Prophylactic surgery in non-cirrhotic portal fibrosis:is it worthwhile?

BACKGROUND: In cirrhotic patients with portal hypertension prophylactic portasystemic shunts have been found to be ineffective as deaths from post-shunt liver failure exceed those from bleeding. However, in patients with non-cirrhotic portal hypertension, variceal bleeding rather than liver failure is the common cause of death. In developing countries shortage of tertiary health-care facilities and blood banks further increases mortality due to variceal bleed. AIM: To study the results of prophylactic operations to prevent variceal bleeding in patients with portal hypertension due to non-cirrhotic portal fibrosis (NCPF). METHODS: Between 1976 and 2001, we performed 45 prophylactic operations in patients with NCPF, if the patients had high-risk esophagogastric varices or symptomatic splenomegaly and hypersplenism. Proximal lienorenal shunt was done in 41 patients and the remaining underwent splenectomy with (2 patients) or without (2 patients) devascularization. RESULTS: There was no operative mortality. Thirty-eight patients were followed up for a mean 49 (range, 12-236) months. Three patients bled - one was variceal and two due to duodenal ulcers; none died of bleeding. There were 2 late deaths (6 weeks and 10 years after surgery), one from an unknown cause and one due to chronic renal failure. The delayed morbidity was 47%. This included 7 patients who developed portasystemic encephalopathy, 4 glomerulonephritis, 2 pulmonary arteriovenous fistulae and 5 ascites requiring treatment with diuretics. Thus only 20 (53%) patients were symptom-free on follow up. CONCLUSIONS: Prophylactic surgery is safe and effective in preventing variceal bleeding in NCPF but at the cost of high delayed morbidity.

Appendiceal tumour--retrospective clinicopathological analysis.

Appendiceal tumours are rare and often discovered unexpectedly in an acute situation in which decision-making is difficult. We report the spectrum of appendiuar tumours seen in our institution over a period of more than 10 years, and discuss the clinicopathological behaviour, investigations, surgical procedures and outcomes in these patients. We have also reviewed the literature with regard to appendiceal tumours. Appendicular tumours were identified from the database of 1646 appendictomies (18% in children) performed in single centre and case notes were reviewed. Clinical presentation, investigations, histopathology, surgical procedures and outcome were analysed. Twelve patients with appendiceal tumours were identified (0.72%): 8 carcinoid, 2 mucinous (mucocele) and 2 adenocarcinoma. All the patients with a carcinoid tumour presented with features suggestive of acute appendicitis and were diagnosed postoperatively following appendicectomy and formal histology. No further surgical intervention was required as these lesions were less than 1cm away from the base of the appendix. One of the patient with mucinous cystadenoma presented acutely and underwent an appendicectomy; in the other patient with chronic pain, apreoperative MRI suggested the diagnosis leading to a planned hemicolectomy as the lesion was close to the base of the appendix. While one of the patient with an adenocarcinoma localized to the appendix did well following a right hemicolectomy, the other patient with disseminated disease succumbed within a year. Carcinoid tumours are the commonest appendiceal tumours, which present often as acute appendicitis. While appendicectomy would be adequate in most of these patients, in patients with a cystadenoma close to the base of the appendix or in case of a carcinoma, a right hemicolectomy is the appropriate option. While the prognosis is good in patients with carcinoid tumour and cystadenoma, it remains dismal in patients with disseminated malignant disease.

Spectrum of hemostatic derangements, in Budd-Chiari syndrome.

BACKGROUND: Hemostatic abnormalities have been reported in various hepatocellular diseases. We evaluated the hemostatic functions in patients with Budd-Chiari syndrome. METHODS: Biochemical liver function tests, and measurement of prothrombin time, activated partial thromboplastin time, and plasma levels of anti-thrombin III (antigen) and activity of protein C were done in 36 patients with Budd-Chiari syndrome. RESULTS: Liver biochemistry was abnormal in 34 patients. Plasma prothrombin time and activated partial thromboplastin time were prolonged in 17 (47%) and 23 (64%) patients, respectively. Antithrombin III antigen levels and protein C activity were reduced in 15 (50%) and 25 (83%) patients, respectively, among the 30 patients studied. Albumin levels showed significant correlation with coagulation test results, levels of anti-thrombin-III, and protein C activity. CONCLUSION: Hepatic synthesis of coagulation factors and anticoagulants is reduced in Budd-Chiari syndrome; this may play a role in recurrence of thrombosis.