peds deck 11 Flashcards

1
Q

children with hereditary spherocytosis will only have cholecystecomy if have __________ & ___________

A

gallstones; cholecystitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

_______________ causes hemolysis in the presence of oxidative stressors

A

G6P dehydrogenase deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

G6P dehydrogenase deficiency typically present with what s/sx

A
  1. anemia and jaundice 2. followed by abdominal pain, renal failure
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what medications would you want to avoid in a pt with G6P dehydrogenase deficiency?

A

any medications that cause methemoglobinemia: 1. hydralazine 2. nitroprusside 3. bactrim 4. sulfonamides 5. nitroglycerin 6. LA: benzocaine, lidocaine, procainamide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

anesthetic management for the pt with G6P dehydrogenase deficiency

A
  1. keep warm and hydrated 2. decrease pain & anxiety 3. avoid triggering agents: drugs that cause methemoglobinemia 4. tx/eliminate precipitating cause of infection
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

common surgeries that the pt with sickle cell presents for

A
  1. cholecystectomy 2. Tonsillectomy & adenoidectomy 3. other ENT surgery 4. port/line placement 5. frequent MRI to check for strokes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

preoperative considerations for pt with coagulation d/o

A
  1. may need to come in early (but not first case) to have factor given 2. ports may need to be accessed or PIV started 3. if need preop labs - consider the time it takes to get those results back 4. hematology consult 5. may need DDAVP (increase release of vWF and VIII)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

considerations for airway, NG/OG and meds for pt with coagulation d/o

A
  1. careful with NG 2. careful consideration/use of nasal ETT 3. caution with ketorolac d/t increased risk of bleeding
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

considerations for the pt with hematologic malignancy

A
  1. primary service = onc 2. preop labs and possible blood products 3. immunocompromised: good hand hygiene & strict aseptic technique
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

patients with _____________ are at increase risk for malignancies

A

trisomy 21

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

common OR procedures the pt with hematologic malignancy presents for

A
  1. central access 2. dental rehabilitation 3. lymph node or other biopsy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

large amount of NS may produce _______________ compared to other balanced electroyte solutions

A

hyperchloremic metabolic acidosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

PRBCs can be stored up to ___________ days

A

42

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

1 unit of plts will increase plt count by ____________ in children and ______________ in adults

A

15,000; 5,000-10,000

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

indications for cryoprecipitate

A
  1. anticipated or active bleeding in those with congenital fibrinogen def 2. vWF dz unresponsive to DDAVP 3. hypofibrinogenemia d/t massive transfusion
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what is in cryo

A

VIII, vwF, fibrinogen, XIII

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

_____________ can tx coagulopathies associated with plt dysfunction, uremia, and cirrhosis

A

DDAVP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

a massive blood transfusion is defined as ?

A

30 mL/kg PRBC or a replacement of entire blood volume in < 4 hours

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

risk of mortality with massive blood transfusion increases when >/= _________ ml/kg of PRBC is administered to a pediatric pt

A

40

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

what is the emergency release blood?

A

O-; but as transfusing and det pts blood type need to switch to that blood asap

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

complications of blood transfusion

A
  1. coagulopathy 2. hypothermia 3. dilutional thrombocytopenia/factor deficiency 4. DIC 5. hyperkalemia 6. hypocalcemia (more with FFP than PRBC) 7. acidosis
22
Q

_______________ is failure of complete closure of the pleural and periotneal cavity –> herniation of abdominal contents into the thorax inhibiting normal lung growth

A

congenital diaphragmatic hernia

23
Q

dx congenital diaphragmatic hernia prenatally

A
  1. U/S = polyhydraminos, intrathoracic gastric bubble, and/or mediastinal shift from herniation site 2. amniocentesis = low levels of alpha-fetoprotein
24
Q

congenital diaphragmatic hernia occurs on the _______________ side more commonly due to later closure

A

left (foramen of bochdalek)

25
Q

if congenital diaphragmatic hernia develops early what is the result

A
  1. a lot gut pressing on the chest 2. compression of developing lung bud 3. results in very small, hypoplastic lung
26
Q

what situation of congenital diaphragmatic hernia would result in no survival

A

bilateral hypoplastic lungs

27
Q

if congenital diaphragmatic hernia develops later, wht is the result

A

normal lung that is compressed by the gut

28
Q

congenital diaphragmatic hernia can inhibit normal lung growth –> ___________ PVR and ___________

A

increased; primary pulmonary htn

29
Q

__________________ is extremely important to survival in patients with congenital diaphragmatic hernia

A

pre-delivery dx

30
Q

what week of inutero development does the diaphragm develop separating the thoracic and abdominal cavities

A

week 7

31
Q

what are the different scenarios for surgical intervention for congenital diaphragmatic hernia

A
  1. delay until pt is stable (24-48 hour to 1 week) 2. if on ECMO preoperatively –> weaned from ECMO then scheduled for surgery 3. can have surgery on ECMO and remain on ECMO postop up to 30 days
32
Q

why are some congenital diaphragmatic hernia patients placed on ECMO

A

due to respiratory compromise and allows times for the surfactant system to mature

33
Q

when is the best time to repair a congenital diaphragmatic hernia

A

when pt is stable and PVR has decreased

34
Q

anesthesia considerations for congenital diaphragmatic hernia repiar

A
  1. avoid harmful effects of volutrauma (maintain small freq Tv and limit PIP <30 mmHg) 2. avoid conditions that increase PVR (hypoxemia, acidosis, hypothermia, hypercarbia) 3. avoid N2O - expands tissues 4. most often remain intubated postop 5. may extubate if lungs are normal/small hernia 6. NGT should be placed prior to induction to empty stomach
35
Q

if you need high PIP during surgery for congenital hernia repair, what could be the result?

A

contralateral pneumothroax

36
Q

what are some different indications for intrauterine surgery

A
  1. twin-twin transfusion syndrome 2. congenital diaphragmatic hernia 3. neck masses (cystic hygroma) 4. neural tube defects (spina bifida)
37
Q

What is ex-utero intrapartum treatment (EXIT)

A

prior to clamping the cord, the neonate is intubated and ventilated

38
Q

what is the most common type of Tracheoesophageal fistula

A

Type IIIB (blind proximal esophageal pouch with distal TEF just above the carina)

39
Q

_______________ is due to an error in separation of the tracheal from the floor of the foregut around the 4-5 week of gestation

A

tracheoesophageal fistula

40
Q

most common causes of death in pt with tracheoesophageal fistula

A
  1. prematurity 2. associated congential heart defect
41
Q

50% of neonates with tracheoesophageal fistula have an associated ___________________

A

congenital anomalie (VACTERL)

42
Q

fully formed esophagus, but has fistula coming off trachea into esophagus

A

type II

43
Q

fistula into a blind pouch, then there is distance between that and the second blind pouch attached to stomach

A

IIIA

44
Q

blind pouch, and then have a fistula above the carina which goes down and into the stomach

A

IIIB (most common)

45
Q

2 fistulas - you have a fistula into the blind pouch and also have fisula connected to more distal esophagus which goes down to stomach

A

IIIC

46
Q

clinical presentations/diagnosing TEF

A
  1. polyhdraminos (inability of fetus to swallow amniotic fluid) 2. polyhydraminos on U/s –> attempt to place NGT and find inability to place 3. early post natal presentation of excess salivation, choking/coughing/regurgitation with first feed 4. distended abdomen
47
Q

what are the primary concerns with TEF

A
  1. aspiration pna 2. dehydration
48
Q

which type of TEF may a G-tube be needed prior to repair due to the high degree of risk for refulx/pna

A

ones with blind pouch (IIIA, B, C)

49
Q

placement of ETT prior to TEF surgery

A

should be placed ABOVE carina and BELOW fistula AVOID excessive PPV consider doing with FOB to ensure proper placement

50
Q

anesthesia consideration for pt presenting for TEF surgery

A
  1. avoid masking and intubation until surgery d/t increased gastric distension 2. ETT placed ABOVE carina and BELOW fistual 3. if G-tube present –> open to air and keep at head of table to avoid kinking/obstruction 4. consider a line if have cardiac abn 5. remain intubated post procedure & paralyzed 6. avoid post repair neck extension 7. minimal pain post op