Flashcards in GI Deck (117)
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1
Esophageal Atresia
- portion of conduit replaced by thin, noncanalized cord with blind pouches above and below atretic segment.
- associated with congenital heart defects, GU malformations, and neurologic disorders.
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presentation: regurgitation during feeding.
2
Imperforate Anus
- most common congenital intestinal atresia.
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failure of cloacal membrane to involute.
3
Esophageal Fistula
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connection btw esophagus and trachea or mainstem bronchus.
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swallowed material or gastric fluid can enter the respiratory tract.
- associated with: congenital heart defects, GU malformations, and neurologic disorders.
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presentation: regurgitation during feeding.
4
Esophageal Stenosis
- incomplete form of atresia.
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lumen reduced by fibrous, thickened wall.
- can be congenital or from inflammatory scarring (chronic reflux, irradiation, scleroderma)
5
Congenital Duplication Cysts
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cystic masses with redundant smooth muscle layers throughout GI tract.
6
Diaphragmatic Hernia
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incomplete formation of diaphragm allows cephalad displacement of abd viscera.
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leads to pulmonary hypoplasia incompatable with life.
7
Omphalocele
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abdominal musculature is incomplete and viscera herniate into ventral membranous sac.
- associated with other birth defects in 40%.
8
Gastroschisis
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all layers of abd wall fail to develop (peritoneum to skin).
- viscera herniate into ventral membranous sac.
9
GI Ectopia
- most common gastric mucosa ectopia is proximal esophagus.
- ⇒ dysphagia and esophagitis.
- in small bowel/colon ⇒ occult blood loss or peptic ulceration.
- ⇒ dysphagia and esophagitis.
10
Pancreatic Heterotopia
- ectopic in esophagus and stomach.
- in pylorus ⇒ inflammation, scarring, and obstruction.
11
Meckel Diverticulum
- 2% of population, 2:1 male:female
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persistence of vitelline duct ⇒ outpouching within 85cm of ileocecal valve.
- can have pancreatic tissue or heterotopic gastric mucosa (with peptic ulceration).
12
Diverticulum
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blind pouch leading off alimentary tract, lined by mucosa and including all three layers of bowel wall (mucosa, submucosa, and muscularis propria)
13
Pyloric Stenosis
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congenital hypertrophic pyloric stenosis: 1 in 500 births. 4:1 male:female.
- associated with Turner syndrome and trisomy 18.
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presentation: regurgitation and projectile vomiting within 3 wks of birth, externally visible peristalsis, palpable firm ovoid mass.
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tx: myotomy (full thickness muscle spitting incision).
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acquired pyloric stenosis: from chronic antral gastritis, peptic ulcers close to pylorus, and malignancy.
- associated with Turner syndrome and trisomy 18.
- presentation: regurgitation and projectile vomiting within 3 wks of birth, externally visible peristalsis, palpable firm ovoid mass.
- tx: myotomy (full thickness muscle spitting incision).
14
Hirschsprung Disease
- aka congenital aganglionic megacolon.
- from arrested migration of neural crest cells into the gut ⇒ aganglionic segment lacking peristaltic contractions ⇒ functional obstruction and progressive dilation and hypertrophy of unaffected proximal colon.
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pathogenesis: loss of function mutation in RET tyrosine kinase receptor in 15% sporadic and most familial.
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4:1 male:female.
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presentation: neonatal failure to pass meconium or abd distention with megacolon.
- risk of peroration, sepsis, or enterocolitis with fluid derangement.
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acquired megacolon with Chagas disease (lose ganglia), bowel obstruction, IBD, and psychosomatic disorders.
- 4:1 male:female.
- risk of peroration, sepsis, or enterocolitis with fluid derangement.
- acquired megacolon with Chagas disease (lose ganglia), bowel obstruction, IBD, and psychosomatic disorders.
15
Esophageal Spasm
- short or long-lived, focal or diffuse.
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diffuse ⇒ functional obstruction.
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↑ wall stress can cause diverticula.
↑ wall stress can cause diverticula.
16
Zenker Diverticulum
- aka pharyngeoesophageal diverticulum.
- occurs immediately above upper esophageal sphincter.
- contains one or more wall layers, can accumulate food if large and present as a mass with food regurgitation.
17
Traction Diverticulum
- occurs at esophageal mid-point.
- contains one or more wall layers, when large can accumulate food and present as mass with food regurgitation.
18
Epiphrenic Diverticulum
- occurs immediately above lower esophageal sphincter.
- contains one or more wall layers, when large can accumulate food and present as mass with food regurgitation.
19
Mucosal Webs
-
ledgelike protrusions of fibrovascular tissue and overlying epithelium.
- mostly in upper esophagus in women >40yrs.
20
Plummer-Vinson Syndrome
- aka Paterson-Brown-Kelly Syndrome.
- constellation of webs, iron deficiency anemia, glossitis, and cheilosis.
21
Esophageal Rings
- aka Schatzki rings.
- similar to webs but circumferential and thicker.
- include mucosa, submucosa, and occasionally hypertrophic muscularis propria.
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A rings = above esophageal junction.
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squamous epithelium.
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B rings = at squamocolumnar junction.
- contain gastric cardia-type mucosa.
- squamous epithelium.
- contain gastric cardia-type mucosa.
22
Achalasia
- triad: incomplete relaxation of LES, ↑ LES tone (cholinergic signaling), and esophageal aperistalsis.
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primary: idiopathic, from failure of distal esophageal neurons to induce LES relaxation during swallowing, or degenerative changes in neural innervation.
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secondary: with Chagas disease, disorders of vagal dorsal motor nuclei (polio, surgical ablation), diabetic autonomic neuropathy, infiltrative disorders (amyloid, sarcoid, cancer).
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tx: myotomy, balloon dilation, botulinum toxin injection to inhibit LES cholinergic neurons.
23
Mallory-Weiss Tears
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longitudinal lacerations at gastroesophageal junction associated with excessive vomiting with alcohol intoxication.
- caused by failure of relaxation of LES preceding vomiting. causes stretching and tearing.
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presentation: hematemesis.
- caused by failure of relaxation of LES preceding vomiting. causes stretching and tearing.
24
Chemical and Infectious Esophagitis
- squamous epithelium damaged by alcohol, corrosive acids or alkalis, excessively hot fluids, heavy smoking, irradiation, chemotherapy, graft-versus-host disease.
- infections common in immunocompromised: HSV, CMV, candida.
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morphology: dense neutrophilic infiltrates.
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granulation tissue if ulceration
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candidiasis: adherent grey-white pseudomembrane of fungal hyphae and inflammatory cells.
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HSV: punched-out ulcers.
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CMV: shallow ulcerations with viral inclusions.
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presentation: pain and dysphagia.
- severe/chronic cases: hemorrhage, stricture, perforation.
- granulation tissue if ulceration
- candidiasis: adherent grey-white pseudomembrane of fungal hyphae and inflammatory cells.
- HSV: punched-out ulcers.
- CMV: shallow ulcerations with viral inclusions.
- severe/chronic cases: hemorrhage, stricture, perforation.
25
Reflux Esophagitis
- foremost cause of esophagitis = GERD.
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Pathogenesis: reflux of gastric juices from ↓ LES tone and/or ↑ abd pressure.
- exacerbated by alcohol, tobacco, obesity, CNS depressants, pregnancy, delayed gastric emptying, ↑ gastric volume.
- can be from hiatal hernia when stomach protrudes into thorax.
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Morphology: hyperemia, edema, basal zone hyperplasia and thinning of superficial epithelial layers, neutrophile and/or eosinophil infiltration.
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Presentation: adults >40yrs with dysphagia, heartburn, regurgitation of gastric contents into mouth.
- complications: ulceration, hematemesis, melena, stricture, Barrett esophagus.
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tx: proton pump inhibitors and/or H2 histamine receptor antagonists.
- exacerbated by alcohol, tobacco, obesity, CNS depressants, pregnancy, delayed gastric emptying, ↑ gastric volume.
- can be from hiatal hernia when stomach protrudes into thorax.
- complications: ulceration, hematemesis, melena, stricture, Barrett esophagus.
26
Eosinophilic Esophagitis
- pts have atopic disorders (dermatitis, asthma, etc.)
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morphology: large numbers of intraepithelial eosinophils.
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presentation: food impaction and dysphagia.
- children: feeding intolerance and GERD-like symptoms.
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tx: dietary restriction and/or steroids.
- children: feeding intolerance and GERD-like symptoms.
27
Barrett Esophagus
- complication of 10% chronic GERD.
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intestinal metaplasia within esophageal squamous mucosa.
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↑ risk of esophageal adenocarcinoma. 0.2-2% pts have pre-invasive dysplasia each yr.
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morphology: patches of red, velvety mucosa up from gastroesophageal junction.
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intsetinal-type columnar epithelium with mucin-secreting goblet cells.
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dysplasia is low or high grade.
- intramucosal carcinoma has neoplastic cell invasion in lamina propria.
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presentation: white male btw 40-60yrs. diagnosed both grossly and with biopsy.
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tx: high grade dysplasia or carcinoma needs esophagectomy.
- intsetinal-type columnar epithelium with mucin-secreting goblet cells.
- dysplasia is low or high grade.
- intramucosal carcinoma has neoplastic cell invasion in lamina propria.
28
Esophageal Varices
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pathogenesis: severe portal HTN ⇒ collateral bypass channels btw portal and caval circulations.
- ⇒ congested subepithelial and submucosal veins in distal esophagus = varices.
- most common cause in west = alcoholic cirrhosis
- most common worldwide = hepatic schistosomiasis
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morphology: tortuous dilated veins in distal esophageal and proximal gastric submucosa.
- irregular luminal protrusion of overlying mucosa with superficial ulceration, inflammation, or adherent blood clots.
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presentation: silent until rupture with catastrophic hematemesis.
- rupture from inflammatory erosion, ↑ venous pressure, ↑ hydrostatic pressure from vomiting.
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50% die of first bleed from exsanguination or hepatic coma.
- 50% chance recurrence.
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tx: scleroherapy, balloon tamponade, band ligation.
- ⇒ congested subepithelial and submucosal veins in distal esophagus = varices.
- most common cause in west = alcoholic cirrhosis
- most common worldwide = hepatic schistosomiasis
- irregular luminal protrusion of overlying mucosa with superficial ulceration, inflammation, or adherent blood clots.
- rupture from inflammatory erosion, ↑ venous pressure, ↑ hydrostatic pressure from vomiting.
- 50% die of first bleed from exsanguination or hepatic coma.
- 50% chance recurrence.
29
Esophageal Adenocarcinoma
- come from dysplasia in Barret mucosa.
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7:1 male:female
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pathogenesis: early chromosomal and p53 mutations, amplification of c-ERB-B2, cyclin D, E genes, mutated RB, p16/INK4a cyclin dependent kinase inhibitor.
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morphology: gross = exophytic nodules to excavated and deeply infiltrative masses in distal 1/3 esophagus.
- micro: form glands, produce mucin, intestinal morphology. signet ring tumors not common.
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presentation: white male with dysphagia, weight loss, hematemesis, chest pian, or vomiting.
- 5 yr survival <25%.
- micro: form glands, produce mucin, intestinal morphology. signet ring tumors not common.
- 5 yr survival <25%.
30
Squamous Cell Carcinoma (Esophageal)
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adults >45yrs. 4:1 male:female, blacks>whites
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risk factors: alcohol, tobacco, caustic esophageal injury, achalasia, Plummer-Vinson syndrome, scalding hot beverages.
- high incidence in Iran, central China, Hong Kong, Brazil, South Africa.
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morphology: 50% in middle 1/3 of esophagus.
- begin in situ as gray-white plaque-like mucosal thickenings.
- expand as exophytic, ulcerate, become diffusely infiltrative with wall thickenings and luminal stenosis.
- submucosal lymphatic network promotes circumferential and longitudinal spread.
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mod to well defined. less comon verrucous, spindle, and basaloid carcinomas.
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presentation: insidious, late symptoms, dysphagia, obstruction, weight loss, hemorrhage, sepsis from ulceration, respiratory fistulae with aspiration.
- 5 yr survival is 75% if superficial, otherwise 9%.
- begin in situ as gray-white plaque-like mucosal thickenings.
- expand as exophytic, ulcerate, become diffusely infiltrative with wall thickenings and luminal stenosis.
- submucosal lymphatic network promotes circumferential and longitudinal spread.
- mod to well defined. less comon verrucous, spindle, and basaloid carcinomas.
- 5 yr survival is 75% if superficial, otherwise 9%.