ETIOLOGY, PRESENTATION AND NATURAL-HISTORY OF EXTRA-HEPATIC PORTAL VENOUS OBSTRUCTION

  • 1 January 1979
    • journal article
    • research article
    • Vol. 48  (192) , 627-639
Abstract
Extra-hepatic obstruction of the portal vein is a well-known cause of hypertension in childhood and 55 out of the 97 patients (57%) seen with this condition presented before they were 15 yr old. Nearly 1/2 of the cases (43%) presented in adult life. Hematemesis was the commonest mode of presentation in both the adult and childhood group, although splenomegaly was common, especially in the children. The severity and frequency of hematemesis increased during puberty in the children. Twelve children and 3 adults have now not bled for between 5 and 30 yr, 10 of them ceasing spontaneously and 5 following surgery. Intra-abdominal infection or septicemia precipitated the portal venous obstruction in 38 patients (39%), though the exact cause of such obstruction in patients who had no cirrhosis was obscure in about 1/2 the cases. Ascites was presented in 18/51 (35%) of the children and 24/35 (69%) of the adults. Its presence was associated with an increased mortality (P < 0.01). Out of 42 (45%) patients with ascites, 19 also had portal-systemic encephalopathy. Of the 24 patients who died, 9 had presented during childhood; variceal hemorrhage was responsible for death in 19 and infection in 5. Sixty-four patients underwent 114 operations for variceal hemorrhage. Mortality was greater in the surgical group compared with those managed conservatively. Surgery is therefore indicated only in the rare case where bleeding cannot be controlled by medical means.

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