Original Article Prophylactic surgery in non-cirrhotic portal fibrosis: is it worthwhile? Sujoy Pal, P Radhakrishna, Peush Sahni, Girish K Pande, Samiran Nundy,* Tushar K Chattopadhyay Department of GI Surgery and Liver Transplantation, All India Institute of Medical Sciences, New Delhi, and *Department of GI Surgery and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi Background: In cirrhotic patients with portal hyper- tension 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, va- riceal bleeding rather than liver failure is the com- mon cause of death. In developing countries short- age 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 per- formed 45 prophylactic operations in patients with NCPF, if the patients had high-risk esophagogastric varices or symptomatic splenomegaly and hyper- splenism. Proximal lienorenal shunt was done in 41 patients and the remaining underwent splenectomy with (2 patients) or without (2 patients) devascular- ization. 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 glom- erulonephritis, 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. [ Indian J Gastroenterol 2005;24:239-242] See editorial on page 233 I n cirrhotic patients the mortality rate from the first episode of variceal bleeding has been reported to range from 30% to 70%. 1,2,3 However, measures to prevent the first bleed, such as endoscopic sclerotherapy or portasystemic shunts, have not been effective. In three of the four early trials 3-6 of prophylactic portasystemic shunts, patients who underwent operation did worse than those who did not, with deaths from post-shunt liver failure exceeding those from bleeding. Non-cirrhotic portal hypertension is common in India and the causes include extrahepatic portal venous obstruction and non-cirrhotic portal fibrosis (NCPF). This disease usually affects poor patients, who live in rural areas far from medical centers and have little or no access to facilities for blood transfusion. In the West, patients with non-cirrhotic etiologies have mortality ranging from 7% to 31% for a single bleeding episode. 7 In a Japanese national survey (1979-1988) of 671 cases of idiopathic portal hy- pertension, 24 were managed conservatively. On long-term follow up, the mortality was higher in the non-operated group and the majority of deaths were because of variceal hemorrhage. 8 Patients with NCPF generally have good liver function and a lower incidence of post-shunt en- cephalopathy. 9,10 For elective therapeutic splenorenal shunt procedures (to forestall a second or subse- quent bleed) the reported mortality rates are also much lower than in cirrhotics. 11,12 We have per- formed 285 elective proximal lienorenal shunt (PLRS) operations in patients with NCPF with a low mor- tality rate (0.8%), low variceal re-bleeding rate (8%) and low post-shunt encephalopathy rate (13%). 12 We therefore reckoned, that in spite of their inef- fectiveness in cirrhosis, it would be justifiable to perform prophylactic operations on a selected group of NCPF patients with high-risk varices and/or hypersplenism who came from rural areas of the country. Methods Between January 1976 and December 2001, 1180 patients with portal hypertension (extrahepatic portal venous obstruction: 720, NCPF: 323, cirrhosis: 94, hepatic venous outflow tract obstruction: 43) were operated on at the Department of Gastrointestinal Surgery, All India Institute of Medical Sciences. Of these, 45 patients (age range 12-45 years, median 23; Copyright © 2005 by Indian Society of Gastroenterology