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GI 2 Pathology--Chapt 16 and 17 > Chapter 17 part 1--GI tract--Congenital Abnormalities and Esophagus > Flashcards

Chapter 17 part 1--GI tract--Congenital Abnormalities and Esophagus Flashcards

1
Q

Congenital Abnormalities–List (4)

A
  • Atresia
  • Fistula
  • Duplication
  • Stenosis
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2
Q

When and how do artesias, fistulas, and duplications present? Tx?

A
  • When in esophagus, they present shortly after birth with regurgitation during feeding
  • need prompt surgical correction!
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3
Q

Esophageal atresia is also associated with??

A
  • Congenital heart defects
  • Genitourinary malformation
  • neurologic disorders
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4
Q

Esophageal Atresia

A

-portion of conduit is replaced by a thin, non canalized cord with blind pouches above and below atretic segment

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5
Q

Most common form of esophageal atresia

A

-Imperforate anus–caused by failure of cloacal membrane to involute

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6
Q

Esophageal fistula

A
  • Connection between esophagus and trachea or a mainstem bronchus
  • Swallowed material or gastric fluids can enter respiratory tract
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7
Q

Esophageal stenosis

A
  • Incomplete form of atresia
  • lumen is reduced by a fibrous thickened wall
  • can be congenital or result from inflammatory scarring (from chronic reflux, irradiation or scleroderma)
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8
Q

Congenital duplication cysts

A
  • cystic masses with redundant smooth muscle layers

- can occur throughout the GI tract

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9
Q

Diaphragmatic hernia

A
  • occurs when incomplete formation of diaphragm allows cephalad displacement of abdominal viscera
  • when substantial, subsequent pulmonary hypoplasia is incompatible with postnatal life
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10
Q

Omphalocele

A
  • occurs when abdominal musculature is incomplete and viscera herniates into ventral membranous sac
  • 40% associated with other birth defects
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11
Q

Gastroschisis

A

-similar to omphalocele except that all layers of abdominal wall (from peritoneum to skin) fail to develop

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12
Q

Ectopia–most common site?

A
  • Ectopic tissues common in GI tract
  • most common site=PROXIMAL ESOPHAGUS leading to dysphagia and esophagitis
  • can also occur in small bowel or colon presenting with occult blood loss or peptic ulceration
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13
Q

Pancreatic heterotopia

A
  • also an ectopia
  • occurs in esophagus and stomach
  • in pylorus, it can cause inflammation, scarring and obstruction
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14
Q

true diverticulum

A

-blind pouch leading off the alimentary tract, lined by mucosa and includes all 3 layers of bowel wall–mucosa, submucosa, and muscular propria

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15
Q

Most common true diverticulum is? cause?

A
  • Meckel diverticula (2% of population)
  • results from persistence o the vitelline duct (connecting yolk sac and gut lumen) leaving a solitary out pouching within 85 cm of ileocechal valve
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16
Q

Meckel’s diverticulum–male vs. female and consequences

A
  • male to female ratio is 2:1

- Heterotopic gastric mucosa or pancreatic tissue can be present and can cause peptic ulceration

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17
Q

Congenital hypertrophic pyloric stenosis–incidence, M/F ratios and associations

A
  • 1/500 births
  • 4:1 male to female
  • complex polygenic inheritance
  • Associated with Turner syndrome and trisomy 18!!
  • Also associated with exposure to erythromycin or analogue in first 2 weeks of life
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18
Q

Congenital hypertrophic pyloric stenosis–clinical presentation

A
  • regurgitation and projectile vomiting wishing 3 weeks of brith
  • externally visible peristalsis and a palpable firm ovoid mass
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19
Q

Congenital hypertrophic pyloric stenosis–Tx

A

-Full-thickness, muscle-splitting incision (myotomy) is curative!!

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20
Q

Acquired pyloric stenosis

A

-complication of chronic antral gastritis, peptic ulcers close to pylorus and malignancy

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21
Q

Hirschsprung Disease

A
  • Aka congenital aganglionic megacolon!
  • results from arrested migration of neural crest cells into gut, yielding ganglionic segment lacking peristaltic contractions
  • Functional obstruction, proximal dilation, progressive dilation and hypertrophy of unaffected proximal colon
  • 1/5000 live births
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22
Q

Hirschsprung Disease–organ involvement

A
  • Rectum is ALWAYS affected!!

- Proximal involvement is more variable

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23
Q

Pathogenesis of Hirschsprung Disease–genetic

A
  • usually has genetic component–heterozygous LoF mutations in RET tyrosine kinase receptor in 15% of sporadic cases and majority of familial cases
  • More than 7 other genes involved in enteric neurodevelopment also associated
  • Penetrance is incomplete influenced by sex-linked factors (males 4x more commonly affected!!) and other genetic and environmental modifiers
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24
Q

Clinical features of Hirschsprung Disease

A
  • presents with neonatal failure to pass meconium or abdominal distention with severely distended megacolon (several cm in diameter!)
  • risk of perforation, sepsis, or enterocolitis with fluid derangement
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25
Q

Acquired megacolon

A
  • occur in Chagas disease, bowel obstruction, IBD, and psychosomatic disorders
  • Ganglia actually lost ONLY IN CHAGAS!!
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26
Q

Esophageal Obstruction–can cause dysphagia especially with sold foods–causes

A
  • Stenosis
  • Spasm
  • Diverticula
  • Mucosal webs
  • Esophageal rings
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27
Q

Esophageal obstruction–spasm

A

-can be short or long lived, focal or diffuse

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28
Q

Diffuse esophageal spasm

A
  • causes functional obstruction

- increased wall stress can cause diverticula to form!

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29
Q

Esophageal diverticula

A
  • can contain one or more layers

- if sufficiently large, they can accumulate enough food to present as a mass with food regurgitation

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30
Q

3 types of esophageal diverticula

A
  • Zenker (pharyggeoesophageal)
  • Traction diverticulum
  • Epiphrenic diverticulum
31
Q

Zener (pharyngeoesophageal) diverticulum occurs where?

A

-occurs immediately above upper esophageal sphincter

32
Q

Traction diverticulum occurs where?

A

-occurs at esophageal midpoint

33
Q

Epiphrenic diverticulum–occurs where?

A

-occurs immediately above the lower esophageal sphincter (LES)

34
Q

Mucosal webs

A
  • ledgelike protrusions of fibrovascular tissue and overlying epithelium
  • most common in the upper esophagus
  • women over 40!!
  • constellation of webs, iron deficiency anemia, glossitis and cheilosis
  • Plummer-Vinsion syndrome
35
Q

Plummer-Vision syndrome

A
  • aka Paterson-Brown Kelly syndrome

- constellation of webs, iron deficiency anemia, glossitis, and cheilosis

36
Q

Esophageal rings (Schatzie rings)

A
  • Similar to webs but are circumferential and thicker

- include mucosa, submucosa and occasionally hypertrophic muscular propria

37
Q

A rings vs. B rings (esophageal rings)

A
  • A rings=ABOVE the gastroesophageal (GE) junction; have SQUAMOUS EPITHELIUM
  • B rings: located at the SQUAMOCOLUMNAR junction; can have gastric-cardia type mucosa
38
Q

Achalasia

A

-triad of incomplete relaxation of LES, increased LES tone (due to cholinergic signaling) and esophageal aperistalsis

39
Q

Primary achalasia

A
  • idiopathic
  • cause: from failure of distal esophageal neurons to induce LES relaxation during swallowing (normally driven by nitric oxide and vasoactive intestinal peptide signaling
  • can also happen with degenerative changes in neural innervation
40
Q

Secondary achalasia

A
  • can occur with:
  • Chagas disease (Trypanosome cruzi)
  • Disorders of the vagal dorsal motor nuclei (polio, surgical ablation)
  • Diabetic autonomic neuropathy, in association with Down syndrome and infiltrative disorders (malignancy, amyloidosis, sarcoidosis
41
Q

Allgrove (Triple A syndrome)

A
  • Autosomal recessive disorder

- characterized by achalasia, alacrima, and adrenocorticotropic hormone-resistant adrenal insufficiency

42
Q

Treatment for achalasia

A

-myotomy, balloon dilation, and/or botulinum toxin injection to inhibit LES cholinergic neurons

43
Q

Esophagitis–causes/types

A
  • Lacerations
  • Chemical and Infectious Esophagitis
  • Reflux esophagitis
  • Eosinophilic Esophagitis
  • Esophageal Varices
44
Q

Esophageal Lacerations leading to esophagitis

A
  • Mallory Weiss Tears

- Boerhaave syndrome

45
Q

Mallory-Weiss tears

A
  • longitudinal lacerations (mm to cm in length) at GE junction associated with excessive vomiting–associated with alcohol intoxication
  • reflex relaxation of the LES precedes anti peristaltic wave associated with vomiting
  • with prolonged vomiting, relaxation fails, resulting in esophageal stretching and tearing
46
Q

Mallory-Weiss tears presentation

A
  • typically present with hematemesis
  • 10% of upper GI bleeding is associated with such tears
  • not fatal; healing is prompt without surgery
47
Q

Boerhaave syndrome

A
  • In contrast to Mallory-Weiss tears, this is much less common but more serious
  • involve transmural rupture of distal esophagus with severe mediastinhtis
  • surgical intervention required!
48
Q

Chemical and Infectious esophagitis–agents that can damage esophageal epithelium

A
  • alcohol
  • corrosive acids or alkalis
  • excessively hot fluids
  • heavy smoking
  • Pills that lodge and dissolve in esophagus
  • irradiation
  • chemotherapy
  • graft vs. host disease (GVHD)=iatrogenic etiology
49
Q

Esophagitis associated with was systemic desquamative disorders?

A
  • Pemphigoid
  • Epidermolysis bulls
  • Crohns disease (CD)
50
Q

Infections associated with esophagitis; who does this happen in?

A
  • immunocompromised
  • HSV
  • CMV
  • Fungal infections (candida most common!)
51
Q

Chief symptoms of chemical and infectious esophagitis

A
  • pain and dysphagia (pain with swallowing)

- in severe and chronic cases, hemorrhage, stricture or perforation can result

52
Q

Morphology of chemical and infectious esophagitis

A
  • depends on etiology
  • most common=dense neutrophilic infiltrates, although chemical injury may initially cause outright necrosis with inflammation
  • Any epithelial ulceration is accompanied by granulation tissue and eventually fibrosis
53
Q

Candidiasis esophagitis morphology

A

-when severe is associated with adherent gray-white PSEUDOMEMBRANES composed of densely matted fungal hyphae and inflammatory cells

54
Q

HSV esophagitis morphology vs. CMV esophagitis

A
  • HSV causes punched out ulcers

- CMV presents with shallower ulcerations with characteristic viral inclusions!

55
Q

Esophageal GVHD lesions and blistering disorders

A

-resemble their counterparts in the skin

56
Q

Reflux esophagitis

A
  • Reflux of gastric contents is the foremost cause of esophagitis!!!
  • clinical condition is called GERD (gastroesophageal reflux disease)
57
Q

Pathogenesis of reflux esophagitis

A
  • Reflux of gastric juices is the major source of mucosal injury
  • in severe cases, duodenal bile reflux exacerbates damage
58
Q

Reflux is caused by

A
  • decreased LES tone and/or increased abdominal pressure and can be caused by alcohol, tobacco use, obesity, CNS depressants, pregnancy, delayed gastric emptying or increased gastric volume
  • Hiatal hernia also causes GERD
59
Q

Hiatal hernia

A
  • can cause GERD
  • occurs when diaphragmatic crura are separated and the stomach protrudes into thorax
  • Hiatal hernias can be congenital or acquired; <10% are symptomatic!
60
Q

Morphology of reflex esophagitis

A
  • Hyperemia and edema
  • Basal zone hyperplasia (exceeding 20% of epithelium) and thinning of superficial epithelial layers
  • Neutrophil and/or eosinophil infiltration
61
Q

Clinical features of GERD

A
  • most common in adults older than 40 yrs

- S/S: dysphagia, heartburn and regurgitation of gastric contents into mouth

62
Q

Complications of long standing reflux include

A
  • ulceration
  • hematemesis
  • melena
  • stricture
  • Barrett esophagus
63
Q

Symptomatic relief of GERD

A
  • with reduced mucosal damage only

- use PPIs and or H2 histamine receptor antagonists

64
Q

Eosinophilic Esophagitis presentation

A
  • food impaction and dysphagia

- children with feeding intolerance and GERD-like symptoms

65
Q

Cardinal histologic feature of Eosinophilic Esophagitis is

A

-Large numbers of intraepithelial eosinophils!!

66
Q

Other conditions associated with Eosinophilic Esophagitis? Tx?

A
  • Many atopic disorders like atopic dermatitis, asthma

- Tx for esophageal disorder involves dietary restriction and/or stroids

67
Q

Pathogenesis of Esophageal varices

A
  • Severe portal HTN induces collateral bypass channels between prowl and naval circulations
  • lead to congested subepithelial and submucosal veins in distal esophagus (varies)
68
Q

Most common cause of esophageal varicies in Western world? second most common cause worldwide?

A
  • Alcoholic cirrhosis!!
  • 90% of cirrhotic patients develop varicose!
  • second most common cause=hepatic schistosomiasis!!
69
Q

Morphology of esophageal varicies

A
  • Tortuous, dilated veins are present in distal esophageal and proximal gastric submucosa
  • irregular luminal protrusion of overlying mucosa with superficial ulceration, inflammation or adherent blood clots
70
Q

Clinical features of esophageal varicies

A
  • Varices present in almost half of patients with cirrhosis

- clinically silent until they rupture with hematemesis (50-80% of patients with varies)

71
Q

Causes of rupture of esophageal varicies

A
  • inflammatory erosion
  • increased venous pressure
  • increased hydrostatic pressure associated with vomiting
72
Q

Bleeding from esophageal varies can be treated with?

A
  • sclerotherapy
  • balloon tamponade or
  • band ligation
73
Q

Esophageal varicies prognosis

A
  • Up to half die with their first bleed either due to exsanguination or following hepatic coma
  • in survivors there is a 50% change of recurrence within a year with the same mortality rate

GI 2 Pathology--Chapt 16 and 17 (14 decks)

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