peds deck 11

Question 1

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

Answer 1

gallstones; cholecystitis

Question 2

_______________ causes hemolysis in the presence of oxidative stressors

Answer 2

G6P dehydrogenase deficiency

Question 3

G6P dehydrogenase deficiency typically present with what s/sx

Answer 3

1. anemia and jaundice 2. followed by abdominal pain, renal failure

Question 4

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

Answer 4

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

Question 5

anesthetic management for the pt with G6P dehydrogenase deficiency

Answer 5

1. keep warm and hydrated 2. decrease pain & anxiety 3. avoid triggering agents: drugs that cause methemoglobinemia 4. tx/eliminate precipitating cause of infection

Question 6

common surgeries that the pt with sickle cell presents for

Answer 6

1. cholecystectomy 2. Tonsillectomy & adenoidectomy 3. other ENT surgery 4. port/line placement 5. frequent MRI to check for strokes

Question 7

preoperative considerations for pt with coagulation d/o

Answer 7

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)

Question 8

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

Answer 8

1. careful with NG 2. careful consideration/use of nasal ETT 3. caution with ketorolac d/t increased risk of bleeding

Question 9

considerations for the pt with hematologic malignancy

Answer 9

1. primary service = onc 2. preop labs and possible blood products 3. immunocompromised: good hand hygiene & strict aseptic technique

Question 10

patients with _____________ are at increase risk for malignancies

Answer 10

trisomy 21

Question 11

common OR procedures the pt with hematologic malignancy presents for

Answer 11

1. central access 2. dental rehabilitation 3. lymph node or other biopsy

Question 12

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

Answer 12

hyperchloremic metabolic acidosis

Question 13

PRBCs can be stored up to ___________ days

Answer 13

42

Question 14

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

Answer 14

15,000; 5,000-10,000

Question 15

indications for cryoprecipitate

Answer 15

1. anticipated or active bleeding in those with congenital fibrinogen def 2. vWF dz unresponsive to DDAVP 3. hypofibrinogenemia d/t massive transfusion

Question 16

what is in cryo

Answer 16

VIII, vwF, fibrinogen, XIII

Question 17

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

Answer 17

DDAVP

Question 18

a massive blood transfusion is defined as ?

Answer 18

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

Question 19

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

Answer 19

40

Question 20

what is the emergency release blood?

Answer 20

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

Question 21

complications of blood transfusion

Answer 21

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

Question 22

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

Answer 22

congenital diaphragmatic hernia

Question 23

dx congenital diaphragmatic hernia prenatally

Answer 23

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

Question 24

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

Answer 24

left (foramen of bochdalek)

Question 25

if congenital diaphragmatic hernia develops early what is the result

Answer 25

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

Question 26

what situation of congenital diaphragmatic hernia would result in no survival

Answer 26

bilateral hypoplastic lungs

Question 27

if congenital diaphragmatic hernia develops later, wht is the result

Answer 27

normal lung that is compressed by the gut

Question 28

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

Answer 28

increased; primary pulmonary htn

Question 29

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

Answer 29

pre-delivery dx

Question 30

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

Answer 30

week 7

Question 31

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

Answer 31

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

Question 32

why are some congenital diaphragmatic hernia patients placed on ECMO

Answer 32

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

Question 33

when is the best time to repair a congenital diaphragmatic hernia

Answer 33

when pt is stable and PVR has decreased

Question 34

anesthesia considerations for congenital diaphragmatic hernia repiar

Answer 34

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

Question 35

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

Answer 35

contralateral pneumothroax

Question 36

what are some different indications for intrauterine surgery

Answer 36

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

Question 37

What is ex-utero intrapartum treatment (EXIT)

Answer 37

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

Question 38

what is the most common type of Tracheoesophageal fistula

Answer 38

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

Question 39

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

Answer 39

tracheoesophageal fistula

Question 40

most common causes of death in pt with tracheoesophageal fistula

Answer 40

1. prematurity 2. associated congential heart defect

Question 41

50% of neonates with tracheoesophageal fistula have an associated ___________________

Answer 41

congenital anomalie (VACTERL)

Question 42

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

Answer 42

type II

Question 43

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

Answer 43

IIIA

Question 44

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

Answer 44

IIIB (most common)

Question 45

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

Answer 45

IIIC

Question 46

clinical presentations/diagnosing TEF

Answer 46

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

Question 47

what are the primary concerns with TEF

Answer 47

1. aspiration pna 2. dehydration

Question 48

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

Answer 48

ones with blind pouch (IIIA, B, C)

Question 49

placement of ETT prior to TEF surgery

Answer 49

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

Question 50

anesthesia consideration for pt presenting for TEF surgery

Answer 50

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