American Journal of Obstetrics and Gynecology (2006) 195, 12933

www.ajog.org

Characteristics and treatment of hepatic rupture caused

by HELLP syndrome
Ana C. P. F. Araujo, MD, PhD,a,b Marcos D. Leao, MD,b Maria H. Nobrega, MD, PhD,a,b
Patricia F. M. Bezerra, MD,a,b Flavio V. M. Pereira, MD,b Edailna M. M.
Dantas, MD,b George D. Azevedo, MD, PhD,b,c Selma M. B. Jeronimo, MD, PhDb,d,*
Department of Gynecology and Obstetrics,a Health Graduating Program, Health Sciences Center,b Departments of
Morphologyc and Biochemistry,d Bioscience Center, Universidade Federal do Rio Grande do Norte, Natal, RN, Brazil
Received for publication September 9, 2005; revised November 22, 2005; accepted January 10, 2006

KEY WORDS
Hepatic rupture
HELLP (hemolysis,
elevated liver
enzymes, and low
platelet count)
syndrome
Preeclampsia
Hepatic artery ligation

Objective: The purpose of this study was to review the management of hepatic rupture caused
by HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome and to assess
maternal and perinatal outcomes of these cases.
Study design: A retrospective study of HELLP syndrome cases that were complicated by hepatic
rupture was conducted.
Results: Ten cases of hepatic rupture were identified. The median maternal age was 42.5 G 5.9
years (median G SD), and the median gestational age at delivery was 35.5 G 4.9 weeks. The most
frequent signs and symptoms of hepatic rupture were the sudden onset of abdominal pain, acute
anemia, and hypotension. Laboratory findings included low platelet count and increased hepatic
enzymes. Surgery was performed in 9 cases. One case was treated nonsurgically. The maternal
mortality rate was 10%, and the perinatal mortality rate was 80%.
Conclusion: A combination of surgical treatment with hepatic artery ligation and omental patching with supportive measures was effective in decreasing the mortality rate in hepatic rupture
caused by HELLP syndrome.
2006 Mosby, Inc. All rights reserved.

Spontaneous hepatic rupture is a rare, but lifethreatening, complication of preeclampsia that frequently is associated with HELLP (hemolysis, elevated
liver enzymes, and low platelet count) syndrome.1 During pregnancy, the incidence of spontaneous hepatic
rupture is reported to be between 1 in 45,000 and 1 in
* Reprint requests: Selma M. B. Jeronimo, Department of Biochemistry, Universidade Federal do Rio Grande do Norte, CP 1624,
Natal, RN, 59078-970, Brazil.
E-mail: smbj@cb.ufrn.br
0002-9378/$ - see front matter 2006 Mosby, Inc. All rights reserved.
doi:10.1016/j.ajog.2006.01.016

225,000 overall deliveries.1-3 The most common clinical

signs of hepatic rupture are right upper quadrant or
epigastric pain, severe right shoulder pain, nausea, vomiting, abdominal distention, and hypovolemic shock.4
The successful management of hepatic rupture has
involved a combination of surgical intervention and
aggressive supportive care. Several surgical approaches
have considerably decreased the morbidity and mortality rates that are associated with this entity,1 but there
is still not an agreement on the best approach to treat
this severe complication of pregnancy.

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Araujo et al

Table I Hepatic rupture that was associated with HELLP

syndrome: Clinical and laboratory findings at admission
Parameter

Median

SD

Systolic blood pressure (mm Hg)

Diastolic blood pressure (mm Hg)
Hematocrit (%)
Platelets (!103/mm3)
AST (U/L)
Alanine aminotransferase (U/L)
LDH (U/L)

150
100
23.5
91.7
322
294
1860

57
40
4.0
57.8
194
161
897

The maternal mortality rate is high when hepatic

rupture occurs, varying from 18% to 86% in dierent
series that have been reported. Death results largely
from complications such as disseminated intravascular
coagulation, pulmonary edema, or acute renal insuciency.5-8 The objective of this study was to review the
management of hepatic rupture caused by HELLP syndrome and to assess the maternal and perinatal outcomes through a retrospective analysis of a large case
series of patients who were treated in a single medical
center in Natal, Brazil, between 1990 and 2002.

Patients and methods

A review was performed of 10 cases of pregnancy with
spontaneous hepatic rupture caused by HELLP syndrome that was treated at the maternity hospital of the
Federal University of Rio Grande do Norte (Maternidade Escola Januario Cicco) in Natal, Brazil, between
1990 and 2002. The University Hospital is a reference
center that manages complications of pregnancies,
mostly for subjects without health insurance, that serves
a population of O1 million women. HELLP syndrome
was dened as a severe preeclampsia, with subjects having
a platelet count of !100,000 per microliter, aspartate
aminotransferase (AST) of O72 U/L, and lactate dehydrogenase (LDH) of O600 U/L.9 The characteristics of
the cases are shown as the median G SD. For the determination of the SD value for the liver enzymes, the outliers were removed because the distribution of the data
was not normal.
Cases were studied retrospectively. The Maternity
Hospital admits an average of 7587 subjects yearly, of
whom 5346 are deliveries. The incidence of HELLP
syndrome was calculated on the basis of 64,156 admissions that were registered between 1990 and 2002.
Charts of 635 of patients with preeclampsia who were
admitted over a 6-month period were reviewed. The
incidence of HELLP syndrome was calculated for this
reference center, with a condence interval of 95%.
Medical records were reviewed for demographic
information, clinical symptoms, laboratory and imaging
ndings, treatment approaches, and maternal and neonatal outcomes. This study was reviewed and approved

by the Universidade Federal do Rio Grande do Norte

Ethical Review Board (CEP-08/01) and the National
Brazilian Ethical Review Board (Comissao Nacional de
Etica em Pesquisa [CONEP] 4586).

Results
Ten cases of hepatic rupture that was due to HELLP
syndrome were treated at the Maternal University
hospital. An average of 1 case of hepatic rupture in
5346 deliveries was observed during the 12 years that
were studied. The incidence of HELLP syndrome and
hepatic rupture were, respectively, 0.6% and 0.015% for
all deliveries, whereas the incidence of complete HELLP
syndrome in subjects with preeclampsia was 3.1%. General characteristics of the hepatic rupture cases were
a median age of 42.5 G 5.9 years, median parity of
4.5 G 5.5, and a median gestational age at delivery of
35.5 G 4.8 weeks.
The major symptoms at admission were vaginal
bleeding (8/10 cases) and abdominal pain (7/10 cases).
The median laboratory values at diagnosis were a low
hematocrit level (23.5% G 4.7%), a low platelet count
(91 ! 103/mm3, with SD 57,819), and an elevated serum
AST level (322 G 194 U/L), alanine aminotransferase
level (ALT) (294 G 161 U/L), and LDH level (1860 G
897 U/L). The median systolic blood pressure was
150 G 57 mm Hg, and the median diastolic blood pressure was 100 G 40 mm Hg. Table I shows the most common clinical and laboratory ndings that were observed
at admission.
Six subjects underwent cesarean deliveries, and 4
subjects had vaginal deliveries. The cesarean delivery
was performed because of vaginal bleeding, sudden
abdominal pain, or acute fetal distress. The diagnosis
of hepatic rupture was made during the cesarean procedure for 4 subjects and by ultrasound evaluation for
5 subjects. Hepatic rupture was diagnosed in the nal
patient during a laparotomy that was performed because of an acute abdomen and hypovolemic shock.
The grade of hepatic involvement in this series varied
from a minor capsular laceration to extensive parenchymal rupture. Most hepatic lesions were found in the right
lobe. One patient with a stable subphrenic hematoma was
treated conservatively. Cases with minor capsular lacerations (n = 3) underwent suture and omental patching.
Patients who had nonfriable and localized lesions (n = 5)
underwent hepatic artery ligation and cholecystectomy.
One of these patients with more extensive lesions had the
hepatic parenchyma compressed with surgical towels
before hepatic artery ligation. Cholecystectomies were
performed to avoid gallbladder necrosis. One patient
who was admitted in hypovolemic shock underwent
immediate laparotomy. A large ruptured hepatic hematoma was observed in the right lobe. This patient died
during the surgical procedure. Almost all subjects

Araujo et al

131

Table II

Demographics and treatment of hepatic rupture

Case

Age (wk)

Time of occurrence

Treatment

Blood products

36

36 Wk pregnancy

44

14

Cryoprecipitate, packed
red blood cells, plasma
No therapy

43

10

Puerperium
(day 1)
24 Wk pregnancy

Hepatic artery ligation

and cholecystectomy
No procedure
(dead at admission)
Omentum patching

48

43

15

Puerperium
(day 1)
37 Wk pregnancy

42

34 Wk pregnancy

45

15

40 Wk pregnancy

42

35 Wk pregnancy

38

38 Wk pregnancy

10

27

33 Wk pregnancy

Parity (n)

received support therapy with the transfusion of blood

products that included plasma, cryoprecipitate, red
blood packs, and platelets. The one exception was a
patient who was treated conservatively and also received
albumin. Table II shows the demographics and types of
treatment for cases in this series.
The median length of hospitalization was 14 G 13.2
days. The patient, who was treated nonsurgically, was
hospitalized for 32 days. The most common complications were hematoma in the surgical site, which later
spontaneously reabsorbed, pleural eusion, and fever.
No infection was observed in any of the patients. The survival maternal rate was 9 of 10 patients. Only 3 fetuses
were born alive, and 1 infant died right after the delivery
because of severe intrauterine anoxia. Table III shows
the major complications and maternal and neonatal
outcomes in hepatic rupture. A more complete review
of the 3 more unique cases is presented.

Surgical stitching
Hepatic artery ligation and
omentum patching
Hepatic artery ligation and
omentum patching
Surgical stitching and
omentum patching
Hepatic artery ligation and
omentum patching
Clinical observation and
monitoring
Hepatic artery ligation and
cholecystectomy

Plasma, cryoprecipitate, packed

red blood cells, platelets
Plasma, cryoprecipitate, packed
red blood cells, platelets
Plasma, cryoprecipitate, packed
red blood cells, albumin
Plasma, cryoprecipitate, albumin
Plasma, cryoprecipitate
Plasma, cryoprecipitate,
platelets, albumin
Albumin
Plasma, cryoprecipitate

768 U/L, an LDH level of 2207 U/L, and a hematocrit

level of 20%. The patients blood pressure and laboratory
ndings grew progressively worse. A cesarean delivery
was performed, and a healthy child was born without
signs of hypoxia. Hemoperitoneum was diagnosed during surgery, and a hepatic hematoma of 9.8 cm diameter
was revealed during laparotomy. No additional sites of
hepatic bleeding were seen. Because the hepatic hematoma was located subphrenically in an area that was difcult to access surgically and because there were no signs
of further bleeding, the patient was monitored clinically
and by serial ultrasound evaluations. The patient experienced dyspnea, tachycardia, thoracic pain, right upper
quadrant abdominal pain, and dry cough. A chest x-ray
revealed a pleural eusion on the right side. The patient
underwent thoracocentesis. A culture of the pleural aspirate was negative. In addition, blood and urine cultures
were also negative. The patient had no further complications and was discharged 32 days after admission.

Case 1
A 38-year-old multiparous woman (G5P4) was admitted
at 38 weeks of gestation because of epigastric pain and
severe preeclampsia, with a blood pressure of 160/100
mm Hg. The patient was treated with magnesium sulfate
(loading dose of 5 g, followed by 2 g/h for 24 hours). The
signs, symptoms, and laboratory evaluation met the
criteria for HELLP syndrome. After the blood pressure
was stabilized, labor was induced with oxytocin, with no
response after 12 hours. Laboratory ndings revealed
a platelet count of 91.4 ! 103/mm3, an AST level of

Case 2
A 36-year-old woman (G3P2) was admitted at 36 weeks
of gestation because of seizures. She initially had a
blood pressure of 90/60 mm Hg, a thready pulse, and
uterine hypertonia. No fetal pulse was detected. A
clinical diagnosis of placental abruption was made.
The major laboratory ndings at admission were a
platelet count of 92.0 ! 103/mm3, an AST level of
1861 U/L, an LDH level 1514 U/L, and a hematocrit
level of 25%. The patient underwent a laparotomy,

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Araujo et al

Table III Complications and maternal and neonatal outcomes in hepatic rupture

Case

Complication

Fever and
hematoma
Death
Pleural effusion
Dyspnea
Thrombophlebitis
and subphrenic
hematoma
Pleural effusion
No additional
complication
Pleural effusion
Fever and dyspnea
No additional
complication

2
3
4
5

6
7
8
9
10

Length of
hospital
stay (d)

Recovery
Maternal

Neonatal

10

Yes

No

0
45
8
16

No
Yes
Yes
Yes

No
No
No
No

25
10

Yes
Yes

No*
No

20
32
12

Yes
Yes
Yes

No
Yes
Yes

* Newborn infant died 6 hours after birth.

and a stillbirth was delivered with signs of recent

death. Extensive abdominal bleeding because of hepatic
laceration was observed. The hepatic artery was ligated,
which was followed by cholecystectomy and suture
of the hepatic lesion. Postsurgical supportive therapy
included plasma, cryoprecipitate, and red blood cell
transfusions. The patient was monitored carefully by
ultrasound scanning. Her postoperative course was complicated by fever on the subsequent postoperative days,
but blood cultures were negative. Serial ultrasound
examinations revealed a hepatic hematoma that formed
after surgery, which was spontaneously reabsorbed after
2 weeks.

Case 3
A 44-year-old woman (G14P14) was admitted to the
hospital 24 hours after the delivery of a dead fetus and
after the development of several postpartum generalized
seizures. She had a history of high blood pressure
throughout the pregnancy. The seizures were followed
by a sudden decrease in blood pressure, cutaneous
pallor, and altered mental status. At the time of admission, the patient was unresponsive and hypotensive.
Physical examination revealed multiple cutaneous ecchymoses and gingival and vaginal hemorrhage. Laboratory ndings showed a platelet count of 80.0 ! 103/
mm3, an AST level of 430 U/L, an LDH level of 2960
U/L and a hematocrit level of 15%. A laparotomy revealed hemoperitoneum and a large hepatic laceration.
The patient had cardio pulmonary arrest during surgery,
which was followed by death.

Comment
Spontaneous hepatic rupture is a rare and life-threatening complication of pregnancy that reportedly occurs in
1 of every 45,000 to 225,000 deliveries.1-3,9 Hepatic rupture occurs in pregnancies that are complicated by preeclampsia, eclampsia, or HELLP syndrome.10 In our
series, a higher incidence of hepatic rupture caused by
HELLP syndrome was seen. Most of these cases did
not receive proper management of the preeclampsia.
In addition, the Maternity Hospital is a reference center
for major obstetrics complications, likely increasing the
incidence of this condition.
A large case series composed of 141 cases of hepatic
rupture was compiled from dierent studies.10 Of these
cases, 58 cases indicated a platelet nadir, and 77.5% of
these cases had platelet counts of !100 ! 103/mm3.11
Our cases fullled the criteria of complete HELLP syndrome. Hepatic rupture in our series occurred more
often in multiparous women who were O40 years old
(mean, 40.8 years; median, 42.5 years). The youngest
woman in the group was 27 years old, and the oldest
woman was 48 years. In contrast, other studies have
not reported age as a risk,12 although some reports
have indicated that multiparous older women are at a
higher risk of the development of intrahepatic hemorrhage than younger or primiparous women.13 One of
our case occurred after delivery. Postpartum hepatic
rupture has been reported in another series.14
The development of abdominal pain in pregnant
women with severe preeclampsia or HELLP syndrome
should be considered ominous and should lead to careful
screening to rule out hepatic lesions.13 Abdominal pain
was the rst clinical sign that was observed in our series.
The appropriate management of hepatic lesions likely
varies from conservative treatment with support therapy
to surgical management in combination with supportive
therapy. Surgical management can vary from the stitching of the lesions, omental patching, hepatic artery ligation, and embolization of the hepatic artery to liver
transplantation. In our series, 50% of the women underwent hepatic artery ligation. The shortcomings of this
procedure and its association with maternal mortality
rates have been discussed in other reports. Nonetheless,
in our cases, no complication of hepatic artery ligation
were observed, and patients were discharged on an average of 16 days after admission.
The mortality rate in hepatic rupture in pregnancy
has decreased from 100% to 30% in the recent case
series.8,10 A substantial reduction in mortality rates has
been attributed to the introduction of several procedures, which include hepatic arterial embolization.10 In
our series, a 10% mortality rate was observed, which
is considerably lower than the reported mortality rate.
Nonetheless, the perinatal child mortality rate in our
series was high, similar to other reports.10

Araujo et al
Nonsurgical management of hepatic rupture has
been considered in cases without coagulopathy.10 In our
series, only 1 case of hepatic rupture was managed
medically, without surgical intervention. This particular
patient had a high surgical risk because of the anatomic
location of the hepatic lesion, and she did not have signs
of active bleeding. We concluded that patients who are
treated with supportive medical care alone should be
carefully followed with adequate hemodynamic support
and regular imaging.15,16
In summary, a diagnosis of hepatic rupture should be
considered when there is a sudden onset of hypotension
and acute anemia in a patient with pregnancy-induced
hypertension. This type of evaluation should lead to
prompt diagnostic tests for hepatic rupture and treatment. In our series, surgical intervention with hepatic
artery ligation was successful. This series illustrates that
patients with hepatic rupture and HELLP syndrome
should be treated by a multidisciplinary team of physicians and other health professionals in a setting in which
adequate supportive care is available to treat this lifethreatening condition.

Acknowledgments
We thank the late general surgeon Dr Expedito Fernandes for his care of the patients described in this
publication; Mary E. Wilson, MD, (University of Iowa)
and Richard D. Pearson, MD, (University of Virginia)
for their helpful suggestions; and Jose W. Queiroz
(Universidade Federal do Rio Grande do Norte) for
the statistical analysis.

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