(Radiographics. 2000;20:367-378.)
© RSNA, 2000
Imaging of Nontraumatic Hemorrhagic Hepatic Lesions1
V. Javier Casillas, MD ,
Marco A. Amendola, MD,
Ana Gascue, MD ,
Nat Pinnar, MD ,
Joe U. Levi, MD and
Juan Manuel Perez, MD
1 From the Departments of Radiology (V.J.C., M.A.A., J.M.P.), Pathology (N.P.), and Surgery (J.U.L.), University of Miami School of Medicine, Miami, Fla; and Centro Médico de Caracas, Caracas, Venezuela (A.G.). Presented as a scientific exhibit at the 1998 RSNA scientific assembly. Received March 22, 1999; revision requested April 21 and received June 15; accepted June 21. Address reprint requests to V.J.C., Department of Radiology, Jackson Memorial Medical Center, 1611 NW 12th Ave, Miami, FL 33136 (e-mail: jcasilla@mednet.med.miami.edu).
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Abstract
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Spontaneous hepatic bleeding is a rare condition. In the absence of trauma or anticoagulant therapy, hepatic hemorrhage may be due to underlying liver disease. The most common causes of nontraumatic hepatic hemorrhage are hepatocellular carcinoma and hepatic adenoma. Such hemorrhage can also occur in patients with other liver tumors, such as focal nodular hyperplasia, hemangiomas, and metastases. Other conditions associated with this entity include HELLP syndrome, amyloidosis, and miscellaneous causes. Imaging plays a significant role in the diagnosis and management of this potentially lethal entity. In the appropriate clinical setting, the diagnosis of a hemorrhagic liver lesion is suggested when a hyperechoic mass or a mass with hyperechoic areas is seen at ultrasonography, a hyperattenuating mass is seen at computed tomography (CT), or a mass with high-signal-intensity areas is seen at T1-weighted magnetic resonance (MR) imaging. The signal intensity of blood can be increased or decreased on MR images depending on when the hemorrhage is imaged. The presence and extent of commonly associated subcapsular hematomas and hemoperitoneum can be easily ascertained with CT. During the first 24–72 hours, acute hematomas are hyperattenuating on nonenhanced CT scans; later, they decrease in attenuation and sometimes develop a pseudocapsule.
Index Terms: Amyloidosis, 761.68 • Angioma, gastrointestinal tract, 761.3194 • HELLP syndrome, 761.659 • Liver, focal nodular hyperplasia, 761.3198 • Liver, hemorrhage, 761.41, 761.77 • Liver neoplasms, diagnosis, 761.3192, 761.323 • Liver neoplasms, secondary, 761.33
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Introduction
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Spontaneous hepatic bleeding is a rare condition. In the absence of trauma or anticoagulant therapy, hepatic hemorrhage may be due to underlying liver disease. The most common causes of nontraumatic hepatic hemorrhage are hepatocellular carcinoma (HCC) and hepatocellular adenoma. Such hemorrhage can also occur in patients with other liver tumors, such as focal nodular hyperplasia (FNH), hemangioma, and metastases. Other conditions associated with spontaneous hepatic hemorrhage include HELLP syndrome, amyloidosis, and miscellaneous causes.
Imaging plays a significant role in the diagnosis and management of this potentially lethal entity. In this article, the clinical, histopathologic, and imaging features and treatment of nontraumatic hemorrhagic hepatic lesions are reviewed.
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Hepatocellular Carcinoma
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Ruptured HCC is an acute surgical emergency with an associated high mortality rate. HCC is the most prevalent malignant disease in the world, killing up to 1.25 million persons annually. Its prevalence has doubled in the United States over the past 30 years. In areas of Asia and Africa where the prevalence of this cancer is high, rupture is reported to occur in 6.9%–14% of cases and represents the most common cause of nontraumatic acute hemoperitoneum in male patients; however, ruptured HCC is relatively uncommon or rarely recognized in Europe and America (1–4). The reason for this difference is not clear, but it could be related to the size of the tumor at diagnosis. In high-prevalence areas such as Taiwan, Mozambique, and southeast China, HCC is commonly diagnosed in the 3rd and 4th decades of life and occurs much more frequently in men (male-female ratio of 8:1) (4). Some patients may have a history of minor blunt abdominal trauma (5). Clinical diagnosis of ruptured HCC may be difficult because there are no specific symptoms; in addition, the symptoms may not be dramatic and may be indistinguishable from those of uncomplicated HCC (6).
A spectrum of the disease exists with relatively minor intrahepatic bleeding progressing to subcapsular hemorrhage and ultimately rupture through the hepatic capsule with intraperitoneal hemorrhage. Subcapsular hematoma of the liver manifests as sudden onset of epigastric or right hypochondrial pain because of acute distention of the Glisson capsule. Hepatic rupture may be heralded by hypotension and rapid organ enlargement. Shock is present in 59%–90% of cases at admission, and 60%–100% of patients have signs of peritonitis or abdominal distention (7–9). Bloodstained ascites exists in almost all cases, and abdominal paracentesis is positive for blood in 86% of patients (3).
There has been speculation about the mechanism of spontaneous rupture of HCC. Some investigators believe that bleeding occurs from rupture of a parasitic artery or vein, such as the inferior phrenic vessel, feeding or draining the tumor (10). Others believe that the hemorrhage originates from laceration of a superficial liver tumor secondary to minor trauma, either from outside the body or from within as a result of repeated respiratory movement (5). Another hypothesis is that the pressure within the tumor increases when the branches of the hepatic veins are blocked as the cancer invades, so that the congestion within the tumor rapidly increases and bleeding and rupture occur when hepatic vein occlusion is complete (5,7,11).
Ruptured HCC has been predicted to occur in patients with large carcinomas located at the periphery of the liver, which protrude into the abdominal cavity and are devoid of overlying normal hepatic parenchyma at computed tomography (CT) (Figs 1, 2) (10).
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Figure 1a. Hemorrhagic HCC in a 79-year-old woman with acute onset of right upper quadrant (RUQ) pain and a history of HCC. (a) Nonenhanced CT scan shows a 4 x 5-cm, well-circumscribed mass of low attenuation (1) in segment 8 of the liver. (b) Contrast material-enhanced CT scan obtained 8 months later shows marked interval growth of the mass, which now has heterogeneous attenuation with central areas of high attenuation suggestive of fresh hemorrhage (arrows). At right hepatectomy, the presence of a hemorrhagic HCC was confirmed.
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Figure 1b. Hemorrhagic HCC in a 79-year-old woman with acute onset of right upper quadrant (RUQ) pain and a history of HCC. (a) Nonenhanced CT scan shows a 4 x 5-cm, well-circumscribed mass of low attenuation (1) in segment 8 of the liver. (b) Contrast material-enhanced CT scan obtained 8 months later shows marked interval growth of the mass, which now has heterogeneous attenuation with central areas of high attenuation suggestive of fresh hemorrhage (arrows). At right hepatectomy, the presence of a hemorrhagic HCC was confirmed.
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Figure 2a. Hemorrhagic HCC in a 53-year-old man with no history of HCC who presented with acute RUQ pain and hypotension. (a) Contrast-enhanced CT scan shows an exophytic mass in the right lobe of the liver (arrows). Note the high-attenuation fluid around the liver and spleen. (b) CT scan obtained at a lower level shows extension of the process inferiorly in the form of a large hemoperitoneum (arrow). Right hepatectomy showed a bleeding HCC associated with a significant hemoperitoneum.
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Figure 2b. Hemorrhagic HCC in a 53-year-old man with no history of HCC who presented with acute RUQ pain and hypotension. (a) Contrast-enhanced CT scan shows an exophytic mass in the right lobe of the liver (arrows). Note the high-attenuation fluid around the liver and spleen. (b) CT scan obtained at a lower level shows extension of the process inferiorly in the form of a large hemoperitoneum (arrow). Right hepatectomy showed a bleeding HCC associated with a significant hemoperitoneum.
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In a patient with an irregular liver and findings consistent with cirrhosis, the diagnosis of a hemorrhagic HCC is suggested when a hyperechoic mass or a mass with hyperechoic areas is seen at ultrasonography (US); when a hyperattenuating mass is seen at CT (Figs 1, 2); or when a mass with high-signal-intensity areas is seen at T1-weighted magnetic resonance (MR) imaging (12–16). However, one-third of HCCs have increased signal intensity on T1-weighted images, and this appearance can be due to steatosis, intracellular glycogen, or copper deposition in addition to hemorrhage (16). Also, the signal intensity of blood may be increased or decreased depending on the time the hemorrhage is imaged. The high attenuation of acute hematomas is most obvious at nonenhanced CT. CT is an effective method of diagnosing hepatic hemorrhage and can often indicate the underlying cause. The extent and age of the hematoma can be easily ascertained with CT. During the acute stage (the first 24–72 hours), the hematoma is hyperattenuating, but it decreases in attenuation and develops a pseudocapsule by 10–30 days (13). A subcapsular hematoma or hemoperitoneum is frequently seen at CT and appears as high-attenuation peritoneal fluid around the liver and spleen, sometimes with layering of the clot (hematocrit effect).
The most effective method of stopping bleeding from a ruptured HCC is transcatheter arterial embolization. The mortality rate for one-stage hepatic lobectomy, which is often performed under hurried and unfavorable conditions, has been as high as 75% (9). Another disadvantage of surgery for a ruptured HCC is the tenuous residual liver that may be left after resection in a patient with cirrhosis. If laparotomy is undertaken, hepatic artery ligation, preferably of the branch supplying the liver lobe bearing the tumor, should be considered together with hemostasis of the rupture site by various means (suture plication, packing, argon beam coagulation, use of microwaves or absolute alcohol) (3–9,17,18). Rupture of an HCC should be suspected in any patient with a history of cirrhosis or HCC who presents with acute epigastric pain. This entity should also be suspected in men from areas of Asia or Africa where HCC is endemic who present with sudden abdominal pain or hemoperitoneum.
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Hepatocellular Adenoma
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Hepatocellular adenoma is a rare benign liver tumor of uncertain origin. Since the introduction of oral contraceptives in 1960, its occurrence has increased dramatically, with an estimated incidence of four cases per year per 100,000 patients using long-term oral contraceptives (19–22). It is also seen in patients with glycogen storage disease, diabetes mellitus, or iron overload secondary to ß-thalassemia and in males using anabolic androgenic steroids (19–21). At histologic analysis, hepatocellular adenoma is characterized by cords of well-differentiated hepatocytes that can produce bile; however, there are no bile ductules present to enable biliary excretion. The hepatocytes are commonly rich in fat and glycogen. The tumor architecture lacks portal vein tracts and terminal hepatic veins, thus helping differentiate it from normal liver tissue or adenomatous hyperplasia. Hepatocellular adenoma manifests as a solitary lesion in 80% of cases and multiple lesions in 20% and is associated with an increased risk of malignant transformation. It may be clinically silent and may be incidentally discovered at laparotomy for an unrelated problem or appear as an asymptomatic RUQ mass at imaging. Hepatic adenomas may rupture and manifest as limited bleeding causing RUQ pain suggestive of acute cholecystitis. On occasion, the initial bleeding may be severe enough to produce hemorrhagic shock. Rapid hemostasis is essential for survival (22).
At US, a hemorrhagic adenoma may appear as a hypoechoic mass with internal hyperechoic areas or as a hyperechoic mass associated with fluid with internal echoes in the subcapsular region or peritoneal cavity (Figs 3, 4). Color Doppler US may demonstrate large vessels in the periphery of the tumor. At nonenhanced CT, the diagnosis is suggested if hyperattenuating areas are noted within a hepatic tumor with an adjacent subcapsular hematoma or hemoperitoneum in a woman taking oral contraceptives (Figs 3, 4). The MR imaging appearance of hepatocellular adenomas can be variable and nonspecific. They are often hyperintense on T1- and T2-weighted images, but most have a heterogeneous appearance. The high signal intensity seen on T1-weighted images correlates with the presence of fat or hemorrhage (23). Early arterial enhancement is seen at dynamic gadolinium-enhanced MR imaging in most cases. The enhancement pattern may be similar to that of FNH, except for the central stellate scar seen in FNH. Occasionally, the only finding at US, CT, or MR imaging is a subcapsular hematoma secondary to a bleeding small adenoma in the periphery of the liver that is not detected at imaging (Fig 4).
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Figure 3a. Hemorrhagic and nonhemorrhagic hepatocellular adenomas in a 34-year-old woman with RUQ pain. (a) Transverse US scan shows a well-circumscribed, complex mass with cystic and solid elements in the periphery of the right hepatic lobe (arrow). (b) Nonenhanced CT scan shows that the mass has central areas of high attenuation, which are suggestive of acute bleeding. There is also a well-defined, low-attenuation mass in the left hepatic lobe (arrows). (c) Contrast-enhanced CT scan shows peripheral enhancement of the mass in the right lobe and heterogeneous enhancement of the mass in the left lobe. (d) T1-weighted MR image shows that the mass in the right lobe has a central, concentric rim of high signal intensity surrounding an area of low signal intensity (blood). Note some areas of low signal intensity in the left lobe, which correspond to the mass seen at contrast-enhanced CT (c). (Courtesy of Didier Mathieu, MD, Centre Hospitalier Universitaire Henri Mondor, Créteil, France.)
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Figure 3b. Hemorrhagic and nonhemorrhagic hepatocellular adenomas in a 34-year-old woman with RUQ pain. (a) Transverse US scan shows a well-circumscribed, complex mass with cystic and solid elements in the periphery of the right hepatic lobe (arrow). (b) Nonenhanced CT scan shows that the mass has central areas of high attenuation, which are suggestive of acute bleeding. There is also a well-defined, low-attenuation mass in the left hepatic lobe (arrows). (c) Contrast-enhanced CT scan shows peripheral enhancement of the mass in the right lobe and heterogeneous enhancement of the mass in the left lobe. (d) T1-weighted MR image shows that the mass in the right lobe has a central, concentric rim of high signal intensity surrounding an area of low signal intensity (blood). Note some areas of low signal intensity in the left lobe, which correspond to the mass seen at contrast-enhanced CT (c). (Courtesy of Didier Mathieu, MD, Centre Hospitalier Universitaire Henri Mondor, Créteil, France.)
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Figure 3c. Hemorrhagic and nonhemorrhagic hepatocellular adenomas in a 34-year-old woman with RUQ pain. (a) Transverse US scan shows a well-circumscribed, complex mass with cystic and solid elements in the periphery of the right hepatic lobe (arrow). (b) Nonenhanced CT scan shows that the mass has central areas of high attenuation, which are suggestive of acute bleeding. There is also a well-defined, low-attenuation mass in the left hepatic lobe (arrows). (c) Contrast-enhanced CT scan shows peripheral enhancement of the mass in the right lobe and heterogeneous enhancement of the mass in the left lobe. (d) T1-weighted MR image shows that the mass in the right lobe has a central, concentric rim of high signal intensity surrounding an area of low signal intensity (blood). Note some areas of low signal intensity in the left lobe, which correspond to the mass seen at contrast-enhanced CT (c). (Courtesy of Didier Mathieu, MD, Centre Hospitalier Universitaire Henri Mondor, Créteil, France.)
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Figure 3d. Hemorrhagic and nonhemorrhagic hepatocellular adenomas in a 34-year-old woman with RUQ pain. (a) Transverse US scan shows a well-circumscribed, complex mass with cystic and solid elements in the periphery of the right hepatic lobe (arrow). (b) Nonenhanced CT scan shows that the mass has central areas of high attenuation, which are suggestive of acute bleeding. There is also a well-defined, low-attenuation mass in the left hepatic lobe (arrows). (c) Contrast-enhanced CT scan shows peripheral enhancement of the mass in the right lobe and heterogeneous enhancement of the mass in the left lobe. (d) T1-weighted MR image shows that the mass in the right lobe has a central, concentric rim of high signal intensity surrounding an area of low signal intensity (blood). Note some areas of low signal intensity in the left lobe, which correspond to the mass seen at contrast-enhanced CT (c). (Courtesy of Didier Mathieu, MD, Centre Hospitalier Universitaire Henri Mondor, Créteil, France.)
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Figure 4a. Bleeding hepatocellular adenoma in a 42-year-old woman with intense RUQ pain and a 15-year history of oral contraceptive use. (a) Transverse US scan shows a subcapsular fluid collection with internal echoes in the right hepatic lobe (arrows). (b) Nonenhanced CT scan shows a large subcapsular liver hematoma (arrow). At surgery, the presence of a subcapsular hematoma was confirmed, and a small bleeding adenoma not evident at imaging was resected from the periphery of the right hepatic lobe.
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Figure 4b. Bleeding hepatocellular adenoma in a 42-year-old woman with intense RUQ pain and a 15-year history of oral contraceptive use. (a) Transverse US scan shows a subcapsular fluid collection with internal echoes in the right hepatic lobe (arrows). (b) Nonenhanced CT scan shows a large subcapsular liver hematoma (arrow). At surgery, the presence of a subcapsular hematoma was confirmed, and a small bleeding adenoma not evident at imaging was resected from the periphery of the right hepatic lobe.
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The treatment of choice in this disease is resection of the tumor with a margin of normal liver parenchyma. In cases in which this treatment is not practical, resectional débridement (ie, removal of the ruptured adenoma along with the surrounding devitalized liver parenchyma) has proved to be an effective alternative (24). Adenomas can regress after discontinuation of oral contraceptives. In women with multiple adenomas with nonbleeding lesions no larger than 4 cm in diameter, a conservative approach with careful clinical and imaging follow-up has been advocated by some authors (25).
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Focal Nodular Hyperplasia
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FNH is the second most common benign liver tumor after hemangioma and is reported to occur in approximately 3% of the population. The natural history of FNH remains unknown. It manifests most commonly in the 3rd and 4th decades of life and has a clear female predominance (2:1 in most reports). Oral contraceptives may promote the growth of FNH (26). The age at presentation ranges from 3 weeks to 74 years. Most lesions are discovered incidentally with few clinical clues. Only 20% of patients report signs and symptoms secondary to the liver mass. FNH does not have an aggressive or malignant course. Unlike hepatic adenoma, which is associated with spontaneous rupture, FNH is rarely reported to bleed. There are few reports of intraperitoneal hemorrhage associated with FNH in the English-language literature (27–29).
At gross analysis, most FNH lesions are firm, nodular masses often located near the liver surface that are well circumscribed but nonencapsulated. A central stellate scar that contains numerous blood vessels is also characteristic. At microscopic analysis, FNH is nodular and composed of multiple spherical aggregates of hepatocytes surrounded by fibrous radiating septa that contain a variable number of small bile ductules. Proliferating hepatocytes are separated by sinusoids that contain both endothelial cells and Kupffer cells (26). Large arteries and veins are present at the periphery and in the central scar. Intraperitoneal hemorrhage may occur from erosion of these prominent vessels (29).
Technetium-99m sulfur colloid scanning may be helpful in diagnosis of FNH because the colloid is taken up by the Kupffer cells in 80% of cases. For the same reason, FNH lesions may show decreased signal intensity on T2-weighted MR images obtained after intravenous administration of superparamagnetic iron oxide particles, thus helping characterize the lesions (26). At nonenhanced MR imaging, the lesion is iso- or hypointense relative to normal liver tissue on T1-weighted images and iso- or mildly hyperintense on T2-weighted images. The central scar is usually hypointense on T1-weighted images and hyperintense on T2-weighted images. The enhancement pattern after administration of gadopentetate dimeglumine parallels that at contrast-enhanced CT (22). At nonenhanced CT, FNH usually appears as a homogeneous, iso- or slightly hypoattenuating mass, with a hypoattenuating central scar in 30% of cases. Because the vascular supply to FNH is predominantly arterial, the lesion shows marked enhancement in the first 20–30 seconds after intravenous injection of a bolus of contrast material, during the so-called arterial phase of dynamic CT. In the portal venous phase, about 70–90 seconds after injection, FNH may become isoattenuating to normal liver tissue with a hypoattenuating central scar. The central scar may demonstrate delayed enhancement on subsequent images due to delayed washout of the contrast material (26). The classic angiographic "spoke wheel" appearance is frequently not present but may be helpful if seen. Hemorrhagic FNH is rare and may manifest as isolated subcapsular or intraparenchymal hemorrhage (Fig 5).
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Figure 5a. Hemorrhagic FNH in a 43-year-old woman who had been taking oral contraceptives for 15 years and presented with a 3-hour history of dull, nonradiating RUQ pain. Laboratory studies showed a hemoglobin level of 9.5 g/dL (95 g/L) and a hematocrit of 27% (0.27). (a) Transverse US scan of the liver shows a large subcapsular fluid collection (arrow). (b) Contrast-enhanced CT scan shows a heterogeneous subcapsular fluid collection with hyperattenuating components (arrow), findings consistent with fresh bleeding around the right hepatic lobe. The patient was taken to surgery with the tentative diagnosis of ruptured hepatic adenoma, and a trisegmentectomy was performed. (c) Photomicrograph shows an organized blood clot (arrow) and a mass that has undergone hemorrhagic necrosis in the center, thus obscuring the histomorphology of the lesion and probably explaining the absence of a central scar. The expansile lesion lacks a capsule and compresses the adjacent hepatic parenchyma, which exhibits a pattern of sinusoidal congestion. Sections stained with reticulin to highlight tissue architecture (not shown) showed that the infarcted lesion contained portal vein tract structures including proliferating bile ducts, features not seen in hepatic adenoma or HCC but typical of FNH. The diagnosis of hemorrhagic, infarcted FNH was established.
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Figure 5b. Hemorrhagic FNH in a 43-year-old woman who had been taking oral contraceptives for 15 years and presented with a 3-hour history of dull, nonradiating RUQ pain. Laboratory studies showed a hemoglobin level of 9.5 g/dL (95 g/L) and a hematocrit of 27% (0.27). (a) Transverse US scan of the liver shows a large subcapsular fluid collection (arrow). (b) Contrast-enhanced CT scan shows a heterogeneous subcapsular fluid collection with hyperattenuating components (arrow), findings consistent with fresh bleeding around the right hepatic lobe. The patient was taken to surgery with the tentative diagnosis of ruptured hepatic adenoma, and a trisegmentectomy was performed. (c) Photomicrograph shows an organized blood clot (arrow) and a mass that has undergone hemorrhagic necrosis in the center, thus obscuring the histomorphology of the lesion and probably explaining the absence of a central scar. The expansile lesion lacks a capsule and compresses the adjacent hepatic parenchyma, which exhibits a pattern of sinusoidal congestion. Sections stained with reticulin to highlight tissue architecture (not shown) showed that the infarcted lesion contained portal vein tract structures including proliferating bile ducts, features not seen in hepatic adenoma or HCC but typical of FNH. The diagnosis of hemorrhagic, infarcted FNH was established.
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Figure 5c. Hemorrhagic FNH in a 43-year-old woman who had been taking oral contraceptives for 15 years and presented with a 3-hour history of dull, nonradiating RUQ pain. Laboratory studies showed a hemoglobin level of 9.5 g/dL (95 g/L) and a hematocrit of 27% (0.27). (a) Transverse US scan of the liver shows a large subcapsular fluid collection (arrow). (b) Contrast-enhanced CT scan shows a heterogeneous subcapsular fluid collection with hyperattenuating components (arrow), findings consistent with fresh bleeding around the right hepatic lobe. The patient was taken to surgery with the tentative diagnosis of ruptured hepatic adenoma, and a trisegmentectomy was performed. (c) Photomicrograph shows an organized blood clot (arrow) and a mass that has undergone hemorrhagic necrosis in the center, thus obscuring the histomorphology of the lesion and probably explaining the absence of a central scar. The expansile lesion lacks a capsule and compresses the adjacent hepatic parenchyma, which exhibits a pattern of sinusoidal congestion. Sections stained with reticulin to highlight tissue architecture (not shown) showed that the infarcted lesion contained portal vein tract structures including proliferating bile ducts, features not seen in hepatic adenoma or HCC but typical of FNH. The diagnosis of hemorrhagic, infarcted FNH was established.
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A specific diagnosis is usually not achievable preoperatively. Most authors have advocated a nonsurgical approach to asymptomatic FNH if the diagnosis is known with reasonable accuracy. Although much less common than in hepatocellular adenoma, rupture may occur in patients with FNH, and this diagnosis should be considered if sudden abdominal pain develops in a young or middle-aged woman taking oral contraceptives (27).
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Hepatic Hemangioma
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Hepatic hemangiomas are the most common tumors of the liver and are found in about 20% of the population in autopsy series. They exhibit a female predilection (female-male ratio of 5:1). The vast majority are small (<3 cm in diameter) and asymptomatic. Hepatic hemangiomas larger than 4 cm in diameter are classified as giant hemangiomas, which are more likely to produce symptoms and show unusual characteristics (22). However, there have been only sporadically reported cases of these lesions leading to severe pain and even spontaneous fatal hemorrhage (30–33). Estrogen may cause growth of hepatic hemangiomas, but there is a paucity of information concerning the effect of pregnancy with its increased estrogen levels on these lesions (33). At histologic analysis, hemangiomas consist of interconnected endothelium-lined vascular channels enclosed within a loose fibroblastic stroma. At analysis of cut sections, areas of fibrosis, hemorrhagic calcification, or thrombosis are frequently seen, especially in larger hemangiomas (22).
In a patient with a known hemangioma, the diagnosis of hemorrhagic hemangioma is suggested if the lesion is enlarged and shows increased echogenicity at US, increased attenuation at CT (Fig 6), or areas of high signal intensity at T1-weighted MR imaging (32,33). These findings may be associated with subcapsular hematoma or hemoperitoneum.
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Figure 6a. Hemorrhagic hemangioma in a 31-year-old man with a cavernous hemangioma of the liver diagnosed 1 year earlier. The patient presented with acute RUQ pain. (a) Nonenhanced CT scan obtained 1 year earlier shows a large mass in the central portion of the liver (arrow). The diagnosis of cavernous hemangioma was made with dynamic contrast-enhanced CT (not shown). (b) Nonenhanced CT scan obtained at the present admission shows no change in the size or configuration of the tumor; however, a large area of increased attenuation is seen within the lesion (arrow), a finding suggestive of intratumoral bleeding. The patient was treated nonsurgically.
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Figure 6b. Hemorrhagic hemangioma in a 31-year-old man with a cavernous hemangioma of the liver diagnosed 1 year earlier. The patient presented with acute RUQ pain. (a) Nonenhanced CT scan obtained 1 year earlier shows a large mass in the central portion of the liver (arrow). The diagnosis of cavernous hemangioma was made with dynamic contrast-enhanced CT (not shown). (b) Nonenhanced CT scan obtained at the present admission shows no change in the size or configuration of the tumor; however, a large area of increased attenuation is seen within the lesion (arrow), a finding suggestive of intratumoral bleeding. The patient was treated nonsurgically.
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Hepatic artery ligation has been suggested as a means of treating hemoperitoneum due to a ruptured hemangioma. However, the procedure of choice is angiographic embolization of the hepatic artery (33–35).
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Hepatic Metastases
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Hemoperitoneum secondary to spontaneous rupture of hepatic metastases from primary tumors of the lung (36), pancreas (37), stomach, kidney, breast, prostate, testicle (38,39), gallbladder (40), skin (melanoma) (41), and nasopharynx (42) and from choriocarcinoma (43) and hepatic lymphoma (14) has been reported. Hepatic metastases from lung carcinoma, renal carcinoma, and melanoma are the most frequent types to cause hepatic bleeding. Clinical findings vary from unexplained blood loss and shock to signs consistent with acute abdomen. Because hemorrhagic hepatic metastases are so uncommon, the diagnosis is rarely made preoperatively, especially in a previously healthy patient. When this condition is associated with acute abdomen, it is most often misdiagnosed as perforated gastric or duodenal ulcer (37). Hemoperitoneum is more frequently seen in association with primary liver tumors, which have greater vascularity than metastatic lesions. The latter tend to be more fibrotic with a poorer blood supply. Fever and leukocytosis, perhaps secondary to tumor necrosis, may be part of the clinical presentation (38).
At imaging, the diagnosis of hemorrhagic metastasis is suggested if blood is identified in one or more liver lesions in a patient with known hepatic metastases or a known primary tumor elsewhere (Fig 7). If the hemorrhage is severe, a subcapsular hematoma or hemoperitoneum may also be noted.
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Figure 7a. Hemorrhagic metastases from malignant melanoma in a 25-year-old woman with a palpable mass and RUQ pain. (a) Nonenhanced CT scan shows a large mass with areas of high attenuation involving the right and left hepatic lobes. An additional smaller lesion of high attenuation compresses the inferior vena cava (arrow). (b) T1-weighted MR image (repetition time msec/echo time msec = 728/16) shows a well-circumscribed mass with areas of high and low signal intensity. An ill-defined lesion is present in the posterior aspect of the right hepatic lobe (arrow). (c) T2-weighted MR image (6,783/160) shows a persistent area of high signal intensity in the posterior aspect of the mass. In addition, a mass of intermediate signal intensity is identified in the left hepatic lobe (arrow). At surgery, multiple lesions were found in the liver. The dominant lesion in the right lobe had multiple areas of hemorrhage. However, melanin could also produce high signal intensity on T1-weighted images due to its paramagnetic characteristics. The histopathologic diagnosis was hemorrhagic metastatic melanoma. On further evaluation, a cutaneous malignant melanoma was discovered in the patient's back.
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Figure 7b. Hemorrhagic metastases from malignant melanoma in a 25-year-old woman with a palpable mass and RUQ pain. (a) Nonenhanced CT scan shows a large mass with areas of high attenuation involving the right and left hepatic lobes. An additional smaller lesion of high attenuation compresses the inferior vena cava (arrow). (b) T1-weighted MR image (repetition time msec/echo time msec = 728/16) shows a well-circumscribed mass with areas of high and low signal intensity. An ill-defined lesion is present in the posterior aspect of the right hepatic lobe (arrow). (c) T2-weighted MR image (6,783/160) shows a persistent area of high signal intensity in the posterior aspect of the mass. In addition, a mass of intermediate signal intensity is identified in the left hepatic lobe (arrow). At surgery, multiple lesions were found in the liver. The dominant lesion in the right lobe had multiple areas of hemorrhage. However, melanin could also produce high signal intensity on T1-weighted images due to its paramagnetic characteristics. The histopathologic diagnosis was hemorrhagic metastatic melanoma. On further evaluation, a cutaneous malignant melanoma was discovered in the patient's back.
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Figure 7c. Hemorrhagic metastases from malignant melanoma in a 25-year-old woman with a palpable mass and RUQ pain. (a) Nonenhanced CT scan shows a large mass with areas of high attenuation involving the right and left hepatic lobes. An additional smaller lesion of high attenuation compresses the inferior vena cava (arrow). (b) T1-weighted MR image (repetition time msec/echo time msec = 728/16) shows a well-circumscribed mass with areas of high and low signal intensity. An ill-defined lesion is present in the posterior aspect of the right hepatic lobe (arrow). (c) T2-weighted MR image (6,783/160) shows a persistent area of high signal intensity in the posterior aspect of the mass. In addition, a mass of intermediate signal intensity is identified in the left hepatic lobe (arrow). At surgery, multiple lesions were found in the liver. The dominant lesion in the right lobe had multiple areas of hemorrhage. However, melanin could also produce high signal intensity on T1-weighted images due to its paramagnetic characteristics. The histopathologic diagnosis was hemorrhagic metastatic melanoma. On further evaluation, a cutaneous malignant melanoma was discovered in the patient's back.
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Treatment of this type of hemoperitoneum depends on the size of the hepatic tumor, its location, and the rate of bleeding, with control of the hemorrhage being the major objective. Regardless of the treatment, the prognosis is very poor, with virtually all patients living less than 6 months and most less than 6 weeks (39).
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HELLP Syndrome
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HELLP syndrome was described by Weinstein (44) in 1982. A small subset of preeclamptic or eclamptic women presented with findings of hemolysis, elevated liver enzymes, and low platelet count. This constellation of findings is known as the HELLP syndrome. This serious obstetric condition, which occurs either before or immediately after birth, may progress rapidly with complications including disseminated intravascular coagulation, hepatic necrosis, and hemorrhagic infarction (44–49).
At histologic analysis, the portal areas of the liver are surrounded by a combination of deposited fibrin, hemorrhage, and hepatocellular necrosis (50–52). Fibrin deposition in sinusoids, portal vein tracts, and hepatic arteries may be secondary to disseminated intravascular coagulation. Another hypothesis suggests that segmental vasospasm induces endothelial cell damage, thus allowing fibrin precipitation. Hypoperfusion secondary to episodes of shock may also contribute to hepatic necrosis and infarction. Nonetheless, hepatic infarction is a much less common result than periportal necrosis or hemorrhage; this difference is presumably due to the dual blood supply of the liver. Although hepatic infarction suggests severe liver involvement, intraparenchymal hemorrhage, which may become subcapsular or even rupture into the peritoneal cavity, is a more serious, life-threatening complication (45,52).
Early in the clinical course of HELLP syndrome, US is frequently used to exclude gallbladder disease as a cause of RUQ pain. Hepatic hematomas (subcapsular and intrahepatic) can be detected with US (45,53). CT is the study of choice for HELLP syndrome because it can demonstrate subcapsular hematoma and hepatic rupture with intraperitoneal bleeding (Fig 8). Hepatic infarction can be seen at CT as low-attenuation wedge-shaped areas in the periphery of the liver without mass effect (Fig 9) (53–55).
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Figure 8. Acute subcapsular hematoma associated with HELLP syndrome in a 38-year-old woman who was 38 weeks pregnant and presented with RUQ pain, hypertension, 4+ proteinuria, and low platelet count. Nonenhanced CT scan shows a large, mostly hyperattenuating, acute subcapsular hematoma (arrow). A diagnosis of HELLP syndrome was established, and a cesarean section was performed. The patient and the neonate did well after this procedure.
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Figure 9. Hepatic infarction associated with HELLP syndrome in a 28-year-old woman with a history of eclampsia and associated HELLP syndrome. Nonenhanced CT scan shows a large, wedge-shaped area of low attenuation in the right hepatic lobe (arrow), a finding suggestive of a hepatic infarction. Note the presence of ascites around the liver.
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In the majority of patients with HELLP syndrome, the management is supportive. Delivery accomplished by whatever route is deemed safest and most expeditious is the cornerstone of therapy. Patients who experience hepatic rupture with intraperitoneal bleeding require immediate surgery or selective embolization of the hepatic arteries (47).
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Amyloidosis
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Amyloidosis may be defined as the extracellular deposition of the fibrous protein amyloid in one or more sites in the body. Amyloid is amorphous, eosinophilic, hyaline, extracellular, and ubiquitous. The involved organs may have a rubbery, firm consistency and a waxy, pink or gray appearance. Organ enlargement, especially of the liver, kidney, spleen, and heart, may be prominent. The liver is frequently involved in amyloidosis; however, hepatocellular rupture is extremely rare. Few cases of spontaneous rupture of the liver associated with hepatic amyloidosis have been reported (56–60).
The pathophysiology of spontaneous hepatic rupture in amyloidosis has been thought to be due to liver enlargement, rigidity of the hepatic parenchyma, and vascular fragility from amyloid involvement (57). Hemoperitoneum or a subcapsular hematoma may be diagnosed with US or CT (Fig 10). With so few cases of hepatic rupture due to amyloid, experience with its management is limited. Death within hours to days has occurred in the majority of reported cases despite a range of surgical approaches (57,59,60).
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Figure 10. Intrahepatic hemorrhage due to primary amyloidosis in a 56-year-old woman with acute abdominal pain and a falling hematocrit. Exploratory laparoscopy revealed a massive hemoperitoneum. The upper right lobe of the liver was found to be the source of the bleeding, and partial right hepatectomy was performed; however, the hospital course was complicated by multiorgan failure. Contrast-enhanced postoperative CT scan shows areas of low attenuation in the left hepatic lobe (arrows). At autopsy, these areas represented old hemorrhage within a liver diffusely infiltrated by amyloid.
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Miscellaneous Causes
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Miscellaneous causes of spontaneous hepatic rupture not illustrated in this article include peliosis hepatis, angiomyolipoma of the liver, nodular regenerative hyperplasia, connective tissue diseases such as polyarteritis nodosa and systemic lupus erythematosus, infectious diseases such as typhoid fever, and parasitic diseases such as malaria (4,40,61).
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Conclusions
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Spontaneous hepatic hemorrhage is a diagnostic and therapeutic challenge for the clinician and the radiologist. Fortunately, this condition is rare. Subcapsular or intrahepatic hematomas in patients who present with acute onset of RUQ pain may be associated with an underlying neoplasm or associated syndrome. Cirrhotic patients may have a ruptured HCC; a young woman taking oral contraceptives may have an occult adenoma that has bled, and preeclamptic or eclamptic patients may have HELLP syndrome. Rarely, patients with FNH, large cavernous hemangiomas, hepatic metastases, amyloidosis, or miscellaneous conditions such as angiomyolipoma, polyarteritis nodosa, and systemic lupus erythematosus may experience spontaneous hepatic hemorrhage. Modern imaging techniques, especially abdominal CT, are instrumental in diagnosis and management of these catastrophic diseases.
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Footnotes
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Abbreviations: FNH = focal nodular hyperplasia
HCC = hepatocellular carcinoma
RUQ = right upper quadrant
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References
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Abstract
Introduction
