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52 O B G M A N A G E M E N T • A p r i l 2 0 0 5

Here’s a disturbing fact: If it lookslike HELLP syndrome, andimpairs the patient like HELLP

syndrome, it isn’t necessarily HELLP syn-drome. A plethora of diagnostic criteriafrom different investigators over the yearshas confused the issue of what constitutesthis syndrome—not to mention how tomanage it.

A management issue has also attract-ed recent attention: use of corticosteroidseither antepartum to enhance maternalstatus so that epidural anesthesia can beadministered, or postpartum to improveplatelets. Such improvements are onlytransient, however, and we lack definitivedata on the benefits.

One thing is certain, however. Thecombination of hemolysis, liver dysfunc-tion or injury, and platelet consumption inwomen with preeclampsia makes adversematernal and perinatal outcomes morelikely and leaves no room for expectantmanagement.

HELLP syndrome also has become amajor issue in litigation against obstetri-cians and medical and surgical consult-ants. Lawsuits usually allege misdiag-nosed preeclampsia, delayed delivery, orimproper recognition and management ofcomplications.

❚ Pinning HELLP downOne of the best tools to identify HELLPsyndrome is a healthy dose of suspicion,since it can affect any pregnant woman atany time: antepartum, intrapartum, orwithin 1 week postpartum. Approximately72% of cases are diagnosed before deliv-ery, and the rest are diagnosed during thefirst week postpartum.

Weinstein noted that the signs andsymptoms of HELLP syndrome can occurwithout clinical evidence of severepreeclampsia (severe hypertension and/orsevere proteinuria). Indeed, he reportedthat hypertension can be mild or absent inmost patients with HELLP, and protein-uria can be mild.

Weinstein coined the term HELLP syn-drome in 1982 to describe these abnormal-ities in women with preeclampsia:

H = hemolysisEL = elevated liver enzymesLP = low plateletsAnother obstacle to early detection:

Patients may have nonspecific signs andsymptoms, none of which are diagnostic ofclassical preeclampsia.

However, HELLP syndrome is mostcommon in women who have already beendiagnosed with gestational hypertensionand/or preeclampsia.

A practical plan to detect and manage HELLP syndromeHow to minimize the risk of serious morbidity, includingtips on distinguishing HELLP from other conditions, stabilizing the patient, and managing labor, delivery, and the postpartum period.

❙ Hallmarks of HELLPPage 55

❙ Conditions thatheighten riskPage 56

❙ Cesarean delivery:Indications and managementPage 63

❙ Rare but life-threatening:Subcapsular liver hematomaPage 64

IN THIS ARTICLE

OBGOBGMANAGEMENT

Baha M. Sibai, MD Professor and ChairmanDepartment of Obstetrics and GynecologyUniversity of Cincinnati Collegeof Medicine

B E S T P R A C T I C E S F O R D I A G N O S I S A N D M A N A G E M E N T O F P R E E C L A M P S I A

C O N T I N U E D

A practical plan to detect and manage HELLP syndrome ▲

w w w. o b g m a n a g e m e n t . c o m A p r i l 2 0 0 5 • O B G M A N A G E M E N T 55

HELLP is more likely

with severe hypertension

Overall, the incidence of HELLP syn-drome in women with gestational hyper-tension/preeclampsia increases with theseverity of the condition. HELLP syn-drome also is more likely in women withearly-onset hypertension/preeclampsia(before 34 weeks’ gestation).

❚ Making the diagnosisHELLP syndrome is diagnosed when all 3of the following are present:

• Hemolysis, defined as the presence ofmicroangiopathic hemolytic anemia.This is the hallmark of the triad.

• Elevated liver enzymes (either aspar-tate aminotransferase [AST] or alanineaminotransferase [ALT]). This compo-nent signifies liver cell ischemia and/ornecrosis.

• Low platelet count (<100,000/mm3).TABLE 1 summarizes the laboratorycriteria for the diagnosis.

When to begin testing

In women with new-onset hypertension,order a complete blood count with plateletsand liver enzyme analysis at the time of diag-nosis and serially thereafter. The frequency ofthese tests depends on the initial test results,severity of disease, and onset of symptoms.

In women without hypertension, I rec-ommend obtaining the same blood tests atthe onset of any of the signs and symptomslisted in TABLE 2.

Assessing test results

Clinicians should be familiar with theupper limit for liver-enzyme tests in theirlaboratory. I suggest a cutoff more thantwice the upper limit for a particular test.

Also keep in mind that these parame-ters are dynamic; some women will meetonly some of the criteria early in the diseaseprocess. Moreover, maternal complications aresubstantially higher when all 3 componentsare present than when only 1 or 2 are present.

Look for these clinical findings

Hypertension. Most women with HELLP

HELLP syndrome is more likely when hypertensionor preeclampsia is diagnosed before 34 weeks

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TA B L E 1

Hallmarks of HELLP: Hemolysis, elevated liver enzymes, and low platelets

IMAGE: MAURA FLYNN

Hemolysis

Diagnosis requires at least 2 of the following:

■ Abnormal peripheral smear (schistocytes, burr cells)

■ Elevated serum bilirubin (≥1.2 mg/dL)

■ Low serum haptoglobin

■ Significant drop in hemoglobin levels, unrelated to blood loss

Elevated liver enzymes

■ Aspartate aminotransferase or alanine aminotransferase at least twice the upper level of normal

■ Lactate dehydrogenase at least twicethe upper level of normal. This valueis also elevated in severe hemolysis

Low platelets

■ <100,000/mm3


A practical plan to detect and manage HELLP syndrome

▲

syndrome have hypertension. In 15% to50% of cases, the hypertension is mild, butit may be absent in 15%. Proteinuria. Most patients also have pro-teinuria by dipstick (≥1+). Proteinuria maybe absent in approximately 13% ofwomen with HELLP syndrome, althoughthey will likely have many of the symp-toms reported by women with severepreeclampsia.

TABLE 3 lists the signs and symptomsto be expected in these patients, along withtheir frequency.

The usual times of onset

Antepartum cases. As was previouslynoted, HELLP syndrome usually developsbefore delivery, with the most frequentonset being before 37 weeks’ gestation(TABLE 4). In the postpartum period, most cases devel-op within 48 hours after delivery. Of these,approximately 90% occur in women whohad antepartum preeclampsia that pro-gressed to HELLP syndrome in the post-partum period. However, approximately20% of postpartum cases develop morethan 48 hours after delivery.

Another important point: HELLP syn-drome can develop for the first time post-partum in women who had no evidence ofpreeclampsia before or during labor. Thus,it is important to educate all postpartumwomen to report new symptoms (listed inTABLE 3) as soon as possible. When thesesymptoms develop, evaluate the patient forboth preeclampsia and HELLP syndrome.

Risk for life-threatening

maternal complications

When all components of HELLP syndromeare present in a woman with preeclampsia,the risk of maternal death and seriousmaternal morbidities increases substantial-ly (TABLE 5). The rate of these complica-tions depends on gestational age at onset,presence of associated obstetric complica-tions (eclampsia, abruptio placentae, peri-partum hemorrhage, or fetal demise) orpreexisting conditions (lupus, renal disease,chronic hypertension, or type 1 diabetes).Abruptio placentae increases the risk ofdisseminated intravascular coagulopathy(DIC), as well as the need for blood trans-fusions. Marked ascites (>1 L) leads to higher ratesof cardiopulmonary complications.

Differential diagnosis

When diagnosing HELLP syndrome, con-firm or exclude the conditions listed inTABLE 6, since the presenting symptomsand clinical and laboratory findings inwomen with HELLP syndrome overlapthose of several microangiopathic disor-

T A B L E 3

CONDITION FREQUENCY (%)

Hypertension 85

Proteinuria 87

Right upper quadrant 40–90or epigastric pain

Nausea or vomiting 29–84

Headaches 33–60

Visual changes 10–20

Mucosal bleeding 10

Jaundice 5

Signs and symptoms

HELLP syndromesometimes appearsfor the first timepostpartum in women who hadno evidence of preeclampsiabefore or duringlabor

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T A B L E 2

• Preeclampsia-eclampsia

Early onset

During expectant management

• Severe gestational hypertension

• Early-onset hypertension, or severeintrauterine growth restriction

• Thrombophilias

• Abruptio placentae

• Nonspecific viral-syndrome-like symptoms

• Right upper quadrant, epigastric, or retrosternal pain

• Persistent nausea or vomiting in thirdtrimester

• Bleeding from mucosal surfaces

• Unexplained hematuria or proteinuria

• Petechial hemorrhages or ecchymosis

Conditions that heighten the risk of HELLP



▲

ders that can develop during pregnancyand/or postpartum. In some women,preeclampsia may be superimposed on oneof these disorders, further confounding analready difficult differential diagnosis.

Because of the remarkably similar clin-ical and laboratory findings of these dis-eases, make every effort to achieve an accu-rate diagnosis, since management and out-comes may differ among these conditions.

❚ Initial management Hospitalize the patient

Because HELLP syndrome usually is char-acterized by progressive and sometimes

sudden deterioration in maternal and fetalconditions, patients should be hospitalizedand observed in a labor and delivery unit.

Initially, assume the patient has severepreeclampsia and treat her with intra-venous magnesium sulfate to prevent con-vulsions and antihypertensive medicationsas needed to keep systolic blood pressurebelow 160 mm Hg and diastolic bloodpressure below 105 mm Hg.

Blood tests should include: • complete blood count with platelet count, • peripheral smear evaluation, • serum AST, • lactate dehydrogenase, • creatinine, • bilirubin, and • coagulation studies.

These tests help confirm the diagnosisand check for the presence of DIC, massivehemolysis, severe anemia, or renal failure.

The first priority is to assess the patientfor the presence of cardiovascular complica-tions, signs of liver hematoma or hemor-rhage, and abruptio placentae. If any is pres-ent—particularly hypotension, hypovolemia,DIC, or pulmonary edema—make everyeffort to stabilize the maternal condition.

Can delivery wait 48 hours

for corticosteroids?

Evaluate fetal status by heart rate monitor-ing or biophysical profile, and confirm ges-tational age. Then decide whether deliveryis indicated or can be delayed for 48 hoursso that corticosteroids can be given.No room for expectant management. Do notconsider expectant management in womenwith true HELLP syndrome. Delivery canonly be delayed for a maximum of 48hours—and only when both mother andfetus are stable, at 24 to 34 weeks’ gestation,and awaiting the benefit of corticosteroids.Corticosteroid dosing. My practice is togive 2 doses of either betamethasone 12mg intramuscularly every 12 hours ordexamethasone 12 mg intravenouslyevery 12 hours. This is to improve mater-nal status, at least temporarily.

Initiate delivery within 24 hours afterthe last steroid dose, with continuousmonitoring in the labor and delivery unit.

T A B L E 5

COMPLICATION FREQUENCY (%)

Death 1

Adult respiratory 1distress syndrome

Laryngeal edema 1–2

Liver failure or hemorrhage 1–2

Acute renal failure 5–8

Pulmonary edema 6–8

Pleural effusions 6–10

Abruptio placentae 10–15

Disseminated intravascular 10–15coagulopathy

Marked ascites 10–15

Maternal complications

In women with true HELLP,delivery can only be delayed for up to 48 hoursfor corticosteroidadministration—and then only ifboth mother and fetus are stable

FAST TRACK

T A B L E 4

RELATION TO DELIVERY PERCENTAGE

Antepartum 72

Postpartum 28

≤48 hours 80

>48 hours 20

GESTATIONAL AGE (WEEKS) PERCENTAGE

17–20 2

21–27 10

28–36 68

>37 20

* Based on 700 cases

Usual times of onset*

C O N T I N U E D


w w w. o b g m a n a g e m e n t . c o m

Avoid pudendal blockbecause of the riskfor bleeding and hematoma formation in this area

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A p r i l 2 0 0 5 • O B G M A N A G E M E N T 63

anesthesiologist if the platelet countexceeds 75,000/mm3.

Some authors report rising plateletcounts after intravenous dexamethasoneand, with the improved platelets, greater useof epidural anesthesia, especially in womenwho achieved a 24-hour latency periodbefore delivery. However, since the platelet

Although some women may demon-strate transient improvement in theirblood tests (eg, increased platelet count ordecreased AST levels), delivery is still indi-cated. Conversely, in some cases, maternaland fetal conditions may deteriorate, man-dating delivery before the 2 doses ofsteroids are completed.

❚ Delivery considerationsHELLP syndrome does not justify

immediate cesarean

Patients with HELLP syndrome in labor orwith rupture of membranes can delivervaginally in the absence of obstetric com-plications. In addition, induction or aug-mentation of labor is acceptable with eitheroxytocin infusion or prostaglandins if thefetal gestational age is 32 weeks or moreand the cervical Bishop score exceeds 5.

TABLE 7 lists the indications for elec-tive cesarean delivery and summarizesmanagement during surgery. It is importantto stabilize the maternal condition, correctcoagulopathy, and have blood or bloodproducts available before initiating surgery.

Watch for oozing from surgical sites

In a cesarean section, generalized oozingfrom the surgical site can occur during theoperation or immediately postpartumbecause of the continued drop in plateletcount in some of these patients. Thus, it isadvisable to insert a subfascial drain andto leave the skin incision open for at least48 hours to avoid hematoma formation inthese areas (FIGURE 1).

Small doses of systemic

opioids are best

For maternal analgesia during labor, givesmall, intermittent doses of systemic opi-oids. For repair of episiotomy or vulvar orvaginal lacerations, use local infiltrationanesthesia.

Avoid pudendal block because of thepotential for bleeding and hematoma for-mation in this area. Epidural anesthesiamay be used after consultation with the

T A B L E 7

Indications for cesarean

• Nonreassuring fetal status

• Abnormal fetal presentation

• <30 weeks’ gestation and Bishop score <5

• <32 weeks’ gestation with intrauterinegrowth restriction or oligohydramnios and Bishop score <5

• Known subcapsular liver hematoma

• Suspected abruptio placentae

Management during cesarean

• General anesthesia for platelet count <75,000/mm3

• Transfuse 6 units of platelets if count <40,000/mm3

• Insert subfascial drain

• Secondary skin closure or leave subcutaneous drain

• Observe for bleeding from upper abdomen prior to closure

Cesarean delivery: Indications and management

T A B L E 6

• Acute fatty liver of pregnancy

• Acute pancreatitis

• Antiphospholipid syndrome

• Cholecystitis

• Disseminated herpes simplex

• Fulminant viral hepatitis

• Hemolytic uremic syndrome

• Hemorrhagic or septic shock

• Immune thrombocytopenic purpura

• Stroke

• Systemic lupus erythematosus

• Thrombotic thrombocytopenic purpura

Differential diagnosis


▲

Profound hypovolemic shockin a previouslyhypertensivepatient may be a sign of rupture of a liver hematoma

FAST TRACK


count may drop again, insert the epiduralcatheter once the desired platelet level (withanesthesiologist approval) is reached.

❚ Suspected liver hematomaA rare and potentially life-threateningcomplication of HELLP syndrome is sub-capsular liver hematoma (FIGURE 2).Unfortunately, the rarity of this complica-tion sometimes causes it to be overlooked.

Early signs and symptoms

Liver hematomas can develop antepartum,during labor, or in the postpartum period.Presenting symptoms may include severeepigastric or retrosternal pain in associa-tion with breathing difficulty (pain oninspiration), with or without shoulder orneck pain.

When profound hypovolemic shockoccurs in a previously hypertensive patient,suspect rupture of a liver hematoma.Diagnosis can be made by ultrasound orcomputed tomography (CT) imaging of theliver, both of which can also confirmintraperitoneal bleeding.

In most cases, rupture involves theright lobe of the liver and is preceded by aparenchymal liver hematoma.

Mortality can exceed 50%

Maternal and fetal mortality increase sub-stantially when a subcapsular liverhematoma is present. In fact, mortalitymay exceed 50% when frank rupture ofthe capsule involves liver tissue.

Choose conservative management

whenever possible

Management of subcapsular liverhematoma depends on maternal hemody-namic status, integrity of the capsule (rup-tured or intact), and the fetal condition.

Conservative management is prefer-able in hemodynamically stable womenwith an unruptured hematoma. It consistsof close monitoring of the patient’s hemo-dynamic and coagulation status and serialassessment of the hematoma with ultra-sound or CT scan. Avoid exogenous trauma to the liver, suchas frequent abdominal palpation, emesis,or convulsions. Any sudden increase inintraabdominal pressure can led to ruptureof the hematoma.

When rupture occurs

This surgical emergency requires an acutemultidisciplinary team, including anOb/Gyn, anesthesiologist, highly qualifiedsurgeon, and a representative of the hospi-tal’s blood bank.

F IGURE 2

Rare but life-threatening:Subcapsular liver hematoma

Liver hematomas can develop antepartum, intrapartum, or postpartum. Presenting symptomsmay include severe epigastric or retrosternal pain in association with respiratory difficulty (pain oninspiration), with or without shoulder or neck pain.

F IGURE 1

Insert subfascial drain at cesarean section

Because generalized oozing from the surgical sitecan occur intraoperatively or immediately postpar-tum, insert a subfascial drain and leave the skin incision open for at least 48 hours to avoidhematoma formation.

C O N T I N U E D


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A sudden drop in blood pressureto hypotensive levels can signify severe hemolysis,unrecognizedintraperitonealblood loss, or sepsis

FAST TRACK

pletely recover within 72 hours, while oth-ers deteriorate further or fail to recover foras long as 1 week after delivery. Thus,some women may require intensive moni-toring for several days because of the riskof pulmonary edema, renal failure, oradult respiratory distress syndrome.

Keep in mind that, in some of thesewomen, the cause of the postpartum dete-rioration may be something other thanHELLP syndrome (TABLE 6).

Watch for sudden hypotension

A sudden drop in blood pressure tohypotensive levels can be an early sign ofsevere hemolysis or unrecognizedintraperitoneal blood loss (from surgicalsites or ruptured liver hematoma), as wellas sepsis.

In a woman with severe hemoconcen-tration (ie, severe vasoconstriction), sud-den hypotension also may indicate exces-sive vasodilation from antihypertensivedrugs such as hydralazine or nifedipine,resulting in relative hypovolemia.

Such a case requires volume resuscita-tion, blood transfusion (if indicated), andevaluation for unrecognized bleeding.

Use of steroids

Some authors recommend giving intra-venous dexamethasone (5 to 10 mg every12 hours) for approximately 48 hours afterdelivery in women who develop antepar-tum or postpartum HELLP. They claim thistreatment improves maternal blood tests,shortens recovery, and reduces maternalmorbidity.

However, at present, no data indicatethis approach has clinical benefit—and therisks are unknown. For these reasons,treatment with intravenous dexametha-sone after delivery remains empiric. ■

B I B L I O G R A P H Y

Abramovici D, Friedman SA, Mercer BM, Audibert F, Kao L,Sibai BM. Neonatal outcome in severe preeclampsia at 24to 36 weeks’ gestation. Does HELLP (hemolysis, elevatedliver enzymes, and low platelet count) syndrome matter?Am J Obstet Gynecol. 1999;180:221–225.

Audibert F, Friedman SA, Frangieh AY, Sibai BM. Clinicalutility of strict diagnostic criteria for the HELLP (hemolysis,

Maternal resuscitation should include: • transfusion of packed red blood cells

to maintain blood pressure and tissueperfusion,

• correction of coagulopathy with freshfrozen plasma and platelets, and

• laparotomy, preferably using a cellsaver.

Options at laparotomy include:

• packing and drainage (preferred), • ligation of the hepatic lacerations, • embolization of the hepatic artery to

the affected liver segment, and • loosely suturing omentum or surgical

mesh to the liver surface.

❚ Postpartum careIn women who develop HELLP prior todelivery, closely monitor postpartum vitalsigns, intake and output, and symptoms inintensive care or a similar facility for atleast 48 hours.

During this time, my practice is to givethe patient intravenous magnesium sulfateand antihypertensive medications as need-ed to keep systolic blood pressure below155 mm Hg (the standard is 160 mm Hg)and diastolic blood pressure below 105mm Hg.

The rationale for this treatment is toprevent bleeding in the brain if the womanhas thrombocytopenia.

When HELLP appears

in the postpartum period

Several maternal complications fromHELLP syndrome may not appear untilimmediately postpartum. Thus, allwomen with preeclampsia require closemonitoring of vital signs, fluid intake andoutput, laboratory values, and pulseoximetry for at least 48 hours.

Also continue magnesium sulfate inthe postpartum period and keep maternalblood pressure below 155 mm Hg systolicand 105 mm Hg diastolic.

Time to recovery

Most patients begin to improve or com-


elevated liver enzymes, and low platelets) syndrome. Am JObstet Gynecol. 1996;175:460–464.

Egerman RS, Sibai BM. Recognizing and managing HELLPsyndrome and its imitators. Contemporary Ob/Gyn. 1997;(October):129–149.

Magann EF, Perry KG Jr, Meydrech EF, Harris RL, ChauchanSP, Martin JN Jr. Postpartum corticosteroids: acceleratedrecovery from the syndrome of hemolysis, elevated liverenzymes, and low platelets (HELLP). Am J Obstet Gynecol.1994;171:1154–1158.

Martin Jr JN, Thigsen BD, Rose CH, et al. Maternal benefitof high-dose intravenous corticosteroid therapy for HELLP.Am J Obstet Gynecol. 2003;189:830–834.

O’Brien JM, Milligan DA, Barton JR. Impact of high-dosecorticosteroid therapy for patients with HELLP (hemolysis,elevated liver enzymes, and low platelet count) syndrome.Am J Obstet Gynecol. 2000;183:921–924.

O’Brien JM, Shumate SA, Satchwell SL, Milligan DA,Barton JR. Maternal benefit to corticosteroid therapy inpatients with HELLP (hemolysis, elevated liver enzymes,and low platelets) syndrome: impact on the rate of region-al anesthesia. Am J Obstet Gynecol. 2002;186:475–479.

Rinehart BK, Terrone DA, Magann EF, Martin RW, May WL,Martin JN Jr. Preeclampsia-associated hepatic hemorrhageand rupture: mode of management related to maternal andperinatal outcome. Obstet Gynecol Surv. 1999;196–202.

Sibai BM, Ramadan MK, Usta I, Salama M, Mercer BM,

Friedman SA. Maternal morbidity and mortality in 442pregnancies with hemolysis, elevated liver enzymes, andlow platelets (HELLP syndrome). Am J Obstet Gynecol.1993;169:1000–1006.

Sibai BM. The HELLP syndrome (hemolysis, elevated liverenzymes, and low platelets): much ado about nothing? AmJ Obstet Gynecol. 1990;162:311–316.

Sibai BM. Diagnosis, controversies, and management ofHELLP syndrome. Obstet Gynecol. 2004;103:981–991.

Tompkins MJ, Thiagarajah S. HELLP (hemolysis, elevatedliver enzymes, and low platelet count) syndrome: the bene-fit of corticosteroids. Am J Obstet Gynecol. 1999;181:304–309.

VanPampus MG, Wolf H, et al. Maternal and perinatal out-come after expectant management of the HELLP syndromecompared with preeclampsia without HELLP syndrome.Eur J Obstet Gynecol Reprod Biol. 1998;76:31.

Weinstein L. Syndrome of hemolysis, elevated liver enzymes,and low platelet count: A severe consequence of hyperten-sion in pregnancy. Am J Obstet Gynecol. 1982;142:159–167.

Weinstein L. Preeclampsia/eclampsia with hemolysis, ele-vated liver enzymes and thrombocytopenia. ObstetGynecol. 1985;66:657–660.

The author reports no financial relationships relevant to thisarticle.


w w w. o b g m a n a g e m e n t . c o m A p r i l 2 0 0 5 • O B G M A N A G E M E N T 69

All women with preeclampsiarequire close monitoring of vitalsigns, fluid intakeand output, lab values, andpulse oximetry for at least 48 hoursafter delivery

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