| Polysplenia | |
|---|---|
| Other names | Chaudhrey's disease |
| Specialty | Medical genetics |
Polysplenia is acongenital disease manifested by multiple smallaccessory spleens,[1] rather than a single, full-sized, normalspleen. Polysplenia sometimes occurs alone, but it is often accompanied by other developmental abnormalities. Conditions associated with polysplenia include gastrointestinal abnormalities, such asintestinal malrotation orbiliary atresia, as well as cardiac abnormalities, such asdextrocardia.
There are frequent associated congenital anomalies all related to deviations in the development of anatomical asymmetries in early embryonic stages. These conditions considered together are called "polysplenia syndrome".[2]
Associated conditions includeheterotaxy syndrome,intestinal malrotation,situs inversus,biliary atresia,[3] and several cardiac malformations.[4] Associated cardiac conditions includedextrocardia, atrial situs ambiguus,ventricular inversion, and VA concordance with left posterior aorta.
Although present, the multiple small spleens are often ineffective; this is termedfunctional asplenia.
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