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ABTS - Thoracic Surgery Notecards > 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)
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2
Q

Most common anomaly of the trachea

A

Trachoesophageal Fistula (TEF)

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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
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4
Q

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

A

TEF

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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)

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6
Q

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

A

Pulmonary sequestration

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7
Q

Arterial supply for pulmonary sequestraton most frequently from _

A

Thoracic aorta

Alternate sources:

abdominal aorta

intercostal artery

multiple sources

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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)

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9
Q

Pulmonary sequestration may be associated with __

A

Esophageal fistula (both arise from forgut)

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10
Q

Frequency of extralobar/intralobar sequestration

A

Extralobar (25%)

Intralobar (75%)

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11
Q

Characteristics of Extralobar sequestration?

A
  • distinct from remaining lung (have own pleura)
  • located above the dome of diaphragm (90% located at the base of left lung)
  • venous return is MC systemic (azygous, hemiazygous)
    • 20% drain to pulmonary veins
  • associated with other congenital anomalies
  • may contain malignancies
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12
Q

MC location of ELS

A

base of left lung (90%)

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13
Q

ELS assocated with what other congenital anomlies

A
  • congenital diaphragmatic hernia
  • congenital cystic adenomatoid malformation (CCAM)
  • pericardial cysts
  • cardiac defects
  • esophageal achalasia
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14
Q

Various locations of ELS

A
  • pericardium
  • diaphragm
  • below diaphragm in retroperitoneum
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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

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16
Q

MC location of ILS

A

posterior segment of LLL

(usually ILS located in right and left lower lobes)

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17
Q

MC location for venous drainage of ILS

A

Pulmonary veins (90% of ILS)

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18
Q

Sx of sequestration

A

Recurrent pulmonary infections and hemoptysis

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19
Q

Dx w/u for sequestration

A
  • CT or MRI
  • UGI series (if concern for enteric communication)
  • Angiography (rare, to identify feeding vessels)
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20
Q

Tx of sequestration

A
  • Lobectomy (MC)
    • Sometimes segmentectomy is sufficient
    • Early ID and control of arterial supply to control bleeding complications
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21
Q

Definition of bronchial cysts

A

Abnormal budding of trachea

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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

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23
Q

Histology of bronchogenic cysts

A

Inner lining of ciliated pseudostratified respiratory epithelium

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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)

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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

A

50%

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
61
Q
A

ABTS - Thoracic Surgery Notecards (48 decks)

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