eP515 - Management of Partially Obstructing Stage IV Colon Cancer in Patient with Ehlers-Danlos Type IV

Purpose/Background: Patients with Ehlers-Danlos Syndrome (EDS) type IV, the vascular type, are prone to spontaneous ruptures in both the vascular and gastrointestinal systems due to a defect in collagen III. Because of their risk for either spontaneous or iatrogenic perforation, patients with EDS type IV are often discouraged from screening colonoscopy. There are a variety of case reports describing the management of acute colon perforations in these patients; however, there are none which describe the non-emergent management of colon tumors. This report describes the presentation of a patient with EDS type IV and the planning strategy for his partially obstructing colon cancer.Methods/Interventions: This is a 60-year-old male with genetically confirmed EDS type IV, who presented to the emergency room with acute lower abdominal pain, without peritonitis. Imaging revealed apple-core lesion in the descending colon with low-grade partial obstruction, and three hypo-enhancing liver lesions, suggesting metastatic disease. Initial CEA was elevated at 9.2 ng/mL. Multidisciplinary discussions between colorectal surgery, gastroenterology and oncology services weighed the risks of non-operative management and subsequent obstruction versus risk of complications due to the friable nature of tissue in patients with EDS type IV. The concluding decision was early operative intervention with diverting transverse loop colostomy.Results/Outcomes: Preoperative planning included platelet function testing, as well as measuring the distance from the xiphoid process to the transverse colon via CT to give a road-map for where to make our incision. An upper midline incision was made over the expected position of the transverse colon, and once the abdominal cavity was entered the transverse colon was easily seen and pulled into view. A transverse loop colostomy was fashioned and intraoperative bleeding was easily controlled. Intra-operative antegrade endoscopy was performed which allowed for evaluation and biopsy of the colonic mass. Postoperatively, the patient had an uncomplicated course with early colostomy function and no bleeding complications. Unfortunately, the intraoperative biopsies obtained did not show evidence for malignancy, so the patient will undergo liquid tumor biopsy as per hematology/oncology recommendations and start chemotherapy based on pathologic results.Conclusion/

Discussion: Our patient presented with a partial bowel obstruction, without perforation. The case reports of patient with EDS type IV and colon pathology only address acute obstructions or perforations and subsequent management. These recommend Hartmann’s procedure with or without eventual ostomy reversal, total colectomy with end ileostomy or ileorectal anastomosis- all concluding the goal of minimal dissection due to high risk for anastomotic breakdown and vascular complications. We were able to extrapolate from case reports on how best to treat our patient in the non-emergent setting, with a focus on decreased tissue handling and dissection.Patients with EDS type IV rarely present with advanced colon cancer due to the rare incidence and shortened life expectancy of the disease. Due to their increased tendency to bleed, extremely friable tissue, and increased risk of perioperative complications, we recommend the least invasive surgical approach while still addressing the underlying problem. In this case, we attempted to minimize operative dissection by localizing the transverse colon based pre-operative imaging in order to create our diverting loop colostomy."Figure 1.

Cross sectional view of apple core lesion
Figure 2.

Coronal view of apple core lesion"