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Ch 23-24 Flashcards

1
Q

what is choanal atresia

A

a narrowing or blockage of the nasal passage by complete membrane or bony septum

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2
Q

what are the clinical presentations of choanal atresia

A

unilateral: respiratory distress and mucoid blockage
bilateral: emergency and acute respiratory distress or RF

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3
Q

what are the clinical manifestations of choanal atresia

A
  1. Cyanosis and retractions
  2. Inspiratory stridor (unilateral defect)
  3. Pulmonary hypertension (hypoxia/acidosis)
  4. Intolerance of feeding (Unable to nurse and feed simultaneously)
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4
Q

what is the incidence of choanal atresia

A
  1. Occurs in 1/700 births
  2. Females affected by 2:1 ratio over males
  3. Unilateral (most common)
  4. Bilateral (emergency)
  5. 50% have associated congenital abnormalities (Craniofacial or CHARGE)
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5
Q

what does CHARGE stand for

A

C – colobomas (division of eyeballs)

H – congenital heart disease

A – choanal atresia

R – retarded growth/development

G – genital anomalies in males

E – ear anomalies and deafness

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6
Q

what is macroglossia

A

abnormal large tongue

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7
Q

what are the clinical presentations of macroglossia

A
  1. Beckwith-Wiedemann and Downs syndrome
  2. protruding tongue
  3. stridor, snoring
  4. feeding difficulties
  5. later: difficulty with speech and abnormal deviated jaw
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8
Q

what is mandibular hypoplasia (macronathia)

A

incomplete or underdeveloped lower jaw

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9
Q

what are the clinical presentations of mandibular hypoplasia

A
  1. Pierre Robin or Treacher Collins syndrome
  2. Minor to severe respiratory distress
  3. Feeding intolerance (Failure to thrive, malnutrition, hyperextension of neck)
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10
Q

what is tracheoesophageal fistula

A
  1. Esophageal atresia (EA) – narrowing or obstruction of esophagus
  2. Tracheoesophageal fistula (TEF) – connection or hole between the lower esophagus and trachea
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11
Q

what are the clinical presentations of tracheoesophageal fistula

A
  1. 1st symptom – drooling with frothing and bubbling at nose and mouth
  2. Coughing, chocking and cyanosis with feeding (Poor feeding)
  3. Continuous or sporadic respiratory distress -severe and progressive (Wheezing)
  4. Recurrent respiratory infections
  5. Blockage (atresia) and aspiration of gastric/salivary contents (fistula)
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12
Q

what is the incidence of tracheoesophageal fistula

A
  1. 1/3000 births

2. male to female is equal

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13
Q

what are the clinical presentations of congenital diaphragmatic hernia

A
  1. Immediate respiratory distress with associated cyanosis
  2. Chest/abdominal asynchrony
  3. Right sided heart sounds
  4. Bowel sound in chest
  5. Scaphoid abdomen - concave
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14
Q

what is the incidence of congenital diaphragmatic hernia

A
  1. 1/2500 births
  2. bochdaleks hernia
    (90% of left side)
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15
Q

what is omphalacele

A

Abdominal wall abnormality of which variable amounts of the abdominal contents (intestines, liver and other organs) protrude through the base of the umbilical cord (belly button) into a thin-walled sac outside the body at the base of the umbilical cord

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16
Q

what is the incidence of omphalacele

A
  1. 1/5386 births
  2. 1/3 have trisomy 13, 18 or 21
  3. Occurs early in fetal development ( about 10 weeks gestation)
  4. Develops during organogenesis (other defects seen) – cardiac, sternal, hindgut, and thoracic cavity
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17
Q

what are the types of omphalacele

A
  1. epigastric (high) - cephalic fold
  2. classic (midabdominal) - lateral fold
  3. hypogastric (low) - caudal fold
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18
Q

what is gastrschisis

A
  1. A birth defect which there is an opening in abdominal wall to the right of the umbilicus allowing abdominal contents to protrude outside the body without a covering
  2. Separate from umbilicus
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19
Q

what is necrotizing enterocolitis

A

a serious gastrointestinal dieses that predominately affects premature infants

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20
Q

what are the clinical presentations of necrotizing enterocolitis

A
  1. abdominal distention
  2. blood stools
  3. poor feeding
  4. green vomit or gastric drainage
  5. infection with lethargy and apnea
  6. diarrhea
  7. temperature instability
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21
Q

what are complications associated with mandibular hypoplasia

A
  1. increased morbidity due to (aspiration, pulmonary HTN, Cor Pulmonale, failure to thrive)
  2. orthodontic problems
  3. speech dysfunction
  4. chronic ear infection
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22
Q

what are complications of choanal atresia

A
  1. respiratory distress
  2. aspiration with feeding
  3. restenosis
  4. tracheostomy
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23
Q

what are complications of tracheoesophageal fistula

A
  1. apnea or bradycardia
  2. feeding difficulty
  3. gastroesophageal reflux
  4. esophageal dysmotility
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24
Q

what are complications of congenital diaphragmatic hernia

A
  1. pneumothoraces
  2. high morality rate
  3. pulmonary infections
  4. post operative (pulmonary HTN, potential stimulation, hypoxemia, acidosis, hypercarbia, R-L shunt)
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25
what are complications of gastroschisis
1. injury to bowel 2. NEC 3. slow gut syndrome
26
what are complications of necrotizing enterocolitis
1. intestinal stricture 2. intestinal perforation 3. sepsis 4. peritonitis
27
how do you diagnose choanal atresia
1. 8 Fr catheter cant be passed 2. secondary anomalies 3. imaging studies
28
how can choanal atresia be treated
1. resuscitation 2. oral airway (ETT, McGovern, Trach) 3. tube feeding 4. surgical repair
29
how do you diagnose macroglossia
family history and physical exam
30
how do you treat macroglossia
1. proneing - mild 2. polysomnography - severity 3. surgery
31
how do you treat mandibular hypoplasia
1. Prone position with towel rolls under shoulders and head 2. Nasogastric tube/feeding tube to prevent aspiration 3. Surgical correction of cleft palate or tying down posterior base of tongue 4. Tracheostomy (severe cases) 5. Buy time
32
how do you diagnose tracheoesophageal fistula
1. Polyhydramnios (ultrasound) 2. Inability to pass NG tube into stomach 3. Chest and abdominal X-rays 4. Presence or absence of gastric bubble 5. ECHO, renal ultrasound, vertebral spine film
33
how do you treat tracheoesophageal fistula
1. No feeding by mouth 2. NG tube until after surgery 3. Prevent aspiration (raise HOB and timing of procedures) 4. Surgical repair 5. MV – severe respiratory distress and LBW infants
34
how to you diagnose congenital diaphragmatic hernia
1. prenatal ultrasound | 2. post birth (respiratory distress with scaphoid abdomen, CXR, tachypnea)
35
how do you treat congenital diaphragmatic hernia
1. Optimize oxygenation while avoiding barotrauma 2. Large vented OG tube 3. Mask CPAP 4. No bag mask ventilation (PIP/PEEP) 5. Intubate and ventilate gently 6. surfactant 7. HFOV MV 8. surgical repair 9. iNO 10. ECMO
36
how do you diagnose omphalocele
1. prenatal diagnostic testing | 2. post birth physical exam
37
how do you treat omphalcele
1. decompress GI tract 2. MV 3. fluid resuscitation 4. temperature control 5. infection control 6. surgical (primary- small, staged - large)
38
how do you diagnose gastrochitis
prenatally - AEF and ultrasound
39
how do you treat gastrochisis
1. GI decompress 2. MV 3. fluid resuscitation 4. temperature control 5. infection control 6. silo
40
how do you diagnose necrotizing enterocolitis
1. physical exam 2. abdominal xray 3. ultrasound
41
how do you treat necrotizing enterocolitis
1. stop feedings 2. bowel decompression 3. IV fluids 4. surgical removal
42
what CXR is this
congenital diaphragmatic hernia
43
what CXR is this
necrotizing enterocolitis
44
what CXR is this: cardiomegaly and pulmonary vascular congestion
AVSD
45
what CXR is this: pulmonary hypertension and cardiomegaly
coarctation of aorta
46
what CXR is this: boot shaped heart
tetralogy of Fallot
47
what CXR is this: cardiomegaly, increased lung markings and right aortic arch
truncus arteriosus
48
what CXR is this: egg on a string, narrow superior mediastinum, increased vascular flow, convexity of right heart border
transportation of the great arteries
49
what defects are R-L shunting
1. hypoplastic left heart syndrome 2. tetralogy of fallot 3. tricuspid of atresia 4. truncus arteriosus 5. transportation of great arteries 6. total anomalous pulmonary venous return
50
what defects are L-R shunting
1. Atrial septal defect 2. Ventricular septal defect 3. A-V canal defect 4. Patent ductus arteriosus 5. Coarctation of the aorta 6. Pulmonary valve stenosis 7. Aortic valve stenosis
51
what defects are ductal dependant
1. obstructed pulmonary or systemic blood flow 2. PDA presence 3. coarctation of aorta and hypoplastic left heart syndrome
52
what shunt is cyanotic
R-L
53
what shunt is acyanotic
L-R
54
what are the clinical findings of AVSD
a. Respiratory distress b. Pulmonary edema c. Poor growth and feeding d. Diaphoresis e. Heart murmurs f. Hepatomegaly
55
what are the clinical findings of coarctation of the aorta
a. Pale skin b. Irritability c. Diaphoresis d. Respiratory distress e. Heart murmur f. Weak pulses at legs and groin compared to arms or neck
56
what are the clinical findings of hypoplastic left heart syndrome
a. cyanosis b. respiratory distress c. heart murmurs d. weak pulses
57
what are the clinical findings of tetralogy of fallot
a. cyanosis b. tet spells c. auscultation
58
what are the clinical findings of truncus arterosus
a. respiratory distress b. signs of CHF c. cyanosis usually not apparent
59
what are the clinical findings of transportation of the great arteries
a. cyanosis b. respiratory distress c. bounding heart with weak pulses
60
what are the characteristics of AVSD
a. Condition that presents with absence of septa between atria and ventricles
61
what are the characteristics of coarctation of the aorta
a. Severe narrowing of the thoracic aorta that may occurs preductal, juxtaductal or postductal
62
what are the characteristics of hypoplastic left heart syndrome
a. Left sided heart structures develop abnormally resulting in inability to provide enough oxygen rich blood to body
63
what are the characteristics of tetralogy of fallot
a. VSD, pulmonary artery stenosis, aortic overriding to right, right ventricular hypertrophy
64
what are the characteristics of truncus arteriosus
pulmonary artery and aorta are a single vessel
65
what are the characteristics of transportation of the great arteries
pulmonary artery and aorta are reversed
66
how is ductal shunting identified
ABG with PaCO2 >20 mmHg difference
67
how are CHDs diagnosed
ECHO | CXR
68
what is the circulation of blood through heart
1. Right atrium 2. tricuspid valve 3. right ventricle 4. pulmonary artery 5. lungs 6. pulmonary veins 7. left atrium 8. mitral valve 9. left ventricle 9. aortic valve 10. aorta 11. inferior and superior vena cava

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