RESUMEN
BACKGROUND: Polyneuropathy organomegaly endocrinopathy M-protein and skin changes (POEMS) syndrome is a rare paraneoplastic syndrome caused by a potential plasma cell tumor. The clinical manifestations of POEMS syndrome are diverse. Due to the insidious onset and lack of specific early-stage manifestations, POEMS syndrome is easily misdiagnosed or never diagnosed, leading to delayed treatment. Neurological symptoms are usually the first clinical manifestation, while ascites is a rare symptom in patients with POEMS syndrome. CASE SUMMARY: A female patient presented with unexplained ascites as an initial symptom, which is a rare early-stage manifestation of the condition. After 1 year, the patient gradually developed progressive renal impairment, anemia, polyserosal effusion, edema, swollen lymph nodes on the neck, armpits, and groin, and decreased muscle strength of the lower extremities. The patient was eventually diagnosed with POEMS syndrome after multidisciplinary team discussion. Treatment comprised bortezomib + dexamethasone, continuous renal replacement therapy, chest and abdominal closed drainage, transfusions of erythrocytes and platelets, and other symptomatic and supportive treatments. The patient's condition initially improved after treatment. However, then her symptoms worsened, and she succumbed to the illness and died. CONCLUSION: Ascites is a potential early manifestation of POEMS syndrome, and this diagnosis should be considered for patients with unexplained ascites. Furthermore, multidisciplinary team discussion is helpful in diagnosing POEMS syndrome.
RESUMEN
BACKGROUND: Intrahepatic portosystemic venous shunt (IPSVS) is a rare hepatic disease with different clinical manifestations. Most IPSVS patients with mild shunts are asymptomatic, while the patients with severe shunts present complications such as hepatic encephalopathy. For patients with portal hypertension accompanied by intrahepatic shunt, portal hypertension may lead to hemodynamic changes that may result in exacerbated portal shunt and increased shunt flow. CASE SUMMARY: A 57-year-old man, with the medical history of chronic hepatitis B and liver cirrhosis, was admitted to our hospital with abnormal behavior for 10 mo. He had received the esophageal varices ligation and entecavir therapy 1 year ago. Comparing with former examination results, the degree of esophageal varices was significantly reduced, while the right branch of the portal vein was significantly expanded and tortuous. Meanwhile, abdominal ultrasound presented the right posterior branch of portal vein connected with the retrohepatic inferior vena cava. The imaging findings indicated the diagnosis of IPSVS and hepatic encephalopathy. Instead of radiologic interventions or surgical therapies, this patient had only accepted symptomatic treatment. No recurrence of hepatic encephalopathy was observed during 1-year follow-up. CONCLUSION: Hemodynamic changes may exacerbate intrahepatic portosystemic shunt. The intervention or surgery should be carefully applied to patients with severe portal hypertension due to the risk of hemorrhage.