Presentation, Diagnosis, and Management of Achalasia John E. Pandolfino, Peter J. Kahrilas  Clinical Gastroenterology and Hepatology  Volume 11, Issue 8, Pages 887-897 (August 2013) DOI: 10.1016/j.cgh.2013.01.032 Copyright © 2013 AGA Institute Terms and Conditions

Figure 1 Subtypes of achalasia. The 3 subtypes are based on esophageal body contractility and pressurization. Type I (left) is associated with absent peristalsis and no discernible esophageal contractility in the context of an elevated IRP. The esophagus is flaccid with some degree of dilatation as evidenced by the associated esophagogram. The IRP threshold value for impaired EGJ relaxation in type I (>10 mm Hg) is lower than for type II or III because there is no potential for esophageal body pressurization. Type II (center) is associated with abnormal EGJ relaxation and panesophageal pressurization in excess of 30 mm Hg. This pressurization is likely related to longitudinal muscle and (nonocclusive) circular contraction compressing the esophageal body compartment. In this example, the proximal esophagus is filled by air, which is evident by the associated esophagogram and the overlying impedance signal showing liquid (purple transparency) only in the distal esophagus. Type III achalasia (right) is associated with premature (spastic) contractions and impaired EGJ relaxation. The diagnostic criteria stipulate that at least 2 swallows be associated with a contraction with distal latency of less than 4.5 seconds; other swallows may have absent peristalsis or rapid contractions, and as in this example, panesophageal pressurization can also be seen. As in this example, the associated esophagogram for type III achalasia is often interpreted as esophageal spasm because this has an extreme corkscrew with a small diverticulum above the distal contraction. With permission from the Esophageal Center at Northwestern. Clinical Gastroenterology and Hepatology 2013 11, 887-897DOI: (10.1016/j.cgh.2013.01.032) Copyright © 2013 AGA Institute Terms and Conditions

Figure 2 The criteria for EGJ outflow obstruction are an abnormal EGJ relaxation pressure associated with some preserved weak or normal peristalsis, thereby not meeting the diagnostic criteria for types I, II, or III achalasia. Ultimately, this pattern may prove to be a phenotype of achalasia as in the case of (A), where the patient also had a large epiphrenic diverticulum. This patient was treated with a laparoscopic myotomy and diverticulectomy, with a good symptomatic and functional response. In contrast, EGJ outflow obstruction can also be associated with mechanical obstruction in the region of the EGJ, as proved to be the case with the patient illustrated in (B). The patient was reported to have a patulous EGJ, and a 9-mm endoscope passed with no resistance noted at the EGJ. However, the IRP was abnormal, and there was compartmentalized pressurization between the preserved peristaltic contraction and the EGJ. The associated esophagogram revealed a subtle stricture just proximal to the EGJ where passage of a 12.5-mm barium tablet was delayed. The patient responded to 18-mm balloon dilation and proton pump inhibitor therapy. With permission from the Esophageal Center at Northwestern. Clinical Gastroenterology and Hepatology 2013 11, 887-897DOI: (10.1016/j.cgh.2013.01.032) Copyright © 2013 AGA Institute Terms and Conditions

Figure 3 Pseudoachalasia. The patient was referred for HRM on the basis of a presumed diagnosis of achalasia from the timed barium esophagogram noting a dilated esophagus with esophageal retention at 5 minutes and a bird beak deformity at the EGJ. Although the patient met criteria for type I achalasia, there was a strong vascular signal noted on the EPT plot that raised suspicion for vascular compression. Endoscopic ultrasound revealed a large thoracic aneurysm compressing the distal esophagus. The patient underwent repair of the aneurysm, and her esophageal function subsequently improved with no esophageal retention on esophagogram. With permission from the Esophageal Center at Northwestern. Clinical Gastroenterology and Hepatology 2013 11, 887-897DOI: (10.1016/j.cgh.2013.01.032) Copyright © 2013 AGA Institute Terms and Conditions

Figure 4 Algorithm for the diagnosis of achalasia. Suspicion for achalasia should be high in patients presenting with dysphagia, especially when accompanied by chest pain or regurgitation in the context of a normal upper endoscopy that has ruled out structural or mucosal (eosinophilic esophagitis) abnormalities. In addition, findings on endoscopy, such as esophageal dilatation, retained food, or resistance at the EGJ, should raise suspicion for achalasia, and a careful retroflexed view of the EGJ is mandatory to evaluate for a subtle tumor. Once the suspicion of achalasia is raised, HRM is indicated to assess EGJ relaxation peristalsis. Six EPT diagnoses are potentially consistent with achalasia, with the caveats indicated on the flow chart. CT, computed tomography; EGD, esophagogastroduodenoscopy; EUS, endoscopic ultrasound; GERD, gastroesophageal reflux disease. Clinical Gastroenterology and Hepatology 2013 11, 887-897DOI: (10.1016/j.cgh.2013.01.032) Copyright © 2013 AGA Institute Terms and Conditions