Polysplenia

Polysplenia is a congenital disease manifested by multiple small accessory spleens, rather than a single, full-sized, normal spleen. Polysplenia sometimes occurs alone, but it is often accompanied by other developmental abnormalities. Conditions associated with polysplenia include gastrointestinal abnormalities, such as intestinal malrotation or biliary atresia, as well as cardiac abnormalities, such as dextrocardia. This condition occurs in 1 per 250,000 live births, with 75% of patients with polysplenia dying before the age of 5 and approximately 10% of patients making it to mid adolescence. Polysplenia occurs very rarely in adults and the elderly, because of the common association of the disorder to congenital heart defects which usually prevents those affected from reaching adulthood. Polysplenia affects more females than males.