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Idiopathic megaesophagus (achalasia) is an esophageal primary motor irregularity. It is characterized by the absence of esophageal peristalsis, together with incomplete relaxation of the lower esophageal sphincter after swallowing.
Differential diagnosis must be made between Chagas disease and esophageal squamous cell carcinoma. The incidence rate ranges from 0.5 to 1 per 100,000 persons-years of study. Although there are several theories, the etiology remains unknown.
The first clinical description was made by Sir Thomas Wills (1672). He used to treat the disease via dilation with a sponge attached to a whalebone. Arthur Hertz was the first to name the disease “achalasia”. Ernest Heller performed the first successful esophagectomy in 1913. Zaaijer was the first to describe the anterior myotomy in 1923.
Other therapeutic procedures include botulinum toxin injection into the lower esophageal sphincter. It has transient effects and patients can develop tolerance to the injections. Another option is endoscopic hydropneumatic dilation, which should be fluoroscopically-guided. When it fails, the efficacy of other therapeutic options decreases. The most serious complication is esophageal perforation.
The diagnostic criteria are based on endoscopic findings. Endoscopy reveals there are food remains as well as esophageal dilation, and decreased motility. X-ray exams show esophageal dilation and narrowing of the lower esophageal sphincter. Manometric findings show decreased esophageal motility, increased lower esophageal sphincter pressure, and incomplete relaxation of the lower esophageal sphincter.
The patient was operated on. Since there was no hiatal hernia, laparoscopic Toupet fundoplication was chosen, based on its efficacy in preventing reflux, as well as in keeping the myotomy free of a wrap.
Her operation was complicated by septic shock due to an esophagogastric fistula with subphrenic abscesses, and on postoperative day 20, she underwent a laparotomy, abscess debridement and drainage, splenectomy and application of cyanoacrylate-based glue, followed by endoscopic positioning of a self-expandable partially coated prosthesis.
Three weeks afterwards, her prosthesis was replaced with a self-expandable fully coated prosthesis due to persistent leak. This prosthesis was finally removed after 7 weeks.
One year after her operation, at gastroscopy, a 25mm submucosal nodule covered with an ulcerated mucosa was found in the proximal antrum. Biopsy was negative, and endoscopic ultrasonography was suggestive of GIST.
She underwent an esophageal manometry, which was indicative of esophagogastric junction outflow obstruction. Her barium swallow test showed a delayed esophageal emptying due to the narrowing and twisting of the proximal part of the stomach. Her abdominal MRI was normal.
An exploratory laparoscopy was indicated for adhesiolysis and removal of the antral lesion.
Total duration of the operation was 3 hours. Her postoperative course was uneventful and she was discharged on postoperative day 6.
Her postoperative swallow study showed the easy passage of the contrast agent with no leaks. The patient completely recovered from her symptoms, and remained asymptomatic after 30 months. Final histology of her lesion evidenced a foreign body granuloma.
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