A case of portal hypertension by presumed as plexiform neurofibroma at the hepatic hilum.
- Author:
Kyung Han LEE
1
;
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
Author Information
- Publication Type:Case Reports
- Keywords: Plexiform neurofibroma; Portal hypertension; Esophageal varix
- MeSH: Abdomen/diagnostic imaging; Endoscopy, Digestive System; Esophageal and Gastric Varices/pathology; Hepatic Artery/diagnostic imaging; Humans; Hypertension, Portal/*diagnosis; Liver/diagnostic imaging; Magnetic Resonance Imaging; Male; Middle Aged; Neurofibroma, Plexiform/*diagnosis/diagnostic imaging; Tomography, X-Ray Computed; Ultrasonography
- From:Clinical and Molecular Hepatology 2016;22(2):276-280
- CountryRepublic of Korea
- Language:English
- Abstract: 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.
