Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

General Thoracic Surgery > Congenital Lung Disease > Flashcards

Congenital Lung Disease Flashcards

1
Q

4 phases of intrauterine pulmonary development

A
  1. Embryonic phase (weeks 1-5)
    1. development of lung buds and primitive 5 lobes
  2. Pseudoglandular phase (weeks 5-16)
    1. development of bronchial tree and pulm vasculature
  3. Cannicular phase (weeks 16-26)
    1. development of bronchioles/alveolar ducts
  4. Terminal sac phase (weeks 26-40)
    1. development of primitive alveoli
    2. surfactant production (type II pneumocytes)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Most common anomaly of the trachea

A

Trachoesophageal Fistula (TEF)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Classification system for TEF

A

Gross Classification

  • Type A (8%): EA without TEF
  • Type B (1%): EA with proximal TEF
  • Type C (87%): EA with distal TEF
  • Type D (1%): EA with proximal and distal TEF
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Excessive air within esophagus, stomach and intestinal tract; inability to pass NGT

A

TEF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

TOC for TEF

A

Right extrapleural thoracotomy

Right cervical incision

Divison of fistula

Repair of esophageal defect

Interposition muscle flp (to reduce likelihood of recurrence)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Abnormal segment of lung tissue that has no communication with tracheobronchial tree

A

Pulmonary sequestration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Arterial supply for pulmonary sequestraton most frequently from _

A

Thoracic aorta

Alternate sources:

abdominal aorta

intercostal artery

multiple sources

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Anatomy of arterial/venous supply to pulmonary sequestration

A

Arterial supply usually enters sequestration away from hilum and most often at base of lung

Venous return most commonly into pulmonary veins (can also be into systemic venous system)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Pulmonary sequestration may be associated with __

A

Esophageal fistula (both arise from forgut)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Frequency of extralobar/intralobar sequestration

A

Extralobar (25%)

Intralobar (75%)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Characteristics of ELS

A
  • distincy from remaining lung (have own pleura)
  • located above dome of diaphragm (90% located at base of left lung)
  • venous return is MC systemic (azygous, hemiazygous)
    • 20% drain to pulmonary veins
  • associted with other congenital anomalies
  • may contain malignancies
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

MC location of ELS

A

base of left lung (90%)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

ELS assocated with what other congenital anomlies

A
  • congenital diaphragmatic hernia
  • congenital cystic adenomatoid malformation (CCAM)
  • pericardial cysts
  • cardiac defects
  • esophageal achalasia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Various locations of ELS

A
  • pericardium
  • diaphragm
  • below diaphragm in retroperitoneum
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Definition of ILS

A

cystic abnormalities within the visceral pleura of a given lung that communicate with the normal lung tissue through the pores of Kohn

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

MC location of ILS

A

posterior segment of LLL

(usually ILS located in right and left lower lobes)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

MC location for venous drainage of ILS

A

Pulmonary veins (90% of ILS)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Sx of sequestration

A

Recurrent pulmonary infections and hemoptysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Dx w/u for sequestration

A
  • CT or MRI
  • UGI series (if concern for enteric communication)
  • Angiography (rare, to identify feeding vessels)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Tx of sequestration

A
  • Lobectomy (MC)
    • Sometimes segmentectomy is sufficient
    • Early ID and control of arterial supply to control bleeding complications
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Definition of bronchial cysts

A

Abnormal budding of trachea

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Characteristics of bronchogenic cysts

A

MC mediastinal cysts (60%)

Typically located along right paratracheal area

May be attached to carina or lobar bronchus

Bronchial communication rare

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Histology of bronchogenic cysts

A

Inner lining of ciliated pseudostratified respiratory epithelium

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Morphology of bronchogenic cysts

A

Unilocular (2-10 cm)

May contain normal bronchial elements (smooth muscle and cartilage)

Maybe filled with blood, mucus, milky fluid, pus (if infected)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Presenting sx and signs of bronchogenic cysts

A

Usually asymptomatic

May cause compressive sx (compresison of PA, atrial fibrillation)

26
Q

Dx of bronchogenic cysts

A

CT or MRI

EUS

27
Q

Tx bronchogenic cysts

A

Complete enucleation with ligation of stalk (avoidance of bleeding and rupture)

Intraparenchymal cysts: segmentectomy or lobectomy

28
Q

Defect makes up 50% of all congenital pulmonary anomalies

A

Congenital emphysema

29
Q

Definition and characteristics of congenital emphysema

A
  • Obstruction of lobar bronchus, resulting in expasion of the distal air spaces without alveolar destruction.
  • Normal pulmonary vasculature to affected lobe
  • Remaining normal lung often compressed
  • Mediastinal shift away from affected lung
30
Q

Anatomic distribution of congenital emphysema

A

Upper airway predominance

LUL > RUL > RML > lower lobes

31
Q

Intrinsic and extrinsic causes of congenital emphysema

A
  • Intrinsic causes:
    • cartilagenous defect (25%)
    • mucus plugging
    • granulation tissue obstructing airway
  • Extrinsic causes:
    • TOF
    • pulmonary stenosis
    • anomalous pulmonary veins
    • adenopathy
    • mediastinal tumors
    • bronchogenic or duplication cysts
  • No apparent cause (50% of cases)
32
Q

Associated anomolies with congenital emphysema

A

Cardiac anomalies (14%)

Rib and chest wall anomalies

33
Q

Acuity of congenital emphysema

A

Acute treatment required

  • 50% mortality within 1 week if no treatment after symtoms (cough, dyspnea, wheezing) develop
  • additional 30-40% of patients die within 1 month
34
Q

Dx modalities used for congenital emphysema

A
  • CXR
  • CT
  • Bronchocoscpy
  • Echocardiography
  • MRA
  • V/Q scan
35
Q

Tx options for congenital emphysema

A
  • Asymptomatic - mildly symptomatic
    • observation (50% normalize during infancy)
  • Symptomatic
    • surgical resection of diseased tissue = lobectomy
      • mortatliy 7%
        • greatest risk during induction
        • selective intubation required to avoid overinflaation of emphysematous tissue
        • be prepared for emergent thoracotomy after induction
36
Q

Characteristics of acquired lobar emphysema

A
  • due to compications of respiratory distress syndrome or prolonged intubation
  • most frequently affects RUL
  • Tx: resection
    • alternative: balloon dilation of airway stenosis
  • Postop patients have progressive respiratory problems
37
Q

Definition of CCAM (congenital cystic adenomatoid malformation)

A

Hyperproliferation of bronchi (cartilage, smooth muscle, bronchial glands, columnar and cuboidal epithelial cells) in the setting of normal pulmonary vasculature and abnormal alveolar development.

  • Diseased tissue does not communicate with tracheobronchial tree
  • Lesions typically single and limited to 1 lobe
38
Q

Incidence of CCAM

A

comprises 25% of all congenital pulmonary anomalies

39
Q

Presentation of CCAM

A

neonatal acute respiratory distress with multiple air-fluid levels on CXR

40
Q

Associated congenital anomalies with CCAM

A
  • pectus excavatum (MC)
  • cardiac
  • pulmonay vessel malformations
41
Q

Stoker classification for CCAM

A
  • Type I (macrocystic, 60-70%)
    • large, widely spaced, irregular cysts (> 2 cm)
    • rarely associated with polyhydramnios or associated anomalies
    • most reach term pregnancy
    • medistinal shift (75%)
    • overall prognosis good
      • 50% develop PNA
  • Type II (mixed, 20-40%)
    • cysts < 2 cm
    • less medistinal shift
    • pregnancy: premature or stillborn
  • Type III (microcystic, 10%)
    • cysts < 0.5 cm
    • mass appears to encase entire affected lobe (LLL most common)
    • very poor prognosis (life expectancy hours after birth)
42
Q

Management of CCAM

A
  • Asymptomatic CCAM
    • observed for first 4-6 months wtih repeat imaging at 6 mo
    • surgical resection after 6 month assessment
      • delayed intervention allows for growth of child (reduced morbidity)
  • Symptomatic CCAM
    • emergent resection after birth
      • lobectomy is TOC
43
Q

Cantrell and Guild classification of tracheal stenosis

A
  • Type 1: involvement of entire trachea
  • Type 2: funnel shaped stenosis of upper, lower, or entire trachea
  • Type 3: segmental stenosis of lower trachea
44
Q

Characteristics of congenital tracheal stenosis

A
  • Stenotic areas have circumferential cartilagenous rings (2-18)
  • 50% associated with pulmonary vascular rings/slings
  • All types may be associated with pulmonary hypoplasia
45
Q

Dx of congenital tracheal stenosis

A
  • CT
  • Bronchoscopy
  • Echocardiography
46
Q

Acuity of congenital tracheal stenosis

A

High

Must be treated aggressively d/t high risk of sudden death

47
Q

SOC (surgery of choice) for congenital tracheal stenosis

A

Partial tracheal resection with reconstruction

* may require CPB support or circulatory arrest if associated vascular rings/slings

48
Q

Reconstruction options following trachea resection

A
  • Primary anastomoiss
  • pericardium
  • aortoic homograft
  • costochondral graft
  • slide tracheostomy
  • tracheal autograft
49
Q

_ % of infant trachea can be removed and still be closed primarily

50
Q

Primary repair ideal when <= _ rings are resected

A

<= 8 rings

*if > 8 rings resected, autograft whoud be considered for reconstruction

51
Q

Nonsugical palliative options for congenital tracheal stenosis in inoperable patients

A
  • Balloon dilation
  • Split posterior tracheoplasty
  • Stenting
  • Local steroid injection
  • Electro resection
  • Cryotherapy
52
Q

2nd most common airway anomalies (after TEF)

A

Bronchial atresia

53
Q

Morphology of bronchial atresia

A

Lobar or segmental bronchus ends blindly, but lung parenchyma distally will expand and become emphysemeatous due to air communication via Pores of Kohn

54
Q

Theories for development of bronchial atresia

A
  1. distal bronchial bud separated but continued to develop
  2. vascular insult to the atretic segments
55
Q

Anatomic distribution of bronchial atresia

A

LUL > LLL > RUL

Segmetnal bronchus > lobar bronchus

56
Q

Indications for surgical resection

A

Enlargement and repeated pulmonary infections

May be resected for presence of abnormal tissue to prevent infection and complications

SOC: lobectomy

57
Q

Other less common pulmonary congenital lesions

A
  • Congenital pulmonary lymphangiectasia
  • Pulmonary hemangiomatosis
  • Pulmonary AV fistula
58
Q

Causes by dilated lymphatics (with resultant pulmonary hypoplasia) due to intrauterine lymphatic obstruction

A

Contenital pulmonary lymphangiectasia

  • Soap-bubble appearance on CXR
    • diffusely granular parenchyma, hyperinflation, prominant interstitum
  • Tx:
    • supportive with drainage of effusions
    • low-fat, high-protein diet with medium chain fatty acids.
  • Prognosis poor
59
Q

Rare, benign vascular tumor characterized by capillary proliferation in pulmonary interstitium

A

Pulmonary hemangiomatosis

  • resp distress, PHTN, consumptive coagulopathy (Kasabach-Merritt syndrome)
  • Tx: supportive
    • IFN and lung transplantation may be beneficial
  • Prognosis grim
60
Q

Rare malformations sometimes confused with Osler-Weber-Rendu syndrome

A

Pulmonary AV fistula

  • asymptomtic vs. respiratory distress, cyanosis, cardiac failure
  • associated with pulmonary hemorrhages
  • complicaitons
    • hemoptysis, cerebral thrombosis, brain abscesses, ptx
  • TOC:
    • embolization of focal lesions
    • wedge resection of larger lesions or lesions that failed embolization

General Thoracic Surgery (36 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions