A 49-year-old female was admitted to our hospital for acute pancreatitis. The abdomen CT scan incidentally showed midline liver with hepatomegaly, centrally located gallbladder, pancreas truncation, right sided small bowel, left sided large bowel, interruption of the inferior vena cava with azygos continuation, preduodenal portal vein, and multiple spleens in the left upper quadrant. In MRCP, the head of pancreas was enlarged and short main pancreatic duct without accessory duct was showed. EUS revealed enlarged ventral pancreas with a main pancreatic duct of normal caliber, absence of the accessory pancreatic duct and the dorsal pancreas. She was diagnosed as polysplenia syndrome with agenesis of dorsal pancreas. It is a rare congenital anomaly frequently associated with various visceral anomalies including multiple spleens, impaired visceral lateralization, congenital heart diseases, gastrointestinal abnormalities and azygos continuation of the inferior vena cava. We report a case of polysplenia syndrome with agenesis of dorsal pancreas presenting acute pancreatitis.
Acute Disease ; Congenital Abnormalities/*diagnosis/ultrasonography ; Endosonography ; Female ; Heterotaxy Syndrome/*diagnosis/ultrasonography ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Pancreas/abnormalities/ultrasonography ; Pancreatitis/*diagnosis ; Tomography, X-Ray Computed

Autosomal dominant polycystic kidney disease is a systemic disorder with cystic manifestations in the kidneys, liver, pancreas, seminal vesicles, and meninges; its noncystic manifestations affect mostly the vascular, cardiac, and connective tissues. Cardiovascular abnormalities, including mitral and aortic valvular prolapse and regurgitation and annuloaortic ectasia, have been considered important extrarenal manifestations of autosomal dominant polycystic kidney disease. But there were no reports with dilated cardiomyopathy in patients with polycystic kidney disease yet. We have experienced a case of dilated cardiomyopathy that is associated by autosomal dominant polycystic kidney disease in 40 year old male patient. Abdominal ultrasonography revealed variable sizes of multiple cysts in both kidneys and echocardiography showed the marked dilatation of left ventricle and severely depressed left ventricular systolic function (ejection fraction=19%). He was treated with diuretics intravenously and orally. Then dyspnea and abdominal distension was improved. This is the first case of dilated cardiomyopathy with autosomal dominant polycystic disease in Korea.
Adult ; Cardiomyopathy, Dilated* ; Cardiovascular Abnormalities ; Connective Tissue ; Dilatation ; Dilatation, Pathologic ; Diuretics ; Dyspnea ; Echocardiography ; Heart Ventricles ; Humans ; Kidney ; Korea ; Liver ; Male ; Meninges ; Pancreas ; Polycystic Kidney Diseases ; Polycystic Kidney, Autosomal Dominant* ; Prolapse ; Seminal Vesicles ; Ultrasonography