Case report: an aortic aneurysm as cause of pseudoachalasia

Case report Open Access Published: 06 March 2020 Case report: an aortic aneurysm as cause of pseudoachalasia Marian Dejaeger1,2, Maud Lormans3, Eddy Dejaeger1,2 & Katleen Fagard1,2  BMC Gastroenterology volume 20, Article number: 63 (2020) Cite this article 122 Accesses Metrics details Abstract Background Pseudoachalasia is a rare disorder which has clinical, radiographic, and manometric findings that are often indistinguishable from primary achalasia. It is usually associated with malignancy. Few reports describe vascular compression as a cause of pseudoachalasia. Case presentation Here we present a case of a 84-year-old woman with anorexia, dysphagia and unintentional weight loss initially diagnosed as achalasia. Upon further investigation a rare cause of pseudoachalasia due to vascular compression of the esophagus was found. It could have been overlooked due to the fact that the initial work-out with a barium swallow, manometry and endoscopy was suggestive for primary achalasia. Conclusion Particularly in older patients with a manometric diagnosis of achalasia, additional investigation to rule out pseudoachalasia is warranted. Although malignant involvement of the esophagus is the most common cause of pseudoachalasia, benign origins have also been described. Peer Review reports Background Dysphagia is defined as the sensation of difficulty or discomfort in swallowing. It is considered a warning symptom for potentially serious underlying causes, especially in the elderly population. Among older persons, dysphagia is a common symptom, with prevalence rates between 30 and 40% in independently living older people and more than 50% in institutionalized older patients [1]. Dysphagia symptoms can present as high or low dysphagia. High or oropharyngeal dysphagia is mostly caused by hypocontractility of the hypopharynx or by incomplete relaxation of the upper esophageal sphincter. Low or esophageal dysphagia is often due to motor dysfunction of the esophagus (insufficient peristalsis or uncoordinated contractions) or incomplete relaxation of the lower esophageal sphincter (LES) muscle, but can also be caused by gastro-esophageal reflux disease or by more rare causes of esophageal stricture [2]. Mechanical obstruction of the esophagus, either caused by intra-luminal or external compression, is a less frequent cause of low dysphagia. Achalasia can be defined as incomplete relaxation of the LES and insufficient peristalsis of the esophagus. In the Chicago classification, a diagnosis of achalasia is based on an elevated median integrated relaxation pressure (> 15 mmHg) in combination with failed peristalsis or spasm [3]. Pseudoachalasia is indistinguishable from achalasia based on manometry. The most common etiology of pseudoachalasia is malignant submucosal involvement of the esophagus. However, other causes, such as previous local surgery or external compression of the esophagus, occur as well. The rare condition in which external compression of the esophagus secondary to an aortic aneurysm or a dissecting or tortuous aorta leads to dysphagia is called dysphagia aortica and was first described by Pape in 1932 [4]. Herein, we present a case of an elderly female with an initial diagnosis of achalasia, but where additional investigations led to the diagnosis of pseudoachalasia due to an aortic aneurysm. Case presentation A 84-year-old Caucasian woman presented to our emergency department after 7 weeks of severe anorexia and unintentional weight loss of 8 kg over the last 3 months to 47.7 kg. She complained of bad taste and dysphagia to solids, but not to liquids. Furthermore she mentioned regurgitation of undigested food. Clinical examination was unremarkable except of the observed weight loss. Her medical history included arterial hypertension, hyperlipidaemia, COPD, chronic atrial flutter, heart failure, previous lung embolism, radioactive iodine therapy for hyperthyroidism and previous drainage of a subdural hematoma of the thoracolumbar spine. Blood analysis showed a digitalis intoxication, a slightly elevated C-reactive protein without leucocytosis and discrete hypoalbuminemia and a grade 2 chronic kidney disease (CKD-epi). Thyroid function was normal. Her chest radiograph showed a widened mediastinum with aortic unfolding and calcification. Since anorexia might be caused by digitalis, this therapy was discontinued. However, without alleviation of the symptoms. To exclude a Zenker’s diverticulum, a barium swallow evaluation was performed, showing mild dilatation of the proximal esophagus with distal stasis of food and marked hypocontractility, presenting as a ‘bird’s beak sign’ (Fig. 1). Endoscopic evaluation was normal, without food remnants or evidence of esophageal neoplasm (fig. S1 A-B). The latter is important to consider in elderly patients with dysphagia to solids. During the procedure a nasogastric tube was placed for supplemental feeding and the patient gained 4 kg. Fig. 1 Barium swallow showing a ‘bird’s beak’ sign at the distal esophagus Full size image Subsequent manometric examination displayed insufficient relaxation of the LES (IRP4 75 mmHg, normal < 20 mmHg) combined with a mean LES resting pressure of 156 mmHg (normal 10-35 mmHg) and a panesophageal pressure rise of > 30 mmHg in more than 20% of swallows, compatible with a type 2 achalasia according to the Chicago classification for esophageal motility disorders (Fig. 2) [3]. Fig. 2 High resolution manometry showing panesophageal pressure rise Full size image Since type 2 achalasia is associated with external compression of the esophagus a computed tomographic (CT) evaluation of the chest was performed to exclude pseudoachalasia, revealing compression of the distal esophagus by a dissecting aorta descendens, type Stanford B (40 mm) (Fig. 3, fig. S2–S3). The patient was not considered suitable for vascular repair given the multiple comorbidities and she was managed with a liquidised diet and discharged home. Her blood pressure was already well controlled with antihypertensive medication. Fig. 3 Coronal CT reconstructed image on esophagus; arrow indicates compression of distal esophagus (with nasogastric tube in situ) by aortic aneurysm Full size image Six weeks later, the patient was readmitted. Since she was not able to maintain a stable body weight and had lost another 6 kg, she was progressively started on nasogastric tube feeding, with close biochemical monitoring to prevent refeeding syndrome. In consent with the patient, a percutaneous endoscopic gastrostomy (PEG) was placed 2 weeks later and enteral feeding was continued. Unfortunately another 2 weeks later the patient developed a pulmonary infection complicated with severe cardiac decompensation and inevitably passed away. Discussion and conclusion Pseudoachalasia was first described by Ogilvie in 1947 as a secondary form of achalasia [5]. Achalasia is an uncommon esophag (...truncated)