BCM
Gastroenterology Grand Rounds - Discussion
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Final Diagnosis: End stage esophagus secondary to Achalasia. |
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Achalasia is a Greek
word that means “does not relax”
The first case was reported 300 years ago by Thomas Willis, the patients cardiospasm responded to dilation with a whalebone. (Visual
1) This is a primary esophageal motor disorder of
unknown etiology characterized manometrically by insufficient lower esophageal sphincter relaxation and loss
of esophageal peristalsis. (Visual 2) |
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The annual incidence is approximately 1 case per 100,000. Usually diagnosed
in patients who are between 25-60 years., men
and women affected equally. Symptoms are (Visual 3) Patients with achalasia
are at increased risk for esophageal squamous
cell carcinoma. A population based study in Radiologically: A barium swallow is the primary screening
test with a diagnostic accuracy
of 95%, showing a dilated esophagus
with a bird beak narrowing by
contracted lower esophageal sphincter. CXR shows a widened mediastinum with
air fluid level and and absence of normal gastric air bubble. (Visual 4) Manometry: 1) elevated resting LES pressure, above 45 mm Hg.
2) Incomplete LES relaxation. 3) Aperistalsis in the body of the esophagus. (Visual 5) MANAGEMENT: |
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The goal of the treatment
is improved esophageal emptying and relief of patient symptoms, as there is no cure for this disorder. No
treatment can restore the the muscular activity to the denervated achalasiac
esophagus; aim is to reduce the
pressure gradient across the LES, thus facilitating esophageal emptying by
gravity. The options are: 1) Pneumatic dilation:PD 2) Heller Myotomy:HM 3) Botox injection and 4) Medical therapy Pneumatic Dilation is the most effective non surgical treatment,
using a Rigiflex balloon, which comes in three sizes, 3, 3.5 and 4 cm. (Visual 9) The clinical response improved in a
graded fashion with increasing size of the balloon diameter (Vaezi and Richter in Journal of Clinical Gastroenterology
1998) a good to excellent relief of symptoms occurred in 50-93% of
patients. Eckhardt et al published (GUT 2004) a prospective study of 54 pts
treated with PD between 1981 and 1991; these pts were followed for a median
of 13.8 yrs. Complete follow up
until 2002 was obtained. A single PD resulted in a five year remission rate
of 40 % and a 10 year remission rate of 36%. Repeated dilations only mildly
improved the clinical response. Patients who were older than 40 yrs had a
significantly better outcome than younger patients. The most significant predictive factor for a favorable long term
outcome was a post dilation lowered esophageal sphincter pressure of less
than 10 mm Hg. Patients who remain in remission for five yrs are likely
to benefit from the long lasting treatment effect of PD. Botulinum Toxin injection inhibits the calcium dependent release of
acetylcholine from nerve terminals , thereby
countering the effect of the selective loss of inhibitory neurotransmitters .
It is initially effective in relieving symptoms in about 85% of pts. How ever
symptoms recur in more than 50% of
these pts within 6 months possibly because of regeneration of the
affected receptors. Older patients
>60 yrs. or with vigorous
achalasia are more likely to have a sustained response. Heller myotomy is a definitive treatment, with a good outcome in younger patients. Long term results of surgery tend to worsen
with time. It may take up to 15 yrs for recurrent symptoms of dysphagia and
the development of symptomatic GERD to become clinically evident. Therefore
the number of patients with good results will diminish with long follow-up
evaluation; Di Simmone et al showed
that post op reflux esophagitis appeared during a mean period of 76.5 months
after operation. After an initial excellent result, there was a progressive
recurrence of symptoms until 15 yrs after the operation, at which point the
number of patients free of dysphagia and reflux were 71.9% and 69.2%
respectively. The predominant cause of
failure was incomplete myotomy, followed by severe reflux and incorrect
diagnosis at the time of initial evaluation. Because of the risk of
GERD, the combination of loose Nissen
fundoplication with myotomy is currently under debate. In a study
published in Surgical endoscopy in Jan 2005, from PD after a failed HM: Zinnotto et
al (Ann of Surg 2002) reported 56% success rate in
nine patients treated with PD after a failed HM, with no perforations
supporting the notion that prior myotomy does not increase the risk of perforation. How about a HM after a failed PD? Gockel et al published their experience in Annals of
Surgery in March 2004. 19 pts who
never reached a clinical remission after PD underwent myotomy. Their clinical
course was compared with that of patients who had reached a clinical
remission after a single (n=34) or multiple (n=14) PDs. The 10 yr remission
rate was 77% in pts undergoing myotomy, 72% in pts successfully treated with
a single PD and 45% in pts undergoing several dilations. Myotomy was therefore shown to be an effective treatment modality in
patients with achalasia who have failed to respond to PD. Redo laparoscopic surgery is safe and feasible as reported by Mayo’s
surgical group (Gorecki et al), study published in
Surgical Endoscopy 2002. Eight
patients underwent redo procedures between 1994 and 1998. The reason for
failure was incomplete myotomy in five, incorrect diagnosis in two, and new
onset of reflux symptoms in one. The redo lap surgery resulted in symptom
improvement (83%) in all, but one, with the quality of life score improving from
poor to good. Surgeon experience and
recognition of the cause for failure of the original operation are most
important in predicting the outcome. This however, is reported to carry a
high morbidity and mortality of 32% and 5.4% respectively, therefore efforts
should be made to exhaust less invasive approaches before subjecting the
patient to a more extensive procedure. (Visual 10) Esophagectomy and
colon interposition is the next approach taken for failed myotomy or PD. Patients with mega esophagus and esophageal diameter > 8 cm or
those with low LES pressure and
persistent symptoms typically do not do well with either PD or HM and may
require an esophagectomy with a gastric pull up or
colon interposition. A recent article published in Annals of Thoracic surgery
2003 , 9 patients underwent limited distal esophagectomy with short colon
interposition through a left thoracoabdominal approach as an alternative to
nearly total esophagectomy with a right thoracotomy or trans hiatal approach.
This procedure was safe and involved less dissection of the intrathoracic
esophagus and easy mobilization of the wrapped EG junction. Another study
from Cleveland Clinic, in a 10-year period, 32 patients underwent esophagectomy with gastric reconstruction for achalasia; 30 (94%) underwent elective surgery, 2
emergent surgery. No postoperative deaths occurred. Of 30 patients, 26 (87%)
felt better after esophagectomy. Esophagectomy with gastric reconstruction relieved
preoperative dysphagia and regurgitation in the
majority of patients. Dietary function and weight maintenance were excellent,
attesting to the durability of the procedure in patients with end-stage achalasia. In a recent article published
in Am. J. of Gastro in 2004 by Marcelo and Richter discussed the complexities of managing achalasia at
a tertiary referral center. 232
pts were evaluated, out of these,184 were untreated,
48 had prior esophageal surgery. PD
was used in111pts. With improvement in symptoms in 86%, and Barium emptying
improve in 54%. 19 (17%) required subsequent Heller myotomy. Perforation rate
was 3/111(2.7%). 16% required PPI for
GERD. HM was used in 72 pts
(81% laparoscopic). 1/3 rd had their procedure combined with an anti reflux
procedure, usually a post partial(Toupet)
or more infrequently an anterior partial(Belsey or dor). It should be noted that the rate of PPI
use was similar either with or without fundoplication(44%vs59%). Symptom and barium emptying improved in
89% and 44% respectively. PPI required in 53%. Botox was used in 39 older patients (mean age 71); symptom
improvement lasted for a mean 6.2 months, with frequent need for repeated
injection (mean:1.7, range: 1-7). About 43% required additional treatment with
a different modality. Esophagectomy was done in three patients. (Visual 7) .
(Visual 8). Patients
with prior surgery (n=48): PD 9(n=10) achieved symptom and barium
emptying improvement in 67% and 11%,
comparable to redo HM (n= 21) with 57% symptom improvement and 38% improved emptying. Redo HM was
performed thru the abdomen with either a lap or open approach. Esophagectomy
was required in eight (17%) despite all the modalities available, their
disease was either too far advanced at the time they sought treatment or they
failed all the other treatments. Esophagectomy was
performed via the trans hiatal route, gastric
reconstruction, cervical anastomosis, and pyloromyotomy. Botox was reserved
for elderly high risk or as a part of multimodality treatment approach In summary the treatment of achalasia can be complex. The best balance of success lies in being able to utilize all the
available therapies, including Botox for palliation, the more definitive
treatment (PD or HM), and esophagectomy for those with end stage disease.
In this study prior Botox treatment did not affect the outcome of PD, similarly prior PDs have no effect on the success of
HM. The treatment of achalasia patients after failing an initial myotomy is
less successful; PD in this group can be performed safely, but repeat HM has
the better chance of improving both symptoms and esophageal emptying. |
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