Splenoportal occlusion associated with a deficiency of factors 5 and 7 presenting with massive oesophageal variceal bleeding in a 40 year old African

RESUMO

Splenoportal occlusion presenting with massive haematemesis is reported in a 40-year old African with no previous history of liver disease. Physical examination was within normal limits except for a splenomegaly. There were no spider naevi. Endoscopy revealed large oesophageal varices extending from the middle third distally. Colour doppler ultra-sound showed the portal vein to be occluded. There was no history of umbilical sepsis or intra-abdominal septicaemia. Haematological investigation revealed a deficiency of factors 5 et 7 but no other congenital defect was present. Oesophageal transection was performed to control rebleeding after a transient relief with propranol. He has continued to do well two-yaers after the acute incident