Abstract

The extrahepatic congenital porto-systemic derivation is a very rare pathology of the venous vascular system of the abdomen described by John Abernethy in 1793. In this malformation there is a passage of substances not metabolized from the liver to the systemic circulation and dilation of the pulmonary capillary bed. It is divided into 2 groups according to the presence (type II) or total absence (type I) of portal flow. We present the case of a 6-year-old child, a twin product of the first pregnancy of a 22-year-old mother, attended at Vicente Corral Moscoso Hospital in the city of Cuenca, with respiratory symptoms without definitive diagnosis. The presence of a portal vascular abnormality was found in a casual abdominal ultrasound study diagnosed as Abernethy type II malformation by abdominal angiography and nuclear magnetic resonance. A thorough bibliographical review and a subsequent discussion of the case were carried out; finding as important data the existence of 80 cases reported until 2014 worldwide. In Latin America there is the report of a case in Venezuela in 2011 and in Argentina in 2016. We conclude by emphasizing the importance of a correct anamnesis, physical examination and the use of different diagnostic techniques in terms of respiratory symptoms due to there are rare pathologies that once an early diagnosed they can be treated successfully, thus giving the patient a normal life. The present case is the only one reported in Ecuador with a successful approach and treatment.

Keywords

malformation, portal vein, pulmonary hypertension, extrahepatic, congenital abnormality