Profile of extrahepatic portal venous obstruction in Mumbai

To study the clinical profile of extrahepatic portal venous obstruction (EHPVO) in a tertiary referral centre in Mumbai.

Retrospective analysis of records of 113 patients with EHPVO, treated between January 1984 and May 1996.

Thirty eight of 54 (70.4%) patients in whom information was available were delivered at home. Eleven of the 50 (22%) had umbilical sepsis after birth. Median disease duration was 5 years (range 4 months-31 years), with age at initial presentation 13 (range 0.5-45) years; 24 (20%) patients presented after age 20 years. Eleven presenting initially with splenomegaly bled after 3 (1-14) years. Number of bleeding episodes per patient was 2.5 (1-12). 13 of 44 (29.6%) patients bled (first bleed or recurrence) after age 20 years. Twenty five (22.3%) had ascites at some time, 17/102 (16.7%) had hypersplenism, and hypoalbuminaemia was present in 20/103 (19.2%). Endoscopic sclerotherapy obliterated varices in 47/52 (90.4%) in 10.5 (3-40) sessions over 7 (1-100) months. Twenty three patients underwent surgery: devascularisation in 20 (with splenectomy in seven), distal lieno-renal shunt in two, and meso-caval shunt in one patient. Follow-up was available in 68 (60.2%) patients. Rebleeding after sclerotherapy occurred in 27/64 (42.2%), with median one (1-5) per patient; recurrence of varices was noted in 15/35 (42.9%) patients over 12 (3-39) months. Varices were present in 12 patients 163 (33-305) months after surgery.

Home delivery and umbilical sepsis may be risk factors in the development of EHPVO. A significant number of patients present or continue to bleed from varices after age 20. Variceal sclerotherapy is effective for eradication of oesophageal varices.