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

Hironori Tanaka; Yoshihito Saijo; Tetsu Tomonari; Takahiro Tanaka; Tatsuya Taniguchi; Shusuke Yagi; Koichi Okamoto; Hiroshi Miyamoto; Masahiro Sogabe; Yasushi Sato; Naoki Muguruma; Koichi Tsuneyama; Masataka Sata; Tetsuji Takayama

doi:10.2169/internalmedicine.5914-20

CASE REPORTS

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

Hironori Tanaka, Yoshihito Saijo, Tetsu Tomonari, Takahiro Tanaka, Tatsuya Taniguchi, Shusuke Yagi, Koichi Okamoto, Hiroshi Miyamoto, Masahiro Sogabe, Yasushi Sato, Naoki Muguruma, Koichi Tsuneyama, Masataka Sata, Tetsuji Takayama

Author information

Hironori Tanaka
Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
Yoshihito Saijo
Department of Cardiovascular Medicine, Tokushima University Graduate School of Biomedical Sciences, Japan
Tetsu Tomonari
Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
Takahiro Tanaka
Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
Tatsuya Taniguchi
Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
Shusuke Yagi
Department of Cardiovascular Medicine, Tokushima University Graduate School of Biomedical Sciences, Japan
Koichi Okamoto
Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
Hiroshi Miyamoto
Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
Masahiro Sogabe
Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
Yasushi Sato
Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
Naoki Muguruma
Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
Koichi Tsuneyama
Department of Pathology and Laboratory Medicine, Tokushima University Graduate School of Biomedical Sciences, Japan
Masataka Sata
Department of Cardiovascular Medicine, Tokushima University Graduate School of Biomedical Sciences, Japan
Tetsuji Takayama
Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan

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

JOURNAL OPEN ACCESS

2021 Volume 60 Issue 12 Pages 1839-1845

DOI https://doi.org/10.2169/internalmedicine.5914-20

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Details

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.

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