Type of disease: Rare conditions

Polysplenia is a disorder in which an individual has multiple spleens that are about equal in size. This disorder is caused by improper connection between parts of the spleen during fetal development. Polysplenia is usually accompanied by other developmental problems, particularly severe heart defects, which often results in death before the age of 5. Polysplenia is believed to be caused by environmental factors that disturb development in utero (teratogenic) or genetic factors. Female infants are affected more often than males.

Complications that are commonly seen with polysplenia include intestinal malrotation, biliary atresia, and cardiac abnormalities. Intestinal malrotation occurs when the intestines do not rotate properly during normal fetal development, and may lead to poor blood supply or physical blockages that prevent the intestines from functioning normally. Biliary atresia is another developmental abnormality in which the bile ducts that normally drain bile from the liver are blocked, and may lead to the accumulation of bile that damages the liver. Cardiac problems include severe defects in the major veins and arteries, displacement of the chambers of the heart, and a heart that is located on the opposite side of the chest. Only about 10% of affected individuals have either minor or no cardiac problems, allowing them to survive into adulthood without symptoms.

Polysplenia is often diagnosed in infants after heart problems are noticed. In less severe cases, polysplenia is diagnosed incidentally after medical imaging or surgery for other reasons. Medication or surgery may be used to treat the symptoms of polysplenia. Surgery is more complicated in affected individuals due to abnormalities in the arrangement and placement of multiple organs. If you or your child has been diagnosed with polysplenia, talk to your doctor about the most current treatment options.

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