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Polysplenia syndrome, also known as left isomerism, is a type of heterotaxy syndrome where there are multiple spleens congenitally as part of left-sided isomerism.
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Polysplenia is seen predominantly in female patients. It is usually diagnosed in childhood or adulthood, later than asplenia syndrome, since associated congenital heart diseases tend to be less severe than those encountered in the latter.
The exact cause of polysplenia is unknown. However it is suggested that it is caused by various factors 10:
Clinical presentation is related to the associated pathology.
Polysplenia results from failure of development of the usual left-right asymmetry of organs and consists of situs ambiguus, with features of bilateral left-sidedness.
It is characterized by multiple splenules without a parent spleen. It occurs on the patient’s left side but may be bilateral. The most common associated feature is inferior vena cava interruption with azygos or hemiazygos continuation 3.
Other characteristic features include:
bilateral hyparterial bronchi
bilateral bilobed lungs
bilateral pulmonary/left atria
congenital heart disease (>50%): especially non-cyanotic, and less complex/severe than in asplenia syndrome 3
semi-annular pancreas / dorsal pancreatic agenesis 4,6,8
midgut malrotation (80%) 7,8
gallbladder agenesis (50%) and biliary atresia
absent C-loop of duodenum
inferior vena cava anomalies 7,8
intrahepatic IVC interruption with azygos/hemiazygos continuation
portal vein anomalies 7,8
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- 9. Yildiz AE, Ariyurek MO, Karcaaltincaba M. Yildiz AE, Ariyurek MO, Karcaaltincaba M. ScientificWorldJournal. 2013 Apr 21;2013:321810. doi: 10.1155/2013/321810. Print 2013. get pdf
- 10. de la Monte SM, Hutchins GM. Sisters with polysplenia. (1985) American journal of medical genetics. 21 (1): 171-6. doi:10.1002/ajmg.1320210125 - Pubmed