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Copyrights © 2013 The Korean Society of Radiology
INTRODUCTION
Hepatic rupture associated with hemolysis, elevated liver en- zyme, and low platelet count (HELLP) syndrome is a cata- strophic complication of pregnancy (1). The incidence of hepat- ic 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, as- sociated with HELLP syndrome, have been described in the lit- erature (3, 4). We recently obtained successful clinical outcome after embolization of the hepatic artery and right inferior phren- ic 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 angiogra- phy, 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 ac- tive bleeding.
CASE REPORT
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 ane- mia (Hg 10.7 g%), mild leukocytosis with neutrophilia (11900/uL, 85.3%), low platelet count (75000/mm³), and elevated liver en- zyme (aspartate transaminase 377 IU/L, and alanine transami- nase 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/
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
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: [email protected]
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, distri- bution, and reproduction in any medium, provided the original work is properly cited.
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 find- ings 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
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
Hepatic Rupture Caused by HELLP Syndrome
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After CT examination, the patient showed low blood pressure (95/75 mm Hg) and elevated heart rate (140/min). Emboliza- tion of the hepatic artery was requested. Conventional hepatic artery angiography showed contrast extravasations from poste- rior 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 Torna- do 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 re- vealed no evidence of contrast extravasation. After embolization, induction of labor was performed using misoprostol (Cytotec®, Pfizer, NY, USA). After embolization, general conditions of the 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 sub- capsular hematoma around an inferior portion of the right he- patic lobe. Color doppler US revealed no fetal heart beat, sug- gesting 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 ir- regular interface between the necrotic hepatic parenchyma and subcapsular hematoma, presumably representing hepatic rup- ture and multiple active contrast extravasations from the right hepatic lobe, especially near the bare area.
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 rep- resenting 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 ne- crotic 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 D
A
E
B C
Hepatic Rupture Caused by HELLP Syndrome Cheong Bok Lee, et al
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pregnant patients with HELLP syndrome before delivery of a live fetus. Patients with this condition usually detour the radiol- ogy section because the obstetrician often conducts bedside ul- trasonography and patients go to the operating room for emer- 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- sulting in hepatic rupture. In a review from the literature, our pa- tient 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. Further- more, 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 op- tion to control hepatic rupture. This method can avoid explor- ative laparotomy and provide better bleeding control with supe- rior maternal survival.
In angiographic intervention, authors examined right inferior phrenic angiography. Right inferior phrenic angiography showed contrast extravasations. The right inferior phrenic artery pro- vides most common sources of extrahepatic blood supply (7, 8).
Right inferior phrenic artery communicates with the intrahe- patic 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 phren- ic angiography and detected another active bleeding.
In summary, we obtained successful clinical outcome after em- bolization 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.
REFERENCES
1. Henny CP, Lim AE, Brummelkamp WH, Buller HR, Ten Cate JW. A review of the importance of acute multidisciplinary 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).
DISCUSSION
Hepatic rupture is the most catastrophic complication of preg- nancy (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 ma- ternal and perinatal morbidity and mortality (6). Although the pathogenesis of this condition remains unclear, histopathologi- cally, vascular microthrombi and intravascular fibrin deposit may lead to intrahepatic sinusoidal obstruction and vascular congestion, which can make hepatic necrosis resulting in paren- chymal and subcapsular hemorrhage, and eventually capsular rupture and hemoperitoneum (1, 5).
Imaging features of HELLP syndrome with hepatic hemor- rhage is documented in some literature. A previous study (3) re- ported that the most frequent abnormal imaging findings of HELLP syndrome were subcapsular hematoma (n = 13), intra- parenchymal 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 nonen- hancing low attenuation, peripheral lesions with vessels cours- ing through and mottled appearance as a characteristic of hepat- ic infarction in patents with HELLP syndrome. In our case, contrast extravasation on CT angiogram is considered as anoth- er 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 con- traindication of iodinated contrast agent in pregnancy, emboli- zation is not recommended as a treatment of hepatic rupture in
Hepatic Rupture Caused by HELLP Syndrome
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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
