Internal medicine (Tokyo, Japan) · 2021
An Adult Case of Congenital Extrahepatic Portosystemic Shunt Successfully Treated with Balloon-occluded Retrograde Transvenous Obliteration.
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Tanaka Hironori, Saijo Yoshihito, Tomonari Tetsu, Tanaka Takahiro, Taniguchi Tatsuya, Yagi Shusuke, Okamoto Koichi, Miyamoto Hiroshi, Sogabe Masahiro, Sato Yasushi, Muguruma Naoki, Tsuneyama Koichi, Sata Masataka, Takayama Tetsuji
Аннотация
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.