What is polysplenia?

Polysplenia syndrome refers to the association of 2 or more multiple spleens with multiple congenital abnormalities in abdomen and chest. However, some cases of polysplenia syndrome have been described with a single bilobed spleen or a single normal splenic gland.

What causes multiple spleens?

Polysplenia, or multiple spleens, is due to the absence of fusion of the primordial germs of the spleen. Therefore, between 1 and 6 small spleens are present in the abdominal cavity with dimensions between 1 cm and 6 cm; however, the total volume does not exceed the volume of a normal spleen [9].

What is heterotaxy syndrome with polysplenia?

In the abdomen, the condition can cause a person to have no spleen (asplenia) or multiple small, poorly functioning spleens (polysplenia). The liver may lie across the middle of the body instead of being in its normal position to the right of the stomach.

What is hetero taxi syndrome?

Heterotaxy syndrome is a rare birth defect that involves the heart and other organs. The beginning of the word (hetero-) means “different” and the end (–taxy) means “arrangement.” There are different forms of heterotaxy syndrome. All usually involve heart defects of varying types and severity.

How common is Mesocardia?

Mesocardia in an uncommon cardiac abnormality, in which the heart is positioned in the center of the thorax and its longitudinal axis lies in the thoracic mid-sagittal plane. The true incidence of this condition is unknown.

What causes Polysplenia?

The exact cause of polysplenia has not been defined. The probable hypotheses are embryonic (accelerated curvature of the embryonic body), genetic causes and teratogenic factors [7]. There is slight female preponderance and rare familial association has also been found [7].

What happens if you have multiple spleens?

One piece often is smaller than the other and is referred to as an accessory spleen. Multiple (accessory) spleens do not cause medical problems, and nothing is done about them. Rarely, accessory spleens may be confused with tumors.

How is heterotaxy diagnosed?

It is often diagnosed before birth, when an ultrasound identifies an abnormal position of one or more organs. Sometimes, heterotaxy is diagnosed with a fetal echocardiogram (a special ultrasound of the heart before birth) when a complex heart defect is identified.