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KMID : 1040620160220020276
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Clinical and Molecular Hepatology
2016 Volume.22 No. 2 p.276 ~ p.280
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A case of portal hypertension by presumed as plexiform neurofibroma at the hepatic hilum
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Lee Kyung-Han
Yoo Sun-Hong
Noh Gi-Tark
Heo Won-Suk
Ko Byung-Seong
Chio Jung-Ah
Cho Hyo-Jin
Choi Jin-Young
Kim Hee-Jun
Sohn Won
Park Sang-Jong
Park Young-Min
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Abstract
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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.
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KEYWORD
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Plexiform neurofibroma, Portal hypertension, Esophageal varix
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