Esophagus Path I

Question 1

VACTERL association

Answer 1

vertebral anomalies
anal atresia
cardiac defects
TEF/esophageal atresia
renal and radial abnormalities
limb defects

non random birth defects - cause of association unknown

Question 2

salivation, choking, vomiting, cyanosis with feeding

Answer 2

TEF - tracheoesophageal fistula

Question 3

polyhydramnios in utero

Answer 3

esophageal atresia

Question 4

most common congenital esophagus defect

Answer 4

blinding ending superior esophagus

fistula of trachea to lower esophagus

Question 5

most common intestinal atresia

Answer 5

imperforate anus

Question 6

pyloric stenosis

Answer 6

projectile vomiting 2 to 8 weeks of age

males

hypertrophied smooth m spasm on emptying of stomach

Question 7

hypokalemic hypochloremic metabolic alkalosis

Answer 7

with pyloric stenosis - loss of gastric acid

also secondary hyperaldosteronism with hypovolemia

Question 8

nipple sign

Answer 8

seen with pyloric stenosis

Question 9

tx of pyloric stenosis

Answer 9

cut smooth m. to correct stenosis

Question 10

double bubble

Answer 10

duodenal atresia

and annular pancreas

Question 11

duodenal atresia

Answer 11

trisomy 21

majority below ampulla of vater

double bubble

Question 12

low lesion imperforate anus

Answer 12

colon close to skin

common in females

anal stenosis or rectum ends in blind pouch

Question 13

high lesion imperforate anus

Answer 13

colon higher up in pelvis
-fistula to bladder, urethra, vagina

look for other congenital defects

like VACTERL association

Question 14

anoplasty

Answer 14

tx of imperforate anus

-making a new butthole

Question 15

persitant cloaca

Answer 15

rectum, vagina, urinary tract joined in single tract/channel

Question 16

diaphragmatic hernia

Answer 16

incomplete formation of diaphragm

-with pulmonary hypoplasia

Question 17

bochdalek hernia

Answer 17

postero-lateral hernia

• more common
• majority on left side

Question 18

morgagni hernia

Answer 18

anterior defect of hernia

-adjacent to xiphoid of sternum

Question 19

diaphragm eventration

Answer 19

abnormal displacement of diaphragm

-results in pulmonary hypoplasia and HTN

Question 20

omphalocele

Answer 20

outpouching of peritoneum at the umbilicus

-covered by amnion

Question 21

gastroschisis

Answer 21

abdominal contents protrude
-no cover by amnion

majority to right of umbilicus

tx- stuff into opening - use gelly band

Question 22

maternal serum alpha-fetoprotein

Answer 22

screening for gastroschisis - second trimester of pregnancy

Question 23

ectopic tissue rest

Answer 23

congenital anomaly
-presence of gastric or pancreatic tissue

can lead to inflammation, bleeding, scarring, and obstruction

inlet patch** - upper 1/3 esophagus

Question 24

inlet patch

Answer 24

island of pink mucosa surrounded by normal white squamous esophagus epithelium

-islant of heterotopic gastric** mucosa - below UES

with ectopic tissue rest

often asymptomatic

rarely - acid - ulceration and dysphagia

also adenocarcinoma risk

often colonized by h pylori

Question 25

meckel diverticulum

Answer 25

most common malformation of small bowel -failure of involution of vitelline duct antimesenteric - with own blood supply true diverticulum - all 3 layers of cells may harbor ectopic tissue - gastric/pancreatic

Question 26

rules of 2s

Answer 26

with meckel diverticulum ``` within 2 feet of ileocecal valve 2 inch length 2 ectopic tissue types 2 years most common age 2:1 male female ration ```

Question 27

meckel clinical

Answer 27

often asymptomatic - silent symptoms - before age 2 - painless rectal bleeding -volvulus, intussesception, obstruction may appear as appendicitis**

Question 28

present like appendicitis

Answer 28

meckel diverticulum

Question 29

technetium 99m scan

Answer 29

diagnosis of meckels detects gastric mcosa presence noninvasive and highly specific/sensitive in kids not as good in adults

Question 30

meckels diverticulum path

Answer 30

proximal vitelline duct failus to regress and involute -has own blood supply

Question 31

hirschsprung disease

Answer 31

megacolon -lack meissner and auerbach plexuses and ganglion cells rectum always affected** receptor kinase susceptibility genes

Question 32

hirschsprung path

Answer 32

normal migration of neural crest cells from cecum to rectum during embryogenesis fails bowel constricts - proximal distension with feces NO ganglion cells present

Question 33

no passing of stool in newborn

Answer 33

hirschsprung green or brown vomit, explosive stools after finger in rectum - abdominal swelling - gas and blood diarrhea

Question 34

finger in rectum - poo explosion!

Answer 34

hirschsprung

Question 35

diagnosis of hirschsprung

Answer 35

suction biopsy lack of ganglion cells gold standard

Question 36

tx hirschsprung

Answer 36

resect part of colon

Question 37

LES

Answer 37

physiologic construct | -prevents reflux and regurg of gastric contents

Question 38

glandular mucosa

Answer 38

1 cell thick

Question 39

squamous mucosa

Answer 39

numerous cells thick

Question 40

odynophagia

Answer 40

pain associated with swallowing

Question 41

pyrosis

Answer 41

heartburn

Question 42

dysphagia

Answer 42

difficulty of discomfort swallowing

Question 43

oropharyngeal dysphagia

Answer 43

difficulty transfer** food to esophagus or initiation of swallowing

Question 44

esophageal dysphagia

Answer 44

difficulty transport** down esophagus nutcracker

Question 45

nutcracker esophagus

Answer 45

disorder of movement of esophagus 60-70yo often chest pain - intermittent

Question 46

dx of nutcracker

Answer 46

esophageal manometry - motility study

Question 47

motility disorders

Answer 47

chest pain or dysphagia

Question 48

steakhouse syndrome

Answer 48

sudden obstruction of esophagus - require urgent treatment