Category: Functional Bowel Disease

P2106 - Ehlers-Danlos Syndrome Type III (EDS) and Visceroptosis: Getting to the Bottom of This Diagnosis

Tuesday, October 9
10:30 AM – 4:00 PM



Category: Functional Bowel Disease       

Ali Rezaie, MD, MSc, Yaniv Raphael, MD, Richard Sukov, MD, Xiaochen Liu, MSc

Cedars-Sinai Medical Center, Los Angeles, CA

Introduction: EDS type III is the most common form of EDS, characterized by joint and ligamentous hyperlaxity. The diagnosis is made entirely by clinical parameters without any genetic testing available. Functional gastrointestinal (GI) symptoms are present in majority of EDS patients with variable severity. Visceroptosis, defined as prolapse of abdominal organs below their natural position, has been proposed as a cause of functional GI symptoms in EDS. To date, no objective radiographic measurement exists to determine and quantify visceroptosis/enteroptosis.

Methods: Subjects with EDS type III fulfilling Rome IV criteria for irritable bowel syndrome were included. Patients with previous abdominal surgeries were excluded. Clinical history and Beighton scores were recorded. Following ingestion of 16 oz barium, the passage of contrast was followed through the small bowel until it reached the colon. At that point, supine and upright radiographs of the abdomen were obtained. Measurements were calculated on supine and fully upright positions with respect to a reference line drawn across the top of the iliac crests. Dynamic measurements included: the lowest point of stomach, bottom of small bowel column in the pelvis, inferior tip of the liver, and top of jejunal column. (Figures 1 & 2) Correction for patients’ height was made by normalizing data to the height of T12 vertebral body.

Results: Seven subjects were enrolled (100% Female, mean age 28.3 years, mean Beighton score 6.9). All subjects had abdominal pain and bloating. Constipation, dyspepsia, and nausea were present in 84% and 14% had diarrhea. Gastric emptying delay and Postural Orthostatic Tachycardia Syndrome (POTS) was present in 3 and 4 subjects, respectively. All four visceroptosis measurements showed a descent in upright position (table 1). The most significant descents were seen with the top of jejunal column (7.6 cm) and the lowest point of stomach (5.3 cm). Measurement of the bottom of small bowel column in the pelvis showed the least quantifiable drop (2.0 cm). Lack of significant variability in symptoms did not allow for correlation analysis. Respectively, stomach descended 2.9 and 1.2 cm more in EDS patients with POTS or gastroparesis; however, it did not reach clinic significance

Discussion: Our study offers a simple, inexpensive and objective radiographic approach to quantify and define visceroptosis/enteroptosis in EDS patients. These results need to be validated in larger controlled-studies.

Disclosures: Does Not Disclose

Ali Rezaie indicated no relevant financial relationships.
Yaniv Raphael indicated no relevant financial relationships.
Richard Sukov indicated no relevant financial relationships.
Xiaochen Liu indicated no relevant financial relationships.



Figure 1.Baseline measurements for assessment of visceroptosis in supine position with respect to line drawn across iliac crest: (A) inferior tip of the liver, (B) bottom of small bowel column in the pelvis, (C) top of jejunal column, (D) lowest point of stomach.


Figure 2: Assessment of the drop of four measurements in upright position. (A) inferior tip of the liver, (B) bottom of small bowel column in the pelvis, (C) top of jejunal column, (D) lowest point of stomach.


Table 1: Absolute and normalized visceroptosis measurements in EDS type III patients.


Citation: Ali Rezaie, MD, MSc, Yaniv Raphael, MD, Richard Sukov, MD, Xiaochen Liu, MSc. EHLERS-DANLOS SYNDROME TYPE III (EDS) AND VISCEROPTOSIS: GETTING TO THE BOTTOM OF THIS DIAGNOSIS. Program No. P2106. ACG 2018 Annual Scientific Meeting Abstracts. Philadelphia, Pennsylvania: American College of Gastroenterology.

Ali Rezaie

Cedars-Sinai Medical Center
Los Angeles, California