Case Presentation

Clients profile
Name: Monica Atilano
Age: 49 y/o
Civil Status: Married
Occupation: Housewife
Past history: (-) DM (-) BA (-)HPN
Chief complaint: Dysphagia
Final Diagnosis: Primary Achalasia
Procedure performed: Laparatomy, Hellers myotomy with dor fundoplication
Illness condition: Achalasia
Achalasia ("no relaxation") is a failure of
smooth muscle fibers to relax, which can cause a
sphincter to remain closed and fail to open when
needed. Without a modifier, "achalasia" usually
refers to achalasia of the esophagus, which is also
called esophageal achalasia, achalasia cardiae,
cardiospasm, and esophageal aperistalsis. Achalasia
can
happen
at
various
points
along
the
gastrointestinal tract; achalasia of the rectum, for
instance, in Hirschsprung's disease.
Esophageal achalasia is an esophageal motility disorder involving the smooth
muscle layer of the esophagus and the
An axial CT image showing marked
lower esophageal sphincter. It is characterized by
dilatation of the esophagus in a
incomplete LES relaxation, increased LES tone, and
person with achalasia.
lack of peristalsis of the esophagus (inability of
smooth muscle to move food down the esophagus) in
the absence of other explanations like cancer or fibrosis.
Achalasia is characterized by difficulty in swallowing, regurgitation, and
sometimes chest pain. Diagnosis is reached with esophageal manometry and
barium swallow radiographic studies. Various treatments are available, although
none cures the condition. Certain medications or Botox may be used in some cases,
but more permanent relief is brought by esophageal dilatation and surgical cleaving
of the muscle (Heller myotomy).
The most common form is primary achalasia, which has no known underlying
cause. It is due to the failure of distal esophageal inhibitory neurons. However, a
small proportion occurs secondary to other conditions, such as esophageal cancer
or Chagas disease (an infectious disease common in South America). Achalasia
affects about one person in 100,000 per year. There is no gender predominance for
the occurrence of disease.
Signs and symptoms

The main symptoms of achalasia are dysphagia (difficulty in swallowing),

regurgitation of undigested food, chest pain behind the sternum, and weight loss.
Dysphagia tends to become progressively worse over time and to involve both fluids
and solids. Some people may also experience coughing when lying in a horizontal
position. The chest pain experienced, also known as cardiospasm and non-cardiac
chest pain can often be mistaken for a heart attack. It can be extremely painful in
some sufferers. Food and liquid, including saliva, are retained in the esophagus and
may be inhaled into the lungs (aspiration).
Mechanism/Pathophysiology
The cause of most cases of achalasia is unknown. LES pressure and
relaxation are regulated by excitatory (e.g., acetylcholine, substance P) and
inhibitory (e.g., nitric oxide, vasoactive intestinal peptide) neurotransmitters. People
with achalasia lack nonadrenergic, noncholinergic, inhibitory ganglion cells, causing
an imbalance in excitatory and inhibitory neurotransmission. The result is a
hypertensive nonrelaxed esophageal sphincter.
Autopsy and myotomy specimens have, on histological examination, shown
an inflammatory response consisting of CD3/CD8-positive cytotoxic T lymphocytes,
variable numbers of eosinophils and mast cells, loss of ganglion cells, and
neurofibrosis; these events appear to occur early in achalasia. Thus, it seems there
is an autoimmune context to achalasia, most likely caused by viral triggers. Other
studies suggest hereditary, neurodegenerative, genetic and infective contributions.
Diagnosis
Due to the similarity of symptoms, achalasia can be mistaken for more
common disorders such as gastroesophageal reflux disease (GERD), hiatus hernia,
and even psychosomatic disorders. Specific tests for achalasia are barium swallow
and esophageal manometry. In addition, endoscopy of the esophagus, stomach, and
duodenum (esophagogastroduodenoscopy or EGD), with or without endoscopic
ultrasound, is typically performed to rule out the possibility of cancer. The internal
tissue of the esophagus generally appears normal in endoscopy, although a "pop"
may be observed as the scope is passed through the non-relaxing lower esophageal
sphincter with some difficulty, and food debris may be found above the LES.
Barium swallow
The patient swallows a barium solution, with continuous fluoroscopy (X-ray
recording) to observe the flow of the fluid through the esophagus. Normal peristaltic
movement of the esophagus is not seen. There is acute tapering at the lower
esophageal sphincter and narrowing at the gastro-esophageal junction, producing a
"bird's beak" or "rat's tail" appearance. The esophagus above the narrowing is often
dilated (enlarged) to varying degrees as the esophagus is gradually stretched over
time. An air-fluid margin is often seen over the barium column due to the lack of
peristalsis. A five-minute timed barium swallow can provide a useful benchmark to
measure the effectiveness of treatment.
Esophageal manometry

Because of its sensitivity, manometry (esophageal motility study) is

considered the key test for establishing the diagnosis. A thin tube is inserted
through the nose, and the patient is instructed to swallow several times. The probe
measures muscle contractions in different parts of the esophagus during the act of
swallowing. Manometry reveals failure of the LES to relax with swallowing and lack
of functional peristalsis in the smooth muscle esophagus.
Characteristic manometric findings are:
Lower esophageal sphincter (LES) fails to relax upon wet swallow (<75% relaxation)
Pressure of LES <26 mm Hg is normal,>100 is considered achalasia, > 200 is nut
cracker achalasia.
Aperistalsis in esophageal body
Relative increase in intra-esophageal pressure as compared with intra-gastric
pressure
Biopsy
Biopsy, the removal of a tissue sample during endoscopy, is not typically
necessary in achalasia but if performed shows hypertrophied musculature and
absence of certain nerve cells of the myenteric plexus, a network of nerve fibers
that controls esophageal peristalsis.
Treatment
Sublingual nifedipine significantly improves outcomes in 75% of people with
mild or moderate disease. It was classically considered that surgical myotomy
provided greater benefit than either botulinum toxin or dilation in those who fail
medical management. However, a recent randomized controlled trial found
Pneumatic Dilation to be non-inferior to Laparoscopic Heller's Myotomy.
Lifestyle changes
Both before and after treatment, achalasia patients may need to eat slowly,
chew very well, drink plenty of water with meals, and avoid eating near bedtime.
Raising the head off the bed or sleeping with a wedge pillow promotes emptying of
the esophagus by gravity. After surgery or pneumatic dilatation, proton pump
inhibitors are required to prevent reflux damage by inhibiting gastric acid secretion,
and foods that can aggravate reflux, including ketchup, citrus, chocolate, alcohol,
and caffeine, may need to be avoided.
Medication
Drugs that reduce LES pressure are useful. These include calcium channel
blockers such as nifedipine and nitrates such as isosorbide dinitrate and
nitroglycerin. However, many patients experience unpleasant side effects such as
headache and swollen feet, and these drugs often stop helping after several
months.[medical citation needed]
Botulinum toxin (Botox) may be injected into the lower esophageal sphincter
to paralyze the muscles holding it shut. As in the case of cosmetic Botox, the effect
is only temporary and lasts about 6 months. Botox injections cause scarring in the

sphincter which may increase the difficulty of later Heller myotomy. This therapy is
recommended only for patients who cannot risk surgery, such as elderly people in
poor health. Pneumatic dilation has a better long term effectiveness than botox.
Pneumatic dilatation
In balloon (pneumatic) dilation or dilatation, the muscle fibers are stretched
and slightly torn by forceful inflation of a balloon placed inside the lower esophageal
sphincter. Gastroenterologists who specialize in achalasia have performed many of
these forceful balloon dilatations and achieve better results and fewer perforations.
There is always a small risk of a perforation which requires immediate surgical
repair. Pneumatic dilatation causes some scarring which may increase the difficulty
of Heller myotomy if the surgery is needed later. Gastroesophageal reflux (GERD)
occurs after pneumatic dilatation in some patients. Pneumatic dilatation is most
effective in the long term on patients over the age of 40; the benefits tend to be
shorter-lived in younger patients. It may need to be repeated with larger balloons
for maximum effectiveness.
Surgery
Heller myotomy helps 90% of achalasia patients. It can usually be performed
by a keyhole approach or laparoscopically. The myotomy is a lengthwise cut along
the esophagus, starting above the LES and extending down onto the stomach a
little way. The esophagus is made of several layers, and the myotomy cuts only
through the outside muscle layers which are squeezing it shut, leaving the inner
muscosal layer intact. A partial fundoplication or "wrap" is generally added in order
to prevent excessive reflux, which can cause serious damage to the esophagus over
time. After surgery, patients should keep to a soft diet for several weeks to a month,
avoiding foods that can aggravate reflux.
Most recommended fundoplication along with Heller's myotomy is Dor's
fundoplication. It consists of 180 to 200 degree anterior wrap around the
esophagus. It provides excellent result as compared to Nissen's fundoplication
which is associated with higher incidence of the post surgery dysphagia.
The shortcoming of laparoscopic esophageal myotomy is the need for a
fundoplication. On one hand the myotomy opens the esophagus and on the other
hand the fundoplication causes an obstruction. Recent understanding of the
Gastroesophageal Antireflux Barrier/Valve has shed light on the reason for the
occurrence of reflux following myotomy. The Gastroesophageal Valve is the result of
infolding of the esophagus into the stomach at the esophageal hiatus. This infolding
creates a valve which extends from 7 o'clock to 4 o'clock (270 degrees) around the
circumference of the esophagus. Laparoscopic myotomy cuts the muscle at the 12
o'clock position resulting in incomptence of the valve and reflux. Recent Robotic
Laparoscopic series have attempted a myotomy at the 5 o'clock position on the
esophagus away from the valve.The Robotic Lateral Esophageal Myotomy preserves
the esophgeal valve and does not result in reflux and obviates the need for a
fundoplication. The Robotic Lateral Esophageal Myotomy has had the best results to
date in terms of ability to eat without reflux.
Since 2010, a new endoscopic treatment modality has been introduced.
Called POEM (peroral endoscopic myotomy), this therapy modality has been
performed on about 2500 patients since 2010.

Follow-up
Follow-up monitoring: Even after successful treatment of achalasia,
swallowing may still deteriorate over time. The esophagus should be checked every
year or two with a timed barium swallow because some may need pneumatic
dilatations, a repeat myotomy, or even esophagectomy after many years. In
addition, some physicians recommend pH testing and endoscopy to check for reflux
damage, which may lead to a premalignant condition known as Barrett's esophagus
or a stricture if untreated.
Nursing interventions
For achalasia patients with NGT
1. Assess for and report signs and symptoms of aspiration of secretions or
foods/fluids (e.g. rhonchi, dull percussion note over affected lung area, cough,
tachypnea, tachycardia, dyspnea, presence of tube feeding in tracheal
aspirate, chest x-ray results showing pulmonary infiltrate).
2. Implement measures to reduce the risk for aspiration:
A. withhold oral foods/fluids and place client in side-lying position if
he/she has a depressed or absent gag reflex, severe dysphagia, and/or
is not alert
B. perform oropharyngeal suctioning, encourage client to use tonsil-tip
suction, and provide oral hygiene as often as needed to remove excess
secretions
C. if client is receiving tube feedings:
I.
check tube placement before each feeding or on a routine basis
if feeding is continuous
II.
do not increase rate of continuous tube feeding infusion unless
ordered; administer intermittent tube feedings slowly
III.
maintain client in a high Fowler's position during and for at least
30 minutes after feeding unless contraindicated
IV.
stop tube feeding and notify physician if residuals exceed
established parameters (usually 75-150 ml)
D. if oral intake is allowed:
I.
perform actions to improve ability to swallow (see Diagnosis 2,
action b)
II.
allow ample time for meals
III.
instruct client to avoid laughing and talking while eating and
drinking
IV.
maintain client in high Fowler's position during and for at least
30 minutes after meals and snacks unless contraindicated
V.
assist client with oral hygiene after eating to ensure that food
particles do not remain in mouth.
3. If signs and symptoms of aspiration occur:
A. perform tracheal suctioning
B. withhold oral intake
C. prepare client for chest x-ray.
Nursing management: Post Op incision
1. Reducing complications from surgery.

2.
3.
4.
5.

Accelerate healing.
Restoring the function of patients as much as possible before surgery.
Maintaining the patient's self concept.
Preparing the patient goes home.

Care after surgery

1. Postoperative nursing actions
a. Monitor consciousness, vital signs, CVP, intake and output
b. Observation and record drain darai properties (color, amount) drainage.
c. In organizing and moving the position of the patient must be careful not to drain
uprooted.
d. Sterile wound care.
2. Food
In the post-surgery patients are generally not allowed to swallow food after
surgery. foods recommended in postoperative patients are foods high in protein and
vitamin C. Protein is needed in the process of wound healing, whereas the
antioxidant vitamin C helps increase body resistance to infection prevention.
diit restriction does is NPO (nothing peroral)
Usually new foods given if:
Abdominal bloating not
Peristaltic normal bowel
Positive flatus
Bowel positive movement
3. Mobilization
Usually the patient is positioned to lie in bed so that the situation is stable. Usually
the initial position is supine, but must still be done in order to avoid changes
decubitus position. Patients undergoing abdominal surgery are encouraged to
perform early ambulation.
4. Meeting the needs of elimination
Urinary system.
- Voluntary control urinary function after 6-8 hours post inhalation anesthesia, IV,
spinal.
Anesthesia, IV infusion, surgery manipulationretained urine.
- Prevention: Inspection, Palpation, Percussionlower abdomen (bladder
distension).
- Dower catheterexamine the color, the amount of urine, urine output < 30 ml /
hourrenal complications.
Gastrointestinal System.
- Nausea vomiting can40% of clients with GA during the first 24 hours lead to
stress and irritation of GI injury and can improve ICT in head and neck surgery and
IOP increases.
- Assess the gastro intestinal function by auscultation of bowel sounds.
- Kaji paralitic ileusbowel sound (-), abdominal distension, no flatus.
- The amount, color, consistency stomach contents every 6-8 hours.
- Insertion of intra-operative NG tube to prevent postoperative complications with
decompresi and gastric drainage.
Increase the break.
Provide an opportunity to cure the GI trac.
Monitor bleeding.

 Preventing bowel obstruction.

Irrigation or drug delivery.