The polysplenia syndrome is a rare hereditary disorder that may go unrecognized until one of its complications occurs.

Features of the polysplenia syndrome:

(1) 2 or more spleens of identical size

(2) other congenital malformations


Congenital malformations may include:

(1) situs inversus

(2) cardiac malformations (atrioventricular septal defects, dextrocardia, etc)

(3) pancreatic malformation (short, semiannular, etc)

(4) vascular malformations affecting portal vein, azygous vein, inferior vena cava, etc, including preduodenal portal vein (PDPV)

(5) hepatic malformations

(6) pulmonary malformations

(7) gastrointestinal malformations

(8) midline gallbladder

(9) genitourinary malformations

(10) central nervous system malformations


Complications may include:

(1) torsion, which may present as acute abdominal pain

(2) infarction

(3) surgical complications (especially during cholecystectomy)


Differential diagnosis:

(1) accessory splenic tissue

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