CASE REPORT: Intrahepatic portal‐hepatic venous shunts associated with a huge pelvic leiomyoma

Abstract

We present a case of portal-systemic encephalopathy due to intrahepatic multiple portal-hepatic venous shunts. A 71-year-old woman was admitted to our hospital because of recurrent episodes of disturbed consciousness. She showed no clinical signs of portal hypertension. Liver function was normal, except for an indocyanine green retention rate of 34% at 15 min and blood ammonia level of 282 microg/dL. Portal venography revealed dilatation of the portal vein and multiple portal-hepatic venous shunts, and a liver biopsy specimen revealed almost normal liver. Further clinical examination revealed a huge pelvic tumour. At laparotomy, two dilated veins were seen to arise from the pelvic tumour with blood flow into the mesentery. The tumour was resected successfully and a histological diagnosis of leiomyoma was made. The blood ammonia concentration decreased to the normal range postoperatively. A follow-up portal venogram demonstrated decreased portal vein dilatation and minor portal-hepatic venous shunts, considered to be congenital in origin. It is concluded that hepatic encephalopathy was produced in this patient due to an excess portal blood flow from the huge pelvic leiomyoma via the mesentery, with portosystemic shunting through pre-existent (probably congenital) intrahepatic anastomoses.

Asymptomatic Aneurysmal Portosystemic Venous Shunt: A Case Report and Review of the Literature

“…There are cases of SIPSVS associated with focal nodular hyperplasia or hereditary hemorrhagic telengiectasia (HHT) (1,15,16,30,32). HHT is an autosomal dominant disorder characterized by telangiectasias and arteriovenous malformations of potentially every organ (3).…”

Gradual improvement of hyperammonemic hepatic encephalopathy after the extirpation of a large uterine leiomyoma in a woman with constipation and liver cirrhosis resulting from autoimmune hepatitis

“…3,4 Only one case report has been published on the association between hyperammonemia and large pelvic leiomyomas in patients with intrahepatic portohepatic venous shunts; two dilated veins connected the pelvic leiomyoma to the mesentery, although the leiomyoma did not originate from the uterine body. 5 In our case, gradual improvement of hyperammonemic hepatic encephalopathy was achieved after the extirpation of a large uterine leiomyoma in a woman with organic constipation and liver cirrhosis resulting from autoimmune hepatitis.…”

“…There have been few reports regarding the improvement of hyperammonemic hepatic encephalopathy after the extirpation of a large uterine leiomyoma. 5 We experienced a case in which a woman with severe constipation, liver cirrhosis resulting from autoimmune hepatitis and a large uterine leiomyoma, developed hyperammonemic hepatic encephalopathy. The extirpation of the uterine leiomyoma resulted in a gradual improvement in her hyperammonemic hepatic encephalopathy and a marked reduction in her serum ammonia levels.…”

Intrahepatic portal-venous shunts during PVE

“…Treatment options include surgical ligation, resection, and endovascular embolization [12] . Complications of intrahepatic portal shunts can range from none to portal hypertension, congestive heart failure, hepatic encephalopathy, cirrhosis, membranoproliferative glomerulonephritis, and cholangitis [13] , [14] , [15] .…”