the 2 major functions of the esophagus: transport of the food bolus from the mouth to the stomach the prevention of retrograde flow of GI contents

the UES remains closed due to the elastic properties of its wall tonic contraction of the cricopharyngeus and inferior

pharingeal constrictor muscles ( produced by continuous neural excitation of the lower motor neurons which innervate these muscles via end plates)

in contrast, the LES remains closed because of its intrinsec myogenic tone and a neural pathway, consisting of preganglionic parasympathetic fibers in the vagus nerve and postganglionic myenteric inhibitory neurons, causes its relaxation.

defective inervation of the smooth muscle portion of the esophageal body and LES

vigorous achalasia less severe neural damage than classic one ( shows a marked reduction in myenteric neurons)

the esophageal body shows elevated resting pressure

in response to swallows, primary peristaltic waves are replaced by simultaneous onset contractions.

these contractions may be of: large amplitude and long duration (vigorous A) poor amplitude (classic A)

administration of the cholinergic muscarinic agonist mecholyl causes a marked increase in baseline esophageal pressure and CCK paradoxically causes contraction of LES ( normally causes a fall in the sphincter pressure )

Measurements are taken from multiple recording levels in the esophagus.

In this example of achalasia, peristalsis and lower esophageal sphincter (LES) relaxation are absent and LES pressure is elevated.

AA=aortic arch; S= swallow.

Manometric representation of normal peristalsis and achalasia.

MEDICAL TREATEMENT: soft foods sedatives nitrates anticholinergic drugs

usually unsatisfactory

Calcium channel antagonists (NIFEDIPINE) BALLON DILATATION –the best available

therapy, effective in 85% of patients; complications: perforation, bleeding

HELLER’S EXTRAMUCOSAL MYOTOMY OF LES

1. On the far left an intramural extravasation (arrow) after distal dilation for achalasia.

2. In the middle an intramural extravasation (arrow) after complicated endoscopy.

3. On the right a perforation after biopsy with extravasation of contrast material (arrow).

COMPLICATIONS

Variants of DESDES ( NUTCRACKER NUTCRACKER ESOPHAGUSESOPHAGUS) occur as a primary disease or in association with a variety of diseases, as well as emotional stress and aging:

Collagen vascular disease Diabetic neuropathy Reflux esophagitis Irradiation esophagitis Esophageal obstruction Drugs ( cholinergic , anticholinergic)

1. Chest pain ± retrosternal , at rest, emotional stress

2. Dysphagia for solids and liquids3. Radiation –to the back

- sides of the chest - both arms - sides of the jaw

may last for a few seconds to several minutes acute and severe ≠ myocardial ischemia

(angina)

BARIUM SWALLOW

74-year-old man with diffuse esophageal spasm who presented with dysphagia. Prone right anterior oblique view from single-contrast esophagram shows multiple nonperistaltic contractions (arrows) of moderate severity without classic corkscrew appearance. Lower

esophageal sphincter opened normally.

uncoordinated simultaneous contractions that produce curling or multiple ripples in the wall, sacculation and pseudodiveticula – “the CORK SCREW “E.

LES opens normally

MANOMETRY

Manometric representation of normal peristalsis and diffuse esophageal spasm. Measurements are taken from multiple recording levels in esophagus. Normal peristalsis is present in upper esophagus, but it is replaced by simultaneous, repetitive contractions below aortic arch (AA). Normal lower esophageal sphincter (LES) relaxation is seen. S, swallow.

reveals prolongued large amplitude and repetitive contractions of simultaneous onset

This is normal esophageal manometry tracing with normal amplitude of the contractions. The contractions are coordinated because the contractions in the proximal esophagus (top of image) occur before the contractions further distal in the esophagus.

Esophageal manometry tracing

demonstrates diffuse esophageal spasm. Note the multiple uncoordinated contractions in the third tracing from the distal esophagus

Esophageal manometry tracing demonstrates

nutcracker esophagus. Note the excessive amplitude of the contractions.

Cold swallows and solid boluses induce chest pain and motor abnormalities

Provocative pharmacologic tests LIMITED

For chest pain and motor disorders

1. MEDICAL TREATMENT Anticholinergies - ↓ value Relaxing smooth muscle agents

nitroglycerin s.l. 0,3-0,6 mg isosorbide dinitrate nifedipine 10-20 before meals

2. ESOPHAGEAL DILATATION with mercury filled rubber dilators – relief as a result of distension of the lower esophagus (largely a placebo effect)

3. REASSURANCE and TRANQUILIZERS are helpful4. BALLON DILATATION5. LONGITUDINAL MYOTOMY of esophageal

circular muscle relieves pain in up to 2/3 of patients

CLINICAL FEATURES:◦Disphagia to solids and liquids in recumbent position

◦heartburn ◦regurgitation

GER stricture

BARIUM SWALLOW

Barium swallow examination in patient with scleroderma, showing long distal oesophageal peptic stricture (large arrow) and mucosal ulceration (small arrow). (Image kindly provided by Dr Hany El-Madbouh).

dilatation and loss of contractions midde and distal esophagus

MOTILITY STUDIES

↓ amplitude smooth muscle contractions

↓ pressure LES

Esophageal mucosal damage resulting from reflux of gastric or intestinal contents into the esophagus

Causative agent:pepticbile (alkaline esophagitis)

Secondary causes: pregnancy, female sex hormones smoking smooth muscle relaxants : β adrenergics ,

aminophyline , nitrates , calcium channel blockers surgical resection myotomy ballon dilatation

The cumulative esophageal reflux, the amount and duration of refluxed material remaining in the esophagus is dependent on the :

amount of refluxed material per episode and frequency of episodes

the clearing of the esophagus by gravity and peristaltic contraction

neutralisation by salivary secretion

Mild esophagitis = microscopic changes of mucosal infiltration with granulocytes or eosynophils ± endoscopic abnormalities

Erosive esophagitis – marked redness , friability, bleeding , superficial linear ulcers , exudates

Peptic stricture- fibrosis that causes constriction of the esophageal lumen

short peptic stricture (1-3cm long) 1/3 distal esophagus

long peptic tubular stricture – persistent vomiting , prolonged nasogastric intubation

Replacement of the squamous epithelium of esophagus with columnar epithelium of esophagus with columnar epithelium (Barret’s esophagus) adenocarcinoma in 5%

Heartburn - angina like Regurgitation (acid)- atypical chest pain

Disphagia – peptic stricture ; progressive disphagia and weight loss indicate Barrett , AC

Bleeding – ulcer Hoarseness Pulmonary aspiration pneumonia, fibrosis , asthma

1. History2. Barium swallow3. Scintiscan 99mTc-sulfur –colloid 4. pH metry – electrode 5cm above the LES

long term (24 h) . De Meester score5. Esophagoscopy – mucosal biopsy6. Berstein test (infusion 0,1 N HCl and

normal saline into the esophagus)7. Esophageal motility studies – motor

function8. Esophageal impedance – both alkaline +

acid reflux

The goals are to : decrease GER neutralize refluxate improve esophageal clearance protect the esophageal mucosa

Weight reduction Sleeping with elevation of the head of the bed Avoid :

smoking, fatty foods, coffee, chocolate, mint, alcohol , orange juice etc

medication calcium channel blockers, antichol. drugs)

Mild forms Mild forms : PPI 20-40mg/day before meals H2 bloking agents (Ranitidine 150 mg,

Famotidine 20mg/bedtime) Antacids 1-3 hours after meals

Severe cases Severe cases : PPI, H2, AA at least 1-2 month Sucralfate 1gx3 before meals (surface

protection) Barrett esophagus- biopsies Antireflux surgery ( Belsey, Nissen’s

fundoplication , Hill repair ) Alkaline reflux – cholestyramine, Aluminium

hydroxide

Due to :•VIRAL•BACTERIAL•FUNGAL•PARASITIC ORGANISM

Acute onset (chest pain, odynophagia, dysphagia)

Bleeding severe cases Nausea,vomiting, fever chills sistemic

manifestations Leucocitosis The persistent infection may lead to

superinfection of denuded esophageal mucosa with fungi/ bacteria or to HSV pneumonia

ENDOSCOPYENDOSCOPY shows vesicles and small punched out superficial ulcerations ± fibrinous exudate

BIOPSYBIOPSY : balloning degeneration ground glass change in the nuclei with

eosinophilic intranuclear inclusions (cow dry type A)

giant cell formation on routine stains positive cultures

ProphylaxisProphylaxis: ACYCLOVIR 800mg po x 2 daily (250 mg/m2 body surface every 12 hours)

Herpes esophagitis. Double-contrast esophagram shows small, discrete ulcers (arrows) in the mid esophagus on a normal background mucosa. Note the radiolucent mounds of edema surrounding the ulcers. In the appropriate clinical setting, this appearance is highly suggestive of herpes esophagitis, since ulceration in candidiasis almost always occurs on a background of diffuse plaque formation.

at children with chickenpox or adults with herpes zoster

DIAGNOSIS – imunohistologically / on culture (≠ HSV)

acquired from blood transfusion

serpiginous ulcers in an otherwise normal mucosa giant ulcers in the distal esophagus involves submucosal fibroblasts and endothelial

cells of the blood vessels but not epithelial cells

Symptoms: painful swallowing, chest pain, haematemesis, nausea ,vomiting

Imagistic exploration Imagistic exploration : Barium swallow (ulcers), Endoscopy+byopsies of the center of the ulcer, mucosal brushing (not useful), imunohystology with monoclonal AB to CMV and in situ hybridization of CMV DNA for early diagnosis

Cytomegalovirus esophagitis in a patient with AIDS. Double-contrast esophagram shows a large, flat ulcer in profile (large arrows) in the mid esophagus with a cluster of small satellite ulcers (small arrows). Because HIV esophagitis may produce identical radiographic findings, endoscopy is required to confirm the presence of cytomegalovirus before patients are treated.

self limited syndrome of acute esophageal ulceration + oral ulcers and a maculopapular skin rash

homosexual men + HIV seroconversion & inversion of lymphocyte T Helper suppressor ratio

electron microscopy – retrovirus – like particles

Two examples of giant HIV esophageal ulcers (arrows) in patients with AIDS. In A, the ulcer is seen in profile, whereas in B, the ulcer is seen en face. Endoscopy is required to exclude cytomegalovirus as the cause of this finding before treating patients.

Lactobacillus β hemolytic streptococci

Immunocompromised host

Patients with AIDS, Cryptosporidium and Pneumocystis carinii may cause nonspecific inflammation of the distal esophagus

In immunodeficiency states (HIV, neoplasms…) occurs in the absence of the above:

predisposing factors bleeding stricture systemic invasion

TREATEMENT 7-10 days: Nystatin (100000U/ml) 10-20 ml/every 6 h Clotrimazol po 10 mg/ 6h Ketoconazole 200-400 mg 1 dose Amphotericine 10-15 mg 300-500 mg total

dose

Candida esophagitis. Double-contrast

esophagram shows linear plaquelike

lesions in the esophagus, with normal

intervening mucosa.

Two examples of advanced Candida esophagitis demonstrate a shaggy esophagus. In both images, the double-contrast esophagram shows a grossly irregular esophageal contour due to innumerable plaques and pseudomembranes, with the trapping of barium between lesions. Patients with this fulminant form of esophageal candidiasis are almost always found to have AIDS.

Candida esophagitis with a foamy esophagus. This patient has a dilated esophagus with beaklike narrowing (arrow) at the gastroesophageal junction as a result of long-standing achalasia. Innumerable tiny bubbles are layering out in the barium column due to infection by the yeast form of candidiasis.

RADIATION ESOPHAGITIS RADIATION ESOPHAGITIS : stricture requires dilatation

CORROSIVE ESOPHAGITIS CORROSIVE ESOPHAGITIS : caustic agents

PILL-induced ESOPHAGITIS PILL-induced ESOPHAGITIS : AB, Aspirin, KCl, Fe, AIS, AINS

ZENKER’ S Diverticula (post-hypopharingeal wall)MID Esophageal D. ± E. motor abnormalitiesEPIPHRENIC D. ± achalasia

Zenker's diverticulum in early and late phase of swallowing

Zenker's diverticulum on chest film, barium study and CT

Zenker's diverticulum

Several epiphrenic diverticula in a patient with reflux esophagitis and a peptic stricture

Large mid-esophageal pulsion diverticulum

On the far left a traction diverticulum (arrow) due to hilar granulomatous disease. Calcified adenopathy (asterisk). In the middle a pulsion diverticulum (arrow) due to high intraluminal pressure.On the right multiple pulsion diverticula (arrows) that preceded Heller myotomy for achalasia.

A traction diverticulum (arrows) secondary to post primary TB.It simulates a cavitary lung lesion on the chest radiograph.

Pseudodiverticula can be seen in reflux esophagitis. A patient with a hiatus hernia, reflux esophagitis, and pseudodiverticula (arrows) at site of proximal stricture

Left: Iatrogenic perforation (arrow). MIDDLE: Communicating esophageal duplication (arrows). RIGHT: Extravasation from iatrogenic perforation of hypopharynx in neonate

congenital / inflammatorysymptomatic hypopharingeal webs with iron-deficiency anemia in middle-aged women Plummer-Vinson SyndromeLE mucosal ring (Schatzki ring) –weblike constriction located at the squamocolumnar mucosal junction at the border of the LESDysphagia to solids-episodic

Esophageal ring due to muscular contraction. It varies during examination and may not persist.

Esophageal A-ring due to muscular contraction. It varies during examination and may not persist.

The esophageal B-ring is located at the squamocolumnar junction, also termed the 'Z' line. The appearance does not change during the examination. On the left a patient with a 'B' ring (arrows) several cm above diaphragm at the apex of sliding hiatus hernia.Note unchanged appearance on these two images.

On the left a 52-year-old man with episodic dysphagia.The image on the far left does not show a abnormality,

but distal esophagus not distended .With dilation of the distal esophagus, a 13 mm wide Schatzki B-ring (arrows) that caused intermittent

obstruction is demonstrated at the apex of a hiatus

hernia (arrowhead).

On the left a 71-year-old man with chest pain after fast food lunch.

Distal obstructing filling defect (arrow) is a piece of meat that passed into stomach during study.

Follow-up esophagram shows Schatzki B-ring

(arrows) that caused obstruction.

Esophageal web

Esophageal web

Multiple esophageal ringsa: In a man with asthma. b: In a 24-year-old man with exercise induced asthma and allergic rhinitis

Schatzki ring

1.1. Sliding H.H. Sliding H.H. – GE jonction (GEJ) and fundus of the stomach slide upward

2.2. Paraesophageal H.Paraesophageal H. – GEJ remaines fixed in its normal location and a pouch of the stomach is herniated beside the GEJ through the esophageal hiatus

COMPLICATIONS 1 +2 incarcerated and strangulated chronic blood loss (gastritis and ulcerations)

Prompt operative treatment in case of : acute chest pain dysphagia mediastinal mass

Large 2 surgically repaired

Hiatal Hernia with Schatzki's Ring. The red arrows point to a slit-like indentation which marks the

position of the esophago-gastric junction and, since it is seen above the diaphragm, therefore defines the presence of a hiatal hernia of the so-called sliding type. This

indentation is called a Schatzki's ring (or "B ring") although that term is reserved by some only for such rings that produce dysphagia. The white arrow points to the herniated stomach while the green arrow points to disordered tertiary waves of

contraction.

Schatzki ring. Prone single-contrast barium esophagogram demonstrating a thin, ringlike narrowing (arrows) in the lower esophagus just above a hiatal hernia. This view is most sensitive for detecting lower esophageal rings, provided adequate esophageal distention is achieved.

Laparoscopic Nissen fundoplication.

Incidence: occurs frequently within a so called Asian extending from the southern shore of the Caspian Sea on the west to northern China ,on the east parts of Iran, Soviet Central Asia , Afghanistan, Siberia and Mongolia

In North America and western Europe the disease is :far more common in blacks than whitesgreater in males than femalesafter age 50associated with lower socioeconomic classes

I. Excess alcohol consumptionII. Cigarette smokingIII. Ingested carcinogens:

nitrates- nitrites smoked opiates fungal toxins in pickled vegetables

IV. Mucosal damage from physical agents: hot tea radiation-induced strictures chronic achalasia

V. Host susceptibility esophageal web with glossitis and iron deficiency congenital hyperkeratosis and pitting of the palms and

soles (tylosis palmaris and plantaris)VI. Dietary deficiencies : vitamin A,ZnVII. Celiac sprueVIII. Chronic gastric reflux ( Barrett’s esophagus)

15 % in the upper 1/3 of the E. (cervical esophagus); 50% in the middle third; 35% in the lower third

85% of the E. tumours are squamous cell carcinomas

Adenocarcinomas develops from columnar epithelium , in the distal esophagus in association with chronic gastric reflux

SPREAD: supraclavicular lymph nodes liver lungs, pleura

Tracheoesophageal fistulas Hypercalcemia – tumor –secreted protein

structurally analogous to parathyroid hormone

Progressive dysphagia

Weight loss of short duration

Odynophagia

Pain radiating to the chest / back

Regurgitation / vomiting

Aspiration pneumonia

CONTRAST RADIOGRAPHS

ENDOSCOPIC BIOPSIES

CYTOLOGIC EXAMINATION OF T. BRUSHINGS

CT SCANS of the chest and abdomen extend of T.

The prognosis is poor… <5% are alive 5 years after the initial diagnosis

Total resection is feasible in 40% of cases ESOPHAGECTOMIES are associated with a

postoperative mortality rate of 20% due to :anastomotic fistulassubphrenic abscessesrespiratory complications

PRIMARY RADIATION THERAPY is not dissimilar to that of radical surgery , sparing patients perioperative morbidity but resulting in less satisfactory palliation of obstructive symptoms

CHEMOTHERAPEUTIC AGENTS CHEMOTHERAPEUTIC AGENTS – ambiguity of “response”; debilitated pysically condition SINGLE AGENT treatment – reduction in size of tumors in

15-25% but 30-60% at the patients treated with DRUG COMBINATION (CISPLATIN)

cure rate increases in the cases with combination CHEMOTHERAPY+RADIOTHERAPY as the initial therapeutic approach , either alone or followed by an attempt at operative resection

UNRESECTABLE PATIENT UNRESECTABLE PATIENT : repeated endoscopic dilatation gastrostomy/jejunostomy for hydratation and

feeding surgical insertion of a polyvinyl prothesis to bypass

tumor Endoscopic fulguration of the obstructing tumor with lasers

appears to be promising