Portal annular pancreas is one of the pancreatic fusion anomalies in which the uncinate process of the pancreas extends to fuse with the dorsal pancreas by encircling the portal vein or superior mesenteric vein. We report two consecutive patients with portal annular pancreas. The first case is a 71-year-old male patient who underwent a pancreaticoduodenectomy for intraductal papillary mucinous neoplasm in the head of pancreas. His preoperative computed tomography scan showed the suprasplenic type portal annular pancreas. The second case is a 74-year-old female patient who underwent a laparoscopic anterior radical antegrade modular pancreatosplenectomy (RAMPS) for pancreatic body cancer. In operative finding, portal confluence (superior mesenteric vein-splenic vein-portal vein) was encased with the uncinate process of pancreas in both cases. Therefore, they required pancreatic division at the pancreatic neck portion twice. During the postoperative period, grade B and A, respectively, postoperative pancreatic fistulas occurred and were controlled by conservative management. Surgeons need to know about this rare pancreatic condition prior to surgical intervention to avoid complications, and to provide patients with well-designed, case-specific pancreatic surgery.
Adenocarcinoma, Mucinous/diagnosis/surgery ; Aged ; Female ; Humans ; Male ; Mesenteric Veins/radiography ; Pancreas/abnormalities ; Pancreatic Diseases/*diagnosis/therapy ; Pancreatic Fistula/etiology ; Pancreatic Neoplasms/diagnosis/surgery ; Pancreaticoduodenectomy/adverse effects ; Portal Vein/radiography ; Splenic Vein/radiography ; Tomography, X-Ray Computed

We report here on a case of primary extraskeletal mesenchymal chondrosarcoma that arose from the pancreas. A 41-year-old man was evaluated by CT to find the cause of his abdominal pain. The CT scans showed a heterogeneously enhancing necrotic mass with numerous areas of coarse calcification, and this was located in the left side of the retroperitoneal space and involved the body and tail of the pancreas. Portal venography via the celiac axis also showed invasion of the splenic vein. Following excision of the mass, it was pathologically confirmed to be primary extraskeletal mesenchymal chondrosarcoma that arose from the pancreas.
Abdominal Pain/etiology ; Adult ; Chondrosarcoma, Mesenchymal/complications/*diagnosis/surgery ; Contrast Media/administration & dosage ; Diagnosis, Differential ; Humans ; Iohexol/analogs & derivatives/diagnostic use ; Male ; Necrosis ; Pancreas/pathology/radiography ; Pancreatic Neoplasms/complications/*diagnosis/surgery ; Portal Vein/radiography ; Radiographic Image Enhancement/methods ; Rare Diseases ; Retroperitoneal Space/radiography ; Splenic Vein/radiography ; Tomography, X-Ray Computed/methods

Protein S (PS), a vitamin K-dependent glycoprotein, performs an important role in the anticoagulation cascade as a cofactor of protein C. Because of the presence of a pseudogene and two different forms of PS in the plasma, protein S deficiency (PSD) is one of the most difficult thrombophilias to study and a rare blood disorder associated with an increased risk of thrombosis. We describe a unusual case of previously healthy 37-year-old man diagnosed with portal-splenic-mesenteric vein thrombosis secondary to PSD. The patient was admitted to the hospital due to continuous nonspecific abdominal pain and nausea. Abdominal computed tomography revealed acute venous thrombosis from inferior mesenteric vein to left portal vein via splenic vein, and laboratory test revealed decreased PS antigen level and PS functional activity. Conventional polymerase chain reaction and direct DNA sequencing analysis of the PROS1 gene demonstrated duplication of the 166th base in exon 2 resulting in frame-shift mutation (p.Arg56Lysfs*10) which is the first description of the new PROS1 gene mutation to our knowledge. Results from other studies suggest that the inherited PSD due to a PROS1 gene mutation may cause venous thrombosis in a healthy young man without any known predisposing factor.
Adult ; Anticoagulants/therapeutic use ; Base Sequence ; Blood Proteins/*genetics ; Codon, Terminator ; Exons ; Humans ; Male ; Mesenteric Veins/radiography ; Polymorphism, Restriction Fragment Length ; Portal Vein/radiography ; Protein S Deficiency/complications/*diagnosis ; Sequence Analysis, DNA ; Splenic Vein/radiography ; Tomography, X-Ray Computed ; Venous Thrombosis/*diagnosis/drug therapy/etiology

Hepatic myelopathy is a rare complication of chronic liver disease that is associated with extensive portosystemic shunts. The main clinical feature of hepatic myelopathy is progressive spastic paraparesis in the absence of sensory or sphincter impairment. Early and accurate diagnosis of hepatic myelopathy is important because patients with early stages of the disease can fully recover following liver transplantation. Motor-evoked potential studies may be suitable for the early diagnosis of hepatic myelopathy, even in patients with preclinical stages of the disease. Here we describe two patients who presented with spastic paraparesis associated with a spontaneous splenorenal shunt and without any previous episode of hepatic encephalopathy. One patient experienced improved neurologic symptoms after liver transplantation, whereas the other patient only received medical treatment, which did not prevent the progression of spastic paraparesis.
Adult ; Disease Progression ; Evoked Potentials, Motor/physiology ; Hepatitis B, Chronic/complications/diagnosis ; Hepatitis C, Chronic/complications/diagnosis ; Humans ; Liver Cirrhosis/*complications/diagnosis ; Liver Transplantation ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Paraparesis, Spastic/etiology/pathology ; Renal Veins/*radiography ; Spinal Cord Diseases/*diagnosis/etiology/radiography ; Splenic Vein/*radiography ; Tomography, X-Ray Computed ; Vascular Fistula/*radiography