Polysplenia syndrome in the adult patient. Case report with review of the literature.

AIMS: 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.

[Portal biliopathy]. / Biliopatía portal.

Portal biliopathy is a rare condition that is usually not diagnosed. It is associated with presence of varix around bile duct with concomitant ischemic damage and structural alterations of bile duct wall; this produces obstructive phenomena. There are scarce reports on the literature this entity. In the present paper, we report two cases in which obstruction of extrahepatic bile duct was associated with cholecystitis with well-documented extrahepatic portal hypertension. Both cases were managed with cholecystectomy and endoscopic placement of endoprothesis. Treatment of portal biliopathy should be adjusted to the individual patient's characteristics. It is focused on the one hand on management of portal hypertension and on the other hand to management of obstructive jaundice. When cholecistitis is found, cholecistectomy should to be performed. If the patient develops concomitant gastrointestinal bleeding due to portal hypertension, management of the problem could require surgical devascularization, shunting procedures, or endoscopic variceal ligature.

Central pancreatectomy as an indication for various benign pancreatic tumors.

Standard pancreatic resections, either proximal or distal ones, result in a considerable loss of pancreatic parenchyma and may cause impairment of the exocrine and endocrine functions. Central pancreatectomy has been indicated for small benign lesions located in the neck or body of the pancreas. It has the potential advantage of lowering the risk of functional impairment of the pancreatic parenchyma, biliary tract, upper gastrointestinal tract, and spleen. We present three cases of patients with benign, isolated pancreatic tumors who underwent a successful central pancreatectomy. From this small series, we believe that central pancreatectomy is an excellent therapeutic option for benign, localized pancreatic tumors.

Current role of surgery for the treatment of portal hypertension.

Portal hypertension surgery has evolved widely in the last decades. Since the first surgical shunt was done in 1945 for the treatment of recurrent hemorrhage, many surgical options have been developed including selective shunts, low diameter shunts and extensive devascularization procedures. Many of them have been studied and compared showing their advantages and disadvantages, evolving also their role in the therapeutic armamentarium. Surgery is nowadays a second line treatment option (after b blockers and endoscopic therapy), and it's main indication is for patients whose main and only problem is history of bleeding, with good liver function (Child-Pugh A). For emergency situations it has a very limited role and for primary prophylaxis virtually has also no role. Patients with good liver function, electively operated with portal blood flow preserving procedures are the patients that benefit from surgical treatment. Patients with a bad liver function are better candidates for a liver transplant.