peds gi Flashcards
cleft lip
congenital fissure in the upper lip
cleft palate
congenital fissure in either the soft palate alone or both hard and soft palates
most common craniofacial malformation
cleft lip with or without cleft palate
cleft lip: pathophysiology
occurs at 6 weeks gestation
- maxillary process fails to merge with the medial nasal elevation on one or both sides
cleft lip: when does it occur?
6 weeks gestation
cleft palate: pathophysiology
occurs 7 to 12 weeks gestation
- lateral palatine processes fail to meet and fuse with each other, the primary palate, or nasal septum
cleft palate: when does it occur?
7 to 12 weeks gestation
cleft lip/palate: etiology
- multifactorial mode of inheritance
- maternal nutrition: FOLIC ACID
- maternal medication 1st trimester
- occurrence of fever, flu 1st trimester
- maternal prenatal cigarette smoking
cleft lip/palate: clinical manifestations
generally evident at birth, if not:
- feeding difficulties
- chronic upper airway congestion/infection
- 7 to 14% have other anomalies!
cleft lip/palate: therapeutic management
- team approach!
- surgical repair PRIORITY ideally before child speaks
cleft lip @ 10 wks or 10 lbs
cleft palate @ 6 months
cleft lip: when should surgical repair be done?
10 weeks or 10 pounds
cleft palate: when should surgical repair be done?
6 months
nutrition considerations for babies with cl/cp
- inability to suck = possible undernutrition
- elevate head during feeds
- special nipples
- gentle, steady pressure on bottle base
- frequent burping
- daily weight: nutritional eval, fluid volume status
- nipple way back in oral cavity
- breck feeder
breck feeder
syringe with feeding tube expansion appropriate for feeding babies with difficulty feeding due to cl/cp
significance of otitis media and cleft palate
improper middle ear drainage due to inefficient eustachian tube function s/t cp = increased middle ear pressure
- recurrent otitis media
- can lead to HEARING IMPAIRMENT
tracheoesophageal fistula aka
esophageal atresia
tef/esophageal atresia
aka tracheoesophageal fistula (tef)
failure of trachea to differentiate and separate from esophagus into separate, distinct structures
failure of esophagus to develop as continuous passage
tef/esophageal atresia % affected with associated anomalies
30-40%
tef/esophageal atresia: clinical manifestations
- inability to swallow saliva, increase in drooling, frothy saliva in mouth/nose
- gastric tube/suction catheter can’t be passed
- choking, cyanosis with feedings; cyanotic or apneic s/t aspiration
- recurrent pneumonia (typically will be caught before this)
- triad: excessive secretions, reflux, respiratory distress
tef/esophageal atresia: hallmark sx triad
excessive secretions + reflux + respiratory distress
tef/esophageal atresia: diagnosis
diagnose before feeding by catching clinical s/s
xray after passing radiopaque catheter into esophagus
tef/esophageal atresia: classic presentation
significant choking, cyanosis with feeds
tef/esophageal atresia: why frequent suctioning?
keeps blind pouch empty; secretions develop even though it is a pouch!
tef/esophageal atresia: gastric decompression
catheter in blind pouch to low wall suction, g tube inserted and open to drain
tef/esophageal atresia: suction considerations
prn with EXTREME CAUTION
- possible interference with fresh postop repair!
tef/esophageal atresia: post operative feeding considerations
with no connection between sucking and satiety, oral aversion is possible; no drive to eat
- start g tube feeds slowly
- start oral feeds slowly after healed
- feeding difficulties not uncommon! MONITOR!
hernia
protrusion of organ through an abnormal opening; become dangerous when constriction leads to impaired circulation or impaired respiration
when do abdominal wall defects occur?
3rd week of gestation
omphalocele
herniation of abdominal contents through umbilical ring; intact peritoneal sac
75%ish survival (associated anomalies)
gastroschisis
herniation of abdominal contents lateral to umbilical ring, no peritoneal sac
85-100% survival
abdominal wall defects: therapeutic management
high risk of infection!! cover with sterile wrap, give abx
IVFs to prevent fluid loss
umbilical hernia
incomplete fusion of umbilical ring where umbilical vessels leave abdominal wall.
most common type of hernia. complications rare. surgery if still present at 2-3 years.
most common type of hernia
umbilical hernia
surgery on umbilical hernia when?
if still present at 2-3 years
congenital diaphragmatic hernia
SIGNIFICANT!
diaphragm not completely formed allowing intestines and/or organs to enter thoracic cavity; restricted lung growth due to competition for space
cdh: manifestations
acute respiratory distress during newborn period; can have significant decreased cardiac output + possible shock
cdh: prenatal diagnosis
media stinal shift, see loops of bowel in chest wall
- ideally picked up in week 20 ultrasound
cdh: postnatal diagnosis
chest x-ray = loops of bowel in chest
cdh: management
- respiratory support via hood or intubation (no bagging because that can introduce air into stomach which will then go into bowel!)
- gi decompression via ngt
- hob elevated
cdh: possible associated outcomes
chronic lung disease, gastroesophageal reflux, growth failure
inguinal hernia
protrusion of small intestines/fluid into groin through a weakness or tear in the abdominal wall
m>f ; premie > term
inguinal hernia: manifestation
mass in groin, can present as painless inguinal swelling; CAN be painful
sac is present at birth but inguinal hernia may not manifest until 2-3 months
inguinal hernia may not manifest until…
2-3 months, though sac IS present at birth
inguinal hernia: surgical considerations
put surgery off until 10 pounds because bigger, stronger babies do better in surgery
surgery is outpatient and not very scary!
hirschsprung’s disease aka
congenital aganglionic megacolon
hirschsprung’s disease
congenital anomaly that results in mechanical obstruction from inadequate motility in part of the intestine; no innervation = no peristalsis AND decreased relaxation of internal rectal sphincter; collapsed rectum can also result
megacolon occurs due to obstruction in portion directly above part of tract with no ennervation
hirschsprung’s: pathophys*
absence of autonomic parasympathetic ganglion cells (of the submucosal plexus of Meissner and the myenteric Auerbauch plexus) in one or more segments of the colon
hirschsprung’s: clinical manifestations - newborn
41-64%; abdominal distension, vomiting, no meconium in 48 hours
big, shiny basketball tummy
hirschsprung’s: clinical manifestations - older infancy/childhood
21-35%/15-26%
chronic constipation, ftt, foul smelling and ribbonlike stools, abdominal distension
hirschsprung’s: classic poo for infancy/childhood diagnosis*
foul smelling and ribbonlike*
hirschsprung’s: stage I management
surgical removal of aganglionic bowel, temporary 6 mo ostomy over stretched out area - it needs to rest
hirschsprung’s: stage II management
8mo - 1yo -OR- 20 lbs
“pull” the end of intact bowel down to rectum for reanastomosis
hirschsprung’s: stage III management
closure of colostomy, usually 3 mo after stage II
hirschsprung’s: conservative therapy for mild
occasional enemas, develop regular poo pattern, at continued risk for development of enterocolitis
(population with mild sx very small)
enterocolitis
inflammation of bowel, esp small intestine
signs: explossive, watery diarrhea, fever, appears significantly ill
hirschsprung’s: high risk for…?
SEPSIS! (prior to surgery)
acute intestinal obstruction: manifestation
- abdominal pain
- distension
- nausea, vomiting
- change in stool
pyloric stenosis: manifestations*
early, nonbilious vomiting beginning around 3 weeks of age, progressing to projectile
olive shaped mass in epigastrum palpable*; can see waves of peristalsis too
pyloric stenosis: classic sign*
olive shaped mass in epigastrum palpable*
pyloric stenosis
narrowing of the pyloric sphincter at the outlet of the stomach, obstructing the flow of food into the small intestines
pyloric stenosis: pathophys
muscle of pyloric sphincter becomes thick, elongated, narrowing the pyloric channel
pyloric stenosis: manifestations*
early, nonbilious vomiting beginning around 3 weeks of age, progressing to projectile
olive shaped mass in epigastrum palpable*; can see waves of peristalsis too
epigastrum
upper, central region of abdomen
pyloric stenosis: diagnosis
- often made by hx, pe
- ultrasound
- possible metabolic alkalosis, severe volume depletion
pyloric stenosis: metabolic consideration
metabolic alkalosis possible, correct before surgery!
pyloric stenosis: postop considerations
- feed 4-6 hours after
- vomiting is not uncommon
- manage pain
intussusception
telescoping of the bowel upon itself
after fixing, only 10% reoccur
intussusception: acute clinical manifestations
triad!
- pain: sudden onset of crampy abdominal pain
- sausage shaped abdominal mass
- currant jelly stool
intussusception: triad - what & when
pain, sausage, currant jelly stool
if ACUTE intussusception
intussusception: chronic clinical manifestations
diarrhea, anorexia, weight loss, vomiting, periodic pain
intussusception: therapeutic management
barium/hydrostatic reduction (ie, enema) - 75% success
surgical manipulation
surgical resection
ger considered gerd when these complications are seen
failure to thrive, anorexia, irritability, recurrent pneumonia (aspirating feedings)
celiac disease
permanent intestinal intolerance to dietary wheat and related proteins that produce mucosal lesions in genetically susceptible individuals
malabsorption syndrome second to cf in cause of malabsorption in children
celiac disease: clinical manifestations
- impaired fat absorption (steatorrhea, foul-smelling poo)
- impaired nutrient absorption (malnutrition, muscle wasting esp in legs/butt, anemia, anorexia, abdominal distension)
- behavioral changes (irritable, uncooperative, apathy)
celiac disease: diagnosis & tx
withdraw gluten from diet = full remission of sx
tx 100% dietary management
steatorrhea
excessively largel pale, oily, frothy stools
- see in children with celiac disease
gastroesophageal reflux
?
ger: diagnostic eval
upper gi swallow study
ger: therapeutic management
feed at higher angle, sit up 30 min after feeds, feed small amount more often, burp a lot, med management possible
ger: therapeutic management
feed at higher angle, sit up 30 min after feeds, feed small amount more often, burp a lot, med management possible, nissen fundoplication
nissen fundoplication
after eating, environment, medication management all tried
stomach is its own cardiac sphincter! (wrap around bottom of esophagus)
gastroesophageal reflux
transfer of gastric contents into esophagus; physiologic phenomenon occurring throughout the day (most frequently after meals and at night)
usually resolves spontaneously by 1 year of age
ger vs gerd
gerd is sx/tissue damage that result FROM ger. ger -> gerd when complications seen:
- failure to thrive, bleeding, dysphagia, anorexia, irritability, recurrent pneumonia (aspirating feedings)
silo
mesh used to house omphalocele that is extremely large or that can’t be pushed back into abdominal cavity because cavity is small.
can be suspended using mild tension. antibacterial ointment applied to prevent infection. compressed on a daily basis. ideally accomplished within 7 to 10 days.
