Radiologic Identification of Visceroptosis in Patients With Hypermobile Ehlers-Danlos Syndrome (hEDS) With Functional Gastrointestinal (GI) Symptoms Compared to Healthy Subjects

Introduction: hEDS is the most common form of EDS, characterized by joint hyperlaxity and a supporting clinical or family history. Visceroptosis, defined as a prolapse of abdominal organs below their natural position, has been proposed as a cause of functional gastrointestinal (GI) symptoms in hEDS. To date, no definitive testing for visceroptosis exists. We aim to develop normative radiologic measurements for visceroptosis from healthy subjects and subsequently assess the prevalence of visceroptosis in hEDS patients. Methods: Healthy controls and subjects with hEDS fulfilling Rome IV criteria for functional GI symptoms were recruited. 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. Correction for patients’ height was made by normalizing data to the height of T12 vertebral body. hEDS subjects with visceroptosis were defined if any of their measurements exceeded two standard deviations above the mean established in the healthy control cohort. (Figure) Results: Eleven healthy and nine subjects with hEDS were enrolled (91% vs 100% Female, mean age 34 ± 13 vs 30 ± 8 years, mean Beighton score 1.8 ± 2.2 vs 7.4 ± 1.3). All hEDS subjects had abdominal pain and bloating. Constipation and mixed pattern were present in 78% and 22%, respectively. Values for the lowest point of stomach and top of the jejunal column how altered distribution between healthy and EDS subjects. Three (33%) hEDS subjects meet the radiographic criteria for visceroptosis. Neither Beighton score, height, weight, or BMI correlated with radiographic evidence of visceroptosis. Lack of significant variability in symptoms and functional GI testing prevented further correlation analysis. (Table) Conclusion: This is the first study to establish a normal range for the dynamic movement of the viscera during supine and upright radiographs of the abdomen. This will offer a simple and objective radiographic approach to define visceroptosis in hEDS patients.Figure 1.: Assessment for visceroptosis was performed by measuring the change in four defined landmarks in supine and upright position with respect to a line drawn across the 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. Table 1. - Normative values for radiographic measurements of visceroptosis in healthy controls along with values observed in hEDS subjects Landmarks Healthy controls hEDS subjects with GI symptoms Absolute change (cm mean ± std) Change corrected for vertebral height(cm mean ± std) Absolute change(cm mean ± std) Change corrected for vertebral height(cm mean ± std) Number of subjects with visceroptosis ( >2 standard deviation) Lowest point of stomach 8.99 (3.13) 2.92 (0.97) 9.89 (7.22) 3.25 (2.28) 3 Bottom of small bowel column 1.96 (1.89) 0.64 (0.59) 2.19 (1.70) 0.74 (0.58) 0 Inferior tip of the liver 4.28 (2.09) 1.47 (0.87) 3.60 (2.78) 1.19 (0.89) 0 Top of jejunal column 5.99 (2.02) 1.99 (0.73) 4.07 (2.91) 1.34 (0.90) 2