peds deck 11 Flashcards
children with hereditary spherocytosis will only have cholecystecomy if have __________ & ___________
gallstones; cholecystitis
_______________ causes hemolysis in the presence of oxidative stressors
G6P dehydrogenase deficiency
G6P dehydrogenase deficiency typically present with what s/sx
- anemia and jaundice 2. followed by abdominal pain, renal failure
what medications would you want to avoid in a pt with G6P dehydrogenase deficiency?
any medications that cause methemoglobinemia: 1. hydralazine 2. nitroprusside 3. bactrim 4. sulfonamides 5. nitroglycerin 6. LA: benzocaine, lidocaine, procainamide
anesthetic management for the pt with G6P dehydrogenase deficiency
- keep warm and hydrated 2. decrease pain & anxiety 3. avoid triggering agents: drugs that cause methemoglobinemia 4. tx/eliminate precipitating cause of infection
common surgeries that the pt with sickle cell presents for
- cholecystectomy 2. Tonsillectomy & adenoidectomy 3. other ENT surgery 4. port/line placement 5. frequent MRI to check for strokes
preoperative considerations for pt with coagulation d/o
- 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)
considerations for airway, NG/OG and meds for pt with coagulation d/o
- careful with NG 2. careful consideration/use of nasal ETT 3. caution with ketorolac d/t increased risk of bleeding
considerations for the pt with hematologic malignancy
- primary service = onc 2. preop labs and possible blood products 3. immunocompromised: good hand hygiene & strict aseptic technique
patients with _____________ are at increase risk for malignancies
trisomy 21
common OR procedures the pt with hematologic malignancy presents for
- central access 2. dental rehabilitation 3. lymph node or other biopsy
large amount of NS may produce _______________ compared to other balanced electroyte solutions
hyperchloremic metabolic acidosis
PRBCs can be stored up to ___________ days
42
1 unit of plts will increase plt count by ____________ in children and ______________ in adults
15,000; 5,000-10,000
indications for cryoprecipitate
- anticipated or active bleeding in those with congenital fibrinogen def 2. vWF dz unresponsive to DDAVP 3. hypofibrinogenemia d/t massive transfusion
what is in cryo
VIII, vwF, fibrinogen, XIII
_____________ can tx coagulopathies associated with plt dysfunction, uremia, and cirrhosis
DDAVP
a massive blood transfusion is defined as ?
30 mL/kg PRBC or a replacement of entire blood volume in < 4 hours
risk of mortality with massive blood transfusion increases when >/= _________ ml/kg of PRBC is administered to a pediatric pt
40
what is the emergency release blood?
O-; but as transfusing and det pts blood type need to switch to that blood asap
complications of blood transfusion
- coagulopathy 2. hypothermia 3. dilutional thrombocytopenia/factor deficiency 4. DIC 5. hyperkalemia 6. hypocalcemia (more with FFP than PRBC) 7. acidosis
_______________ is failure of complete closure of the pleural and periotneal cavity –> herniation of abdominal contents into the thorax inhibiting normal lung growth
congenital diaphragmatic hernia
dx congenital diaphragmatic hernia prenatally
- U/S = polyhydraminos, intrathoracic gastric bubble, and/or mediastinal shift from herniation site 2. amniocentesis = low levels of alpha-fetoprotein
congenital diaphragmatic hernia occurs on the _______________ side more commonly due to later closure
left (foramen of bochdalek)
if congenital diaphragmatic hernia develops early what is the result
- a lot gut pressing on the chest 2. compression of developing lung bud 3. results in very small, hypoplastic lung
what situation of congenital diaphragmatic hernia would result in no survival
bilateral hypoplastic lungs
if congenital diaphragmatic hernia develops later, wht is the result
normal lung that is compressed by the gut
congenital diaphragmatic hernia can inhibit normal lung growth –> ___________ PVR and ___________
increased; primary pulmonary htn
__________________ is extremely important to survival in patients with congenital diaphragmatic hernia
pre-delivery dx
what week of inutero development does the diaphragm develop separating the thoracic and abdominal cavities
week 7
what are the different scenarios for surgical intervention for congenital diaphragmatic hernia
- 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
why are some congenital diaphragmatic hernia patients placed on ECMO
due to respiratory compromise and allows times for the surfactant system to mature
when is the best time to repair a congenital diaphragmatic hernia
when pt is stable and PVR has decreased
anesthesia considerations for congenital diaphragmatic hernia repiar
- 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
if you need high PIP during surgery for congenital hernia repair, what could be the result?
contralateral pneumothroax
what are some different indications for intrauterine surgery
- twin-twin transfusion syndrome 2. congenital diaphragmatic hernia 3. neck masses (cystic hygroma) 4. neural tube defects (spina bifida)
What is ex-utero intrapartum treatment (EXIT)
prior to clamping the cord, the neonate is intubated and ventilated
what is the most common type of Tracheoesophageal fistula
Type IIIB (blind proximal esophageal pouch with distal TEF just above the carina)
_______________ is due to an error in separation of the tracheal from the floor of the foregut around the 4-5 week of gestation
tracheoesophageal fistula
most common causes of death in pt with tracheoesophageal fistula
- prematurity 2. associated congential heart defect
50% of neonates with tracheoesophageal fistula have an associated ___________________
congenital anomalie (VACTERL)
fully formed esophagus, but has fistula coming off trachea into esophagus
type II
fistula into a blind pouch, then there is distance between that and the second blind pouch attached to stomach
IIIA
blind pouch, and then have a fistula above the carina which goes down and into the stomach
IIIB (most common)
2 fistulas - you have a fistula into the blind pouch and also have fisula connected to more distal esophagus which goes down to stomach
IIIC
clinical presentations/diagnosing TEF
- 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
what are the primary concerns with TEF
- aspiration pna 2. dehydration
which type of TEF may a G-tube be needed prior to repair due to the high degree of risk for refulx/pna
ones with blind pouch (IIIA, B, C)
placement of ETT prior to TEF surgery
should be placed ABOVE carina and BELOW fistula AVOID excessive PPV consider doing with FOB to ensure proper placement
anesthesia consideration for pt presenting for TEF surgery
- 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
