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SM_166a: Congenital and Pediatric Disorders Flashcards

1
Q

What are the five phases of development?

A
  1. Embryonic
  2. Pseudoglandular
  3. Canalicular
  4. Saccular
  5. Alveolar
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2
Q

Embyronic stage of development is during ____ and involves _____

A

Embyronic stage of development is during first 1-3 weeks of gestation and involves formation of the airways as an outpouching of the foregut

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3
Q

Pseudoglandular stage of development is during ______ and involves ______

A

Pseudoglandular stage is during weeks 6-16 and involves airways reaching the level of terminal bronchioles and the beginning of formation of the acinar structure

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4
Q

Canalicular stage of development is during ______ and involves ______

A

Canalicular stage of development is during weeks 16-26 and involves epithelium differentiating into type I and II alveolar cells and beginning of surfactant production

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5
Q

Saccular stage of development is during ______ and involves ______

A

Saccular stage of development is during weeks 24-36 and involves further development of capillaries and increased production of surfactant

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6
Q

Alveolar stage of development is during ______ and involves ______

A

Alveolar stage of development is during weeks 36 to after birth and involves development of alveoli and increased production of surfactant

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7
Q

Respiratory distress syndrome is diagnosed via _____ and ______

A

Respiratory distress syndrome is diagnosed via progressive respiratory failure and characteristic chest radiograph demonstrating low lung volume and diffuse reticulogranular ground-glass appearance with air bronchograms

(diffuse white stuff in lungs on X-ray)

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8
Q

Respiratory distress syndrome is most often seen in ________

A

Respiratory distress syndrome is most often seen in premature infants

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9
Q

Respiratory distress syndrome results from ________

A

Respiratory distress syndrome results from inadequate production of surfactant, which causes increased surface tension in the air-liquid interface of the terminal respiratory units

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10
Q

Bronchopulmonary dysplasia (chronic lung disease of infancy) is a term given to _______

A

Bronchopulmonary dysplasia (chronic lung disease of infancy) is a term given to infants with RDS who develop long-term abnormalities of the airway and parenchyma

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11
Q

If an infant is on oxygen at day 28, that infant has ________

A

If an infant is on oxygen at day 28, that infant has bronchopulmonary dysplasia (chronic lung disease of infancy)

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12
Q

Gestational age is _______, while post-menstrual age is _______

A

Gestational age is number of weeks patient was in uterus, while post-menstrual age is total number of weeks patient was in uterus and outside the uterus

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13
Q

Which factors increase the risk of BPD?

A
  • Lower gestational age
  • Lower birth weight
  • Maternal or neonatal infections
  • Positive pressure ventilation
  • Oxygen toxicity
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14
Q

Which factors decrease the risk of BPD?

A
  • Antenatal steroids given to mother before delivery
  • Surfactant therapy for infant
  • Nutritional support and ventilator management
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15
Q

Antenatal steroids ___________, decreasing RDS, morbidity and mortality from premature births

A

Antenatal steroids accelerate morphologic development of type 1 and 2 pneumocytes, decreasing RDS, morbidity and mortality from premature births

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16
Q

Antenatal steroids are recommended for ________

A

Antenatal steroids are recommended for women at risk of preterm delivery prior to 34 weeks gestation

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17
Q

Surfactant delivery into lungs ______ incidence and severity of RDS, mortality, and associated complications

A

Surfactant delivery into lungs decreases incidence and severity of RDS, mortality, and associated complications

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18
Q

Which congenital lung malformations are airway abnormalities?

A
  • Laryngomalacia
  • Tracheo-bronchomalacia
  • Tracheoesophageal fistula
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19
Q

Laryngomalacia

  • Presentation:
  • Etiology:
  • Diagnosis:
  • Treatment:
A

Laryngomalacia

  • Presentation: stridor
  • Etiology: omega shaped epiglottis, short aryepiglottic folds, prolapsed arytenoids
  • Diagnosis: bronchoscopy
  • Treatment: surgical if severe, evaluation and treatment for aspiration, and evaluation and treatment for reflux
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20
Q

Clinical presentation of laryngomalacia is _______

A

Clinical presentation of laryngomalacia is stridor

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21
Q

Etiology of laryngomalacia is ______. _______. and _______

A

Etiology of laryngomalacia is omega shaped epiglottis, short aryepiglottic folds, and prolapsed arytenoids

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22
Q

Diagnosis of laryngomalacia is ______

A

Diagnosis of laryngomalacia is bronchoscopy

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23
Q

Treatment of laryngomalacia involves ______, ______, and ______

A

Treatment of laryngomalacia involves surgery if severe, evaluation and treatment for aspiration, and evaluation and treatment for reflux

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24
Q

Tracheo-bronchomalacia

  • Presentation:
  • Etiology:
  • Diagnosis:
  • Treatment:
A

Tracheo-bronchomalacia

  • Presentation: noisy breathing
  • Etiology: not completely known, but likely alterations of structural support (cartilage and aggrecans)
  • Diagnosis: expert opinion and diagnosis
  • Treatment: primarily supportive but surgical repair if severe
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25
Q

Clinical presentation of tracheo-bronchomalacia is ______

A

Clinical presentation of tracheo-bronchomalacia is noisy breathing

26
Q

Etiology of tracheo-bronchomalacia is _______

A

Etiology of tracheo-bronchomalacia is likely alterations of structural support (cartilage and aggrecans)

27
Q

Diagnosis of tracheo-bronchomalacia is _______

A

Diagnosis of tracheo-bronchomalacia is expert opinion and bronchoscopy

28
Q

Treatment of tracheo-bronchomalacia is _______

A

Treatment of tracheo-bronchomalacia is primarily supportive but surgical repair if severe

29
Q

Tracheoesophageal fistula

  • Presentation:
  • Etiology:
  • Diagnosis:
  • Treatment:
A

Tracheoesophageal fistula

  • Presentation: drooling due to inability to swallow secretions, choking when attempting feeds, usually within first 24 hours of life
  • Etiology: incomplete separation of esophagus from laryngotracheal tube
  • Diagnosis: inability to pass gastric tube (radiographic evaluation)
  • Treatment: surgical
30
Q

Clinical presentation of tracheoesophageal fistula is ________

A

Clinical presentation of tracheoesophageal fistula is drooling due to inability to swallow secretions, choking when attempting feeds, usually within first 24 hours of life

Often associated with other anomalies

31
Q

Etiology of tracheoesophageal fistula is ______

A

Etiology of tracheoesophageal fistula is incomplete separation of esophagus from the laryngotracheal tube

32
Q

Diagnosis of tracheoesophageal fistula is ________

A

Diagnosis of tracheoesophageal fistula is inability to pass gastric tube (radiographic evaluation)

33
Q

Treatment of tracheoesophageal fistula is _______

A

Treatment of tracheoesophageal fistula is surgery

34
Q

Which congenital lung malformations are parenchymal abnormalities?

A
  • Pulmonary sequestration
  • Congenital pulmonary airway malformation
  • Congenital lobar emphysema
35
Q

Parenchymal abnormalities

  • Presentation:
  • Diagnosis:
  • Treatment:
A

Parenchymal abnormalities

  • Presentation: ultrasound in prenatal, respiratory distress in neonatal, recurrent infections in older age
  • Diagnosis: radiographic evaluation
  • Treatment: surgical
36
Q

Clinical presentation of parenchymal abnormalities is _______ in prenatal, _______ in neonatal, and _______ in older age

A

Clinical presentation of parenchymal abnormalities is ultrasound in prenatal, respiratory distress in neonatal, and recurrent infections in older age

37
Q

Diagnosis of parenchymal abnormalities is via _______

A

Diagnosis of parenchymal abnormalities is via radiographic evaluation

38
Q

Treatment of parenchymal abnormalities is ______

A

Treatment of parenchymal abnormalities is surgery

39
Q

What is pulmonary sequestration?

A

Normal non-functioning lung tissue with no connection to the bronchial tree receives blood supply from the systemic circulation (usually descending thoracic aorta or abdominal aorta)

40
Q

What are the types of pulmonary sequestration?

A
  • Intralobar
  • Extralobar
  • Bronchopulmonary foregut malformation: abnormal lung tissue connected to GI tract

(3:1 intralobar:extralobar)

41
Q

Intralobar pulmonary sequestration

  • Definition:
  • Etiology:
  • Other:
A

Intralobar pulmonary sequestration

  • Definition: completely covered by normal lung tissue or by a segment of visceral pleura of the lung lobe within which the sequestration occurs
  • Etiology:
    • Congenital: arising from lung bud
    • Acquired: sequence of bronchial obstruction, pneumonia, parasitization of pulmonary arteries
  • No lobar predominance
  • Venous drainage is via pulmonary veins
  • Equal among males and females
42
Q

Extralobar pulmonary sequestration

  • Definition:
  • Etiology:
  • Other:
A

Extralobar pulmonary sequestration

  • Definition: accessory lung covered by visceral pleura and separated from functioning lung
  • Etiology: congenital
  • Generally in left lower lobe (80%) but some below diaphragm (10%)
  • Venous drainage via systemic venous system (azygos or portal vein)
  • 80% males
43
Q

The etiology of intralobar pulmonary sequestration is _______, while the etiology of extralobar pulmonary sequestration is _______

A

The etiology of intralobar pulmonary sequestration is congenital or acquired, while the etiology of extralobar pulmonary sequestration is congenital

44
Q

Intralobar pulmonary sequestration has _____ lobe predominance, while extralobar pulmonary sequestration has _____ lobe predominance

A

Intralobar pulmonary sequestration has no lobe predominance, while extralobar pulmonary sequestration has left lower lobe predominance

45
Q

Venous drainage in intralobar pulmonary sequestration is via ______, while venous drainage in extralobar pulmonary sequestration is via ______

A

Venous drainage in intralobar pulmonary sequestration is via pulmonary arteries, while venous drainage in extralobar pulmonary sequestration is via systemic venous system (azgos or portal vein)

46
Q

Patients with intralobar pulmonary sequestration are _____ to be males as/than females, while extralobar pulmonary sequestration are _____ to be males as/than females

A

Patients with intralobar pulmonary sequestration are equally likely to be males as females, while extralobar pulmonary sequestration are more likely to be males than females

47
Q

What is congenital pulmonary airways malformation?

A

Congenital pulmonary airways malformation (previously called congenital cystic adenomatoid malformation)

  • Heterogeneous group of congenital cystic and non-cystic lung masses characterized by extensive overgrowth of the primary bronchioles, which are in communication with the abnormal bronchial tree lacking cartilage
  • 25-30% of all congenital lung malformations
  • No lobar or side predominance - most frequently in lower lobes and 85-95% in only one lobe
48
Q

______ are the characteristic imaging finding of congenital pulmonary airways malformation

A

Cysts are the characteristic imaging finding of congenital pulmonary airways malformation

49
Q

What is congenital lobar emphysema?

A

Congenital lobar emphysema

  • Overinflation and distention of one or more pulmonary lobes
  • Etiology: intrinsic (weakness or absence of underlying bronchial cartilage) or extrinsic (mass effect bronchial narrowing with subsequent air trapping)
  • Left upper lobe most frequently affected (rarely bilateral or multifocal)
50
Q

The intrinsic etiology of congenital lobar emphysema is ________, while the extrinsic etiology of congenital lobar emphysema is ________

A

The intrinsic etiology of congenital lobar emphysema is weakness or absence of underlying bronchial cartilage, while the extrinsic etiology of congenital lobar emphysema is mass effect bronchial narrowing with subsequent air trapping

51
Q

Which congenital lung malformations are vascular abnormalities?

A
  • Vascular rings/slings
  • Pulmonary arteriovenous malformations
52
Q

Vascular rings/slings

  • Presentation:
  • Etiology:
  • Diagnosis:
  • Main types:
A

Vascular rings/slings

  • Presentation: trouble swalling, stridor, respiratory distress, recurrent pneumonia or apnea
  • Etiology: developmental abnormality of the aortic arch system which leads to compression of the esophagus and trachea
  • Diagnosis: barium swallow
  • Main types: sling (aberrant left pulmonary artery and right subclavian artery), ring (double aortic arch), many other
53
Q

Clinical presentation of vascular rings/slings is ____, _____, _____, _____, or _____

A

Clinical presentation of vascular rings/slings is trouble swallowing, stridor, respiratory distress, recurrent pneumonia, or apnea

54
Q

Etiology of vascular rings/slings is ________

A

Etiology of vascular rings/slings is developmental abnormality of aortic arch system which leads to compression of the esophagus and trachea

55
Q

Diagnosis of vascular rings/slings is ______

A

Diagnosis of vascular rings/slings is barium swallow

56
Q

Types of vascular rings/slings are _____, ______, and ______

A

Types of vascular rings/slings are sling (aberrant left pulmonary artery and aberrant right subclavian artery), ring (double aortic arch), and things (many other variants)

57
Q

Pulmonary arteriovenous malformations

  • Definition:
  • Presentation:
  • Diagnosis:
  • Treatment:
A

Pulmonary arteriovenous malformations

  • Definition: abnormal connection between arteries and veins
  • Presentation: pulmonary hemorrhage, hemoptysis, or hypoxia
  • Diagnosis: chest radiograph (angiography is second-line)
  • Treatment: embolization
58
Q

Clinical presentation of pulmonary arteriovenous malformations is _____, _____, or ______

A

Clinical presentation of pulmonary arteriovenous malformations is pulmonary hemorrhage, hemoptysis, or hypoxia

59
Q

Diagnosis of pulmonary arteriovenous malformations is ______

A

Diagnosis of pulmonary arteriovenous malformations is chest radiograph

60
Q

Treatment of pulmonary arteriovenous malformations is ______

A

Treatment of pulmonary arteriovenous malformations is embolization

Pulm (32 decks)

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