Idiopathic mesenteric phlebosclerosis: a rare but important disease in Asian populations

Abstract

Idiopathic mesenteric phlebosclerosis (IMP) is a rare form of ischemic colitis that predominantly impacts Asian populations. Despite some recognizable signs, there is a significant lack of awareness about IMP. In this review, we explore the etiology, pathogenesis, imaging manifestations, endoscopic traits, and therapeutic modalities of IMP. In addition, we discuss the deficiencies in the current comprehension of IMP and the potential research orientations in future.

Keywords: Chinese herbal medicines; High venous pressure injury; Idiopathic mesenteric phlebosclerosis; Ischemic colitis; Venous calcification.

Figure1

A case from our institution's records. Thread-like calcification (white arrowhead) and intestinal obstruction (black arrowhead) on plain films (A). Prior to treatment, computed tomography revealed thickening and calcification (arrowhead) of the ascending colon (B). Colonoscopy showed dark purple discoloration and ulceration (arrowhead) of the mucosa pre-treatment (C). Repeat computed tomography and colonoscopy displayed healing ulcers with persistent dark purple mucosa (D), while bowel wall thickening resolved but mesenteric vein calcification persisted (E, arrowhead). Endoscopic biopsy specimens revealed inflammatory cell infiltration in the lamina propria, fibrous wall thickening, and hyalinization of small submucosal vessels (white arrowhead), along with collagenous fiber proliferation around the mesenteric vein (black arrowhead) (F, × 400)

Figure2

The pathogenesis hypothesis of IMP. High venous pressure injury: chronic diseases lead to increased venous blood pressure and abnormal shear stress, resulting in venous injury. Toxicity mechanism: Geniposide is metabolized to genipin and is absorbed into the mesenteric veins, causing intimal hyperplasia and thickening and fibrosis of the venous wall, resulting in “mummification” changes. Genipin can act directly on the intestinal wall and also lead to formation of blue color, staining the vein and intestinal wall during absorption. Both high venous pressure and toxic substances can lead to calcification and obstruction of the veins, subsequently cause damage to the intestinal wall, forming erosions /ulcers and fibrosis of intestinal wall. By Figdraw