is defined as an opening made by cleaving or a crack or crevice
Cleft
lip has a notched vermillion border,
variably-sized clefts involve the alveolar ridge and dental abnormalities
Cleft
palate —nasal distortion, midline or bilateral
cleft with variable extension from the uvula and soft and hard palates with
exposed nasal cavities
Diagnosis of cleft lip/palate
Based on observations at birth
May be detected by palpation if the
palate is involved
Feeding abnormalities if not detected
sooner
May be seen prenatally by ultrasound
management of cleft lip/palate.
The
defect requires a team of experts including surgeons, nurses, geneticists,
orthodontists, ENT’s,
and therapists
Early
surgery for cleft lip at 3-6 months helps promote bonding
Cleft
lip may require modification at 4-5 years of age
Cleft
palate repair depends on the severity of the defect and closure by 6-24 months
aids speech development
Additional
nursing concerns of cleft lip/palate?
Feeding
difficulties
Risk
for aspiration
Emotional
issues for parents
Surgery
pre-op education
Postoperative
care to preserve suture site and prevent infection
Use
of “no-no”
restraints
Upright
feeding
At
high risk for otitis media infection
in a cleft lip how does it result?
the medial nasal and maxillary processes fail to join at 6-8 weeks gestation.
THe cleft palate results from what?
failure of the primary palatal shelves or processes that fuse at 7-12 weeks of gestation.
What is the first sign of a cleft palate?
Formula coming from the nose.
how can you confirm diagnosis of cleft palate?
a gloved finger placed in the mouth ot feel the defect of the palate or visual examination with a flashlight.
What device can be used to expand or realign parts of palate or decrease the size of a wide lip cleft.
A lathan device.
when is cleft lip repair usually done?
3-6 months
When is cleft palate repaired?
between 6-12 months
Esophageal
atresia with tracheoesophageal fistula (TEF)
Actually
2 disorders where either the esophagus terminates before it reaches the stomach
or…
A
fistula is present that causes an unnatural tracheal connection
Incidence
1:2000-4000 births
Often
affects premature or low birth weight infants
Symptoms of Esophageal atresia with tracheosophageal fistula?
At higher risk if pregnancy was polyhydramnios
Failure to pass NG tube or catheter at
newborn exam (10-11 cm beyond gum line)
Excessive oral secretions
Coughing, choking, cyanosis
Vomiting or abnormal appearing abdomen
How can atresia be suspected?
If the NG tube cannot be passes 10-11 cm beyond the gum line?
Why do you keep the infant supine with the head of the bed elevated in atresia?
To decrease the chance of gastric secretions entering the lungs.
What are the three C's of tracheoesophageal fitula?
COUGH
chocking
cynosis
What do you do with the g-tube in the immediate postop of atresia?
Leave it open to drain in order to allwo gastric contents and air to escape.
Fistula
Oral
intake goes into lungs, the baby will cough and choke with feeding, the abdomen
is distended.
Aspiration—pneumonia—respiratory distress.
Atresia
Risk
remains for aspiration of saliva and fluids.
The abdomen will appear airless and scaphoid
Management of atresia or fistula?
Surgery
Staged repair
G-tube and cervical esophagostomy with later anastomosis, colon
interposition, and dilation
Nursing
considerations
Importance
of newborn exam
Assess
for 3 “c’s”—coughing,
choking, and cyanosis
Keep
baby supine to prevent aspiration preoperatively
Tube
feedings
Keep
baby warm and well oxygenated
Postop--expect
chest tubes and g-tube
Imperforate
anus
Incidence is 1:4000-5000 births
Failure to pass meconium stool within 24
hours
Absence of anorectal canal
Presence of anal membrane
External fistula to perineum
Diagnosis
Newborn
exam with rectal temp
No
stool within 24-48 hours
Newborn
exam may note skin dimpling
Observation
may include stool in urine or vagina
X-ray,
Ultrasound, CT
Management varies from repetitive
dilation to surgery that could involve a colostomy and bowel pull-through
procedure
Gastroschisis
An
embryonic weakness in the abdominal wall causing herniation of the gut on one
side of the umbilical cord during early fetal development. Results in viscera outside of the abdominal
cavity and “free
floating” in
the amniotic sac
Incidence
is 1:4000
Mortality
is 10-15%
Delivery
may or may not be a c/section
Management
issues include—IV, NG, TPN, coverage of gut, slow re-introduction of contents
into abdomen (about 28 days), staged surgery to repair the defect and also if
there was any bowel necrosis.
Omphalocele
Large
herniation of gut into umbilical cord.
Viscera are outside of abdomen but inside a sac.
Incidence
is 1:5000-6000 births
Mortality
is 20-30% due to risk for sepsis and obstruction
Management
and nursing care are very similar to gastroschisis
Umbilical
hernia
Not
as serious a defect and involves imperfect closure of the umbilical ring
allowing the gut to push outward and with increased abdominal pressure the
defect is more obvious.
Incidence
is most common among African American babies and if baby was low birth weight.
Management---no
intervention unless the defect persists beyond age 5—usual regression by age
one
If
strangulated or size increases--surgery may be indicated
Nursing
considerations for umbilical hernia
Do not bind
Monitor size
Monitor for irreducible mass or change in
bowel patterns
Teach parents ER warnings including:
signs of strangulation, vomiting, pain, or an irreducible mass at umbilical
Congenital
diaphragmatic hernia
An
opening in the diaphragm resulting in abdominal contents herniated into the
thoracic cavity—usually has some pulmonary effects
Mortality
50-80% and improving to 40% if the baby has ECMO.
Incidence
1:220-5000
Surgical
intervention within 6-18 hours after birth and management at a tertiary care
center
Postop
respiratory assistance until lungs are functional—no O2 face masks to avoid air
into stomach
What is silastic used for?
To cover the intestines in gastroschisis.
Gastroesphageal reflux disease
regurgitation of gastric contents
back into esophagus
Delayed gastric emptying
Drugs
Hiatal hernia
Infants have a short lower abdominal
esophageal sphincter-usually resolves by age 1-2
Increased
intra-abdominal pressure
Obesity
Swallowing
dysfunctions
Symptoms
of GER
Vomiting
Spitting up after meals
Hiccupping
Recurrent otitis media
Weight loss
Severe may see hematoemesis, melena, anemia
Failure
to thrive
Irritable
Discomfort
and abdominal pain
Respiratory—coughing,
choking, wheezing, pneumonia, apnea, bradycardia
Management
of GER
Based
on severity of symptoms
Positioning
at 30 degrees prone
Lying
on right side
Excepted
from usual positioning to prevent SIDs
Medications
may be indicated such as PPI’s,
H2 blockers, antacids, mucosal protectants, and metocloproamide
Dietary
Interventions for GER
Small,
frequent feedings
Predigested
baby formula
Rice
thickened cereals may decrease vomiting but does not reduce reflux since it
delays gastric emptying
Eliminate
caffeine
May
need NG or other supplemental feedings if failure to thrive
Additional
considerations of GER
Acute
bleeding is a complication of long-standing GER. Treated with stomach lavage but not iced
saline. May require interventions to coagulate bleeder
15%
require surgery—fundoplication which “tightens”
the LES. Postoperatively, these patients
are at risk for “gas
bloat” due
to difficulty burping.
Why do infants have gastroesophageal reflux more often?
they have a shorter abdominal lower esophageal sphincter (LES)
What are the hallmarks of GERD?
Vomiting or spitting up after meals
hiccuppin
recurent otitis media related to pooled secretions in the nasopharynx during sleep.
What can sever GERD result in?
hematemiesis or melena and anemia
Constipation
and encopresis
Infrequent
or difficult passage of hard stool
When
constipation persists in the child over 4 years of age, encopresis defined
as repeated and involuntary defecation with no disorders of the GI
tract
Encopresis
secondary to recurrent fecal impaction and an enlarged rectum due to chronic
constipation
Predisposing
factors of constipation and encpresis?
Poor toilet training and habits
Psychological stressors such as starting
to school, new sibling, etc.
Typical age of onset 3-7 years and more
common among boys
Also seen more frequently with lower
socioeconomic and learning disabled
S/S of Constipation?
Absence of expected amount of stool
Abdominal pain
Cramping without distention
Palpable, movable fecal mass
Decreased bowel movements
Malaise
Anorexia
Headache
Nausea
Vomiting
Anal
fissure
At
risk for UTI’s
S/S Encopresis
Soiled
clothing
Fecal
odor
Anal
irritation
Social
withdrawal
Avoidance
of overnight outings
Increased
UTI’s or
incontinence
Management of constipation and encopresis?
Disimpaction—enemas
until clear using fleets at 1 ounce per kg or if over 20 kg use adult dose q 12
hours—usually effective within 48 hours.
Stool
softeners
Laxatives
Managment of Encoporesis
Maintenance
Mineral
oil 2 ml/kg with bid dosing and mixed with food of fluids—may note leakage of
oil
Lactulose
or milk of magnesia for children older than 6 months
Limit
milk and sugar while increasing water and residue
Changing the Retention Habit
Sitting
on toilet 20-30 minutes after meals
Behavior
charts
Positive
reinforcement
Change
of clothes with school nurse
Goal—for
child to pass 2-3 soft painless stools daily and wean off medications
If an
infant is constipated?
Do not encourage rectal stimulation, may try dietary such as barley cereal,
fructose (prune juice), lactulose, high fiber vegetables
Irritable
bowel syndrome
Diffuse
abdominal pain
Alternating
diarrhea and constipation
Undigested
food and mucous seen in stools
Child
has normal growth pattern and is negative for other GI disorders
May
have past history of colic
Occurs
after infancy and is common among toddlers and adolescents
Management
of irritable bowel syndrome
Encourage child to eat slowly
Eliminate carbonated drinks
Excess diet drinks may cause diarrhea
Moderate fiber intake
Reassure family that disease is
self-limiting and usually resolves without affecting growth
Health promotion and exercise
Counseling
Ulcers—loss of tissue in areas of
digestive tract exposed to pepsin
Multiple
factors such as H. pylori, lack or prostiglandins, excessive acid secretion, medications,
and physiological stress may cause ulcers
Symptoms--the
child complains of pain (burning, cramping) when their stomach is empty
Child
may also experience awakening at night or early morning with pain
Hematoemesis, melena
Infectious
gastroenteritis
Giardia
is the most common found in daycare centers
Rotavirus
most often affects infants and children
20
million cases among those younger than 5 and U.S. mortality is 300-400 children
yearly.
S/S of infectious gastroenteritis?
Diarrhea,
vomiting, abdominal pain, tenesmus
(ineffective, painful, or continuous urge to defecate), and fever.
Children
under 2 at increased risk for dehydration
Pathogens
include—shigella,
salmonella, E. Coli, campylobacter, giardia lambia,
rotavirus, and clostidium
difficile—see
text p. 1087
Managment concerns for infectious gastrenterities?
Oral rehydrating solutions at 50-100
ml/kg over 4-6 hours
Avoid high sugar drinks and juices
Avoid anti-diarrheal medications
Electrolyte management
Antibiotics for giardiasis and shigella
Possible vaccine for rotavirus
Appendicitis
Inflammation
and infection of the vermiform appendix located at the end of the cecum.
Incidence of appendicitis?
Most
common type of emergency surgery for children
Most
cases occur adolescence or early adulthood
80,000
appendectomies each year in U.S>
Young
children are harder to diagnose and more prone to rupture
Perforation
rate for children under 2 years if age is 70%
Symptoms of appendicitis?
Progressive
pain RLQ at McBurney’s
point.
Abdominal
pain—may ask child to stand on tiptoes and drop to flat feet
Tenderness
Guarding
Often
supine flexing right leg or knee-chest
Increased
WBC’s
Fever,
chills
Diarrhea
or constipation
Pain
may subside temporarily with rupture
Following
rupture—symptoms worsen, rigid abdomen, fever and shock, dehydration.
Differential
If vomiting precedes the pain, it is more likely gastroenteritis. Appendicitis presents with sudden and
simultaneous pain, anorexia, or nausea and vomiting with fever
Management of Appendicites?
Appendectomy
Nursing
care pre-op
No
enemas or laxatives
No
heat to abdomen
sMay
provide cold or reposition
Pain
medication
Hydration
Management of post op appendectomy?
Hydration
If
ruptured more serious
NG tube
Antibiotics
Wound care
Infection prevention
Elevate head of bed or right side
positioning
Advance diet slowly
ER warnings postop infection or
obstruction
Inflammatory
bowel disease
Chronic
inflammatory conditions of the small and large intestines such as Crohn’s
and ulcerative colitis
Crohn’s
disease
Pathology—affects
entire GI tract
Transmural
involvement
Fistulas
are common
Marked
by remissions and exacerbations
Biopsy
reveals skip lesions with deep fissures, granulomatous, and intermittent normal
mucosa
Ulcerative
colitis
Involves
only the colon
Mucosa
and submucosa
only
Rare
fistulas
Remissions
are uncommon
Biopsy
reveals continuous spreading—ulcers are superficial and no areas of normal
mucosa are seen
Crohn’s
Incidence
5:10,000
Equal
gender
Not
seen in infants, peak incidence teen’s
and 20’s
Clusters
in families
Higher
socioeconomic
Ulcerative colitis
Incidence 5:100,000
Equal gender
Peaks at 15-40 years
Clusters in families
Affects more caucasians
How does Crohn's present?
Abdominal pain
Non-bloody diarrhea
Fever
Palpable abd. mass
Anorexia, weight loss
Perianal, anal lesions
Fistulas, obstructions
Arthritis, arthralgias
Ulcerative colitis Presents with?
Rare abdominal pain
Bloody diarrhea, may hemorrhage
No masses
Moderate weight loss
Rare lesions
Mild growth impairment
Risk for toxic megacolon
Crohn's cont
Life expectancy not reduced
50-70% will require surgery for fistulas,
obstructions, etc.
Surgery is not curative
Dietary--often see malnutrition
NG
feedings indicated
Supplements
should include—iron, fish oil, folic acid, and glutamine
Ulcerative colitis?
12-15 % mortality
10% chance of cancer after 10 years with
disease
Removal of colon cures disorder
Diet should avoid milk
Low fiber, low residue, high protein
Occasional TPN
Nursing care for Chrons and ulcerative colitis?
Ongoing
evaluation of child’s
growth and development
Nutrition
management
Medication
administration
At
risk for oral lesions, perianal skin breakdown, emotional issues, social
problems
“Chronicity”
ER
warnings—weight loss, blood loss, severe abdominal pain
Necrotizing
enterocolitis (NEC
Most
commonly acquired GI disorder of neonates involving intestinal lesions—usually
in proximal colon and ileum
Necrotizing enterocolitis cont?
Incidence
for all NICU admissions is 1-15% and of those 80-90% premature births
Usually
occurs first 14 days of life
Baby
develops apnea, bradycardia,
temperature instability, lethargy, abdominal distention, skin turns shiny and
discolored, and at latter stages—hypotension and shock
Diagnosis of Necrotizing enteroclitis?
X-ray will reveal dilated bowel loops and
presence of air in intestinal wall, portal vein and/or abdominal cavity
Altered white count, decreased platelets,
metabolic acidosis, electrolyte imbalances
Managment of Necrotizing enteroclities?
Prevention using immunoglobin IgA, IgG via breast milk,cortocosteroids, slow feeding protocols
Decompress GI by NG tube and keep baby
NPO
Sepsis control
Fluid
resuscitation
Measure
abdomen
Respiratory
support
Hemodynamic
Decrease
stimulation
Position—not
prone
High
risk for recurrence for several weeks
Obstructive
disorders
Pyloric
stenosis, intussusception, volvulas,
and Hirschsprung’s
disease
(Hypertropic)
pyloric stenosis
Occurs when the muscle surrounding the pyloris
hypertrophies and obstructs gastric emptying
Symptoms of hypertonic pyloric stenosis?
Progressive, non-bilious projectile
vomiting
Blood tinged vomitus
Movable, palpable firm olive-shaped mass
that is visible in RUQ
Visible, gastric peristaltic waves
Managment and prognosis of hypertonic pyloric stenosis?
Pyloromyotomy—incision
of pyloric muscle to release the obstruction
Performed
within 24-48 hours
Excellent prognosis
Intusussusception
Pediatric
emergency where a section of the intestine invaginates
into the distal bowel causing an obstruction—rare diagnosis—most common cause
of bowel obstruction for children under 2.
Presenting symptoms of intusussusce?
Passage of bloody mucous, diarrhea, and
currant jelly stools in a previously healthy child
Sausage shaped abdominal mass
Paroxysmal pain
vomiting
Diagnosis of intusussusce?
X-ray
and ultrasound
May
be able to remedy disorder with barium, water, or air enema ultrasound guided
Surgery
if bowel damage is evident
Best prognosis if detected within 24
hours
Nursing care supportive and explaining
diagnostic tests, condition, and treatment to parents
Hirschsprung’s
Disease (Mega-colon)
Lacking
ganglion cells in colon
A
congenital condition
Without
ganglion, normal peristalsis is impossible
Accounts
for 25% of neonatal obstructions
Affects
1:5000 births
More
common among males and babies with Down’s
Symptoms of Hirschsprungs disease?
Delayed
passage of meconium
Child
is constipated
Infrequent
stools
May
present with bowel obstruction—abdominal pain, vomiting, failure to thrive
Managment of Hirschsrung disease?
Mild
to moderate disease—with stool softeners and rectal irrigations
Moderate
to severe disease—removing abnormal areas surgically, temporary colostomy, and
complete repair once the child reaches 18-20 pounds
Malabsorption disorders
Lactose
intolerance
Celiac
disease
Lactose
intolerance
Unable to digest lactose as a congenital
or acquired problem
Lactose is the sugar found in dairy
products
Congenital presents initially and is more
severe
Developmental onset is usually at age 3
Clinical
findings and treatment of Lactose intolerance?
Frothy,
but not fatty, diarrhea
Abdominal
distention
Cramping
and abdominal pain
Excessive
flatus
Presumptive
diagnosis—eliminate lactose and patient will improve
If
breast feeding, the mother should eliminate milk…formula babies should be given
lactose-free formula
Celiac
disease
Gluten
enteropathy
or tropical sprue—unable
to digest the protein part of wheat, barley, rye, and oats
Affects
1:1000 births in U.S. with higher incidence in Europe
Usual
onset at 9-12 months due to dietary introductions
Symptoms of Celiac disease?
Foul
smelling and fatty diarrhea
Failure
to grow—below 25th
percentile
Abdominal
distention
Irritable
Anorexia,
muscle wasting
Edema
Folate
deficient
managment of celiac disease?
Eliminate
rye, wheat, barley, and oats
Replace
with corn and rice
Vitamin
supplements—especially folate
Expect
life-long dietary changes
Celiac crisis
can present with profuse, watery diarrhea and metabolic acidosis—usual cause is
infection or ingestion of unknown gluten product—this is an emergency
Hepatic
disorders
Hepatitis
Biliary
atresia
Cirrhosis
Viral
and non-viral hepatitis
Acute or chronic liver inflammation
May be secondary to rubella, CMV,
Epstein-Barr, HSV, etc.
Infectious hepatitis A-E
Hepatitis A and E spread oral-fecal
Hepatitis B and C via blood or body
fluids and are transmissible to fetus
Symptoms
of hepatitis
May
be asymptomatic or mild flu-like.
Older
children more prone to fever, malaise, anorexia, nausea, jaundice
Icteric
stage—jaundice, urticaria,
dark urine, clay-colored stools
Fulminant
is capable of producing bleeding abnormalities, encephalopathy, ascites, and
acute liver failure--rare
Management
of Hep A
Prevention
of further spread
A
virulent pathogen able to live outside of the body for weeks
Immune
A globulin
Vaccine
for those at risk—chronic liver disease, homosexual males, foreign travel,
healthcare workers, hemophiliacs, inmates
Reportable
disease
Management
of Hep B
Universal
precautions
Hep
B vaccine
Hep
B immune globulin
Nursing
care is supportive and generally patients are not hospitalized unless
neurological changes, bleeding, ascites—no over-the-counter medications or
alcohol
Biliary
atresia
Obstruction or absence of extrahepatic
bile ducts. Normal newborn and as liver
damage increases—symptoms worsen
Affects 1:10,000-20,000 births
Most common cause of liver transplants among children
Symptoms Biliary atresia?
Healthy
at birth
Evolves
into bile-stained and acholic
stools
Enlarged
liver—spleen may also be affected
Increased
abdominal girth
Failure
to thrive, rickets, pruritis,
skin infections
Persistent
jaundice of newborn
Lab
findings and management of biliary atresia?
Increased
ALT, AST, clotting time
Liver
biopsy—bile plugs, edema, fibrosis
Treat
malnutrition, skin care, vitamins, at risk for GI bleed, liver transplant
candidate
Serum PH <7.35
Acidosis
Serum ph >7.45
Alkalosis
Absence of urine formation?
Anuria
Fluid found outside the cell?
Extracellular Fluid (ECF)
Serum sodium >150. meq/L
Hypernatremic (hypertonic) dehydration
Condition that occurs when sodium concentration falls below that of normal body fluids
Hyponatremic (Hypotonic) dehydration
Fluid found within the cells
Intracellular fluid (ICF)
Fluid surrounding the cells including lymph fluid?
Interstital fluid.
Fluid within the blood vessels?
Intravascular fluid
Condition that occurs when sodium concentration is identical to that of body fluids
Isonatremic isotonic dehydration
Diminished amounts of urine output.
oliguria
T/R- Isotonic dehydration occurs when water and electrolytes are lost in the same proportion as they occure in the body?
True
T/F- In dehydration fluid loss occurs first in the ICF
False
T/F- an infant who has lost 7% of his body weight has moderate dehydration?
True
T/F-Inital therapy in sever dehydration is aimed at preventing shock
true
When a child is severly dehydrated electrolytes such as potassium are replaced by admin them by slow IV Push
False
Either ringer lactate solution or 0.9% sodium chloride is an appropriate fluid choice for parenteral rehydration?
True
Diluted Gatorade is an acceptable oral rehydrating solution for a toddler
false
wahat is diarrhea?
An increase in the frequency fluidity and volume of stools
Diarrhea caused by infection is usually called?
gastroenteritis
List four causes of diarrhea?
Food intolerance
Medications
malabsoprtion
colon disease
irritable bowel syndrome
stress infectious
Why are sports drinks and colas inappropriate fluid choices for a child with diarrhea?
The high carbohydrate content of thees drinks may worsen diarrhea in addition they don not replace electrlytes lost through diarrhea.
Explain why an infant with diarrhea should continue to revceive breast milk or full strenght formula?
To prevent dehydration to reduce stool frequency and volume and to reduce duration of diarrhea.
What common foods are generally well tolerated during diarrhea?
Chose from complex carbohydrates (rice,bread, cereals, noodle, potaotes crackers) yogurt cooked vegtables and lean meats avoid fatty foods and simple sugars.
HOw can skin breakdown be prevented in infants with diarrhea?
Change diaper immediately after each bowel movement wash skin with mild soap and pat dry apply barrier oinment A&D and avoid using commercial baby wipes reposition at least every 2 hours.
T/F- Vomiting can result from allergic reactions
True
T/F- Emesis with a fecal odor may indicate lower intestinal obstruction
True
T/F- Projectile vomiting is usually a result of overfeeding
False
T/F- Oral rehydrating solution cannot be used when a child has vomited?
False
An otherwise healthy infant is admitted to teh hopital with dehydration and diarrhea she is hyperirritable and agitated and she has flushed skin and dry mucous membranes. The nurse supects
Hypernatremia
An infant who has been vomiting frequentlly is at risk for
Metabolic alkalosis
Which statement about the compensatroy mechanisms that maintain normal PH is true?
If serum PH rises above normal teh kidneys conserve hydrogen ions.
Indicators of dehydration in a 4 year old child include?
Thirst and specific gravity of 1.038
Infants with dehydration must be monitored for signs of impending shock. A late sign of shock in the infant is?
decreased B/P
A preschooler with moderate diarrhea who is just admitted to the hospital and who is not nothing by mouth NPO status wants something to drink teh nurse best choice is to offer
An oral rehydration solution.
an infant with diarrhea has tolerted clear liquids but begins having diarrhea again when she returns to her regular formula her diet should be changed to
Soy formula
A child weighing 14 kg is reciveing maintenance IV fluids. How many milliliters per hour should the child receive.
50 mm
An infant weighed 6.4 kg at the peiatricians office 2 days ago he has had diarrhea since then he now weighs 5.9 kg his mucous membranes are dry his fontanel is sunken and his capillary refill is approximately 5 seconds the infant has
moderate dehydration
An infant has viral gastroenteritis to prevent its spread to other family members teh nurse teaches the family
