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ABTS - Thoracic Surgery Notecards IA > 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
Presenting sx and signs of bronchogenic cysts
Usually asymptomatic May cause compressive sx (compresison of PA, atrial fibrillation)
26
Dx of bronchogenic cysts
CT or MRI EUS
27
Tx bronchogenic cysts
Complete enucleation with ligation of stalk (avoidance of bleeding and rupture) Intraparenchymal cysts: segmentectomy or lobectomy
28
Defect makes up 50% of all congenital pulmonary anomalies
Congenital emphysema
29
Definition and characteristics of congenital emphysema
* 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
Anatomic distribution of congenital emphysema
Upper airway predominance LUL \> RUL \> RML \> lower lobes
31
Intrinsic and extrinsic causes of congenital emphysema
* 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
Associated anomolies with congenital emphysema
Cardiac anomalies (14%) Rib and chest wall anomalies
33
Acuity of congenital emphysema
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
Dx modalities used for congenital emphysema
* CXR * CT * Bronchocoscpy * Echocardiography * MRA * V/Q scan
35
Tx options for congenital emphysema
* 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
Characteristics of acquired lobar emphysema
* 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
Definition of CCAM (congenital cystic adenomatoid malformation)
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
Incidence of CCAM
comprises 25% of all congenital pulmonary anomalies
39
Presentation of CCAM
neonatal acute respiratory distress with multiple air-fluid levels on CXR
40
Associated congenital anomalies with CCAM
* pectus excavatum (MC) * cardiac * pulmonay vessel malformations
41
Stoker classification for CCAM
* 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
Management of CCAM
* 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
Cantrell and Guild classification of tracheal stenosis
* 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
Characteristics of congenital tracheal stenosis
* Stenotic areas have _circumferential cartilagenous rings_ (2-18) * 50% associated with pulmonary vascular rings/slings * All types may be associated with pulmonary hypoplasia
45
Dx of congenital tracheal stenosis
* CT * Bronchoscopy * Echocardiography
46
Acuity of congenital tracheal stenosis
High Must be treated aggressively d/t high risk of sudden death
47
SOC (surgery of choice) for congenital tracheal stenosis
Partial tracheal resection with reconstruction \* may require CPB support or circulatory arrest if associated vascular rings/slings
48
Reconstruction options following trachea resection
* Primary anastomoiss * pericardium * aortoic homograft * costochondral graft * slide tracheostomy * tracheal autograft
49
\_ % of infant trachea can be removed and still be closed primarily
50%
50
Primary repair ideal when \<= _ rings are resected
\<= 8 rings \*if \> 8 rings resected, autograft whoud be considered for reconstruction
51
Nonsugical palliative options for congenital tracheal stenosis in inoperable patients
* Balloon dilation * Split posterior tracheoplasty * Stenting * Local steroid injection * Electro resection * Cryotherapy
52
2nd most common airway anomalies (after TEF)
Bronchial atresia
53
Morphology of bronchial atresia
Lobar or segmental bronchus ends blindly, but lung parenchyma distally will expand and become emphysemeatous due to air communication via Pores of Kohn
54
Theories for development of bronchial atresia
1. distal bronchial bud separated but continued to develop 2. vascular insult to the atretic segments
55
Anatomic distribution of bronchial atresia
LUL \> LLL \> RUL Segmetnal bronchus \> lobar bronchus
56
Indications for surgical resection
Enlargement and repeated pulmonary infections May be resected for presence of abnormal tissue to prevent infection and complications **SOC: lobectomy**
57
Other less common pulmonary congenital lesions
* Congenital pulmonary lymphangiectasia * Pulmonary hemangiomatosis * Pulmonary AV fistula
58
Causes by dilated lymphatics (with resultant pulmonary hypoplasia) due to intrauterine lymphatic obstruction
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
Rare, benign vascular tumor characterized by capillary proliferation in pulmonary interstitium
Pulmonary hemangiomatosis * resp distress, PHTN, consumptive coagulopathy (Kasabach-Merritt syndrome) * Tx: supportive * IFN and lung transplantation may be beneficial * Prognosis grim
60
Rare malformations sometimes confused with Osler-Weber-Rendu syndrome
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

ABTS - Thoracic Surgery Notecards IA (48 decks)

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