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Pseudoachalasia presenting 20 years after Nissen fundoplication: a case report
© The Author(s). 2016
Received: 2 February 2016
Accepted: 29 June 2016
Published: 7 July 2016
Pseudoachalasia is a rare diagnosis manifested by clinical and physiologic symptoms of achalasia, with alternative etiology for outflow obstruction. While malignancy is a frequent cause of pseudoachalasia, prior surgical intervention especially surgery involving the esophagogastric junction, may result in a misdiagnosis of achalasia.
We present a case of a 70 year-old male with dysphagia and weight loss after undergoing a Billroth I and Nissen fundoplication several decades ago. His preoperative studies suggested achalasia and he was therefore referred for an endoscopic myotomy. However, careful interpretation of all the data and intra-operative findings revealed a classic mechanical and functional obstruction requiring takedown of his prior wrap.
Individualized interpretation of preoperative studies in the setting of prior foregut surgery is critical to appropriate diagnosis and intervention. This case highlights the significance of endoscopic findings and features of high-resolution manometry specific to pseudoachalasia, which contrasts with classical features of achalasia.
Achalasia is characterized by the absence of peristalsis along with impaired relaxation of the lower esophageal sphincter (LES) . Clinically, this results in dilatation of the esophagus and symptoms of regurgitation and progressive dysphagia. Pseudoachalasia is a rare disease that has many clinical features similar to primary achalasia. It is frequently attributed to malignancies of the distal esophagus or gastric cardia . These tumors result in chronic esophageal outflow obstruction that in turn result in muscular dysfunction of the esophageal body. Benign causes of pseudoachalasia include vascular obstruction (dysphagia aortica), submucosal tumors, congenital muscular rings of the distal esophagus, and mechanical obstruction from prior foregut surgeries. Usually, albeit not always, these patients develop dysphagia soon after their surgical intervention. In literature series, pseudoachalasia has been attributed to prior surgeries in about 12 % of the cases . Here, we present a case of a patient with pseudoachalasia secondary to chronic esophagogastric junction (EGJ) outflow obstruction from prior Nissen fundoplication. The case illustrates the significance of recognizing some key features of pseudoachalasia on preoperative studies that differentiate it from achalasia, before committing patients to a surgical myotomy.
Postoperatively, the patient did well and did not have any complications. After obtaining a repeat esophagram on postoperative day 6 that did not demonstrate a leak, he was started on oral intake and weaned off his tubefeeds over the next month. One year later, the patient remains symptom free and has resumed a normal diet.
A small percentage of patients who undergo fundoplication develop chronic dysphagia. In most cases of dysphagia, it is due to postoperative edema, slipped or migrated fundoplication, or even disrupted fundoplication and it usually presents in the acute setting . However, in some circumstances, prolonged mechanical outflow obstruction may lead to a burnt-out esophagus, thus mimicking a clinical picture of achalasia. It is important for clinicians to distinguish this phenomenon from primary achalasia.
Our patient had dysphagia almost 2 decades after his Nissen fundoplication and his endoscopy and esophagram were suggestive of achalasia. Several clinical features suggested otherwise. First, careful interpretation of the manometry clearly indicated normal relaxation of the LES and IRP . In addition, at the time of the revisional surgery, the endoscope met considerable resistance at the EGJ, another feature inconsistent with primary achalasia . During the laparoscopic dissection, we discovered that his fundoplication wrap had resulted in esophageal torsion, constricting the distal esophagus and confirming the diagnosis of pseudoachalasia. Once we took down the original fundoplication, corrected the torsion, and replaced it with a Dor partial fundoplication, he was able to resume normal feeding without further regurgitation. As suggested by Poulin et al., our particular case was most likely a type 2 pseudoachalasia, which is due to extensive development of scar tissue and/or tight fundic wrap that improves with removal of scar tissue and reconstruction of the wrap .
Patients who undergo foregut surgeries can develop outflow obstruction that can mimic primary achalasia. Our case highlights the importance of an aggressive evaluation and careful interpretation of preoperative studies in any patient presenting with dysphagia after a remote history of fundoplication. These patients should be referred for surgery as appropriately indicated, and surgeons should then formulate a surgical plan after carefully reviewing the upper endoscopy and high-resolution manometry to determine alternative causes of outflow obstruction.
EGD, esophagogastroduodenoscopy; EUS, esophageal ultrasound; LES, lower esophageal sphincter; EGJ, esophagogastric junction; HRM, high-resolution manometry; IRP, integrated relaxation pressure; UGI, upper gastrointestinal series
CNL and PG prepared the manuscript; MPK, KK, and BJD edited and reviewed the manuscript. All authors read and approved the final manuscript.
The authors declare that they have no competing interests.
Consent for publication
Patient signed an informed consent to release his clinical history and imaging for research purposes.
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