Noncirrhotic Portal Vein Thrombosis Causing Sudden Unexpected Death

Abstract

Noncirrhotic portal vein thrombosis (PVT) is a rare disease that usually presents with small nonfatal "herald bleeding" with low mortality. Classic findings of noncirrhotic PVT include esophageal varices, splenomegaly, a normal liver, and an organized thrombus of the portal vein. We present a case of previously unreported sudden unexpected death from noncirrhotic PVT in an asymptomatic elderly woman, review the literature of this entity, and examine the pathophysiology of the formation of fatally ruptured varices. The portal vein must be carefully examined in all cases where there is no coexisting cirrhosis.

Esophageal Causes of Sudden and Unexpected Death

“…Varices most often arise from portal hypertension due to liver cirrhosis from a number of entities including alcoholic and primary biliary cirrhosis. Increased venous pressure within the communicating and gastroepiploic veins of the stomach and short gastric veins of the spleen results in varices within the lower esophagus (24). Victims of this condition often present to autopsy with histories of being found dead alone at their home address surrounded by copious amounts of vomited blood and fresh melena.…”

“…Portal vein thrombosis may be caused by a variety of other conditions, including sepsis, pancreatitis, metastatic carcinoma, biliary tract surgery, myeloproliferative and thrombotic disorders. In 50% of the cases, it is idiopathic and is usually associated with a benign clinical outcome, although unexpected death may occur (24). Bleeding esophageal varices have also been described as a rare complication of portal hypertension because of sarcoidosis (29).…”

Extrahepatic Portal Vein Obstruction in Egyptian Children

“…Clinical splenomegaly was present in 94% of our patients. Enlarged spleen is one of the classic findings of noncirrhotic PVT together with esophageal varices and normal liver (19)(20)(21). Thus, the possibility of PVT should be raised and a Doppler ultrasound performed in children and adolescents with upper gastrointestinal bleeding or isolated finding of splenomegaly during clinical examination (22).…”