Esophagus

Question 1

Atresia and fistulae

Answer 1

structral developmental anomalies that disrupt normal gastrointestinal transit and typically present early in life

Question 2

imperforate anus

Answer 2

the most common form of congenital intestinal atresia, while the esophagus is the most common site of fistulization

Question 3

Stenosis

Answer 3

may be developmental or acquired. Both forms are characterized by a thickened wall and partial or complete luminal obstruction. Acquired forms are often due to inflammatory scarring

Question 4

Diaphragmatic hernia

Answer 4

is characterized by incomplete diaphragm development and herniation of abdominal organs into the thorax. This often results in pulmonary hypoplasia.

Question 5

omphalocele and gastroschisis

Answer 5

refer to ventral herniation of abdominal organs

Question 6

ectopia

Answer 6

refers to the presence of normally formed tissues in an abnormal site.

Question 7

ectopic gastic mucosa

Answer 7

common in the upper third of the esophagus

Question 8

meckel diverticulum

Answer 8

presence of all three layers of the bowel wall reflecting failed involution of the vitelline duct. It is common and is a frequent site of gastric ectopia, which may result in occult bleeding

Question 9

congenital hypertrophic pyloric stenosis

Answer 9

a form of obstruction that presents between the third and sixth weeks of life. There is an ill-defined genetic component to this disease, which is most common in males.

Question 10

Hirschsprung disease

Answer 10

caused by the absence of neural crest derived ganglion cells within the colon. It causes functional obstruction of the affected bowel and proximal dilation. Theh defect always begins at the rectum, but extends proximally for variable lengths.

Question 11

Most common type of tracheoesophageal fistula

Answer 11

blind upper segment with fistula between the lower segment and trachea

Question 12

Nutcracker esophagus

Answer 12

describes patients with 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.

Question 13

Diffuse esophageal spasm

Answer 13

is characterized by repetitive, simultaneous contractions of the distal esophageal smooth muscle.

Question 14

hypertensive lower esophageal sphincter.

Answer 14

Lower esophageal sphincter dysfunction, such as high resting pressure or incomplete relaxation, are present in many patients with nutcracker esophagus or diffuse esophageal spasm. In the absence altered patterns of esophageal contraction, these sphincter abnormalities are termed * hypertensive LES

Question 15

Esophageal Obstruction

Answer 15

Because wall stress is increased, esophageal dysmotility may result in development of small diverticulae, primarily the epiphrenic diverticulum located immediately above the lower esophageal sphincter.

Question 16

Zenker diverticulum

Answer 16

impaired relaxation and spasm of the cricopharyngeus muscle after swallowing can result in increased pressure within the distal pharynx and development of a Zenker diverticulum (pharyngoesophageal diverticulum), which is located immediately above the upper esophageal sphincter.

Zenker diverticulae are uncommon, but typically develop after age 50 and may reach several centimeters in size. When small they may be asymptomatic, but larger Zenker diverticulae may accumulate significant amounts of food, producing a mass and symptoms that include regurgitation and halitosis

Question 17

Esophageal mucosal webs

Answer 17

Paterson-Brown-Kelly or Plummer-Vinson syndrome

Schatzki rings: A rings and are covered by squamous mucosa; in contrast, those located at the squamocolumnar junction of the lower esophagus are designated B rings and may have gastric cardia-type mucosa on their undersurface

Question 18

Achalasia

Answer 18

Achalasia is characterized by the triad of incomplete LES relaxation, increased LES tone, and aperistalsis of the esophagus

Symptoms include dysphagia for solids and liquids, difficulty in belching, and chest pain

Question 19

Primary achalasia

Answer 19

is the result of distal esophageal inhibitory neuronal, that is, ganglion cell, degeneration

Question 20

Secondary achalasia

Answer 20

may arise in Chagas disease, in which Trypanosoma cruzi infection causes destruction of the myenteric plexus, failure of peristalsis, and esophageal dilatation

Question 21

Mallory-Weiss tears

Answer 21

Longitudinal mucosal tears near the gastroesophageal junction are termed Mallory-Weiss tears, and are most often associated with severe retching or vomiting secondary to acute alcohol intoxication

Question 22

Boerhaave syndrome

Answer 22

esophageal rupture

can come from trauma or cancer, e.g.

Question 23

Chemical and Infectious Esophagitis

Answer 23

The stratified squamous mucosa of the esophagus may be damaged by a variety of irritants including alcohol, corrosive acids or alkalis, excessively hot fluids, and heavy smoking

Question 24

GERD

Answer 24

LES is not working properly
inflammation from the reflux

GERD 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. Rarely, chronic GERD is punctuated by attacks of severe chest pain that may be mistaken for heart disease.

Treatment with proton pump inhibitors, which have replaced H2 histamine receptor antagonists, to reduce gastric acidity typically provides symptomatic relief

Question 25

Hiatal hernia

Answer 25

can give rise to symptoms, such as heartburn and regurgitation of gastric juices, that are similar to those of GERD. It is characterized by separation of the diaphragmatic crura and protrusion of the stomach into the thorax through the resulting gap

Question 26

Eosinophilic Esophagitis

Answer 26

The majority of individuals with eosinophilic esophagitis are atopic and many have atopic dermatitis, allergic rhinitis, asthma, or modest peripheral eosinophilia.

Treatments include dietary restrictions to prevent exposure to food allergens, such as cow’s milk and soy products, and topical or systemic corticosteroids

Question 27

Esophageal Varices

Answer 27

Portal hypertension results in the development of collateral channels at sites where the portal and caval systems communicate

–> esophageal varices
and huge hemorrhoids
and caput medusa

Question 28

clinical features of gastroesophageal varices

Answer 28

are present in nearly half of the patients with cirrhosis, and 25-40% of patients with cirrhosis develop variceal bleeding.

Approximately 12% of previously asymptomatic varices bleed each year. Variceal hemorrhage is an emergency that can be treated medically by inducing splanchnic vaso­constriction or endoscopically by sclerotherapy (injection of thrombotic agents), balloon tamponade, or variceal ligation.

Despite these interventions, 30% or more of patients with variceal hemorrhage die as a direct consequence of hemorrhage such as hypovolemic shock, hepatic coma, or other complications. Furthermore, more than 50% of patients who survive a first variceal bleed have recurrent hemorrhage within 1 year, and this carries a mortality rate similar to that of the first episode

Question 29

Barrett Esophagus

Answer 29

Barrett esophagus is a complication of chronic GERD that is characterized by intestinal metaplasia within the esophageal squamous mucosa

The greatest concern in Barrett esophagus is that it confers an increased risk of esoph­ageal adenocarcinoma
**

Question 30

clinical features of barrett esophagus

Answer 30

can only be identified thorough endoscopy and biopsy, which are usually prompted by GERD symptoms

Intramucosal or invasive carcinoma requires thera­peutic intervention. Treatment options include surgical resection, or esophagectomy, as well as newer modalities such as photodynamic therapy, laser ablation, and endoscopic mucosectomy

Multifocal high-grade dysplasia, which carries a significant risk of progression to intra­mucosal or invasive carcinoma, is treated as intramucosal carcinoma. Many physicians follow low-grade dysplasia or a single focus of high-grade dysplasia with endoscopy and biopsy at frequent intervals.

Question 31

Esophageal Tumors

Answer 31

The vast majority of esophageal cancers fall into one of two types, adenocarcinoma and squamous cell carcinoma

Question 32

Most esophageal adenocarcinomas arise

Answer 32

from Barrett esophagus

Question 33

Adenocarcinoma

Answer 33

Molecular studies suggest that the progression of Barrett esophagus to adenocarcinoma occurs over an extended period through the stepwise acquisition of genetic and epigenetic changes

Chromosomal abnormalities, mutation of *TP53, and downregulation of the cyclin-dependent kinase inhibitor *CDKN2A, also known as * p16/INK4a, are detected at early stages.

In the case of CDKN2A, both allelic loss and hypermethylation-induced epigenetic silencing have been described. Later during progression there is amplification of *EGFR, ERBB2, MET, cyclin D1, and cyclin E genes.**

Question 34

lower esophageal tumor more likely to be an

Answer 34

adenocarcinoma

Question 35

a mid-to upper- 1/3 lesion on the esophagus is more likely to be a

Answer 35

squamous cell

Question 36

clincial features of adenocarcinomas

Answer 36

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 submucosal lymphatic vessels. As a result of the advanced stage at diagnosis, overall 5-year survival is less than 25%.

In contrast, 5-year survival approximates 80% in the few patients with adenocarcinoma limited to the mucosa or submucosa

Question 37

Squamous Cell Carcinoma

Answer 37

Risk factors include alcohol and tobacco use, poverty, caustic esophageal injury, achalasia, tylosis, Plummer-Vinson syndrome, diets that are deficient in fruits or vegetables, and frequent consumption of very hot beverages

Question 38

Clinical Features

of squamous cell carcinomas

Answer 38

The onset of esophageal squamous cell carcinoma is insidious and it most commonly presents with dysphagia, odynophagia (pain on swallowing), or obstruction. Patients subconsciously adjust to the progressively increasing obstruction by altering their diet from solid to liquid foods. Prominent weight loss and debilitation result from both impaired nutrition and effects of the tumor itself

Increased prevalence of endoscopic screening has led to earlier detection of esophageal squamous cell carcinoma. This is critical, because 5-year survival rates are 75% in individuals with superficial esophageal squamous cell carcinoma but much lower in patients with more advanced tumors.

Lymph node metastases, which are common, are associated with poor prognosis. The overall 5-year survival rate in the United States remains less than 20%