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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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Background/Aims Transjugular intrahepatic portosystemic shunt(TIPS) is a promising method of treatment for gastric and esophageall variceal bleeding. Immediate technical and short-term clinical results have been reportn!. This study is performed to evaluate long-term outcome after TIPS in patients who underwent the pracedure for variceal bleeding. Methods:Forty patients who underwent TIPS hetween August 1991 and February 199S were followed up by clinical examination, upper gastrointestina! Endoscopy and Duplex sonogrphy. Results:The mean portohepatic pressure gradient prior to TIPS was 30.1+ 8.7cmH ancl dropped to 16.6+ 6.7cmH2O after shunt(p<0.001). The cumulative survival rate was 67.5% at 6 months. 57.4% at 1 year, 37.1% at 2 years and 26.8% at 3 years. Survival after TIPS was inversely related to Child-Pugh class. The incidence of recurrent variceal bleeding was 25%. The causes of death were hepatic failure(53.6 %), recurrent variceal bleeding(28.6'%), sepsis(7.1 %) and unknown causes(10.7'%). Conelueien:TIPS is an effective method for treatment of variceal bleeding in unsuccessful cases by other treatments including endoscopic therapy and the most important prognostic factor is preprocedual hepatic resenre(Child-Pugh class), TIPS by itself is not defioite therapy, but in combination with careful follow-up surveillance and percutaneous shunt revision is very effective therapeutic strategy. TIPS is particularly valuable in tlreating patients with variceal bleeding hefor liver transplantation and in treating patients with poor liver function.
Gyu Hyun Lee, M.D., Yong Jun Shin, M.D, Young Yun Ko, M.D.
Jun Ho Ko, M.D., Ho Dong Kim, M.D., Byeong Moo Yoo, M.D.
Kwang Jae Lee, M.D., Young Soo Kim, M.D., Ki Baik Hahm, M.D.
Jin Hong Kim, M.D. and Sung Won Cho, M.D.
Background /Aim: Endoscopic sclerotherapy and band ligation have been well documented beneficial therapeutic options of esophageal variceal bleeding. But acute variceal bleeding is refractory to sclerotherapy in upto one-third of patients, and rebleeding occurs in 30% to 50%. Recently alternative endoscopic modality, N-butyl-2-cyanoacrylate (Histoacryl) injection is performed in intravariceal sclerotherapy but its efficacy and safty are not clearly established. We evaluated the efficacy of Histoacryl on esophageal variceal bleeding and compared with that of endoscopic band ligation in the present study. Materials/Methods: From March 1994 to March 1998, ninety seven patients with endoscopically documented esophageal variceal bleeding were enrolled. Histoacryl injection (Histoacryl group, n=33) or endoscopic band ligation (EVL group, n=64) was done for esophageal variceal bleeding. We evaluated the rebleeding rate and in-hospital mortality in both groups. Results: Baseline characteristics were similar but active bleeding on first endoscopic session was significantly higher in Histoacryl group (Histoacryl group; 90.7%, EVL group; 26.6%, p=0.002). Successful hemostasis was done at 87.9% in Histoacryl group, 95.3% in EVL group (not significant). There were no significant differences on early rebleeding rate (18.2% vs 23.4%), late rebleeding rate (39.4% vs 37.5%) and in-hospital mortality (24.2% vs 15.6%) between Histoacryl group and EVL group. There were no technique-related fatal complications at Histoacryl injection group. Conclusion: Therapeutic efficacy of Histoacryl injection was similar to the endoscopic band ligation in patients with esophageal varix bleeding in terms of hemostasis and rebleeding. Histoacryl is effective therapeutic option for esophageal variceal bleeding as well as gastric variceal bleeding. (Korean J Hepatol 1999;5:306－313)
Sung Bum Cho , Kang Jin Park , Jung Soo Lee , Wan Sik Lee , Chang Hwan Park , Young Eun Joo , Hyun Soo Kim , Sung Kyu Choi , Jong Sun Rew , Sei Jong Kim
Background/Aims: Terlipressin and octreotide had been used to control acute variceal bleeding and to prevent early rebleeding after endoscopic hemostasis. We compared the efficacy and safety of terlipressin and octreotide combined with endoscopic variceal ligation (EVL) for the treatment of acute esophageal variceal bleeding and we evaluated their clinical significance as related to rebleeding. Methods: The eighty eight cirrhotic patients were randomized to the terlipressin group (n=43; 2 mg i.v. initially and 1 mg i.v. at every 4 hours for 3 days) or the octreotide group (n=45; continuous infusion of 25 μg/h for 5 days) combined with EVL for the treatment of acute esophageal variceal bleeding. Results: The initial hemostasis rates were 98% (42/43 cases) in the terlipressin group and 96% (43/45 cases) in the octreotide group. The 5-day and 42-day rebleeding rates were 12% (5/43 cases) and 28% (12/43 cases), respectively, in the terlipressin group and 9% (4/45 cases) and 24% (11/45 cases), respectively, in the octreotide group. No significant difference was demonstrated between the terlipressin and octreotide groups. The mortality at 42 days was similar in both group, but a high mortality rate (48%) was shown to be related to 42-day rebleeding. The risk factors related to 42-day rebleeding were Child-Pugh class C (aOR=30.2, 95% CI=7.7-117.9), ascites above grade II (aOR=6.6, 95% CI=2.2-19.2) and advanced hepatocellular carcinoma (aOR=4.6, 95% CI=1.1-18.9). Conclusions: Comparing terlipressin and octreotide combined with EVL showed them to be equally safe and effective therapeutic agents in patients with acute esophageal variceal bleeding. The high risk factors related to early rebleeding were poor liver function and advanced hepatocellular carcinoma.
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