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Clinical Reviews


A practical plan to detect and manage HELLP syndrome

How to minimize the risk of serious morbidity, including tips on distinguishing HELLP from other conditions, stabilizing the patient, and managing labor, delivery, and the postpartum period.

April 2005 · Vol. 17, No. 4

Here’s a disturbing fact: If it looks like HELLP syndrome, and impairs the patient like HELLP syndrome, it isn’t necessarily HELLP syndrome. A plethora of diagnostic criteria from different investigators over the years has confused the issue of what constitutes this syndrome—not to mention how to manage it.

A management issue has also attracted recent attention: use of corticosteroids either antepartum to enhance maternal status so that epidural anesthesia can be administered, or postpartum to improve platelets. Such improvements are only transient, however, and we lack definitive data on the benefits.

One thing is certain, however. The combination of hemolysis, liver dysfunction or injury, and platelet consumption in women with preeclampsia makes adverse maternal and perinatal outcomes more likely and leaves no room for expectant management.

HELLP syndrome also has become a major issue in litigation against obstetricians and medical and surgical consultants. Lawsuits usually allege misdiagnosed preeclampsia, delayed delivery, or improper recognition and management of complications.

Pinning HELLP Down

One of the best tools to identify HELLP syndrome is a healthy dose of suspicion, since it can affect any pregnant woman at any time: antepartum, intrapartum, or within 1 week postpartum. Approximately 72% of cases are diagnosed before delivery, and the rest are diagnosed during the first week postpartum.

Weinstein noted that the signs and symptoms of HELLP syndrome can occur without clinical evidence of severe preeclampsia (severe hypertension and/or severe proteinuria). Indeed, he reported that hypertension can be mild or absent in most patients with HELLP, and proteinuria can be mild.

Weinstein coined the term HELLP syndrome in 1982 to describe these abnormalities in women with preeclampsia:

  • H = hemolysis
  • EL = elevated liver enzymes
  • LP = low platelets

Another obstacle to early detection: Patients may have nonspecific signs and symptoms, none of which are diagnostic of classical preeclampsia.

However, HELLP syndrome is most common in women who have already been diagnosed with gestational hypertension and/or preeclampsia.

HELLP is more likely with severe hypertension

Overall, the incidence of HELLP syndrome in women with gestational hypertension/preeclampsia increases with the severity of the condition. HELLP syndrome also is more likely in women with early-onset hypertension/preeclampsia (before 34 weeks’ gestation).

Making The Diagnosis

HELLP syndrome is diagnosed when all 3 of the following are present:

  • Hemolysis, defined as the presence of microangiopathic hemolytic anemia. This is the hallmark of the triad.
  • Elevated liver enzymes (either aspartate aminotransferase [AST] or alanine aminotransferase [ALT]). This component signifies liver cell ischemia and/or necrosis.
  • Low platelet count (<100,000/mm3). TABLE 1 summarizes the laboratory criteria for the diagnosis.

When to begin testing

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

In women without hypertension, I recommend obtaining the same blood tests at the onset of any of the signs and symptoms listed in TABLE 2.

TABLE 2

Conditions that heighten the risk of HELLP

  • Preeclampsia-eclampsia
  • Severe gestational hypertension
  • Early-onset hypertension, or severe intrauterine growth restriction
  • Thrombophilias
  • Abruptio placentae
  • Nonspecific viral-syndrome-like symptoms
  • Right upper quadrant, epigastric, or retrosternal pain
  • Persistent nausea or vomiting in third trimester
  • Bleeding from mucosal surfaces
  • Unexplained hematuria or proteinuria
  • Petechial hemorrhages or ecchymosis

Assessing test results

Clinicians should be familiar with the upper limit for liverenzyme tests in their laboratory. I suggest a cutoff more than twice the upper limit for a particular test.

Also keep in mind that these parameters are dynamic; some women will meet only some of the criteria early in the disease process. Moreover, maternal complications are substantially higher when all 3 components are present than when only 1 or 2 are present.

Look for these clinical findings

Hypertension. Most women with HELLP syndrome have hypertension. In 15% to 50% of cases, the hypertension is mild, but it may be absent in 15%.

Proteinuria. Most patients also have proteinuria by dipstick (≥1+). Proteinuria may be absent in approximately 13% of women with HELLP syndrome, although they will likely have many of the symptoms reported by women with severe preeclampsia.

TABLE 3 lists the signs and symptoms to be expected in these patients, along with their frequency.

TABLE 3

Signs and symptoms

CONDITION

FREQUENCY (%)

Hypertension

85

Proteinuria

87

Right upper quadrant or epigastric pain

40–90

Nausea or vomiting

29–84

Headaches

33–60

Visual changes

10–20

Mucosal bleeding

10

Jaundice

5

The usual times of onset

Antepartum cases. As was previously noted, HELLP syndrome usually develops before delivery, with the most frequent onset being before 37 weeks’ gestation ( TABLE 4).

In the postpartum period, most cases develop within 48 hours after delivery. Of these, approximately 90% occur in women who had antepartum preeclampsia that progressed to HELLP syndrome in the postpartum period. However, approximately 20% of postpartum cases develop more than 48 hours after delivery.

Another important point: HELLP syndrome can develop for the first time postpartum in women who had no evidence of preeclampsia before or during labor. Thus, it is important to educate all postpartum women to report new symptoms (listed in TABLE 3) as soon as possible. When these symptoms develop, evaluate the patient for both preeclampsia and HELLP syndrome.

TABLE 4

Usual times of onset*

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

Risk for life-threatening maternal complications

When all components of HELLP syndrome are present in a woman with preeclampsia, the risk of maternal death and serious maternal morbidities increases substantially (TABLE 5). The rate of these complications depends on gestational age at onset, presence of associated obstetric complications (eclampsia, abruptio placentae, peripartum hemorrhage, or fetal demise) or preexisting conditions (lupus, renal disease, chronic hypertension, or type 1 diabetes).

Abruptio placentae increases the risk of disseminated intravascular coagulopathy (DIC), as well as the need for blood transfusions.

Marked ascites (>1 L) leads to higher rates of cardiopulmonary complications.

TABLE 5

Maternal complications

COMPLICATION

FREQUENCY (%)

Death

1

Adult respiratory distress syndrome

1

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 coagulopathy

10–15

Marked ascites

10–15

Differential diagnosis

When diagnosing HELLP syndrome, confirm or exclude the conditions listed in TABLE 6, since the presenting symptoms and clinical and laboratory findings in women with HELLP syndrome overlap those of several microangiopathic disorders that can develop during pregnancy and/or postpartum. In some women, preeclampsia may be superimposed on one of these disorders, further confounding an already difficult differential diagnosis.

Because of the remarkably similar clinical and laboratory findings of these diseases, make every effort to achieve an accurate diagnosis, since management and outcomes may differ among these conditions.

TABLE 6

Differential diagnosis

  • 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

Initial Management

Hospitalize the patient

Because HELLP syndrome usually is characterized by progressive and sometimes sudden deterioration in maternal and fetal conditions, patients should be hospitalized and observed in a labor and delivery unit.

Initially, assume the patient has severe preeclampsia and treat her with intravenous magnesium sulfate to prevent convulsions and antihypertensive medications as needed to keep systolic blood pressure below 160 mm Hg and diastolic blood pressure 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 diagnosis and check for the presence of DIC, massive hemolysis, severe anemia, or renal failure.

The first priority is to assess the patient for the presence of cardiovascular complications, signs of liver hematoma or hemorrhage, and abruptio placentae. If any is present—particularly hypotension, hypovolemia, DIC, or pulmonary edema—make every effort to stabilize the maternal condition.

Can delivery wait 48 hours for corticosteroids?

Evaluate fetal status by heart rate monitoring or biophysical profile, and confirm gestational age. Then decide whether delivery is indicated or can be delayed for 48 hours so that corticosteroids can be given.

No room for expectant management. Do not consider expectant management in women with true HELLP syndrome. Delivery can only be delayed for a maximum of 48 hours—and only when both mother and fetus are stable, at 24 to 34 weeks’ gestation, and awaiting the benefit of corticosteroids.

Corticosteroid dosing. My practice is to give 2 doses of either betamethasone 12 mg intramuscularly every 12 hours or dexamethasone 12 mg intravenously every 12 hours. This is to improve maternal status, at least temporarily.

Initiate delivery within 24 hours after the last steroid dose, with continuous monitoring in the labor and delivery unit.

Although some women may demonstrate transient improvement in their blood tests (eg, increased platelet count or decreased AST levels), delivery is still indicated. Conversely, in some cases, maternal and fetal conditions may deteriorate, mandating delivery before the 2 doses of steroids are completed.

Delivery Considerations

HELLP syndrome does not justify immediate cesarean

Patients with HELLP syndrome in labor or with rupture of membranes can deliver vaginally in the absence of obstetric complications. In addition, induction or augmentation of labor is acceptable with either oxytocin infusion or prostaglandins if the fetal gestational age is 32 weeks or more and the cervical Bishop score exceeds 5.

TABLE 7 lists the indications for elective cesarean delivery and summarizes management during surgery. It is important to stabilize the maternal condition, correct coagulopathy, and have blood or blood products available before initiating surgery.

TABLE 7

Cesarean delivery: Indications and management

Indications for cesarean

  • Nonreassuring fetal status
  • Abnormal fetal presentation
  • <30 weeks’ gestation and Bishop score <5
  • <32 weeks’ gestation with intrauterine growth 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

Watch for oozing from surgical sites

In a cesarean section, generalized oozing from the surgical site can occur during the operation or immediately postpartum because of the continued drop in platelet count in some of these patients. Thus, it is advisable to insert a subfascial drain and to leave the skin incision open for at least 48 hours to avoid hematoma formation in these areas (FIGURE 1).

FIGURE 1 Insert subfascial drain at cesarean section

Because generalized oozing from the surgical site can occur intraoperatively or immediately postpartum, insert a subfascial drain and leave the skin incision open for at least 48 hours to avoid hematoma formation.

Small doses of systemic opioids are best

For maternal analgesia during labor, give small, intermittent doses of systemic opioids. For repair of episiotomy or vulvar or vaginal lacerations, use local infiltration anesthesia.

Avoid pudendal block because of the potential for bleeding and hematoma formation in this area. Epidural anesthesia may be used after consultation with the anesthesiologist if the platelet count exceeds 75,000/mm3.

Some authors report rising platelet counts after intravenous dexamethasone and, with the improved platelets, greater use of epidural anesthesia, especially in women who achieved a 24-hour latency period before delivery. However, since the platelet count may drop again, insert the epidural catheter once the desired platelet level (with anesthesiologist approval) is reached.

Continued...
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