[Downloaded free from http://www.njcponline.com on Tuesday, April 05, 2016, IP: 41.132.79.253] Review Article Pseudoachalasia: A review U Abubakar, MB Bashir1, EB Kesieme2 Department of Surgery, Cardiothoracic Surgery Unit, Usmanu Danfodiyo University, Usmanu Danfodiyo Teaching Hospital, 1Department of Surgery, General Surgery Unit, Usmanu Danfodiyo University, Usmanu Danfodiyo University Teaching Hospital, Sokoto, 2Department of Surgery, Cardiothoracic Surgery Unit, Irua Specialist Teaching Hospital, Irua, Nigeria Abstract Pseudoachalasia presents typically like achalasia. It account for only 2.4-4% of patients presenting with achalasia-like symptoms. Clinical, radiologic and endoscopic findings resemble those of achalasia but treatment and prognosis are different in these conditions. The aim of this review is to give an overview of the condition and highlight challenges in diagnosis and distinguishing features between the two conditions. A review of the publications obtained from Medline search, medical libraries, and Google on ‘pseudoachalasia’ and ‘secondary achalasia’ was done. A total of 50 articles were retrieved and used for this review. There has been tremendous efforts towards establishing the diagnosis of pseudoachalasia both clinically and with the use of modern investigative modalities but to date its still difficult to distinguish it from achalasia. Endoscopy, endoscopic ultrasonography and computerized tomography scan have shown promising results. Key words: Dysphagia, pseudoachalasia, secondary achalasia Date of Acceptance: 02-Nov-2015 Introduction rate of up to 25% of endoscopic biopsies in the diagnosis of the primary cancer.[2] Pseudoachalasia is characterized by achalasia‑like symptoms caused by secondary etiologies in most instances an occult It is a rare clinical entity accounting for only 2.4–4% of tumor. Clinical, radiologic, and endoscopic findings resemble patients presenting with achalasia‑like symptoms. those of achalasia, but treatment and prognosis are different in these conditions.[1] This condition may be difficult to We aim to update clinicians on this rare disease entity diagnose in the early phase because of low diagnostic yield and highlight the challenges in making diagnosis and of either barium or endoscopic findings and false‑negative treatment. Methods Address for correspondence: Dr. U Abubakar, A literature review of pseudoachalasia was done from Department of Surgery, Cardiothoracic Surgery Unit, Usmanu Danfodiyo University, Usmanu Danfodiyo 1970 to date using manual library search, journal Teaching Hospital, Sokoto, Nigeria. publications on the subject, and Medline using search E-mail: [email protected] This is an open access article distributed under the terms of the Creative Access this article online Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows Quick Response Code: Website: www.njcponline.com others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms. For reprints contact: [email protected] DOI: 10.4103/1119-3077.179275 How to cite this article: Abubakar U, Bashir MB, Kesieme EB. PMID: ******* Pseudoachalasia: A review. Niger J Clin Pract 2016;19:303-7. © 2016 Nigerian Journal of Clinical Practice | Published by Wolters Kluwer - Medknow 303 [Downloaded free from http://www.njcponline.com on Tuesday, April 05, 2016, IP: 41.132.79.253] Abubakar, et al.: Pseudoachalasia terms “pseudoachalasia” and “secondary achalasia”. Pathogenesis Full texts of the materials, including those of relevant Two patterns of tumor involvement have been described.[10] references, were collected and studied. Information relating The most common type consists of malignant stricture of to historical perspective, etiology, pathogenesis, clinical the cardia that acts as a physical barrier to the passage of features, diagnosis, and treatment were extracted from food. A less frequent type is strictly related to the malignant these materials. submucosal infiltration with secondary impairment of inhibitory neurons of the esophageal myenteric plexus by Historical perspective tumor cells, which let the manometric pattern of achalasia be Horvath first suggested this entity in 1919.[3] Ogilvie later stable even after any treatment. Indeed, many malignancies described the syndrome in 1947.[4] He described it as a form as common causes of pseudoachalasia directly involve the of achalasia as a result of the involvement of the cardia esophageal myenteric plexus by neoplastic cells infiltrating region from gastric carcinoma. Park, in 1952, and Asherson, the mucosa at the cardia as the main pathogenetic in 1953, reported on patients with apparent “cardiospasm”, mechanism.[19‑21] However, neuronal degeneration distant in whom gastric cancer was eventually diagnosed.[5,6] from the primary tumor site with a reduction in ganglion cells in the dorsal nucleus of the vagal nerve or in the vagal Incidence and prevalence nerve itself has been also proposed.[22‑24] This interaction Owing to the lack of a large series, there are no reliable of tumor factors with the esophageal neuronal plexus epidemiological data on the incidence and prevalence of without a direct infiltration of the esophagogastric junction, the disease. Campo et al. in their review reported 4.7% of even infrequently, and serological antineuronal nuclear patients who fulfill the manometric criteria of achalasia antibody can be detected in these patients, suggesting a were eventually diagnosed with a malignant disease, directly paraneoplastic syndrome. Another form of pseudoachalasia or indirectly involving the cardia, or following antireflux occurs following antireflux surgery.[17] Three explanations surgery.[5] have been proposed: Misdiagnosed idiopathic achalasia with evidence of dysphagia just after surgery, achalasia Etiology occasionally developing for the underlying gastroesophageal Primary malignancies of the esophagus and esophagogastric reflux, and development of scar tissue and/or an overly tight [25‑27] junction account for 50% of cases of pseudoachalasia.[7] fundic wrap. This is followed by secondary malignancies (18%) such as metastases, which primarily originated from the lungs and The mechanism of esophageal dilation and dysmotility breast. Malignancies from different parts of the body have postgastric banding is unknown. Lower esophageal also been reported.[8‑12] sphincter (LES) pressure and LES residual pressure are increased after LAGB. A proposed mechanism is the Benign causes of pseudoachalasia include benign mesenchymal high outflow resistance caused by the gastric band at tumors, secondary amyloidosis, and peripheral neuropathy. the LES that creates a high‑pressure area leading to a Others include pancreatic pseudocyst, sarcoidosis, progressive weakening of the esophageal musculature. The neurofibromatosis,[13,14] esophageal leiomyomatosis,[15] inflammation around the laparoscopic band, with fibrosis esophageal mesenchymal tumors,[ 15] histiocytosis‑x,[16] aortic or neuromuscular damage, may account for the variability [9] aneurysm, juvenile Sjogren syndrome, chronic idiopathic of manometric studies. intestinal pseudo‑obstruction, familial glucocorticoid deficiency syndrome, and a few surgeries (vagotomy, bariatric, The mechanism of pseudoachalasia in EPS is unclear, and Nissen fundoplication).[1] They account for 12% of the but authors of the single case report of EPS causing causes of pseudoachalasia. Three explanations have been pseudoachalasia postulated that thickened and sclerotic proposed for antireflux surgery causing pseudoachalasia. peritoneum on the abdominal surface of the diaphragm and These are misdiagnosed idiopathic achalasia with evidence the lower esophagus caused a mechanical constriction of the [18] of dysphagia just after surgery, achalasia occasionally lower esophageal sphincter presenting as pseudoachalasia. developing for the underlying gastroesophageal reflux, and development of scar tissue and/or an overly tight fundic Clinical Features wrap.[17] Pseudoachalasia has also been reported in a patient that had laparoscopic adjustable gastric banding (LAGB).[9] The classic symptom presentation of short‑term interval Encapsulating peritoneal sclerosis (EPS) as a complication weight loss, progressive dysphagia, and chest pain are of peritoneal dialysis has also been reported as a cause of secondary to impaired esophageal emptying at the distal pseudoachalasia.[18] esophagus. The demographic study has demonstrated gender predisposition with a median age of 61 years.[28] Rare causes include paraneoplastic syndromes from small Patients with pseudoachalasia are usually over 50 years cell carcinoma of the lung, bronchial carcinoid, gastric of age; they present with a short history of progressive carcinoma, and pleural mesothelioma. dysphagia usually <1 year and significant weight loss 304 Nigerian Journal of Clinical Practice • May-Jun 2016 • Vol 19 • Issue 3 [Downloaded free from http://www.njcponline.com on Tuesday, April 05, 2016, IP: 41.132.79.253] Abubakar, et al.: Pseudoachalasia usually >7 kg and retrosternal pain.[29] They may present occurring secondary to distal esophageal or esophagogastric with other features of primary malignancy. junction carcinoma. The hallmark pathologic characteristics of this condition are an invasion and disruption of the Diagnosis myenteric plexus or a paraneoplastic autoimmune‑mediated depletion of myenteric ganglion cells.[27,35] Kahrilas et al.[16] in Pseudoachalasia