Korean J Radiol.  2008 Aug;9(4):386-389. 10.3348/kjr.2008.9.4.386.

Radiological Findings in a Case of Multiple Focal Nodular Hyperplasia Associated with Portal Vein Atresia and Portopulmonary Hypertension

• Lee IJ
• Jeong SH
• Choi JW
• Park HS
• Lee KH
• Kim H

• KMID: 1076468
• DOI: http://doi.org/10.3348/kjr.2008.9.4.386

Abstract

We present here the radiological findings of a rare case of multiple focal nodular hyperplasia that was associated with portal vein atresia and portopulmonary hypertension in a young woman. This case illustrates and supports the pathophysiological hypotheses that were previously proposed for the coexistence of these three abnormalities.

Keyword

Focal nodular hyperplasia; Portal vein atresia; Pulmonary hypertension; Computed tomography (CT), angiography

MeSH Terms

Adult
Female
Focal Nodular Hyperplasia/*radiography
Humans
Hypertension, Portal/*complications
Hypertension, Pulmonary/*complications
Portal Vein/*abnormalities

Figure

• 

  Fig. 1 30-year-old woman with multiple focal nodular hyperplasia, portal vein atresia and portopulmonary hypertension. A. Chest radiography shows enlarged pulmonary arteries (arrows) and cardiomegaly. B. Arterial (left side) and delayed (right side) phase CT images show multiple nodules (arrowheads) in liver. Note hypertrophied hepatic artery (arrow) and portal vein at hepatic hilum is not visualized. C. Lesions (arrowheads) show peripheral hyperintensity with central hypointense portion on T1-weighted image (left side) and they show peripheral hypointensity with central hyperintense portion on T2-weighted fast spin echo image (right side). D. On gadolinium enhanced dynamic MR scans, central hypointense portion of nodule (arrows) is filled with contrast media for length of time and they show persistent intense enhancement. E. On SPIO-enhanced T2-weighted gradient echo image, nodules show decreased signal intensity except for their central portions (C). F. Delayed phase of superior mesenteric arteriography shows absence of portal vein. Only fine collateral vessels are shown at hepatic hilum (open arrowheads). Flows in superior mesenteric vein (arrow) and splenic vein (arrowhead) drains into inferior vena cava (I) through collaterals. G. Low power magnification shows dense fibrous septum containing numerous vascular structures (left side, Hematoxylin & Eosin staining; original magnification, ×100). Vascular walls are thickened (arrowheads), surrounding hepatic parenchyme shows liver cell plates with increased thickness and well-formed bile ducts are absent in portal tract (right upper, Hematoxylin & Eosin staining; original magnification, ×200). Eccentric thickening of vascular wall is seen in portal tract (right lower, Masson's trichrome; original magnification, ×400).

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