Robert Dick

Primary sclerosing cholangitis: a review of its clinical features, cholangiography, and hepatic histology.

Twenty-nine patients with primary sclerosing cholangitis were reviewed. Males predominated (2:1). Seventy-six per cent presented with cholestasis and cholangitis, 17% with cirrhosis and portal hypertension, and 7% were asymptomatic, presenting with a raised serum alkaline phosphatase. The serum immunoglobulin IgM concentration was raised in 45% of the patients, but no patient had serum mitochondrial antibody present. Seventy-two per cent had ulcerative proctocolitis. There was no relationship between either duration or severity of ulcerative proctocolitis and the development of primary sclerosing cholangitis. Four patients were not benefited by colectomy. None of the patients ahd Crohns disease. The prognosis was variable. Corticosteriods and azathioprine were ineffective. Eleven patients (38%) had died with a mean survival time of seven years from diagnosis. Three patients with ulcerative proctocolitis developed bile duct carcinoma. The cholangiograms and liver biopsies were reported without reference to clinical information together with 41 patients with other biliary diseases. Cholangiography was diagnostic in 18/22 (82%). Hepatic histology was diagnostic in 8/22 (36%). Ten showed features of large bile duct disease and three were misdiagnosed as primary biliary cirrhosis. Reduced numbers of bile ducts, ductular proliferation, portal inflammation, and substantial copper deposition, in combination with piecemeal necrosis, are commonly seen in primary sclerosing cholangitis and indicate the need for cholangiography.

Controlled Trial of Propranolol for the Prevention of Recurrent Variceal Hemorrhage in Patients with Cirrhosis

We conducted a prospective randomized trial of propranolol for the prevention of recurrent variceal bleeding in 48 patients with cirrhosis of the liver. During a follow-up period of up to 21 months, 12 of 26 patients in the propranolol group and 11 of 22 in the control group had rebleeding from esophageal varices. There was no significant difference in rebleeding between the two groups. This contrasts with a previous report of the efficacy of propranolol in preventing recurrent gastrointestinal bleeding in alcoholic cirrhosis. The difference in results may be due to the inclusion in our study of patients with other causes of cirrhosis and more severe liver disease. Propranolol may not be indicated for the prophylaxis of variceal rebleeding in such patients, and we advocate that its use be limited at present to controlled clinical trials.

Gianturco self-expanding stents: Clinical experience in the vena cava and large veins

Twenty-five patients with stenosis of the vena cava (21) and other large veins (4) have been treated with self-expanding Gianturco metallic stents. Eighteen patients had superior vena cava syndrome. In 17, the stricture was due to malignant superior vena cava compression recurrent after maximum tolerance radiotherapy and/or chemotherapy. In 16 of these patients there was early symptomatic relief. In 1 patient with a benign posttraumatic superior vena cava stricture, the stenosis was not relieved, and occlusion occurred after 1 month. Stenoses associated with dialysis shunts were relieved in 2 other patients. Two malignant and one benign inferior vena cava stenoses were relieved either until death, or in the benign case, for 30 months. One malignant subclavian vein obstruction occluded after 24 h due to stent misplacement and another with extrinsic mediastinal compression remained patent until death, extensive thrombus having been lysed prior to stent placement. The results of this short series suggest that the Gianturco self-expanding stent in the vena cava and large veins is easy and safe to place, and in most cases produces almost immediate palliation of the distressing effects of venous obstruction, often in a preterminal and inoperable patient.

PORTAL HYPERTENSION WITH VARICES IN UNUSUAL SITES

Abstract Seven patients with liver disease and portal hypertension are described in whom varices occurred in sites other than the stomach and œsophagus. All but one presented with overt bleeding from a colonic, rectal, or ileal varix. The diagnosis was confirmed by proctosigmoidoscopy or barium enema in all cases. In two patients splenic venography showed portal venous collaterals and manometry demonstrated raised portal-vein pressures. In six cases varices formed in the territory of the inferior mesenteric vein; in the seventh they were tributaries of the superior mesenteric vein. Treatment for uncontrollable haemorrhage by transfusion was required in two cases; both patients died after operation. The series suggests that varices in unusual sites comprise a minor but significant cause of gastrointestinal bleeding in patients with liver disease.

Incidence, risk factors, management, and outcome of portal vein abnormalities at orthotopic liver transplantation.

Portal vein thrombosis is often considered a contraindication to orthotopic liver transplantation. We have analyzed the incidence, risk factors, management and outcome of patients with portal vein thrombosis undergoing orthotopic liver transplantation. During the period from October 1988 to October 1992 140 grafts were performed on 132 patients. Fourteen had portal vein thrombosis with either partial (n = 7) or complete (n = 7) occlusion of the portal vein at surgery. Portal vein thrombosis was more common in patients with autoimmune chronic active hepatitis (3/5 vs. 11/127, chi 2 = 13.3, P < 0.001), cryptogenic cirrhosis (4/12 vs. 10/120, chi 2 = 7.2, P < 0.01), or those with tumors (6/22 vs. 10/110, chi 2 = 5.7, P < 0.05). In 13 of the 14 portal inflow was reestablished by flushing, balloon thrombectomy, or passage of a graduated dilator. In one patient complete fibrous obliteration necessitated a portal vein to right gastroepiploic vein anastomosis. On follow-up there have been 6 deaths in this group (6/14 = 43%) from recurrent cancer (n = 1), sepsis (n = 4), and cardiac and renal failure (n = 1). Four of these 6 patients had confirmation of PV patency on imaging. The remaining 8 patients are alive and well (median follow-up 37 months, range 6-53 months). Post-transplant portal vein thrombosis occurred in 3 of the 14 patients (21%) with a portal vein abnormality at surgery and in two of the 118 patients with a normal portal vein (3/14 vs. 2/118, chi 2 = 8.5, P < 0.01). Four of the 5 cases were successfully treated by surgical thrombectomy.

Treatment of small hepatocellular carcinomas.

There is growing interest in screening to detect symptomless hepatocellular carcinoma (HCC), which should be easier to treat than symptomatic tumours. Combined alpha-fetoprotein and ultrasound monitoring can detect HCCs of 1 cm, and Lipiodol retention can be detected in tumours smaller than 1 cm. A number of treatment options are available. Surgical resection may be curative in selected patients with a single small tumour, but the cirrhotic patient is left with a diseased liver and the risk of tumour recurrence or death from underlying liver dysfunction. Orthotopic liver transplantation is a rational treatment for patients with decompensating cirrhosis and a small HCC, but it is expensive and necessitates immunosuppression. A variety of targeted or local therapies, either individually or in combination, can be used to treat HCC. These include percutaneous alcohol injection into an HCC, which may be an alternative to surgical resection. Tumour necrosis can be seen after targeted Lipiodol chemotherapy or radiotherapy. Transcatheter arterial embolisation selectively embolises the feeding artery, and can be combined with Lipiodol chemotherapy. Small tumours are thus amenable to treatment, even in patients who cannot have surgery. Screening and treatment for symptomless HCC seems justified, unless controlled trials teach us differently.

Factors related to early mortality after transjugular intrahepatic portosystemic shunt for failed endoscopic therapy in acute variceal bleeding

BACKGROUND Uncontrolled variceal haemorrhage is the main indication for transjugular intrahepatic portosystemic shunt. However, mortality is 50% for this high-risk group. We have evaluated clinical and laboratory variables prior to transjugular intrahepatic portosystemic shunt in order to establish predictors of mortality, validated prospectively. METHOD Over a 4-year period, 367 patients were admitted with variceal bleeding. In 54 patients endoscopic therapy for acute variceal bleeding failed and they had emergency transjugular intrahepatic portosystemic shunt. Failure of therapy was defined as continued bleeding after 2 endoscopy sessions (n=39) or vasoconstrictor-resistant bleeding from gastric/ectopic varices (n=15). Thirty-three variables were analysed from data available immediately prior to transjugular intrahepatic portosystemic shunt. RESULTS Twenty-six patients died within 6 weeks. In a multivariate analysis, 6 factors had independent prognostic value: moderate/severe ascites, requirement for ventilation, white cell blood count (WBC), platelet count (PLT), partial thromboplastin time with kaolin (PTTK) and creatinine. A prognostic index (PI) score was derived, in which presence of moderate/severe ascites, or need for ventilation, scored 1: PI=1.54 (Ascites)+1.27 (Ventilation)+1.38 Ln (WBC)+2.48 ln (PTTK)+1.55 Ln (Creat)-1.05 Ln (PLT). Using this equation, 42% (n=10) of deaths occurred in the fifth quintile (PI > or = 18.52), where the mortality was 100%. The score was prospectively validated in a further 31 patients, giving 100% positive predictive value. Eleven further patients died, including all seven with a PI >18.5. No survivors had a PI >18.3. CONCLUSION Despite immediate control of bleeding by transjugular intrahepatic portosystemic shunt, patients with uncontrolled variceal haemorrhage have a high mortality, particularly when associated with markers of advanced liver disease, sepsis and multi-organ failure. The use of transjugular intrahepatic portosystemic shunt is probably not justified in this subgroup. Our prognostic index can help identify such patients, and, if validated elsewhere, will help in deciding when to use transjugular intrahepatic portosystemic shunt.

Use of gianturco self-expandable stents in the tracheobronchial tree

Gianturco self-expandable stents were used successfully in the management of 5 patients with tracheobronchial pathology. Placement was performed under endoscopic and fluoroscopic guidance. None of the patients has experienced complications secondary to the stent placement, and in all of them the clinical problems resolved satisfactorily. Longer follow-up is required to determine the place of tracheobronchial stenting in patients with respiratory compromise.

Single portal pressure measurement predicts survival in cirrhotic patients with recent bleeding

Background Height of portal pressure correlates with severity of alcoholic cirrhosis. Portal pressure indices are not however used routinely as predictors of survival. Aims To examine the clinical value of a single portal pressure measurement in predicting outcome in cirrhotic patients who have bled. Methods A series of 105 cirrhotic patients who consecutively underwent hepatic venous pressure measurement were investigated. The main cause of cirrhosis was alcoholic (64.8%) and prior to admission all patients had bled from varices. Results During the follow up period (median 566 days, range 10–2555), 33 patients died, and 54 developed variceal haemorrhage. Applying Cox regression analysis, hepatic venous pressure gradient, bilirubin, prothrombin time, ascites, and previous long term endoscopic treatment were the only statistically independent predictors of survival, irrespective of cirrhotic aetiology. The predictive value of the pressure gradient was much higher if the measurement was taken within the first or the second week from the bleeding and there was no association after 15 days. A hepatic venous pressure gradient of at least 16 mm Hg appeared to identify patients with a greatly increased risk of dying. Conclusions Indirectly measured portal pressure is an independent predictor of survival in patients with both alcoholic and non-alcoholic cirrhosis. In patients with a previous variceal bleeding episode this predictive value seems to be better if the measurement is taken within the first two weeks from the bleeding episode. A greater use of this technique is recommended for the prognostic assessment and management of patients with chronic liver disease.

A prospective controlled study comparing brush and bile exfoliative cytology for diagnosing bile duct strictures.

Imaging of biliary strictures may suggest malignancy but cytology can provide a tissue diagnosis. The aim of this study is to compare the diagnostic value of brush cytology and bile cytology. Thirty two patients (20 males, 12 females, median age 66 years, range 31-84) with biliary strictures at endoscopic retrograde cholangio pancreatography (24) or percutaneous transhepatic cholangiography (8) had bile cytology and brush cytology. Brushings were taken using a modified Geenan cytology brush (6 Fr gauge, Wilson Cook) passed alongside a guide wire placed through the stricture. Bile was aspirated after insertion of an internal/external catheter or an endoprosthesis. Bile and brushings were examined by one experienced cytologist (AD) and was reported as positive or negative for malignant cells. Twenty nine patients had malignant strictures. Sixteen were confirmed by histology and 13 had malignancy suggested by clinical follow up. Three patients had resection of histologically benign strictures. The overall sensitivity of brush cytology (17 of 29 positive, 59%) was significantly greater than bile cytology (seven of 29 positive, 24%) (p < 0.01) as was the diagnostic accuracy (63 v 31%, p < 0.01). None of the patients had positive bile cytology with negative brush cytology. There were no procedure related complications and the average sampling time once the guide wire had been inserted was less than five minutes. It is concluded that brush cytology is more sensitive than bile cytology and with the technique described is safe and rapid.