pathologies of esophagus Achalasia Cardia.ppt

pathologies of esophagus gerd & achalasia cardia and management of achalasia and its surgical treatment

1. AchalasiaAchalasia
Prof. Khawaja Muhammad Azim FRCSProf. Khawaja Muhammad Azim FRCS
Head of Surgery DepartmentHead of Surgery Department

2. DefinitionDefinition

A Greek term that means "does not A Greek term that means "does not
relax“relax“

NormallyNormally

The act of swallowing (deglutition) The act of swallowing (deglutition)
normally initiates a peristaltic wavenormally initiates a peristaltic wave

Deglutition also triggers relaxation of Deglutition also triggers relaxation of
the lower esophageal sphincter (LES)the lower esophageal sphincter (LES)

3. DefinitionDefinition

Achalasia is a disease of unknown Achalasia is a disease of unknown
cause in which there is a loss of cause in which there is a loss of
peristalsis in the distal esophagus peristalsis in the distal esophagus
(whose musculature is comprised (whose musculature is comprised
predominantly of smooth muscle) predominantly of smooth muscle)
and a failure of LES relaxation.and a failure of LES relaxation.

5. PathophysiologyPathophysiology

Achalasia results from the Achalasia results from the
degeneration of neurons in the degeneration of neurons in the
esophageal wall.esophageal wall.

Histologic examination reveals Histologic examination reveals
decreased numbers of neurons decreased numbers of neurons
(ganglion cells) in the myenteric (ganglion cells) in the myenteric
plexuses, plexuses,

7. Cholecystokinin (CCK) test Cholecystokinin (CCK) test

In normal individuals, the intravenous In normal individuals, the intravenous
administration of CCK octapeptide stimulates both administration of CCK octapeptide stimulates both
the contraction of smooth muscle cells in the LES, the contraction of smooth muscle cells in the LES,
and the release of inhibitory neurotransmitters and the release of inhibitory neurotransmitters
from ganglion cells in the wall of the esophagus.from ganglion cells in the wall of the esophagus.

Thus, the weak, direct stimulatory effect of CCK on Thus, the weak, direct stimulatory effect of CCK on
the sphincter muscle is opposed by the CCK-the sphincter muscle is opposed by the CCK-
induced release of inhibitory neurotransmitters.induced release of inhibitory neurotransmitters.

The inhibitory effects predominate, and the net The inhibitory effects predominate, and the net
result is a fall in LES pressure.result is a fall in LES pressure.

In contrast, when CCK octapeptide is administered In contrast, when CCK octapeptide is administered
to patients with achalasia, the direct stimulatory to patients with achalasia, the direct stimulatory
effect of the hormone on smooth muscle is effect of the hormone on smooth muscle is
unopposed and LES pressure rises. unopposed and LES pressure rises.

9. EtiologyEtiology

The cause of the inflammatory The cause of the inflammatory
degeneration of neurons in degeneration of neurons in
achalasia is not known.achalasia is not known.

The observations that achalasia is The observations that achalasia is
associated with HLA-DQw1 and that associated with HLA-DQw1 and that
affected patients often have affected patients often have
circulating antibodies to enteric circulating antibodies to enteric
neurons suggest that achalasia neurons suggest that achalasia
may be an autoimmune disorder.may be an autoimmune disorder.

10. EtiologyEtiology

MalignancyMalignancy: the most common cause of : the most common cause of
pseudoachalasia in most populations. In one series, pseudoachalasia in most populations. In one series,
for example, six patients with pseudoachalasia and for example, six patients with pseudoachalasia and
161 patients with primary idiopathic achalasia were 161 patients with primary idiopathic achalasia were
seen over a 14 year period.seen over a 14 year period.

Chagas' disease: Chagas' disease: seen in Central and South America, seen in Central and South America,
esophageal infection with the protozoan parasite esophageal infection with the protozoan parasite
Trypanosoma cruzi can result in a loss of intramural Trypanosoma cruzi can result in a loss of intramural
ganglion cells leading to aperistalsis and incomplete ganglion cells leading to aperistalsis and incomplete
LES relaxation.LES relaxation.

Other causes:Other causes: A variety of other diseases have been A variety of other diseases have been
associated with achalasia-like motor abnormalities. associated with achalasia-like motor abnormalities.
These include amyloidosis, sarcoidosis, These include amyloidosis, sarcoidosis,
neurofibromatosis, eosinophilic gastroenteritis, neurofibromatosis, eosinophilic gastroenteritis,
multiple endocrine neoplasia type 2B, juvenile multiple endocrine neoplasia type 2B, juvenile
Sjögren's syndrome, chronic idiopathic intestinal Sjögren's syndrome, chronic idiopathic intestinal
pseudo-obstruction, and Fabry disease. pseudo-obstruction, and Fabry disease.

11. EpidemiologyEpidemiology

Achalasia has an annual incidence of Achalasia has an annual incidence of
approximately 1 case per 100,000.approximately 1 case per 100,000.

Men and women are affected with equal Men and women are affected with equal
frequency.frequency.

The disease can occur at virtually any The disease can occur at virtually any
age, but onset before adolescence is age, but onset before adolescence is
decidedly unusual.decidedly unusual.

Achalasia is usually diagnosed in Achalasia is usually diagnosed in
patients who are between the ages of 25 patients who are between the ages of 25
and 60 years. and 60 years.

12. Clinical ManifestationsClinical Manifestations

Dysphagia for solids (91 percent) Dysphagia for solids (91 percent)
and liquids (85 percent) is the and liquids (85 percent) is the
primary clinical feature of achalasia.primary clinical feature of achalasia.

Although dysphagia for liquids can Although dysphagia for liquids can
occur in patients with other occur in patients with other
esophageal motility disorders this esophageal motility disorders this
symptom is most characteristic of symptom is most characteristic of
achalasia and strongly suggests the achalasia and strongly suggests the
diagnosis. diagnosis.

14. Radiographic studies Radiographic studies

A barium swallow is the primary A barium swallow is the primary
screening test when achalasia is screening test when achalasia is
suspected on clinical grounds.suspected on clinical grounds.

The diagnostic accuracy of barium The diagnostic accuracy of barium
swallow for achalasia is approximately swallow for achalasia is approximately
95 percent.95 percent.

16. Manometry Manometry

Elevated resting LES pressure — In Elevated resting LES pressure — In
the LES, the loss of inhibitory the LES, the loss of inhibitory
neurons typically causes LES neurons typically causes LES
pressures to rise to hypertensive pressures to rise to hypertensive
levels.levels.

18. Medical TherapyMedical Therapy

No treatment reliably restoresNo treatment reliably restores

Nitrates and calcium channel blockers Nitrates and calcium channel blockers
relax the smooth muscle of the LES both relax the smooth muscle of the LES both
in normal individuals and in patients in normal individuals and in patients
with achalasia, and these agents have with achalasia, and these agents have
been used to treat the disorder with been used to treat the disorder with
limited success.limited success.

The drugs usually are taken sublingually 10 The drugs usually are taken sublingually 10
to 30 minutes before meals. to 30 minutes before meals.

19. Dilation of the LESDilation of the LES

excellent short-term results in 60 to 85 excellent short-term results in 60 to 85
percent of patients with achalasiapercent of patients with achalasia

Approximately 50 percent of patients with Approximately 50 percent of patients with
achalasia who are treated initially with a achalasia who are treated initially with a
single pneumatic dilation will require further single pneumatic dilation will require further
therapy within five yearstherapy within five years

Esophageal perforation is the most common Esophageal perforation is the most common
serious complication of pneumatic dilation, serious complication of pneumatic dilation,
occurring in most large series of experienced occurring in most large series of experienced
endoscopists in 2 to 6 percent of cases endoscopists in 2 to 6 percent of cases

21. Surgical MyotomySurgical Myotomy

Surgical myotomy via the modified Surgical myotomy via the modified
Heller approach results in good to Heller approach results in good to
excellent relief of symptoms in 70 excellent relief of symptoms in 70
to 90 percent of patients with few to 90 percent of patients with few
serious complications.serious complications.

The surgeon weakens the LES by The surgeon weakens the LES by
cutting its muscle fibers.cutting its muscle fibers.

The mortality rate (approximately The mortality rate (approximately
0.3 percent) is similar to that 0.3 percent) is similar to that
reported for pneumatic dilation. reported for pneumatic dilation.

32. Botulinum ToxinBotulinum Toxin

Botulinum toxin injected into the LES of Botulinum toxin injected into the LES of
patients with achalasia poisons the patients with achalasia poisons the
excitatory (acetylcholine-releasing) excitatory (acetylcholine-releasing)
neurons that increase LES smooth muscle neurons that increase LES smooth muscle
tone, thereby producing a therapeutic tone, thereby producing a therapeutic
decrease in LES pressure.decrease in LES pressure.

A number of studies have demonstrated A number of studies have demonstrated
the efficacy of botulinum toxin injection the efficacy of botulinum toxin injection
for producing short-term symptomatic for producing short-term symptomatic
improvement in patients with achalasia.improvement in patients with achalasia.

The long-term safety and efficacy remain The long-term safety and efficacy remain
uncertain. uncertain.