Massive esophageal bleeding in long-standing achalasia complicated by esophageal carcinoma and aspirin-induced stasis ulcer

Abstract

Rationale: Esophageal hemorrhage may occasionally develop subsequent to esophagitis and stasis ulcer, but potentially fatal esophageal bleeding is very uncommon in primary achalasia. Patient concerns: We describe a case of a 64-year-old man with long-standing achalasia and megaesophagus who presented acute episodes of life-threatening upper gastrointestinal bleeding. Diagnoses and interventions: Five esophagogastroduodenoscopies (EGD) were conducted and during each large amount of static food, bloody material, and clots should be removed from the esophagus because of impaired esophageal transit. Eventually, diffuse multiple irregular ulcers were observed in the middle and lower portions of the esophagus that were presumed to have been caused by aspirin stasis based on considerations of previous drug use. EGD also revealed a 2.0 × 2.5 cm flat nodular lesion with central ulceration at the mid-to-lower esophagus and adherent blood clots suggestive of bleeding stigma. The biopsy specimen demonstrated esophageal cancer. Accordingly, a diagnosis of massive esophageal hemorrhage in long-standing achalasia complicated by squamous cell carcinoma, possibly triggered by acute mucosal irritation and ulcer caused by aspirin stasis, was made. The patient then successfully underwent the Ivor-Lewis operation. Resultantly, the tumor was diagnosed as moderately differentiated squamous cell carcinoma stage IIA (T2N0M0). Outcomes: The patient's postoperative course was uneventful, and no evidence of tumor recurrence or metastasis has been found during the 6 months of follow-up examination. He was tolerating normal food with only minimal reflux symptoms. Lessons: Although, fortunately in the described case, esophageal cancer was diagnosed at a relatively early stage because it is the acute presentation of life-threatening upper gastrointestinal bleeding, this report cautions that when symptoms of dysphagia are aggravated, taking drugs capable of acting as local irritants, such as aspirin, could cause fatal esophageal hemorrhage in achalasia.

A Rare Case of a Life-Threatening Massive Upper Gastrointestinal Bleed and Airway Obstruction in a Patient With a Megaesophagus Secondary to Longstanding Achalasia

“…Achalasia is an esophageal motility disorder that is characterized by impaired relaxation of the LES during swallowing and aperistalsis in the lower two-thirds of the esophagus due to parasympathetic denervation [2,7,10]. Although the exact etiology of achalasia has not been elucidated yet, the neuronal degeneration has been associated with neurodegenerative or autoimmune processes as patients present with increased serum neural autoantibodies and with histopathological evidence of a T-lymphocyte mediated reaction [14].…”

“…Upper GI bleeding in achalasia is an extremely rare phenomenon and is associated with underlying mucosal ulcers caused by pill esophagitis, malignancies, or stasis ulcers [2,[4][5]8]. Typically esophageal ulcers are formed due to the loss of the protective mucosal barrier caused by gastric acid refluxing through a weak LES as seen in GERD or repeated episodes of vomiting, as seen in bulimia nervosa [16].…”

“…Achalasia is an idiopathic primary motor disorder characterized by esophageal aperistalsis and incomplete relaxation of the lower esophageal sphincter (LES) [1][2][3]. The underlying pathophysiology is attributed to a functional loss of the myenteric plexus of Auerbach, resulting in an impairment of the inhibitory postganglionic neurons in the distal esophagus and LES [4][5][6].…”

“…Achalasia is a relatively rare disorder with an incidence rate of 1 in 100,000 and a prevalence of 1 in 10,000 in the United States with an equal distribution in males and females [2,6]. Clinically, the diagnosis of achalasia is often delayed because of its similar presentation with other more prevalent diseases such as gastroesophageal reflux disease (GERD) [6][7].…”

“…Furthermore, in extremely rare situations, long-standing primary achalasia can also result in fatal esophageal hemorrhage due to underlying mucosal irritation and ulcer formation [2,8,12]. To our knowledge, the etiology of upper gastrointestinal (GI) bleeds in achalasia were due to cytomegalovirus (CMV) esophagitis, esophageal varices, excessive ingestion of tannins, esophagopulmonary fistula, non-Hodgkin esophageal lymphoma, and excessive aspirin ingestion [2,[4][5][12][13]. We hope to further contribute to the current understanding of this topic by demonstrating a rare case of a patient with achalasia mediated megaesophagus who presented with acute respiratory obstruction and life-threatening esophageal bleeding from underlying pressure ulcers.…”