Oct10 M3-Embryonic congenital anomalies Flashcards
thing in common between resp system and foregut and why
share common origin (primitive pharynx or esophagus gives tracheal bud)
most common atresia and fistula of foregut (2) and type name
esophageal atresia (type A): 8% tracheo-esophageal fistula (bottom esoph) and atresia (of esoph) (type C): 85%
Type B fistula or atresia def
tracheo-esophageal fistula at upper esoph and atresia of esoph
Type D fistula or atresia def
tracheo-esophageal fistulas at upper and lower esophagus + esophageal atresia
Type E fistula or atresia def
tracheo-esophageal fistula
symptoms of esophageal atresia and tracheo-esophageal fistula (4)
excessive salivation, respiratory distress, choking on feeds, MATERNAL POLYHYDRAMNIOS
diagnosis of esophageal atresia and tracheo-esophageal fistula
NG tube through esophagus and look at CXR
bronchopulmonary foregut cyst def
cyst remnant of budding process found on mediastinum
bronchopulmonary foregut cyst composition
GI and respiratory components (GI muscle pattern) and ciliated columnar cell of resp)
bronchopulmonary foregut cyst problems outcomes (3)
- asymptomatic
- aero-digestive tract symptoms
- prone to infection
lung dev at week 4
buds
lung dev at week 5
secondary bronchi form
lung dev at 8 weeks
segmental bronchi form
lung dev at 24 weeks
17 orders of branches have formed
17 orders of branching in the lung: normal prenatally?
yes. 7 additional orders develop after birth
Type A atresia stomach size
smaller stomach bc don’t swallow amniotic fluid
Type A atresia on CXR
Tip of NG tube is high in thorax below neck
Type C atresia on CXR
air in stomach and bowel bc of fistula. tip of NG tube high in thorax below neck
Bronchopulmonary foregut cyst on CXR
shifting of the right of upper thorax organs
4 periods of lung dev and time
pseudoglandular (6-16 wks)
canalicular (16-26 wks)
terminal saccular (26-23 wks)
alveolar (32 wks-8 yrs)
at what phase lung can do gas exchange and in what structure
canalicular phase (at week 26) at terminal saccules (primordial alveoli)
terminal saccular period: what happens
Type 1 cells become thin
Type 2 cells increase and secrete surfactant
Capillaries bulge in developing alveoli
what phase surfactant formed
terminal saccular phase
surfactant deficiency of foetus is when
prenatally until last 4-6 weeks of pregnancy
surfactant gross def
complex mixture of phospholipids
surfactant composition and relevance clinically
Lecithin and Sphingomyelin. If L:S ratio less than 2, fetal surfactant deficiency
how can treat surfactant deficiency before birth (antenataly)
steroid therapy (promotes type 2 pneumocyte development and lung maturation)
consequences of untreated surfactant deficiency (what we try to minimize with the steroid therapy) (2)
Risk of respiratory distress syndrome (RDS)
Risk of hyaline membrane disease (HMD)
how can treat surfactant deficiency after birth (antenataly)
surfactant delivery by tracheal route
surfactant delivery by tracheal route can help treat what other thing
lung hypoplasia in case of diaphragmatic hernia
3 factors required for normal lung dev
- thoracic space for lung growth
- fetal breathing movements
- adequate amniotic fluid volume
Potters syndrome def
congenital kidney agenesis: lack of amniotic fluid. lung hypoplasia
congenital lobar emphysema: what it is
hyperinflation (of affected lobe) and severe air trapping
congenital lobar emphysema: cause
failure of bronchial mesenchyme. dysplastic bronchial cartilage collapses during expiration
congenital cystic adenomatoid malformation (CCAM) def
segment of lung arrested in dev at pseudoglandular period and is cystic and glandular (adenomatoid)
symptoms of CCAM frequency and cause
usually asymptomatic.
symptom causes: large non functional mass or delayed infections
pulmonary sequestration def
non functional mass of normal lung tissue (intra or extra lobal) with systemic vascular supply and not communicating with tracheobronchial tree
2 examples of pleural cavity pathologies
empyema (pleura infection
pneumothorax
fetal circulation main points
right to left heart shunt through foramen ovale. high pulmonary pressure. placenta provides supply with umbilicus
neonatal circulation main points
oxygen is trigger for drop in pulmonary vascular resistance. foramen ovale closes.
4 structures contributing to the diaphragm (development
- septum transversum
- pleuroperitoneal membrane
- mesentery of the esophagus
- muscular ingrowth from body wall
congenital diaphramatic hernia: 2 ways it leads to resp failure
- hernia-hypoplasia of lung-poor gas exchange
- hyperplastic and hypersensitive pulm vasculature-pulm hypertension-rightleft shunt
eventration of diaphragm def
paralysis to do deficient innervation
eventration of diaphragm 2 types
congenital acquired
congenital diaphragm eventration def
defective dev or innervation of diaphragm
acquired diaphragm eventration causes (3)
- injury to phrenic nerve
- traction injury at birth
- iatrogenic surgical injury
diaphragm eventration symptoms
(can be asymptomatic)
- respiratory distress
- abdominal paradox
persistent fetal circulation what and cause
persistent fetal circulation after birth bc hypertension in pulm vasculature
