Anatomy and Motility Disorders

Anatomy and Motility Disorders
• Normal anatomy and physiology
• Abnormal anatomy:
– Congenital
– Acquired
• Manometry and motility disorders
• Miscellaneous
– Eosinophilic esophagitis
– Pill esophagitis
Steven Shay MD
CLASSIC DIVISIONS AND ADJACENT STRUCTURES
Distance from incisors
Normal narrowings
Lipham et al. In: Surgery of the Chest. Sabiston, 2010, p517.
1
Hypopharynx-UESProximal Esophagus
EG JUNCTION
(Posterior view)
ESOPHAGEAL MUSCULATURE
AND RELEVANCE TO DISORDERS
From Mashimo H et al. GI Motility on Line 2006.
2
Lymphatics in Submucosa:
Importance in Esophageal Cancer
2009
Note: 1) Small lymphatics extend to just below epithelium
2) No serosa
SMOOTH MUSCLE IN NORMAL PERISTALSIS
A
Stimulus
B
Stimulus
A. Latency to contraction increases down esophagus after initial panesophageal relaxation
B. Cholinergic excitatory vs noncholinteric inhibitory
Goyal et al. In: The Esophagus, Ed 4. Castell, 2004, p17.
3
Barium Bolus Progression:
Simultaneous Manometry and Flouroscopy
Note:1) Leading edge of barium bolus thrust to mid/distal esophagus by oropharynx
2) Barium clears from each site before clearing contraction begins
Kahrilas et al. Gastroenterology 1988; 94:75.
Deglutative Inhibition
Note: Latency persists with multiple swallows until they end;
then, the contraction can propagate thru the esophagus
Modified from Goyal et al. In: The Esophagus, Ed 4. Castell, 2004, p13.
4
DYSPHAGIA LUSORIA
Normal great vessel anatomy
Abnormal right subclavian take off
Note: Oblique ascending extrinsic
compression of esophagus trapped
between trachea and right subclavian
Levitt B, Richter JE. Dis Esoph 2007; 20:455-460
DUPLICATION CYST
Note: Extrinsic compression of the esophagus. EUS shows this to
be a cyst with hyperechoic proteinaceous material (white arrow) and
fluid (black area) adjacent to esophagus.
5
Hiatal hernia types
Sliding
Paraesophageal
Type 1
Type 2
Hiatal hernia types
TYPE 3: MIXED
TYPE 4: Hiatal hernia +
Other Viscera
6
EPIPHRENIC DIVERTICULUM
EPIPHRENIC DIVERTICULUM
Endoscopic view of EG junction
LES
opening
7
STEP 1: RESECT DIVERTICULUM
STEP 3: PARTIAL FUNDOPLICATION
STEP 2: HELLER MYOTOMY
8
What is true regarding Zenkers diverticulum?
a. It protrudes thru weakness in the muscle layer at Killians triangle.
b. Measuring UES pressure at manometry is very valuable in
management?
c. Cricopharyngeal myotomy and diverticulectomy is standard surgical
therapy?
d. Aspiration pneumonia is not a complication.
1. a only
2. a, b, c only
3. a, c only
4. All are true
Hypopharynx-UES- Proximal Esophagus
(Posterior view)
9
CRICOPHARYNGEAL BAR
UES SPASM)
(
BAR
Early Zenker’s
ZENKER’S DIVERTICULUM
Body
10
Zenker’s diverticulum
Large, mixed hiatal hernia
Note: Same patient with large mixed hiatal hernia below the Zenker’s diverticulum
SURGERY FOR ZENKERS DIVERTICULUM:
Myotomy and Diverticulectomy
Note: Surgery done via neck incision
11
ENDOSCOPIC CRICOPHARYNGEAL MYOTOMY
Modified from GI Motilility on line; May 2006
What is true regarding achalasia?
a. Poor LES relaxation and aperistalsis are characteristic findings at
esophageal manometry?
b. Endoscopy is not necessary once the diagnosis is made by barium
swallow and esophageal manometry.
c. Pneumatic dilation gives much poorer results in intial therapy
compared to Heller myotomy / partial fundoplication.
d. Aspiration pneumonia is not a complication.
1. a only
2. a, b, c only
3. b, d only
4. d only
5. All are true
12
Traditional manometry
High Resolution manometry
Pressure
scale
cm
(mmHg)
30
29
28
27
26
25
24
23
22
21
20
20 cm
19
18
17
16
15
15 cm
14
13
12
11
10
10 cm
9
8
7
6
5
5 cm
4
3
2
1
0
LES
-1
-2
-3
-4
-5
-6
1960-
Time (seconds)
Time (seconds)
2007-
Distance
Pressure
Rice T, Shay S. A primer of high-resolution esophageal manometry. Sem Thor and Cardiovas Surg 2011; 181-90.
Anatomic Segment
Time (seconds)
0
10
20
Hypopharynx
(mmHg) (cm)
UESp falls to 0 with swallow
Striated muscle contraction
Rice T, Shay S. Sem Thor and Cardiovas Surg 2011; 181-90.
Amplitude Bolus pressure
65 mmHg
65 mmHg
3 cm/s
Basal
Relax
15 mmHg
0 mmHg
70 mmHg
5 mmHg
Smooth muscle Contraction
Peristalsis parameters
Antegrade
Overshoot
35 mmHg
LES pressure
Intragastric
13
ESOPHAGEAL MOTILITY
DISORDERS
• Achalasia
• Diffuse esophageal spasm
• Hypotensive peristalsis (e.g. scleroderma)
• Hypertensive peristalsis (“nutcracker”)
Modified from Table 42-2. Kahrilas, P et al. GI and Liver
Disease. Schlesinger and Fordtran, 2010; 42: 699.
ESOPHAGEAL MOTILITY
DISORDERS
• Impaired LES (EGJ) relaxation (>15 mmHg)
– Achalasia, 3 types: Absent peristalsis every swallow
– Functional EGJ obstruction (eg stricture): Some normal
peristalsis
• Normal LES (EGJ) relaxation
–
–
–
–
Esophageal spasm: >20% simultaneous contraction >30 mmHg
No contraction every swallow (eg, scleroderma)
Hypotensive peristalsis every swallow (eg, GERD)
Hypertensive peristalsis
Modified from Table 42-2. Kahrilas, P et al. GI and Liver
Disease. Schlesinger and Fordtran, 2010; 42: 699.
14
Achalasia
- Simultaneous contraction,
- LES high, poor relaxation
Normal
Pressure
scale
(mmHg)
Rice T, Shay S. A primer of high-resolution esophageal manometry. Sem Thor and Cardiovas Surg 2011; 181-90.
Three Types of Achalasia
Type I
Type 2
75
50
25
Pressure
Scale
75
50
25
100
Pressure
Scale
75
50
25
100
UES
UES
Type 3
Type
3
Type 2
Pharynx
Pressure
Scale
Pharynx
Type 1
75
50
25
25
100
13-cm
50
75
25
50
25
25
50
25
75
50
100
Gastric
25
75
25
75
50
25
100
LES
50
50
100
3-cm
3-cm
25
100
75
LES
50
50
75
Gastric
75
75
8-cm
8-cm
13-cm
75
LES >30 mmHg thruout
LES >25 mmHg thruout
Esophageal pressurization
<25 mmHg thruout esophagus
Esophageal pressurization
>40 mmHg thruout esophagus
75
50
25
CCF © 2009
Vigorous achalasia
Type 2: Best response to surgery
15
ADVANCED ACHALASIA
Fig 42-9. Kahrilas P et al. GI and Liver Disease.
Schlesinger and Fordtran; 42: 694, 2010.
Achalasia
Primary
“Bird beak” appearance
Secondary
Mass effect on EGJ and
cardia from cancer
From Levine MS et al. Clin Gastroenterol Hepatol 2008;6:11-25.
16
Achalasia Treatment
• Pneumatic dilation
• Laparoscopic Heller-Dor
– Initial symptom relief
in ~ 90%
– 2-4% perforation rate
• Open thoracotomy
– Initial symptom relief in ~
90%
– Risks
• General anesthesia
• Surgery
• GERD ± stricture
Note: Target of treatment is palliation by reducing LES pressure
since there is no therapy to reverse underlying neuropathology.
Which of the following are true?
a. The diagnosis of DES by manometry requires both normal peristalsis
after some swallows and simultaneous contractions after > 20% .
b. Possible treatments for DES include smooth muscle relaxants and
botox injection of the spastic segment of the esophagus.
c. Scleroderma affects distal esophageal muscle but proximal
esophageal muscle is preserved.
d. Nutcracker esophagus causes a severe delay in bolus transit.
1. a only
2. a, b, c only
3. b, d only
4. d only
5. All are true
17
ESOPHAGEAL MOTILITY
DISORDERS
• Impaired LES (EGJ) relaxation (>15 mmHg)
– Achalasia, 3 types: Absent peristalsis every swallow
– Functional EGJ obstruction (eg stricture, tight fundoplication):
Some normal peristalsis
• Normal LES (EGJ) relaxation
–
–
–
–
Esophageal spasm: >20% simultaneous contraction >30 mmHg
No contraction every swallow (eg, scleroderma)
Hypotensive peristalsis every swallow (eg, GERD)
Hypertensive peristalsis
Modified from Table 42-2. Kahrilas, P et al. GI and Liver
Disease. Schlesinger and Fordtran 2010; 42: 699.
Functional Obstruction EG Junction
A
A
B
B
A – Very high Intrabolus pressure: mean 55 mmHg (nl < 15)
B – Increased contraction pressure: 250 mmHg; nl <180
Rice T, Shay S. A primer of high-resolution esophageal manometry. Sem Thor and Cardiovas Surg 2011; 181-90.
18
ESOPHAGEAL MOTILITY
DISORDERS
• Impaired LES (EGJ) relaxation (>15 mmHg)
– Achalasia, 3 types: Absent peristalsis every swallow
– Functional EGJ obstruction (eg stricture): Some normal peristalsis
• Normal LES (EGJ) relaxation
–
–
–
–
Esophageal spasm: >20% simultaneous contraction >30 mmHg
No contraction every swallow (eg, scleroderma)
Hypotensive peristalsis every swallow (eg, GERD)
Hypertensive peristalsis
Modified from Table 42-2. Kahrilas, P et al. GI and Liver
Disease. Schlesinger and Fordtran, 2010; 42: 699.
DIFFUSE ESOPHAGEAL SPASM
Normal peristalsis > 30 mmHg Simultaneous >30 mmHg
Rice T, Shay S. A primer of high-resolution esophageal manometry. Sem Thor and Cardiovas Surg 2011; 181-90.
19
Diffuse Esophageal Spasm
From Levine MS et al. Clin Gastroenterol Hepatol 2008;6:11-25.
39
DIFFUSE ESOPHAGEAL SPASM:
Therapy
• Smooth muscle relaxants
– Calcium channel blockers
– Sublingual nitroglycerin
– Sildenafil
• Botox injection of spastic segment
• Esophageal dilation
20
ESOPHAGEAL MOTILITY
DISORDERS
• Impaired LES (EGJ) relaxation (>15 mmHg)
– Achalasia, 3 types: Absent peristalsis every swallow
– Functional EGJ obstruction (eg stricture): Some normal peristalsis
• Normal LES (EGJ) relaxation
–
–
–
–
Esophageal spasm: >20% simultaneous contraction >30 mmHg
No contraction every swallow (eg, scleroderma)
Hypotensive peristalsis every swallow (eg, GERD)
Hypertensive peristalsis
Modified from Table 42-2. Kahrilas, P et al. GI and Liver
Disease. Schlesinger and Fordtran, 2010; 42: 699.
B
SCLERODERMA
300
Pressure
Scale
200
100
75
Pharynx
Pressure
Scale
Pharynx
A
NUTCRACKER
50
25
75
UES
UES
300
200
100
50
25
75
13-cm
13-cm
300
200
100
50
25
75
8-cm
8-cm
300
200
100
50
25
3-cm
75
200
100
50
25
300
75
200
50
LES
LES
3-cm
300
100
25
75
Gastric
Gastric
300
200
100
50
25
CCF © 2009
Antegrade esophageal contraction
No LES pressure
Increased contraction amplitude
No contraction smooth muscle
21
ESOPHAGEAL MOTILITY
DISORDERS
• Impaired LES (EGJ) relaxation (>15 mmHg)
– Achalasia, 3 types: Absent peristalsis every swallow
– Functional EGJ obstruction (eg stricture): Some normal peristalsis
• Normal LES (EGJ) relaxation
–
–
–
–
Esophageal spasm: >20% simultaneous contraction >30 mmHg
No contraction every swallow (eg, scleroderma)
Hypotensive peristalsis every swallow (eg, GERD)
Hypertensive peristalsis
Modified from Table 42-2. Kahrilas, P et al. GI and Liver
Disease. Schlesinger and Fordtran, 2010; 42: 699.
Hypotensive
Note: Large defects in 30-mmHg isobar
(i.e., waveform outlined by 30 mmHg line)
Failed
Note: No waveform
with 30-mmHg isobar
Rice T, Shay S. A primer of high-resolution esophageal manometry. Sem Thor and Cardiovas Surg 2011; 181-90.
22
MISCELLANEOUS TOPICS
• Eosinophilic esophagitis (EoE)
• Pill esophagitis
EOSINOPHILIC ESOPHAGITIS:
DEFINITION
“EoE represents a chronic, immune / antigen
mediated, esophageal disease characterized
clinically by symptoms of esophageal
dysfunction and histologically by eosinophilpredominant inflammation”
Liacouras C et al, J Allergy Clinc Immunol 2011
23
EOSINOPHILIC ESOPHAGITIS:
HISTOLOGY
Note: at least one biopsy must have > 15 eos in hpf
Furuta G, et al. Gastroenterology 2007;133:1342-63
EOSINOPHILIC ESOPHAGITIS
SYMPTOMS
• Chest pain
• Dysphagia
• Food impaction
CLUES
• FH esophageal dilations or recalcitrant GERD
• “Pretreatment” may mask EoE
(i.e., topical steroids for other atopic diseases)
Straumann et al, Allergy 2012.
24
Eosinophilic Esophagitis:
Endoscopy
EGD normal
in 10%
Gonsalves N et al. Gastrointest Endosc 2006;64:313-9.
EOSINOPHILIC ESOPHAGITIS:
TREATMENT
• Traditional initial therapy
– PPI’s
– Topical fluticasone / viscous budesonide
• Other therapy that may be necessary
– Esophageal dilation (slight increased risk
of perforation, though rare)
– Diet therapy
• Adult: 6-food elimination diet; Child:elemental
• After food allergy testing?
– Systemic steroids
25
EOSINOPHILIC ESOPHAPGITIS
6-Food Elimination Diet
•
•
•
•
•
•
Nuts
Fish/shellfish
Wheat
Eggs
Dairy
Soy
EOSINOPHILIC ESOPHAGITIS:
TREATMENT
• Traditional initial therapy
– PPI’s
– Topical fluticasone / viscous budesonide
• Other therapy that may be necessary
– Esophageal dilation (slight increased risk
of perforation, though rare)
– Diet therapy
• Adult: 6-food elimination diet; Child:elemental
• After food allergy testing?
– Systemic steroids
26
Which of the following pills can occasionally cause
deep esophageal ulcers and/or strictures?
a. Potassium supplements
b. Foxamax
c. NSAID’s
d. Tetracycline
1. a only
2. a, b, c only
3. b, d only
4. d only
5. All are true
Pill Esophagitis
Clinical Presentation
• Odynophagia
• Chest pain
• Dysphagia
27
PILL ESOPHAGITIS
(75% of 1088 cases in largest publication to date)
• Severe damage ocasionally (deep ulcer/stricture)
–
–
–
–
–
Potassium supplements (n=33)
Quinidine (n=13)
Fosomax (All biphosphonates; n=127)
Non-steroidal anti-inflammatory (n=121)
Ferrous sulfate (sustained release form; n=24)
• Mild (superficial ulcers)
– Tetracycline (n=437)
Kikendall JW. Diseases of the Esophagus. 2006
28