P3.47: Multimodality Surgical Management of Patients with Global Gut Dysmotility: Techniques and Long-Term Outcome

Introduction: Global gut dysmotility (GGD) is a severely disabling disorder with the potential to develop gut failure with impaired quality of life. With the lack of effective medical therapy and pacing technology, surgical management with different modalities including gut transplantation (GTx) has recently evolved as part of the multidisciplinary treatment approach for this orphan population. Methods: Between August 2012 and January 2019, a total of 172 patients with the diagnosis of GGD were referred to Cleveland Clinic, Center of Gut Rehabilitation and Transplantation (CGRT). TPN dependence was documented in 116 (76%). Of those, 82 (71%) underwent rehabilitative surgery (RS) and/or GTx. The diagnosis was confirmed by exclusion of gastrointestinal mechanical obstruction with radiologic evidence of global dysmotility including capsule endoscopy and sitz marker methodology. Most patients were adult female (88%) with extensive psychiatric history in more than 50% of the cases. Previous management included pharmacologic therapy, gastric pacing, partial colon resection, and external venting. Results: With the intent to treat or as a bridge to GTx, 62 (76%) underwent RS with completion colectomy and ileorectal anastomosis (n=47), pyloroplasty (n=27) and chimney ileostomy (n=40). GTx with liver-free (n=19) and liver-contained (n=1) allograft was performed in a total of 20 (24%). Of these transplant recipients, 7 (35%) failed RS at our institution. With a mean follow up of 32 + 15 months, 160 patients are currently alive with an overall survival rate of (93%). In those with TPN dependency, the survival rate was better after RS (94%) compared to GTx (80%). Restoration of oral tolerance with achievement of full nutritional autonomy was accomplished in 20 (32%) and 10 (63%) of the current survivors, respectively. Conclusion: Gut Rehabilitative surgery and transplantation are effective treatment modalities for GGD patients. RS can be utilized as a long-term therapy or bridge for gut transplantation.