Clin Mol Hepatol.  2016 Jun;22(2):276-280. 10.3350/cmh.2015.0021.

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

• Lee KH
• Yoo SH
• Noh GT
• Heo WS
• Ko BS
• Chio JA
• Cho HJ
• Choi JY
• Kim HJ
• Sohn W
• Park SJ
• Park YM

• KMID: 2373125
• DOI: http://doi.org/10.3350/cmh.2015.0021

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.

Keyword

Plexiform neurofibroma; Portal hypertension; Esophageal varix

MeSH Terms

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