A GEDANKEN EXPERIMENT
The oval represents the muscular wall of the thorax and abdomen lined, in the
case of the latter, with peritoneum. In the static case, the abdominal contents
act as a "bag of water" and, with normal abdominal tone, will tend to extrude
the peritoneum through the gaps in the wall forming ventral hernias at v
and inguinal and femoral hernias at I/f by hydrostatic pressure. There
is no hydrostatic pressure on the top of the bag of water. If anything, the
membrane closing the hiatal gap h would sag downward.
If the muscular wall contracts, the abdominal pressure increases and the hernias
are exaggerated. However, any pressure gradient at the hiatus is equalized by
upward or downward motion of the diaphragm.
(2A) Belching: Male age 58. CC "Gas", "Feels like I'm going to explode!",
heartburn, nocturnal laryngospasm, cheilosis. Lost teeth at age 19, lump-in-throat
symptom.
There was esophagitis grade 2 and grade 2 reflux. Here the patient has been
induced to belch. Note how LM traction elevates the PEL above the dome of the
diaphragm. Loss of diaphragmatic sharpness is a subtle sign of LMC. Despite
a life-long history of LMT symptoms, there is no stretching of the PEL to present
as a "hiatus hernia." Such relaxation or even rupture may have a beneficial
effect on symptoms.
(2.2) Belching produces a HH. Over the course of a lifetime, belching,
gagging, rapid swallowing and vomiting generally stretch the PEL beyond its
elastic limits - a limit that is also decreasing with age.
(4-3a and 4-3b) Advanced achalasia: [A] Dilated, redundant esophagus.
[B] Narrowing just behind but above the dome of the diaphragm.
(53) Globus due to enlarged lingual tonsil.: CC: "Feels like a peach
pit caught in my throat." Nocturnal reflux. On fluoroscopy, grade ii reflux.
Captive bolus, duodenitis, 7 cm HH, esophagitis. Normally, there is an air space
between epiglottis and the lingual tonsil. An increased anterior curl of the
former or enlargement of the latter brings the two in contact and this is perceived
symptomatically as "globus." Reflux is the probable cause of the tonsilar in
enlargement but lymphosarcoma and other tumors cannot be excluded.
(6A) Enlargement of lingual tonsil causes globus or a "lump in the throat"
sensation. The patient states, "It feels like food or a pill is stuck in my
throat." Not relieved by drinking water. Reports cheilitis but no wet spot on
pillow. On fluoroscopy, there was a moderately severe esophagitis, copious reflux
in response to the dC maneuver, hiatus hernia and a grade iii duodenitis .
Normally, there is an air space between the tip of the epiglottis and the base
of the tongue. When reflux causes edema of the epiglottis or, as is shown here,
lingual tonsillitis, the air space is lost. In lateral projection [A] the epiglottis
is plastered against the lingual tonsil. In the frontal view [B] the contact
zone appears as a nearly circular ring above the median raphe of the valleculae.
This physical contact between the two structures is perceived symptomatically
as a foreign object in the hypopharynx.
(7) Cricopharyngeus "spasm": The force of LM contraction is considerable.
Shortening 40% or more of its length, it can tear the lower esophagus from the
diaphragm, stretching or avulsing the PEL. A force of equal magnitude and opposite
direction is consequently applied to the hypopharyngeal attachment of the organ.
The pathological findings in excised specimens of the so-called "cricopharyngeal
bar" are those of old hemorrhage and fibrosis - typical of repeated soft-tissue
injuries. When the larynx elevates it no longer stretches the upper esophageal
sphincter properly giving rise to this appearance. Note that a post-cricoid
web is also present causing the marked turbulence (B). This homolog of the "Shatzki
ring" further demonstrates the similar mechanics of causation.
The patient had heartburn almost daily. Occasional wet spot on his pillow in
AM. Lost his teeth at age 32. At fluoroscopy, grade iii esophagitis, grade iv
reflux, tertiary contractions, HH and duodenitis, grade iii, were also noted.
The p-wave showed impaired cleanup.
(8A,B) LMC causes hiatal transtraction ("hiatus hernia") (A) Severe
reflux esophagitis apparently without HT. (B) A few seconds later LM shortening
pulls the fundus through a distensible hiatus. Note the soft tissue investments.
(8A) LM tension causes the gas/bloat symptom: Here the patient is trying
to vomit. He habitually induces vomiting to relieve the sensation of left upper
abdominal pressure. Severe heartburn. Acid regurgitation but no cheilosis. Frequently
a wet spot on pillow in AM. Numerous dental caries, frequent sore tongue. Mild
duodenitis.
[A] Severe esophagitis: There are no signs of HH with the LM relaxed. [B] The
remarkable force of LMC has stretched the PEL as the patient retches. Spontaneous
mass contraction drew the fundus 8.5 cm above the diaphragm. This happened slowly
enough over a period of about 10 seconds that the patient could be asked whether
he was having the "pressure" sensation that was his chief complaint. He emphatically
responded that he was. Questioned a few seconds later, after the LMC had subsided,
he reported that the "pressure" sensation was gone.
In vomiting this sequence occurs almost instantly. The LM has contracted 37%
of its length (8.5 cm/23 cm). One can imagine what this would do to reconstructive
surgery about the hiatus.
(11) The esophagus "sees" foreign bodies. In [A] a barium tablet is
arrested by a LER. Although it appears arrested below the diaphragm, it is merely
projected below the dome. In [B] LM contraction has been provoked an attempt
to dislodge the tablet. Hiatal squeeze is still enough to prevent escape of
gas from fundus. The diaphragm is becoming unsharp. At [C], the entire fundus
and perihiatal region is elevated by the LM tension that has drawn a gas filled
tube of stomach through the hiatus. The PEL tent surrounds this tube. [D] Later
a Valsalva maneuver applies external pressure to the tube producing the "empty
segment" appearance by inflating the PEL up to its insertion..
It cannot be assumed that the introduction of foreign bodies such as endoscopes,
pH meters, catheters, balloons and transducers will be physiologic. Here a small
tablet has caused marked LMC and orad sphincter displacement. These and other
effects such as sphincter release can invalidate manometric measurements or
render them uninterpretable. The air filled gastric tube (arrow) could be mistaken
for esophagus lined with gastric mucosa.
(15) Rupture of the phrenoesophageal ligament.: The very elastic PEL
provides both the inferior attachment of the esophagus and the force that restores
the esophagus to its normal resting length and reduces the sliding HH. In a
huge HH such as this, the esophagus is permanently shortened because the elastic
PEL is ruptured. Consequently these HH's do not "slide." Resolution of the force
of LMC by the PEL also creates the sphincter-opening vectors. When the PEL ruptures,
this mechanism is destroyed and the sphincter does not efface well although
a bolus will partially distend it. This non-effacement is a not uncommon cause
of dysphagia.
By the same token, LMC and hiccups can no longer open the sphincter. These
patients usually experience symptomatic remission! This is the explanation for
the paradox that the largest HH's are the least symptomatic. Note that the true
length of the LES (8 mm corrected for magnification) is much less than it is
judged to be by manometric methods. Although one can infer that gastric mesentery
herniates along with the fundus in HH, this illustration shows it directly (arrows)
proving that it extends to the GE junction.
Without the restoring elasticity of the PEL, the esophagus does not alternate
between sort and long. The mucosa no longer needs an accordion pleat, therefore,
and LERs are seldom seen after rupture of the PEL.
(18) HH with a wide hiatus. Because of the wide hiatus, there is no
hiatal constriction of the gastric blood supply, hence there is no swelling
of the gastric mucosal folds above the diaphragm. Such patients do not get achalasia.
(19) "Globus hystericus": [A] The patient had reflux, esophagitis, HH,
impaired p-wave and a LER. This deformity of the epiglottis can cause the globus
symptom by impinging on the lingual tonsil. [B] There is no laryngeal ventricle
"fishmouth" shadow due to swelling of the false or true cords. Reflux can cause
a characteristic posterior laryngitis.
(20) With a neutralized CD receptor, LMC causes gross reflux.: The patient has just been placed in the prone RAO position after the dC test has turned off the protective Cannon-Daugherty reflex. A powerful LMC retracts 6 cm of stomach through the hiatus, opens the sphincter and causes gross GE reflux of the water-diluted barium. History of ++ heartburn, nocturnal laryngospasm, "lot of gas" and bloating. Fluoroscopy also showed a captive bolus, esophagitis, duodenitis. Note that the sphincter region is less distensible. This is often misinterpreted as a "smooth stricture" and thought to be a sign of Barrett's esophagus especially when seen in air contrast.
(22) "Gas bloat" with a slipped Nissen 10 years after the operation.: A
Nissen fundoplication slips because LM tension pulls the the esophagus through
the encircling cuff of stomach, everting the latter in the process and resulting
in the complex topology seen here. The esophagus can pass through the hiatus
as can a portion of the fundus that is drawn out into a tube [B] The ora serrata
(arrow) is 2.5 cm above the diaphragm (arrow) but the stomach bulk must stay
behind forming the mass of tissue which now separates the fundus from the diaphragm.
No more dramatic illustration of the power of LMC can be found than its ability
to achieve this complication. Because of it, the LM now has a lower purchase
on the stomach and need shorten the esophagus correspondingly less to produce
the gas/bloat sensation or, from another point of view, the same degree of LMC
will produce worse symptoms. Hence the frequency of gas/bloat after this procedure.
Extreme LM tension transmitted to the diaphragm can also produce nausea, a
symptom this patient has experienced for 10 years. He also has nocturnal reflux
which he believes is affecting his lungs. He has lost 7 teeth in the last 3
years.
(23) Iatrogenic hiatus hernia.: This is the sole example of a "paraesophageal
HH" I have been able to collect. Although, it is iatrogenic, this is the way
one should look. The esophagus is attached to the diaphragm and the stomach
protrudes alongside it. If HH's were due to chronic or intermittent increases
in intra abdominal pressure, this should be the most common variety of all.
(24) Chronic esophageal shortening.: The patient has complaints of "gas",
nausea and acid regurgitation. Meat sticks substernally. Must sleep in a chair.
[A] The HH is obviously under tension which varies somewhat but never relaxes
entirely. It is easy to see how endoscopically this would appear to be a Barrett
esophagus - there is gastric mucosa in an esophagus-sized tube 4.5 cm above
the hiatus and the sphincter region resembles a stricture. Constant LM traction
on the sphincter keeps it open constantly, hence the air esophogram.[B &
C] Further LM traction pulls the fundus through the hiatus.
On endoscopy, there was no mass at the gastroesophageal junction (arrow). This
probably represents extrinsic pressure by a ruminant of the torn PEL.
(26) Esophageal folds: Normal folds measure 1 mm or less and one can
count 5 or 6 of them. They are caused by the circular muscle. Not the m.
mucosa that is also longitudinal. There are no transverse folds unless,
for one reason or another, the esophagus can shorten with LMC.
(27) Pseudo tumors of the fundus are reduced hiatus hernias: Enlarged,
friable mucosal folds in the fundus can be mistaken for a neoplasm. These folds
become swollen when the venous return from the fundus is impaired by hiatal
constriction. They persist when it reduces.
(28) Rupture of the PEL and the angle of His.: Because of the acute
angle of His, it has been assumed that this condition is a paraesophageal hernia.
However, it obvious that the esophagus is not attached to the diaphragm at any
point due to complete rupture of the PEL. The stomach floats into the chest
either alongside the shortened esophagus, producing the acute angle of His seen
here, or else telescopes over it to produce the "molar tooth" appearance. Once
the PEL ruptures, the patient's reflux may be cured! This is probably responsible
for the belief that the angle of His prevents reflux. Note the fatty mesentery
along the greater curvature.
(29) LMC and the trumpet GE junction.: The force of LMC is resolved
into 2 vector components both of which are well displayed here. a.) One component
stretches the PEL, and b.) One opens the sphincter. Because these forces exist
in 3 dimensions, are affecting elastic structures and are modified by the oblique
PEL insertion, a striking, trumpet-like flaring of the GE junction results.
(30) Tertiary contractions: An esophagus without a functional p-wave
will contract en masse. This is the fallback mode when obstruction is
encountered. Tertiary contractions are a complex manifestation of a.) LM shortening.
[Note the loss of the posterior bowing of the esophagus as the TC's form.] b.)
Mass circular muscle contraction. c.) Self buffering - an en masse contraction
of circular muscle cannot compress the liquid contents of the organ. The stronger
muscle bundles overpower the weaker resulting in alternate constricted and widened
segments. Modern anatomical research has shown that the muscle of the body is
in part spirally arranged, a fact that is well shown during such contractions.
There appear to be two spirals - one ascending from below, the other
descending from above.
The velocity of both LM and CM contraction is remarkable as can be seen on
these exposures second apart.
(31 ) The lower esophageal sphincter. : Here the bulb-syringe action
of mesentery crowding into the tented PEL is powerful enough to override the
sphincter, forcing the captive bolus back into the body of the esophagus. At
this instant, the true length of the sphincter is seen to be only 7-8 mm, far
shorter than has been supposed from the evidence of manometry. The sphincter
remains closed against this considerable force from below, but when it does
yield (after 3 seconds) it then relaxes completely within a second. Gastric
mesentery crowded into the PEL tent by the Valsalva maneuver is the driving
force.
(31X) Belch: LMC jerks the stomach into the chest to the end of the
tethering PEL.
(32) Lower esophageal ring formation:
This sequence illustrates why LER's are at the GE mucosal junction. The advancing
p-wave milks any mucosal redundancy to the limit of its travel. It cannot push
it farther, because this is where peristalsis stops - in this case about 5 mm
above the mucosal junction. In other cases this short aperistaltic segment may
be 2.5 or even 3 cm in length. One never sees peristalsis rolling over a LER.
Note that peristalsis consists of a shortening cone of contraction.
(33) The De Carvalho test: Frame [A] shows barium in a short tube of
stomach above the diaphragm. In frame [B], it is diluted by water being swallowed
by the patient in the RPO position (supine, right side down). The sphincter
is still closed, however. In frame [C] refluxing barium is diluted by water
in the esophagus as the CD receptor is flushed. By frame [D] the entire esophagus
is flooded with refluxed barium. This is the time to ask the patient a.) whether
he can feel something coming back up and b.) whether it reproduces his symptom
in all but degree.
Both cats (Cannon) and ruminants (Daugherty) have a sensory area near the mouth
of the esophagus that inhibits LM contraction when stimulated by ingesta. The
de Carvalho maneuver, as shown here, washes this area free of ingesta allowing
reflux to occur in patients with high LM tension.
(34) Longitudinal muscle contraction causes transverse folds.: The widely
distended bowel could be due to paralysis or distention. Folds cannot form unless
the lumen is obliterated. Numerous transverse folds, misnamed valvulae conniventes
prove that the LM is contracting and thus distinguish mechanical obstruction
from paralytic ileus in which atony of the m. propria prevents any fold
formation.
(35) Achalasia can clear in seconds: Frame (A) shows an esophagus emptying
slowly by hydrostatic pressure alone as there is no peristalsis. Note the "tram-lines"
similar to those seen in hypertrophic pyloric stenosis passing through the hiatus
and the "bird beak" termination at the diaphragm. The latter appears to be partly
surrounded by a 3 cm soft tissue mass in the gastric air bubble. The beak of
the "bird" tends to approach the horizontal because the esophagus is redundant
because of the 4.5 cm HT that is not seen until frame [C].
Later frames showed normal peristalsis with excellent peristaltic cleanup despite a grade ii esophagitis. The soft tissue mass has vanished! Frame [D] shows the subtle, yet unmistakable edge of a trumpet which indicates powerful LMC that has released the trapped fundus by reducing hiatal squeeze.
(35X) Transient achalasia: There is overnight retention of mucus globules
[A] in the lower esophagus. Emptying was by gravity only. A Valsalva maneuver
demonstrated a transtract [C] and when this reduced all signs of achalasia vanished.
(36) Terminal annular constriction [A] is not a carcinoma, a
stricture or "terminal esophagitis" of Schatzki but a sphincter that cannot
efface because of rupture of the PEL [B].
(37) A slightly more severe stage of achalasia: The nearly horizontal
"bird-beak" configuration [frames A &B] and tramline shadows (frames 2-5)
are shown to be in the herniated stomach by the fact that the p-wave - which
stops at the sphincter - ends well above the diaphragmatic constriction. A good
clean-wiping p-wave is still able to force the partial obstruction [C &
D]. During this stage, the CM will undergo work hypertrophy.
(38) Failure of sphincter latching: This patient with chronic reflux
had a mild impairment of the p-wave. The feeble p-wave cannot latch the sphincter
and, on encountering back-pressure because of the temporarily occluded PEL,
gives way and allows reflux back into the esophageal body [D]. This may be an
important factor in the muscle hypertrophy of achalasia.
(39a) Pseudo tumor of the fundus : The venous return is compromised
when a gastric segment is trapped above the diaphragm [A]. The result
is engorged and friable mucosa. Clinically, this accounts for the tendency of
HH's to bleed. Such mucosa may bleed 1 unit/month without turning stools guaiac
positive. When the HH is again normally situated below the diaphragm [B], this
engorged mucosa - and gastric wall as well - present as a tumor-like mass in
the fundus. Provoking the HH [B] will cause the pseudotumor to vanish as shown
here.
(40) LMC opens the sphincter. : In some extreme cases of excessive LMT
the sphincter may remain open indefinitely as in this patient. The trumpet shape
is the geometrical resultant of resolution of the force of LMC into sphincter-opening
and PEL-stretching components. Receptors in the fundus, when stimulated by acid/pepsin,
inhibit LMC. The appearance seen here may be reproduced by washing the posterior
wall of the stomach with water (the de Carvalho maneuver) thus "turning off"
the inhibitory reflex.
(41) The p-wave stops at the sphincter. : The cone of peristalsis has
its base on the sphincter. It becomes progressively shorter, but never passes
beyond the sphincter. Even without landmarks such as the LER seen here, this
fact makes sphincter identification easy. In this patient, as is very common,
there is an aperistaltic segment - the region below the sphincter and
above the LER. Here it measures 2.4 cm, but may be much shorter or even non-existent.
(42) Non-effacement of the sphincter. : The PEL is ruptured in this
patient with 50% of the stomach in the chest destroying the normal mechanism
for sphincter effacement. The sphincter may be pylorus-like as in this case,
neither opening or closing. This may cause mild dysphagia. The 1 cm sphincer
length is far shorter than the 4.5 cm or more derived from manometry.
(44) Disappearing LER: When the HH is demonstrated with the Valsalva
maneuver (A), a typical LER forms. When it is evoked by inducing belching (B)
there is no trace of a ring. The main difference is that there was no peristaltic
wave in (B) to milk mucosal redundancy distally. Also note the long aperistaltic
segment between ring and sphincter. The slight hourglass constriction in [B]
(arrow) is the less distensible sphincter region. This may be misinterpreted
as a stricture in air an distended esophagus and diagnosed Barrett's esophagus..
(45) LMC produces reflux: LMC is evident from the marked tenting of
the fundus into an unusually wide hiatus. The lateral stretching of the GE junction
explains the fact that, although produced by longitudinal traction, Mallory-Weiss
tears are also longitudinal. Barium in the distal esophagus is reflux. The hiatus
itself is widened by the lateral resolution of LM force. If a transducer is
measuring hiatal squeeze, it will register decreased pressure when the LM contracts!
(46) "Inflammatory" gastro-esophageal polyp: The term is probably a misnomer. Note the tight hiatus which constricts circulation in the portion of the stomach retracted above the diaphragm.
This is a minor degree of pseudotumor of the fundus.
(47) The hiatus itself may be a cause of dysphagia. : A 12.5 mm barium
tablet was arrested at the diaphragm [A] Compare tablet with the hiatal size
in frame B. The patient also had an apparently normal, but fixed pylorus which
neither contracted or expanded. His symptoms ("Feels like there is a clamp on
my stomach.") were completely relieved by endoscopic dilatation of the pylorus.
(48) Severe esophagitis, grade iii. : 2 folds occupy the entire width
of the relaxed body.
(49) Corkscrew esophagus: Every book on the esophagus has at least one
of these. This elderly gentleman had been followed for many years with frequent
GI exams at leading institutions. The spectacular curling allowed the obstruction
(arrow) to go unnoticed. It is easy to see spiral muscle bundles in this case.
The tracheal aspiration was asymptomatic and did not elicit a cough reflex.
(50) Symptomatic post-cricoid ring with Zenker's diverticulum: CC:"Food
sticks in throat." Comparing these landmarks with the first rib or a cervical
vertebrae shows they have an upward excursion of 2.8 cm at the outset of deglutition.
This exerts an abrupt, forceful tug on the esophagus which, transmitted to the
PEL, may supply all the force needed to open the sphincter. The sharp tug may
also trigger a stretch reflex causing LMC.
As is invariably the case with Zenker's diverticula, the patient also had a
HH. The association of the two is due to the circumstance that powerful LMC
stretches or disrupts the esophageal attachment at the hypopharynx as well as
at the diaphragm.
(51) Peristalsis stops at the sphincter.: Frames 1,4, 9, 10, 11 of a
sequence made at 2 frames/sec. (A) The esophagus is flooded with barium from
a large bolus. The thick ring sometimes called the "A" ring or "muscular ring,"
(arrow) is actually the sphincter. In (B) a mucosal ring comes into view as
the esophagus shortens. The sphincter is partially obliterated by increased
intraesophageal pressure from the advancing p-wave. Despite mild esophageal
varices, the p-wave is clean-wiping to the lower edge of the sphincter. The
short segment between the sphincter and the ring is aperistaltic. Note the LM
relaxation as soon as the p-wave reaches the sphincter. The patient had gross
GE reflux, easily provoked by dorsiflexion of the cervical spine.
(52) Long PEL: Although the PEL is difficult or impossible to see in most cases, it produces effects which are unmistakable. Here a Valsalva effort collapses the stomach in the PEL distal to its attachment because hydrostatic pressure is confined to the (in this case) tubular PEL tent. This extreme example shows the mechanism responsible for the "empty segment." It has no part in preventing reflux. Tissues slowly crowding into the PEL have been mistaken for prolapsing mucosa.
(54) The Cannon-Dougherty reflex: If the posterior wall of the stomach
is flushed with water in the supine position [A] it may "turn off" the CD reflex
that normally inhibits reflux. A hypertonic LMC then produces gross reflux of
either air or gastric contents when the patient assumes the prone RAO position
[B & C]. When LMC occurs at this point one simply asks the patient if he
has the gas or bloat symptom at that instant.
The deCarvalho maneuver causes reflux in patients with a hypertonic LM because
it neutralizes the CD inhibitory reflex.
Compare the appearance of the gastric segment (a traction cone in [B] &
[C]) when a hiatal transtraction is produced by this method with that (captive
bolus) produced by the Valsalva test in the same patient [D] The faint outline
of the PEL tent can be seen here.
(55) Nocturnal laryngospasm is a frequent symptom of reflux. Aspirated
barium defining the pyriform sinuses, true and false cords and laryngeal ventricle.
It may cause a posterior laryngitis with intermittent hoarseness.
(56) Post-cricoid ring: In many ways analogous to the LER, it is not
restricted to PVS. Oddly enough, unlike LERs, there is no resistance in academia
to the idea that these rings are mucosal plications. Note flow disturbance below
ring.
(58) Belch: (A) Before, (B) After. Although the release of air from
the stomach may be explosive, unless the superior constrictor releases, the
esophagus may remain air-distended for 8-24 seconds affording an excellent opportunity
to note the traction effects of LMC and their correlation with the opening of
the sphincter. Barium is dilute from the dC test which has turned off the CD
receptor that acts as a guardian of the gate to prevent reflux when submerged
in acid/pepsin. The trumpet shape explains the orad directed wedge shape of
Mallory-Weiss tears.
(60) Candida infection: Male, age 26 with HIV-III virus infection. There
are actually 3 types of abnormal folds: a.) Thickened longitudinal folds similar
to those of reflux esophagitis, b) Mammillary, rice-grain nodularity and 3)
Transverse folds. The latter produce the saw-tooth appearance in profile when
the stiffened mucosa cannot take up the slack as LMC shortens the organ.
(61) Transverse folds with eventration of the diaphragm: Although the
patient did not have an elongated PEL, eventration of the left leaf of the diaphragm
provided enough slack for development of transverse folds upon LMC. Longitudinal
folds are increased in thickness and decreased in number due to mucosal thickening
by esophagitis.
(64A) The pinchcock at the diaphragm: The constriction is actually well
above the diaphragm and obviously much thicker than the diaphragm. On this lightly
exposed film, one can just make out the bell-like tent of the PEL which, by
constraining mesentery protruding through the hiatus, chokes off the fundus
attached at the apex.
(64B) Severe grade 3 reflux esophagitis. Grade = 5 minus number of folds.
(68) Achalasia and DES: Work hypertrophy in achalasia/DES is not idiopathic.
It is secondary to intestinal obstruction.. The normal response to obstruction
is contraction of the LM and en masse contraction of the circular CM.
Chronic obstruction of the esophagus causes both "tertiary contractions" and
work hypertrophy of the circular muscle. The following table was calculated
to determine the cross-sectional area of the esophageal wall at the levels shown
on this exposure, a part of a 2-second burst at 10 frames/sec.
| OD | ID | Area | % Increase | |
| 24.4 | 16.2 | A | 2.62 cm2 | 0.00 |
| 22.9 | 11.6 | B | 3.05 cm2 | 16 |
| 22.9 | 7.5 | C | 3.65 cm2 | 39 |
| 25.2 | 9.8 | D | 4.19 cm2 | 60 |
| 27.1 | 8.6 | E | 5.20 cm2 | 96 |
Measurements were made with a dial gage and corrected for minification of the
105 mm format camera. A 12.5 mm barium tablet was used to calibrate the latter.
Although this hypertrophy is always referred to as idiopathic, work hypertrophy
is the obvious cause. Aganglionosis, on the contrary, causes loss of muscle
tissue. The reason the hypertrophy increases aborally is that the intra luminal
pressure increases as the p-wave moves distally (There is less length of lumen
to contain the same volume.) so progressively more work must be done.
The obstruction is in the soft tissue mass between the stomach and the diaphragm
[G]. This is a small piece of stomach or omentum trapped in the PEL tent and
hiatus.
[B/C/D] The speed of CM contraction can be appreciated from these exposures
made at 1/10ths second intervals. Again note greatly increased wall thickness.
68 [E/F] The sphincter (arrow) would be difficult to distinguish from
the other constrictions were it not for its usual relation just above a LER
(open arrow). Wide open, it is not causing obstruction.
68 [G] In LAO Note faint tramline and a knuckle of HH passing through the diaphragm.
Interposed tissues separate the fundus from the under surface of the diaphragm.
The obstruction is due to these tissues caught in the small hiatus. This narrowing
is generally thought to be the sphincter but is far longer than the sphincter
and is, in fact, compressed stomach.
T-1 Reflux into salivary ducts: Note opacified Wharton and Stenson ducts.
Apparently this was asymptomatic in this patient with neurogenic dysphagia,
although if acid/pepsin were involved gland inflammation would be anticipated.
Evidently this is not on the PVS palate.
T-2 "Terminal esophagitis": Shatzki believed this appearance
was inflammatory, however, it occurs so frequently in patients with ruptured
PELs that it seems more probable it is due to non-effacement of the sphincter.
When the esophagus shortens, it becomes thicker for purely geometrical reasons.
T-3 Omentum crowding into the PEL tent. Note the PEL insertion at the
sphincter. Although the rest of the PEL is largely invisible, it can be appreciated
by the way it constrains the infra diaphragmatic tissues after they protrude
through the hiatus.
T-4 An unusual sphincter variant: The sphincter, initially double (A),
was demonstrated in the usual way when pressure from the CB forced it. Two frames
later [C] it has merged into one.
T-5 Systemic sclerosis look-alike: The circular muscle of the esophagus
atrophies in systemic sclerosis while the LM is unaffected. The result is this
typical appearance. The esophagus is constantly short. The HH never reduces.
The sphincter never closes. As a result, there is a constant air esophogram
which can be seen even on chest films. The superior constrictor does not relax
which, at least in the upright position, prevents gastric fluids contacting
the esophagus. There is no peristalsis below the striated muscle portion. Note
the relatively small hiatus and turgid gastric mucosal folds in the transtracted
stomach.
The patient is a 71 year old undifferentiated schizophrenic on long-term haloperidol
(Haldol) medication. He also had megaduodenum and pseudo-intestinal obstruction
but did not exhibit Renaud's phenomenon. Such drugs may mimic systemic sclerosis.
T-6 LMC with tubular HH: The short esophagus with a tubular HH can easily
be mistaken for an esophagus lined with gastric mucosa. Note the mucosal transition
and compare with published cases of BE. Distention of the esophagus with air
causes reflex LMC producing this appearance.
T-7 Multiple LERs: Can each one have a different etiology? Or are they
accordion pleats?
T-8 So-called "intramural diverticula:" The name is an oxymoron as if
they are intramural, they are, by definition, not diverticula. The only thing
they could be due to is barium in ectatic mucus glands, the glands that provide
esophageal lubrication.[Case provided by O. Arthur Stiennon, III]
T-9 Fundic peristalsis: The most proximal gastric p-wave I have ever
encountered was in this severely nauseated patient. Gastric peristalsis cannot
take over when the esophageal p-wave stops.
T-10 Candida infection with AIDS: Note the enlargement of the mucus
glands.
T-11 The "Burnoose phenomenon:" Note how a hood appears about the mouth
of the esophagus and then vanishes as the LM draws it upward. It has been aptly
likened to an Arab headdress by Jutras.
X-10 A toroidal Angelchik prosthesis: closely mimics the toroidal appearance
of gastric omentum in the PEL tent and thus the appearance of "achalasia."
Y1 The cause of achalasia: The outlines of the PEL and its contents
can be seen here as LMC elevates the latter through the hiatus. Even a tag of
fat trapped in a small hiatus when the LM again relaxes can cause obstruction
causing the "achalasia" appearance.
Y2 (A) Epiphrenic diverticulum for 34 years. These diverticula form
as a buffer because, if there is obstruction at the hiatus, there is no other
place for the bolus to go when peristalsis reaches the end of the esophagus.
For the same reason, they are epiphrenic. Note the small hiatus. (B)The
diverticulum is no longer in contact with the diaphragm because LMC has drawn
7 cm of stomach into a tube in an unsuccessful attempt to clear the obstruction.
Shortly after this film was made she was operated on for obstructive symptoms
and the diverticulum resected! The result was satisfactory, however, probably
because the hiatus was widened incidentally.
Y 3(A) Classical decompensated, redundant esophagus of achalasia. (B)
Note the small hiatus.
Y-4 *Achalasia can occur overnight and reduce spontaneously: Fluoroscopic
note:"Barium entered the esophagus to mix with a great deal of secretions
and food particles. There were spectacular 'tertiary contractions' which were
ineffective in emptying the organ. A barium tablet lodged in the distal esophagus.
There were 2 narrowings. One was 3 cm above the diaphragm corresponding to the
sphincter (frames 1-4) and the other was at the actual diaphragmatic hiatus.
The tablet lodged below the sphincter. At this point a longitudinal contraction
of the esophagus occurred (frame 5-9) that tented the diaphragm. Simultaneously
both areas of constriction disappeared relieving the obstruction."
This sequence illustrates the function of LMC in dislodging obstructing food
particles. It not only obliterates the sphincter but widens the hiatus itself.
Here a small hiatus is holding up passage of a barium tablet. Note how the GE
junction, that appears to be below the diaphragmatic dome in frames 1-4, is
abruptly elevated above the dome as the tablet clears the hiatus in frames 7-9.
Y5 Hypertonic longitudinal muscle: Frames 1-17. LER, HH, esophagitis,
gr. 2 reflux. For a 19 second exposure, the sphincter alternately opened and
closed depending on strength of LMT. The sliding HH never reduced completely.
Note the "alpenhorn" configure of the GE junction when maximally stressed. I
have seen a sphincter remain open under LMT for over 28 seconds in a patient
with a hypertonic LM and indefinitely in myotonia dystrophia and scleroderma.
The LM opens the sphincter. '
Y6 Non effacement of sphincter: Rupture of PEL demonstrated by (A) the
dC maneuver, and (B) by inducing belching. The force of LMC is resolved into
sphincter-opening vectors by the PEL. When it ruptures this mechanism fails
giving the appearance that Schatzki attributed to "terminal esophagitis." It
is actually a non-effaced sphincter. A mild dysphagia may result.
Y7 Barrett esophagus: The short esophagus + stricture + tubular HH shown here may appear to the endoscopist to be a Barrett esophagus. Acid reflux can cause esophageal shortening. If films are exposed for low contrast and low density, one can often see the external surfaces of these structures.
Y9 Longitonia: Reflux, esophagitis, vallecular sign, aspiration. For
30 seconds the sphincter remained open, the LM contracted and the diaphragm
tented. Despite marked aspiration there was no record of pneumonitis.
Y10 Every Zenker's diverticulum (A) has an associated HH. Noting the
damage LMC has caused at the lower esophageal attachments (C= belch), it is
surprising there are not more Zenker's diverticula as the force on the upper
attachments is the same. The so-called "cricopharyngeus spasm" is also the result
of local muscular avulsion. This patient had a persistently taunt LM (B). Only
a slight notch at the sphincter identifies the GE junction. These tubular HHs
are mistaken for esophagus lined with gastric mucosa and the less distensable
sphincter area is mistaken for a stricture.
Esophageal thickening must be interpreted cautiously. A 33% shortening of the length will cause a 50% increase in thickness.
Y11 Non effacement of sphincter with ruptured PEL. The sphincter-opening
vectors generated by LMC require resolution by the obliquely inserted PEL. Such
cases are often labeled "terminal esophagitis."
Y12 LM tension is the cause of gas/bloat. A sustained forceful LM contraction
shortens the esophagus 30% here as it draws the stomach through the hiatus (A),
opens the sphincter (B) and causes reflux (B-C). Severe bloat with severe subxiphoid
pressure "Making it difficult to breath," ++ pyrosis. A calcium channel blocker
(10 mg Nifedipine) relieved the gas/bloat symptoms within minutes. The effect
of such drugs on the CM is well known. S = sphincter.
Y13 Hiatal canal obstruction: "Trouble swallowing food." (A) A 12 mm
barium tablet was held up at the hiatus. (B) This small hiatal canal was the
cause of the obstruction. Note that usual ROA projection gives the false impression
that the GE junction is below the diaphragm.
Y14 A typical "bird beak" of achalasia. The "gastroesophageal polyp"(arrow)
identifies stomach above the narrowing.
Y15 " Longatonia" Persistent LM contraction keeps the sphincter open
after the Cannon-Dougherty receptor has been turned off by the dC maneuver.
Typically such patients complain of gas-bloat, reflux and its complications.
Y16 LM power and its effects: This sequence illustrates both the remarkable power of LM contraction and the equally remarkable elasticity of the PEL. In frame 1 there is just a nubbin of stomach above the diaphragm. The diaphragm is sharp. In frames 2-6, made during a belch, the stomach is relentlessly transtracted through the hiatus 7.6 cm as the esophagus shortens 35% of its length. The diaphragm itself is tented and the hiatus stretched to its widest extent (frame 5). LM contraction, as here, widens the hiatus thus affecting measurements of hiatal squeeze. Note the wide open sphincter and alpenhorn sign as the LM relaxes slightly in frame 7. Further relaxation closes the sphincter.
Y17 The captive bolus test: Three things are necessary to demonstrate
the captive bolus. 1) A normal p-wave, 2) Enough elongation of the PEL to form
a tent, and 3) A Valsalva maneuver to increase intraabdominal pressure, force
mesentery into the PEL and so obstruct the fundus. This is the optimal way of
studing swallowing against resistance
_____________________________________________________________________________
This patient has a somewhat elongated PEL making a captive bolus possible. Here he is doing a prolonged maximal Valsalva maneuver. It is easy to confirm many of the points made in this book, namely:
The p-wave stops at the sphincter, frames H-L, thus identifying both the sphincter
and its location.
Separate crinkles of esophageal mucosa (frames A,B) coalesce into a LER (frame
C and onward). This demonstrates why LERs occur where they do - the p-wave cannot
force them into the stomach because it goes no farther - and why they form:
there is 39 mm of redundant mucosal length that must go somewhere when the esophagus
shortens. The two surfaces of the ring account for 19 mm of this leaving 2 cm
for the elastisticity of the m. mucosae and slippage.
Mesentery crowding into the tented PEL acts, first as an obstruction inciting
a maximal p-wave [C] then as a piston, by crowding into the confined space of
the tented PEL. This force may be sufficient, as here, to eject barium back
through the contracted sphincter (frames J-T). Note the smaller size of the
CB in the later frames.
The sphincter, which is still activated, then stands in sharp contrast to the
CM (frames Q-T) which has relaxed in the wake of the p-wave as proved by its
easy distention by the jet of refluxing barium (frames N through R). A dial
gage measurement corrected for minification gives a sphincter length of 9.6
mm - far less than the 3.5-4.5 cm squeeze measured by manometry.
It should be noted that in this position - 4 cm above the diaphragm - there
is no possibility of the sphincter receiving auxiliary support from the angle
of His, pouting of the gastric mucosa, the valve of Guberoff, intraabdominal
pressure or any of the myriad of postulated mechanisms upon which surgical treatment
of reflux is based. In other cases, despite the considerable force which intraabdominal
pressure - acting like a thumb on a bulb syringe - exerts to force the contents
of the gastric sphere back through it, the sphincter maintained perfect competence
as long as the patient could sustain the Valsalva erffort.
By measuring the relationship of the LER to the diaphragm - stationary throughout the maneuver - we obtain an accurate index of the state of contraction of the LM. This shows that there is a rapid LM contraction while the p-wave is in the proximal esophagus. By the time the p-wave cone is in the distal esophagus, LM shortening is maximal. It peaks just as the p-wave enters the distal 10 cm of the esophagus when it abruptly relaxes.
___________________________________________________________________________
Maximal LM contraction of 2.8 cm + the height of the CB for a total of 7.7
cm occurs by frame H as the p-wave merges into the sphincter area. Thereafter
the LM relaxes. [Relaxation is not as noticeable because the transtracted stomach
holds it up.]
In frames G through M a very faint spherical soft tissue density surrounds
the CB. This is mesenteric fat inflating the PEL tent. High contrast or over-penetrated
films will not show it.
LM contraction causes fundic transtraction ("hiatus hernia") not increased intraabdominal pressure.
The long constriction in the PEL tent (B) is similar to the obstructive appearance
in achalasia. Note tram lines.
The reader may note that there is no difficulty in assigning state formulas
to each frame in swallowing against resistance.
(Y17-V) The same patient as in Y17A-T. Here the LM "knows" the barium tablet is caught by the LER and contracts in an effort to expand the esophagus and pass the tablet. In doing so it produces a HH that has an entirely different morphology than when elicited by the Valsalva maneuver. The presence of foreign bodies (tubes and transducers) in the esophagus may invalidate a manometric study.
X1A,B Signs of LMC. Male, 42 with life-long reflux, severe cheilitis,
wet spot on pillow in AM. Chronic sore throat, lost his teeth at 19. [A] Grade
2 reflux with de Carvalho test -RPO. [B]Cannon-Dougherty reflex was turned off
by dC test and patient turned to RAO provoking belch. Note trumpet configuration
of GE junction and loss of diaphragmatic sharpness. L. Gr. 2+ esophagitis. C.)
Such patients almost invariably have duodenitis.
X2 Gas-bloat with LMT. Male 56, pyrosis, bloat, nocturnal laryngospasm,
lost teeth at 26. An uneffaced sphincter serves for measurement of LMC. Note
shortening from A to B. The esophagus was constantly shortened. This causes
traction on the diaphragm producing the "gas-bloat" symptom. This patient also
has an enlarged lingual tonsil, gr. 2 vallecular sign and the p-wave was ineffectual.
X4 LMC after deCarvalho test. [A] Barium is dilute from the dC test
which has turned off the CD reflex.LMC lifts the perihiatal tissues [B] until
the sphincter yields [C]. This effect of LMC widens the hiatus, opens the sphincter
and translates the latter proximally. It is likely that these effects invalidate
many manometric measurements purporting to be of sphincter pressure. LMC is
one cause of the unpredictable transient complete loss of LESP. The other is
a hiccup.
X5 "Watermelon stomach": Once the PEL ruptures, the stomach remains
stationary while the hiatus slides down and up with inspiration [A] and expiration
[B]. This can cause stripe-like erosions of the gastric folds as they rub together.
X7 Ruptured PEL: A slight molar tooth shape results when the esophagus
invaginates the stomach. Note the poor effacement of the LES.
35xa Transient achalasia: may exhibit all of the classic signs yet have
them vanish during the course of the examination.[A] Overnight rentetion of
mucus globules with tramlines passing through the diaphragm and narrowed PEL
tent are typical of achalasia. [B] Gastric folds above diaphragm. [C] A Valsalva
maneuver demonstrates the hiatal transtract that is causing the obstruction.
It also dislodged the fundus from the hiatus and thereafter there was normal
peristalsis. This and "elevator esophagus" are typical for transient achalasia.
Z1 Post poliomyelitis: The gluteal musculature in this patient with
right sided paralysis shows what happens to aganglionic muscle.
Z2 The longitudinal muscle opens the sphincter. Obvious signs of LMC in [B] correlate with the open sphincter. Note gastric mucosa above the diaphragm. The repeated stretching of the PE attachments over a lifetime will elongate or even rupture them.
ILLUSTRATIONS TO BE COMPUTER GENERATED
CG-1
t1 Thickness of mucosa in distended organ.
t2 Thickness after lumen obliterating contraction
r1 Radius of distended organ - center to muscularis propria.
r2 Radius of contracted organ.
When the m. Propria contracts, it obliterates the lumen. While the x-sectional
area of the lumen goes to zero, the circumference of its lining mucosa can not.
It is necessarily thrown into folds.
CG-2
If the set of patients with both malady A and malady B is empty, ie., ~(A&B)
by the calculus of Boolean algebra equals ~A or ~B. That is, patients are spared
either one disorder or the other but not both. This appears to be the case in
practice: a patient can have achalasia or HH but not both. This implies that
either one disease prevents the other (which is obviously not true) or that
one causes the other. The latter can be true if A & B are the same disorder
- now diagnosed one way, now the other.
CG-2
In the 1990 to date Medline database, although there were many articles on HH and on BE, there were only 11 in which the abstract mentioned both keywords. After deleting the miscodes, only 4 of this 11 mentioned patients with both disorders. As both the BE and the HH populations are subsets of the GE reflux population, one would expect that at least 70% of the BE articles or 253 would deal with patients who had HHs as well, not the 4 that actually did so. Equally remarkable, 97% of the articles on HH failed to mention BE. The conclusion is that HHs are being diagnosed BE and vice versa or, more likely, they are the same thing.