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Management of splenomegaly with thrombocytopenia is important in the treatment of portal hypertension. We propose a new concept: "Splanchnic Caput Medusae" in which enlarged spleen is her face and portal collateral pathways are her snake hairs. We report 2 demonstrable cases who were treated based on this new concept. Case 1 with refractory esophageal varices and splenomegaly was treated by stepwise partial splenic embolization (PSE) and endoscopic injection sclerotherapy with ligation. Spleen/liver volume ratio changed from 0.33 to 0.10. Hepatic venous pressure gradient changed from 19 to 14 mmHg. Case 2 with mesenteric shunt and splenomegaly was treated by stepwise PSE and retrograde obliteration. Spleen/liver volume ratio changed from 0.70 to 0.05. Hepatic venous pressure gradient changed from 11 to 7 mmHg. In these 2 cases, 3D-CT reconstruction images after treatment revealed that spleen- portal system reversed almost to normal form. We conclude that splenomegaly and portal collateral pathways could be considered as "Splanchnic Caput Medusae" and have to be treated by stepwise PSE.

Flood syndrome is a rare complication of cirrhosis of liver accompanied by ascites and a sudden rupture of umbilical hernia causing drainage of ascitic fluid from abdominal cavity. We report management of a case of Flood syndrome which was caused by rupture of incisional hernia. The clinical picture was similar to well described and widely accepted Flood syndrome. A 70-year-old female with decompensated hepatitis C cirrhosis was transported to the emergency department with a sudden drainage of ascitic fluid after sudden dehiscence of pre-existing incisional hernia and diffuse abdominal tenderness. Initially, she was managed by applying ostomy bag and diuretics to reduce the ascites. On 8th day of admission, a 16 Fr. drain was percutaneously placed in the left lower abdominal quadrant to divert the fluid from the abdominal wall defect. On 13th day, 80% partial splenic embolization (PSE) was attempted to control portal hypertension to reduce the ascites volume. After PSE, the hepatic venous pressure gradient reduced from 28 to 21cm H2O. The peritoneal drain was removed on 16th day and she was discharged on 22nd day. We conclude that PSE and temporary percutaneous peritoneal drainage are useful option to manage Flood syndrome.

We report a case of hemoperitoneum and sepsis from transhepatic gallbladder perforation in an 87-year-old male with acute cholecystitis who had past history of endoscopic sphincterotomy for common bile duct stone. Contrast-enhanced computed tomography (CT) showed intrahepatic and subcapsular low density areas. A wall defect of gallbladder was seen in coronal and sagittal - sections at the liver bed. Fluids obtained through the paracentesis were hemorrhagic. Percutaneous transhepatic gallbladder drainage (PTGBD) was attempted. First cholangiography revealed an orifice of fistula. Further injection of contrast medium drained into the intrahepatic secondary abscess and intraperitoneal cavity confirming the diagnosis of transhepatic gallbladder perforation. We conclude that contrast-enhanced CT with coronal and sagittal - sections and cholangiography via PTGBD tube are useful to confirm diagnosis of transhepatic gallbladder perforation.

Platelets participate in not only thrombosis and hemostasis but also other pathophysiological processes, including tumor metastasis and inflammation. However, the putative role of platelets in the development of solid organs has not yet been described. Here, we report that platelets regulate lung development through the interaction between the platelet-activation receptor, C-type lectin-like receptor-2 (Clec-2; encoded by Clec1b), and its ligand, podoplanin, a membrane protein. Clec-2 deletion in mouse platelets led to lung malformation, which caused respiratory failure and neonatal lethality. In these embryos, α-smooth muscle actin-positive alveolar duct myofibroblasts (adMYFs) were almost absent in the primary alveolar septa, which resulted in loss of alveolar elastic fibers and lung malformation. Our data suggest that the lack of adMYFs is caused by abnormal differentiation of lung mesothelial cells (luMCs), the major progenitor of adMYFs. In the developing lung, podoplanin expression is detected in alveolar epithelial cells (AECs), luMCs, and lymphatic endothelial cells (LECs). LEC-specific podoplanin knockout mice showed neonatal lethality and Clec1b-/--like lung developmental abnormalities. Notably, these Clec1b-/--like lung abnormalities were also observed after thrombocytopenia or transforming growth factor-β depletion in fetuses. We propose that the interaction between Clec-2 on platelets and podoplanin on LECs stimulates adMYF differentiation of luMCs through transforming growth factor-β signaling, thus regulating normal lung development.