Congenital angiodysplasia in a woman presenting with idiopathic jejunal varicosis on angiography

Abstract

Angiography is a useful diagnostic tool in cases with massive gastrointestinal bleeding such as angiodysplasia and varicosis when endoscopy is not available. Angiodysplasia and varicosis have distinguishable characteristic features on angiography, such as the presence of a nidus, visible late-draining veins, and the typical vascular tuft. We recently treated a rare case of congenital angiodysplasia without the characteristic angiodysplasia features on angiography. Instead, the patient presented with a very rare case of idiopathic jejunal varicosis. A 42-year-old woman visited the emergency room with the chief complaint of melena for three days and a hemoglobin level of 5.9 g/dL. An abdominal CT angiogram showed varicosis at the jejunal mesentery. Angiography of the superior and inferior mesenteric arteries showed tortuous and dilated jejunal and ileal branches during the venous phase, suggesting a vascular malformation such as varicosis of the jejunum. Surgical exploration with intraoperative endoscopy revealed diffuse engorged veins and a 1.0-cm-diameter superficial ulcer covered with a blood clot that was 70 cm from the ligament of Treitz. A 100-cm segment of jejunum was resected. Histological examination revealed that the lesion was angiodysplasia, not varicosis. The final diagnosis was congenital angiodysplasia.

Keywords: Angiography; Congenital angiodysplasia; Idiopathic varicosis; Jejunum.

Fig. 1

Axial CT image of the arterial phase showing no abnormal vessels. (B) Axial CT image of the portal phase showing enhancing abnormal veins at the antimesenteric border of the small bowel (arrow). (C) Axial CT image of the portal phase showing enhanced abnormal veins of the mesentery at the small bowel loop (arrow). (D) Maximum-intensity projection reconstruction image of CT angiography showing tortuous abnormal veins in the left upper abdomen (arrow). (E) Superior mesenteric artery (SMA) arteriography of the arterial phase showing no abnormal vessels. (F) SMA arteriography of the portal phase showing enhancing abnormal veins at the mesentery of the small bowel loop (arrow).

Fig. 2

(A) Intraoperative endoscopy showing a 1.0-cm-diameter superficial ulcer covered with a blood clot that was 70 cm from the ligament of Treitz. (B) Gross examination revealed a 0.9-cm ulceration in the mucosa as a bleeding focus (arrowhead) and one blue-black lesion in the submucosal layer, with venous distension (arrow).

Fig. 3

Microscopic images of the blue-black-colored polypoid submucosal lesion (A and B) and subserosal lesion (C and D). A and C: Ectatic thin-walled vessels with a few smooth muscle cells (arrowheads). B and D: Some foci of vascular walls were lined by only a single layer of endothelial cells (arrows) (H&E staining; A, ×40: B, ×200: C, ×12.5: D, ×200).