Hepatic rupture associated with hemolysis, elevated liver enzyme, and low platelet count (HELLP) syndrome is a catastrophic complication of pregnancy (1). The incidence of hepatic rupture in pregnancy ranges between one in 45000 and one in 225000. Maternal mortality in patients with hepatic rupture is reported to be as high as 60 to 86%. Fetal mortality can reach up to 60 to 86% (2). Imaging manifestations of hepatic rupture, associated with HELLP syndrome, have been described in the literature (3, 4). We recently obtained successful clinical outcome after embolization of the hepatic artery and right inferior phrenic artery as the first treatment in a pregnant patient with HELLP syndrome causing hepatic rupture and hemoperitoneum. Until now, contrast extravasation on CT and conventional angiography, in case with HELLP syndrome causing hepatic rupture and hemoperitoneum, has not been reported in the literature. We report the CT and conventional angiographic findings in the case of HELLP syndrome resulting in hepatic rupture with active bleeding.

A 28-year-old woman at 28 weeks of gestation presented with fever and right upper quadrant pain for one day. She had high blood pressure (171/102 mm Hg) and significant proteinuria (4+) indicating severe pre-eclampsia. Laboratory findings showed anemia (Hg 10.7 g%), mild leukocytosis with neutrophilia (11900/uL, 85.3%), low platelet count (75000/mm³), and elevated liver enzyme (aspartate transaminase 377 IU/L, and alanine transaminase 369 IU/L). Initial laboratory findings indicated HELLP syndrome. Prothrombin time (international normalized ratio: 0.95) and activated prothrombin time (26.3 second) showed normal range. Fibrinogen degradation product level (147 µg/mL) was elevated and D-dimer was positive.

Ultrasonography (US; iU-22, Philips, Bothell, WA, USA) was requested to evaluate the reason for right upper quadrant pain and elevated liver enzyme. US revealed a large amount of subcapsular hematoma around an inferior portion of the right hepatic lobe. Color doppler US revealed no fetal heart beat, suggesting intrauterine fetal death.

CT angiography (LightSpeed VCT, GE, Milwaukee, WI, USA) was performed after reconfirmation of intrauterine fetal death by obstetrician. Contrast-enhanced CT (Fig. 1A, B) revealed irregular interface between the necrotic hepatic parenchyma and subcapsular hematoma, presumably representing hepatic rupture and multiple active contrast extravasations from the right hepatic lobe, especially near the bare area.

After CT examination, the patient showed low blood pressure (95/75 mm Hg) and elevated heart rate (140/min). Embolization of the hepatic artery was requested. Conventional hepatic artery angiography showed contrast extravasations from posterior segmental branch of the right hepatic artery (Fig. 1C). Right inferior phrenic artery angiography also showed focal contrast extravasations (Fig. 1D). The selective coil (3 mm × 2 cm Tornado coil, Cook, Bloomington, IN, USA) and gelatin sponge sheet (Spongostan, Johnson & Johnson, Skipton, UK) embolization of the posterior segmental hepatic artery and right inferior phrenic artery was performed. The post-embolization angiography revealed no evidence of contrast extravasation. After embolization, induction of labor was performed using misoprostol (Cytotec®, Pfizer, NY, USA). After embolization, general conditions of the patient improved gradually and the level of hepatic enzymes and coagulation profile were normalized. Follow-up contrast-enhanced CT two months after embolization showed large post-hemorrhagic pseudocyst formation in necrotic right lobe of the liver and perihepatic space (Fig. 1E).

Hepatic rupture is the most catastrophic complication of pregnancy (1). This rare condition is usually associated with HELLP syndrome, which was first defined by Weinstein (5) in 1982. A rare complication of this syndrome is hepatic hemorrhage that may result in hepatic rupture, significantly increasing both maternal and perinatal morbidity and mortality (6). Although the pathogenesis of this condition remains unclear, histopathologically, vascular microthrombi and intravascular fibrin deposit may lead to intrahepatic sinusoidal obstruction and vascular congestion, which can make hepatic necrosis resulting in parenchymal and subcapsular hemorrhage, and eventually capsular rupture and hemoperitoneum (1, 5).

Imaging features of HELLP syndrome with hepatic hemorrhage is documented in some literature. A previous study (3) reported that the most frequent abnormal imaging findings of HELLP syndrome were subcapsular hematoma (n = 13), intraparenchymal hematoma (n = 6), and rupture (n = 4) in their study with 34 cases. They documented that the hepatic rupture was most frequently involved in the right hepatic lobe. Henny et al. (1) documented that hematomas were present in the right lobe in 75% of cases, in the left lobe in 11%, and in both lobes in 14%. Zissin et al. (4) described a CT features of multiple nonenhancing low attenuation, peripheral lesions with vessels coursing through and mottled appearance as a characteristic of hepatic infarction in patents with HELLP syndrome. In our case, contrast extravasation on CT angiogram is considered as another imaging finding that suggests active bleeding and requires prompt intervention.

The treatment of hepatic rupture with HELLP syndrome is emergent delivery and bleeding control, including exploratory laparotomy or intervention, such as hepatic artery embolization. Because of radiohazard of angiographic intervention, and contraindication of iodinated contrast agent in pregnancy, embolization is not recommended as a treatment of hepatic rupture in pregnant patients with HELLP syndrome before delivery of a live fetus. Patients with this condition usually detour the radiology section because the obstetrician often conducts bedside ultrasonography and patients go to the operating room for emergent delivery and exploratory laparotomy. Therefore, hepatic artery embolization was requested mainly as a post-op bleeding control after laparotomy in patients with HELLP syndrome resulting in hepatic rupture. In a review from the literature, our patient is a rare case who received only hepatic artery embolization as a first treatment for bleeding control due to hepatic rupture with HELLP syndrome before delivering a dead fetus. Furthermore, our patient is the first reported radiologic case, showing a contrast extravasation on CT and conventional angiography.

Rinehart et al. (2) found that maternal survival rate of hepatic rupture in HELLP syndrome was highest in a group treated with embolization. Hepatic artery embolization is a better option to control hepatic rupture. This method can avoid explorative laparotomy and provide better bleeding control with superior maternal survival.

In angiographic intervention, authors examined right inferior phrenic angiography. Right inferior phrenic angiography showed contrast extravasations. The right inferior phrenic artery provides most common sources of extrahepatic blood supply (7, 8). Right inferior phrenic artery communicates with the intrahepatic arteries typically in the caudate lobe and posterior segment (8). In this presenting case, hepatic artery angiography showed contrast extravasations, especially from the posterior segment near the bare area. Therefore, we examined right inferior phrenic angiography and detected another active bleeding.

In summary, we obtained successful clinical outcome after embolization in the hepatic artery and right inferior phrenic artery in a pregnant patient with HELLP syndrome causing hepatic rupture. Hepatic artery embolization can be a better option to control hepatic rupture and the right inferior phrenic artery should be evaluated in a patient with HELLP syndrome, causing hepatic rupture and active bleeding from the posterior segment near the bare area.