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cleft lip and palate - what is it
monst common cogenital craniofacial anomaly
development starts early in pregnancy
lip d/t improper fuse during embryonic stage at week 5
palate d/t absence of midline fusion by week 7
can occur together or on there own
can be unilateral or bilateral
30% have genetic syndrome
claft lip/palate - risk factors
maternal smoking
prenatal infection
advanced maternal age
medication use - anticonvulsants, steroids
cleft lip/palate - complications
feedings difficulties: improper suction, excessive feeding times, inadequate intake, nasal regurgitation
altered dentition: missing, malformed, malposition
delayed or altered speech development
otits media: fluid buildup in mid ear, temporary/permanent loss of hearing
Cleft lip/palate - therepeutic management
repair of lip: 2-3months of age
surgical repair of palate: 6-9 months of age
revisions necessary as time grows
cleft lip/palate - nursing management
prevent injury to suture line
promote adequate nutrition
encourage infant parent bonding
provide emotional support
esophageal artesia - what is it
congenitally interrupted esophagus
the poximal and distal ends dont communicate
transeophageal fistula
abnormal communication between trachea and esophagus
esophageal artesia/ transesophageal fistula - clinical manifestations
polyhydraminos is first indication - ususally w/ artesia
excessive salivaiton (frothy)
drooling
abd distention
unable to pass feeding tube
rattling resp.
the 3 Cs - coughing, choking, cyanosis
esophageal artesia/ transesophageal fistula - nursing management
preop: NPO, baseline Vitals, Assessments, labs, IV insertion
Postop: incision care/monitoring, NG tube proteciton, IV fluids, O2, nutrition, pain management
omphalocele - what is it
defect of the umbilical ring allows evisceration of abd content into external periotneal sac
Gastroschisis - what is it
herniation of abd wall contents d/t abd wall defect
usually to left or right of umbilicus
no membrane
requires pediatric surgeon available at delivery
omphalocele/Gastroschisis - nursing assessment
review maternal hx
illness and infxn
drug use, smoking
genetic abnormalities
associated cogenital anomalies
cardiovascular
genitourinary
CNS
omphalocele/Gastroschisis - Nursing management
preventing hypothermia
maintain perfusion to eviscerated abd contents
protect exposed abd contents
minimize fluid loss
prevent trauma and infxn
postop care
promote parent newborn interaction
dehydration
occurs more readily in infants and children
inc. extracellular fluid percent
inc. in body water
inc. metabolic rate
inc. ratio BSA to body mass
immature renal fxn
inc. insensible loss through temp eval.
these can also cause overhydration
dehydration - s/sx
sunken fontanelle
sunken eyes
lack of tears when crying
excessive thirst but vomits after drinking lots of water
dec. UO
infants/babies - no wet diapers in 6-8hrs, little dark yellow urine
toddlers - very little dark yellow urine
dehydration - history
diarrhea
vomitting
dec. oral intake
sustained high fever
DKA
extensive burns
dehydration - nursing assessment
hydration status
heart rate
BP
skin tugor
fontanels
oral mucosa
eyes
temp and color of extremeties
mental status
UO
dehydration - nursing management
promote F/E balance
oral rehydration (pedialyte) - should have 75mmol/L NaCl and 13.5 g/L glucose
tap water, milk, undiluted fruit juice, soup, broth = not good for this
children w/ mild to moderate require 50-100ml/kg ORS over 4 hrs
mild to moderate - withhold oral feedings 1-2hrs after last emesis, give 0.5-2oz Q15mins
IV fluids if oral replacement not successful
restore fluid vol.
prevent progression to hypovolemia -
IV fluids for severe dehydration - 20ml/kg NS or LR
maintenance fluids
Vomiting
ginger capsules, ginger tea, candied ginger reduces nausea and is safe in children <2yo
Vomitting - Nursing Assessment
onset and progression - hours after meal, middle of night
Effortless or projectile
contents and character - bilious is never normal, suggest obstruction
past medical history
risk factors
Diarrhea - what is it
most commonly caused by infxn
acute or chronic
inc. in frequency or dec. in consitency
diarrhea - nurisng assessment/diagnostics
details about stool
other symptoms ?
gained through travel or household illness
hydration status
abd assessment
electrolytes
stool cultures/stool OandP
stool for occult blood
abd radiograph
Diarrhea - nursing managment
restore F&E balance
provide family education
med. instruciton PRN
prevention if pertinent
proper handwashing
avoid excessive formula, water, fruit juice
hypertrophic pyloric stenosis
circular muscle of pyloris becomes atrophied, causing thickness in luminal side of pyloric canal, causing gastric outlet obstruction, causing nobilious vomitting
presents at 3-6 wks of life
more in males than fems
requires surgical interventions
hypertrophic pyloric stenosis - nursing assessment
common symptoms
forecful nonbilious vomitting unrelated to feeding positiomn
hunger after vomitting
weight loss
progressive dehydration w/ lethargy
possible fam. history
palpate for moveable “olive” in right upper quadrant
hypertrophic pyloric stenosis - nursing management
preop
fluid management
correcting electrolyte values
emotional support
education
postop
NPO
IV
dextrose
family talks w/ surgeon
intussusception
occurs when proximal segment of bowel telescopes into distal segment
causes edema, vascular compromise, partial/total bowel obstruction
occurs at 1-2 yrs of age
intussusception - common symptoms
sudden intermittent, crampy abd pain
severe pain
vomitting
diarrhea
currant - jelly stools, gross blood, hemoccult positive stool
lethargy
intussusception - nursing assessment
assess risk factors
cystic fibrosis
celiacs disease
sausage shaped mass in upper mid abd
diagnosed with air or barium enema
intussusception - nursing management
IV fluids, ABX prior to diagnostic testing
emotional support
spontaneous reduction
barium enema reduciton
surgical reduction
appendicits - what is it
acute inflammation of the appendix
ruptures if untreated
abd pain suddenly relieved w/o intervention, suspect perforation
diffuse abd tenderness/distention = peritonitis
appendicitis - physical assessment
ill appearing child
maximal tenderness over mcburneys point in RLQ
rebound tenderness
daignos w/ CT, CBC, CRP
appendicitis - nursing management
preop care
postop care
abx if ruptured or gangrenous
education
Hirschsprung disease (Cogenital Aganglionic Megacolon) - what is it
disorder of the motility of intestinal tract resulting in obstruction
lack of ganglion cells in intestine
4x more common in boys
rectal biopsy is definitve exam
Hirschsprung disease (Cogenital Aganglionic Megacolon) - Nursing assessment
stool pattern is key - did newborn pass meconium in 24-48hrs or life? rectal stim required? meconium plug present ?
family history - down syn. hirschprung dz
palpate - presence of stool, rectal tone, forceful expulsion of fecal matter while finger withdrawn from rectum
Hirschsprung disease (Cogenital Aganglionic Megacolon) - therapeutic management
surgical resection - usually w/ ostomy
reanstomosis of intestine
ostomy closure at later date
Hirschsprung disease (Cogenital Aganglionic Megacolon) - nursing management
postop ostomy care
child and fam education
prompt treatment if show signs of entercolitis
Celiac Dz
immunologic disorder where gluten cause damage in villi in small intestine causing malnutrition
strict gluten free diet is only treatment
autotissue stranglutemase IgA is first line test
atiendomysium IgA test specific to celiac dz
celiac dz - Nursing assessment
diarrhea
steatorrhea
constipation
failure to thrive/weight loss
abd distention/bloating
poor muscle tone
irritability/listlessness
dental disorders
anemia
delayed onset of puberty or amenorrhea
nutritional deficiencies