Oct10 M3-Embryonic congenital anomalies Flashcards

1
Q

thing in common between resp system and foregut and why

A

share common origin (primitive pharynx or esophagus gives tracheal bud)

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2
Q

most common atresia and fistula of foregut (2) and type name

A
esophageal atresia (type A): 8%
tracheo-esophageal fistula (bottom esoph) and atresia (of esoph) (type C): 85%
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3
Q

Type B fistula or atresia def

A

tracheo-esophageal fistula at upper esoph and atresia of esoph

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4
Q

Type D fistula or atresia def

A

tracheo-esophageal fistulas at upper and lower esophagus + esophageal atresia

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5
Q

Type E fistula or atresia def

A

tracheo-esophageal fistula

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6
Q

symptoms of esophageal atresia and tracheo-esophageal fistula (4)

A

excessive salivation, respiratory distress, choking on feeds, MATERNAL POLYHYDRAMNIOS

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7
Q

diagnosis of esophageal atresia and tracheo-esophageal fistula

A

NG tube through esophagus and look at CXR

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8
Q

bronchopulmonary foregut cyst def

A

cyst remnant of budding process found on mediastinum

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9
Q

bronchopulmonary foregut cyst composition

A

GI and respiratory components (GI muscle pattern) and ciliated columnar cell of resp)

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10
Q

bronchopulmonary foregut cyst problems outcomes (3)

A
  • asymptomatic
  • aero-digestive tract symptoms
  • prone to infection
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11
Q

lung dev at week 4

A

buds

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12
Q

lung dev at week 5

A

secondary bronchi form

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13
Q

lung dev at 8 weeks

A

segmental bronchi form

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14
Q

lung dev at 24 weeks

A

17 orders of branches have formed

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15
Q

17 orders of branching in the lung: normal prenatally?

A

yes. 7 additional orders develop after birth

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16
Q

Type A atresia stomach size

A

smaller stomach bc don’t swallow amniotic fluid

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17
Q

Type A atresia on CXR

A

Tip of NG tube is high in thorax below neck

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18
Q

Type C atresia on CXR

A

air in stomach and bowel bc of fistula. tip of NG tube high in thorax below neck

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19
Q

Bronchopulmonary foregut cyst on CXR

A

shifting of the right of upper thorax organs

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20
Q

4 periods of lung dev and time

A

pseudoglandular (6-16 wks)
canalicular (16-26 wks)
terminal saccular (26-23 wks)
alveolar (32 wks-8 yrs)

21
Q

at what phase lung can do gas exchange and in what structure

A

canalicular phase (at week 26) at terminal saccules (primordial alveoli)

22
Q

terminal saccular period: what happens

A

Type 1 cells become thin
Type 2 cells increase and secrete surfactant
Capillaries bulge in developing alveoli

23
Q

what phase surfactant formed

A

terminal saccular phase

24
Q

surfactant deficiency of foetus is when

A

prenatally until last 4-6 weeks of pregnancy

25
Q

surfactant gross def

A

complex mixture of phospholipids

26
Q

surfactant composition and relevance clinically

A

Lecithin and Sphingomyelin. If L:S ratio less than 2, fetal surfactant deficiency

27
Q

how can treat surfactant deficiency before birth (antenataly)

A

steroid therapy (promotes type 2 pneumocyte development and lung maturation)

28
Q

consequences of untreated surfactant deficiency (what we try to minimize with the steroid therapy) (2)

A

Risk of respiratory distress syndrome (RDS)

Risk of hyaline membrane disease (HMD)

29
Q

how can treat surfactant deficiency after birth (antenataly)

A

surfactant delivery by tracheal route

30
Q

surfactant delivery by tracheal route can help treat what other thing

A

lung hypoplasia in case of diaphragmatic hernia

31
Q

3 factors required for normal lung dev

A
  • thoracic space for lung growth
  • fetal breathing movements
  • adequate amniotic fluid volume
32
Q

Potters syndrome def

A

congenital kidney agenesis: lack of amniotic fluid. lung hypoplasia

33
Q

congenital lobar emphysema: what it is

A

hyperinflation (of affected lobe) and severe air trapping

34
Q

congenital lobar emphysema: cause

A

failure of bronchial mesenchyme. dysplastic bronchial cartilage collapses during expiration

35
Q

congenital cystic adenomatoid malformation (CCAM) def

A

segment of lung arrested in dev at pseudoglandular period and is cystic and glandular (adenomatoid)

36
Q

symptoms of CCAM frequency and cause

A

usually asymptomatic.

symptom causes: large non functional mass or delayed infections

37
Q

pulmonary sequestration def

A

non functional mass of normal lung tissue (intra or extra lobal) with systemic vascular supply and not communicating with tracheobronchial tree

38
Q

2 examples of pleural cavity pathologies

A

empyema (pleura infection

pneumothorax

39
Q

fetal circulation main points

A

right to left heart shunt through foramen ovale. high pulmonary pressure. placenta provides supply with umbilicus

40
Q

neonatal circulation main points

A

oxygen is trigger for drop in pulmonary vascular resistance. foramen ovale closes.

41
Q

4 structures contributing to the diaphragm (development

A
  • septum transversum
  • pleuroperitoneal membrane
  • mesentery of the esophagus
  • muscular ingrowth from body wall
42
Q

congenital diaphramatic hernia: 2 ways it leads to resp failure

A
  • hernia-hypoplasia of lung-poor gas exchange

- hyperplastic and hypersensitive pulm vasculature-pulm hypertension-rightleft shunt

43
Q

eventration of diaphragm def

A

paralysis to do deficient innervation

44
Q

eventration of diaphragm 2 types

A

congenital acquired

45
Q

congenital diaphragm eventration def

A

defective dev or innervation of diaphragm

46
Q

acquired diaphragm eventration causes (3)

A
  • injury to phrenic nerve
  • traction injury at birth
  • iatrogenic surgical injury
47
Q

diaphragm eventration symptoms

A

(can be asymptomatic)

  • respiratory distress
  • abdominal paradox
48
Q

persistent fetal circulation what and cause

A

persistent fetal circulation after birth bc hypertension in pulm vasculature