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

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