Polysplenia syndrome in the adult patient. Case report with review of the literature.
OBJECTIVE
To report a case of polysplenia syndrome (PSS) in an adult patient.


BACKGROUND
The PSS is a form of situs ambiguous with multiple spleen, cardiac anomalies, abdominal heterotaxia, short pancreas, major venous system and bronquial malformations. It is a rare syndrome, more often found in childhood, and only the 10% of the patients that do not have cardiac anomalies can reach adulthood.


RESULTS
A 56 y/o male with obstructive jaundice and intestinal obstruction who was submitted to an abdominal laparotomy suspecting cholangiocarcinoma. He had choledocolithiasis, duodenal kinking by a preduodenal portal vein, intestinal levorotation, hypoplasic vena cava with a prominent acigos vein, short pancreas and polysplenia. A cholecistectomy, biliodigestive and gastroyeyunal bypasses were performed without any complications and with a successful evolution.


CONCLUSIONS
In conclusion, PSS is a rare hereditary syndrome that often occurs in childhood and its discovery in an adult is frequently fortuitus. Surgical treatment is an excellent therapeutic option, however is reserved just for complications. The outcome is good and the final evolution depends on the degree of the cardiac anomalies.
Plata-Muñoz
Juan José
JJ
Surgery Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, CP. 14000, Mexico City, Mexico.
Hernández-Ramírez
Daniel
D
Anthón
Francisco Javier
FJ
Podgaetz
Eitan
E
Avila-Flores
Francisco
F
Chan
Carlos
C
D000015:Abnormalities, Multiple / Q000473:pathology*; D006801:Humans; D007415:Intestinal Obstruction / Q000473:pathology*; D041781:Jaundice, Obstructive / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D013154:Spleen / Q000002:abnormalities*; D013577:Syndrome; D014057:Tomography, X-Ray Computed