Case Report

pISSN 1738-2637
J Korean Soc Radiol 2013;68(5):407-410
http://dx.doi.org/10.3348/jksr.2013.68.5.407

Hepatic Rupture Caused by Hemolysis, Elevated Liver Enzyme, and

Low Platelet Count Syndrome: A Case Report with Computed
Tomographic and Conventional Angiographic Findings
: CT 1
Cheong Bok Lee, MD, Jae Hong Ahn, MD, Soo-Jung Choi, MD, Jong Hyeog Lee, MD,
Man Soo Park, MD, Seung Mun Jung, MD, Dae Sik Ryu, MD
Department of Radiology, Asan Foundation, Gangneung Asan Hospital, University of Ulsan College of Medicine, Gangneung, Korea

The authors recently obtained successful clinical outcome after embolization of the
hepatic artery and right inferior phrenic artery in a pregnant patient with hemolysis,
elevated liver enzyme, and low platelet count (HELLP) syndrome causing hepatic
rupture. We report the computed tomographic and conventional angiographic findings in a case of HELLP syndrome, resulting in hepatic infarction and rupture with
active bleeding.
Index terms
Hemolysis, Elevated Liver Enzyme, and Low Platelet Count Syndrome
Hepatic Rupture
Angiography
Embolization

INTRODUCTION
Hepatic rupture associated with hemolysis, elevated liver en-

Received January 14, 2013; Accepted March 7, 2013

Corresponding author: Jae Hong Ahn, MD
Department of Radiology, Asan Foundation, Gangneung
Asan Hospital, University of Ulsan College of Medicine,
38 Bangdong-gil, Sacheon-myeon, Gangneung 210-711,
Korea.
Tel. 82-33-610-3486 Fax. 82-33-610-3490
E-mail: jhahn@gnah.co.kr
This is an Open Access article distributed under the terms
of the Creative Commons Attribution Non-Commercial
License (http://creativecommons.org/licenses/by-nc/3.0)
which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the
original work is properly cited.

report the CT and conventional angiographic findings in the

case of HELLP syndrome resulting in hepatic rupture with active bleeding.

zyme, 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

CASE REPORT

225000. Maternal mortality in patients with hepatic rupture is

A 28-year-old woman at 28 weeks of gestation presented with

reported to be as high as 60 to 86%. Fetal mortality can reach up

fever and right upper quadrant pain for one day. She had high

to 60 to 86% (2). Imaging manifestations of hepatic rupture, as-

blood pressure (171/102 mm Hg) and significant proteinuria (4+)

sociated with HELLP syndrome, have been described in the lit-

indicating severe pre-eclampsia. Laboratory findings showed ane-

erature (3, 4). We recently obtained successful clinical outcome

mia (Hg 10.7 g%), mild leukocytosis with neutrophilia (11900/uL,

after embolization of the hepatic artery and right inferior phren-

85.3%), low platelet count (75000/mm), and elevated liver en-

ic artery as the first treatment in a pregnant patient with HELLP

zyme (aspartate transaminase 377 IU/L, and alanine transami-

syndrome causing hepatic rupture and hemoperitoneum. Until

nase 369 IU/L). Initial laboratory findings indicated HELLP

now, contrast extravasation on CT and conventional angiogra-

syndrome. Prothrombin time (international normalized ratio:

phy, in case with HELLP syndrome causing hepatic rupture and

0.95) and activated prothrombin time (26.3 second) showed

hemoperitoneum, has not been reported in the literature. We

normal range. Fibrinogen degradation product level (147 g/

Copyrights 2013 The Korean Society of Radiology

407

Hepatic Rupture Caused by HELLP Syndrome

mL) was elevated and D-dimer was positive.

After CT examination, the patient showed low blood pressure

Ultrasonography (US; iU-22, Philips, Bothell, WA, USA) was

(95/75 mm Hg) and elevated heart rate (140/min). Emboliza-

requested to evaluate the reason for right upper quadrant pain

tion of the hepatic artery was requested. Conventional hepatic

and elevated liver enzyme. US revealed a large amount of sub-

artery angiography showed contrast extravasations from poste-

capsular hematoma around an inferior portion of the right he-

rior segmental branch of the right hepatic artery (Fig. 1C). Right

patic lobe. Color doppler US revealed no fetal heart beat, sug-

inferior phrenic artery angiography also showed focal contrast

gesting intrauterine fetal death.

extravasations (Fig. 1D). The selective coil (3 mm 2 cm Torna-

CT angiography (LightSpeed VCT, GE, Milwaukee, WI, USA)

do coil, Cook, Bloomington, IN, USA) and gelatin sponge sheet

was performed after reconfirmation of intrauterine fetal death

(Spongostan, Johnson & Johnson, Skipton, UK) embolization of

by obstetrician. Contrast-enhanced CT (Fig. 1A, B) revealed ir-

the posterior segmental hepatic artery and right inferior phrenic

regular interface between the necrotic hepatic parenchyma and

artery was performed. The post-embolization angiography re-

subcapsular hematoma, presumably representing hepatic rup-

vealed no evidence of contrast extravasation. After embolization,

ture and multiple active contrast extravasations from the right

induction of labor was performed using misoprostol (Cytotec,

hepatic lobe, especially near the bare area.

Pfizer, NY, USA). After embolization, general conditions of the

D
E
Fig. 1. A 28-year pregnant woman with HELLP syndrome.
A. Axial contrast-enhanced CT reveals active contrast extravasation (arrow) in peripheral right hepatic lobe.
B. Axial contrast-enhanced CT reveals active contrast extravasation (long arrow) in right central hepatic lobe near to bare area. The liver surface
shows the irregular interface (short arrows) between subcapsular hematoma (asterisk) and necrotic hepatic parenchyma (clover) presumably representing hepatic rupture.
C. Conventional hepatic angiography shows multiple contrast extravasations (arrows) from posterior segmental branch of liver. The right hepatic
surface is compressed by subcapsular hematoma.
D. Right inferior phrenic angiography shows contrast extravasations (arrows).
E. Follow-up axial contrast-enhanced CT two months after embolization shows large post-hemorrhagic pseudocyst formation (asterisk) in necrotic right lobe of liver and perihepatic space. The embolization coil (arrow) in right inferior phrenic artery is visible.
Note.-HELLP = hemolysis, elevated liver enzyme, and low platelet count

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Cheong Bok Lee, et al

patient improved gradually and the level of hepatic enzymes

pregnant patients with HELLP syndrome before delivery of a

and coagulation profile were normalized. Follow-up contrast-

live fetus. Patients with this condition usually detour the radiol-

enhanced CT two months after embolization showed large post-

ogy section because the obstetrician often conducts bedside ul-

hemorrhagic pseudocyst formation in necrotic right lobe of the

trasonography and patients go to the operating room for emer-

liver and perihepatic space (Fig. 1E).

gent delivery and exploratory laparotomy. Therefore, hepatic

artery embolization was requested mainly as a post-op bleeding
control after laparotomy in patients with HELLP syndrome re-

DISCUSSION

sulting in hepatic rupture. In a review from the literature, our pa-

Hepatic rupture is the most catastrophic complication of preg-

tient is a rare case who received only hepatic artery embolization

nancy (1). This rare condition is usually associated with HELLP

as a first treatment for bleeding control due to hepatic rupture

syndrome, which was first defined by Weinstein (5) in 1982. A

with HELLP syndrome before delivering a dead fetus. Further-

rare complication of this syndrome is hepatic hemorrhage that

more, our patient is the first reported radiologic case, showing a

may result in hepatic rupture, significantly increasing both ma-

contrast extravasation on CT and conventional angiography.

ternal and perinatal morbidity and mortality (6). Although the

Rinehart et al. (2) found that maternal survival rate of hepatic

pathogenesis of this condition remains unclear, histopathologi-

rupture in HELLP syndrome was highest in a group treated

cally, vascular microthrombi and intravascular fibrin deposit

with embolization. Hepatic artery embolization is a better op-

may lead to intrahepatic sinusoidal obstruction and vascular

tion to control hepatic rupture. This method can avoid explor-

congestion, which can make hepatic necrosis resulting in paren-

ative laparotomy and provide better bleeding control with supe-

chymal and subcapsular hemorrhage, and eventually capsular

rior maternal survival.

rupture and hemoperitoneum (1, 5).

In angiographic intervention, authors examined right inferior

Imaging features of HELLP syndrome with hepatic hemor-

phrenic angiography. Right inferior phrenic angiography showed

rhage is documented in some literature. A previous study (3) re-

contrast extravasations. The right inferior phrenic artery pro-

ported that the most frequent abnormal imaging findings of

vides most common sources of extrahepatic blood supply (7, 8).

HELLP syndrome were subcapsular hematoma (n = 13), intra-

Right inferior phrenic artery communicates with the intrahe-

parenchymal hematoma (n = 6), and rupture (n = 4) in their

patic arteries typically in the caudate lobe and posterior segment

study with 34 cases. They documented that the hepatic rupture

(8). In this presenting case, hepatic artery angiography showed

was most frequently involved in the right hepatic lobe. Henny et

contrast extravasations, especially from the posterior segment

al. (1) documented that hematomas were present in the right

near the bare area. Therefore, we examined right inferior phren-

lobe in 75% of cases, in the left lobe in 11%, and in both lobes in

ic angiography and detected another active bleeding.

14%. Zissin et al. (4) described a CT features of multiple nonen-

In summary, we obtained successful clinical outcome after em-

hancing low attenuation, peripheral lesions with vessels cours-

bolization in the hepatic artery and right inferior phrenic artery

ing through and mottled appearance as a characteristic of hepat-

in a pregnant patient with HELLP syndrome causing hepatic

ic infarction in patents with HELLP syndrome. In our case,

rupture. Hepatic artery embolization can be a better option to

contrast extravasation on CT angiogram is considered as anoth-

control hepatic rupture and the right inferior phrenic artery

er imaging finding that suggests active bleeding and requires

should be evaluated in a patient with HELLP syndrome, causing

prompt intervention.

hepatic rupture and active bleeding from the posterior segment

The treatment of hepatic rupture with HELLP syndrome is

near the bare area.

emergent delivery and bleeding control, including exploratory

laparotomy or intervention, such as hepatic artery embolization.
Because of radiohazard of angiographic intervention, and con-

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