Peroral endoscopic myotomy for achalasia combined with esophageal varices
Article information
Achalasia is a rare disease characterized by incomplete relaxation of the lower esophageal sphincter and impaired esophageal motility, with a mean incidence rate of 0.3 to 1.63 per 100,000 persons.1 Various treatment methods have been explored. Peroral endoscopic myotomy (POEM) is a promising treatment for achalasia than botulinum toxin injection and pneumatic dilatation and non-inferior to laparoscopic Heller myotomy regarding effectiveness.2-4 Patients with achalasia rarely present with esophageal varices. Persistent food stasis and progressive esophageal dilatation because of achalasia can increase the risk of esophagitis and ulcer formation, increasing the likelihood of variceal bleeding.5 Therefore, providing a safe and effective treatment in patients with achalasia and esophageal varices is important in terms of the increased risk of bleeding.
A multimodal approach is crucial in deciding whether to perform POEM in patients with achalasia and esophageal varices owing to concerns regarding bleeding and perforation. Endoscopy helps assess bleeding risk by evaluating the cut-surface area of varices or dilated vessels and identifying factors contributing to varix formation.6 Endoscopy is necessary to determine the grade and location of the varices and assess the severity and other characteristic features of achalasia.7 Besides assessing achalasia symptoms through questionnaires, its severity should be objectively evaluated using esophagography, high-resolution manometry, and EndoFLIP (Medtronic). Specifically, EndoFLIP provides three-dimensional images of the esophageal lumen, using high-resolution impedance planimetry to measure pressure, diameter, and volume changes, which help determine the extent of the myotomy.8 Moreover, identifying the cause of varices and assessing liver function using diagnostic tests, such as computed tomography, ultrasonography, FibroScan (Echosens), and relevant laboratory tests for factors related to portal hypertension, is important while considering treatment options for improvement. This thorough evaluation allows POEM to be performed to the appropriate length, guiding procedural decisions and addressing potential challenges effectively.
At our institution, a 40-year-old man with hepatitis B-related F2 grade varices and type 1 achalasia (Eckardt score of 9, EndoFLIP distensibility index 0.7 mm²/mmHg, non-sigmoid type) underwent POEM after taking propranolol (40 mg twice daily) for several months. The varices were on the anterior esophagus; therefore, the myotomy was directed to the opposite side, at the 6 o’clock position, with a 6-cm esophageal and 2-cm gastric myotomy. Minor thermal mucosal injuries were managed with clipping, and the procedure lasted 80 min. The patient improved significantly, with a final Eckardt score of 1 and no severe complications (Fig. 1).
Progression before and after the intervention observed in a case. (A) Esophageal varices are present up to the lower esophagus without a red sign (F2CbLi). (B) Esophageal varices are prominent on endoscopic ultrasonography. (C) Esophagogastrography reveals a dilated esophagus with passage disturbance. (D) Esophageal varices are observed during submucosa tunneling, and the peroral endoscopic myotomy is carefully conducted in a direction opposite to that of the varices. (E) When small full-thickness mucosal perforation occurred due to burning while performing myotomy, clipping was performed to close it. (F) On the 5-minute delayed esophagogram, no residual barium contrast is observed.
Effective management of bleeding or mucosal injuries is crucial in patients with achalasia and esophageal varices. Propranolol administered for several months before POEM, along with 50 μg of intravenous octreotide immediately before the procedure,9 can help reduce the risk of variceal bleeding. During POEM, the myotomy should be directed away from esophageal vascular changes, and diluted indigo carmine dye should be injected before dissection to visualize vessels and surrounding structures, minimizing the risk of thermal injury.10 Pre-emptive coagulation of dilated vessels is recommended to prevent bleeding from vessel laceration, with minor bleeding controlled using a coagrasper. For persistent bleeding, epinephrine injections and clipping are employed. In patients with severe cirrhosis, POEM may be performed after transjugular intrahepatic portosystemic shunt or variceal embolization.11 Reducing or eradicating varices with sclerotherapy, argon plasma coagulation, or band ligation before POEM may be considered.12,13 However, these interventions may induce submucosal fibrosis, increasing the risk of mucosal injury and complicating POEM.12 Nonetheless, appropriate strategies to address uncontrolled bleeding should be developed. Histoacryl injections have demonstrated long-term safety and efficacy in low-grade gastric varices.14 Cipolletta et al.15 reported that an emergency administration of histoacryl is safe and effective in treating acute bleeding from esophageal varices. Therefore, accurate injection can be considered a fast and safe method to stop bleeding during POEM, which is difficult to control with other techniques.
Although patients with achalasia and esophageal varices have an increased risk of bleeding during POEM, careful evaluation and the use of various treatment modalities before the procedure, effective bleeding prevention, and management during POEM can make it a successful treatment option even in such challenging case. All procedures were approved by our Ethics Committee and Institutional Review Board of CHA Gangnam Medical Center (approval number: GCI 2023-08-002).
Notes
Conflicts of Interest
Joo Young Cho is currently serving as a senior consultant of Clinical Endoscopy; however, he had not been involved in the peer reviewer selection, evaluation, or decision process of this article. Ah Young Lee has no potential conflicts of interest to declare.
Funding
None.
Author Contributions
Conceptualization: JYC; Data curation: AYL; Formal analysis: AYL; Investigation: AYL; Methodology: AYL; Supervision: JYC; Visualization: JYC; Writing–original draft: AYL; Writing–review & editing: JYC.