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Case
presentation:
A 69-year-old woman was sent to the emergency room because
of vomiting for 10 days and dyspnea for 3 days.
She was a case of cervical cancer s/p
ATH 2 years earlier and ventral hernia s/p laparoscopic
herniorrhaphy last year. Postprandial vomiting occurred
occasionally during the past two decades. In the past years,
vomiting progressed with increased frequency, accompanied by
progressive dysphagia for solid foods and liquids. She also
experienced dyspepsia and acid regurgitation. Chest X-ray
(CXR) showed a tortuous structure with air-fluid level at
paratracheal region (Fig.
1 ) one year ago, when she
underwent laparoscopic herniorrhaphy. Upper gastrointestinal
(UGI) barium study showed slow passage of barium through a
tortuous, dilated esophagus with prolonged transitional time
(Fig.
2,Fig
3). Abdominal CT scan showed dilated esophagus with fluid
filling and air-fluid level (Fig.
4,Fig
5
),
but no mass or space-taking lesion at gastro-esophageal
junction. The lower-esophageal-sphincter (LES) manometry with
stationary pull through method showed that basal LES pressure
was 38-44mmHg and relaxation of LES was incomplete. Achalasis
was impressed but the patient refused UGI endoscopy and
surgical intervention. However, she had a weight loss of 5 kgw
during the last 3 months. Since 10 days prior to the emergency
room, she started to have aggravated vomiting with a frequency
of 10 times per day, followed by productive cough and dyspnea
on exertion for 3 days. Physical examination revealed the body
temperature was 39℃ and bilateral coarse breathing sounds.
Routine laboratory tests reveal leukocytosis with left
shifting. Pneumonia was diagnosed after CXR confirmation.
Course and Treatment:
Antibiotic therapy was started for aspiration pneumonia. In
addition, nasogastric tube was inserted for decompression.
After pneumonia resolved, Heller esophagomyotomy was
performed. Operative findings included stenosis and muscular
hyperplasia at gastro-esophageal junction, proximal esophageal
dilatation above gastro-esophageal junction, and severe and
diffuse lung adhesion, especially at diaphragm. Post-operative
course was uneventful. She tried oral intake 2 days after
operation and was discharged 10 days after operation. She
reported neither dysphagia nor food regurgitation at OPD
thereafter.
Case
Analysis:
Achalasia is the most recognized, primary esophageal
motility disorder characterized by absence of distal
peristalsis, abnormal lower esophageal sphincter relaxation,
and raised lower esophageal sphincter pressure (>45 mm Hg).
The cause of achalasia is currently unknown. Hereditary,
degenerative, autoimmune, and infectious factors are possible
causes. Among them, the latter two are the most commonly
accepted. Pathological changes seen in the esophageal
myenteric (Auerbach's) plexus include a prominent but patchy
inflammatory response, loss of ganglion cells, and some degree
of myenteric neural fibrosis.
As the disease progresses, UGI barium study may show that
the esophagus becomes more dilated and tortuous and contains
retained food and saliva with air-fluid level. Esophageal
manometry is the key test to establish the diagnosis. The
pressure of the lower esophageal sphincter is usually raised
(never low), but can be normal (10–45 mm Hg) in up to half of
patients. Abnormal lower-esophageal-sphincter relaxation is
seen in all achalasia patients, and about 70–80% of patients
have absent or incomplete relaxation with wet swallows. Some
tumors of the gastro-esophageal junction can produce
pseudoachalasia. Upper gastrointestinal endoscopy, endoscopic
ultrasonography, and/or computed tomography scans are needed
to help make the diagnosis of pseudoachalasia.
Treatment option for achalasia is limited to reducing the
pressure gradient across the lower esophageal sphincter, thus
facilitating esophageal emptying by gravity. Pneumatic
dilation is the most effective non-surgical treatment option
for patients with achalasia. About 30% of patients might
require subsequent dilations. The main adverse event with
pneumatic dilation, which occurs at a cumulative rate of 2%,
is esophageal perforation.
Surgical myotomy for achalasia involves carrying out an
anterior myotomy across the lower esophageal sphincter
(Heller's myotomy). Myotomies are usually done
laparoscopically through the abdomen with a 1–2 cm distal
myotomy onto the stomach. The major complication is
uncontrolled gastro-esophageal reflux in about 10% of
patients. Up to 30% of patients can feel better and still have
poor esophageal emptying. Simple objective testing such as
follow-up manometry or a timed barium swallow might help to
define objective improvement after treatment.
Smooth-muscle relaxants including sublingual isosorbide
dinitrate or calcium blockers can be taken prophylactically
before meals or as necessary for pain or dysphagia. Endoscopic
injection of botulinum toxin type A into the lower esophageal
sphincter is the most recent treatment alternative for
achalasia.
Reference:
1. Lancet 2001;358:823-28
2. Am J Gastroenterol
1999;94:3406-3412
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Case Discussion
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