Congenital intrahepatic portosystemic shunt diagnosed during intrauterine life
نویسندگان
چکیده
OBJECTIVE To report a patient with prenatal diagnosis of portosystemic shunt; a rare condition in humans. CASE DESCRIPTION 17-day-old female infant admitted for investigation of suspected diagnosis of portosystemic shunt, presumed in obstetric ultrasound. The hypothesis was confirmed after abdominal angiography and liver Doppler. Other tests such as echocardiography and electroencephalogram were performed to investigate possible co-morbidities or associated complications, and were normal. We chose conservative shunt treatment, as there were no disease-related complications and this was intrahepatic shunt, which could close spontaneously by the age of 2 years. COMMENTS Portosystemic shunt can lead to various complications such as hepatic encephalopathy, hypergalactosemia, liver tumors, and hepatopulmonary syndrome. Most diagnoses are done after one month of age, after such complications occur. The prenatal diagnosis of this patient provided greater security for the clinical picture management, as well as regular monitoring, which allows the anticipation of possible complications and perform interventional procedures when needed.
منابع مشابه
Congenital multiple intrahepatic portosystemic shunt: an autopsy case.
Multiple intrahepatic portosystemic shunt (IPSS) without portal hypertension, now thought to be congenital in origin, is very rare. The presence of IPSS, unlike other congenital diseases, may not be recognized for several decades due to the time it takes to develop hepatic encephalopathy. In this article, we report an autopsy case of an 80-year-old Japanese woman with a one-month history of hyp...
متن کاملCongenital Intrahepatic Portosystemic Venous Shunt and Liver Mass in a Child Patient: Successful Endovascular Treatment with an Amplatzer Vascular Plug (AVP)
A congenital intrahepatic portosystemic shunt is a rare anomaly; but, the number of diagnosed cases has increased with advanced imaging tools. Symptomatic portosystemic shunts, especially those that include hyperammonemia, should be treated; and various endovascular treatment methods other than surgery have been reported. Hepatic masses with either an intra- or extrahepatic shunt also have been...
متن کاملAsymptomatic Congenital Intrahepatic Portosystemic Shunt
Congenital (spontaneous) intrahepatic portosystemic shunt is rare in the English literature. Most cases of portosystemic shunt occur after trauma, surgery, liver biopsy or as a result of chronic portal hypertension. Chronic shunting may result in encephalopathy, bleeding or hyperinsulinism. We report a case of an asymptomatic adult female with a presumed congenital intrahepatic portosystemic sh...
متن کاملLiver resection for the treatment of a congenital intrahepatic portosystemic venous shunt.
Intrahepatic portosystemic shunts (IPSS) are rare congenital anomalies arising from disordered portal vein embryogenesis. It has been described in both children and adults and may be asymptomatic or be associated with a variety of neurophysiological and pulmonary complications. When recognized, early intervention to occlude the shunt will reverse the associated complications. Literature review ...
متن کاملCongenital intrahepatic portosystemic shunt with variant inferior right hepatic vein.
We present a case of congenital intrahepatic aneurysmal portosystemic shunt in which the right portal vein communicates with both a variant inferior right hepatic vein and the right hepatic vein. We also describe the importance and efficacy of three dimensional computed tomography reconstruction images in determining subtle, small shunt vessels.
متن کامل