Esophagus Path I Flashcards
VACTERL association
vertebral anomalies anal atresia cardiac defects TEF/esophageal atresia renal and radial abnormalities limb defects
non random birth defects - cause of association unknown
salivation, choking, vomiting, cyanosis with feeding
TEF - tracheoesophageal fistula
polyhydramnios in utero
esophageal atresia
most common congenital esophagus defect
blinding ending superior esophagus
fistula of trachea to lower esophagus
most common intestinal atresia
imperforate anus
pyloric stenosis
projectile vomiting 2 to 8 weeks of age
males
hypertrophied smooth m spasm on emptying of stomach
hypokalemic hypochloremic metabolic alkalosis
with pyloric stenosis - loss of gastric acid
also secondary hyperaldosteronism with hypovolemia
nipple sign
seen with pyloric stenosis
tx of pyloric stenosis
cut smooth m. to correct stenosis
double bubble
duodenal atresia
and annular pancreas
duodenal atresia
trisomy 21
majority below ampulla of vater
double bubble
low lesion imperforate anus
colon close to skin
common in females
anal stenosis or rectum ends in blind pouch
high lesion imperforate anus
colon higher up in pelvis
-fistula to bladder, urethra, vagina
look for other congenital defects
like VACTERL association
anoplasty
tx of imperforate anus
-making a new butthole
persitant cloaca
rectum, vagina, urinary tract joined in single tract/channel
diaphragmatic hernia
incomplete formation of diaphragm
-with pulmonary hypoplasia
bochdalek hernia
postero-lateral hernia
- more common
- majority on left side
morgagni hernia
anterior defect of hernia
-adjacent to xiphoid of sternum
diaphragm eventration
abnormal displacement of diaphragm
-results in pulmonary hypoplasia and HTN
omphalocele
outpouching of peritoneum at the umbilicus
-covered by amnion
gastroschisis
abdominal contents protrude
-no cover by amnion
majority to right of umbilicus
tx- stuff into opening - use gelly band
maternal serum alpha-fetoprotein
screening for gastroschisis - second trimester of pregnancy
ectopic tissue rest
congenital anomaly
-presence of gastric or pancreatic tissue
can lead to inflammation, bleeding, scarring, and obstruction
inlet patch** - upper 1/3 esophagus
inlet patch
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
meckel diverticulum
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
rules of 2s
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
meckel clinical
often asymptomatic - silent
symptoms - before age 2 - painless rectal bleeding
-volvulus, intussesception, obstruction
may appear as appendicitis**
present like appendicitis
meckel diverticulum
technetium 99m scan
diagnosis of meckels
detects gastric mcosa presence
noninvasive and highly specific/sensitive in kids
not as good in adults
meckels diverticulum path
proximal vitelline duct failus to regress and involute
-has own blood supply
hirschsprung disease
megacolon
-lack meissner and auerbach plexuses and ganglion cells
rectum always affected**
receptor kinase susceptibility genes
hirschsprung path
normal migration of neural crest cells from cecum to rectum during embryogenesis fails
bowel constricts - proximal distension with feces
NO ganglion cells present
no passing of stool in newborn
hirschsprung
green or brown vomit, explosive stools after finger in rectum - abdominal swelling - gas and blood diarrhea
finger in rectum - poo explosion!
hirschsprung
diagnosis of hirschsprung
suction biopsy
lack of ganglion cells
gold standard
tx hirschsprung
resect part of colon
LES
physiologic construct
-prevents reflux and regurg of gastric contents
glandular mucosa
1 cell thick
squamous mucosa
numerous cells thick
odynophagia
pain associated with swallowing
pyrosis
heartburn
dysphagia
difficulty of discomfort swallowing
oropharyngeal dysphagia
difficulty transfer** food to esophagus or initiation of swallowing
esophageal dysphagia
difficulty transport** down esophagus
nutcracker
nutcracker esophagus
disorder of movement of esophagus
60-70yo
often chest pain - intermittent
dx of nutcracker
esophageal manometry - motility study
motility disorders
chest pain or dysphagia
steakhouse syndrome
sudden obstruction of esophagus - require urgent treatment
