DOI:10.2214/AJR.04.0817
AJR 2005; 185:1205-1210
© American Roentgen Ray Society
Abdominal Imaging Features of HELLP Syndrome: A 10-Year Retrospective Review
John O. Nunes1,
Mary Ann Turner and
Ann S. Fulcher
1 All authors: Department of Radiology, Virginia Commonwealth University/Medical
College of Virginia Hospitals and Physicians, 1101 E Marshall St., Sanger
Hall, Rm. 4-050, PO Box 980470, Richmond, VA 23298-0470.
Received May 24, 2004;
revised November 3, 2004;
Address correspondence to A. S. Fulcher.
Abstract
OBJECTIVE. The purpose of this study was to describe the abdominal
imaging features associated with HELLP (hemolysis, elevated liver enzymes, low
platelets) syndrome, a complication of pregnancy.
CONCLUSION. The abdominal imaging features of HELLP syndrome include
intraparenchymal and perihepatic hematomas and hemoperitoneum as noted on
sonography, CT, and angiography.
Introduction
HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome describes
a pregnancy-related condition in which the pregnant patient shows hemolysis,
elevated liver enzymes, and low platelets. This syndrome was originally
described in 1954 by Pritchard et al.
[1], with the acronym coined in
1982 by Weinstein [2]. A rare
complication of this syndrome is hepatic hemorrhage that may result in hepatic
rupture, thus significantly increasing both maternal and perinatal morbidity
and mortality. Urgent intervention, usually with emergent delivery of the
fetus and exploratory laparotomy, is necessary for treatment. Although hepatic
hemorrhage–complicating HELLP syndrome is well documented in the
obstetrics and surgical literature
[3–8],
there is scant mention of the abdominal imaging features of HELLP syndrome in
the radiology literature
[9–13].
The purpose of this study was to describe the abdominal imaging features of
HELLP syndrome and thereby heighten the awareness of radiologists to this rare
but life-threatening complication of pregnancy.
Materials and Methods
A computerized medical records search spanning the 10-year period of
1992–2002 was conducted at our tertiary care medical center using the
codes from the ninth revision of the International Classification of
Diseases (ICD-9) [14] for
preeclampsia and HELLP syndrome. This search revealed 568 patients with the
diagnosis of preeclampsia or HELLP syndrome. Of these 568 patients, 310
underwent imaging examinations in the department of radiology. Of this subset
of patients, 37 underwent abdominal imaging examinations including
conventional radiography, sonography, CT, and angiography. Four of the 37
patients were excluded because their medical records were not available for
review.
Review of medical records and reports of radiologic examinations in the
remaining 33 patients was conducted by one author who recorded patient age,
race, gravity and parity, age of gestation, signs and symptoms at presentation
and duration of signs and symptoms, blood pressure, hemoglobin value, liver
function test results (i.e., aspartate aminotransferase [AST] and alanine
aminotransferase [ALT]), coagulation test results, platelet count, treatment
method, abdominal imaging examination findings, surgical findings, and fetal
and maternal outcomes. Specifically, with respect to abdominal imaging
examination findings, an assessment was made for hepatic hemorrhage or
rupture, subcapsular or perihepatic hematoma, and hemoperitoneum as
complications of HELLP syndrome previously reported in the radiology
literature
[9–12].
This review revealed that three of the 33 patients had these abdominal
findings. An assessment was also made for other abdominal imaging findings
presumably unrelated to HELLP syndrome.
Abdominal imaging examinations of two of the three patients were evaluated
in consensus by two abdominal radiologists. In the case of the remaining
patient whose abdominal imaging examinations had been purged, the dictated
reports of the examinations were reviewed. Examinations reviewed included
conventional radiography, sonography, CT, and angiography. Radiologic features
assessed included presence and location of intrahepatic hemorrhage or
hematoma, characteristics of adjacent liver parenchyma, presence and location
of subcapsular or perihepatic hematoma, presence of hemorrhage at angiography,
and presence of hemoperitoneum. Approval was granted by the institutional
review board for this retrospective study; informed consent was not
required.
Results
Of the 568 patients diagnosed with preeclampsia or HELLP on the basis of
the ICD-9 codes, a review of the medical records and radiologic reports
revealed three (0.53%) with abdominal imaging findings. The clinical and
imaging characteristics of these three patients are summarized in
Table 1. These patients had a
mean age of 32.7 years and a mean age of gestation of 31.3 weeks. All three
patients had hemolysis, thrombocytopenia, and elevated liver enzymes and
thereby met the criteria for HELLP syndrome
[3].
Of the three patients, all showed surgically confirmed hepatic rupture and
acute hemoperitoneum. Emergent laparotomy and cesarean delivery preceded
imaging in each of the three patients owing to deterioration of clinical
status. Intrahepatic hematomas involving the anterior and posterior segments
of the right and medial segment of the left hepatic lobe were present in each
of the three patients as noted at surgery and imaging examinations.
On sonography, the intrahepatic hematoma was depicted as a hypoechoic focus
relative to the adjacent hepatic parenchyma and was indistinguishable in
echogenicity from the perihepatic hematoma in patient 1
(Fig. 1A). In patient 2, the
intrahepatic hematoma was not seen as a focal hypoechoic abnormality on
sonography, but as diffuse heterogeneity involving most of the right hepatic
lobe and medial segment of the left hepatic lobe
(Fig. 2A). A perihepatic
hematoma was also noted (Fig.
2B).
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Fig. 1A —38-year-old woman with HELLP (hemolysis, elevated liver
enzymes, low platelet count) syndrome and liver rupture (patient 1 in
Table 1). Transverse sonogram
of liver shows intraparenchymal hematoma (H) that is hypoechoic relative to
uninvolved liver (L) and isoechoic relative to adjacent perihepatic hematoma
(asterisks).
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Fig. 2A —33-year-old woman with HELLP (hemolysis, elevated liver
enzymes, low platelet count) syndrome and liver rupture (patient 2 in
Table 1). Transverse sonogram
of liver shows heterogeneity (H), which is representative of hematoma,
involves right hepatic lobe and medial segment of left. Perihepatic hematoma
(asterisk) is slightly hypoechoic relative to liver.
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Fig. 2B —33-year-old woman with HELLP (hemolysis, elevated liver
enzymes, low platelet count) syndrome and liver rupture (patient 2 in
Table 1). Sagittal sonogram of
liver reveals perihepatic hematoma (asterisk) is slightly hypoechoic
relative to adjacent liver.
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Fig. 1B —38-year-old woman with HELLP (hemolysis, elevated liver
enzymes, low platelet count) syndrome and liver rupture (patient 1 in
Table 1). Contrast-enhanced CT
scan obtained at level similar to A reveals low-attenuation hematoma
(H) in right hepatic lobe is isoattenuating to perihepatic hematoma
(asterisks). Irregular interface between intraparenchyma and
perihepatic hematoma presumably represents site of rupture.
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Fig. 1C —38-year-old woman with HELLP (hemolysis, elevated liver
enzymes, low platelet count) syndrome and liver rupture (patient 1 in
Table 1). Contrast-enhanced CT
scan obtained 3.2 cm cephalad to B shows that hematoma involves medial
segment of left hepatic lobe (M) and most of right hepatic lobe.
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Fig. 1D —38-year-old woman with HELLP (hemolysis, elevated liver
enzymes, low platelet count) syndrome and liver rupture (patient 1 in
Table 1). Contrast-enhanced CT
scans of lower abdomen (D) and pelvis (E) reveal hemoperitoneum
(asterisk) with in vivo hematocrit effect (arrow) in right
paracolic gutter and pelvic cul-de-sac, respectively.
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Fig. 1E —38-year-old woman with HELLP (hemolysis, elevated liver
enzymes, low platelet count) syndrome and liver rupture (patient 1 in
Table 1). Contrast-enhanced CT
scans of lower abdomen (D) and pelvis (E) reveal hemoperitoneum
(asterisk) with in vivo hematocrit effect (arrow) in right
paracolic gutter and pelvic cul-de-sac, respectively.
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Fig. 1F —38-year-old woman with HELLP (hemolysis, elevated liver
enzymes, low platelet count) syndrome and liver rupture (patient 1 in
Table 1). Conventional celiac
angiogram shows multiple small hemorrhages (arrows) associated with
peripheral branches of right hepatic artery and larger hemorrhage
(arrowheads) in dome of liver.
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Fig. 1G —38-year-old woman with HELLP (hemolysis, elevated liver
enzymes, low platelet count) syndrome and liver rupture (patient 1 in
Table 1). Conventional celiac
angiogram obtained after embolization with gelatin sponge (Gelfoam, Upjohn)
and polyvinyl alcohol-soaked sponges reveals complete resolution of
hemorrhage. Minor degree of vasospasm is present and is likely related to
patient's hypovolemic state.
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Contrast-enhanced CT performed in all three patients revealed that the
intraparenchymal hepatic hematomas were, in general, lower in attenuation than
the adjacent liver and were associated with perihepatic hemorrhage-yielding
attenuations of 50–70 H (Figs.
1B,
1C, and
2C,
2D,
2E). The interface between the
intraparenchyma and perihepatic hemorrhage was irregular in each patient and
presumably represented the site of capsular rupture. Hemoperitoneum was noted
in each of the three patients and showed an in vivo hematocrit effect in some
locations (Figs. 1D and
1E).
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Fig. 2C —33-year-old woman with HELLP (hemolysis, elevated liver
enzymes, low platelet count) syndrome and liver rupture (patient 2 in
Table 1). Unenhanced CT scan of
liver shows high-attenuation perihepatic hematoma (asterisks)
adjacent to unenhanced liver (L).
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Fig. 2D —33-year-old woman with HELLP (hemolysis, elevated liver
enzymes, low platelet count) syndrome and liver rupture (patient 2 in
Table 1). Contrast-enhanced CT
scan obtained at level similar to C reveals multifocal low-attenuation
intraparenchymal hematomas (H) involving right hepatic lobe and medial segment
of left. Perihepatic hematomas (asterisks) are again noted.
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Fig. 2E —33-year-old woman with HELLP (hemolysis, elevated liver
enzymes, low platelet count) syndrome and liver rupture (patient 2 in
Table 1). Contrast-enhanced CT
scan obtained 3 cm caudad to D shows multifocal intraparenchymal
hematomas (H), perihepatic hematoma (asterisk), and hemoperitoneum
(arrow).
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The imaging features that were noted correlated with the surgical findings
in each of the three patients.
Angiography was performed in patient 1 because complete hemostasis was not
achieved during surgery. The preembolization angiogram revealed multifocal
hemorrhages associated with peripheral hepatic arteries
(Fig. 1F). The postembolization
angiogram showed no evidence of hemorrhage
(Fig. 1G).
Nonspecific findings in the additional 30 patients who underwent abdominal
imaging examinations but who had no liver abnormalities or hemoperitoneum
included nonhemorrhagic free fluid (n = 3), dilatation of the renal
collecting system (n = 4), and dilatation of the bowel (n =
4).
Discussion
HELLP syndrome is a poorly understood pregnancy-related condition for which
the treatment and cure ultimately include delivery of the fetus. The criteria
of the syndrome are debated but include hemolysis as evidenced by an abnormal
peripheral blood smear; platelet count of less than 100 x
109/L; and serum AST value of greater than 70 U/L, serum lactate
dehydrogenase value of greater than 600 U/L, or total bilirubin value of
greater than 1.2 mg/dL [3].
This syndrome is typically seen in patients with severe preeclampsia, although
it can occur in the absence of preeclampsia. Clinically the diagnosis of HELLP
syndrome may be challenging because patients may present with vague symptoms
including nausea, vomiting, headache, malaise, or viral-like symptoms
[3].
A life-threatening complication of HELLP syndrome is hepatic hemorrhage and
rupture. Hepatic rupture occurring as a complication of HELLP syndrome is
rare, with approximately 100 cases reported in the English-language literature
[6]. In the current study,
hepatic rupture occurred in only three (0.53%) of 568 patients with
preeclampsia or HELLP syndrome over a period of 10 years.
Affected patients may present with vague abdominal pain, right upper
quadrant pain, shoulder pain, or hypovolemic shock
[4]. A low index of suspicion
is warranted in patients with such symptoms to prompt emergent imaging and to
allow rapid diagnosis of hepatic hemorrhage and rupture. With increasing
severity of disease from preeclampsia to severe preeclampsia to HELLP syndrome
and to eventual liver hemorrhage, there is a significant increase in maternal
and perinatal morbidity and mortality, with hepatic hemorrhage representing
one of the most severe consequences of severe preeclampsia or the HELLP
syndrome [5]. Although most
liver-related complications of HELLP syndrome occur during the third or late
in the second trimester, some have been reported to occur during the immediate
postpartum period [4].
Although the pathogenesis of this condition remains unclear,
histopathologic findings in the liver include intravascular fibrin deposits
that presumably may lead to hepatic sinusoidal obstruction, intrahepatic
vascular congestion, and increased intrahepatic pressure with ensuing hepatic
necrosis, intraparenchymal and subcapsular hemorrhage, and eventually capsular
rupture [2,
5,
6,
15].
Barton and Sibai [4]
reported in their series of 34 patients with HELLP syndrome and right upper
quadrant pain that the most frequent imaging findings were subcapsular
hematoma (n = 13), intraparenchymal hematoma (n = 6), and
rupture (n = 4), with the latter most frequently involving the right
hepatic lobe. Henny et al. [8]
noted that hematomas were present in the right lobe in 75% of cases, in the
left lobe in 11%, and in both lobes in 14%. In our study, we found
intraparenchymal and perihepatic hematomas and hepatic rupture in all three
patients. Although the hematomas in our series involved the right hepatic
lobes and medial segment of the left in all, the right lobe was involved to a
greater degree.
In the past, patients with this condition bypassed the radiology department
because the obstetrician often performed bedside sonography and the patient
went immediately to the operating room for emergent cesarean delivery and
exploratory laparotomy. More recently, imaging of these patients is being
performed by radiologists with sonography, CT, MRI, and angiography. Although
sonography can be performed rapidly, it is possible that CT may more easily
characterize the intrahepatic abnormalities as hematomas and may better
determine the extent of the intraperitoneal hemorrhage if present. In some
instances, as occurred in one patient in our series, arterial embolization is
successful in the treatment of hepatic hemorrhage. In their review of
preeclampsia-associated hepatic hemorrhage and rupture, Rinehart et al.
[7] reported that maternal
survival was highest in the group treated with arterial embolization. These
changes in practice have effectively placed radiologists at the forefront of
diagnosis and treatment of patients with HELLP syndrome and liver hemorrhage.
Therefore, familiarity with this life-threatening condition is paramount.
Although an algorithm for when and how to image patients with HELLP
syndrome for suspected complications cannot be constructed on the basis of our
small series, Barton and Sibai
[4] recommend imaging in
patients with complaints of right upper quadrant and neck pain, shoulder pain,
or relapsing hypotension. Furthermore, they recommend follow-up with serial
CT, MRI, or sonography in patients with subcapsular hematoma of the liver
until the defect resolves.
In summary, this 10-year retrospective review of patients with preeclampsia
or HELLP syndrome in a tertiary care center revealed three of 568 patients
with liver rupture diagnosed with CT, sonography, or both and treated in one
instance with arterial embolization. Given the life-threatening nature of
HELLP syndrome complicated by liver rupture, proper evaluation, prompt
diagnosis and treatment, and heightened awareness of radiologists about this
condition are critical for improved maternal and perinatal outcomes.
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Abstract
Introduction
