Achalasia

4.
With respect to achalasia:
a) Describe the anatomy of the oesophagogastric junction. (30)
Anatomic Landmarks:
The oesophagogastric junction is the distal end of the abdominal

oesophagus. The abdominal oesophagus begins as the oesophagus
passes through the diaphragm at the level of the body of the 11th vertebra.
It marks the anatomical transition from oesophagus to stomach and is
made up of the lower oesphageal sphincter (LES). Unlike the Upper
Oesophageal sphincter which is demarcated by the crycopharyngeas
muscle the LES can be more difficult to discern.
There are four anatomic points that mark the LES:
1. Two of which can be noted on endoscopy:
a. The Z-Line – the squamocolumnar junction (provided the patient

does not have metaplasia/barrett’s oesophagus)
b. The beginning of rugae/normal gastric folds
2. Two of which can be seen external to the oesophagus:
a. The Collar of Helvetius/Loop of Willis marks where the circular

fibres of the oeophagus join the oblique fibres of the stomach
b. The Gastro-oesophageal fat pad
Arterial Supply
Arterial supply to the oesophago-gastric junction comes from the paired

inferior phrenic arteries, which is the first branches off the abdominal aorta
and the left gastric artery, a branch off the celiac axis.
Venous Drainage
Venous drainage parrallels the arterial supply, with the inferior phrenic veins
draining into the IVC and the left gastric vein and short gastric veins draining
into the portal circulation. The dual venous drainage into both the systemic as
well as portal circulation is the patho-physiological mechanism by which
oesophageal varices develop in the situation of portal hypertension.
Lymphatics
The lymphatic drainage of the abdominal oesohpagus and oesophago-gastric

junction tends to be to nodes around the left gastric artery and celiac axis
nodes.
Innervation
The distal thoracic oesophagus receives sympathetic fibres from the greater
splanchnic nerve and the abdominal oesophagus from sympathetic fibres
arising posteriorly around the left gastric artery. The left and right vagus nerve
come to lie anterior and posterior, respectively in relation to the oesophagus
providing it with parasympathetic innervation.
b) Outline the steps in confirming the diagnosis. (30)
Diagnosis of achalasia includes taking a careful history and clinical examination

of the patient. A dynamic barium swallow usually confirms suspicion, while
oesophageal manometry is the gold-standard in diagnostic evaluation. The role of
endoscopy is to exclude distal carcinoma and oesophagitis and provides little
else in the diagnostic workup (endoscopy does have a role to play in the
therapeutic management).
History & Clinical Examination
A strong suspicion of the diagnosis can usually be made on taking a careful

history from the patient. The classic triad of presenting symptoms consists of
dysphagia, regurgitation, and weight loss.
Dysphagia usually begins with liquids and progresses to solids. Most patients eat
slowly and use large volumes of water to help wash the food down into the
stomach. As the water builds up pressure, retrosternal chest pain is experienced
and can be quite severe until the LES opens and is quickly relieving. The
dysphagia worsens over years, and patients adapt their lifestyle to accommodate.
Regurgitation of undigested, foul-smelling foods is common, and with progressive

disease, aspiration can become life-threatening. Pneumonia, lung abscess, and
bronchiectasis often result from long-standing achalasia.
Patients often do not seek medical attention until their symptoms are quite
advanced and will present with marked distension of the esophagus. They maybe
cachectic and have features of Chronic Lung disease secondary to chronic
aspiration and infection.
Barium Swallow
The diagnosis of achalasia is usually made from a Barium Swallow, which

provides both an oesophagogram and a motility study.
Findings:
1. May vary, depending on the stage of the disease

2. A dilated esophagus with a distal narrowing referred to as the classic
“bird's beak” appearance of the barium-filled esophagus
3. Sphincter spasm and delayed emptying through the LES, as well as
dilation of the esophageal body, are observed
4. A lack of peristaltic waves in the body and failure of relaxation of the LES
are observed.
5. Lack of a gastric air bubble is a common finding on the upright portion of
the esophagram and is a result of the tight LES not allowing air to pass
easily into the stomach
6. In the more advanced stage of disease, massive esophageal dilation,
tortuosity, and a sigmoidal esophagus (megaesophagus)
Oesophageal Manometry
Manometry is the gold standard test for diagnosis and will help eliminate other
potential esophageal motility disorders.
In typical achalasia, the manometry tracings show five classic findings: two
abnormalities of the LES and three of the esophageal body:
1. The LES will be hypertensive with pressures usually above 35 mm Hg

2. The LES will fail to relax with deglutition
3. The body of the esophagus will have a pressure above baseline
(pressurization of the esophagus) from incomplete air evacuation
4. Mirrored contractions with no evidence of progressive peristalsis
5. Low-amplitude waveforms indicating a lack of muscular tone (Fig. 41-27).
These five findings provide a diagnosis of achalasia.
Endoscopy
An endoscopy is performed to evaluate the mucosa for evidence of esophagitis

or cancer. It otherwise contributes little to the diagnosis of achalasia.
c) Discuss the management options. (40)
The management options include both surgical and nonsurgical treatments; all
are directed toward relieving the obstruction caused by the LES. Apart from
oesophagectomy none of these treatments addresses the issue of decreased
motility in the esophageal body therefore all are palliative treatments.
Nonsurgical treatment options include medications and endoscopic interventions.
Medical therapy:
1. In the early stage of the disease, medical treatment with sublingual

nitroglycerin, nitrates, or calcium channel blockers may offer hours of relief
of chest pressure before or after a meal.
Endoscopic therapy:
1. Bougie dilation up to 54 French may offer several months of relief but

requires repeated dilations to be sustainable.
2. Injections of botulinum toxin (Botox) directly into the LES blocks
acetylcholine release, preventing smooth muscle contraction, and
effectively relaxes the LES. With repeated treatments, Botox may offer
symptomatic relief for years, but symptoms recur more than 50% of the
time within 6 months.
3. Balloon Dilation with a Gruntzig-type (volume-limited, pressure-control)
balloon is effective in 60% of patients and has a risk for perforation less
than 4%; however, perforation is life-threatening and must be weighed
carefully in otherwise unhealthy patients.
Medical and endoscopic therapy offers a short term solution with high recurrence
rates. In addition the risk of perforation with balloon dilatation is higher than that
for surgical oesophagomyotomy (4% vs 1%) which would carry significant
mortalitiy and morbidity in a chronically ill, cachectic patient with possible poor
respiratory reserve. In addition it must be noted that previous balloon dilatation
may have deleterious consequences for subsequent attempts at surgical
oesophagomyotomy.
Surgical treatments:
Surgical esophagomyotomy offers superior results and is less traumatic than

balloon dilation.
The modified Heller myotomy that was described originally by a laparotomy in

1913. A variety of changes have been made to the originally described operation;
however, the modified laparoscopic Heller myotomy is now the operation of
choice. It is done open or with video or robotic assistance.
The decision to perform an antireflux procedure remains controversial. Most

patients post-Hellers will experience some symptoms of reflux. The addition of a
partial antireflux procedure, such as a Toupet or Dor fundoplication, will restore a
barrier to reflux and decrease postoperative symptoms. This is especially true in
patients whose esophageal clearance is greatly impaired.
The indications for an oesophagectomy include:

1. Any symptomatic patient with a tortuous esophagus (megaesophagus) or
sigmoid esophagus
2. Failure of more than one myotomy
3. An undilatable reflux stricture.
Fewer than 60% of patients undergoing repeat myotomy benefit from surgery,
and fundoplication for treatment of reflux strictures is even more dismal. In
addition to definitively treating the end-stage achalasia, esophageal resection
also eliminates the risk for carcinoma.
A transhiatal esophagectomy with or without preservation of the vagus nerve

offers a good long-term result.
Results of medical, interventional, and surgical procedures all point to surgery as

being the safest and most effective treatment of achalasia.
When comparing balloon dilation to Botox injections, remission of symptoms

occurred in 89% versus 38% of patients at 1 year, respectively. Studies done to
compare balloon dilation versus surgery show perforation rates of 4% and 1%
and mortality rates of 0.5% and 0.2%, respectively. Results were considered
excellent in 60% of patients undergoing balloon dilation and in 85% of those
undergoing surgery.
Studies looking at laparoscopic versus open myotomy have all demonstrated

superior results with a minimally invasive technique. Shorter length of stay, less
pain, and excellent relief of dysphagia with an improved heartburn score have all
been documented with a laparoscopic approach.
Furthermore, laparoscopic myotomy appears to be safe and effective even after

treatment with Botox or balloon dilation, or with a massively dilated esophagus.
Most patients in the South African public hospital setting present with advanced
disease, end-stage achalasia. In these late presentations, a surgical myotomy is
less likely to be effective.

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4.

With respect to achalasia:

a) Describe the anatomy of the oesophagogastric junction. (30)

The oesophagogastric junction is the distal end of the abdominal

There are four anatomic points that mark the LES:

1. Two of which can be noted on endoscopy:

a. The Z-Line – the squamocolumnar junction (provided the patient

2. Two of which can be seen external to the oesophagus:

a. The Collar of Helvetius/Loop of Willis marks where the circular

Arterial supply to the oesophago-gastric junction comes from the paired

The lymphatic drainage of the abdominal oesohpagus and oesophago-gastric

b) Outline the steps in confirming the diagnosis. (30)

Diagnosis of achalasia includes taking a careful history and clinical examination

History & Clinical Examination

A strong suspicion of the diagnosis can usually be made on taking a careful

Regurgitation of undigested, foul-smelling foods is common, and with progressive

The diagnosis of achalasia is usually made from a Barium Swallow, which

1. May vary, depending on the stage of the disease

1. The LES will be hypertensive with pressures usually above 35 mm Hg

These five findings provide a diagnosis of achalasia.

An endoscopy is performed to evaluate the mucosa for evidence of esophagitis

c) Discuss the management options. (40)

Nonsurgical treatment options include medications and endoscopic interventions.

1. In the early stage of the disease, medical treatment with sublingual

1. Bougie dilation up to 54 French may offer several months of relief but

Surgical esophagomyotomy offers superior results and is less traumatic than

The modified Heller myotomy that was described originally by a laparotomy in

The decision to perform an antireflux procedure remains controversial. Most

The indications for an oesophagectomy include:

A transhiatal esophagectomy with or without preservation of the vagus nerve

Results of medical, interventional, and surgical procedures all point to surgery as

When comparing balloon dilation to Botox injections, remission of symptoms

Studies looking at laparoscopic versus open myotomy have all demonstrated

Furthermore, laparoscopic myotomy appears to be safe and effective even after

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