An Adult Case of Congenital Extrahepatic Portosystemic Shunt Successfully Treated with Balloon-occluded Retrograde Transvenous Obliteration

Abstract

A 42-year-old woman visited our hospital due to syncope. Contrast-enhanced CT revealed portosystemic shunt, portal vein hypoplasia, and multiple liver nodules. The histological examination of a liver biopsy specimen exhibited portal vein hypoplasia and revealed that the liver tumor was positive for glutamine synthetase. The patient was therefore diagnosed with congenital extrahepatic portosystemic shunt type II, and with focal nodular hyperplasia (FNH)-like nodules. She had the complication of severe portopulmonary hypertension and underwent complete shunt closure by balloon-occluded retrograde transvenous obliteration (B-RTO). The intrahepatic portal vein was well developed at 1 year after B-RTO, and multiple liver nodules completely regressed. Her pulmonary hypertension also improved.

Keywords: B-RTO; FNH-like nodule; congenital extrahepatic portosystemic shunt; portopulmonary hypertension.

Figure 1.

Abdominal CT of portal vein hypoplasia and portosystemic shunt and histology of FNH-like lesions. (a, b) Abdominal contrast-enhanced CT showed tapering of the left and right portal vein (arrowhead). (c) Coronal CT images showed a narrowed main trunk of the portal vein. (d) Three-dimensional computed tomography showed shunt flow from the portal vein draining into the left renal vein. (e, f) A histological examination of the liver tissue revealed a decreased portal area and marked portal vein hypoplasia. No cirrhotic changes were observed. FNH: focal nodular hyperplasia

Figure 2.

Gadoxetic acid (Gd-EOB-DTPA) enhanced magnetic resonance imaging. (a, b) T1 and T2-weighted MRI images. (c-f) Following the administration of the contrast agent Gd-EOB-DTPA, enhancement of the nodule was observed at the arterial phase (c), the enhancement persisted through the portal phase (d), and delayed phase (e), and Gd-EOB-DTPA was taken up into the cells in the hepatocellular phase (f).

Figure 3.

Abdominal ultrasonography imaging. (a) B-mode imaging showed multiple low echoic masses. (b-d) Contrast-enhanced ultrasonography showed enhancement in the arterial phase and sustained enhancement in the portal and post-vascular phases.

Figure 4.

Histological findings of a biopsy sample from an FNH-like nodule in the liver. (a, b) Low and high magnification of Hematoxylin and Eosin staining sections. The liver cell density was slightly increased. (c) Reticulin staining showed reticulin fibrosis along the liver sinusoids. (d, e) Cells in the nodule were negative for glypican 3 (d) and HSP 70 (e). (f, g) Cells in the nodule were positive for glutamine synthetase (f) and serum amyloid A (g). (h) Berlin blue staining showed hemosiderin deposition.

Figure 5.

Chest X-ray, electrocardiography (ECG) and ultrasound imaging of the heart. (a) A chest X-ray showed remarkable hilar pulmonary artery dilatation; the cardiothoracic ratio was 62%. (b) ECG showed regular sinus rhythm and right ventricular hypertrophy. (c, d) The parasternal short-axis view showed an enlarged right ventricle and flattened ventricular septum. (e) A Color Doppler examination showed severe tricuspid regurgitation. The tricuspid regurgitation pressure gradient was calculated to be 85 mmHg.

Figure 6.

Balloon-occluded retrograde transvenous venography (B-RTV). (a) B-RTV revealed severe hypoplasia of the portal vein and dilated left gastric vein (LGV) and post gastric vein (PGV) before treatment. (b) Four months after the first embolization of PGV, B-RTV resulted in improvement of PGV dilatation and increased the blood flow of the portal vein, while LGV dilatation remained. (c) At 9 months after the first embolization (5 months after the embolization of LGV), B-RTV showed residual blood flow in the LGV with improvement of LGV dilatation.

Figure 7.

Abdominal CT and MRI findings after balloon-occluded retrograde transvenous obliteration (B-RTO). (a, b) Axial CT images after B-RTO showed development of intra- and extrahepatic portal veins. (c) Three-dimensional computed tomography imaging showed intra- and extrahepatic portal vein development. The coils were placed on the left and post gastric veins, shown in the image, and the shunt vessel disappeared completely at 6 months after B-RTO. (d) Gd-EOB-DTPA enhanced MRI at 6 months after B-RTO showed complete regression of multiple FNA-like lesions in the hepatobiliary phase.