Polysplenia can be isolated or associated with other congenital
malformations that are part of the polysplenia syndrome.
Polysplenia syndrome is a rare congenital disease characterized
by the presence of two or more spleens (Figure 1) and affection
of other asymmetric organs It is more commonly identified in
childhood than in maturity. It’s an uncommon heterotaxy condition
that affects one out of every 250 000 live births. Several studies
have attempted to categorize the wide range of abnormalities into
asplenia and polysplenia. Polysplenia has an underlying etiology
that is unknown. it has been linked to a variety of causes, including
embryonic, genetic, and teratogenic components [1,2].
Figure 1: Abdominal CT scan in an axial section without injection of contrast medium showing in the left hypochondrium
multiple nodular formations, well limited, homogeneous with clear contours (red arrows) concerning a polysplenia syndrome.
It is characterized by an abnormal arrangement of thoracic
and abdominal organs. The variable involvement of these different
organs results in a clinical polymorphism. Adults may experience
atypical biliary and pancreatic duct discharge, cholecystitis, and
intestinal blockage as a result of symptomatic polysplenia .
More than 40% of reported instances had cardiac abnormalities,
and the majority of these children do not survive past the age of
five; atrial septal defect (78 %), and azygous continuation of the
inferior vena cava (65%), and ventricular septal defect are the
most frequent cardiac defects (63 %) . There is no specific
pathognomic aberration that characterizes polysplenia syndrome,
although it has a wide spectrum of abnormalities. Multiple spleens
of comparable volume, visceral heterotaxia, right-sided stomach,
left-sided or huge midline liver, malrotation of the intestine, a short
pancreas, and inferior vena cava anomalies are only a few of the
abnormalities . The splenic structures are separated into masses
of roughly the same size, ranging in number from 2 to 6 and in
diameter from 1 to 6 cm. The splenic structures are separated into
masses of roughly the same size, ranging in number from 2 to 6 and
in diameter from 1 to 6 cm. The total quantity is equal to the mass
of a typical spleen (Figure 1). The spleens can be found in either the
left or right upper quadrant . Polysplenia is often discovered by
coincidence during an abdominal ultrasound or CT scan for another
reason . Adult polysplenia is frequently discovered during
the investigation for other reasons. To summarize, polysplenia
syndrome is a rare condition that can be accompanied by a variety
of systemic disorders.