RESUMO
Von Gierke disease is occasionally complicated by hepatic adenomas (HAs) causing great concern owing to the current difficulties in monitoring them regarding malignant transformation. Orthotopic liver transplantation (OLT) is proposed as a therapeutic tool when multiple HAs and poor metabolic control are present, owing to the lack of a clear-cut criterion to detect early malignant transformation, whether or not associated with growth retardation. Focal nodular hyperplasia (FNH) has never been described together with multiple adenomas in von Gierke disease. We report a 26-year-old woman with von Gierke disease complicated by multiple HAs concomitant with FNH who underwent OLT and is now free from disease symptoms with good long-term outcome. In conclusion, although FNH could have been managed clinically, when multiple adenomas are present, OLT should be planned for some patients, mainly for those with poor metabolic control.
Assuntos
Adenoma/complicações , Hiperplasia Nodular Focal do Fígado/complicações , Doença de Depósito de Glicogênio Tipo I/complicações , Doença de Depósito de Glicogênio Tipo I/terapia , Neoplasias Hepáticas/complicações , Transplante de Fígado , Adenoma/diagnóstico , Adenoma/terapia , Adulto , Feminino , Hiperplasia Nodular Focal do Fígado/diagnóstico , Hiperplasia Nodular Focal do Fígado/terapia , Doença de Depósito de Glicogênio Tipo I/diagnóstico , Humanos , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/terapia , Prognóstico , Transplante Homólogo , Resultado do TratamentoRESUMO
At the present time several therapeutic options are used for the treatment of bleeding esophageal varices in patients with portal hypertension. We will review the main medical publications on transjugular intrahepatic portosystemic shunt (TIPS), a procedure seldom used among us. TIPS works as a portocaval side-to-side shunt and decreases the risk of esophageal bleeding through lowering of the portal system pressure and a decrease of the portal hepatic pressure gradient. TIPS consists in the percutaneous insertion, through the internal jugular vein, of a metallic stent under fluoroscopic control in the hepatic parenchyma creating a true porta caval communication. There are several studies demonstrating the efficacy of TIPS, although only a few of them are randomized and control-matched to allow us to conclude that this procedure is safe, efficient and with a good cost benefit ratio. In this review, we search for the analysis of the TIPS utilization, its techniques, its major indications and complications. TIPS has been used in cases of gastroesophageal bleeding that has failed with pharmacologic or endoscopic treatment in patients Child-Pugh B and C. It can be used also as a bridge for liver transplantation. Others indications for TIPS are uncontrolled ascites, hepatic renal syndrome, and hepatic hydrothorax. The main early complications of TIPS using are related to the insertion site and hepatic encephalopathy and the stent occlusion is the chief late complication.