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A prospective study of hepatic encephalopathy (HE) including neuropsychiatric and psychometric evaluation, electroencephalography, and determination of arterial ammonia levels was performed in 55 cirrhotic patients treated consecutively by transjugular intrahepatic portosystemic shunt (TIPS). The cumulative HE rate increased from 23.6% within the 3-month interval before TIPS to 50. 9% within the first 3-month interval post-TIPS (P = .003). Significant and independent predictors of HE post-TIPS were the presence of HE pre-TIPS and reduced liver function. The cumulative HE rate declined in the second 3-month interval post-TIPS and reached the pre-TIPS level. Chronic forms of HE exceeding grade I were not observed. In a subgroup of 22 nonencephalopathic TIPS patients, the prevalence of subclinical HE did not change after TIPS. Among individual psychometric tests, the block design test gave the highest proportion of pathological results (about 50%), whereas selective reminding gave the lowest (10%-25%). Electroencephalography (EEG) showed a temporary increase of pathological results at 1 month after TIPS, when patients with overt HE (grade I) were included (proportion of 21.1% before vs. 57.1%, P = .005). Arterial ammonia concentration increased from a mean of 94 +/- 26 microgram/dL to 140 +/- 28 microgram/dL at 3 months after TIPS (P < .001). Elevated ammonia levels persisted. TIPS led to a temporary increase of HE incidence within 3 months. The decline of the HE rate beyond 3 months despite a sustained increase of arterial ammonia levels could not entirely be explained by reduction of shunt flow, nor by alteration of liver function. Instead, cerebral adaptation to gut-derived neurotoxins might be anticipated.

Shunt insufficiency due to shunt occlusion or stenosis is frequent after TIPS (about 50% after one year). Controversially discussed is whether Doppler sonography is effective in detecting shunt stenosis or whether regular angiographies are required. The experience with a noninvasive method of surveillance primarily based on Doppler sonography is reported here. 58 patients (35 men, 23 women, mean age 55 years, range 33-82 years) were treated by TIPS because of complications of portal hypertension (43 x gastroesophageal bleeding, 14 x refractory ascites, 1 x venoocclusive disease). Liver cirrhosis (alcoholtoxic etiology in 63%) was present in 55 cases, according to Child-Pugh's classification 23 patients = A, 19 patients = B and 13 patients = C. Within a mean observation period of 14 months, Doppler sonography was performed in three months intervals, endoscopy in six months intervals and angiography only when shunt insufficiency was suspected by Doppler sonography and/or because of clinical events, e.g. recurrent bleeding. Immediately after TIPS, maximal flow velocity and flow volume in the portal vein increased by 116% and 115%, respectively. Three months later, a significant increase of portal vein diameter of about 15% was measured. Shunt flow was initially 2.700 ml/min (one week after TIPS) and decreased progressively by about 30% within the first nine months of follow-up. Correspondingly, angiographically proven shunt insufficiency was present in 22 patients (33 episodes). A total of twelve bleeding episodes recurred in seven patients (rebleeding rate of 16% after one year and 19% after two years). In the remaining 15 patients (68%; 21 episodes) shunt insufficiency could be corrected prior to complications because of detection by Doppler sonography (19x) and endoscopy (2x). Therefore, Doppler sonography is an effective diagnostic tool for the detection of shunt insufficiency and should be performed at three months intervals for at least 18 months. In this context it appears allowable to avoid routine angiographies.

A 65-year-old italian patient developed complicated portal hypertension immediately after orthtopic liver transplantation (OLT) necessitating shunt creation. One to five weeks after OLT, massive ascitic fluid losses of up to 121/day developed. Vascular and major hepatic-parenchymal abnormalities were excluded by duplexsonography, angiography and initial histology, respectively. A peritoneovenous shunt (Denver-shunt) on day 31 after OLT reduced (by about 50%) but did not stop ascitic fluid losses. Furthermore, three variceal bleedings occurred after implantation of the Denver-shunt. Direct portography on day 45 after OLT revealed portal hypertension (pressure gradient of 26 mmHg) requiring the implantation of a transjugular intrahepatic portosystemic stent-shunt (TIPS) leading to a reduction of the pressure gradient to 13 mmHg. Subsequently, ascites resolved within ten days and esophageal varices improved. Liver function parameters normalized inspite of recurrence of HCV infection with detection of HCV RNA in serum already in the fifth week after OLT. During follow-up, histological findings deteriorated from mild changes to extended fibrosis at day 61 after OLT, which might have contributed to the maintenance of portal hypertension. The deterioration of liver histology was accompanied by an improvement/normalization of liver graft function. There was no evidence for additional viral liver infections, e.g. hepatitis B or cytomegalovirus infection. This case illustrates an etiologically unclear syndrome developing directly after OLT and reaffirms the effectiveness of TIPS in the treatment of complicated portal hypertension even after liver transplantation.

History and Clinical Findings: A 55-year-old woman with known primary biliary cirrhosis (PBC) was hospitalized because of increasing dyspnoea. A year before she had for the first time experienced a right-sided pleural effusion which had to be drained every 4 weeks. Physical examination revealed dullness on percussion and greatly decreased breath sounds on auscultation over the entire right thorax. In addition there were signs of moderate ascites and leg oedema., Investigations: Chest radiograph showed a homogeneous shadowing of the right thorax without mediastinal shift. Diagnostic thoracocentesis produced a serous effusion, a transudate on chemical analysis, comparable to the composition of the ascitic fluid. Bacteriological and cytological tests on both fluids were unremarkable., Treatment and Course: The right pleural effusion was presumed to be due to a hydrothorax from the ascites caused by portal hypertension associated with the PBC. Despite continuous diuretic treatment and thoracocentesis with albumin substitution every 3 days there was no improvement and implantation of a transjugular intrahepatic portosystemic stent shunt (TIPSS) was performed. This effectively lowered portal pressure and markedly improved the patient's condition so that further thoracocentesis were no longer necessary. 3 weeks after TIPSS implantation she was discharged in good condition. Radiography 3 weeks later demonstrated continued reduction in the hydrothorax., Conclusion: Hydrothorax is a rare complication of liver cirrhosis. TIPSS implantation can provide lasting resolution and corresponding clinical improvement of a hydrothorax, especially in those conditions which are refractory to diuretic treatment and thoracocentesis.

History and Clinical Findings: A 41-year-old woman, known for 10 month to have polycythaemia vera, developed severe right upper abdominal pain. The abdomen was tense from marked ascites and the liver enlarged by 18 cm in the mid-clavicular line., Investigations: Serum bilirubin was slightly elevated to 2.2mg/dl, liver synthesis being much reduced (recalcifying time minimally 23%, albumin minimally 2.8 g/dl). Doppler sonography detected no flow in the right and middle hepatic veins, indicating Budd-Chiari syndrome. Portal vein flow was diminished., Treatment and Course: Heparin treatment had to be stopped because of heparin-associated type II thrombocytopenia and hirudin was substituted. Attempted lysis with a total of 100 mg r-tPA failed. As the patient's condition deteriorated a TIPSS was implanted to provide portal decompression. Incomplete portal vein thrombosis was demonstrated and worsened during the procedure until nearly complete occlusion. Local lysis treatment for 2 days with urokinase, 50,000-60,000 U/h, and two shunt revisions finally succeeded in completely dissolving the thrombus. Portocaval pressure fell from 32 to 21 mm Hg, and the size and function of the liver became almost normal and the ascites disappeared. Anticoagulation with a coumarin derivative was started and hydrocarbamide again given for recurrent thrombocytosis. The patient remained largely symptom-free one year after TIPSS., Conclusion: This case demonstrates the effectiveness of TIPSS in Budd-Chiari syndrome, even in complicated portal vein thrombosis.

A 69-yr-old man with known carcinoid syndrome treated with octreotide and interferon-alpha 2b developed diarrhea, with six to eight watery to semiliquid stools per day. Diminished stool frequency and increased stool consistency were obtained by treatment with the 5-hydroxytryptamine-3 receptor antagonists ondansetron and tropisetron. Successful alleviation of the diarrhea was also observed with the alpha 2-receptor agonist clonidine. These observations indicate that these classes of drugs should be evaluated in a controlled trial in patients with carcinoid-associated diarrhea.