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Case Reports
. 2018 Jun 8;10(6):e2768.
doi: 10.7759/cureus.2768.

Portal Hypertension and a Stiff Liver

Felicia D'Alitto  1 , Amina Scherz  2 , Cristina Margini  1 , Hendrik Von Tengg-Kobligk  3 , Matteo Montani  4 , Thomas Pabst  5 , Annalisa Berzigotti  6
Affiliations
Case Reports

Portal Hypertension and a Stiff Liver

Felicia D'Alitto et al. Cureus. .

Abstract

Portal hypertension (PH) is a common clinical syndrome leading to severe complications. In the western world, about 90% of cases of PH are due to liver cirrhosis, and thanks to the availability of ultrasound elastography methods, this diagnosis is usually confirmed at bedside. We report a case of a patient presenting with PH and ascites initially suspected of suffering from liver cirrhosis. The finding of large hepatomegaly and a massive increase in liver stiffness prompted us to perform a liver biopsy. This revealed no fibrosis, but diffuse primary amyloidosis (AL amyloidosis). We discuss the diagnostic and treatment of this case, with emphasis on non-invasive imaging methods available for diagnosis and follow up.

Keywords: amyloidosis; elastography; hvpg; liver biopsy; liver cirrhosis; multiple myeloma.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Ultrasound and 2D-SWE findings at presentation (October 2015, upper row) and 18 months after diagnosis (ongoing VGPR to chemotherapy; March 2017, lower row)
The antero-posterior diameter of the right liver lobe measured at the mid-clavicular line was markedly enlarged on presentation (Panel A, arrow) and decreased on VGPR (Panel B, arrow). Similarly, liver stiffness by 2D-SWE was very high on presentation (Panel C, arrow) and decreased markedly on VGPR (Panel D, arrow). Intrahepatic portal blood flow was reversed (hepatofugal) on presentation (Panel E, arrow) and returned to normality (hepatopetal) on VGPR (Panel F, arrow). In addition patency of paraumbilical vein was noted on presentations (Panel G, arrow), and was no longer seen on VGPR (Panel H, arrow). 2D-SWE: 2-dimensional shear wave elastography; VGPR: very good partial response.
Figure 2
Figure 2. Findings on cross sectional imaging on presentation and during the follow-up
Computed tomography (CT) scan on presentation showing a clearly enlarged liver (Panel A). Magnetic resonance imaging (MRI) in February 2016 showed a stable hepatomegaly (Panel B), which improved substantially on VGPR one year later (volumetric reconstruction of MRI liver images; Panel C). VGPR: very good partial response.
Figure 3
Figure 3. Liver histology on diagnosis
Extensive amyloid deposition with obliteration of sinusoids was observed (arrows). Hematoxylin & Eosin 200x.
Figure 4
Figure 4. Liver histology on diagnosis
Extensive amyloid deposition with obliteration of sinusoids was observed (arrows). Congo red 100x.
See this image and copyright information in PMC

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