title: Polysplenia syndrome in the adult patient. Case report with review of the literature.
abstract: 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.
authors.affiliation: Surgery Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, CP. 14000, Mexico City, Mexico.
authors.forename: Juan José
authors.initials: JJ
authors.lastname: Plata-Muñoz
authors.forename: Daniel
authors.initials: D
authors.lastname: Hernández-Ramírez
authors.forename: Francisco Javier
authors.initials: FJ
authors.lastname: Anthón
authors.forename: Eitan
authors.initials: E
authors.lastname: Podgaetz
authors.forename: Francisco
authors.initials: F
authors.lastname: Avila-Flores
authors.forename: Carlos
authors.initials: C
authors.lastname: Chan
mesh_terms: 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