236 Eosinophilic Myenteric Ganglionitis: A Rare Cause of Chronic Intestinal Pseudo-obstruction. (11th January 2018)

• Record Type: Journal Article
• Authors: Agarwal, Indu; Ahmad, Muhammad; Watkin, William
• Is Part Of: American journal of clinical pathology. Volume 149(2018)Supplement 1
• Page Start: S100
• Page End: S101
• Publication Date: 2018-01-11
• Subjects: Diagnosis, Laboratory -- Periodicals; Pathology -- Periodicals; 616.07
• DOI: 10.1093/ajcp/aqx123.235
• Languages: English
• ISSNs: 0002-9173

Abstract

Abstract: Introduction: Eosinophilic myenteric ganglionitis (EMG) is a rare cause of chronic intestinal pseudo-obstruction (CIPO) characterized histopathologically by infiltration of the myenteric plexus by eosinophils. There are only five reported cases in the literature, four occurring in children and one in an elderly woman. Herein we present an additional case of this rare entity and discuss the clinicopathological features. Methods: A 49-year-old man presented to the emergency room with signs and symptoms of intestinal obstruction with obstipation. CT imaging demonstrated features suggestive of colonic obstruction at the level of sigmoid colon and a distal sigmoidectomy with colostomy was performed. Gross examination revealed a few shallow diverticula. Histopathological examination demonstrated marked eosinophilic inflammation concentrated within nerves and ganglia of the myenteric plexus, and focally in the adjacent muscularis propria and submucosa. Mucosal eosinophilia was not present and peripheral blood eosinophilia was absent too. His symptoms resolved after the resection and the patient is currently well after 25 months. Conclusions: EMG is a type of eosinophilic gastroenteropathy in which functional gastrointestinal obstruction is associated with eosinophilic inflammation involving nerves of the myenteric plexus. In contrast to more common forms of eosinophilic gastroenteritis, the mucosa, muscularis propria, and serosa are not significantly involved in EMG. It has been proposed that EMG results from myenteric neuronal expression of interleukin-5, a powerful chemoattractant for eosinophils. Similar to our case, all five previously reported cases have clinically demonstrated intestinal pseudo-obstruction. All pediatric cases were responsive to steroids with or without immunosuppressants. EMG should be considered in the clinical and histopathological differential diagnosis of motility and eosinophilic disorders of the bowel. The diagnosis can only be made in a full-thickness biopsy as mucosal involvement is typically absent.