Type:
Educational Exhibit
Keywords:
Arteriovenous malformations, Contrast agent-intravenous, Ultrasound-Colour Doppler, CT-Angiography, CT, Liver, Anatomy, Abdomen, Congenital
Authors:
F. Tavanti1, D. Sergi2, C. Bernardi1, C. De Vincentiis1, M. Di Pietropaolo3, A. Pallavicino1, L. Stefanetti3, V. David1; 1Roma/IT, 2reggio calabria/IT, 3Rome/IT
DOI:
10.1594/ecr2013/C-1934
Background
Congenital extra-hepatic porto-systemic shunts (Abernethy malformations) are extremely rare vascular malformations.
Their gross anatomy has not been well defined but a review of the literature indicates that are classified into two types: type 1 if the portal vein is absent and the entire portal blood drains to the vena cava,
type 2 when the portal blood is diverted into vena cava through extra-hepatic communication with a hypoplastic portal vein [1-3].
Differentiation between these two variants plays an essential role in determination of the definitive method of treatment.
While in patients with type 2 CEPS shunt occlusion can be an effective treatment,
in patients with type 1 CEPS liver transplantation is the only possible therapeutic approach when they develops symptoms of hepato-pulmonary syndrome or porto-systemic encephalopathy [4].
Diagnosis is usually made in childhood and only few cases affecting adults have been described [5].
Clinical presentations are not common in elder patients,
in which are usually related to chronic hepatopathy signs as hyperammonemia (usually secondary to a porto-systemic shunting) and regenerative nodules [6].
Literature describes also other congenital abnormalities findings,
as multiple cardiac defects,
skeletal deformities or skin lesions and reports an increasing in neoplastic-risk [7].