Subsequent Problems That Can Develop Due to Portal Hypertension:
Hepatomegaly (enlarged liver), splenomegaly (enlarged spleen due to increased pressure in the liver), esophageal varices (distended, weakened, and blood-filled veins under pressure, or sacs with a thin esophageal wall, having the potential to bleed suddenly and unexpectedly into the esophagus). Severe, abrupt bleeding of esophageal varices can cause the stomach to fill with large amounts of blood, causing bloody vomitus (throw-up). This is a life-threatening medical emergency due to the large loss of blood volume (see care of bleeding esophageal varices below). Proper care is needed immediately.
Portal circulation refers to the passage of blood in the portal vein, spleen and gastrointestinal tract (GI tract). Veins from the stomach and intestines, which feed the portal vein, may also be under pressure. Bleeding from the GI tract may result in stools that are bloody, black or tarry in consistency and medical care should be sought immediately.
Hypersplenism is an exaggeration of the hemolytic (blood destruction) function of the spleen and may develop with advanced splenic enlargement, resulting in deficiency of blood elements. Hypersplenism results in thrombocytopenia (low blood platelet count, utilized for blood clotting), anemia (low red blood count, oxygen carrying component of blood), and leukopenia (low blood leukocyte level, functions are complicated, but chiefly fight bacteria and other microorganisms).
Cholangitis is an infection of the bile and bile ducts with microorganisms and occurs due to bile sluggishness or stasis. Severe cholangitis can be life threatening. Caroli’s syndrome or disease refers to cystic dilatation of segments or the entire intrahepatic biliary system that may be present with ARPKD. These persons are at an increased risk for cholangitis. The threat of cholangitis lessens with age, and not every child with CHF has Caroli’s. Caroli’s is associated with chronic cholestasis and liver stones in young ARPKD adults. There are a few documented cases of liver cancer associated with CHF that have occurred in adulthood that may be due to years of chronic inflammation.