Peroral endoscopic myotomy in a patient with sigmoid achalasia, end-stage liver disease, and esophageal varices as a bridge to liver transplantation. Peroral endoscopic myotomy is a minimally invasive endoscopic procedure for the treatment of achalasia and related esophageal motility disorders. The experience with this technique considered portal hypertension and compensated cirrhosis as contraindications to this procedure. There are a few case reports on the use of POEM in patients with cirrhosis with or without esophageal varices. In the largest series of 14 patients with achalasia and esophageal varices, botulinum toxin injection was performed in six patients, pneumatic dilation was performed in five, and POEM in three. Endoscopic methods illustrated in this case include submucosal injection, mucosal incision, submucosal dissection, full thickness, myotomy of the gastrointestinal wall, and clip closure. This case is a 66-year-old male with decompensated cirrhosis secondary to treated hepatitis C. He also had medically controlled ascites, lower extremity edema, encephalopathy, and esophageal varices. MELD score was 22. He related dysphagia for eight months to solids and liquids. Esophageal manometry confirmed type 2 achalasia. His current ECRT score was 8. Initial treatment was with pneumatic dilation using a 30-millimeter balloon, but this caused no change in symptoms. A repeat dilation with a 35-millimeter balloon was done and provided one month of symptom relief. Timed barium esophagram one month after 35-millimeter balloon dilation showed a dilated sigmoid esophagus, however, at one, two, and eventually at five minutes, 50% of the contrast ingested was retained. The patient was listed for a liver transplant at two different hospitals, however, surgeons at both institutions refused to operate due to his history of recurrent aspiration pneumonia and inability to reliably take oral medications and nutrition. After discussion with the patient, he was offered and agreed to undergo POEM. Labs showed a platelet count of 53,000 and INR of 2.4. A thromboelastography or TEG-guided preoperative transfusion strategy mandated two units of FFP and two units of platelets. The patient received an additional 10 milligrams of vitamin K and 30 minutes before POEM received a 0.3 microgram per kilogram IV infusion of DD-AVP. Upper endoscopy showed a tortuous sigmoid-shaped esophagus with at least three columns of varices measuring three to four millimeters in diameter. The lower esophageal sphincter was tight. There was mild portal gastropathy, but no evidence of gastric varices. An injection of a mixture of saline and methylene blue was injected along a posterior orientation. A mucosal incision was made, followed by tunnel entry. Injection of the same mixture with a T-type electrosurgical knife was made. A wide tunnel was created by submucosal dissection. Penetrating blood vessels were treated with coagulation forceps. In the soft coagulation mode, care was taken to minimize any risk of thermal injury to the overlying mucosa. At the lower esophageal sphincter, meticulous dissection was made to ensure a wide enough tunnel to access the lower esophageal sphincter. Dissection was continued into the gastric cardia for about two centimeters. The endoscope was withdrawn from the tunnel and retroflex BU confirmed adequate length of tunneling. Using endocut Q-mode, the electrosurgical knife was used to start the myotomy. A circular myotomy was intended to minimize the risk of any potential bleeding. The myotomy was carried out to the lower esophageal sphincter. At this level, the tunnel became quite tight. Therefore, an upward deflection and downward deflection of the electrosurgical knife was required to complete adequate circular myotomy. Myotomy was carried out about one centimeter into the gastric cardia. A circular myotomy measuring about six centimeters was performed. The endoscope was backed out of the tunnel and passage into the stomach demonstrated no evidence of resistance. The mucosal incision site was then closed with five clips to complete the procedure. The esophagram the same day after POEM showed less than 10% of the amount ingested remained at one minute. This was in distinction to esophagram performed prior to POEM, which showed 50% retention at five minutes. There were no post-procedure adverse events. The patient was admitted overnight and discharged the following day tolerating a full liquid diet. Three weeks after POEM, the patient underwent an uneventful liver transplantation and six months following POEM, ECRT score was zero. Clinical implications from our case are first, clinicians must be prepared to implement more invasive strategies if safer options fail to provide necessary outcomes. Second, therapeutic endoscopy in patients with end-stage liver disease requires a multidisciplinary discussion for safe and optimal management. Third, a thromboelastography or TEG-guided transfusion strategy may be part of a plan to minimize the risk of periprocedural bleeding in patients with end-stage liver disease. And finally, symptomatic relief of dysphagia after treatment in some of these high-risk patients may permit performance of additional life-saving care, such as liver transplantation. In conclusion, POEM may be successfully performed in patients with achalasia, end-stage liver disease, and esophageal varices. Additional evaluation of strategies to minimize the risk of adverse events associated with these patients is warranted.

peroral endoscopic myotomy

POEM

achalasia

liver transplantation

esophageal varices