[2] Improvements in imaging and awareness have contributed to an increase in cases[3][4] Thus a large part of medical and scientific literature on the subject is grounded in veterinary medicine.
Blood leaving the digestive tract is rich in nutrients, as well as in toxins, which under normal conditions undergo processing and detoxification in the liver.
This blood passes by the network of capillaries before being evacuated by the hepatic veins into the inferior vena cava and subsequently the heart.
[6] Neonatal cholestasis, liver tumours, hepatopulmonary syndrome, pulmonary hypertension and encephalopathy are common clinical manifestations of CPSS.
[6] In adults, the discovery of a CPSS is often fortuitous but can also occur in response to the detection of one or several characteristic complications such as hepatic encephalopathy, hepatopulmonary syndrome and pulmonary hypertension.
[10] Other complications of CPSS are hyperandrogenism, pancreatitis, vaginal bleeding, and lower urinary tract symptoms like nephrolithiasis (kidney stones) and haematuria (presence of blood in the urine).
The clinical manifestations of intra- and extra- hepatic portal systemic shunts can be similar; however the pathophysiology and treatment of the two types are distinct.
Upon discovery of a CPSS in a child, it is important to rule out portal hypertension or hepatic hemangioma as the cause of the shunt, either of which would require a specific treatment.