A case of portal hypertension by presumed as plexiform neurofibroma at the hepatic hilum

Autor: Kyung Han Lee, Sun Hong Yoo, Gi Tark Noh, Won Suk Heo, Byung Seong Ko, Jung Ah Chio, Hyo Jin Cho, Jin Young Choi, Hee Jun Kim, Won Sohn, Sang Jong Park, Young Min Park
Jazyk: angličtina
Rok vydání: 2016
Předmět:
Zdroj: Clinical and Molecular Hepatology, Vol 22, Iss 2, Pp 276-280 (2016)
Druh dokumentu: article
ISSN: 2287-2728
2287-285X
DOI: 10.3350/cmh.2015.0021
Popis: Neurofibromas can occur anywhere in the body, but they usually involve the head, neck, pelvis, and extremities. Abdominal visceral involvement is rare, and intrahepatic involvement is even less common. We describe a patient who suffered from plexiform neurofibromatosis with liver involvement. A 49-year-old man, who had previously been diagnosed with neurofibromatosis, underwent esophagogastroduodenoscopy and abdominal ultrasonography for screening purposes. Esophagogastroduodenoscopy showed grade 2 esophageal varices and abdominal ultrasonography showed conglomerated nodules with echogenic appearances in the perihepatic space. Magnetic resonance imaging showed presumed plexiform neurofibroma involving the lesser sac and hepatic hilum and encasing the common hepatic artery celiac trunk and superior mesenteric artery left portal triad. We report an unusual case of portal hypertension attributed to the compressive narrowing of the portal vein by presumed as plexiform neurofibroma at the lesser sac and hepatic hilum.
Databáze: Directory of Open Access Journals