Gastric Electrical Stimulation for Gastroparesis Associated with Mitochondrial Disease and Dysautonomia in Children

Background: Gastroparesis (GP) is a debilitating condition in many children and is challenging to treat with limited therapies available. Gastric electrical stimulation has been used successfully in children with recalcitrant GP. Patients with dysautonomia syndromes (DA) or mitochondrial disorders (MD) frequently include GP as part of the symptom complex and are an especially challenging group to manage. We report the use and outcomes of GES in a group of patients with these conditions.

Methods: A retrospective analysis of patients who underwent either temporary or permanent GES was performed, and cases with DA or MD were included for this study. Clinical data including Gastroparesis Cardinal Symptom Index (GCSI) were collected and analyzed with a paired t test.

Results: Overall 275 patients underwent temporary and 152 permanent GES. 50 cases were found to have associated DA. Majority (88%) were female, with a mean age of 15.36 years and 19 had features of both DA and Ehlers-Danlos (EDS). 82% responded to temporary GES and had permanent implanted. In the patients with permanent GES (n=41), 24 reported improvement in GCSI scores at all visits with a reduction in mean GCSI score from16.44 to 9.95, with a mean follow up of 4.88 years. 14 patients improved their route of feeds while 5 maintained the same route of feeding, and 2 required supplemental nutrition. 14 patients required device removal due to progression of disease and remission.

12 patients with MD had temporary GES and 7 responders had a permanent. Responders were older than non-responders (2.6 vs. 5.6 years). Three of the patients with permanent GES were male, and all 7 had improvement in parent reported symptoms at 3 and 6-months and beyond. All had weight gain post GES placement, and improvement in route/volume of feedings. Hospital utilization reduced in these cases, with a long term mean follow up of 5.7 years, with one patient requiring explantation.

Conclusion: Patients with GP associated with DA or EDS, and MD are very challenging groups, requiring extensive care. We found GES to be very effective in selected patients with quality-of-life improvements and a significant reduction in GP symptoms, improved feeding method, and reduction in antiemetic medications needed. Consideration should be given to early use of GES in children with MD and DA syndromes with recalcitrant GP.