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 헬프 증후군에 의한 간파열: CT와 고식적 혈관조영술 영상소견 1예 보고 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:
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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/mm³), 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 μg/
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
A
B
C
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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J Korean Soc Radiol 2013;68(5):407-410
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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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헬프 증후군에 의한 간파열: CT와 고식적 혈관조영술 영상소견 1예 보고 이청복 · 안재홍 · 최수정 · 이종혁 · 박만수 · 정승문 · 류대식 최근에 저자들은 간파열이 동반된 헬프 증후군 임신 환자의 간동맥과 우하횡경맥동맥을 색전하여 성공적인 임상 결과를 얻었다. 저자들은 간경색, 간파열, 활동성 출혈을 보인 헬프 증후군 환자에 있어서 CT와 고식적인 혈관조영술 소견을 보 고하고자 한다. 울산대학교 의과대학 강릉아산병원 영상의학과
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