Polysplenia syndrome

Definition:

Polysplenia syndrome (PSS) is a rare subtype of heterotaxy syndrome with multiple spleens. It has been reported that most patients die before 5 years of age because the disease is often associated with congenital anomalies, such as cardiovascular anomalies. Some patients have a normal heart or only minor cardiac defects, are often diagnosed incidentally in patients being treated for other disease. The patient has bilateral bilobed lungs with hyparterial bronchi, abnormal location of abdominal solid organs and malrotation of bowels with multiple spleens. The etiology of PSS is not known yet. The causative factors of PSS are thought to be association of embryonic, genetic and teratogenic components.

References:

[1]. Güliz Yılmaz, et al. Polysplenia syndrome detected after chest symptoms in two adult patients: case report and review of literature. Pol J Radiol. 2014 Sep 13;79:311-4. [Content Brief]

[2]. Hyung-Il Seo, et al. Polysplenia syndrome with preduodenal portal vein detected in adults. World J Gastroenterol. 2008 Nov 7;14(41):6418-20. [Content Brief]

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