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.
