University of Oklahoma College of Medicine Oklahoma City, OK
Christopher A. Bouvette, MD, Katherine Janike, MD, Jalal Gondal, MD, Amir Rumman, MD University of Oklahoma College of Medicine, Oklahoma City, OK
Introduction: We describe the novel use peroral endoscopy myotomy (POEM) for an exceedingly rare presentation of calcineurin inhibitor (CNI) - associated achalasia.
Case Description/Methods: A 68-year-old male was referred for dysphagia, regurgitation, and chest spasms. He had history of allogenic bone marrow transplant for acute myeloid leukemia, complicated by refractory graft-versus-host-disease (GVHD). Thus, he was receiving tacrolimus. Dysphagia symptoms developed shortly after starting the tacrolimus. He had multiple admissions due to dehydration from poor oral intake and lost over 50lbs during this time. Eckhardt score (ESS) was 10/12.
Upper endoscopy revealed a tight LES. Endoscopic impedance planimetry revealed a distensibility index of 1.5 and minimal diameter of 7mm at 60cc inflation. The patient could not tolerate high resolution esophageal manometry. Timed barium esophagram demonstrated a 4cm standing column of contrast at 5 minutes.
Given the close temporal relationship between the onset of symptoms and the initiation of tacrolimus, we suspected CNI-induced achalasia. In discussion with the patient’s oncologist, discontinuation of tacrolimus or switching to an alternative was not possible given history of refractory GVHD with favorable treatment response to tacrolimus. Given high ESS and recurrent admissions, the patient was offered POEM.
A 9cm full-thickness posterior myotomy (6cm esophageal and 3cm cardiac) was performed. Post myotomy impedance planimetry showed minimal DI of 5 and minimal diameter of 15.5mm. The mucosotomy was closed with suture.
The patient resumed regular diet within 2 weeks with complete resolution of symptoms. ESS 1-month post-POEM was 0. Three months later, the patient remained symptom-free on tacrolimus.
Discussion: CNI-associated achalasia is exceedingly rare. This is the fourth case reported in the literature, and the first report of treatment with POEM. Prior case reports suggest discontinuation or switching to an alternative CNI. In our case, switching or discontinuing CNI therapy was not possible, and given the patient’s high ESS and multiple admissions for dehydration, Botox injection was felt to be insufficient. This case demonstrates POEM is effective treatment for CNI-associated achalasia and should be considered in patients with severe symptoms and when switching or discontinuing CNI therapy is infeasible.
Figure: Figure 1
Disclosures:
Christopher Bouvette indicated no relevant financial relationships.
Katherine Janike indicated no relevant financial relationships.
Jalal Gondal indicated no relevant financial relationships.
Amir Rumman indicated no relevant financial relationships.
Christopher A. Bouvette, MD, Katherine Janike, MD, Jalal Gondal, MD, Amir Rumman, MD. P4544 - Calcineurin Inhibtor-Associated Achalasia Treated With Peroal Endoscopic Myotomy, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.
