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N320 Neo Peds Pathophysiology > Wk 6 GI Diseases > Flashcards

Wk 6 GI Diseases Flashcards

1
Q

What is gastroesophageal reflux?

A

Intrusion of acids into the dilated esophagus resulting into the deterioration of the esophagus’ epithileum & stimulation of the vomiting reflex.

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2
Q

What is the etiology of gastroesophageal reflux?

A

Low cardiac sphincter pressure

Short cardiac sphincter

Transient/prolonged relaxation of LES

Poor coordination of esophageal motility

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3
Q

What are signs & symptoms of GERD?

A

Vomiting

Persistent cough

Crying w/ feeding

Choking/gagging with feeding

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4
Q

What medical problems can GERD lead to?

A

Poor weight gain

Aspiration

Pneumonia

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5
Q

How do you diagnose GERD?

A

Clinical s/s often adequate to diagnose

Esophageal pH monitoring w/ a probe for 24 hrs

Endoscopy

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6
Q

What is the treatment for GERD?

A

Usually resolves w/ age

Mild GERD = no treatment.

Upright positioning

Thickened feeds OR small frequent feeds w/ freq. burping

Gastric acid blockers, metoclopramide (reglan), & antacids

Surgery (gastropexy or fundoplication - recreates a valve by wrapping fundus of stomach around the esophagus)

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7
Q

What is esophageal atresia (EA)?

A

When the esophagus ends in a blind pouch & does not connect to the stomach

Can happen in conjunction w/ tracheoesophageal fistula

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8
Q

What is a tracheoesophageal fistula (TEF)?

A

An abnormal connection btwn the esohagus & the trachea

Can happen in conjunction with esophageal atresia

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9
Q

What is the etiology of esophageal atresia & tracheosophageal fistula?

A

An incomplete division of the oforegut into the respiratory & digestive portions at wk 4 of gestation.

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10
Q

Esophageal atresia & tracheoesophageal fistula can be an isolated defect or appear as part of what syndrome(s)?

A

VATER, VACTERL, & CHARGE

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11
Q

What does VATER syndrome/ VATERL syndrome stand for?

A

Anomalies of the following:

Vertebrae

Anus

Trachea

Esophagus

Renal

Limb

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12
Q

What does CHARGE syndrome stand for?

A

CNS anomalies

Heart defects

Atresia of nasal passage/choana

Retardation of growth/dvlpment

Genital/urinary defects

Ear anomalies/deafness

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13
Q

What are prenatal s/s of esophageal atresia & tracheosophageal fistula?

A

polyhydramnios

no stomach bubble in US

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14
Q

What are postnatal s/s of esopheal atresia & tracheoesophageal fistula?

A

OG/NG tube does not pass

Excess salivation

Nonbilious vomiting

Feeding intolerance

Respiratory distress w/ feeds

Abdominal distention (TEF when abdomen fills w/ air)

Intermittent cyanosis

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15
Q

What is the treatment for esophageal atresia & tracheosophageal fistula?

A

Usu. diagnosed @ birth

Inability to pass NG/OG tube

prenatal US

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16
Q

What medical problems can esophageal atresia & tracheoesophageal fistula lead to?

A

recurrent aspiration

pneumonia

atelectasis

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17
Q

What is the treatment for esophageal atresia & tracheoesophageal fistula?

A

Surgery to restore esophageal continuity & eliminate fistula.

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18
Q

What is duodenal atresia?

A

The complete closure of the duodenum - causes an intrinsic obstruction

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19
Q

What is the etiology of duodenal atresia?

A

Failure of bow to recannulize in 2nd month of gestation.

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20
Q

What are prenatal s/s of duodenal atresia?

A

Polyhydramnios

Distended duodenum

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21
Q

What symptom is a medical emergency until proven otherwise?

A

Bilious emesis

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22
Q

What are postnatal s/s of duodenal atresia?

A

Bilious emesis - 1st 24 hrs of life

Abdominal distention of upper quadrants

KUB = double bubble (see pic showing air trapped in stomach & air trapped in proximal duodenum; separated by the pyloric sphincter - why looks like double bubble)

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23
Q

What is the treatment for duodenal atresia?

A

Decompression

Surgery

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24
Q

What disorders have a high rate of association w/ duodenal atresia?

A

Trisomy 21

CHD

Malrotation

Annular pancreas

EA

Genitourinary anomalies

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25
Q

What extrinsic causes could result in duodenal obstruction?

A

Malrotation/volvulus

Annular pancreas

Bowel duplications

Retroperitoneal tumors

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26
Q

What is an annular pancreas?

A

when the pancreas surrounds the second part of the duodenum causing constriction or blockage of the intestines.

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27
Q

What is the etiology of the annular pancreas?

A

when the ventral bud rotates counter-clockwise

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28
Q

What are the s/s of annular pancreas?

A

Polyhdramnios

Feeding intolerance

Low birth weight

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29
Q

What is the treatment for annular pancreas?

A

Surgical bypass of the blocked part of the duodenum

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30
Q

What is a malrotation?

A

When the intestines fail to rotate properly and affix improperly (the cecum ends up in the RUQ instead RLQ) & an abnormal membrane called the Ladd’s band may obstruct the duodenum (see pic)

Leads to an increase likelihood of volvulus.

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31
Q

What is volvulus?

A

When the loops of the intestine twist on themselves causing obstruction due to malrotation

Can cause an infarct or necrosis of the entire mid-gut.

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32
Q

What is the etiology of malrotation?

A

Arrest of intestinal -270 degree rotation of the bowel in the 2nd month of gestation

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33
Q

What percentage of children pressent w/ s/s of malrotation/volvulus by their 1st week, 1st month, & 1st year?

A

1st week - 50%

1st month - 80%

1st year - 90%

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34
Q

What are the s/s of malrotation/volvulus?

A

Bilious emesis

Volvulus - acute episode of rectal bleeding & shock

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35
Q

How do you diagnose malrotation?

A

Clinical s/s

X ray shows gas bubbles & distention proximal to the site of obstruction.

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36
Q

What is the treatment of malrotation/volvulus?

A

Malrotation = Ladd’s procedure - surgical division of Ladd’s bands, widening of small intestine’s mesentary, appendectomy, & correctional placement of colon & cecum

Volvulus = opening abdomen & reducing volvulus manually & performing Ladd’s procedure; necrotic bowel can be resected & good bowel anastomosed.

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37
Q

What is the most common cause of exocrine pancreatic insufficiency?

A

Cystic fibrosis

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38
Q

What is the most common cause of endocrine pancreatic insufficiency?

A
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39
Q

What is pancreatic insufficiency? Treatment.

A

The deficient production of prancreatic enzymes required to digest proteins, carbohydrates, & fats. The main problem is maldigestion of fats –> results in malnutrition, weight loss, & steatorrhea (large amt of fat in stool)

Treatment = lipase supplementation

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40
Q

Within what timeframe should a newborn pass stool?

A

Within 48 hrs

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41
Q

What is jejunal-ileal atresia?

A

When the intestine ends blindly w/ or w/o a gap in the mesentary.

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42
Q

Jejunal-ileal atresia is more common than duodenal or colonic atresia. True or false.

A

True

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43
Q

What is the etiology of jejunal-ileal atresia?

A

Mesenteric vascular occlusion in utero

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44
Q

What are the s/s of jejunal-ileal atresia?

A

SGA

1/3 polyhydramnios (rare in ileal atresia)

1/3 jaundice

Bilious emesis

Abd. distention - the more distention, the lower the lesion

May or may not pass meconium

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45
Q

How do you diagnose jejunal-ileal atresia?

A

KUB = dilated loops w/ air-fluid levels, the more air, the lower the obstruction

Calcifications

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46
Q

What is the treatment for jejunal-ileal atresia?

A

Surgery - take out the necrosed bowel & re-anastomose the rest.

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47
Q

Which disease is known as the disease of 2s?

A

Meckel diverticulum

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48
Q

What is Meckel diverticulum?

A

Outpouching from the small intestine involving all 3 intestinal layers

Can end in a blind pouch, a blind ending passage frm the umbilicus, a cyst, or cord-like attachment frm the umbilicus to the intestine (can cause obstruction if intestine twists around the band)

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49
Q

Why is Meckel diverticulum known as the disease of 2s?

A

Occurs in 2% of all infants at 2 yrs of age with boys 2x more affected

2 inch outpouching of ileum, 2 feet frm the ileocecal valve

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50
Q

What are the s/s of Meckel diverticulum?

A

Painless rectal bleeding

Bowel obstruction

Diverticulitis

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51
Q

How do you diagnose Meckel diverticulum?

A

radionuclide scan Tc-99m - detects gastric mucosa

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52
Q

What is the treatment for Meckel diverticulum?

A

Surgical resection

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53
Q

What is the etiology of Meckel diverticulum?

A

Remannt of the vitelline duct that involutes in 5-7wks during gestation

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54
Q

What is Hirschsprung disease?

A

Absence of gangion cells in the bowel wall causing an obstruction

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55
Q

Hirschsprung dx is also known as…

A

Congenital aganglionic megacolon

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56
Q

What is the etiology of Hirschsprung disease?

A

Failure of the neural crest cells to migrate frm the proximal to distal bowel at 8-10 wks gestation

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57
Q

What is the most frequent GI anomaly?

A

Meckel diverticulum

58
Q

Is Hirschsprung disease more common in males or females?

A

Males 4:1

59
Q

What is the most common cause of lower intestinal obstruction in neonates?

A

Hirschsprung disease

60
Q

What are the s/s of Hirschsprung disease?

A

No meconium in 1st 24-48 hrs

Abd. distention

Constipation / foul smelling diarrhea

Bloody diarrhea

FTT

urinary obstruction

Acute bacterial enterocolitis (25-30% mortality)

61
Q

What would increase your risk for Hirschsprung dx?

A

Positive family hx (incr. w/ length of affected bowel)

Siblings w/ Hirschsprung

Trisomy 21

62
Q

What is meconium plug syndrome?

A

Transient obstruction of the colon caused by functional immaturity of the colon characterized by a delayed passage of meconium & intestinal dilation

Presents clinically the same as meconium ileus

63
Q

Meconium plug syndrome is also known as…

A

Small left colon syndrome

64
Q

What are the s/s of meconium plug?

A

no stool w/in 48 hrs

abdominal distention

emesis

65
Q

When does meconium plug syndrome occur?

A

1st 3 days of life

66
Q

What is the etiology of meconium plug syndrome?

A

IDM (50%) due to hypoglycemia

Hypermagnesia - causes reduced acetylcholine release & myoneural depression –> neonate can be this is mom has received Mag sulfate as treatment

Systemic sepsis (hypoglycemia)

Functional immaturity of myenteric plexus

67
Q

What is the treatment for meconium plug?

A

Rectal exam

Contrast enema

Surgery (in rare cases)

Rectal biopsy for Hirschsprung’s dx & testing for CF

68
Q

What is meconium ileus?

A

Obstruction of the ileus caused by hyperviscous secretions frm the mucous glands of the small intestine.

Meconium has decr. H20 content & adheres to intestinal lining & has elevated concentrations of albumin

69
Q

What medical condition is a concern & should be tested for if a neonate is found to have meconium ileus?

A

Cystic fibrosis - 90% have meconium ileus

70
Q

What are the s/s of meconium ileus?

A

no stool w/in 48 hrs

abdominal distention

palpable bowel loops

bilious emesis

71
Q

What is the treatment for meconium ileus?

A

Hypertonic enemas - Mucomyst enemas (60% success rate)

Surgery

72
Q

What other conditions can complicate meconium ileus?

A

Volvulus

Intestininal necrosis

Perforation

Meconium peritonitis - rupture of bowel prenatally leading to inflammation of the peritoneum due to fetal stool in the space.

Meconium pseudocyst - when the meconium is walled off in the peritoneal space.

73
Q

What is gastrochisis?

A

When abdominal contents protrude b/c of an abdominal wall defect without a sac to the right of the umbilicus.

74
Q

What is an omphalocele?

A

When abdominal contents protrude out the abdominal cavity covered by a sac (unless ruptured) due to a failure of the gut to retract back to the abdominal cavity from the umbilical cord at 8-11 wks gestation.

75
Q

What is the treatment for omphalocele?

A

Surgery at 6-12 mos. to allow abdominal cavity to enlarge.

Gradual reduction by enlarging abdominal cavity accomodate intestinal contents. A plastic pouch is placed over the abdominal contents to contain the bowel & aid in reduction until surgical closure.

76
Q

What is the treatment for gastroschisis?

A

Silicone pouch (silo) placed over the bowel & gradually pushed slowly back into the abdomen over a period of days & abdominal opening closed surgically.

77
Q

Which has greater incidence, gastroschisis or omphalocele?

A

Gastroschisis

78
Q

When comparing gastroschisis to omphalocele, is there a difference in occurence with regards to gender ?

A

Gastroschisis - occurs equally

Omphalocele - more males 3:1

79
Q

Btwn gastroschises and omphalocele, omphalocele is more likely to occur in babies of young mothers. True or false.

A

False - gastroschisis is more likely to occur in babies of young mothers.

80
Q

Comparing gastroschisis to omphalocele, is there a difference in the location & size of the defect?

A

Gastroschisis - right lateral to umbilicus & < 4cm

Ompalocele - central (umbilical ring) & > 4cm

81
Q

In comparison to omphalocele, gastroschisis is more frequently associated w/ anomalies. True or false.

A

False. Gastroschisis infreq. associated w/ anomalies (if it is, usu. GI atresias)

Omphalocele is frequently associated with CV, GU, craniofacial, & skeletal anomalies

82
Q

In comparison to omphalocele, gastroschisis is a better diagnosis & has a better survival rate. True or false.

A

True - gastroschisis = >90% survival rate

Omphalocele = 90% survival rate w/o other anomalies, 60-70% survival rate w/ anomalies

83
Q

What is an ileus?

A

Paralysis / partial paralysis of intestinal propulsion from non-mechanical reasons causing obstruction of the bowel

84
Q

What are s/s of ileus?

A

abd. pain

constipation

abd. distention

N/V

lack of flatulence or BM

85
Q

What is the etiology of ileus?

A

Surgery (postsurgical ileus)

Drugs

Inflammation

Electrolyte imbalance - hypermagnesia = slow bowel movement

86
Q

Name 3 syndromes associated w/ GI malformations.

A

Trisomy 21 (down syndrome)

Beckwith-Wiedemann

Pentalogy of Cantrell

87
Q

Which GI anomalies are children w/ trisomy 21 more likely to have?

A

TEF

Pyloric stenosis

Duodenal atresia

Hirschsprung dx

Annular pancreas

Imperforate anus

88
Q

What percentage of Trisomy 21 children have GI anomalies?

A

12%

89
Q

What is Beckwith-Wiedemann?

A

Chromosmal defect of chromosome 11 causing visceral marosomia (large organs) associated w/ macroglossia (enlarged tongue), diastesis recti (separation of abdominal muscles), omphalocele, ear creases/pits, cryptorchidism (undescended testes)

90
Q

What is pentalogy of Cantrell?

A

Failure of growth & fusion of the lateral & cephalic folds.

5 distinct defects: 1) Cleft sternum (failed fusion of sternum)

2) Anterior midline diaphragmatic defect
3) Pericardial defect
4) CHD including ectopic cordis (when heart partially or totally outside of thorax)
5) High omphalocele

91
Q

What is biliary atresia?

A

Absence or closure of ducts that drain bile from the liver causing bile to be trapped in the liver –> can cause damage, cirrhosis, & liver failure

92
Q

What is the etiology of biliary atresia?

A

Uknown

93
Q

Biliary atresia is more common in females & Asians & African Americans. True or false.

A

True

94
Q

At what age should you be suspicious of biliary atresia if the child presents w/ jaundice?

A

4 wks

95
Q

What are the s/s of biliary atresia?

A

Jaundice

Hepatomegaly

Darker urine

Lack of color to stools

FTT

96
Q

How would you diagnose biliary atresia?

A

Abdominal xray, US, HIDA scan (in 4 & 24 hrs)

Labs: liver enzymes, conjugated (direct) bilirubin - hyperbilirubinemia does not lead to kernicterus

Liver biopsy

97
Q

Desribe the difference between intra-hepatic & extra-hepatic biliary atresia.

A

Intra-hepatic: failure of duct formation in fetus

Extra-hepatic: progressive inflammatory process, ducts outside liver are affected 1st –> WBCs invade the ducts which get damaged & become withered or disappear.

98
Q

What is the treatment for biliary atresia?

A

Kasai procedure by 1st 3 months of life - removal of atretic biliary ducts & attaching small intestine directly to liver at the spot where bile expected to drain.

Liver transplant - 50% of children will need by age 5

99
Q

What congenital anomaly is associated with pyloric stenosis?

A

Tracheoeophageal fistula

100
Q

What is pyloric stenosis?

A

Narrowing of the pylorus due to muscle hypertrophy

101
Q

What is the etiology of pyloric stenosis?

A

Uknown

102
Q

What factors may increase the occurrence of pyloric stenosis?

A

Male (first born) 4:1

Family hx (mother > father)

Type B & O

103
Q

What are s/s of pyloric stenosis?

A

non-bilious projectile vomiting

“olive,” visible peristaltic wave

not anorexic - child still hungry

104
Q

How do you diagnose pyloric stenosis?

A

Clinical s/s

US (thickness of pylorus > or = 4mm, length > or = 14mm)

UGI

105
Q

What is the treatment for pyloric stenosis?

A

Correct fluid, acid-base, & electrolyte losses

Ramstedt pyloromyotomy - dividing the muscle of the pylorus to widen it’s opening

106
Q

What is nectrotizing enterocolitis (NEC)?

A

An ischemic inflammatory condition of the bowel that causes necrosis, perforation, & death if untreated.

107
Q

What is the greatest risk factor of NEC?

A

prematurity

108
Q

Cocaine is a risk factor for NEC. True or false.

A

True

109
Q

When is NEC likely to occur?

A

3 days - 3 wks postnatally

110
Q

What is the etiology of NEC?

A

Unknown

111
Q

Describe the pathophysiology of NEC.

A

1) Mucosal injury & poor host protection
2) Hypoxic insult
3) Enteral feedings
4) Flora
5) Inflammatory mediators
6) Migratory motor complexes - waves of activity that sweep through the intestine during a fasting state –> stimulate peristalsis & transports bacteria frm small intestine to large intestine

112
Q

What are the enteric s/s of NEC?

A

Abd. distention & tenderness

Abd. wall mass, erythema, & induration

Ascites

Bloody stools

Emesis

Gastric aspirates (can be + / - bilious)

Ileus

113
Q

What are the systemic s/s of NEC?

A

Resp: Respiratory distress, apnea

CV: Bradycardia, poor peripheral profusion, hypotension

Neuro: Lethargy, irritability, temp instability

GI/GU: Poor feeding, acidosis, oliguria

Heme: bleeding diathesis (tendency to bleed)

114
Q

Besides clinical s/s how do you diagnose NEC?

A

Blood work - look for blood work triad

Stool analysis: hemoccult test, clinitest (carbohydrate malabsorption)

Cultures: (blood, urine, stool, CSF)

KUB - bowel gas, ileus, bowel wall edema, ascites, air

115
Q

What is the blood work triad of NEC?

A

Acidosis

Hyponatremia

Thrombocytopenia

116
Q

What is pneumoperitoneum?

A

Air trapped in the peritoneal cavity outside the lumen of the bowel –> signals perforation

117
Q

What is pneumatosis?

A

The presence of gas WITHIN the walls of the intestine, indicative of NEC

118
Q

What are the 3 diffrent types of pneumatosis? (inner to outer)

A

Linear submucosal pneumatosis - btwn mucosa & submucosa

Cystic submucosal pneumatosis - bubbly air trapped w/in the submucosa

Linear subserosal pneumatosis - underneath the serosa

119
Q

What is Bell Staging?

A

Criteria in stages for NEC

120
Q

What is Bell Stage I?

A

Suspect NEC

Apnea, bradycardia, temp instability, positive stools/frank blood

121
Q

What is Bell Stage II?

A

Definite NEC (mild-moderate)

Stage I + abd. tenderness, absent bowel sounds, abd. wall discoloration, R lower quadrant mass

Mild metabolic acidosis, mild thrombocytopenia

Intestinal dilation, pneumatosis, portal venous gas

122
Q

What is Bell Stage III?

A

Severe NEC

Stage II + peritonitis, hypotension, shock

Severe metabolic acidosis, DIC, neutropenia, hyponatremia

Ascites, pneumoperitoneum

123
Q

What is treatment for NEC?

A

NPO, parenteral nutrition support

Bowel decompression

Broad spectrum abx

Respiratory support

Correct acidosis, hypotension, thrombocytopenia, anemia, & DIC

Monitor renal function

Serial labs & radiographs

Surgery: perforation, necrosis, portal venous air

124
Q

What are medical sequelae to NEC?

A

Strictures, enteric fistulas, short bowel syndrome, malabsorption, chronic diarrhea, dumping syndromes, FTT

Hepatitis & cholestasis (frm longterm TPN)

Infection (frm longterm central line)

Prolonged need for respiratory support

Dev delay, metabolic bone dx

125
Q

What can we do to prevent NEC?

A

Prevent prematurity

Antenatal steroids

Conservative feeding approach

Oral immunoglobulins

Arginine supplementation

Oral probiotics

126
Q

What is intussusception?

A

When a segment of the bowel into a segment distal to it; gets caught in downstream peristalsis & is pulled into the distal bowel –> can cause necrosis & perforation

Can occur anywhere in the small bowel & colon

127
Q

What are the s/s of intussusception?

A

Classic triad - intermittent colicky pain, vomiting, bloody stool

Dance sign

Tender & distended abdomen = signs of peritoneal irritation/necrosis or perforation

128
Q

What is the classic triad s/s of intussusception?

A

Classic triad: intermittent / colicky abdominal pain, non-bilious vomiting, bloody currant jelly stools

129
Q

What is the Dance sign of intussusception?

A

Pathognomic sign

Palpation of the abdomen may reveal a sausage-like mass in the right upper quadrant representing the actual intussusception, and an empty space in the right lower quadrant representing the movement of the cecum out of its normal position.

130
Q

When is the most likely age for intussusception?

A

3 mos - 6 y/o

131
Q

What is the most common type of intussusception?

A

Ileocolic intussusception

132
Q

Distinguish btwn intussusceptum and intussuscipiens.

A

Intussusceptum - proximal intestine that prolapses

Intussuscipiens - distal intestine into which the proximal intestine prolapses

So in ileocolic intussusception, the intussusceptum is the ileus & the intussuscipiens is the colon.

133
Q

How would you diagnose intussusception?

A

Clinical s/s

CBC / manual diff, electrolytes, glucose

KUB: look for normal vs obstructive, free air

Contrast air enema

US

134
Q

In intussusception, what is the gold standard for diagnosis & therapy? Describe what intussusception looks like on film.

A

Contrast air enema

Coiled spring appearance on the film, “cervix-like” mass as contrast reaches intussusception

135
Q

Can intussusception reoccur?

A

Yes

136
Q

What is toxic megacolon?

A

An acute form of colonic distention - a complication of inflammatory bowel disease (IBS)

137
Q

What is appendicitis?

A

Obstruction of the appendix with bacterial overgrowth, inflammation, ischemia & perforation

138
Q

Which gender & at what age is appendicitis more likely?

A

Males

Peaks in late teens - early 20s

139
Q

What is diverticulitis?

A

herniations or saclike outpouchings of mucosa through muscle layers of colon wall

140
Q

What is the etiology of diverticulitis?

A

Unknown, but associated with decreased dietary fiber and intracolonic pressure

141
Q

What is the pathogenesis of diverticulitis?

A

Habitual consumption of low residue diet decreases fecal bulk and decreases diameter of colon

Hypertrophy and contraction of muscle layers increase intraluminal pressure and degree of herniation

Can cause abscess formation, fistula, peritonitis or obstruction

142
Q

What are s/s of diverticulitis?

A

Carmping pain

Diarrhea / constipation

Diverticula w/ obstructed opening b/comes inflamed or forms an abscess = fever, leukocytosis, tenderness in LLQ

Rare: hemorrhage, perforation with peritonitis, bowel obstruction, and fistula

N320 Neo Peds Pathophysiology (3 decks)

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