a 42-year - old female visited our emergency room ( er ) with complaints of acute , diffuse abdominal pain .
she had been treated for three days at a local clinic because of fever and right upper quadrant pain .
the laboratory findings showed mild leukocytosis ( 11.210 cells/l ) and an elevated c - reactive protein level , and the hepatic parameters were markedly elevated .
the serum hemoglobin level was 7.8 g / dl and she was transfused with whole blood .
the ultrasound examination performed on an atl machine ( hdi 5000 , bothell , wa ) revealed a huge and heterogeneous lesion at the subcapsular area of the right hepatic lobe and also a perihepatic fluid collection ( fig .
the differential diagnosis was hepatic subcapsular abscess or a hepatic mass such as a cavernous hemangioma draping over the liver .
mdct was performed with a 4-channel scanner ( volume zoom ; siemens , erlangen , germany ) with using following parameters ; 120 kvp , 165 mas , 3 mm collimation and a 1.5 mm reconstruction interval for the arterial and portal venous phases , and 7 mm collimation and a 5 mm reconstruction interval for the unenhanced and equilibrium phase images .
after injection of 120 ml of non - ionic contrast material , the arterial phase images were obtained with a 40 sec delay , and 80 sec and 3 min delays were used for the portal venous and delayed phases , respectively .
coronal multiplanar reconstruction ( mpr ) was done at each phase . on the unenhanced images , about a 9 cm - sized , low density , irregular - shaped lesion was seen at the right hepatic dome . most of the lesion was not enhanced , but multiple , irregular and linear , septa - like densities were demonstrated which were of isoattenuation to the adjacent normal parenchyma on all phases ( fig .
the unenhanced images also revealed a huge , heterogeneous and mainly hyperdense lesion occupying the entire right subcapsular area , and this was suggestive of acute hematoma ( fig .
most of the lesion was not enhanced , except for multiple small enhancing foci that were gradually enlarged and isodense on all phases as compared with the liver parenchyma .
the margin of the lesion at the junction with the normal parenchyma was shaggy and irregular .
another 1 cm - sized lesion was seen at the right hepatic lobe , and the lesion was of low density on the arterial phase ; it showed peripheral isodense enhancement on the portal venous phase and it became totally isodense or slightly hyperdense on the delayed phase ( fig .
the mpr images helped us view all the above - mentioned findings at a glance ( fig .
angiography was performed for vascular embolization under the impression of rupture of hepatic tumor such as hepatic adenoma after the ct scan .
the angiogram revealed a huge hypovascular area that conformed to the entire right subcapsular portion , which was compatible with hematoma , and an irregular parenchymal defect was seen in the hepatic dome without any feeding vessels , definite contrast accumulations or vascular malformations ( fig .
so , interventional procedures were not done and the diagnosis was not changed even after angiography .
the patient was given supportive care after angiography , but she suffered cardiac arrest that necessitated cardiopulmonary resuscitation .
finally , emergency right lobectomy was performed about 24 hours after her er admission . at laparotomy ,
most of the portion of the right hepatic lobe was replaced by hematoma , and some inflammatory patches were present on the liver surface .
a mass - like lesion was noted at segment 8 , which had ruptured , and this resulted in a large amount of blood and necrotic material in the peritoneal cavity . on microscopic examination ,
the lesion was diagnosed as peliosis hepatis which was a blood filled cavity lined by fibrinous material and hepatocytes ( fig .
she was discharged about one month after surgery and has lived healthy without any symptoms or abnormal findings on follow - up ct examinations two years later .
the etiology of peliosis hepatis is unknown ; however , a number of causes have been postulated and its pathogenesis remains uncertain ( 10 ) .
peliosis hepatis has been considered as a rare , incidental autopsy finding that 's associated with chronic wasting diseases such as tuberculosis and malignancies .
it has been associated with renal transplantation , hematologic disorders and infections such as pulmonary tuberculosis and human immunodeficiency virus .
it has also been reported in patients treated with long - term with anabolic steroids , oral contraceptives , hormones or azathioprine ( 7 ) . in 25 - 50% of the cases ,
no associated condition has been identified , the same as in this case ( 9 ) .
they range from asymptomatic to signs of acute infection and even to hepatic failure and intraabdominal hemorrhage .
the patients with complicated fatal hemorrhage probably could have been successfully managed by surgical intervention such as partial hepatic resection or hepatic dearterialization , interventional selective hepatic arterial embolization or liver transplantation and adequate supportive care . although there were some cases in which the patients died after treatment , the other patients who had survived the disease became healthy without any symptoms ( 2 , 3 ) .
two morphologic patterns of hepatic peliosis were described by yanoff and rawson ( 10 ) . in the phlebectatic type ,
the blood - filled spaces are lined with endothelium and they are based on by aneurismal dilatation of the central vein ; in the parenchymal type , the spaces have no endothelial lining and they usually are associated with hemorrhagic parenchymal necrosis .
senf as reviewed by zak ( 1 ) considered the two morphological patterns as one process initiated by focal necrosis of the liver parenchyma , which transforms into an area of hemorrhage ( the parenchymal pattern ) .
this pattern may progress to formation of fibrous walls and an endothelial lining around the hemorrhage ( the phlebectatic type ) , or heal by fibrin deposition , thrombosis and sclerosis of the vascular spaces . when reviewing the reports that have described the radiologic findings of peliosis hepatis with using various modalities , including angiography , us , ct and mri
the imaging findings were so variable , depending on the histopathologic findings , including lesion size , the extent of communication with the sinusoids and the presence of thrombosis or hemorrhage within a lesion ( 8) . as for the angiographic appearances of peliosis hepatis , multiple small contrast accumulations or puddles have been described ( 2 - 4 ) . yet
such findings were not shown in the present case because the major portion of the affected area was necrotized and replaced by hemorrhage , which attributed to delayed diagnosis and was the reason why radiologic intervention was not performed .
hypoechoic or hyperechoic nodule or diffuse heterogeneous hepatic echotexture have been reported as us findings ( 5 , 6 ) . in our case ,
only a heterogeneous subcapsular mass - like lesion was noted and interpreted as abscess that correlated to the signs of clinical infection , but in retrospect , we could have found a hyperechoic nodule at segment 8 , which was considered to be a lesion that was demonstrated on ct scan .
the mri findings for peliosis hepatis largely depend on the age or status of hemorrhage , with hypo- , iso- , or hyperintensities on t1-weighted images and hyperintensity on t2-weighted images
. the contrast enhancement may be usually slow and late ( 7 - 9 ) .
on the unenhanced ct , the lesions are usually of low density , but the attenuation will vary with regard to the age of the lesion , as well as the presence of hemorrhage . they have been reported to usually become isodense or slightly hyperdense at the late venous or equilibrium phase with or without a central unenhancing portion , although with the presence of hemorrhage they may show little enhancement ( 8) .
( 7 ) reported a unique pattern of the early and high enhancement with centrifugal progression on triphasic ct ; kleinig et al .
( 5 ) reported a small lesion of which density was high on the arterial and portal venous phases , respectively . in our case ,
a small lesion in the right lobe showed gradual and centripetal enhancement , and the attenuation was nearly the same as that of parenchyma .
we suggest that this feature might be a more usual dynamic enhancement pattern on triphasic - enhanced ct , including the arterial , portal and delayed phases , in the lesions without necrotic change or other complications .
until now , there has been no report describing the contrast - enhanced dynamic ct features in a case with peliosis hepatis with hemorrhagic necrosis , intrahepatic hemorrhage and rupture .
here we demonstrated several features that can provide radiologists with clues suggesting that the cause of extensive subcapsular hemorrhage is a peliosis hepatis , or that a low density , mass - like lesion may be peliosis hepatis with hemorrhagic necrosis .
the first clue is multiple small enhancing foci that are located at the boundary of the acute subcapsular hemorrhage and enlarge according to the passage of time .
these densities are isodense to the parenchyma on all phase images , with the highest hounsfield number on the portal venous phase .
this finding is considered to correspond to multiple small accumulations of contrast material on angiography , and this finding was first described by pliskin ( 4 ) . in our case
, the densities were not visualized on us or angiography and it is now clear that visualization of these small foci is attributed to the excellent spatial resolution of mdct .
the second clue is a shaggy border of parenchyma with hemorrhage , which may give an impression that the subcapsular high density is not just simple hemorrhage , but it is associated with peliosis hepatis .
the last clue is multiple , irregular and linear , septa - like structures inside a low density mass , which show isoattenuation compared to the adjacent parenchyma on all phases .
the structures may suggest uncomplicated peliotic parenchyma in contrast to the surrounding , hemorrhagic necrosis .
the differential diagnosis of peliosis hepatis may be different according to the presented imaging findings . in the case of hypervascular ,
well - enhancing lesions , the differential diagnosis includes benign hepatic tumors such as hepatic hemangioma , adenoma , focal nodular hyperplasia , hemangiosarcoma , hypervascular metastasis or arteriovenous malformation . a hypovascular lesion must be differentiated from abscess , hypovascular metastasis or other necrotic masses .
yet the addition of the delayed phase imaging can help rule out abscess or metastasis via the finding of disappearance or a decrease in size of the lesion , as compared to the arterial or portal phase images . in conclusion ,
familiarity with the imaging characteristics and the possible clinical manifestations of peliosis hepatis with or without complications can help radiologists make the early and correct diagnosis .
thin - section , triphasic - enhanced mdct can provide quite useful and conclusive information for the diagnosis of peliosis hepatis .
 we report here on an uncommon case of peliosis hepatis with hemorrhagic necrosis that was complicated by massive intrahepatic bleeding and rupture , and treated by emergent right lobectomy . 
 we demonstrate the imaging findings , with emphasis on the triphasic , contrast - enhanced multidetector ct findings , as well as reporting the clinical outcome in a case of peliosis hepatis with fatal hemorrhage . 
CASE REPORT
DISCUSSION