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Flashcards in peds gi Deck (84):
1

cleft lip

congenital fissure in the upper lip

2

cleft palate

congenital fissure in either the soft palate alone or both hard and soft palates

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most common craniofacial malformation

cleft lip with or without cleft palate

4

cleft lip: pathophysiology

occurs at 6 weeks gestation
- maxillary process fails to merge with the medial nasal elevation on one or both sides

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cleft lip: when does it occur?

6 weeks gestation

6

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

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cleft palate: when does it occur?

7 to 12 weeks gestation

8

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

9

cleft lip/palate: clinical manifestations

generally evident at birth, if not:
- feeding difficulties
- chronic upper airway congestion/infection
- 7 to 14% have other anomalies!

10

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

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cleft lip: when should surgical repair be done?

10 weeks or 10 pounds

12

cleft palate: when should surgical repair be done?

6 months

13

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

14

breck feeder

syringe with feeding tube expansion appropriate for feeding babies with difficulty feeding due to cl/cp

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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

16

tracheoesophageal fistula aka

esophageal atresia

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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

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tef/esophageal atresia % affected with associated anomalies

30-40%

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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

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tef/esophageal atresia: hallmark sx triad

excessive secretions + reflux + respiratory distress

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tef/esophageal atresia: diagnosis

diagnose before feeding by catching clinical s/s

xray after passing radiopaque catheter into esophagus

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tef/esophageal atresia: classic presentation

significant choking, cyanosis with feeds

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tef/esophageal atresia: why frequent suctioning?

keeps blind pouch empty; secretions develop even though it is a pouch!

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tef/esophageal atresia: gastric decompression

catheter in blind pouch to low wall suction, g tube inserted and open to drain

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tef/esophageal atresia: suction considerations

prn with EXTREME CAUTION
- possible interference with fresh postop repair!

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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!

27

hernia

protrusion of organ through an abnormal opening; become dangerous when constriction leads to impaired circulation or impaired respiration

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when do abdominal wall defects occur?

3rd week of gestation

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omphalocele

herniation of abdominal contents through umbilical ring; intact peritoneal sac

75%ish survival (associated anomalies)

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gastroschisis

herniation of abdominal contents lateral to umbilical ring, no peritoneal sac

85-100% survival

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abdominal wall defects: therapeutic management

high risk of infection!! cover with sterile wrap, give abx

IVFs to prevent fluid loss

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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.

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most common type of hernia

umbilical hernia

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surgery on umbilical hernia when?

if still present at 2-3 years

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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

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cdh: manifestations

acute respiratory distress during newborn period; can have significant decreased cardiac output + possible shock

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cdh: prenatal diagnosis

media stinal shift, see loops of bowel in chest wall
- ideally picked up in week 20 ultrasound

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cdh: postnatal diagnosis

chest x-ray = loops of bowel in chest

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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

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cdh: possible associated outcomes

chronic lung disease, gastroesophageal reflux, growth failure

41

inguinal hernia

protrusion of small intestines/fluid into groin through a weakness or tear in the abdominal wall

m>f ; premie > term

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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

43

inguinal hernia may not manifest until...

2-3 months, though sac IS present at birth

44

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!

45

hirschsprung's disease aka

congenital aganglionic megacolon

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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

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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

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hirschsprung's: clinical manifestations - newborn

41-64%; abdominal distension, vomiting, no meconium in 48 hours

big, shiny basketball tummy

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hirschsprung's: clinical manifestations - older infancy/childhood

21-35%/15-26%
chronic constipation, ftt, foul smelling and ribbonlike stools, abdominal distension

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hirschsprung's: classic poo for infancy/childhood diagnosis*

foul smelling and ribbonlike*

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hirschsprung's: stage I management

surgical removal of aganglionic bowel, temporary 6 mo ostomy over stretched out area - it needs to rest

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hirschsprung's: stage II management

8mo - 1yo -OR- 20 lbs
"pull" the end of intact bowel down to rectum for reanastomosis

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hirschsprung's: stage III management

closure of colostomy, usually 3 mo after stage II

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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)

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enterocolitis

inflammation of bowel, esp small intestine

signs: explossive, watery diarrhea, fever, appears significantly ill

56

hirschsprung's: high risk for...?

SEPSIS! (prior to surgery)

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acute intestinal obstruction: manifestation

- abdominal pain
- distension
- nausea, vomiting
- change in stool

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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

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pyloric stenosis: classic sign*

olive shaped mass in epigastrum palpable*

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pyloric stenosis

narrowing of the pyloric sphincter at the outlet of the stomach, obstructing the flow of food into the small intestines

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pyloric stenosis: pathophys

muscle of pyloric sphincter becomes thick, elongated, narrowing the pyloric channel

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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

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epigastrum

upper, central region of abdomen

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pyloric stenosis: diagnosis

- often made by hx, pe
- ultrasound
- possible metabolic alkalosis, severe volume depletion

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pyloric stenosis: metabolic consideration

metabolic alkalosis possible, correct before surgery!

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pyloric stenosis: postop considerations

- feed 4-6 hours after
- vomiting is not uncommon
- manage pain

67

intussusception

telescoping of the bowel upon itself

after fixing, only 10% reoccur

68

intussusception: acute clinical manifestations

triad!
- pain: sudden onset of crampy abdominal pain
- sausage shaped abdominal mass
- currant jelly stool

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intussusception: triad - what & when

pain, sausage, currant jelly stool
if ACUTE intussusception

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intussusception: chronic clinical manifestations

diarrhea, anorexia, weight loss, vomiting, periodic pain

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intussusception: therapeutic management

barium/hydrostatic reduction (ie, enema) - 75% success
surgical manipulation
surgical resection

72

ger considered gerd when these complications are seen

failure to thrive, anorexia, irritability, recurrent pneumonia (aspirating feedings)

73

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

74

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)

75

celiac disease: diagnosis & tx

withdraw gluten from diet = full remission of sx

tx 100% dietary management

76

steatorrhea

excessively largel pale, oily, frothy stools
- see in children with celiac disease

77

gastroesophageal reflux

?

78

ger: diagnostic eval

upper gi swallow study

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ger: therapeutic management

feed at higher angle, sit up 30 min after feeds, feed small amount more often, burp a lot, med management possible

80

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

81

nissen fundoplication

after eating, environment, medication management all tried

stomach is its own cardiac sphincter! (wrap around bottom of esophagus)

82

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

83

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)

84

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.