I.

INTRODUCTION
The word dysphagia is derived from the Greek words "dys" (with
difficulty) and "phagia" (to eat). Dysphagia is a subjective sensation that
suggests the presence of an organic abnormality in the passage of liquids or
solids from the oral cavity to the stomach. Patients' complaints range from the
inability to initiate a swallow to the sensation of solids or liquids being hindered
during their passage through the esophagus into the stomach. The term
"odynophagia" refers to pain with swallowing.1
Dysphagia is considered to be an alarm symptom, indicating the need for
an immediate evaluation to define the exact cause and initiate appropriate
therapy. Dysphagia in elderly subjects should not be attributed to normal aging.
Aging alone causes mild esophageal motility abnormalities, which are rarely
symptomatic. Dysphagia can be classified as either oropharyngeal or
esophageal. Oropharyngeal dysphagia, also called transfer dysphagia, arises
from disorders that affect the function of the oropharynx, larynx, and upper
esophageal sphincter. Neurogenic and myogenic disorders as well as
oropharyngeal tumors are the most common underlying mechanisms for
oropharyngeal dysphagia. Esophageal dysphagia arises within the body of the
esophagus, the lower esophageal sphincter, or cardia, and is most commonly
due to mechanical causes or a motility disturbance.1
In some patients no cause can be identified; these patients have been
categorized as having functional dysphagia . Oral dysfunction can lead to
drooling, food spillage, sialorrhea, difficulty initiating a swallow, piecemeal
swallows, and dysarthria. Pharyngeal dysfunction can lead to a sensation of
"food getting stuck" immediately upon swallowing, postnasal regurgitation,
coughing or choking during food consumption, and dysphonia. In addition,
patients with oropharyngeal dysphagia point toward the cervical region when
asked to identify the site of their symptoms. In contrast, patients with
esophageal dysphagia usually describe the onset of symptoms several seconds
after initiating a swallow. They frequently point to the suprasternal notch or
behind the sternum when asked to localize the area causing symptoms.
Retrosternal dysphagia usually corresponds with the location of the lesion,
while suprasternal dysphagia is commonly referred from below.1

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 Sir Thomas Willis described achalasia in 1672. In 1881, von Mikulicz
described the disease as a cardiospasm to indicate that the symptoms were due
to a functional problem rather than a mechanical one. In 1929, Hurt and Rake
realized that the disease was caused by a failure of the lower esophageal
sphincter (LES) to relax. They coined the term achalasia, meaning failure to
relax.2
Achalasia is a primary esophageal motility disorder characterized by
failure of a hypertensive LES to relax and the absence of esophageal peristalsis.
These abnormalities cause a functional obstruction at the gastroesophageal
junction. Achalasia occurs with an incidence of approximately 1: 100,000 with
an equal gender distribution. it occurs at all ages with an increase in incidence
observed after the seventh decade.2

II. ANATOMY
The esophagus is a mucosally lined neuromuscular tube that proples food
from the mounth and pharynx to the stomch,while minimizing acid and bile
reflux.it is the narrowest tube of the gastrointestinal tract,and within the
esophagus there are three regions of natural constriction:the cricopharynges,the
point at which the aorta and left mainstem bronchus cross anteriorly,and the
lower esophagus sphincter (LES).the cricopharyngeus is the narrowest point in
the entire gastrointestinal tract and is a common location to find food
impactions,foreign bodies,and perforations from instrumentation.3,4
The esophagus is composed of an outer muscular layer,a middle
submucosal layer,and an inner mucosal layer.the esophagus lacks a serosal lacks
a serosal lining-an important anatomic barrier or the spread of infection and
neoplastic disease.Without a serosal layer,the esophagus is more susceptible to
perforations and has less structural integrity to aid in surgical repair or
anastomoses.the upper one-third of the muscular layer is striated,whereas the
lower two-thirds are composed of smooth muscle .the muscular layer can be
further divided into outer,longitudinal and inner,cicular fibers.startified
squamous epithelium lines the majority of the esophagus ,with only the distal 1
to 3cm lined by columner epithelium.3,4
Understanding the anatomy and physiology of the UES and LES is
essential to comprehending the reflux and motility disorders that affect the

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esophagus. The inferior constrictor muscle consists of two parts: the oblique
thyropharyngeal muscle and the transverse cricopharyngeus muscle, which
forms the inferior border of the inferior constrictor. This arrangement leaves a
sparse area of musculature known as Killian triangle. The cricopharyngeus
muscle originates from the posterolateral aspects of the cricoid cartilage. It is
the thickest and strongest of the pharyngeal constrictors and forms the UES.
Coordinated relaxation and contraction of the cricopharyngeus occur with
normal deglutition, allowing passage of food from the pharynx into the upper
esophagus. The UES is maintained in a constant state of contraction.
Manometry has confirmed the radial asymmetry of the UES; resting pressures in
the anteroposterior orientation average 100 mm Hg, whereas laterally oriented
pressures are less than 50 mm Hg. The high resting pressure of the UES is
important because the body of the esophagus maintains a resting pressure of -5
mm Hg as a result of its intrathoracic location. The resting state of contraction
of the UES prevents air influx during the normal respiratory cycle. If the body
of the esophagus is exposed to acid or if volume changes occur (e.g., fluid bolus
or balloon distention), the UES pressure increases significantly. These reflexes
prevent regurgitation and possible aspiration.
The true nature of the LES continues to be studied and debated. It is not
thought to be a discrete anatomic structure; rather, it is a zone of increased
pressure that is 2 to 4 cm long. The LES functions through a combination of
anatomic and physiologic factors, preventing the reflux of gastric contents into
the lower esophagus. During swallowing, the LES relaxes, allowing ingested
material to reach the stomach. Incompetency of the LES can lead to
gastroesophageal reflux disease (GERD) and its associated complications. If the
LES fails to relax properly, dysphagia results and esophageal motility is
adversely affected.
The blood supply of the esophagus follows a sagmental
organization,although signification variation exists.in addition,the esophagus
maintains a complete net work of vessels within the wall,allowing for
mobilization over a long distance.the lymphatic drainage does not follow this
segmental patten.the cervical and mid-esophagus tend to drain cephalad to the
paraesophagel cervical nodes and then to the lower jugular noddes.the
tymphatic channels from the thoraric esophagus drain to superior mediastinal,

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 peribronchial,hilar,and paraesophadeal nodes. the abdominal esophagus drains
into the left gastric nodes and the celiac nodes.there is an basence of lymphatic
within the superfical mucosa,which may explain why intramural spread of
cancer occurs primary within the submucossa and why esophageal cancer have
a high postoperative recurrence rate at the resection line.3,4
The esophagus is innervated by the vagus nerve(parasympathetic)and
sympathetic chain.the cervical esophagus receives innervation from the
recurrent laryngeal nerves.in the superior medistinum,the vagus nerves arborize
to from an esophageal plexus.thers fibers then coalesce,forming two vegel
trunks; the left anterior vagus and the right posterior vagus.motor function is
thought to be under vagal control;the sympathetic nerves relax the muscular
wall,contract the sphincters,and increase glandular and peristaltic
activity.striated muscles have direct synaptic connections to motor
endplates,whereas smooth muscles synapse with neurons of the myenteric
ganglia.
In addition to the prevention of regurgitation and ventilation of excess
gaseous materials from the stomach,the primary function of the esophagus is the
transportation of nutrients during the esophageal phase of swallowing.as the
pharygeal constrictors contract,the upper esophageal sphincter (UES)
relaxes,propelling food into the esophagus and initiating a peristaltic wave.this
is know as primary peristalsis,and the contraction proceeds distally along the
lenght of the esophagus at a rate of approximetely 5 to 10cm per
second.secondary peristalsis clears any remaining food and is initiated by
esophagael distention or by gastroesophagel reflux.
Teriary contractions are nonperistaltic and can occur spontaneously or
after swallowing,the normal swallow is a highly coordinated sequence of events
orchestrated thourgh the swallowing center in the medulla and requires input
from crainal nerves V,VII,IX,X and XII. Lesser roles are played by both gravity
and the relative negative pressure in the lower esophagus. 3,4

III. ETIOLOGY
The exact cause of achalasia is not known. Various theories have been
advocated : 1) degeneration of Auerbach’s plexus causing failure of relaxation
of the cardiac orifice, 2) degeneration in utero due to ischaemia during rotation

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 of the gut, 3) abnormal pinch-cock action of the right crus of the diaphragm, 4)
chaga’s disease.4

IV. PATHOLOGY
It is believed that due to degeneration of the Auerbach’s plexus, no
coordinated peristalsis occurs in the oesophagus and the lower oesophagael
sphincter fails to relax in response to food.this results in retention of food and
dilatation of its lumen.4

V. CLINICAL FEATURES
The condition affects young persons of both sexes. Symptoms usually
develop insidiously but in rare instances the onset is relatively sudden,after a
nervous shock or childbirth. Substernal pain or felt burn at the beginning and
than followed by epigastric pain. Dysphagia follows later and is more marked
for fluids than for solids. the epigastric discomfort is,however,sometime
relived by drinking more fluids. Regurgitation of undigested food,sometime
taken the day before. Loss of weight may occur but is rarely severe.there may
be remissions of symptoms over periodes of years. the patient rarely becomes
ill or cachectic unless enhalation pneumonia or carcinoma of the oesephagus
supervene.4

VI. DIAGNOSIS
Achalasia can be diagnosed from clinical features, radiology using barium
flow , oesophagoscopy and manometric test. The appearances on a barium
swallow are typical. There is marked or enormous dilatation at 2/3 distal part
with smooth, rounded lower border and narrowing at distal part
gastroesophageal junction showing a mouse tail or bird beak appearance. There
is usually a fluid level in the oesophagus about 20cm from the cardia.
From oesophagoscopy examination shows dilated oesophagus containing
undigested food, and also excluded malignancy. Manometric test can be
performed to see motoric function of oesophagus with examining pressure
inside the lumen and oesophagus sphincter. Manometric studies reveal a nadir
LES pressure greater than 8 mm Hg above gastric pressure and aperistalsis.3,4
VII. TREATMENT

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 The treatment of achalasia can be perform with : 1) Lavage with a
stomach tube,is useful as a preliminary to other forms of treatment, 2)
Anticholinergic drug such as Amyl or octyl nitrite inhaled from capsules before
meals,may give relaxation and follow comfortable swallowing, 3) Dilatation of
the lower end is done under direct vision with bougies and then a hydrostatic
bag.Repeated dilatation may have to be done,even by the patient himself,using
hurt’s mercury bougies.this simple practice has lost favour in recent years and
operation is preferred in obstinate cases, 4) Cardiomyotomy (heller’s
operation)is the operation of choice.the cardiac sphincter is incised down to
mucosa, 5) Anastomotic operations. Anastomic between stomach and
oesophagus may be indicated when the oesophagus is grossly lengthened and
kinked.3,4,6
Traditionally, pneumatic dilatation has been the first line of treatment for
esophageal achalasia, while surgery was reserved for patients who had
persistent dysphagia after multiple dilatations or who had suffered a perforation
during dilatation. Today, minimally invasive surgery has completely changed
this treatment algorithm and a laparoscopic Heller myotomy and partial
fundoplication is preferred by most gastroenterologists and surgeons as the
primary treatment modality. When properly performed, a Heller myotomy can
be expected to result in permanent relief of dysphagia in 85-100% of patients.
Critical details of the operation include a generous myotomy of the lower
esophagus, extending well onto the gastric wall. Because of the lack of
esophageal peristalsis, a partial (Dor or Toupet), rather than a total
fundoplication is frequently added to prevent reflux. A recent prospective,
randomized study demonstrated that Heller myotomy plus a partial
fundoplication is superior to Heller myotomy alone in regard to the incidence of
postoperative reflux as measured by 24 hour pH testing. Patients can usually eat
the morning of the first postoperative day, and can be discharged home after one
or two days. In the only prospective, randomized trial performed comparing
balloon dilation with surgery, myotomy outperformed balloon dilation 95% to
65%.3,4,6
Historically, the most popular treatment for achalasia has been by forceful
pneumatic dilation. The success rate of this procedure is 55-70% with a single
dilation but can be increased to nearly 90% with multiple dilations. However,

6
 the risk of perforation with each dilation is at least 3-5% and has been reported
as high as 12% in some series. These patients may require open surgery to close
the perforation and perform a myotomy. Furthermore, when stratified by age,
balloon dilation is less than 50% effective in patients younger than 40 years old
and is rarely effective in adolescents.
Occasionally the degree of esophageal aperistalsis is so advanced that
myotomy alone will not relieve the dysphagia and the patient is better served
with esophagectomy. Esophagectomy should be considered in a patient who has
had a previous myotomy, with a resting LES pressure of less than 10 mmHg,
and a dilated sigmoid esophagus. The need for esophagectomy for achalasia is
very uncommon, even in the presence of a dilated esophagus, and should be
reserved for failures after myotomy. All patients undergoing treatment for
achalasia should be followed by surveillance endoscopy, because they are at
increased risk for development of both squamous and adenocarcinoma.3,4

VIII. CASE REPORT

A Woman, 48 years old came to policlinic ENT of Dr sardjito hospital
with main complain: difficult to swallow, since 4 months ago patient felt
difficult to swallow especially for hard food. Patient only able to eat soft food
and drink. Patient felt want to vomit after 2 minutes. There is a decrease in
bodyweight around 6 kg for the last four month. Patient complaint vomit after
finished eating. No ear and nose complaint. No sore throat. Pregnancy (-). No
history of gastritis.
On the physical examination, the general condition is good, compos
mentis, moderate nutritional status. Vital sign: the blood pressure 110/60 mmhg,
pulse rate 85 x/minutes, respiration rate 20x/minutes,is the temperature is
afebris.for local examination MAE with normal limit,rinoscopy anterior and
posterior with normal limit, ororpharnyx and laryx with normal limit.
Oesophagography was performed and found beak bird appearance in
oesophagus suggesting achalasia oesophagus. The patient was performed
esophagogastroduodenoscopy view and found no esophageal mass, normal
esophageal size, food found in 35 cm depth of esophagus, gastritis with multiple
erosion, suggesting paralysis at Nervus IX and XI.

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 The patient was diagnosed with dysphagia ec achalasia. The patient treat
with esophagoscopy and dilatation with bougie but failed. And then the
patient consulted to digestive surgery and plan for cardiomyotomy.

IX. DISCUSSION
On this case the diagnosis of achalasia stage from resume taking and
oesophagography. From the esophagography seen mouse tail appearance . patient was
planed to performed esophagography and dilatation using bougie. But when the
dilatation performed there is something block the bougie to enter and was suspected

8
as tumor. Then the patient was planed to perform esophagogastroduodenoscopy and
the result is no masses and suspected parese NIX and XI with erosion in esophagus
and gaster. The internist assesed this patient with gastritis erosiva. And when
consulted to the neurology division about the parese, they found no sign of parese.
The patient was returned home from ENT due to no problem in ENT and send
to follow up to Internist division. From the internist division and consulted to
digestive surgery division the patient was planned to be performed cardiomiotomy.
Many cause of dysphagia and become problem for the person that has the
problem. A good anamnese and physical examination supported with additional
examination was needed in assesed the correct diagnose that cause dysphagia. In this
patient the anamnese and the esophagography show achalasia as the cause of
dysphagia. Due to failure of dilatation with bougie this patient was planned to perform
cardiomiotomy.

X. CONCLUSION
Has been reported a case of dysphagia ec achalasia and the patient treated
with esophagoscopy and dilatation with bougie but failed and then cadiomiotomy
was performed for this patient.

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2. Fisichella CM, Patti MG. Achalasia. C2008 [Updated 2008 Jun 30 : cited
2009 Aug 31] Avaible from
http://emedicine.medscape.com/article/169974-overview
3. Shockley WW, Das S. Esophageal Disorders. In: Bailey BJ, Johnson JT,
Newlands SD, editors. Head & Neck Surgery – Otolaryngology. 4 th ed.
Lippincott Williams & Wilkins ; 2006
4. Bhargava KB, Bhargava SK, Shah TM. Examination of the Oesophagus :
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