cartilage.
may be filled with blood, mucus, milky material,
or pus (if infected).

13. Diagnosis
 Symptoms :-
asymptomatic and found incidentally on CXR as
an air-filled cyst.
intraparenchymal cysts may be presented with
infection.
 Investigations :-
CT chest & EUS

14. Differential diagnosis
 Sequestration
 Teratoma
 Lymphadenopathy.
 Hemangioma.
 Lipoma.
 Hamartoma .
 Neurogenic tumors.
 pericardial cysts.

18. Treatment
 Complete excision of cyst.
 Mediastinal cysts can be enucleated and the stalk
ligated.
 Intraparenchymal cysts may require a segmentectomy
or lobectomy.
 The bronchial defect and any other associated
communications should be repaired.

19.  50% of all congenital pulmonary anomalies.
 It is the obstruction of a lobar bronchus that results in
expansion of the distal air spaces without alveolar
destruction.
 Common sites :- (LUL > RUL > RML > lower lobes).
 The remaining normal lung is often compressed and there
is mediastinal shift away from the affected lung.

20. Aetiology
 50% of cases, there is no apparent cause.
 (A) Intrinsic causes:-
*cartilaginous defect.
*mucous plugging, and granulation tissue
obstructing the airway.
 (B) Extrinsic causes :-
*mediastinal tumors
*bronchogenic or duplication cyst .
*tetralogy of Fallot.
*pulmonary stenosis.

21. Diagnosis
 asymptomatic at birth.
 patients may develop cough, dyspnea , wheezes .
 There is a male predominance of 3:1 .
 80% of those affected will show symptoms by the age
of 6 months.
 Associated anomalies :- cardiac (14%) and rib or chest
abnormalities.
 If symptoms begin & treatment is not administered
immediately , 50% of patients will die within 1 week,
and another 30% to 40% will die within 1 month.

22.  Investigations :-
*CXR
*CT
*bronchoscopy
*echocardiography
*MRA

25. Treatment
 Asymptomatic patients >>>> conservative .
 symptomatic patients >>>>> surgical removal of
diseased segment or lobe .
 The procedural mortality rate is 7%, with the greatest
risk at the time of induction.
 Selective intubation will minimize overinflation of the
emphysematous tissue.
 surgeon should be prepared for immediate
thoracotomy after induction.

26.  Def :- a hyper-proliferation of bronchi (cartilage,
smooth muscle, bronchial glands, columnar and
cuboidal epithelial cells) with normal pulmonary
vasculature, and abnormal alveolar development.
 25% of all congenital pulmonary anomalies.
 Lesions are single and localized to one lobe.

27. Diagnosis
 presents as neonatal acute respiratory distress.
 CXR :- multiple air fluid levels.
 C.T chest used for confirmation .
 Associated anomalies:-
(1) pectus excavatum (most common).
(2) cardiac and pulmonary vessel malformations.

28.  (A) Type I (macrocystic, 60-70%):-
Large, widely-spaced, irregular cysts (> 2 cm).
rarely associated with polyhydramnios or other
anomalies.
Most patients will reach term, but some are stillborn
Mediastinal shifting can be seen in 75% of patients
with some associated cyanosis and grunting.
50% of cases develop pneumonia during infancy or
early childhood.
good prognosis .

29.  (B) Type II (mixed, 20-40%):-
smaller than 2 cm.
have the appearance of more bronchioles with
increased proliferation.
less mediastinal shift.
Patients tend to be premature or stillborn.

30.  (3) Type III (microcystic, 10%):-
smaller than 0.5 cm.
the mass is firmer.
Prognosis is very poor.
life expectancy :- hours after birth.

35. Treatment
 (1) Asymptomatic :-
observed for the first 4 to 6 months.
repeat imaging at the age of 6 months to assess
for enlargement or malignant degeneration.
After 6 months resection occurs
This delay allows further growth of the child &
reducing operative morbidity.

36. (2) Symptomatic lesions
 after birth should be resected without delay.
 Most of the time a lobectomy is required.
 After successful resection, long term outcomes are
excellent.

37. other rare anomalies:-
1) Cong. pulmonary lymphangiectasia:-
* very rare.
*characterized by dilated lymphatics (with
resultant pulmonary hypoplasia) due to intrauterine
lymphatic obstruction.
*CXR shows a “soap bubble” appearance with
diffusely granular parenchyma, hyperinflation, and a
prominent interstitium.

38. Treatment
 Conservative.
 drainage of effusions
 a low-fat, high-protein diet with medium-chain fatty
acids

39. 2)Pulmonary hemangiomatosis
 rare benign vascular tumor.
 characterized by proliferation of capillaries in the
interstitium.
 present with respiratory distress, pulmonary
hypertension, and consumptive coagulopathy
(Kasabach-Merritt syndrome).

40.  Treatment is supportive.
 Prognosis >>>>> bad.
 lung transplantation may be useful

41. Reference :-
 TSRA_Review_of_Cardiothoracic_Surgery.
 Johns Hopkins Textbook of Cardiothoracic Surgery, 2E
[New Medical Books].