Primary Achalasia Research Articles

Idiopathic (primary) achalasia: a review.

Idiopathic achalasia is a primary esophageal motor disorder characterized by loss of esophageal peristalsis and insufficient lower esophageal sphincter relaxation in response to deglutition. Patients with achalasia commonly complain of dysphagia to solids and liquids, bland regurgitation often unresponsive to an adequate trial of proton pump inhibitor, and chest pain. Weight loss is present in many, but not all patients. Although the precise etiology is unknown, it is often thought to be either autoimmune, viral immune, or neurodegenerative. The diagnosis is based on history of the disease, barium esophagogram, and esophageal motility testing. Endoscopic assessment of the gastroesophageal junction and gastric cardia is necessary to rule out malignancy. Newer diagnostic modalities such as high resolution manometry help in predicting treatment response in achalasia based on esophageal pressure topography patterns identifying three phenotypes of achalasia (I-III) and outcome studies suggest better treatment response with types I and II compared to type III. Although achalasia cannot be permanently cured, excellent outcomes are achieved in over 90 % of patients. Current medical and surgical therapeutic options (pneumatic dilation, endoscopic and surgical myotomy, and pharmacologic agents) aim at reducing the LES pressure and facilitating esophageal emptying by gravity and hydrostatic pressure of retained food and liquids. Either graded pneumatic dilatation or laparoscopic surgical myotomy with a partial fundoplication are recommended as initial therapy guided by patient age, gender, preference, and local institutional expertise. The prognosis in achalasia patients is excellent. Most patients who are appropriately treated have a normal life expectancy but the disease does recur and the patient may need intermittent treatment.

Primary versus secondary achalasia: New signs on barium esophagogram.

Aim:To investigate new signs on barium swallow that can differentiate primary from secondary achalasia.Materials and Methods:Records of 30 patients with primary achalasia and 17 patients with secondary achalasia were reviewed. Clinical, endoscopic, and manometric data was recorded. Barium esophagograms were evaluated for peristalsis and morphology of distal esophageal segment (length, symmetry, nodularity, shouldering, filling defects, and “tram-track sign”).Results:Mean age at presentation was 39 years in primary achalasia and 49 years in secondary achalasia. The mean duration of symptoms was 3.5 years in primary achalasia and 3 months in secondary achalasia. False-negative endoscopic results were noted in the first instance in five patients. In the secondary achalasia group, five patients had distal esophageal segment morphology indistinguishable from that of primary achalasia. None of the patients with primary achalasia and 35% patients with secondary achalasia had a length of the distal segment approaching combined height of two vertebral bodies. None of the patients with secondary achalasia and 34% patients with primary achalasia had maximum caliber of esophagus approaching combined height of two vertebral bodies. Tertiary contractions were noted in 90% patients with primary achalasia and 24% patients with secondary achalasia. Tram-track sign was found in 55% patients with primary achalasia. Filling defects in the distal esophageal segment were noted in 94% patients with secondary achalasia.Conclusion:Length of distal esophageal segment, tertiary contractions, tram-track sign, and filling defects in distal esophageal segment are useful esophagographic features distinguishing primary from secondary achalasia.

Primary versus secondary achalasia: A diagnostic conundrum.

A coherent distinction between primary and secondary achalasia is considerably important to clinicians. This is because the therapeutic interventions for the two are entirely different. Whilst pneumatic dilation is the standard treatment in primary achalasia, the same treatment, if instituted in malignant stenosis carries potential risk to the patient and delays appropriate therapy of the underlying disorder. This distinction however, is extremely difficult. None of the available clinical, manometric, endoscopic or imaging criteria can reliably exclude secondary achalasia. However, suspicion may be raised and in such cases, an exhaustive work up including repeat biopsies and even surgery should be considered. In this review, we discuss the various problems in differentiating primary from secondary achalasia.

478 Surgical Endoscopic Vacuum Therapy for Defects of the Upper GI Tract

S A T A b st ra ct s of esophagogastric junction (EGJ) relaxation. Indeed, the diagnosis of achalasia is established by HRM on the basis of an IRP>15mmHg and absence of normal peristalsis in the esophageal body. Our aim was to assess the effect of Heller myotomy on IRP in achalasia patients. Patients and Methods: We evaluated all consecutive patients who underwent laparoscopic Heller myotomy as first treatment from 2009-2014 and had a HRM evaluation before and after surgery. Patients who had already been treated for achalasia (with Heller myotomy, endoscopic treatment) were excluded from the study. The diagnosis of primary achalasia was established by esophageal manometry on the basis of accepted esophageal motility characteristics (i.e. absence of normal peristalsis in the esophageal body). Symptoms were collected and scored using a detailed questionnaire for dysphagia, regurgitation, and chest pain; barium swallow, endoscopy, HRM were performed, before and 6 months after surgical treatment. Treatment failure was defined as a postoperative symptom score >10th percentile of the preoperative score (i.e. > 10). Results: 139 consecutive achalasia patients (M:F= 72:67) represented the study population. All the patients had 100% simultaneous waves but 11 had a IRP<15 mmHg. According to the HRM classification, patients were classified as having: 58 (42.3%) type I, 63 (46%) type II and 16 (11.7%) type III. At a median follow-up of 24 months, the symptom score was significantly lower after surgery (median preoperative score 18 [IQR 11-20] vs median postoperative score 0 [IQR 0-3]; p<0.0001). The resting LES pressure (median preoperatively 27 [IQR 19-36] vs median postoperatively 11 [IQR 8-14]; p<0.001) and IRP (median preoperatively 27.4 [IQR 20.4-35] vs median postoperatively 7.1 [IQR: 4.4-9.8]; p<0.001). The surgical procedure was completed laparoscopically in all the patients. The failures of surgical treatment were 7 (5%). At univariate analysis IRP was correlated with the gender, LES basal and resting pressure, and the dysphagia score. Conclusion: This is the first study evaluating the role of IRP in achalasia and its modifications after surgery. An increased preoperative IRP directly correlated with dysphagia severity in achalasia patients. Heller myotomy was able to resolve this symptom by reducing the IRP to a value lower than 10 mmHg.

New endoscopic indicator of esophageal achalasia: "pinstripe pattern".

Background and Study AimsEndoscopic diagnosis of esophageal achalasia lacking typical endoscopic features can be extremely difficult. The aim of this study was to identify simple and reliable early indicator of esophageal achalasia.Patients and MethodsThis single-center retrospective study included 56 cases of esophageal achalasia without previous treatment. As a control, 60 non-achalasia subjects including reflux esophagitis and superficial esophageal cancer were also included in this study. Endoscopic findings were evaluated according to Descriptive Rules for Achalasia of the Esophagus as follows: (1) esophageal dilatation, (2) abnormal retention of liquid and/or food, (3) whitish change of the mucosal surface, (4) functional stenosis of the esophago-gastric junction, and (5) abnormal contraction. Additionally, the presence of the longitudinal superficial wrinkles of esophageal mucosa, “pinstripe pattern (PSP)” was evaluated endoscopically. Then, inter-observer diagnostic agreement was assessed for each finding.ResultsThe prevalence rates of the above-mentioned findings (1–5) were 41.1%, 41.1%, 16.1%, 94.6%, and 43.9%, respectively. PSP was observed in 60.7% of achalasia, while none of the control showed positivity for PSP. PSP was observed in 26 (62.5%) of 35 cases with shorter history < 10 years, which usually lacks typical findings such as severe esophageal dilation and tortuosity. Inter-observer agreement level was substantial for food/liquid remnant (k = 0.6861) and PSP (k = 0.6098), and was fair for abnormal contraction and white change. The accuracy, sensitivity, and specificity for achalasia were 83.8%, 64.7%, and 100%, respectively.Conclusion“Pinstripe pattern” could be a reliable indicator for early discrimination of primary esophageal achalasia.

Extragastroesophageal Malignancy-Associated Secondary Achalasia: A Rare Association of Pancreatic Cancer Rendering Alarm Manifestation.

Secondary achalasia or pseudoachalasia is a rare esophageal motor abnormality, which mimics primary achalasia; it is not easily distinguishable from idiopathic achalasia by manometry, radiological examination, or endoscopy. Although the majority of reported pseudoachalasia cases are associated with neoplasms at or near the esophagogastric (EG) junction, other neoplastic processes or even chronic illnesses such as rheumatoid arthritis can lead to the development of pseudoachalasia, for example, mediastinal masses, gastrointestinal (GI) tumors of the liver and biliary tract, and non-GI malignancies. Therefore, even if a patient presents with the typical findings of achalasia, we should be alert to the possibility of other GI malignancies besides EG tumors. For instance, pancreatic cancer was found in the case reported here; only four such cases have been reported in the literature. A 47-year-old man was admitted to our center with a 3-month history of dysphagia. His endoscopic and esophageal manometric findings were compatible with primary achalasia. However, unresponsiveness to diverse conventional achalasia treatments led us to suspect secondary achalasia. An active search led to a diagnosis of pancreatic mucinous cystadenocarcinoma invading the gastric fundus and EG junction. This rare case of pseudoachalasia caused by pancreatic carcinoma emphasizes the need for suspecting GI malignancies other than EG tumors in patients refractory to conventional achalasia treatment.

Laparoscopic management of achalasia cardia

Background: Primary idiopathic Achalasia is a quite rare disease. Achalasia Cardia is a motor disorder of the esophagus and lower esophageal sphincter. More specifically, it is a neuromuscular disorder characterized by degenerative changes of the myenteric plexus leading to a selective loss of inhibitory nerve endings. Irreversible disruption of peristaltic contractions and impaired relaxation of the lower esophageal sphincter in response to deglutition are the consequences of this damage. There is always a need to find out the best possible management options for this condition. Methods: We studied 22 cases of Achalasia Cardia in a span of 2 years from November 2012 to November 2014. To confirm the diagnosis we subjected the patients to barium swallow, upper GI endoscopy and CT scanning. Esophageal Manometry and 24hr PH monitoring were not done, because of non-availability of it in our institute. Operative technique used was Laparoscopic Heller’s Cardiomyotomy with Dor’s 180 degree Partial Anterior Fundoplication. Results: Following laparoscopic surgery there was significant improvement in preoperative symptoms like dysphagia (95.5%), regurgitation (95.5%), chest pain (90.90%) and epigastric pain (100%). After addition of anti-reflux procedure there was significant reduction in post-operative reflux without development of any dysphagia which has to be noted. Conclusions: Laparoscopic Heller’s Cardiomyotomy with Dor’s Procedure is feasible, safe and better than open procedures. Laparoscopic Heller’s Cardiomyotomy should be the management of choice in achalasia cardia in expert hands and proper setup.

Endoscopic pneumatic dilation versus botulinum toxin injection in the management of primary achalasia.

Achalasia is an oesophageal motility disorder, of unknown cause, which results in increased lower oesophageal sphincter (LOS) tone and symptoms of difficulty swallowing. Treatments are aimed at reducing the LOS tone. Current endoscopic therapeutic options include pneumatic dilation (PD) or botulinum toxin (BTX) injection. To undertake a systematic review comparing the efficacy and safety of two endoscopic treatments, PD and intrasphincteric BTX injection, in the treatment of oesophageal achalasia. Trials were initially identified by searching MEDLINE (1966 to August 2008), EMBASE (1980 to September 2008), ISI Web of Science (1955 to September 2008), The Cochrane Library Issue 3, 2008. Searches in all databases were conducted in October 2005 and updated in September 2008 and April 2014. The Cochrane highly sensitive search strategy for identifying randomised trials in MEDLINE, sensitivity maximising version in the Ovid format, was combined with specific search terms to identify randomised controlled trials in MEDLINE. The MEDLINE search strategy was adapted for use in the other databases that were searched. Randomised controlled trials comparing PD to BTX injection in individuals with primary achalasia. Two review authors independently performed study quality assessment and data extraction. Seven studies involving 178 participants were included. Two studies were excluded from the meta-analysis of remission rates on the basis of clinical heterogeneity of the initial endoscopic protocols. There was no significant difference between PD or BTX treatment in remission within four weeks of the initial intervention; with a risk ratio of remission of 1.11 (95% CI 0.97 to 1.27). There was also no significant difference in the mean oesophageal pressures between the treatment groups; with a weighted mean difference for PD of -0.77 (95% CI -2.44 to 0.91, P = 0.37). Data on remission rates following the initial endoscopic treatment were available for three studies at six months and four studies at 12 months. At six months 46 of 57 PD participants were in remission compared to 29 of 56 in the BTX group, giving a risk ratio of 1.57 (95% CI 1.19 to 2.08, P = 0.0015); whilst at 12 months 55 of 75 PD participants were in remission compared to 27 of 72 BTX participants, with a risk ratio of 1.88 (95% CI 1.35 to 2.61, P = 0.0002). No serious adverse outcomes occurred in participants receiving BTX, whilst PD was complicated by perforation in three cases. The results of this meta-analysis suggest that PD is the more effective endoscopic treatment in the long term (greater than six months) for patients with achalasia.

Diagnostics and therapy of achalasia

The low incidence (1:100,000) makes primary idiopathic achalasia a problem of special importance. Patients often have a long medical history of suffering before the diagnosis is established and adequate therapy provided. Surgeons who perform antireflux surgery must be certain of detecting achalasia patients within their collective of gastroesophageal reflux disease (GERD) patients to avoid contraindicated fundoplication. The current gold standard for establishing the diagnosis of achalasia is manometry. Especially in early stages, symptom evaluation, endoscopy and barium swallow lack adequate sensitivity. High-resolution manometry (HRM) is increasingly used and allows characterization of different achalasia types (i.e. type I classical achalasia, type II panesophageal pressurization and type III spasmodic achalasia) and differentiation from other motility disorders (e.g. distal esophageal spasm, jackhammer esophagus and nutcracker esophagus). For patients over 45 years of age additional endoscopic ultrasound and computed tomography are recommended to exclude pseudoachalasia. A curative treatment restoring normal esophageal function does not exist; however, there are good options for symptom control. Therapy aims are abolishment of dysphagia, improvement of esophageal clearance, prevention of reflux and abolishment of chest pain. The current standard treatment is cardiomyotomy, which was first described 100 years ago by the German surgeon Ernst Heller and has been shown to be clearly superior when compared to endoscopic treatment (e.g. botox injection and balloon dilatation). Heller's myotomy procedure is preferentially performed via the laparoscopic route and combined with partial fundoplication. Currently, an alternative to performing Heller's myotomy via the endoscopic route is under intensive investigation in several centers worldwide. The peroral endoscopic myotomy (POEM) procedure has shown very promising initial results and warrants further clinical evaluation.

Utility of chest CT for differentiating primary and secondary achalasia

To determine the computed tomography (CT) findings of primary and secondary achalasia and to assess the utility of CT for differentiating these conditions. A computerized search revealed 13 patients with primary achalasia and 15 with secondary achalasia who underwent chest CT during a 10-year period. The images were reviewed to determine whether there was distal oesophageal narrowing (including the length/contour of narrowing), oesophageal dilation, oesophageal wall thickening (including degree/symmetry/pattern of thickening), a soft-tissue mass at the gastro-oesophageal junction, mediastinal adenopathy, or other findings of malignant tumour. Eleven (85%) of 13 patients with primary achalasia had distal oesophageal narrowing at CT that was smooth in all patients; four (31%) had oesophageal wall thickening that was smooth and symmetric in all patients; none had a soft-tissue mass at the gastro-oesophageal junction or mediastinal lymphadenopathy; and two (15%) had pulmonary metastases from unrelated lung cancers. In contrast, 12 (80%) of 15 patients with secondary achalasia had distal oesophageal narrowing at CT; 11 (73%) had distal oesophageal wall thickening that was nodular/lobulate and asymmetric in seven (64%) and smooth and symmetric in four (36%); six (40%) had a soft-tissue mass at the gastro-oesophageal junction; seven (47%) had mediastinal lymphadenopathy; and all 15 had other findings of malignant tumour. CT is a useful technique for differentiating primary and secondary achalasia. Distal oesophageal wall thickening that is nodular/lobulate and asymmetric, a soft-tissue mass at the gastro-oesophageal junction, mediastinal lymphadenopathy, and pulmonary, hepatic, or osseous metastases are findings that favour secondary achalasia.

Association between gastroesophageal reflux disease after pneumatic balloon dilatation and clinical course in patients with achalasia.

Background/AimsThe occurrence of gastroesophageal reflux disease (GERD) is known to be associated with lower post-treatment lower esophageal sphincter pressure in patients with achalasia. This study aimed to elucidate whether GERD after pneumatic balloon dilatation (PD) has a prognostic role and to investigate how the clinical course of GERD is.MethodsA total of 79 consecutive patients who were first diagnosed with primary achalasia and underwent PD as an initial treatment were included in this retrospective study. Single PD was performed using a 3.0 cm balloon. The patients were divided into two groups: 1) who developed GERD after PD (GERD group) and 2) who did not develop GERD after PD (non-GERD group). GERD was defined as pathological acid exposure, reflux esophagitis or typical reflux symptoms.ResultsTwenty one patients (26.6%) developed GERD after PD during follow-up. There were no significant differences between the two groups in demographic or clinical factors including pre- and post-treatment manometric results. All patients in GERD group were well responsive to maintenance proton pump inhibitor therapy including on demand therapy or did not require maintenance. During a median follow-up of 17.8 months (interquartile range, 7.1–42.7 months), achalasia recurred in 15 patients (19.0%). However, the incidence of recurrence did not differ according to the occurrence of GERD after PD.ConclusionsGERD often occurs after even a single PD for achalasia. However, GERD after PD is well responsive to PPI therapy. Our data suggest that GERD after PD during follow-up does not appear to have a prognostic role.

Timing of Pharyngeal Swallow Events in Chagas' Disease.

BackgroundEsophageal involvement by Chagas’ disease causes a significative decrease in the number of neurons of the esophageal myenteric plexus, causing an impairment of esophageal motility with the same alterations described in primary esophageal achalasia. There is also a longer duration of pharyngeal transit, which might be consequent of the involvement of the central control of swallowing by the disease, or an adaptation of the pharynx to the difficult bolus transit through the esophagus, which could contribute to the complaint of dysphagia.MethodsWe studied, by videofluoroscopy, the sequence and timings of pharyngeal bolus transit in 16 patients with esophageal involvement by Chagas’ disease and 12 healthy volunteers. Each subject swallowed in duplicate 5 mL and 10 mL of liquid and paste boluses.ResultsThere was no difference between Chagas’ disease patients and normal volunteers in the sequence and timing of events associated with pharyngeal bolus flow, for liquid and paste boluses, and for 5 mL and 10 mL.ConclusionThe timing and sequence of swallow pharyngeal events of patients with Chagas’ disease do not differ from that of control subjects, which suggested that the central control of swallowing is not impaired by the disease.

For Patients with Primary Achalasia the Clinical Success of Pneumatic Balloon Dilatation Can Be Predicted from the Residual Fraction of Radionuclide During Esophageal Transit Scintigraphy

Esophageal transit scintigraphy (ETS) and esophagography have long been used to evaluate patients with achalasia. The objectives of our study were to evaluate the efficacy of endoscopic pneumatic dilatation (EPD) as treatment for Koreans with achalasia and to determine which findings from ETS and esophagography predict successful treatment of achalasia. Patients with achalasia who were treated by EPD between April 2002 and January 2012 were recruited. We defined the success of EPD as 6months or more of clinical remission without symptoms or a decrease in the Eckardt scores by at least two points and a total Eckardt score not exceeding 3. We reviewed the percentage of maximum scintigraphic activity retained in the esophagus at 30s (R 30) and the post-PD rate of reduction of R 30 ((Pre R 30-Post R 30)/Pre R 30×100) by ETS. Possible predictive factors determined by ETS and esophagography were analyzed. Our study included 53 eligible patients. The median symptom score (Eckardt score) was 5 (4-8). R 30 and T 1/2 were, respectively, 61.8% and 38.5min before EPD and 20% and 4.19min after EPD. Successful EPD was achieved for 40 of 53 (75.47%) patients. Age (≥40, p=0.027) and post-PD rate of reduction of R 30 (>20%, p=0.003) were best prognostic indicators of clinical success. There were no perforations related to EPD. Older age and a post-PD rate of reduction of R 30 were strongly associated with better outcomes. Examination with ETS before and after EPD can be used to objectively assess a patient's short-term response to EPD.

Su1516 The Residual Fraction of Radionuclide Is a Significant Marker for Prediction After Balloon Dilatation in Patients With Primary Achalasia

included elderly patients ( 60 years) with achalasia. The diagnosis was made based on clinic, endoscopic, radiologic and manometric parameters. Characteristics of the patients before, during and after BS placement were documented. Mellow score was used to evaluate dysphagia (0 no dysphagia/1 normal solids/2 dysphagia to soft solids/3 to solids and liquids/4 inability to swallow saliva), we measure the LES pressure and the barium column height at 1 min after swallow. The patients were followed weekly during the first three months after placement then each 15 days until the sixth month and then monthly P 0.05 was considered statically significant. Results: 10 patients with achalasia were included (7men and 3 women). Mean age was 76 7.8 years. Main symptoms were dysphagia and weight loss. Endoprostheses were successfully placed in all cases without complications however all presented chest pain during the first 72hours that was controlled with oral medication. 6 months (3-10) was the mean following period none of the stent was removed. The mean placement time was 35 8.3min. Migration occurred in 2 patients after 7 and 15days but none presented intestinal obstruction or other complication and both then received BTI. The mean degradation time was 80 20 days. Dysphagia score improved in all patients and 2 were able to eat normal diet at the end of the treatment. The mean basal LES pressure decreased 11.7 2.1mmHg (p 0.01) after treatment. Barium column height decreased 4.2 2.5cms after treatment (p 0.022). Final mean weight gain after treatment was 25 8% (p 0.017). Table 1 Conclusion: BS represents a safe and effective treatment in elderly patients with achalasia

Water Flush Technique: A Noninvasive Method of Optimizing Visualization of the Distal Esophagus in Patients With Primary Achalasia

The purpose of this study was to assess the use of a water flush technique to optimize visualization of the distal esophageal mucosa during barium esophagography of patients with moderate to severe primary achalasia. The water flush consists of having patients drink tap water at end esophagography in an attempt to clear the standing barium column. The water flush technique is a simple, noninvasive maneuver that improves the extent of diagnostic visualization of the distal esophageal mucosa in patients with moderate to severe primary achalasia. It facilitated detection of a tumor in one patient and identification of additional pathologic changes in two other patients in this series.

Contrast-enhanced ultrasound (CEUS) and CEUS-guided biopsy in the diagnosis of lung abscess in a patient with achalasia: Case report

Primary achalasia is a rare disorder of unknown aetiology characterized by dysfunction of esophageal motility secondary to degeneration of esophageal neurons. Some diseases, in particular neoplastic diseases, can cause symptoms similar to achalasia, and this condition is called pseudoachalasia. Pseudoachalasia can be indistinguishable from primary achalasia, and many investigations are often necessary to detect or exclude an underlying neoplasia. We report a case in which thoracic contrast-enhanced ultrasound (CEUS) and percutaneous CEUS-guided lung biopsy played a central role in differentiating aspiration pneumonia secondary to achalasia and complicated by lung abscess from pseudoachalasia secondary to lung carcinoma, after computed tomography (CT) yielded inconclusive findings. US contrast agent SonoVue® has an exclusively intravascular distribution, and its ability to discriminate between contrast vascular signal and tissue signal is greater than that of CT, making CEUS superior to CT in distinguishing perfused, viable tissues from avascular necrotic tissues. Although its use in pleuropulmonary pathology is still off-label, CEUS can play a useful role in characterizing peripheral pulmonary masses in selected cases.

Predictive Factors Associated with the Success of Pneumatic Dilatation in Japanese Patients with Primary Achalasia: A Study Using High-Resolution Manometry

Background/Aims: A new classification of achalasia using high-resolution manometry (HRM) has recently been suggested. Pneumatic dilatation (PD) is a common treatment for primary achalasia. The usefulness of the new classification and HRM for the treatment and follow-up of patients after PD is unknown. The aim of this study was to evaluate the PD effectiveness and the predictive factors of success in Japanese patients with achalasia using HRM and the new classification of achalasia. Methods: Twenty-five patients were diagnosed with primary achalasia using HRM and treated by PD in our hospital. We evaluated symptom scores and esophageal manometry 6 and 12 months after the first PD. Results: After the first PD treatment, remission occurred in 24 out of 25 (96.0%) patients at 6 months and in 19 out of 25 (76.0%) patients at 12 months. With the new classification of achalasia, the success rates were 83.3, 80.0 and 50% for types I, II and III, respectively, 12 months after PD. The median age of the successful group was significantly greater than that of the failure group (47.1 vs. 37.0 years, p <0.05). The median residual lower esophageal sphincter (LES) pressure 6 months after PD in the successful group was significantly lower than that of the failure group (9.0 vs. 15.5 mm Hg, p <0.05). Conclusion: Good predictors of PD success were old age (>40 years) and residual LES pressures less than 15 mm Hg 6 months after PD.

Pseudoachalasia Secondary to Intramural Esophageal Cancer Diagnosed by EUS-FNA

Purpose: Intramural esophageal malignancy is rare and can be indistinguishable from primary achalasia. EUS-FNA has been shown to be useful in the evaluation of pseudochalasia. We report a case of an intramural squamous cell carcinoma of the esophagus mimicking achalasia diagnosed by EUS-FNA. A 62-year-old woman s/p cholecystectomy presented with a 2-year history of right upper pain. MRCP revealed mild biliary dilation. She was lost to follow-up and returned years later with increasing epigastric pain, weight loss and progressive dysphagia. EGD with esophageal biopsies was unremarkable. She did not have improvement of her dysphagia with empirical dilation. An esophagram revealed concentric narrowing at distal esophagus with proximal esophageal dilation. Repeat EGD with biopsies was normal and an esophageal stricture was not seen. High resolution manometry was consistent with type II achalasia. EUS was performed to further evaluate the dilated biliary tree, revealing a mildly dilated CBD with normal tapering to the level of the ampulla. An intramural circumferential lesion was noted in the distal esophagus, the layer of origin could not be determined. FNA was performed which revealed squamous cell carcinoma. A subsequent PET scan demonstrated metastases to the liver, mediastinum, and retroperitoneal lymph nodes. This was staged T3N3M1. She was treated with chemo radiation. This case demonstrates the utility of EUS-FNA in the evaluation of pseudoachalasia. EUS should be considered in the work-up of suspected pseudoachalasia, especially if prior diagnostic modalities were unrevealing. A clinical suspicion of secondary achalasia should prompt use of EUS as it can identify intramural malignancy.

A Unique Case of Systemic Sarcoidosis Presenting as Pseudoachalasia with Involvement of the Gastric Cardia

Background: A 59-year-old African American male presented to the Gastroenterology Clinic with a nine month history of solid food dysphagia. Endoscopy revealed nodular erythematous tissue at the gastric cardia, biopsies showed noncaseating granulomas. Chest imaging demonstrated bilateral hilar lymphadenopathy, confirming the diagnosis of systemic sarcoidosis, and oral corticosteroid therapy was initiated. High-resolution manometry showed incomplete relaxation of the lower esophageal sphincter consistent with possible achalasia. Further evaluation with EUS showed paraesophageal lymphadenopathy causing extrinsic esophageal compression. The patient's dysphagia responded to serial esophageal dilations, however, he was referred to surgery for consideration for cardiomyotomy or lymphadenectomy as a definitive treatment. Discussion: Gastric involvement in patients with systemic sarcoidosis has been reported in 2.5% of cases. Although our patient developed granulomatous involvement of the gastric cardia, it is the gastric antrum that is most frequently affected. To our knowledge only one case of pseudoachalasia due to gastric cardia sarcoidosis has been previously reported, and was successfully treated with cardiomyotomy. Sarcoidosis has been implicated as a cause of pseudoachalasia in roughly 2-4% of symptomatic patients with a manometric pattern of primary achalasia. Previous reports have described dysphagia symptoms from mechanical compression by lymphadenopathy or granulomatous infiltration of the mucosal musculature and nerve fibers. Multiple cases of esophageal sarcoidosis have been reported in the literature; some of which responded to medical management with corticosteroid therapy, endoscopic dilations, or botulinum toxin injections. Definitive surgical interventions in the form of cardiomyotomy or lymphadenectomy will be pursued should the patient's symptoms become refractory to medical management. Conclusion: To our knowledge, our patient is the second reported case of pseudoachalasia secondary to gastric cardia sarcoidosis. Surgery has been suggested for patients with esophageal and gastric sarcoidosis in the form of cardiomyotomy or lymphadenectomy to patients refractory to medical management. Our patient, however, has responded to a combined medical and endoscopic approach.Figure: No Caption available.

Pattern of esophageal eosinophilic infiltration in patients with achalasia and response to Heller myotomy and Dor fundoplication

Eosinophilic esophagitis (EoE) is now recognized as a common cause of dysphagia. Eosinophilic infiltration of the esophagus has also been associated with other conditions, such as gastroesophageal reflux disease (GERD); however, the incidence, pattern, and clinical significance of eosinophilic infiltration in achalasia are poorly documented. We sought to characterize this histological finding in patients undergoing Heller myotomy (HM) for achalasia. Ninety-six patients undergoing laparoscopic HM for primary achalasia between 1999 and 2008 were identified from a prospective database. Serial mid and distal per-endoscopic esophageal biopsies taken from patients before and after surgery were assessed for the presence of elevated intraepithelial eosinophils (EIEs). Slides from patients with reports suggestive of EIE were reviewed independently by two pathologists, and the highest eosinophil count/high-power field (eos/hpf) was recorded. Dysphagia scores (0 = none to 5 = severe dysphagia), GERD health-related quality of life scores (0 = best to 45 = worst), and 24-hour pH results were compared before and 3 months after surgery. We related the highest eos to the symptoms and response to HM. Data are presented as median (range). Paired t-test and Wilcoxon signed-rank test determined significance, *P < 0.05. Of 96 patients with achalasia, 50 had undergone pre-HM biopsies revealing EIE in 17/50 (34%), with a median of 3 eos/hpf (1-21). Two patients were found to have superimposed esophageal candidiasis. One patient met the pathologic criteria for EoE. Twenty-five of 50 (50%) postoperative biopsies demonstrated a median of 5 eos/hpf (1-62) for a total of 28/50 patients (56%) with EIE in either the preoperative or postoperative period. Four patients (8%) met the pathologic criteria for EoE, and two demonstrated persistent esophageal candidiasis. A decrease in eosinophils was found in 6/28 patients (21%) from 3/hpf (1-21) to 0.5/hpf (0-4). Increase in eosinophils was found in 22/28 patients (79%) from 0.5/hpf (0-8) to 5/hpf (1-62). Preoperative and postoperative dysphagia scores were available in 23 patients. Dysphagia scores improved in 22/23 patients. (3 [0-5] to 0 [0-2])*. Preoperative and postoperative GERD scores were available in 21 patients. GERD scores improved in 20/21 patients (10 [3-38] to 2 [2-14])*. Four of 13 patients (30.7%) demonstrated significant reflux in the postoperative period. No difference in clinical response to HM was detected between patients with preoperative EIE compared with patients with no EIE. No correlation between postoperative esophageal pH and eos was observed. A significant number of patients with achalasia demonstrate esophageal eosinophilic infiltration even at numbers demonstrable in patients with EoE (8% 4/50). While the interaction between achalasia and esophageal eosinophilic infiltration needs further investigation, this does not represent a distinct clinical entity. Thus, the presence of esophageal eosinophils in patients presenting with dysphagia should not preclude further work-up for other etiologies, including achalasia.