J. B. Dilawari

Hepatic outflow obstruction (Budd-Chiari syndrome). Experience with 177 patients and a review of the literature.

Budd-Chiari syndrome (BCS) may not be as uncommon as was once believed. Our study has substantiated the existence of 2 major clinical forms. The acute syndrome is invariably associated with extensive blockage of the major hepatic veins, resulting in congestive liver cell necrosis. In a small, but significant, number of patients the inferior vena cava (IVC) is also occluded. The important etiologic factors are related to hypercoagulability of blood. Immediate placement of a shunt improves survival. The chronic syndrome is characterized by portal hypertension and is associated with a variable abnormal vascular anatomy. The causes of the chronic syndrome are not clear, but a substantial number of cases are related to the presence of an IVC membrane. Shunt surgery is effective but procedures aimed at the primary pathology are likely to be even more so. The natural history of BCS should be viewed over a long period of time. The very long survival of several patients urges a more cautious approach to surgical remedies. Budd-Chiari syndrome probably represents a spectrum of disease caused primarily by a hypercoagulable state and having a varied presentation depending on the balance between rate of formation and the extent of the thrombosis and the bodys own rate of thrombolysis and recanalization. The extent and efficacy of the individuals collateral circulation and the rate of development of liver fibrosis are other determinants. It is thus possible to view BCS as a continuum of a single pathogenetic spectrum. Pregnancy-related BCS in India probably has strong social determinants, and is usually acute and fulminant. We have, however, documented a chronic form not described earlier. Children usually do not have acute BCS, but chronic BCS in children and adolescents is similar to that in adults. Membranous obstruction of the inferior vena cava (MOVC) is common and was found even at a young age. The association of MOVC with hepatocellular carcinoma, however, did not appear to be as clear as was previously believed. There has been a wide geographical variability in the causes and manifestations of BCS. Our study has clearly shown that--Kiplings categorical statement to the contrary--East and West do meet in India, in the Budd-Chiari syndrome.

Non‐cirrhotic portal fibrosis (idiopathic portal hypertension): Experience with 151 patients and a review of the literature

Background: Non‐cirrhotic portal fibrosis (NCPF), the equivalent of idiopathic portal hypertension in Japan and hepatoportal sclerosis in the United States of America, is a common cause of portal hypertension in India. The clinical features, portographic and histological findings, and management of 151 patients with non‐cirrhotic portal fibrosis are presented.

Portal hypertensive biliopathy

Extrahepatic portal venous obstruction (EHPVO) is a common cause of portal hypertension in the developing countries, and constitutes up to 40% of all patients with portal hypertension.1,2 EHPVO is a common cause of major upper-gastrointestinal bleeding among children.2–4 The most common presentation in children is well-tolerated variceal bleeding and splenomegaly. In adults, EHPVO is often recognised when evaluating for other disorders or with uncommon presentations such as jaundice, pruritus, acute cholecystitis-like syndrome, ascites and so on, resulting from prolonged portal hypertension.5–7 The portal vein in EHPVO is transformed into a cavernoma, which is a bunch of multiple collateral veins around the obstructed portion of portal vein (fig 1). Marked improvements in the management of variceal bleeding in patients with EHPVO have resulted in an improved survival, thus presenting with unusual symptoms in adulthood. Figure 1  Splenoportovenogram showing multiple collaterals (portal cavernoma) replacing the portal vein (arrows) in a patient with extrahepatic portal venous obstruction. The splenic vein is normal (arrowheads). The reasons for EHPVO are obscure in approximately half of the patients. Omphalitis and intra-abdominal sepsis are the common causes in neonates and children. Adults develop EHPVO due to increased blood coagulability, local inflammation, intra-abdominal sepsis, myeloproliferative disorders, underlying cirrhosis, or tumours in the liver, bile ducts or pancreas.7,8,9,10 Gibson et al 11 first reported the relationship between EHPVO and jaundice in 1965. Since then, several cases of obstructive jaundice due to common bile duct (CBD) obstruction caused by cavernomatous transformation of portal vein (portal cavernoma) have been described. Williams et al 12 were the first to report cholangiographic changes caused by choledochal varices. We, for the first time, describe abnormalities on endoscopic retrograde cholangiography (ERC) in a prospective study.13 These abnormalities were similar to those of primary sclerosing cholangitis and …

Efficacy of lactulose in cirrhotic patients with subclinical hepatic encephalopathy.

To investigate the role of lactulose in the treatment of cirrhotic patients with subclinical hepatic encephalopathy (SHE), 40 cirrhotic patients, 33 males and 7 females, were included in the study. The diagnosis of SHE was made by quantitative psychometric tests including the number connection test (NCT), figure connection test (FCT) parts A and B, and two performance subtests of Wechsler adult intelligence scale, ie, picture completion (PC) and block design (BD) tests. SHE was diagnosed in 26 (65%) of 40 patients. Of these 26 patients, 14 patients were randomized to treatment group (lactulose 30–60 ml/day for three months, SHE-L) and 12 patients to no treatment group (no lactulose, SHE-NL). Psychometric tests were repeated in all patients in both groups and in six patients with no SHE (group NSHE, N = 14) after three months. The mean scores and number of the abnormal psychometric tests at entry were significantly higher in patients in groups SHE-L and SHE-NL than in patients in group NSHE; however, there was no significant difference between SHE-L and SHE-NL. The mean number of the abnormal psychometric tests decreased in patients in group SHE-L after three months of treatment with lactulose (2.9 ± 0.9 vs 0.8 ± 1.2; P = 0.004); however, there was no change in patients in group SHE-NL after three months (3.7 ± 1.5 vs 3.5 ± 1.3; P = NS). While SHE improved in 8 of 10 patients in group SHE-L, none of the patients in group SHE-NL improved after three months of follow-up (P < 0.001). Two patients in group SHE-NL also developed overt encephalopathy during the study period. We conclude that lactulose treatment in cirrhotic patients with SHE is effective.

Minimal hepatic encephalopathy: Consensus statement of a working party of the Indian National Association for Study of the Liver

Hepatic encephalopathy (HE) is a major complication that develops in some form and at some stage in a majority of patients with liver cirrhosis. Overt HE occurs in approximately 30–45% of cirrhotic patients. Minimal HE (MHE), the mildest form of HE, is characterized by subtle motor and cognitive deficits and impairs health‐related quality of life. The Indian National Association for Study of the Liver (INASL) set up a Working Party on MHE in 2008 with a mandate to develop consensus guidelines on various aspects of MHE relevant to clinical practice. Questions related to the definition of MHE, its prevalence, diagnosis, clinical characteristics, pathogenesis, natural history and treatment were addressed by the members of the Working Party.

Prognostic Evaluation of Early Indicators in Fulminant Hepatic Failure by Multivariate Analysis

Viral hepatitis is the commonest cause offulminant hepatic failure (FHF) in developing countries.We evaluated the early indicators of prognosis in thesepatients by multivariate analysis. The records of 204 consecutive patients with acute liverfailure admitted with hepatic encephalopathy over fiveyears were studied. The etiology of these patientsincluded virus related in 186 (91.1%), drug induced in 15 (7.4%), Wilsons disease in one (0.5%),acute Budd-Chiari syndrome in one (0.5%), and malignantinfiltration in one (0.5%). Patients with FHFcomplicating viral hepatitis were analyzed by univariate and multivariate analysis. These patients werefurther subclassified depending upon the intervalbetween the onset of jaundice and the onset ofencephalopathy into hyperacute (HALF; interval 0-7days), acute (ALF; interval 8-28 days) and subacuteliver failure (SALF; interval 4-12 weeks). Sixty (32.3%)patients with viral hepatitis survived. Univariateanalysis showed that the interval between onset of encephalopathy and onset of jaundice, grade ofencephalopathy, raised intracranial pressure,prothrombin time, and serum bilirubin levels onadmission were related to outcome in these patients.Multivariate logistic regression analysis showed that thepresence of raised intracranial pressure at the time ofadmission, prothrombin time >100 sec on admission,age (>50 yr), and onset of encephalopathy seven days after onset of jaundice were associated withpoor prognosis. Forty seven (37.0%) of 129 patients withHALF survived compared with 9 (22.5%) of 40 with ALF and4 (21.1%) of 19 with SALF (P = NS). Raised intracranial pressure was more frequent in patients withHALF (48.8%) than in patients with ALF (32.5%) and SALF(15.8%; P = 0.01), while clinically detectable asciteswas more frequent in patients with SALF (78.9%) compared with HALF (19.7%) and ALF (37.5%; P< 0.0001). The factors adversely affecting theoutcome in our patients with FHF complicating viralhepatitis include presence of overt clinical features of raised ICP at the time of hospitalization,prothrombin time (>100 sec) on admission, age (>50yr), and onset of encephalopathy seven days after onsetof jaundice.

Endoscopic sclerotherapy of gastric variceal bleeding with N-butyl-2-cyanoacrylate.

Background Bleeding from gastric varices is a life-threatening complication of portal hypertension. Fundal and isolated gastric varices are at high risk for variceal bleeding. In this study, we report our experience with n-butyl-2-cyanoacrylate (BC) in patients with large gastric varices. Study Twenty-nine patients (15 male, 14 female) with large fundal varices (active bleed, 5; passive bleed after eradication of esophageal varices, 13; unbled fundal varices with red color sign, 11) underwent endoscopic sclerotherapy with BC. Cirrhosis was present in 13 patients; extrahepatic portal venous obstruction, in 13; and noncirrhotic portal fibrosis, in 3. N-Butyl-2-cyanoacrylate after mixing with lipiodol (1:1) was given to the initial 10 patients and was given in undiluted form to the remaining patients, followed by injection of 0.7 mL of distilled water to rinse the injection catheter. One to three injections (0.5–1 mL) were given until all gastric varices became hard. All patients were on long-term endoscopic sclerotherapy or variceal ligation programs for eradication of esophageal varices. Results Acute variceal bleeding was controlled in all five patients with BC injections. Eradication of gastric varices was achieved in 27 (93.1%) patients (20 patients in 1 session, 4 patients in 2, and 3 patients in 3–6). Rebleeding occurred in three (10.3%) patients who responded to repeat BC injections. Complications related to the procedure occurred in two (6.9%) patients. In one patient, the needle became impacted into the tissue adhesive. This patient died 5 days later because of massive upper gastrointestinal bleeding. In the other patient, there was distal embolization. Conclusions Sclerotherapy of gastric varices with BC is a safe and an effective treatment for control of bleeding and eradication. The needle should be withdrawn immediately after the BC injection to prevent its impaction into the tissue adhesive.

Duplex Doppler sonography in patients with Budd-Chiari syndrome.

Background : Angiography has been the mainstay for diagnosis of Budd–Chiari syndrome even though other modalities are increasingly being used. We have evaluated our findings of duplex Doppler sonography (DDS) in patients with Budd–Chiari syndrome.

Primary sclerosing cholangitis : An experience from India

Primary sclerosing cholangitis (PSC) is considered to be rare in India. The aim of the present study was to investigate the incidence, clinical profile and outcome of PSC seen in a tertiary care centre. Over a period of 10 years (July, 1984‐June, 1994) 18 patients of PSC were diagnosed at cholangiography (14 patients by endoscopic retrograde cholangiopancreatography, two patients by percutaneous transhepatic cholangiography and two patients by both methods). The presence of secondary causes, such as choledocholithiasis, biliary tract surgery, congenital biliary tract anomalies, cholangiocarcinoma and pancreatic diseases, were excluded. These patients were evaluated retrospectively with respect to their clinical presentation, radiological findings, presence of associated idiopathic ulcerative colitis (IUC), treatment instituted and outcome. The mean (±s.d.) age at diagnosis of PSC was 39.0 (±16.1) years with a male: female ratio of 1.57:1. Nine (50%) patients had associated IUC. The diagnosis of IUC preceded that of PSC in all but one case. Fifteen (83.3%) patients had cholestatic jaundice at presentation, while three (16.7%) patients had asymptomatic rise of alkaline phosphatase. Three (16.7%) patients had recurrent cholangitis and five (27.8%) patients developed portal hypertension during the course of the disease. At cholangiography, intrahepatic radicles were involved in all and extrahepatic radicles in 12 (66.6%) cases. Patients were managed with steroids (n= 7), colchicine (n= 3), ursodeoxycholic acid (UDCA; n= 2) and methotrexate (n= 1), along with symptomatic measures. Mean duration of follow up available in 11 (61%) patients was 20.1 months (range: 1 month‐8 years). Four (36.4%) patients died. Steroids and colchicine did not have any effect while the one patient on UDCA and one on methotrexate showed improvement. In conclusion, in India PSC does not seem to be a rare entity. Its clinical profile and outcome are somewhat similar to those seen in Western countries.

Portal hemodynamics by duplex Doppler sonography in different grades of cirrhosis

Not much is known about the relationship betweenportal hemodynamics and the grades of cirrhosis. Usingpulsed Doppler ultrasonography, we studied portal veindiameter, portal flow velocity, and portal blood flow rate in 37 patients with livercirrhosis (11 Childs A, 13 Childs B, and 13 Childs C)and 10 healthy controls. There was no difference in themaximum inner diameter of the portal vein in cirrhotics and controls. However, there was a significantdecrease in the portal flow velocity in patients withChilds C cirrhosis, as compared to controls andpatients with Childs A and Childs B cirrhosis. The portal blood flow rate in Childs B and ChildsC cirrhosis was also significantly less as compared tocontrols and patients with Childs A cirrhosis. Patientswith ascites and encephalopathy had significantly lower portal flow velocities and blood flowrate as compared to those without ascites andencephalopathy, respectively. This study indicates thatportal flow significantly decreased in cirrhoticpatients with worsening Childs grade ofcirrhosis.