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The source of chronic pain may include:

• Cyst formation - Cyst formation causes increase in size and compression of the kidney and may be associated with constant, but not necessarily severe, pain which is worse when standing or walking. Cyst formation in the liver can be extensive and even more painful than in the kidneys.

• Mechanical changes of the spine - The size and location of the cysts (kidney or liver) can cause changes in abdominal girth which impact posture and can be associated with spinal changes. When the abdomen enlarges, it places pressure on the lumbar spine which can cause changes on muscles supporting the spine which can cause significant pain.

Extrarenal Cysts

Extrarenal cysts are cysts that develop outside of the kidney. Approximately 60% of patients with PKD develop cysts in the liver which can be even more painful than kidney cysts. Women who have had multiple pregnancies seem to develop larger cysts and more cysts than men. Liver cysts can become quite massive, however, liver failure is rare. The incidence of liver cysts increases with age.

Cysts in the kidneys or liver can become so large that they compress the stomach and cause a feeling of satiety in the patient. These patients must be carefully monitored for unintentional weight loss.

Approximately 5% of patients develop cysts in the pancreas and approximately 3% of individuals with PKD develop cysts in the colon or spleen.

Intracranial Aneurysm

An intracranial aneurysm is a bulge or ballooning of the wall of a blood vessel in the brain. If the aneurysm ruptures, it can lead to the accumulation of blood in the brain and can cause massive brain swelling and even death. Intracranial aneurysms are a serious complication and occur in 4-10% of people with PKD. Rupture of an intracranial aneurysm usually occurs in patients under the age of 50 whose blood pressure is not well controlled.

Circulatory Disturbances

Circulatory disturbances in patients with PKD may include:

• Anemia - underproduction of red blood cells
• Cardiac hypertrophy - enlargement of the heart
• Mitral valve prolapse - failure of the mitral valves in the heart to open and close properly which allows for a small amount of blood to flow back into the heart with each contraction (heartbeat). Mitral valve prolapse develops in approximately 25% of people with PKD.
• Aortic aneurysm - a bulging or widening of the aorta (the large blood vessel that carries blood from the heart to the rest of the body).

Diverticular Disease

Diverticular disease refers to a range of conditions that develop from the presence of one or more small pouches (diverticula) that protrude from the wall of the large intestine (colon). Symptoms may include abdominal pain, constipation, diarrhea, and rectal bleeding. Diverticular disease occurs in up to 8% of patients with PKD.