PMC2847873_01
(a) Coronal plane maximum-intensity-projection reformatted CT image shows the fusiform dilatation of the main portal vein (thick arrow). There is also marked dilatation and tortuosity at coronary vein (thin arrow). Note that the caliber of superior mesenteric vein is normal (arrowhead). (b) Axial half-Fourier acquisition single-shot turbo spin-echo (HASTE) MR image demonstrates thick lymphatic channels in the retrocrural space (arrows). Also, there is hydnonephrosis at the right kidney. (c) MR-Lymphangiography image (a half-Fourier single-shot turbo spin-echo 2D sequence with breath-hold technique with maximum intensity projection (MIP)) demonstrates two tortuous tubular structures on each side of the aorta representing dilated cisterna chyli (thin arrows). The meshwork of saccular lymphatic channels in the lumbar region inferior to the cisterna represents the dilated lumbar lymphatics (thick arrows).
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fig1
On abdominal computerized tomography (CT) a giant portal vein aneurysm and dilated coronary vein (Figure 1(a)) was present. Also, grade IV right hydronephrosis due to stricture at the ureteropelvic junction (Figure 1(b)) was detected. For assesment of lymph drainage, magnetic resonance (MR) lymphangiography was performed which showed significant dilatation of cisterna chili and saccular changings of lymphatic channels (Figures 1(b)-1(c)). 
(a), (b) Edema of the submucosa and muscularis mucosa, and dilatation of the submucosal lymphatic vessels (asterixes). (c) Blood filled cavities (x) in the liver biopsy, (d) peritoneal biopsy displaying dilated lymphatic (asterixes) and capillary (v) vessels. m: mucosa, sm: submucosa, mm: muscularis mucosa, h: hepatocytes.
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fig2
In the histopathological examination of the endoscopic biopsy samples retrieved from the large bowel mucosa, dilated submucosal lymphatic vessels as well as mucosal and submucosal edema (Figures 2(a), 2(b)) were evident. Core biopsy of the liver showed peliotic changes, that is, blood filled cavities in the parenchyma not lined by endothelial cells (Figure 2(c)). Omental biopsy analysis showed nonspesific mild chronic inflammatory infiltration, proliferation of mesothelial cells while peritoneal biopsy analysis revealed diffuse lymphangiectasia and papillary proliferation of mesothelial cells with vascular congestion (Figure 2(d)).
Diffuse polypoid lesions sized 2-5 mm throughout the small intestine in capsule endoscopy.
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fig3
To rule out intestinal pathology, capsule endoscopy was performed which showed that diffuse polypoid lesions sized 2-5 mm throughout the small intestine was noted (Figure 3).