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.

Case Report: A Rare Syncope Case Caused by Abernethy II and a Review of the Literature

“…All three syncope patients had some of the same characteristics including high blood ammonia, PAH and PH. Our patient is also quite different from the other syncope cases ( 11 , 14 ). Both of the other two reported cases were women.…”

“…The clinical manifestations of AM are diverse, and there are also some rare clinical manifestations, such as those with nephrotic syndrome (10) as the primary manifestation, or endocrine diseases such as hyperinsulinemia (11)(12)(13). In addition to the atypical manifestations above, there were two patients previously reported to experience syncope (11,14).…”

“…Of the two syncope cases, the first was a 42-year-old female patient, diagnosed with AM type II ( 14 ). The other was a 17-year-old female with AM Ib, manifesting as repeated syncope ( 11 ).…”

“…The other was a 17-year-old female with AM Ib, manifesting as repeated syncope ( 11 ). Due to abnormal liver blood perfusion ( 15 ), liver nodules were reported in both patients ( 11 , 14 ). Our patient also had this abnormality.…”

Shrinkage of Enlarged Hepatic Nodules by an Embolizing Congenital Extrahepatic Portosystemic Shunt

“…Franchi-Abella et al reported that complete or partial regression of benign hepatic nodules occurred after shunt embolization in 10 children ( 5 ). Regarding adult cases, there have been two case reports of congenital portosystemic shunts with hepatic nodules that were successfully treated with endovascular shunt embolization ( 6 , 7 ). It is thought that increased portal vein blood flow related to shunt embolization normalizes the hepatic blood flow, leading to nodule reduction, but no detailed mechanism has yet been reported.…”