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WeBSurg, the e-surgical reference of Laparoscopic surgery

Clinical Case

PATIENT REFERRED WITH ACHALASIA


F Jamali, MD, J Leroy, MD, Hôpitaux Universitaires de Strasbourg, Strasbourg, France

1. Description


This case illustrates the clinical scenario of pseudoachalasia, ie a patient presenting with typical symptoms of achalasia secondary to a tumor of the gastroesophageal (GE) junction. It emphasizes the dictum that malignancy should be ruled out in every case of achalasia.

2. Keywords


Achalasia, esophageal carcinoma, dysphagia

3. Patient


Female, 77-year-old

4. Past medical history


- insulin-dependent diabetes mellitus;
- lower extremity vascular insufficiency (claudication), treated medically.

5. History of present illness


The patient presents with new onset dysphagia, which has progressively worsened over the course of the last year. The dysphagia was initially limited to solids but grew progressively more severe leading to significant weight loss.

6. Upper endoscopy


- stenosis of the gastroesophageal junction that is easily dilatable with the endoscope;
- no suspicious masses, ulcerations or other anomalies are noted.

7. Contrast swallow


Stenosis of gastroesophageal junction with a bird's beak deformity at the level of the G-E junction suggestive of achalasia. There is significant retention of contrast in the dilated esophagus and a somewhat irregular appearance to the G-E junction.
Figure
Figure 7.c

8. Manometry


- basal LES pressure = 21 mm Hg;
- poor esophageal peristalsis;
- failure of relaxation of LES consistent with achalasia.

9. Clinical progression I


The patient undergoes a pneumatic dilatation with temporary relief of the dysphagia. However the symptoms recur rapidly with continued weight loss. After a second failed pneumatic dilatation attempt the patient is referred for a surgical myotomy.

10. Procedure


This brief video illustrates the diagnostic laparoscopy performed. The surgeon was surprised by the discovery of a suspicious lesion at the cardia involving the crura, especially the left crus. A biopsy is performed and the procedure is terminated.
video
Video 10

11. Pathology


Biopsy reveals well differentiated adenocarcinoma.

12. Clinical progression II


Because of the invasion of the diaphragm at the level of the left crus seen and confirmed at the time of the laparoscopic exploration, the patient is considered to be unresectable and a metallic expandable stent is placed for palliative purposes, followed by radiation chemotherapy.

13. Discussion


Achalasia is an esophageal motility disorder of unknown etiology, characterized by increased lower esophageal sphincter pressure and absence of peristalsis in the lower esophagus. Histologic studies of autopsy and surgical specimens of the esophageal wall have documented a loss of ganglion cells in the myenteric plexus as well as neuronal degeneration.
Patients typically present with complaints of progressive difficulty swallowing over a period of several years. The classic triad of presenting symptoms in achalasia includes dysphagia, regurgitation, and weight loss. Complications of achalasia include esophagitis, aspiration and possibly an increased risk of esophageal carcinoma 1-10% at 15-25 years (Rosati et al. , 1995).
The diagnosis of achalasia is confirmed by esophageal manometry. The manometric criteria of achalasia are failure of the LES to relax reflexively with swallowing and lack of progressive peristalsis throughout the length of the esophagus. The roentgenographic hallmark of achalasia on barium swallow examination is the distal bird-beak tapering of the esophagogastric junction.
Both the non-surgical and surgical treatments of achalasia are directed toward relieving the obstruction caused by the non-relaxing LES. Medical treatment options include pneumatic dilatation, esophageal bougienage, nitrates, calcium channel blockers and botulinum toxin injections. The primary technique of surgical treatment is the Heller myotomy, in which longitudinal incisions are made in the muscle fibers of the lower esophageal sphincter to reduce sphincter pressure. Historical series comparing myotomy to forceful dilatation for achalasia show superior results in the surgical arm with a lower perforation rate (1% vs 4%) and improved long-term relief from dysphagia (85% vs 65%) (Okike et al. , 1979). Frequently, a fundoplication is performed in addition to the myotomy to decrease the likelihood of development of gastroesophageal reflux, a complication reported in 3-50% of cases.
This case illustrates the clinical scenario of pseudoachalasia, ie: a patient presenting with typical symptoms of achalasia secondary to a tumor of the G-E junction. It emphasizes the dictum that malignancy should be ruled out in every case of achalasia. Although this patient had undergone several endoscopies, the lesion was missed leading to the misdiagnosis. In retrospect, one view of the contrast swallow was highly suspicious and should have prompted a preoperative CT scan for proper evaluation of the G-E junction.
Figure
Figure 13

14. References


  1. Okike N, Payne WS, Neufeld DM, Bernatz PE, Pairolero PC, Sanderson DR. Esophagomyotomy versus forceful dilation for achalasia of the esophagus: results in 899 patients. Ann Thorac Surg 1979;2:119-25.
  2. Rosati R, Fumagalli U, Bonavina L, Segalin A, Montorsi M, Bona S, et al. Laparoscopic approach to esophageal achalasia. Am J Surg 1995;4:424-7.