DSA Ch. 17 1-3 (Dobson) Flashcards
(124 cards)
1
Q
Importantly, because many organs develop simultaneously during embryogenesis, the presence of congenital GI disorders should prompt what?
A
Evaluation of other organs!
2
Q
This is a common congenital anomaly that occurs in one out of every 3,500 live births. It is commonly associated with other congenital malformations, particularly cardiac defects.
A
Tracheoesophageal (TE) Fistula
3
Q
________ _______ is a related congenital malformation with a presentation similar to that of a TE fistula and can occur with or without the presence of a fistula.
A
Esophageal Atresia
4
Q
Esophageal Atresia consists of a thin, noncanalized cord that replaces a segment of the esophagus, most commonly at the tracheal bifurcation. It is usually associated with a _________ connecting the upper or lower esophageal pouches to a bronchus or trachea.
A
Fistula
***Can also be fistula without atresia (TE fistula)!
5
Q
TE fistulas occur due to abnormal septation of the caudal foregut during which weeks of embryonic development?
A
4th and 5th weeks
6
Q
In embryonic development, the trachea normally forms as a _________ of the foregut and develops a complete septum that separates the esophagus from the trachea.
A
Diverticulum
7
Q
This occurs in the Ileum, and is due to the failed involution of the Vitelline Duct (which connects the lumen of the developing gut to the yolk sac).
A
Meckel Diverticulum
8
Q
The mucosal lining of Meckel diverticula may resemble that of normal small intestine, but ectopic ________ or ________ tissue may also be present. The latter may secrete acid, cause peptic ulceration of adjacent small intestinal mucosa, and present with occult bleeding or abdominal pain resembling acute appendicitis or obstruction.
A
Pancreatic
Gastric
9
Q
What are the rule of 2’s for Meckel Diverticulum?
A
- 2% of population
- Within 2 ft. of Ileocecal valve
- Approx. 2 inches long
- 2x more common in males
- Symptoms by age 2 (usually)
***Although, only 4% of cases every show symptoms at all!
10
Q
This occurs when there is thickened muscle around the pylorus, preventing food from leaving the stomach.
A
Pyloric Stenosis
11
Q
Pyloric Stenosis occur 3-5x more often in males, and also has a much higher rate of concordance in (DIZYGOTIC/MONOZYGOTIC) twins, with a 200-fold increase if one of the twins is affected. In (DIZYGOTIC/MONOZYGOTIC) twins, there is just a 20-fold increase in risk for siblings of affected individuals.
A
Monozygotic
Dizygotic
12
Q
What chromosomal syndromes give an increased chance of Pyloric Stenosis?
A
Turner Syndrome
Edward Syndrome
13
Q
What antibiotics can give an infant increased risk of developing Pyloric Stenosis? Consumed either orally or via mother’s milk in their 1st 2 weeks of life.
A
Erythromycin
Azithromycin
14
Q
This disease occurs in approximately 1 of 5000 live births, and occurs when normal migration of NCCs from cecum to rectum is arrested prematurely or when enteric ganglion cells undergo premature death.
A
Hirschsprung Disease (Aganglionic Megacolon)
15
Q
Hirschsprung Disease may be isolated or occur in combination with other developmental abnormalities. 10% of all cases occur in children with _______ _______ and serious neurologic abnormalities are present in another 5%.
A
Down Syndrome
16
Q
Hirschsprung Disease produces a distal intestinal segment that lacks which plexuses?
A
Meissner Submucosal Plexus
Auerbach Myenteric Plexus
17
Q
In Hirschsprung Disease, coordinated peristaltic contractions are absent and functional occurs, resulting in _________ proximal to the affected segment.
A
Dilation
***This is what causes megacolon!
18
Q
In Hirschsprung Disease, the ________ is always involved.
A
Rectum
19
Q
Hirschsprung Disease is more common in males, but when it occurs in females what happens?
A
Females have longer length of colonic involvement
20
Q
After birth, what occurs that makes us suspicious of Hirschsprung Disease?
A
Failure to pass meconium
21
Q
Intrinsic innervation of much of the alimentary canal is provided by the _______ _______ _______, which runs from the esophagus to the anus, and contains approximately 100 million motor, sensory, and interneurons (unique to this system compared to all other parts of the PNS). These neurons are grouped into two plexuses.
A
Enteric Nervous System
22
Q
This plexus lies in the muscularis layer of the alimentary canal and is responsible for motility, especially the rhythm and force of the contractions of the muscularis.
A
Auerbach Myenteric Plexus
23
Q
This plexus lies in the submucosal layer and is responsible for regulating digestive secretions and reacting to the presence of food.
A
Meissner Submucosal Plexus
24
Q
The ________ develops from the cranial portion of the foregut and is recognizable by the 3rd week of gestation. Development of the GI system results from a series of highly regulated biochemical processes and folding patterns.
A
Esophagus
25
The GI tract is subdivided functionally into what?
Foregut
Midgut
Hindgut
26
The esophagus is a muscular tube that is how long (in adults)?
18-22 cm
27
What provides the blood supply to the esophagus?
Upper 1/3 = Inferior Thyroid A.
Middle 1/3 = Branches of Thoracic Aorta
Lower 1/3 = Left Gastric A.
28
What provides nerve supply to the esophagus?
Sympathetic Trunks
| Parasympathetic Nerve - Vagus
29
What is the function of the esophagus?
Move bolus of food from mouth to stomach
***Motility!
30
If a patient presents with difficulty and/or pain with swallowing stating that "food or liquid gets stuck" then what issue should we think of?
Upper GI obstruction or inflammation
31
If a patient presents with chest pain or says it feels like "heart burn", or they are coughing, choking, and have a sour taste in their mouth, what issue should we think of?
If they have chest pain, HAVE to rule out MI first!
Otherwise, think incompetence of LES or reflux of gastric acid.
32
If a patient presents with "fatigue" and are lightheaded/fainting with pallor, what should we think of?
Blood loss/anemia
33
If a patient presents with weight loss, what should we think of?
Inadequate nutrition or cancer
34
What are the types of esophageal obstruction?
Functional
Structural/Mechanical
Achalasia
35
_________ causes of esophageal obstruction create disruptions in coordinated peristalsis.
Functional
36
This is a type of functional esophageal obstruction where patients have high-amplitude contractions of the distal esophagus that are, in part, due to loss of the normal coordination of inner circular layer and outer longitudinal layer smooth muscle contractions.
Nutcracker Esophagus (Jackhammer Esophagus)
37
This is a type of functional esophageal obstruction that presents as repetitive, simultaneous contraction of the distal esophageal smooth muscle.
Diffuse Esophageal Spasm
38
LES dysfunction consists of high resting pressure or incomplete relaxation, and is often present in many patients with Nutcracker Esophagus or Diffuse Esophageal Spasm. How does LES dysfunction differ from Achalasia?
Achalasia does this, but also includes reduced esophageal peristaltic contractions.
39
This is a disease that can also cause functional esophageal obstruction due to CREST syndrome.
Systemic Sclerosis
40
Another functional esophageal obstruction can be from complications due to increased intraesophageal pressure. An example of this is ________ ________.
Zenker Diverticulum
***Like a balloon animal, squeeze on one end then it balloons on the other end!
41
Zenker Diverticulum is located right above the UES. What are the symptomatic differences from small or large Zenker Diverticula?
Small -- Usually asymptomatic
Large -- Can trap food and cause regurgitation and halitosis
42
Structure/Mechanical esophageal obstruction is due to stricture or stenosis that is either ________ or not ________ related.
Cancer
| Cancer
43
What are the "not cancer" causes of structure/mechanical esophageal obstruction?
- - Esophageal Webs
- - Esophageal Rings
- - Achalasia
- - Inflammation and scarring (esophagitis)
44
This type of structural esophageal obstruction is an idiopathic ledge-like protrusion of mucosa that may cause obstruction. They are uncommon, and occur most often in females over 40 yo.
Esophageal Webs
45
The main symptom of esophageal webs is non progressive ________ associated with incompletely chewed food.
Dysphagia
46
In the upper esophagus, webs may be accompanied by IDA, glossitis, and cheilosis as part of the _______ or _______. With these, there is also a risk of esophageal cancer!
P-B-K (Paterson-Brown-Kelly)
| P-V-S (Plummer-Vinson Syndrome)
47
What are other disease associations with Esophageal Webs?
- - GERD
- - C-GVHD
- - Celiac Disease
48
Esophageal Rings are called _______ ______, and are categorized as type A or type B. These rings are circumferential and thicker, and include the mucosa, submucosa, and occasionally hypertrophic muscularis propria.
Schatzki Ring
49
This type of Schatzki Ring (Esophageal Ring) is present in the distal esophagus above the gastroesophageal junction and covered in squamous mucosa.
Type A rings
50
This type of Schatzki Ring (Esophageal Ring) is present at the squamocolumnar junction of the lower esophagus and may have gastric cardia-type mucosa on their undersurface.
Type B rings
51
This is a type of esophageal obstruction where patients present with dysphagia for solids and liquids, difficulty belching, and chest pain.
Achalasia
52
Achalasia is characterized by what triad?
1) Incomplete LES relaxation
2) Increased LES tone
3) Aperistalsis of the esophagus
53
(PRIMARY/SECONDARY) Achalasia is the result of distal esophageal inhibitory neuronal (ganglion cell) degeneration.
Primary
54
(PRIMARY/SECONDARY) Achalasia may arise in Chagas disease, in which Trypanosoma cruzii infection causes destruction of the myenteric plexus, failure of peristalsis, and esophageal dilatation.
Secondary
55
Secondary Achalasia can occur due to Chagas, achalasia-like disease caused by _______ _______ _______, infiltrative disorders, lesions of DMN, syndromes, HSV1, and autoimmune disease.
Diabetic Autonomic Neuropathy
56
What are treatments for Achalasia?
- - Laparoscopic myotomy
- - Pneumatic balloon dilatation
- - Botox injections to inhibit LES cholinergic neurons
57
This type of esophageal laceration occurs due to longitudinal mucosal tears near the gastroesophageal junction. It is most often associated with severe retching or vomiting secondary to acute alcohol intoxication. It is an incomplete tear.
Mallory-Weiss Syndrome
58
T/F. Mallory-Weiss Syndrome is a catastrophic event and requires immediate surgery.
False. Mallory-Weiss Syndrome does not usually require surgery, and healing tends to be rapid and complete.
***Boerhaave Syndrome is a catastrophic event!
59
This type of esophageal laceration is much less common but more severe. It is characterized by transmural tearing and rupture of the distal esophagus (complete tear). It is a catastrophic event that produces mediastinitis and generally requires surgical intervention.
Boerhaave Syndrome
60
With Boerhaave Syndrome, because patients can present with severe chest pain, tachypnea, and shock, the initial ddx can include...
Myocardial Infarction
61
What were some of the main esophageal causes of hematemesis that we discussed?
- - Lacerations (ie, Mallory-Weiss)
- - Esophageal perforation (ie, cancer or Boerhaave)
- - Varices (cirrhosis)
- - Infectious esophagitis (Candida, HSV)
- - Barrett Esophagus
- - Adenocarcinoma
- - SCC
***There are more, but we talked about these the most! See Table 17-1 for others!
62
Esophageal varices are present in up to 50% of _________ and 24-40% of these develop _________.
Cirrhotics (due to Alcoholic Liver Disease)
| Bleeding
63
Variceal hemorrhage is a medical emergency that can be treated medically by...
-- Inducing splanchnic vasoconstriction
-- Endoscopically by sclerotherapy (injection of thrombotic agents)
-- Balloon tamponade
-- Variceal ligation
64
Venous blood from the GI tract passes through the liver, via the portal vein, before returning to the heart. This circulatory pattern is responsible for the first-pass effect in which drugs and other materials absorbed in the intestines are processed by the liver before entering the systemic circulation. Diseases that impede this flow cause _______ _______ and can lead to the develop of esophageal varies, an important cause of esophageal bleeding.
Portal HTN
65
Portal HTN results in the development of collateral channels at sites where the portal and canal systems communicate. Varices develop in the vast majority of which patients? Second most common cause?
Majority is cirrhotic patients, most commonly in association with alcoholic liver disease.
Second most common cause is hepatic schistosomiasis.
66
When there is blood loss from the GI tract, is acute or chronic blood loss a medical emergency?
Acute blood loss
67
Is the upper or lower GI tract more likely to have blood loss?
UGIB is 4x more common than LGIB
***UGIP is proximal to ligament of Treitz!
68
When there is an UGIB, the stool can appear black and tarry due to blood. This type of stool is called...
Melena
69
Where there is a LGIB, there can be fresh blood in the anus or stool. This is called...
Hematochezia
70
_________ is a cause of structural/mechanical obstruction. Symptoms range from self-limited pain, particularly on swallowing (odynophagia), to hemorrhage, stricture, or perforation in severe cases.
Esophagitis
71
What are the main types of esophagitis discussed?
1) Radiation
2) Chemical
3) Infectious
4) Eosinophilic
5) Reflux esophagitis
72
Chemical esophagitis can occur due to caustic substances that were recently ingested. The main example is...
When kids consume household cleaning products
73
What occurs in pill-induced esophagitis?
Pills will lodge in the esophagus and dissolve there rather than in the stomach (where they're supposed to).
74
What are the most common causes of infectious esophagitis?
HSV
CMV
Fungus
75
Esophageal infections in otherwise healthy individuals are uncommon and most often due to...
HSV
76
Infections in patients that are debilitated or immunosuppressed is more common and caused by HSV, CMV, or fungi. What are the most common fungi?
- - Candidiasis
- - Mucormycosis
- - Aspergillosis
77
This fungi is characterized by adherent, gray-white pseudomembranes composed of densely matted fungal hyphae and inflammatory cells covering the esophageal mucosa.
Candidiasis
78
This fungi typically causes punched-out ulcers. Biopsy shows nuclear viral inclusions within a rim of degenerating epithelial cells at margin of ulcer.
HSV
79
This fungi causes shallower ulcerations and characteristic nuclear and cytoplasmic inclusions within capillary endothelium and stromal cells.
CMV
80
This type of esophagitis has been increasing in incidence since 1978. Symptoms include food impaction, dysphagia, or feeding intolerance in infants and children. Most patients have food or seasonal allergies (allergies, allergic rhinitis, atopic dermatitis).
Eosinophilic esophagitis
***Cardinal histologic feature is large numbers of intraepithelial eosinophils!
81
Atopic dermatitis is very common with eosinophilic esophagitis. What are the common locations of atopic dermatitis for infants, children, and adults?
Infantile type = Face, scalp, trunk, extensor surfaces of extremities
Childhood type = Flexural folds of extremities (antecubital, popliteal fossa), neck, ankles
Adult type = Upper arms, back, wrists, hands, fingers, feet, toes
82
Eosinophilic Esophagitis has a very characteristic appearance via endoscopy. It has the appearance of ridges and looks very similar to the ________.
Trachea
***Reminds me of a spider-web!
83
________ refers to the genetic tendency to develop allergic diseases such as allergic rhinitis, asthma, and atopic dermatitis (eczema).
Atopy
84
T/F. Atopy is typically associated with heightened immune responses to common allergens, especially inhaled allergens and food allergens.
True
85
Reflux of gastric contents into the lower esophagus is the most frequent cause of ________ and the most common outpatient GI diagnosis in the US.
Esophagitis
86
Reflux esophagitis, aka _______, is due to transient LES relaxation.
GERD
87
Normally, release of _______ _______ and _______ _______ _______ from inhibitory neurons, along with interruption of normal cholinergic signaling, allows the LES to relax during swallowing. This process is disrupted in esophagitis, because the LES will be pathologically relaxed.
Nitric Oxide
| Vasoactive Intestinal Polypeptide
88
What are things that can cause the pathogenesis of LES relaxation?
- - Vagal mediated pathways
- - Increased intra-abdominal pressure
- - Alcohol and tobacco
- - Obesity
- - Hiatal hernia
- - Delayed gastric emptying (gastroparesis)
- - Idiopathic
89
This is the term for the protrusion of the stomach through the diaphragm into the chest.
Hiatal hernia
90
_______ is most common in individuals older than age 40 but also occurs in infants and children. The most frequent clinical symptoms are "heartburn", dysphagia, and regurgitation of sour-tasting gastric contents (______ _______).
GERD
| Water brash
91
Rarely, chronic GERD is punctuated by attacks of severe chest pain that may be mistaken for...
Heart disease
92
Treatment with ______, which have replaced H2 histamine receptor antagonists, to reduce gastric acidity typically provides symptomatic relief.
PPIs (Proton Pump Inhibitors)
93
Complication of reflux include ulceration, hematemesis, Selena, stricture development, and _________.
Metaplasia
94
This is the term for a change in phenotype of differentiated cells, often in response to chronic irritation, that makes cells better able to withstand the stress, usually induced by altered differentiation pathway of tissue stem cells. May result in reduced functions or increased propensity for malignant transformation.
Metaplasia
95
This is a complication of chronic GERD that is characterized by intestinal metaphases within the esophageal squamous mucosa. Squamous cells are changed to columnar epithelial cells, to better handle the acidity.
Barrett Esophagus
96
What is the greatest concern associated with Barrett Esophagus?
Esophageal Adenocarcinoma
97
Is Barrett Esophagus diagnosed by symptoms?
No -- have to do endoscopy
98
T/F. Biopsy by endoscopy is used for Barrett Esophagus to obtain tissue for histology. This is the only way Barrett Esophagus can be identified. GERD symptoms are usually what prompt the endoscopy and biopsy.
True
99
The metaplastic mucosa of Barrett Esophagus alternates with residual, smooth, pale squamous (________) mucosa and interfaces with light-brown columnar (_______) mucosa distally.
Esophageal
| Gastric
100
The issue with Barrett Esophagus is the development of ________, which can initially be low-grade, then high-grade, and eventually adenocarcinoma. The influences that predispose to metaplasia, if persistent, can initiate malignant transformation in metaplastic epithelium.
Dysplasia
***Dysplastic tissue doesn't ALWAYS progress to cancer, but it has much higher chance!
101
Esophageal tumors that are benign neoplasms are causes of ________. Most are "submucosal".
Obstruction
102
Most benign esophageal tumors are mesenchymal, with smooth muscle tumors being the most common. These are called...
Leiomyomas
103
What are the malignant forms of esophageal cancers we need to watch out the most for?
Adenocarcinoma
| SCC
104
What is the geographic distribution at the highest risk for adenocarcinoma?
US
UK
Canada
Australia
105
What is the highest risk group for adenocarcinoma?
Caucasians -- 7x more common in men
106
What are risk factors for adenocarcinoma?
Barrett Esophagus
Tobacco
Radiation
H. pylori
107
For unknown reasons, the increases in esophageal adenocarcinoma have been restricted to white and Hispanic men and white women in the US. As a result, esophageal adenocarcinoma, which represented less than _____ of esophageal cancers before 1970, now accounts for more than _______ of all esophageal cancers in the US.
5%
| 50%
108
This type of esophageal cancer will initially appear as flat or raised patches in otherwise intact mucosa, large masses of 5 cm or more in diameter may develop. Alternatively, tumors may infiltrate diffusely or ulcerate and invade deeply. Usually located in distal third of esophagus, because that's where highest portion of acid comes out.
Adenocarcinoma (mostly mucinous adenocarcinoma)
109
Molecular studies suggest that the progression of Barrett Esophagus to adenocarcinoma occurs over an extended period through the stepwise acquisition of _______ and ________ changes.
Genetic
| Epigenetic
110
Although esophageal adenocarcinomas are occasionally discovered in evaluation of GERD or surveillance of Barrett Esophagus, they more commonly present with pain or difficulty in swallowing, progressive weight loss, hematemesis, chest pain, or vomiting. By the time symptoms appear, the tumor has usually spread to the...
Submucosal lymphatic vessels
111
For esophageal adenocarcinoma, overall 5-year survival is less than _______ in high stage disease. In contrast, 5-year survival approximates ______ in the few patients with adenocarcinoma limited to the mucosa or submucosa.
25%
| 80%
112
What is the geographic distribution at highest risk for esophageal SCC?
Iran
Central China
Hong Kong
113
What group is the highest risk for esophageal SCC?
Males (4:1) >45 yo
| 8x more common in African Americans
114
What are the risk factors for esophageal SCC?
- - Alcohol and tobacco
- - Poverty
- - Caustic esophageal injury
- - Achalasia
- - Tylosis
- - Radiation
- - Plummer-Vinson Syndrome
- - Diets deficient in fruits or veggies
- - Frequent consumption of very hot beverages
- - Mursik (fermented milk type drink in Kenya)
115
What are the major causes of esophageal SCC in Europe/US vs. other countries?
Europe/US = Alcohol and tobacco
Other countries = Nutritional deficiencies and mutagenic carcinogens
116
In this type of esophageal cancer, early lesions appear as small, gray-white, plaque-like thickenings. Over months to years they grow into tumor masses that may be polypoid, or exophytic, and protrude into and obstruct the lumen. Other tumors are either ulcerated or diffusely infiltrative lesions that spread within the esophageal wall and cause thickening, rigidly, and luminal narrowing.
Esophageal SCC
117
Where does 50% of esophageal SCC occur?
Middle third of esophagus
118
This risk factor for esophageal SCC is due to a RHBDF2 mutation, that can cause Howel-Evans Syndrome (marked squamous hyperplasia, looking like thick, crusty skin).
Tylosis
119
This is a risk factor for esophageal SCC in high-risk areas, but not in low-risk areas.
HPV
120
This autoimmune disease causes an increased risk of stomach and esophageal malignancies (ie, esophageal SCC).
HIV
121
The onset of esophageal SCC is insidious and it most commonly presents with dysphagia, odynophagia, or obstruction. Patients subconsciously adjust to the progressively increasing obstruction by altering their diet from solid to liquid foods. Prominent _______ ______ and debilitation result from both impaired nutrition and effects of the tumor itself.
Weight Loss
122
In esophageal SCC, hemorrhage and sepsis may accompany tumor ulceration, and symptoms of iron deficiency are often present. Occasionally, the first symptoms are caused by aspiration of food via a...
TE Fistula
***This is how TE fistula could be acquired later in life!
123
Increased prevalence of endoscopic screening has led to earlier detection of esophageal SCC. 5-year survival rates are ______ in individuals with superficial esophageal SCC but much lower in patients with more advanced tumors. ______ _____ metastases, which are common, are associated with poor prognosis.
75%
| Lymph Node
124
The overall 5-year survival rate of esophageal SCC in the US remains less than ______, and varies by tumor stage and patient age, race, and gender.
20%
