Malformation of abernethy type 2. Presentation of a clinical case and review of literature.

Abstract

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 a case of a 6-year-old child, the product of twin pregnancy of the first pregnancy of a 22-year-old mother, who was treated at the Vicente Corral Moscoso Hospital in Cuenca, with respiratory symptoms, without an initial definite diagnosis. The presence of a portal vascular abnormality was found in a casual abdominal ultrasound study, which was diagnosed as Abernethy type II malformation by abdominal angiography and nuclear magnetic resonance.

A bibliographical review and subsequent discussion of the case was carried out; finding as important data, the existence of 80 cases reported until 2014 worldwide. In Latin America there is a case report in Venezuela in 2011 and in Argentina in 2016.

We conclude by emphasizing the importance of a correct anamnesis, physical examination and use of different diagnostic techniques in terms of respiratory symptoms due to there are rare pathologies that being diagnosed early, 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.

Key words: Congenital Abnormalities, Vascular Malformations, abernethy.