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Flashcards in Peds Deck (92):
1

What are the most common causes of respiratory distress in newborns?

- Transient tachypnea of newborn: residual pulmonary fluid in lung
- Hyaline membrane disease / respiratory distress syndrome
- Meconium aspiration syndrome

2

In a newborn with respiratory distress, what should we do to confirm/rule out common surgical diagnoses?

Place OGT/NGT
Get CXR

3

What signs in a newborn suggest congenital diaphragmatic hernia (CDH)?

Severe resp distress
Absent breath sounds
Scaphoid abdomen

4

How is CDH usually diagnosed?

In utero: prenatal US

5

Where do CDH most commonly occur?

L side: posterolateral/Bochdalek hernia

6

What does herniation of abdominal contents in CDH cause?

Pulmonary hypoplasia: ipsi and contra

7

What is the pathophysiology of respiratory distress in CDH?

Pulm HTN --> Decreased pulm blood flow / hypoxia
Pulm hypoplasia --> decreased gas exchange and CO2 retention

8

What common associated anomalies occur with CDH?

Chromosomal defects
Rotational
Cardiac (VSD/ASD)
CNS
Limb
GU

9

What is mortality related to in CDH?

Degree of pulmonary hypoplasia / Pulmonary HTN
Presence of congenital anomalies

10

What is the first step in management of newborn respiratory distress?

Immediate intubation

11

What is the first step in management of newborn respiratory distress?

Immediate intubation

12

What is survival related to in CDH?

Degree of pulmonary hypoplasia and HTN

13

What is the goal of treatment for CDH?

provide pulmonary support w/o further damaging the lungs

14

When is the optimal timing for surgery for CDH: immediate or wait?

Delay: allow lungs to mature and pulmonary HTN to improve

15

Before surgery for CDH, what must be done?

Evaluate for other congenital anomalies

16

Why must blow-by oxygen or excessive bag mask ventilation be avoided in CDH?

Worsens lung compression and mediastinal shift

17

What happens with CDH?

Failure of septum transversum to completely divide the pleural and coleomic cavities during fetal development

18

What's the most important step in clinical management for CDH?

Immediate intubation

19

What's the best initial diagnostic test for newborn respiratory distress?

OGT + CXR

20

What do we think: bilious vomiting in a newborn 0-1 month?

Surgical problem until proven otherwise

21

Does passage of meconium rule out obstruction?

No: mucuous and epithelium is shed along entire length of intestine - distal to the obstruction, it may still pass!

22

In a stable newborn with bilious vomiting, what is the first step?

Plain abdominal XR to rule out perforation, prox vs. distal obstruction, presence or absence of distal gas

23

What are the key features on XR of duodenal obstruction/atresia?

Double bubble sign + no distal gas

24

If a newborn with bilious vomiting has distal gas, what's the most likely diagnosis?

Malrotation + midgut volvulus before duodenal web or partial duodenal obstruction

25

What causes duodenal atresia?

failure of recanalization early in development

26

>50% of patients with duodenal atresia have what associated anomalies?

Trisomy 21
Annular pancreas
Cardiac (most common)

27

What is the management for a newborn with bilious vomiting?

1. Get IV access: Correct fluid/electrolyte abnormalities
2. Place NGT
3. Rule out other anomalies prior to surgery

28

If a newborn with bilious vomiting is unstable, what do we suspect? Next steps?

Suspect malrotation with midgut volvulus
Go to OR emergently

29

What is the procedure of choice for duodenal atresia?

Duodenoduodenostomy

30

How can duodenal atresia present with non bilious vomiting?

Obstruction proximal to the ampulla: present with nonbilious emesis

31

What key comorbidity must be identified before surgery for duodenal atresia? Why?

Annular pancreas: Injury to the ring of pancreatic tissue can lead to pancreatic enzyme leak and pancreatitis

32

What is bilious emesis?

Green or yellow emesis

33

What type a problem is bilious emesis in an infant?

Surgical until proven otherwise!

34

In a stable infant with bilious emesis, what is the first step?

Plain abdominal radiograph first to exclude gross perforation

35

If initial XR is negative in an infant with bilious emesis, what is the next step?

UGI contrast study: evaluate duodenum and proximal SI

36

In infants with bilious vomiting, what MUST we suspect first due to danger?

Malrotation with midgut volvulus

37

What is the midgut embryologically?

SMA: Second portion of duodenum to 2/3 of transverse colon

38

What is malrotation in the gut due to?

Developmental failure of normal 270 degree counterclockwise midgut rotation

39

What is the classic pattern of congenital malrotation?

narrow mesenteric base
Ligament of Treitz located R of midline, cecum in epigastrium, Ladd's bands from cecum to RUQ crossing duodenum

40

What is a volvulus in setting of malrotation?

Midgut rotates around SMA axis causing duodenal obstruction and vascular compromise of bowel

41

What is the classic UGI radiograph of volvulus in setting of malrotation?

"corkscrew" appearance of contrast in bowel lumen

42

What is the management of treating midgut volvulus?

1. Place NGT to decompress stomach
2. Give ABx / IVF while prepping for laparotomy
3. Laparotomy

43

What is the first management step of treating hemodynamically unstable acute GI obstruction in an infant?

1. Rapid fluid resuscitation
2. Immediate surgical intervention w/o additional studies!

44

What is Ladd's procedure?

Relieving obstruction by untwisting the bowel and brooding the mesenteric base to prevent future episodes

45

How can malrotation with midgut volvulus present (2 ways)?

Bilious or non bilious vomiting depending on location

46

What is the most common bowel gas pattern in malrotation with midgut volvulus?

Normal

47

During surgery in an infant with bilious emesis, what must be ruled out?

Duodenal stenosis / atresia

48

In newborn with bilious emesis whose XR shows proximal obstruction: distal bowel gas + "double bubble", what is the next step?

UGI contrast study: should show malrotation +- midgut volvulus

49

In a newborn with bilious emesis whose XR shows "double bubble" w/o distal gas, what is the diagnosis?

Duodenal atresia

50

In an newborn with bilious emesis whose XR shows distal obstruction with multiple loops of dilated small bowel, what is the next step and likely diagnoses?

Contrast enema:
- Intestinal atresia
- Meconium ileus
- Hirschsprung's disease

51

Of patients with non bilious emesis in infancy, which disease are managed medically?

Acute gastroenteritis
GERD
Metabolic d/o
Pylorospasm

52

Of patients with non bilious emesis in infancy, which disease are managed surgically?

Antral web
Enteric duplication cyst
GERD
Pyloric atresia / stenosis

53

What is the most common surgical cause of non bilious vomiting in an infant?

Hypertrophic pyloric stenosis (HPS)

54

What is classic PE sign of HPS?

Palpable RUQ "olive" mass with visible peristalsis over the epigastrium

55

If diagnosis of HPS is unclear, what should be done?

US

56

What is the diagnostic criteria for HPS?

- Pyloric length > 15mm
- Thickness > 3mm

57

What electrolyte abnormalities are common in HPS?

- Hypochloremic, hypokalemic, metabolic acidosis
- Paradoxical aciduria

58

What is pathophysiology of HPS?

Hyperplasia and hypertrophy of pylorus --> GOO

59

What is the first treatment for HPS?

1. Fluid resuscitation
2. Correct electrolyte imbalances

60

What is the gold standard surgery for HPS?

Ramstedt pyloromyotomy: incise and split the muscular layers, leaving the mucosa and submucosa intact

61

When is the optimal timing of surgery for HPS?

Delay until infant resuscitated and electrolyte levels are normal

62

When does feeding start after HPS repair? What is common?

After surgery - vomiting may occur but should resolve

63

If a patient has equivocal US suspicious for HPS or concern for malrotation with midgut volvulus, what else should be done? What is the risk

Contrast UGI
- Risk of aspiration

64

Is HPS a medical or surgical emergency?

Medical!

65

If an infant has persistent fever after surgery for HPS, what should be considered?

incomplete pyloromyotomy

66

If an infant has HPS surgery and has post-op fever and tachycardia, what must it be until proven otherwise?

perforation

67

How do we diagnose abdominal wall defects in newborns?

Prenatal ultrasound usually

68

What are key identifying features of gastroschisis?

Paraumbilical (R) and exposed bowel

69

What are key identifying features of omphalocele?

Sac from the umbilicus, associated with more congenital defects

70

What are the associated syndromes with omphalocele?

Beckwith-Wiedemann Syndrome
Trisomies 13, 18
Pentalogy of Cantrell

71

What is the etiology of gastroschisis?

In utero vascular insult causing abdominal wall defect

72

What is the etiology of omphalocele?

Arrest of cell migration causing incomplete return of midgut to the peritoneal cavity

73

What is the most important first 2 steps in management of abdominal wall defect in newborn?

1. Ventilation: secure airway
2. Normothermia and fluid management:
- Radiant heater
- Orogastric suction
- Protect exposed viscera with plastic wrap
- IV fluids + broad spectrum Abx

74

What is the definitive treatment for gastroscisis or omphalocele?

- Protective silo and serial reduction OR
- Surgical repair

75

What must a gastroschisis patient be evaluated for?

Atresia, ischia or volvulus

76

What must be given to a gastroschisis patient while they are awaiting reduction of the bowel or surgery? Why?

TPN - intestine likely inflamed

77

What are key considerations after post-op repair of gastroschisis or omphalocele?

1. Ventilator if needed
2. TPN if needed
3. Monitor for abdominal compartment syndrome!

78

What's in the differential for feeding intolerance in a newborn?

- Anatomic malformation of naso- and oropharynx, tracheobronchial tree and esophagus
- GERD
- Extrinsic esophageal compression
- Food sensitivity
- Neurologic d/o

79

What do desaturations only while feeding imply in a newborn?

Anatomic or functional problem w/ proximal aerodigestive tract

80

What is the best initial diagnostic test in a newborn with feeding intolerance?

AP and lateral chest XR after NG/OGT placement

81

What is the pathophysiology of EA +/- TEF?

Defect in development of longitudinal tracheoesophageal fold separating foregut into trachea and esophagus

82

What is the most common type of TEF?

Distal: Type C: Trachea with esophagus going off of it

83

Most patients with TEF +/- EA have what type of anomalies?

VACTERL:
Vertebral
Anorectal
CV
Tracheoesophageal
Renal
Limb

84

In patients with TEF +/- EA, what is prognosis for those with comorbidites or requiring surgery?

Excellent w/o comorbidiites
* Significant sequelae from surgery!

85

What is management in newborns with respiratory compromise?

Intubation/mechanical ventilation

86

What is the preoperative management goal in patient with TEF +/- EA?

Minimize risk of aspiration: NGT of upper esophageal pouch, head elevation, AB

87

In patients with TEF +/- EA, what must be done before surgery?

Eval for associated anomalies: echo, renal US, XRays

88

What is the surgical repair for TEF +/- EA?

Division of fistula tract, repair of trachea, primary anastomosis of esophagus

89

What are the post-operative complications from surgery for TEF +/- EA and how common are they?

Common!
- Anastomotic leak
- Stricture
- GERD

90

Why do we avoid contrast esophagram in newborns with suspected TEF +/- EA?

Risk of aspiration pneumonitis

91

Why do we avoid distending GI tract in patients with TEF +/- EA?

Avoid further aspiration and lung injury, esp in patients on ventilator support

92

What must be done before surgery for TEF +/- EA?

Assess vascular anatomy:
- IVC can drain into RA via azygous system
- 5% patients have R sided aortic arch