Baylor University Medical Center Proceedings April 2014, Volume 27, Number 2 | Page 43

DISCUSSION Edvard Ehlers (1863–1937) in Denmark and Henri-Alexandre Danlos (1844–1912) in France independently published lucid descriptions of the condition that would later bear both their names; Ehlers published in 1901 and Danlos in 1908 (1). Now many types of EDS are recognized. All three patterns of Mendelian inheritance are represented (autosomal dominant, autosomal recessive, and X-linked recessive) (2). EDS type IV, also known as the vascular type, is a rare connective tissue disorder with autosomal dominant transmission (McKusick catalog number 130050) caused by mutations in the COL3A1 gene. As a result, these patients have increased fragility of connective tissue with arterial, intestinal, and uterine ruptures. The estimated prevalence for types of EDS varies between 1 in 10,000 and 1 in 25,000, with EDS type IV representing approximately 5% to 10% of cases (3). The median age of death is 50 years (3, 4). Search of medical publications to 1959 revealed only 39 cases of isolated celiac artery dissections, making it the least common visceral artery dissection reported. Treatment options include surgery, endovascular repair, or medical management (3). 1. 2. 3. Figure. CT angiogram of the abdomen (sagittal view) showing an isolated celiac artery dissection. April 2014 4. Johns Hopkins Medicine. Online Mendelian Inheritance in Man. Available at www.omim.org. Genetests.org (for online information about genetic testing, and for brief reviews) Germain DP. Ehlers-Danlos syndrome type IV. Orphanet J Rare Dis 2007;2:32. Ganesamoni R, Agarwal S, Stephen E, Narayan RL. Isolated celiac artery dissection with splenic infarction: report of a case. Eur J Vasc Endovasc Surg 2007;13(4):58–59. Celiac artery disease and fatal rupture of a hepatic artery aneurysm in the Ehlers-Danlos syndrome 117