Fig. 4

MRI findings in KTS with anorectal and anorectosigmoid VM. Panel A: Dilated, incompetent middle rectal vein (rightward arrow), perirectal VM (downward arrow), and thickening of rectal wall by VM involving (leftward arrow). Panel B: Sigmoid lumen became narrowed by thickening of the wall (asterisk). The mesentery of sigmoid colon become thickened and edematous (arrow). Panel C: Dilated, incompetent IIV (rightward arrow) is demonstrated. Typical fluid signal in the lumen is indicative of stagnation and/or reflux of blood. Panel D: Part of VM in rectosigmoid colon (rightward arrow) drains into the superior rectal vein (leftward arrows). Panel E and G: Ectatic and incompetent inferior mesenteric vein is identified (arrow). Heterogeneous signal in the lumen is indicative of blood stagnation, turbulent flow and reflux. Panel F: Dilation and tortuosity of the sigmoid vein in the mesentery (arrow). It drains into inferior mesenteric vein. Thickening and edema of the sigmoid mesentery also can be identified (asterisk). Panel H and I: Multiple cystic lymphatic malformations within the spleen (arrow). Splenectomy is indicated for massive splenomegaly (Panel I). Panel J: Main branches of portal vein become dilated (leftward arrow), and spleen enlarged (rightward arrow), which is suggestive of portal hypertension, although obstruction is not evident. Panel K: Main branches of portal vein become dilated and obstructed (arrow). Circumferential edema is notable. The spleen was resected because of portal hypertension and massive splenomegaly by lymphatic malformations