Secondary Achalasia Research Articles

S3410 A Rare Case of Pancreatic Cancer Causing Secondary Achalasia

S3410 A Rare Case of Pancreatic Cancer Causing Secondary Achalasia

Biallelic NDC1 variants that interfere with ALADIN binding are associated with neuropathy and triple A-like syndrome

Nuclear pore complexes (NPC) regulate nucleocytoplasmic transport and are anchored in the nuclear envelope by the transmembrane nucleoporin NDC1. NDC1 is essential for post-mitotic NPC assembly and the recruitment of ALADIN to the nuclear envelope. While no human disorder has been associated to one of the three transmembrane nucleoporins, biallelic variants in AAAS, encoding ALADIN, cause Triple-A syndrome (Allgrove syndrome). Triple A syndrome, characterized by alacrima, achalasia and adrenal insufficiency, often includes progressive demyelinating polyneuropathy and other neurological complaints. In this report, diagnostic exome and/or RNA-sequencing was performed in 7 individuals from 4 unrelated consanguineous families with AAAS negative triple A syndrome. Molecular and clinical studies followed to elucidate the pathogenic mechanism. The affected individuals presented with intellectual disability, motor impairment, severe demyelinating with secondary axonal polyneuropathy, alacrima and achalasia.. None of the affected individuals has adrenal insufficiency. All individuals presented with biallelic NDC1 in-frame deletions or missense variants, that affect amino acids and protein domains required for ALADIN binding. No other significant variants associated with the phenotypic features were reported. Skin fibroblasts derived from affected individuals show decreased recruitment of ALADIN to the NE and decreased post-mitotic NPC insertion, confirming pathogenicity of the variants. Taken together, our results implicate biallelic NDC1 variants in the pathogenesis of polyneuropathy and a Triple-A-like disorder without adrenal insufficiency, by interfering with physiological NDC1 functions, including the recruitment of ALADIN to the NPC.

Modern insights into the pathophysiology and treatment of pseudoachalasia.

Secondary achalasia or pseudoachalasia is a clinical presentation undistinguishable from achalasia in terms of symptoms, manometric, and radiographic findings, but associated with different and identifiable underlying causes. A literature review was conducted on the PubMed database restricting results to the English language. Key terms used were "achalasia-like" with 63 results, "secondary achalasia" with 69 results, and "pseudoachalasia" with 141 results. References of the retrieved papers were also manually reviewed. Etiology, diagnosis, and treatment were reviewed. Pseudoachalasia is a rare disease. Most available evidence regarding this condition is based on case reports or small retrospective series. There are different causes but all culminating in outflow obstruction. Clinical presentation and image and functional tests overlap with primary achalasia or are inaccurate, thus the identification of secondary achalasia can be delayed. Inadequate diagnosis leads to futile therapies and could worsen prognosis, especially in neoplastic disease. Routine screening is not justifiable; good clinical judgment still remains the best tool. Therapy should be aimed at etiology. Even though Heller's myotomy brings the best results in non-malignant cases, good clinical judgment still remains the best tool as well.

Pseudoachalasia as a paraneoplastic event in a patient with Hodgkin's disease.

Pseudoachalasia or secondary achalasia (5% of achalasias that are deemed primary achalasias) is an esophageal motor disorder with manometric criteria for achalasia, but it appears in the context of an underlying pathology that can be attributed to its origin. Usually appears in >60 years with rapid evolution of symptoms (<1 year). The main cause of pseudoachalasia is neoformative etiology, but there are others. Our patient started with rapid progression dysphagia and was diagnosed with type II achalasia within a Hodgkin's lymphoma. In the radiological-metabolic studies, disease involvement was ruled out as an extrinsic compression of the esophagogastric junction as well as signs of its activity at this level. Chemotherapy has not been shown to play a role in the development of this pathology. On the other hand, radiotherapy has been associated with an esophageal motor disorder, but, in our case, it was after its onset. Therefore, we propose that the mechanism of pseudoachalasia in our case is a paraneoplastic event. This hypothesis is related to other similar cases reported, and it reflects the importance of continuing to investigate this clinical condition that is indistinguishable by manometry from primary achalasia. In addition, it usually presents differential clinical characteristics whose early recognition has implications for the diagnostic, therapeutic, and prognostic management of the patient.

Successful Management of Secondary Achalasia in a Patient with Myotonic Dystrophy

Successful Management of Secondary Achalasia in a Patient with Myotonic Dystrophy

Cross-Sectional Study of Swallowing Phases in Cases of Megaesophagus Caused by Chagas Disease.

BackgroundChagas disease, caused by the flagellate protozoan Trypanosoma cruzi, is an infectious cause of secondary achalasia and megaesophagus. Moreover, the oral and pharyngeal phases of swallowing may also be affected, which may contribute to dysphagia and increase the possibility of airway aspiration during and/or after swallowing. This cross-sectional study evaluated, with videofluoroscopy, the oral, pharyngeal, and esophageal phases of swallowing in patients with megaesophagus caused by Chagas disease. The hypothesis is that there is impairment of the pharyngeal phase of swallowing that may increase the risk of airway aspiration.MethodsA total of 29 patients, aged 48 - 73 years (mean: 63.8 ± 5.1 years), with dysphagia, radiological changes in the esophagus, and positive serologic test for Chagas disease, participated in the study. They were submitted to the videofluoroscopic evaluation of oral, pharyngeal, and esophageal phases, swallowing twice 10 mL of liquid and 10 mL of thickened barium boluses.ResultsThe most frequent findings were: oral residues and ineffective ejection in the oral phase; residues in vallecula, pharynx, and pyriform sinuses in the pharyngeal phase; abnormal esophageal motility, longer clearance, and longer transit in the esophageal phase. Laryngeal penetration was seen in 28% of the patients. Patients with increased esophageal diameter had more pharyngeal residues than patients without increased esophageal diameter. None of the patients had airway aspiration.ConclusionMegaesophagus caused by Chagas disease may affect all phases of swallowing, with an increase in oral and pharyngeal residues which suggest the impairment of oral and pharyngeal efficiency. None of the patients had airway aspiration.

Management of Esophageal Dysphagia in Chagas Disease.

Chagas disease, caused by the infection of the protozoan parasite Trypanosoma cruzi, has clinical consequences in the heart and digestive tract. The most important changes in the digestive tract occur in the esophagus (megaesophagus) and colon (megacolon). Esophageal dysfunction in Chagas disease results from damage of the esophageal myenteric plexus, with loss of esophageal peristalsis, partial or absent lower esophageal sphincter relaxation, and megaesophagus, which characterizes secondary esophageal achalasia. The treatment options for the disease are similar to those for idiopathic achalasia, consisting of diet and behavior changes, drugs, botulinum toxin, peroral endoscopic myotomy (POEM), pneumatic dilation of the lower esophageal sphincter, laparoscopic Heller myotomy, and esophagectomy. Chagas disease causes a life-threatening cardiopathy, and this should be considered when choosing the most appropriate treatment for the disease. While some options are palliative, for temporary relief of dysphagia (such as drugs, botulinum toxin, and pneumatic dilation), other therapies provide a long-term benefit. In this case, POEM stands out as a modern and successful strategy, with good results in more than 90% of the patients. Esophagectomy is the option in Chagas disease patients with advanced megaesophagus, despite the increased risk of complications. In these cases, peroral endoscopic myotomy may be an option, which needs further evaluation.

Resection of Fibrous Pseudocapsule in a Patient with Secondary Achalasia and Persistent Symptoms After Removal of Adjustable Gastric Band: Case Report and Review of the Literature

Background: Secondary achalasia in the setting of an adjustable gastric band is a known, but rare complication. Most of the symptoms usually resolve after deflating or removing the gastric band, bu...

Diagnosis and treatment of pseudoachalasia: how to catch the mimic

: Pseudoachalasia, or secondary achalasia, is an uncommon esophageal dysmotility syndrome with symptoms and manometric findings indistinguishable from primary achalasia, but due to any mechanism other than idiopathic degeneration of the inhibitory neurons of the esophageal submucosal myenteric plexus. Whilst pseudoachalasia is rare, affecting some 1.4–5.4% of all achalasia patients, it is essential this diagnosis is always considered and excluded, as the treatment and outcomes for these patients will be very different from those with true achalasia. Pseudoachalasia can be difficult to differentiate from primary or “idiopathic achalasia”. Several particular clinical features have been described as more common in patients with pseudoachalasia than in achalasia, but because of the low prevalence of this condition, the positive predictive value remains low. The majority of patients with pseudoachalasia have an underlying malignancy, predominantly gastro-esophageal adenocarcinoma, which is usually advanced. Management revolves around treating the underlying cause where possible, as this may lead to reversal of the esophageal dysmotility. In patients presenting with symptoms and manometry findings consistent with achalasia, the diagnosis initially should be one of an achalasia-like syndrome. Idiopathic achalasia can then only be confirmed after other potential causes have been considered and excluded. We describe a case of pseudoachalasia encountered in our clinical practice, followed by a review of current practice regarding diagnosis and management of pseudoachalasia.

Peroral endoscopic myotomy (POEM): a comparative study between Chagasic and idiopathic achalasia.

Background and study aims Achalasia can be classified as either primary (idiopathic) achalasia or secondary achalasia, which is a consequence of another systemic disease. Peroral endoscopic myotomy (POEM) is an effective and safe treatment for achalasia. We evaluated the efficacy and safety of POEM in patients with Chagasic achalasia compared to idiopathic achalasia. Patients and methods We evaluated POEM procedures performed at a single institution from November 2016 to January 2018. Demographic data, Eckardt score, lower esophageal sphincter (LES) pressure, body mass index, post-operative erosive esophagitis, adverse events, length of hospital stay, and procedure-related parameters were analyzed. Results Fifty-one patients underwent POEM as a treatment for achalasia in this period (20 patients with Chagasic and 31 with Idiopathic etiology). The overall clinical success rate was 92.1 %, with no statistical difference between groups (90 % in the Chagasic group vs. 93.5 % in the Idiopathic group, P = 0.640). Both groups had significant reduction in Eckardt score and in LES pressure, and increase in bodey mass index (BMI) at 1-year follow-up. There was no statistical difference between groups regarding Eckardt score ( P = 0.439), LES pressure (p = 0.507), BMI ( P = 0.254), post erosive esophagitis (35 % vs. 38.7 %, P = 0.789), adverse events (30 % vs. 12 %, P = 0.163,) length of hospital stay (3.75 days vs. 3.58 days, P = 0.622), and operative time (101.3 min vs. 99.1 min, P = 0.840). Conclusion POEM is an effective and safe treatment for patients with achalasia. There is no difference in POEM outcomes for those patients with Chagasic or Idiopathic achalasia.

Pseudoachalasia: a systematic review of the literature.

Pseudoachalasia, also known as secondary achalasia, is a clinical condition mimicking idiopathic achalasia but most commonly caused by malignant tumors of gastroesophageal junction (GEJ). Our aim was to systematically review and present all available data on demographics, clinical features, and diagnostic modalities involved in patients with pseudoachalasia. A systematic search of literature published during the period 1978-2019 was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines (end-of-search date: June 25th, 2019). Two independent reviewers extracted data with regards of study design, interventions, participants, and outcomes. Thirty-five studies met our inclusion criteria and were selected in the present review. Overall, 140 patients with pseudoachalasia were identified, of whom 83 were males. Mean patient age was 60.13years and the mean weight loss was 13.91kg. A total of 33 (23.6%) patients were wrongly 'treated' at first for achalasia. The most common presenting symptoms were dysphagia, food regurgitation, and weight loss. The median time from symptoms' onset to hospital admission was 5 months. Most common etiology was gastric cancer (19%). Diagnostic modalities included manometry, barium esophagram, endoscopy, and computed tomography (CT). Pseudoachalasia is a serious medical condition that is difficult to be distinguished from primary achalasia. Clinical feature assessment along with the correct interpretation of diagnostic tests is nowadays essential steps to differentiate pseudoachalasia from idiopathic achalasia.

447 Clinical Characteristics of Secondary Achalasia Associated With a Malignancy: A 20-Year Review From a Cancer Hospital

INTRODUCTION: Achalasia is an uncommon neurodegenerative disorder of esophageal motor function with an annual incidence of 1.6:100, 000. Most cases are idiopathic. Achalasia secondary to neoplasia is extremely rare, with an incidence of 1: 750,000. AIM: To evaluate the incidence and characteristics of achalasia in patients with a malignancy at our institution between 1999-2019. METHODS: Electronic medical records were retrospectively reviewed from January 1999 to May 2019 to identify patients with a diagnosis of achalasia and a malignancy. Patiets with direct tumor invasion of the distal esophagus were excluded. We identified 28 patients who had a malignancy and a diagnosis of achalasia. RESULTS: The median age at diagnosis of achalasia was 58 years (19-85). There were 14 (50%) male and 14 (50%) female patients. The most common associated cancer was a hematologic malignancy (N = 9, 32%: lymphoma N = 4, leukemia N = 4, myeloma N = 1), followed by breast cancer, (N = 7, 25%). Other cancers included lung, (N = 3, 11%), carcinoid tumor, (N = 2, 7%), and one each of thymoma, sarcoma, esophagus, stomach, thyroid, larynx, and prostate. The median interval between the diagnosis of cancer and achalasia was 78 months (range 2- 364). In 21 patients, the cancer preceded the diagnosis of achalasia. All patients underwent endoscopy to rule out psuedoachalasia. Where high resolution manometry was done (N = 10/28, 36%), type 2 achalasia was seen in 8/10 patients and type 1 achalasia in 2/10 patients. In seven patients, achalasia was diagnosed before the cancer. The median time interval between diagnosis of achalasia and diagnosis of a primary malignancy in these patients was 72 months (range 12-354 months). Anti-Hu antibodies were present in 2/8 patients who were checked for it, suggestive of a paraneoplastic process. Eight patients underwent endoscopic dilatations, 12 had Botulinum Toxin A injection into the lower esophageal sphincter and 6 had both procedures. 7 patients underwent a Heller myotomy. One is awaiting surgery. Response to treatment was favorable when assessed for improvement in dysphagia and eating ability for 6 months after diagnosis in 24/ 28 patients (85%). CONCLUSION: Malignancy associated secondary achalasia is very rare. In our population heme associated malignancy and breast cancer were the 2 cancers most commonly associated with achalasia. Patients with cancer who develop dysphagia should be screened for achalasia, as response to treatment is favorable.

3099 Endoscopic Botulinum Toxin Injection for Tacrolimus-Induced Achalasia in a Renal Transplant Recipient

INTRODUCTION: Multiple medications have been associated with secondary achalasia. Here we present a patient who developed dysphagia 4 days after his renal transplant and was found to have achalasia thought to be due to his tacrolimus; he was successfully treated with botulinum injection. To our knowledge, this is the first case of tacrolimus-induced achalasia ever reported in a renal transplant patient; it is also the first case in a transplant patient to be treated endoscopically with botulinum injection. CASE DESCRIPTION/METHODS: A 58 year old man with a history of end stage renal disease secondary to focal segmental glomerulosclerosis presented with progressive renal failure and underwent deceased kidney transplant without complications. At clinic, follow-up on post-operative day #4, the patient reported difficulty in swallowing since post-operative day two. A modified barium swallow study (Figure 1) demonstrated severe esophageal dysmotility and lower esophageal sphincter dysfunction concerning for achalasia. Upper endoscopy (Figure 2) one week later revealed a dilated esophagus with mild edema but without focal lesions or ulcerations. Esophageal manometry (Figure 3) evidenced outflow obstruction of the esophagogastric junction and weak peristalsis in the esophageal body, confirming the diagnosis of achalasia. Surgical therapy and changing his calcineuron inhibitor for achalasia were considered too high risk in this patient so shortly after transplant. The patient ultimately underwent therapy with endoscopic botulinum toxin injection (100 units) of the lower esophageal sphincter. He had excellent results with immediate and complete resolution of dysphagia. DISCUSSION: The proposed mechanism for CNI induced changes in esophageal motility involves inhibition of nitric oxide synthase by CNIs. Nitric oxide (NO) is responsible for esophageal peristalsis as well as relaxation of the lower esophageal sphincter (LES). Although it is unclear why switching between different CNIs would lead to symptom resolution, individual variation in the reaction to the different chemical structures of tacrolimus and cyclosporine has been proposed. Botulinum toxin can be considered as a therapeutic option in those patients who are not candidates for a change in their immunosupression. To our knowledge, this is the first case of botulinum induced achalasia reported after a kidney transplant and also the first successfully treated with botulinum toxin injection.

1708 Case of Secondary Achalasia Presenting Several Years After Nissen Fundoplication With Improvement After Endoscopic Dilatation: “Rare but Not Forgotten”

INTRODUCTION: Secondary Achalasia is an esophageal motor disorder presenting with clinical, radiographic and manometric features that mimic those of primary achalasia except that it has a known etiology such as gastroesophageal junction (GEJ) tumor, antireflux surgery, gastric banding or gastrectomy. We present a rare case of secondary achalasia as a remote consequence of a laparoscopic Nissen fundoplication (LNF). CASE DESCRIPTION/METHODS: A 59 year old woman with a history of diabetes mellitus, gastroesophageal reflux disease (GERD), hiatal hernia status post LNF with hernia repair eight years ago, presented with dysphagia to solids and liquids with a 24-pound weight loss over the last 3 months. On presentation, physical exam and initial laboratory data were unremarkable. Computerized tomography of the chest showed esophageal dilatation with an obstruction at the GEJ. Upper endoscopy revealed a large amount of impacted food in the lower third of the esophagus which was removed with a Roth net, and the GEJ was traversed by the scope with significant resistance. On retroflexion evidence of a Nissen fundoplication was found at the GEJ with a tight appearing wrap. The GEJ was dilated using Savary dilators over a guide up to a diameter of 14 mm with remarkable improvement of the symptoms. An esophagogram done post dilatation showed transit of the barium contrast through the GEJ. DISCUSSION: GERD is the most common pathologic condition affecting the upper gastrointestinal tract. While most patients respond to medical therapy, LNF has become the gold standard for antireflux surgery in patients with medically refractory disease. Transient dysphagia occurs in 40% to 70% of patients after LNF in the early postoperative period due to edema, slipped, migrated or too tight fundoplication or a missed diagnosis of primary achalasia and nutcracker esophagus. However, it can also occur many years after the operation due to hiatal stenosis secondary to severe fibrotic reaction and anterior angulation of the GEJ. EGD is a good initial diagnostic tool and resistance or inability to pass a gastroscope through the EGJ should raise suspicion of secondary achalasia with a background history of LNF. GEJ tumor should always be ruled out with careful examination. Dilatation of the GEJ is done using wire guided Savary dilators or through the scope balloon dilators. Our case highlights the importance of an aggressive evaluation and careful interpretation of a patient presenting with dysphagia with a remote history of fundoplication.

Functional assessment of achalasia

The treatment of achalasia is dependent on evaluation of esophageal function of patients with this progressive disorder. This begins with an evaluation of its symptoms and their severity. The Eckardt scale is the most widely used, though it has limitations. Timed barium swallow evaluation provides a simple inexpensive means to evaluate esophageal emptying across a non-relaxing lower esophageal sphincter, before and after therapy. High resolution esophageal manometry has improved our ability to assess both esophageal body and sphincteric function. It has allowed identification of manometric phenotypes, which help prognosticate response to therapy. Esophageal impedance measurements of liquid swallows and esophageal emptying are a new and promising advance. Endoscopic ultrasound can measure esophageal muscle thickness, but has an adjunctive role mainly aimed at excluding structural abnormalities, such as malignancy causing secondary achalasia.

Do we need endoscopic ultrasonography for the workup of patients with esophageal motility disorder?

Endoscopic ultrasonography (EUS) is advised in the workup of achalasia patients to rule out secondary achalasia or pseudoachalasia, and search for a typical esophageal wall thickening. The purpose of this study was to assess the clinical contribution of EUS findings in achalasia and other esophageal motility disorders (EMD). We conducted a single center retrospective study at a tertiary referral centre. We included all patients with an EUS for the workup of a suspected EMD from January 2012 to December 2017. Sixty-nine patients were included, 52% were men, with a median (±SD) age of 61 ± 14 years. Median (±SD) Eckardt Score was 7 ± 2. EUS was normal in 26 (38%) patients, and showed an esophageal wall thickening in 43 (62%) patients. Three cases of secondary achalasia were diagnosed at mucosal biopsies: 2 esophageal carcinomas and one eosinophilic esophagitis. Esophageal wall thickening was not significantly associated with the type of EMD or achalasia subtype and there was no statistical correlation between the presence of a wall thickening at EUS and therapeutic outcomes. In our work, the presence of an esophageal wall thickening was not predictive of the type of EMD nor achalasia subtype or treatment outcome. The contribution of endoscopic ultrasonography in achalasia and other EMD seems limited.

Clinical Characteristics of Malignancy-associated Secondary Achalasia: An 18-Year Assessment at a Cancer Hospital

Introduction: Achalasia is an unusual disorder of esophageal motor abnormalities with an incidence of 1.6:100, 000 individuals. Achalasia secondary to neoplasia is extremely rare, the incidence is approximately 1:750,000 people. Achalasia in malignancy may be caused by direct infiltration of the lower esophageal sphincter, as a neoplastic infiltration of the myenteric plexus, pseudoachalasia, (PA) or as a paraneoplastic process. The purpose of this study is to evaluate the characteristics of achalasia associated with malignancy. Methods: Electronic medical records were retrospectively reviewed from January 1999 to May 2017 to identify patients with a diagnosis of achalasia and a malignancy. We identified 19 patients who had a nonesophageal malignancy and a diagnosis of achalasia. Age at diagnosis of cancer, at diagnosis of achalasia, sex, type of cancer and its stage, time interval between cancer and diagnosis of achalasia, treatment and responses were reviewed. Results: The median age at diagnosis of Achalasia was 56 years (19-85). There were 11 (58%) male and 8 (42%) female patients. The most common associated malignancy was Breast cancer, N=5 (27%), and Lymphoma, N=4 (21%). Other cancers included Lung, N=3 (16%), Carcinoid tumor, N=2 (11%), Myeloma, N=1 (5%), CLL, N=1 (5%), AML, N=1 (5%), Thymoma, N=1(5%) and Sarcoma, N=1 (5%). The median time interval between the diagnosis of cancer and a diagnosis of Achalasia was 58 months (range 2-120 months). In most patients the cancer diagnosis preceded the achalasia. All patients underwent confirmation by esophageal manometry. In 15/19 patients barium studies were available. All patients underwent endoscopy to rule out psuedoachalasia. In four patients achalasia was diagnosed before the cancer was diagnosed. The median time interval between Achalasia and onset of a primary malignancy in these patients was 24 months (range 12-72 months). Patients were treated for their primary cancers with chemotherapy, radiation and/or surgery. Eight patients underwent endoscopic dilatations, 11/19 had Botulinum Toxin A injection, 6/19 had both. Two underwent Heller myotomy. Response to treatment was favorable for 6 months after treatment, except in three patients. The median overall survival time was 3 years (0-16) after the achalasia onset. Conclusion: Malignancy associated secondary achalasia is very rare. The most common malignancy according to the literature is gastric but in our population breast cancer and lymphoma were seen.Table: Table. Clinical presentation of achalasia in cancer patients

Achalasia: A Review of Etiology, Pathophysiology, and Treatment

Achalasia was a condition marked by peristaltic movement absent in lower esophageal sphincter and segment that hypertonic result in imperfect relaxation during food ingestion. Achalasia incidence did not differ between men and women, account for 1 in 100.000 people every year with prevalence of 10 in 100.000 people, unrelated specifically with ethnic, and has its highest incidence on 30-60 age group.Based on its etiology, it was divided into primary and secondary Achalasia, while based on its motility, it was into hypermotil, hypomotil, and amotil Achalasia. Until present, several therapeutic modalities were available to treat Achalasia, among them was pharmacology therapy, botulinum toxin injection via endoscopy, pneumatic dilatation, Heller myotomy surgery, and Per Oral Endoscopy Myotomy (POEM).

Is secondary achalasia a reality? Primary versus secondary achalasia: Role of barium swallow

This study was conducted in the Department of Radiology Mayo Hospital, Lahore from January 2002 to December 2002. 30 Patients with findings on barium swallow of Achalasia were taken. Post procedure follow up was carried out either in the shape of endoscopic biopsy or post operative biopsy. The results showed that 25 out of 30 had Primary Achalasia and 5 had Secondary Achalasia. The results were compared with age of the patients and it was found that Secondary Achalasia, which was due to carcinoma of the lower esophagus or fundus of stomach, was virtually non existent before the age of 30. These were 5 patients all above 30 years of age.

Pseudoachalasia: A review.

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.