Re: Botox vs Surgery or dilatation - & Joan

December 28, 2004

Joan - I agree with your concern about the doctor saying no scar

tissue would result from a Botox injection. I'm proof that it does.

During my lap heller myotomy, i had a perforation (The tissue of the

esophagus fused with the surrounding muscle and had a tear) that was

at the injection site of the botox (done a couple of years prior) and

ended up with an extended stay at the hospital.

I've learned to question the " no's " and " not possibles " with this

disease and prefer to follow up with my own research. Especially since

it seems like Achalasia is just a fly buzzing around on all these

doctors and researchers desks.

- please take everyone's concerns to heart when they say find

an EXPERIENCED doctor. It makes all the difference.

Take care

k

> Hi,

> The fact that your gastroenterologist says that Botox does not cause

> scarring makes me wonder!!! Perhaps he had a hidden agenda or just

has not

> heard about the damage that can be done. I attach a paper by Dr

Vaesi et al.

> who are world renound achalasia experts from Cleveland Clinic for you to

> read. It may be a bit technical, but I am sure the message will come

across.

>

> We are all nervous of surgery but if it is done while you are young

and your

> oesophagus is not stretched out too much there is a very good record of

> success. If it is done with a laparoscope it is a relatively minor

> procedure. You must find an experienced surgeon. Please tell us

where you

> are from and hopefully some one will know of a surgeon - there is also a

> list in the Website. You may have to travel to a neighbouring city

but it

> will be worth it,

> Please think long and hard before you take the road of dilatations

and botox

> before surgery. Read up as much as you can on the Web.

> Best wishes,

> Joan

>

> Practice Guidelines

>

> December 1999

> Volume 94, Number 12

> Pages 3406-3412

>

--------------------------------------------------------------------------

>

> Diagnosis and Management of Achalasia

>

> F. Vaezi, M.D., Ph.D.,a and E. Richter, M.D.,a

for the

> American College of Gastroenterology Practice Parameter Committee*

>

--------------------------------------------------------------------------

>

> aCenter for Swallowing and Esophageal Disorders, Department of

> Gastroenterology, The Cleveland Clinic Foundation, Cleveland, Ohio

>

--------------------------------------------------------------------------

>

> Preamble

>

> Guidelines for clinical practice are intended to suggest

preferable

> approaches to particular medical problems as established by

interpretation

> and collation of scientifically valid research, derived from extensive

> review of the published literature. When data are not available that

will

> withstand objective scrutiny, a recommendation may be made based on a

> consensus of experts. Guidelines are intended to apply to the clinical

> situation for all physicians without regard to specialty. Guidelines are

> intended to be flexible, not necessarily indicating the only acceptable

> approach, and should be distinguished from standards of care that are

> inflexible and rarely violated. Given the wide range of choices in any

> health care problem, the physician should select the course best

suited to

> the individual patient and the clinical situation presented. These

> guidelines are developed under the auspices of the American College of

> Gastroenterology and its Practice Parameters Committee. These

guidelines are

> also approved by the governing boards of the American

Gastroenterological

> Association and the American Society for Gastrointestinal Endoscopy.

Expert

> opinion is solicited from the outset for the document. Guidelines are

> reviewed in depth by the committee, with participation from experienced

> clinicians and others in related fields. The final recommendations

are based

> on the data available at the time of the production of the document

and may

> be updated with pertinent scientific developments at a later time. The

> following guidelines are intended for adults and not for pediatric

patients.

>

> Definition

>

> Achalasia is a primary esophageal motor disorder of unknown

etiology

> characterized manometrically by insufficient lower esophageal sphincter

> (LES) relaxation and loss of esophageal peristalsis, and

radiographically by

> aperistalsis, esophageal dilation, minimal LES opening with a

" bird-beak "

> appearance, and poor esophageal emptying of barium.

>

> Achalasia is a well-recognized primary esophageal motor

disorder of

> unknown etiology. Available data suggest hereditary, degenerative,

> autoimmune, and infectious factors as possible causes for achalasia, the

> latter two being the most commonly accepted possible etiologies (1, 2).

> Pathological changes found at autopsy or from myotomy specimens are

in the

> esophageal myenteric (Auerbach's) plexus with a prominent but patchy

> inflammatory response consisting of T-lymphocytes and variable

numbers of

> eosinophils and mast cells, loss of ganglion cells, and some degree of

> myenteric neural fibrosis (3). The end result of these inflammatory

changes

> is the selective loss of postganglionic inhibitory neurons, which

contain

> both nitric oxide and vasoactive intestinal polypeptide. The

postganglionic

> cholinergic neurons of the myenteric plexus are spared leading to

unopposed

> cholinergic stimulation (4). This produces high basal LES pressures,

and the

> loss of inhibitory input results in insufficient LES relaxation.

> Aperistalsis is related to the loss of the latency gradient along the

> esophageal body—a process mediated by nitric oxide.

>

> Diagnosis

>

> The diagnosis of achalasia should be suspected in anyone

complaining

> of dysphagia for solids and liquids with regurgitation of food and

saliva.

> The clinical suspicion should be confirmed by a barium esophagram

showing

> smooth tapering of the lower esophagus leading to the closed LES,

resembling

> a " bird's beak. " Esophageal manometry establishes the diagnosis showing

> esophageal aperistalsis and insufficient LES relaxation. All

patients should

> undergo upper endoscopy to exclude pseudoachalasia arising from a

tumor at

> the gastroesophageal junction.

>

> Most achalasia patients are symptomatic for years before seeking

> medical attention. The most common symptoms are dysphagia for solids and

> liquids, regurgitation, and chest pain. Although the dysphagia may

initially

> be for solids only, as many as 70-97% of patients with achalasia have

> dysphagia for both solids and liquids at presentation (2). This

contrasts

> with patients having strictures or ring whose dysphagia is limited to

> solids. Achalasia patients localize their dysphagia to the cervical or

> xiphoid areas. Over the years, patients learn to accommodate to their

> problem by using various maneuvers, including lifting the neck or

drinking

> carbonated beverages to help empty the esophagus. Regurgitation

becomes a

> problem with progression of the disease, especially when the esophagus

> begins to dilate. Regurgitation of bland, undigested, retained food, or

> accumulated saliva occurs in about 75% of achalasia patients (5). It

occurs

> most commonly in the recumbent position, awaking the patient from sleep

> because of coughing and choking. Chest pain or discomfort, located

in the

> xiphoid area, is experienced by nearly 40% of patients with

achalasia (5).

> It may mimic angina by location and character, but differs in not being

> aggravated by exercise or relieved by rest. About 60% of achalasia

patients

> may have some degree of weight loss at presentation because of poor

> esophageal emptying and decreased or modified food intake (6). However,

> weight loss is usually minimal, and some patients are obese.

Surprisingly,

> heartburn is reported by nearly 40% of achalasia patients. However,

it is

> not related to the reflux of acidic gastric contents, but most likely to

> production of lactic acid from retained food or exogenous ingested

acidic

> materials such as carbonated drinks.

>

> When the diagnosis of achalasia is suspected, a barium

esophagram with

> fluoroscopy is the single best diagnostic study. This test will

reveal loss

> of primary peristalsis in the distal two-thirds of the esophagus with

> to-and-fro movement in the supine position. In the upright position,

there

> will be poor emptying with retained food and saliva often producing a

> heterogeneous air-fluid level at the top of the barium column. Early

in the

> disease, the esophagus may be minimally dilated, but more chronic

disease is

> associated with sigmoid-like tortuosity and sometimes massive

dilation of

> the esophageal body. There is a smooth tapering of the lower esophagus

> leading to the closed LES, resembling a " bird's beak. " When the

esophagus is

> minimally dilated, this may be misinterpreted as a peptic stricture. The

> presence of an epiphrenic diverticulum suggests the diagnosis of

achalasia

> (7). Hiatal hernias are infrequent findings in patients with

achalasia with

> reported prevalence of 1-14% compared with 20-50% found in the general

> population (8). The presence of a hiatal hernia on barium esophagram may

> make the diagnosis of achalasia less likely, but it does not rule it

out,

> and does not change the management of these patients.

>

> Esophageal manometry is the key test for establishing the

diagnosis of

> achalasia (9) (Table 1). Because achalasia involves the smooth muscle

> portion of the esophagus, the manometric abnormalities are always

confined

> to the distal two-thirds of the esophagus. In the body of the esophagus,

> aperistalsis is always present. This means that all wet or dry

swallows are

> followed by simultaneous contractions that are classically identical

to each

> other (isobaric or mirror images). The contraction amplitudes are

typically

> low (10-40 mm Hg) and may be repetitive (2). The term " vigorous

achalasia "

> is sometimes used when there is aperistalsis with normal or even high

> amplitude contractions in the esophageal body (10). Patients with

vigorous

> achalasia usually have normal esophageal diameter on barium

esophagram, but

> otherwise do not differ from patients with classic achalasia. Some

> manometric abnormality of the LES is always present in patients with

> achalasia. The LES pressure is usually elevated but may be normal

(10-45 mm

> Hg) in up to 45% of patients; however, a low LES pressure is never

seen in

> patients with untreated achalasia. Abnormal LES relaxation is seen

in all

> achalasia patients. About 70-80% of patients with achalasia have

absent or

> incomplete LES relaxation with wet swallows. In the remaining

20-30%, the

> relaxations are complete to the gastric baseline but are of short

duration

> (usually <6 s) and functionally inadequate as assessed by barium and

nuclear

> emptying studies (11).

>

--------------------------------------------------------------------------

>

> Table 1. Radiographic and Manometric Features of Achalasia

>

> --------------------------------------------------------------------

>

> Barium esophagram

>

> Essential features:

> · " bird's beak " appearance of the LES with incomplete

opening

>

> · loss of primary peristalsis

>

> · delayed esophageal emptying

>

> Supportive features:

> · dilated or sigmoid-like esophagus

>

> · epiphrenic diverticula

>

> Manometry

>

> Essential features:

> · aperistalsis in distal of the esophagus

>

> · abnormal LES relaxation

>

> Supportive features:

> · hypertensive LES pressure

>

> · low amplitude esophageal contractions

>

--------------------------------------------------------------------------

>

> Pseudoachalasia results from a tumor at the esophagogastric

junction

> or in an adjacent area. These patients mimic classic achalasia

clinically

> and manometrically. The diagnosis should be suspected in patients with

> advanced age, shorter duration of symptoms, and marked weight loss

(5, 12).

> However, the predictive accuracy of this triad of symptoms and signs

is only

> 18% (13), possibly related to the low prevalence of the disease.

Although

> the gastric cardia may be assessed radiographically, its sensitivity

is poor

> in detecting tumors of the gastroesophageal junction causing

> pseudoachalasia. Therefore, all patients with suspected achalasia should

> undergo upper gastrointestinal endoscopy with close examination of the

> cardia and gastroesophageal junction. At endoscopy, the esophageal body

> usually appears dilated, atonic, and often tortuous with normal

appearing

> mucosa. Sometimes, the mucosa is reddened, friable, thickened, or even

> superficially ulcerated secondary to chronic stasis, pills, or Candida

> esophagitis. Retained secretions, usually saliva, liquids, or

sometimes food

> debris may be encountered. Patients with a markedly dilated

esophagus may

> need esophageal lavage or a clear liquid diet for several days before

> endoscopy to avoid aspiration and to allow adequate visualization of the

> esophagus. The LES region usually has a " rosette " appearance and remains

> closed with air insufflation; however, the endoscope will easily

traverse

> this area with gentle pressure allowing examination of the stomach. If

> excess pressure is required, the presence of pseudoachalasia should be

> highly suspected, the gastroesophageal junction and cardia closely

examined,

> and biopsies taken. Tumors of the gastroesophageal junction may be

missed

> endoscopically in up to 60% of patients with pseudoachalasia (5, 14).

> Endoscopic ultrasonography may prove useful in patients with a

nondiagnostic

> endoscopy and a high degree of clinical suspicion for

pseudoachalasia, but

> it is not recommended as a routine test in achalasia (15). The role of

> computed tomography scans is limited in the diagnosis of pseudoachalasia

> (14).

>

> The Management of Patients With Achalasia

>

> Although there is no cure for achalasia, the goal of treatment

should

> be relief of patient symptoms and improved esophageal emptying. The

two most

> effective treatment options are graded pneumatic dilation and surgical

> myotomy. For patients who are at high risk for pneumatic dilation or

> surgery, endoscopic injection of the LES with botulinum toxin or

> pharmacological treatment with nitrates or calcium channel blockers

may be

> acceptable alternatives.

>

> No treatment can restore the muscular activity to the denervated

> achalasiac esophagus. Esophageal aperistalsis and impaired LES

relaxation

> are rarely, if ever, reversed by any mode of therapy. Therefore, all the

> current treatment options for achalasia are limited to reducing the

pressure

> gradient across the LES, thus facilitating esophageal emptying by

gravity.

> This can be accomplished most effectively by pneumatic dilation and

surgical

> myotomy or less effectively by pharmacological agents injected

> endoscopically into the LES (botulinum toxin) or taken orally (calcium

> channel blockers and nitrates).

>

> Pneumatic dilation is the most effective nonsurgical treatment

option

> for patients with achalasia. All patients considered for pneumatic

dilation

> should be surgical candidates, since esophageal perforation may

result from

> the procedure. Pneumatic dilation uses air pressure to

intraluminally dilate

> and disrupt the circular muscle fibers of the LES. A variety of dilators

> were used in the past to treat patients with achalasia, including the

> Rider-Moeller, Sippy, Mosher, and Brown-McHardy dilators (1). Today, the

> most commonly used achalasia balloon dilators in the United States

are the

> nonradiopaque graded size polyethylene balloons (Microvasive Rigiflex

> dilators). A less frequently used balloon is the over-the-endoscope

Witzel

> dilator. Table 2 lists the recommended technique for performing

pneumatic

> dilation using these graded balloons. Pneumatic dilation should

always be

> carried out with sedation and under fluoroscopy. These dilators come in

> three different balloon diameters (3, 3.5, and 4 cm), and are positioned

> over a guidewire usually placed at endoscopy. The most important

aspect of

> an effective pneumatic dilation is accurate positioning of the balloon

> across the LES and effective obliteration of the balloon waist

visualized

> under fluoroscopy. The effectiveness of dilation does not depend on

balloon

> distention time so long as the balloon waist is appropriately

positioned and

> fully distended (17). After pneumatic dilation, all patients should

undergo

> a gastrograffin study followed by barium swallow to exclude esophageal

> perforation (18). This procedure is usually performed as an

outpatient with

> patients observed postprocedure for 4-6 h for chest pain and fever.

>

--------------------------------------------------------------------------

>

> Table 2. Recommended Technique for Pneumatic Dilation Using

the Graded

> Balloons*

>

> --------------------------------------------------------------------

>

> 1.

> Fasting for at least 12 h before procedure .

>

> 2.

> Esophageal lavage with a large-bore tube (if needed).

>

> 3.

> Sedation and endoscopy in RIGHT lateral position.

>

> 4.

> Guidewire positioned in stomach and balloon passed over the

> guidewire.

>

> 5.

> Initial dilation with 3-cm diameter balloon; subsequent

> progression to 3.5-cm and 4-cm balloons may be required at separate

> sessions.

>

> 6.

> Accurate placement of balloon across gastroesophageal

junction

> fluoroscopically.

>

> 7.

> Balloon distention to obliterate the waist, which usually

> requires 7-10 psi (this is the key to a successful dilation).

>

> 8.

> Gastrograffin study followed by barium swallow to exclude

> esophageal perforation.

>

> 9.

> Observation for 4 h for chest pain and fever.

>

> 10.

> Discharge with follow-up in 1 mo.

>

> --------------------------------------------------------------------

>

> *Before proceeding with pneumatic dilation, it is

important to

> ensure that a cardiothoracic surgeon is available in case of an

esophageal

> perforation.

>

--------------------------------------------------------------------------

>

> Studies to date indicate that by using the graded dilators,

> good-to-excellent relief of symptoms occurs in 50-93% of patients

(Table 3)

> (1). The clinical response improves in a graded fashion with

increasing size

> of the balloon diameter. Cumulatively, dilation with 3-, 3.5-, and 4-cm

> balloon diameters results in good-to-excellent symptomatic relief in

74%,

> 86%, and 90% of 359 treated patients, respectively (19, 20, 21, 22,

23, 24,

> 25, 26, 27, 28, 29, 30, 31) with an average follow-up of 1.6 yr

(range 0.1-6

> yr). Additionally, studies show that the rate of perforation may be

lower

> with the serial balloon dilation approach (25); therefore, most experts

> start with the smallest, 3-cm balloon, except in patients who have

had prior

> pneumatic dilations. The need for further dilation is based upon the

> persistence of symptoms usually assessed 4 weeks postprocedure or the

> recurrence of symptoms overtime.

>

--------------------------------------------------------------------------

>

> Table 3. Cumulative Effectiveness of the Graded Pneumatic

Dilators in

> Achalasia

>

> --------------------------------------------------------------------

>

> Reference

> Number of Patients

> Study Design

> Dilator (Size/cm)

> Objective Assessments

> % Sx Improvement

> Follow-up (yr) Mean (Range)

> Perforation (%)

>

> --------------------------------------------------------------------

>

> --------------------------------------------------------------------

>

> %LES Pressure

> Excellent/Good

>

> --------------------------------------------------------------------

>

> (19)

> 7

> Prospective

> 3

>

> 86

> 0.8(0.5-1)

> 0

>

> Gelfand (20)

> 24

> Prospective

> 3, 4

> 60,68

> 70,93

>

> 0

>

> Barkin (21)

> 50

> Prospective

> 3.5

>

> 90

> 1.3(0.1-3.4)

> 0

>

> Stark (22)

> 10

> Prospective

> 3.5

>

> 74

> 0.5

> 0

>

> Makela (23)

> 17

> Retrospective

> 3, 3.5, 4

>

> 50,75,75

> 0.5

> 5.9

>

> Levine (24)

> 62

> Retrospective

> 3, 3.5

>

> 85,88

>

> 0

>

> Kadakia (25)

> 29

> Prospective

> 3, 3.5, 4

> 67

> 62,79,93

> 4(0.3-6)

> 0

>

> Kim (26)

> 14

> Prospective

> 3, 3.5

> 39

> 75

> 0.3

>

> Lee (27)

> 28

> Prospective

> 3, 3.5, 4

>

> 7

>

> Abid (28)

> 36

> Retrospective

> 3.5, 4

>

> 50,75,75

> 0.5

> 5.9

>

> Levine (24)

> 62

> Retrospective

> 3, 3.5

>

> 85,88

>

> 0

>

> Kadakia (25)

> 29

> Prospective

> 3, 3.5, 4

> 67

> 62,79,93

> 4(0.3-6)

> 0

>

> Kim (26)

> 14

> Prospective

> 3, 3.5

> 39

> 75

> 0.3

>

> Lee (27)

> 28

> Prospective

> 3, 3.5, 4

>

> 7

>

> Abid (28)

> 36

> Retrospective

> 3.5, 4

>

> 88,89

> 2.3(1-4)

> 6.6

>

> Wehrmann (29)

> 40

> Retrospective

> 3, 3.5

> 42

> 89

> 2-5

> 2.5

>

> Lambroza (30)

> 27

> Retrospective

> 3

>

> 67

> 1.8(0.1-4.8)

> 0

>

> Bhatnagar (31)

> 15

> Prospective

> 3, 3.5

>

> 73,93

> 1.2(0.3-3)

> 0

>

> --------------------------------------------------------------------

>

> Total

> 359

>

> size 3

> 125/168=74%

> 1.6(0.1-6)yr

> 7/345=2%

>

> size 3.5

> 184/214=86%

>

> size 4

> 90/100=90%

>

--------------------------------------------------------------------------

>

> Overall, studies find a 2% cumulative perforation rate using the

> graded balloons, although some centers report higher perforation

rates (1).

> Patients with prompt recognition of perforation and surgical repair have

> comparable outcomes to those undergoing elective surgery (16); however,

> surgery for perforation is via an open thoracotomy approach. It is

important

> to note that the rate of perforation is variable and highly

dependent on the

> skill of the endoscopist. Physicians who do not perform pneumatic

dilations

> on a regular basis should consider referral to specialized centers with

> expertise in performing this procedure. Other less prevalent

complications

> of pneumatic dilation include gastroesophageal reflux (0-9%), aspiration

> pneumonia, gastrointestinal hemorrhage, and esophageal hematoma (32).

> Patients with a dilated and tortuous esophagus, esophageal

diverticula, or

> previous surgery at the gastroesophageal junction may be at an increased

> risk for esophageal perforation with balloon dilation and should be

> considered for surgical myotomy as the first treatment option.

>

> Traditionally, symptom improvement is used to assess the

success of

> pneumatic dilation. However, a recent study suggests that subjective and

> objective parameters of improvement are discordant in about 30% of

patients

> postpneumatic dilation (33), suggesting that subjective improvement

alone

> may give a false sense of success in those with less than optimal

relief of

> their distal esophageal obstruction. Objective tests to better assess

> improvement after pneumatic dilation include manometry (LES pressure

<10 mm

> Hg), esophageal scintigraphy, and the timed barium esophagram (34,

35, 36).

> The adjunctive use of these tests may help to improve the long-term

success

> of pneumatic dilation, but this premise is still speculative.

>

> Surgical myotomy for achalasia involves performing an anterior

myotomy

> across the LES (Heller's myotomy) usually associated with an antireflux

> procedure (loose Nissen, incomplete Toupet, or Dor fundoplication).

In the

> past, the myotomy was done by an open procedure through a thoracic or

> abdominal incision. The hospital stay was 7-10 days with a substantial

> postoperative recovery period. The abdominal approach limits

extension of

> the myotomy proximally, with usually only a 1- to 2-cm distal

myotomy onto

> the stomach to decrease the frequency of postprocedure gastroesophageal

> reflux. The transthoracic approach allows a longer proximal

extension of the

> myotomy to the level of the major pulmonary vessels, but the extension

> distally onto the stomach may be limited. The results from published

> studies, using either the abdominal or thoracic approaches, show

> good-to-excellent symptom improvement in 83% of 2660 patients undergoing

> myotomy through the abdominal approach and in 83% of 1210 patients

who had a

> transthoracic esophagomyotomy with a mean follow-up of ~7 yr (1).

The main

> late complication of a Heller's myotomy is gastroesophageal reflux

disease.

> The cumulative rates of heartburn and reflux disease reported in the

studies

> are 22% for the abdominal and 10% for the transthoracic approach

(1). The

> operative mortality for both procedures is very low (0.2% vs 1%),

with most

> studies reporting no deaths directly related to the operation.

>

> The advent of minimally invasive surgery and laparoscopic

myotomy has

> resulted in shorter patient hospital stay (2 days), reduced

morbidity, and

> quicker return to daily activity, making the procedure an attractive

initial

> management option for healthy patients with achalasia. Studies show that

> laparoscopic cardiomyotomy has a cumulative good-to-excellent clinical

> response rate of 94% in 254 treated patients (Table 4) (37, 38, 39,

40, 41,

> 42, 43, 44, 45, 46, 47, 48). However, long-term outcome of patients

> undergoing this procedure is unknown with current studies having a

> cumulative mean follow-up time of only 1 yr (range 0.1-4 yr). Before

> laparoscopic surgery, the most common indication for myotomy was the

patient

> with recurrent symptoms after graded pneumatic dilations. However,

> laparoscopic surgery is increasingly performed as initial therapy for

> healthy patients, if a skillful surgeon is available. The cumulative

rate of

> heartburn and reflux disease after laparoscopic myotomy is

approximately 11%

> (1). Patients with megaesophagus (esophageal diameter >8 cm) or

those with

> low LES pressure and persistent symptoms typically do not do well with

> either pneumatic dilation or surgical myotomy and may require an

> esophagectomy with a gastric pullup or colon interposition.

>

--------------------------------------------------------------------------

>

> Table 4. Cumulative Effectiveness of the Laparoscopic Surgical

Myotomy

> in Achalasia

>

> --------------------------------------------------------------------

>

> Reference

> Number of Patients

> Study Design

> Antireflux Procedure

> Objective Assessments

> % Sx Improvement

> Follow-up (yr) Mean (Range)

> % Complications

>

> --------------------------------------------------------------------

>

> --------------------------------------------------------------------

>

> --------------------------------------------------------------------

>

> %LES Pressure

> Excellent/Good

> GERD

> Mortality

>

> --------------------------------------------------------------------

>

> ti (37)

> 25

> Retrospective

> yes

> 61

> 96

> 1(1-2)

>

> 0

>

> Ancona (38)

> 17

> Retrospective

> yes

> 72

> 100

> 0.7

> 6

> 0

>

> Esposito (39)

> 8

> Retrospective

> yes

>

> 100

> 0.9(0.8-1)

>

> 0

>

> Raiser (40)

> 29

> Prospective

> yes

>

> 90

> 1.2(1-2)

> 27

> 0

>

> Morino (41)

> 18

> Prospective

> yes

> 60

> 100

> 0.7(0.2-2)

> 6

> 0

>

> Anselmino (42)

> 43

> Prospective

> yes

> 68

> 95

> 1(0.3-4)

> 6

> 0

>

> Delgado (43)

> 12

> Prospective

> yes

> 42

> 83

> 0.3(0.1-1)

> 0

>

> Slim (44)

> 8

> Retrospective

> yes

> 67

> 100

> 1

>

> 0

>

> Bonovina (45)

> 33

> Retrospective

> yes

> 61

> 97

> 1(0.3-2)

>

> on (46)

> 9

> Retrospective

> no

>

> 88

> 1.1(1-1.9)

> 13

> 0

>

> Swanstrom (47)

> 12

> Retrospective

> yes

> 42

> 100

> 1.3

> 16

> 0

>

> Hunter (48)

> 40

> Retrospective

> yes

>

> 90

> 1

>

> 0

>

> --------------------------------------------------------------------

>

> Total

> 254

>

> 59%

> 240/254 = 94%

> 1(0.1-4) yr

> 16/141=11%

> 0%

>

> --------------------------------------------------------------------

>

> GERD = gastroesophageal reflux disease.

>

--------------------------------------------------------------------------

>

> Endoscopic injection of botulinum toxin, type A, into the LES

is the

> most recent treatment alternative for achalasia. Botulinum toxin acts by

> inhibiting the calcium-dependent release of acetylcholine from nerve

> terminals, thereby counterbalancing the effect of the selective loss of

> inhibitory neurotransmitters in achalasia (49, 50). It is commercially

> supplied as lyophilized powder (Oculinum; Allergan, Irvin, CA) in vials

> containing 100 units each. The powder must be diluted with normal saline

> (5-10 ml) and used within 4 h of reconstitution without agitation of the

> solution because of the toxin's instability at room temperature.

Botulinum

> toxin is injected endoscopically via a 5-mm sclerotherapy needle

into the

> LES region as identified by a " puckered " appearance just above the

> gastroesophageal junction. Aliquots equaling 20-25 units of the

toxin are

> injected into each of four quadrants for a total of 80-100 units.

>

> Available data indicate that botulinum toxin is effective in

relieving

> symptoms initially in about 85% of patients (1). However, symptoms

recur in

> more than 50% of patients within 6 months possibly because of

regeneration

> of the affected receptors (49). Older patients (>60 yr) and those with

> vigorous achalasia, defined as esophageal amplitude >40 mm Hg, are more

> likely to have a sustained response (up to 1.5 yr) to botulinum toxin

> injection (51). In those responding to the first injection, 76% will

respond

> to a second botulinum toxin injection with decreasing response to

further

> injections, usually from antibody formation to this foreign protein.

Less

> than 20% of patients failing to respond to the first injection will

respond

> to a second injection of botulinum toxin. Studies have shown that

botulinum

> toxin is less effective than pneumatic dilation long term (52, 53).

> Additionally, some reports indicate that cardiomyotomy may be more

difficult

> and less effective in patients who were previously treated with repeated

> botulinum toxin injections, possibly because of submucosal scar

formation in

> the esophagus at the site of injection (54). Finally, the long-term

safety

> of repeated injections of botulinum toxin in achalasia patients is

unknown.

> Therefore, botulinum toxin injection should be reserved for elderly

patients

> or patients who are at high surgical risk or refuse pneumatic

dilation and

> surgical myotomy.

>

> Calcium channel blockers and long-acting nitrates are effective in

> reducing LES pressure and temporally relieving dysphagia, but do not

improve

> LES relaxation or improve peristalsis. Both agents are used

sublingually by

> opening the capsule and placing the contents under the tongue 15-45 min

> before meals with doses ranging from 10-30 mg for nifedipine and

5-20 mg for

> sublingual isosorbide dinitrate (55, 56). These drugs decrease LES

pressure

> by approximately 50% with the long-acting nitrates having a shorter

time to

> maximum effect (3-27 min) compared to sublingual nifedipine (30-120

min).

> Overall, calcium channel blockers improve patient symptoms by 0-75%,

whereas

> sublingual nitrates result in symptom improvement in 53-87% of

patients with

> achalasia (1). The clinical response to these pharmacological agents is

> short acting; they usually do not provide complete symptom relief, and

> efficacy decreases with time. Side effects such as headache,

hypotension,

> and pedal edema are common limiting problems. Given these limitations,

> calcium channel blockers and nitrates are recommended only for

patients who

> are very early in their disease with a nondilated esophagus, for

symptomatic

> patients who are not candidates for pneumatic dilation or surgical

myotomy,

> or for those who refuse invasive therapy and fail botulinum toxin

> injections.

>

> A suggested treatment algorithm for patients with achalasia is

shown

> in Figure 1. Symptomatic patients with achalasia who are good surgical

> candidates should be given the option of either graded pneumatic

dilation or

> laparoscopic cardiomyotomy. The choice between the two procedures

depends on

> institutional preference and experience. In patients unresponsive to

graded

> pneumatic dilation, laparoscopic myotomy should be performed. In myotomy

> failures, repeat pneumatic dilation can be attempted. In patients

who are

> poor candidates for surgery, initial treatment with botulinum toxin is

> currently the preferred approach. Nifedipine or isordil may prove to be

> beneficial in those unresponsive to botulinum toxin. Those with a

> megaesophagus (sigmoid esophagus and diameter >8 cm), or those with

low LES

> pressure with persistent symptoms may require esophagectomy.

>

--------------------------------------------------------------------------

>

--------------------------------------------------------------------------

>

> Figure 1. Suggested treatment algorithm for patients with

achalasia.

> *Initial dilation with a 3-cm balloon followed by 3.5-cm and

then 4-cm

> balloons in the nonresponders.

>

--------------------------------------------------------------------------

>

> References

>

> 1. Vaezi MF, Richter JE. Current therapies for achalasia:

Comparison

> and efficacy. J Clin Gastroenterol 1998;27:21-35.

>

> 2. Birgisson S, Richter JE. Achalasia: What's new in diagnosis and

> treatment? Dig Dis 1997;15:1-27.

>

> 3. Goldblum JR, Whyte RI, Orringer MB, et al. Achalasia: A

morphologic

> study of 42 resected specimens. Am J Surg Pathol 1994;18:327-37.

>

> 4. Holloway RH, Dodds WJ, Helm JF, et al. Integrity of cholinergic

> innervation to the lower esophageal sphincter in achalasia.

Gastroenterology

> 1986;90:924-9.

>

> 5. Rozeman RW Jr, Achkar E. Features distinguishing secondary

> achalasia from primary achalasia. Am J Gastroenterol 1990;85:1327-30.

>

> 6. Wong RKH, Maydonovitch CL. Achalasia. In: Castell DO, ed. The

> esophagus, 2nd ed. New York: Little Brown, 1995:219-47.

>

> 7. Debas HT, Payne WS, Cameron AJ, et al. Physiopathology of

the lower

> esophageal diverticulum and its complications for treatment. Surg

Gynecol

> Obstet 1980;151:593-600.

>

> 8. Ott DJ, Hodge RG, Chen MYM. Achalasia associated with hiatal

> hernia: Prevalence and potential implications. Abdom Imaging

1993;18:7-9.

>

> 9. Ergun GA, Kahrilas PJ. Clinical application of esophageal

manometry

> and pH monitoring. Am J Gastroenterol 1996;91:1077-89.

>

> 10. Goldenberg SP, Burrell M, Fette GG, et al. Classic and

vigorous

> achalasia: A comparison of manometric, radiographic, and clinical

findings.

> Gastroenterology 1991;101:743-8.

>

> 11. Katz PO, Richter JE, Cowan R, et al. Apparent complete lower

> esophageal sphincter relaxation in achalasia. Gastroenterology

> 1986;90:978-83.

>

> 12. Tucker HJ, Snap WJ Jr, Cohen S. Achalasia secondary to

carcinoma:

> Manometric and clinical features. Ann Intern Med 1978;89:315-8.

>

> 13. Sandler RS, Bozymski EM, Orlando RC. Failure of clinical

criteria

> to distinguish between primary achalasia and achalasia secondary to

tumor.

> Dig Dis Sci 1982;27:209-13.

>

> 14. Tracey JP, Traube M. Difficulties in the diagnosis of

> pseudoachalasia. Am J Gastroenterol 1994;89:2014-8.

>

> 15. Van Dam J, Falk GW, Sivak MV, et al. Endosonographic

evaluation of

> the patient with achalasia: Appearance of the esophagus using the

> echoendoscope. Endoscopy 1995;27:185-90.

>

> 16. Schwartz HM, Cahow CE, Traube M. Outcome after perforation

> sustained during pneumatic dilation for achalasia. Dig Dis Sci

> 1993;38:1409-13.

>

> 17. Khan AA, Shah WH, Alam A, et al. Pneumatic balloon dilation in

> achalasia: A prospective comparison of balloon distention time. Am J

> Gastroenterol 1998;93:1064-7.

>

> 18. Ott DJ, Richter JE, Wu WC, et al. Radiographic evaluation

of the

> achalasia esophagus immediately after pneumatic dilation. Gastrointest

> Radiol 1987;32:962-7.

>

> 19. J, Buckton GK, JR. Balloon dilatation in

achalasia: A

> new dilator. Gut 1986;27:986-9.

>

> 20. Gelfand MD, Kozarek RA. An experience with polyethylene

balloon

> for pneumatic dilation for achalasia. Am J Gastroenterol 1989;84:924-7.

>

> 21. Barkin JS, Guelrud M, Reiner DK, et al. Forceful balloon

dilation:

> An outpatient procedure for achalasia. Gastrointest Endosc

1990;36:123-6.

>

> 22. Stark GA, Castell DO, Richter JE, et al. Prospective

randomized

> comparison of Brown-McHardy and Microvasive balloon dilators in

treatment of

> achalasia. Am J Gastroenterol 1990;85:1322-6.

>

> 23. Makela J, Kiviniemi H, Laitinen S. Heller's cardiomyotomy

compared

> with pneumatic dilation for the treatment of oesophageal achalasia.

Eur J

> Surg 1991;157:411-4.

>

> 24. Levine ML, Moskowitz GW, Dorf BS, et al. Pneumatic dilation in

> patients with achalasia with a modified Gruntzig dilator (Levine) under

> direct endoscopic control: Results after 5 years. Am J Gastroenterol

> 1991;86:1581-4.

>

> 25. Kadakia SC, Wong RKH. Graded pneumatic dilation using Rigiflex

> achalasia dilators in patients with primary esophageal achalasia. Am J

> Gastroenterol 1993;88:34-8.

>

> 26. Kim CH, Cameron AJ, Hsu JJ, et al. Achalasia: Prospective

> evaluation of relationship between lower esophageal sphincter pressure,

> esophageal transit, and esophageal diameter and symptoms in response to

> pneumatic dilation. Mayo Clin Proc 1993;68:1067-73.

>

> 27. Lee JD, Cecil BD, Brown PE, et al. The Cohen test does not

predict

> outcome in achalasia after pneumatic dilation. Gastro[zhy]intest Endosc

> 1993;39:157-60.

>

> 28. Abid S, Champion G, Richter JE, et al. Treatment of

achalasia: The

> best of both worlds. Am J Gastroenterol 1994;89:979-85.

>

> 29. Wehrmann T, i V, Jung M, et al. Pneumatic dilation in

> achalasia with a low-compliance balloon: Results of a 5-year prospective

> evaluation. Gastrointest Endosc 1995;42:31-6.

>

> 30. Lambroza A, Schuman RW. Pneumatic dilation for achalasia

without

> fluoroscopic guidance: Safety and efficacy. Am J Gastroenterol

> 1995;90:1226-9.

>

> 31. Bhatnagar MS, Nanivadekar SA, Sawant P, et al. Achalasia

cardia

> dilatation using polyethylene balloon (Rigiflex) dilators. Indian J

> Gastroenterol 1996;15:49-51.

>

> 32. Reynolds JC, Parkman HP. Achalasia. Gastroenterol Clin

North Am

> 1989;18:223-55.

>

> 33. Vaezi MF, Baker ME, Richter JE. Assessment of esophageal

emptying

> post-pneumatic dilation: Use of timed-barium esophagram. Am J

Gastroenterol

> 1999;94:1802-7.

>

> 34. Eckhardt VF, Aignherr C, Bernhard G. Predictors of outcome in

> patients with achalasia treated by pneumatic dilation. Gastroenterology

> 1992;103:1732-8.

>

> 35. Levine ML, Dorf BS, Moskowitz GW, et al. Pneumatic dilation in

> achalasia under endoscopic guidance: Correlation pre- and

post-dilation by

> radionuclide scintiscan. Am J Gastroenterol 1987;82:311-4.

>

> 36. de Oliveira JM, Birgisson S, Doinoff C, et al. Timed barium

> swallow: A simple technique for evaluating esophageal emptying in

patients

> with achalasia. AJR 1997;169:473-9.

>

> 37. ti R, Fumagalli U, Bonavina L, et al. Laparoscopic

approach to

> esophageal achalasia. Am J Surg 1995;169:424-7.

>

> 38. Ancona E, Anselmino M, Zaninotto G, et al. Esophageal

achalasia:

> Laparoscopic versus conventional open Heller-Dor operation. Am J Surg

> 1995;170:265-70.

>

> 39. Esposito PS, Sosa JL, Sleeman D, et al. Laparoscopic

management of

> achalasia. Am Surgeon 1997;63:221-3.

>

> 40. Raiser F, Perdikis G, Hinder RA, et al. Heller myotomy via

minimal

> access surgery: An evaluation of anti-reflux procedures. Arch Surg

> 1996;131:593-8.

>

> 41. Morino M, Rebecchi F, Festa V, et al. Laparoscopic Heller

> cardiomyotomy with intraoperative manometry in the management of

oesophageal

> achalasia. Int Surg 1995;80:332-5.

>

> 42. Anselmino M, Zaninotto G, Costantini M, et al. One-year

follow-up

> after laparoscopic Heller-Dor operation for esophageal achalasia. Surg

> Endosc 1997;11:3-7.

>

> 43. Delgado F, Bolufer JM, ez-Abad M, et al. Laparoscopic

> treatment of esophageal achalasia. Surg Lap Endosc 1996;2:83-90.

>

> 44. Slim K, Pezet D, Chipponi J, et al. Laparoscopic myotomy for

> primary esophageal achalasia: Prospective evaluation.

> Hepato-Gastroenterology 1997;44:11-5.

>

> 45. Bonovina L, ti R, Segalin A, et al. Laparoscopic

Heller-Dor

> operation for the treatment of oesophageal achalasia: Technique and

early

> results. Ann Chir Gynaecol 1995;84:165-8.

>

> 46. on GSM, Lloyd DM, Wicks ACB, et al. Laparoscopic

Heller's

> cardiomyotomy without an anti-reflux procedure. Br J Surg 1995;82:957-9.

>

> 47. Swanstrom LL, Pennings J. Laparoscopic esophagomyotomy for

> achalasia. Surg Endosc 1995;9:286-92.

>

> 48. Hunter JG, Trus TL, Branum GD, et al. Laparoscopic Heller

myotomy

> and fundoplication for achalasia. Ann Surg 1997;225:655-65.

>

> 49. Tsui JKS. Botulinum toxin as a therapeutic agent.

Pharmacol Ther

> 1996;72:13-24.

>

> 50. Pasricha PJ, Ravich WJ, Henrix TR, et al. Intrasphincteric

> botulinum toxin for the treatment of achalasia. N Engl J Med

1995;322:774-8.

>

> 51. Pasricha PJ, Rai R, Ravich WJ, et al. Botulinum toxin for

> achalasia: Long-term outcome and predictors of response.

Gastroenterology

> 1996;110:1410-5.

>

> 52. Vaezi MF, Richter JE, Wilcox M, et al. Botulinum toxin versus

> pneumatic dilation in the treatment of achalasia: A randomized

trial. Gut

> 1999;44:231-9.

>

> 53. Prakash C, Freedland KE, Chan MF, et al. Botulinum toxin

> injections for achalasia symptoms can approximate the short-term

efficacy of

> a single pneumatic dilation: A survival analysis approach. Am J

> Gastroenterol 1999;94:328-33.

>

> 54. Gordon JMI, Eager ELY. Prospective study of esophageal

botulinum

> toxin injection in high-risk achalasia patients. Am J Gastroenterol

> 1997;92:1812-7.

>

> 55. Gelfond M, Rozen P, Gilat T. Isosorbide dinitrate and

nifedipine

> treatment of achalasia: A clinical, manometric and radionuclide

evaluation.

> Gastroenterology 1982;83:963-9.

>

> 56. Bortolotti M, Coccia G, Brunelli F, et al. Isosorbide

dinitrate or

> nifedipine: Which is preferable in the medical therapy of achalasia?

Ital J

> Gastroenterol 1994;26:379-82.

>

> Appendix

>

> Ad Hoc Committee on Practice Parameters:

>

> Nimish Vakil, M.D., F.A.C.G., Chair

> Freda L. Arlow, M.D., F.A.C.G.

> Alan N. Barkun, M.D.

> W. , Jr., M.D., F.A.C.G.

> J. Caddick, M.D.

> H. Caldwell, M.D.

> D. Carey, M.D., F.A.C.G.

> P. Cheney, M.D., F.A.C.G.

> Sita S. Chokhavatia, M.D.

> R. DeVault, M.D., F.A.C.G.

> Francis A. Farraye, M.D., F.A.C.G.

> Kris V. Kowdley, M.D.

> Simon K. Lo, M.D., F.A.C.G.

> S. Pratt, M.D.

> Dawn Provenzale, M.D., F.A.C.G.

> M. Simon, M.D., F.A.C.G.

> Amy M. Tsuchida, M.D., F.A.C.G.

> R. Viggiano, M.D., F.A.C.G.

> J. Waring, M.D., F.A.C.G.

> M. Wo, M.D.

> Marc J. Zuckerman, M.D., F.A.C.G.

>

--------------------------------------------------------------------------

>

> Reprint requests and correspondence: F. Vaezi, M.D.,

Ph.D.,

> Center for Swallowing and Esophageal Disorders, Department of

> Gastroenterology, The Cleveland Clinic Foundation, 9500 Euclid Avenue,

> Cleveland, OH 44195.

>

> Received Aug. 23, 1999; accepted Aug. 23, 1999.

>

--------------------------------------------------------------------------

>

> Published by Elsevier Science Inc.

>

> You are not the only one

>

> >

> > Hi :

> > I was diagnosed with Achalasia last June after five years of

> > swallowing problems. No doctor could find out what was wrong with

> > me until my last upper GI and mamometry tests in June. They were

> > treating me for GERD. By last August I was only consuming liquids

> > and Ensure. I lost a great deal of weight. I had Botox in

> > September. The doctor found that I had a bad fungus infection in my

> > esophagus. That might have been the reason why I didn't have as

> > much improvement in my esophagus as they hoped for. It did improve

> > somewhat as I was able to eat some soft foods. I aked my GI about

> > scar tissue when using Botox. He said no. Recently I caught a bad

> > cold and now I'm back to square one. My doctor is talking about

> > dilation or another Botox next September. I know Myotomy is a more

> > permanent treatment, but frankly I find it very scary and I'm trying

> > to build up my courage to have it done. I did see a surgeon, but he

> > hadn't done very many myotomies so I didn't have the surgery. I

> > know how you feel when you see other people eating and enjoying

> > their food, as I feel the same way. I also felt alone, but this is

> > a great group. You are not alone. I wish there was a better

> > treatment that is permanent. Maybe one day. I find that working

> > helps me cope better with my Achalasia. I don't think about it when

> > I work. Please ask any questions you have and I hope we can help

> > you.

> >

December 29, 2004

said : 'Especially since it seems like Achalasia is just a fly buzzing

around on all these

doctors and researchers desks.'

How true ! A nuisance but not enough irritation to be taken seriously

! Time we insisted on some in-depth research and recognition.

Joan

You are not the only one