Eosinophilic myenteric ganglionitis (EMG) in a patient with chronic intestinal pseudo-obstruction and Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, and Stroke-like episodes (MELAS) syndrome: a case report and systematic literature review

Aims We performed endoscopic full thickness biopsy on a 33-year old woman with chronic intestinal pseudo-obstruction (CIPO). Due to stool impaction, we could only sample the rectum, showing no pathologic findings. Conservative treatment failed and the patient underwent Hartmann’s procedure, complicated by lactic acidosis and neurologic symptoms. Mitochondrial DNA 3243A>G mutation was identified and Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, and Stroke-like episodes (MELAS) syndrome was diagnosed. Surgical specimen histology showed eosinophilic myenteric ganglionitis (EMG). We investigated whether the concomitance of MELAS and EMG had been previously described [1] [2] [3] [4] [5] [6] [7] [8] [9] [10] [11] [12] [13] [14].

Methods We conducted a systematic literature review on PubMed using the searching terms “eosinophilic” + “myenteric” + “ganglion” and included all studies and case reports which described the finding of histological features of EMG.

Results Our search yielded a total of 41 results, among which 6 case series and case reports described a total of 12 cases of adult patients with histopathological diagnosis of EMG. Although concomitance of MELAS syndrome and CIPO has been previously reported, ours is the first case of EMG-associated CIPO in a MELAS patient.

Conclusions MELAS patients have a high risk of severe post-surgical complications. It has been recommended that the site of full thickness biopsy in CIPO should be the most dilated area. Unfortunately, stool impaction prevented endoscopic access to the affected colonic area in our late-presenting case. Conversely, if CIPO is suspected early, endoscopic full thickness biopsy sampling could avoid post-surgical diagnosis.

Conflicts of interest

Authors do not have any conflict of interest to disclose.

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Publication History

Article published online:
14 April 2023