Autor(es)

Soutzopoulos Χ, Voultsinou D, Anastasiadou K, Avramidis O, PagKalidou E, Palladas P.

Paciente

féminino, 73 año(s)

A 74 year old female with history of liver cirrhosis re-evaluated for her disease. On clinical examination of the abdomen there was not any abdominal tenderness or pain. The patient underwent ultrasound.

A 74 year old female with history of cryptogenic liver cirrhosis with portal hypertension development, re-evaluated for her disease. On clinical examination of the abdomen there was no abdominal tenderness or pain. The patient underwent ultrasound. The liver was atrophic with nodular appearance (Fig 1) of the parenchyma and its margins. The nodules were discriminated by thick hyperechoic bands (Fig 2) attributed to the existence of fibrous tissue. A periportal cuffing (Fig 3) also demonstrated mainly at the left hepatic lobe. The portal vein (Fig 4a), its tributaries (Fig 4b) and the splenomesenteric confluence (Fig 4b) were patent and of normal size. The patient was under therapy and there were no signs of portal hypertension at the time. Splenic parenchyma (Fig 6) visualized with multiple tiny hyperechoic foci without acoustic shadowing.
No ascites, or portosystemic shunts (splenorenal, splenogastric, gastroesophageal or umbilical) were observed.

Liver cirrhosis is the terminal condition of liver diseases resulting from various aetiologies. Several explanations may be offered as possible underlying aetiologies. These include occult alcohol abuse, occult viral (non-B, non-C) hepatitis, silent autoimmune hepatitis, or progression of non-alcoholic steatohepatitis (NASH). Despite the recent development of diagnostic tools, no recognizable aetiology can be detected in approximately 5-31% of cirrhotic patients who are therefore diagnosed as having cryptogenic cirrhosis. a1 Antitrypsin phenotype abnormalities, such as phenotype MZ, are sometimes present among patients with cryptogenic cirrhosis in the absence of frank deficiency, but heterozygous carriage of these phenotypes is usually thought to potentiate some other condition rather than to explain cirrhosis.
Portal hypertension leads to splenomegaly with hyperplasia of the cells of the reticulo-endothelial system, which cover the sinusoids. Prolonged transit time of the blood and pressure increase disintegration of cells, leading to bleeding into the red pulp with deposition of siderin adjacent to the thickened collagen tissue, forming the so-called Gamna-Gandy bodies (GGB). Therefore, GGB contain fibrous tissue, hemosiderin and calcium. The lesions vary in size, but generally have a diameter ranging from a few millimetres up to at the most 1 cm.
Ultrasonography features of GGB include multiple, punctate, hyperechoic foci of echogenic nodules without acoustic shadowing. Unenhanced CT may detect GGB as multiple faint high-attenuation spots in the spleen. These spots represent calcification in siderotic nodules. CT however is far inferior to MRI in detecting GGB.
These nodules show in MRI, signal voids on all pulse sequences, due to their hemosiderin content. Gradient-echo sequences are considered the most sensitive for their detection. Lesion depiction improves on post-gadolinium-contrast images, because the spleen enhances and leads to increased signal-to-noise, therefore improving GGB detection.
Although GGB are the end-result of haemorrhages in the spleen caused by portal hypertension, they are not specific for portal hypertension. They are also seen in patients with portal vein or splenic vein thrombosis, haemolytic anaemia, leukaemia or lymphoma, acquired hemochromatosis, paroxysmal nocturnal hemoglobinuria and patients receiving blood transfusions, although they are rare findings in these diseases.