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A 43-year-old female was admitted to our intensive care liver unit because of hypovolemic shock secondary to a spontaneous liver rupture. When she was twenty years old, she developed an adrenal carcinoma requiring left adrenalectomy, nephrectomy and splenectomy. Since then, she has been treated with mitotane, alongside hydrocortisone and fludrocortisone for mitotane-related adrenal insufficiency. She also developed primary hypogonadism due to mitotane treatment and was on hormonal treatment with estradiol and norethisterone. She attended the emergency department with non-traumatic sudden-onset right upper abdominal pain. Physical examination revealed painful hepatomegaly but no fever. Blood tests showed a mild elevation in aspartate aminotransferase 254 U/L and alanine aminotransferase 149 U/L (upper limit of normal: 40 U/L) and a gamma glutamyltransferase level of 151 U/L (upper limit of normal: 40 U/L). Otherwise, blood tests were normal. She suddenly developed hemodynamic instability with severe anemia and required massive blood transfusions and fluid resuscitation. CT scan showed a liver rupture in the posterior surface of segments VI-VII with intraperitoneal contrast leakage. The liver parenchyma showed diffuse hypoattenuating lesions with subtle centripetal enhancement without fill-in and no wash-out (Fig. 1). In the operating room we observed a rupture of the liver capsule of posterior segments VI-VII with active bleeding. We performed a temporary Pringle maneuver and used abdominal packing to achieve hemostasis. To minimize rebleeding risk we performed percutaneous arterial embolization of branches for segments VI-VII. Subsequently, a segmentectomy of segments VI-VII was performed due to hepatic ischemia. The resection piece was transferred for pathological analysis.
Fig. 1Coronal view of contrast-enhanced CT scan showing a subhepatic hematoma.
Coronal view of contrast-enhanced CT scan in arterial (A) and venous phase (B) showing a subhepatic hematoma with active bleeding (arrows) suggesting liver rupture. Multiple hepatic hypoattenuating lesions with subtle centripetal enhancement are noted (stars).
The macroscopic examination of the liver resection piece showed multiple blood-filled hepatic cavities (Fig. 2A). Microscopic examination revealed blood-filled cystically dilated spaces bordered by hepatocytes communicating with sinusoidal spaces (Fig. 2B,C). Specific staining showed rupture of the reticulin fibers. No signs of malignancy were detected. The imaging and pathological findings along with a history of estrogen and steroid exposure were consistent with a diagnosis of peliosis hepatis. The patient recovered without rebleeding or liver failure and was discharged from the hospital 25 days after admission.
Fig. 2Macroscopic and microscopic examination of resected tissue.
(A) Gross examination shows irregular blood-filled hepatic cavities (B,C) H&E-stained sections showing the presence of large spaces filled with blood lined by hepatocytes, areas of sinusoidal dilation and hepatocyte necrosis (B: x20 magnification; C: x100 magnification).
Peliosis hepatis as a cause of spontaneous liver rupture
Peliosis hepatis is a rare and benign condition characterized by the presence of sinusoidal dilatation causing multiple blood-filled lacunar cavities within the liver.
It can affect other organs like the spleen or lymph nodes. It should be distinguished from bacillary peliosis hepatis which is a different lesion attributed to Bartonella species infection that affects patients with AIDS. The blood-filled cysts found in peliosis can range from less than 1 millimeter to several centimeters in diameter. The lesions are distributed randomly across the liver without zonal preference.
Peliosis is associated with many conditions including malnutrition, neoplasms such as hepatocellular carcinoma and leukemia, tuberculosis, vasculitis, organ transplantation and drug therapy. Drug-related peliosis is associated typically with hormonal therapy: anabolic steroids, estrogens, and tamoxifen. Other associated drugs are thiopurines and azathioprine. Pathogenesis remains poorly understood and is thought to be related to dysfunction of the reticuloendothelial system, breakdown of sinusoidal borders, dilatation of the central lobular vein or hepatic cell necrosis forming blood-filled cavities.
Patients with hepatic peliosis are generally asymptomatic, but it may manifest as hepatomegaly, and rare cases of portal hypertension, cholestasis and liver failure have been reported.
Liver rupture is a rare but often fatal complication of peliosis and manifests typically as abdominal pain with hemodynamic instability and anemia. On sonography, stable peliotic lesions are typically hyperechoic but may be heterogeneously hypoechoic if complicated with hemorrhage. The preferred imaging technique if liver rupture is suspected is contrast-enhanced CT scan. Peliotic lesions do not have a typical radiological pattern. Lesions are usually hypoattenuating, with early globular enhancement in the arterial phase and centrifugal progression during the portal phase without wash-out. Centripetal progression of enhancement can also be seen. In the case of spontaneous liver rupture due to peliosis, a contrast leakage in the area affected may be seen on multiphasic CT.
In the non-traumatic liver rupture scenario, differential diagnosis should also include pyogenic hepatic abscess, hemangioma, hepatocellular adenoma, and neoplastic lesions such as hypervascular metastases and primary liver cancer. On pathological analysis, the main hallmark of peliosis is an extreme sinusoidal dilatation that breaks the reticulin fibers holding hepatocytes and sinusoids. In case of rupture, treatment should be tailored but aggressive bleeding-control strategies are recommended.
Liver resections should be minimized since the natural history of hepatic peliosis is generally a regression of the lesions after drug withdrawal or resolution of the etiological factor.
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The authors received no financial support to produce this manuscript.
Conflicts of interest
The authors declare no conflicts of interest that pertain to this work. Please refer to the accompanying ICMJE disclosure forms for further details.
Authors’ contributions
All authors contributed equally to the conceptualization, data curation, formal analysis, writing and revision of the manuscript.
Supplementary data
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