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OBGMANAGEMENT BEST PRACTICES FOR DIAGNOSIS AND MANAGEMENT OF PREECLAMPSIA

Baha M. Sibai, MD A practical plan to detect Professor and Chairman Department of and Gynecology and manage HELLP syndrome University of Cincinnati College of Medicine 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 .

ere’s a disturbing fact: If it looks ❚ Pinning HELLP down like HELLP syndrome, and One of the best tools to identify HELLP H impairs the patient like HELLP syndrome is a healthy dose of suspicion, syndrome, it isn’t necessarily HELLP syn- since it can affect any pregnant woman at drome. A plethora of diagnostic criteria any time: antepartum, intrapartum, or from different investigators over the years within 1 week postpartum. Approximately has confused the issue of what constitutes 72% of cases are diagnosed before deliv- this syndrome—not to mention how to ery, and the rest are diagnosed during the IN THIS ARTICLE manage it. first week postpartum. A management issue has also attract- Weinstein noted that the signs and ❙ Hallmarks ed recent attention: use of symptoms of HELLP syndrome can occur of HELLP either antepartum to enhance maternal without clinical evidence of severe Page 55 status so that epidural anesthesia can be preeclampsia (severe and/or administered, or postpartum to improve severe ). Indeed, he reported ❙ Conditions that . Such improvements are only that hypertension can be mild or absent in transient, however, and we lack definitive most patients with HELLP, and protein- heighten risk data on the benefits. uria can be mild. Page 56 One thing is certain, however. The Weinstein coined the term HELLP syn- combination of , liver dysfunc- drome in 1982 to describe these abnormal- ❙ Cesarean delivery: tion or injury, and consumption in ities in women with preeclampsia: Indications women with preeclampsia makes adverse H = hemolysis and management maternal and perinatal outcomes more EL = elevated liver enzymes Page 63 likely and leaves no room for expectant LP = low platelets management. Another obstacle to early detection: ❙ HELLP syndrome also has become a Patients may have nonspecific signs and Rare but major issue in litigation against obstetri- symptoms, none of which are diagnostic of life-threatening: cians and medical and surgical consult- classical preeclampsia. Subcapsular ants. Lawsuits usually allege misdiag- However, HELLP syndrome is most liver nosed preeclampsia, delayed delivery, or common in women who have already been Page 64 improper recognition and management of diagnosed with complications. and/or preeclampsia. CONTINUED

52 OBG MANAGEMENT • April 2005 A practical plan to detect and manage HELLP syndrome ▲

TABLE 1 Hallmarks of HELLP: Hemolysis, elevated liver enzymes, and low platelets

Hemolysis Diagnosis requires at least 2 of the following:

■ Abnormal peripheral smear (, burr cells)

■ Elevated serum (≥1.2 mg/dL)

■ Low serum

■ Significant drop in levels, unrelated to loss Elevated liver enzymes

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

■ at least twice the upper level of normal. This value is also elevated in severe hemolysis Low platelets

■ <100,000/mm3

IMAGE: MAURA FLYNN

HELLP is more likely When to begin testing with severe hypertension In women with new-onset hypertension, Overall, the incidence of HELLP syn- order a complete blood count with platelets drome in women with gestational hyper- and liver enzyme analysis at the time of diag- FAST TRACK tension/preeclampsia increases with the nosis and serially thereafter. The frequency of HELLP syndrome severity of the condition. HELLP syn- these tests depends on the initial test results, drome also is more likely in women with severity of disease, and onset of symptoms. is more likely early-onset hypertension/preeclampsia In women without hypertension, I rec- when hypertension (before 34 weeks’ gestation). ommend obtaining the same blood tests at or preeclampsia the onset of any of the is diagnosed listed in TABLE 2. before 34 weeks ❚ Making the diagnosis Assessing test results HELLP syndrome is diagnosed when all 3 Clinicians should be familiar with the of the following are present: upper limit for liver-enzyme tests in their • Hemolysis, defined as the presence of laboratory. I suggest a cutoff more than microangiopathic . twice the upper limit for a particular test. This is the hallmark of the triad. Also keep in mind that these parame- • Elevated liver enzymes (either aspar- ters are dynamic; some women will meet tate aminotransferase [AST] or alanine only some of the criteria early in the disease aminotransferase [ALT]). This compo- process. Moreover, maternal complications are nent signifies liver cell ischemia and/or substantially higher when all 3 components . are present than when only 1 or 2 are present. • Low platelet count (<100,000/mm3). TABLE 1 summarizes the laboratory Look for these clinical findings criteria for the diagnosis. Hypertension. Most women with HELLP

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TABLE 2 The usual times of onset Conditions that heighten Antepartum cases. As was previously the risk of HELLP noted, HELLP syndrome usually develops before delivery, with the most frequent • Preeclampsia- onset being before 37 weeks’ gestation Early onset (TABLE 4). During expectant management In the postpartum period, most cases devel- • Severe gestational hypertension op within 48 hours after delivery. Of these, • Early-onset hypertension, or severe approximately 90% occur in women who intrauterine growth restriction had antepartum preeclampsia that pro- • Thrombophilias gressed to HELLP syndrome in the post- • Abruptio placentae partum period. However, approximately • Nonspecific viral-syndrome-like 20% of postpartum cases develop more symptoms than 48 hours after delivery. • Right upper quadrant, epigastric, or Another important point: HELLP syn- retrosternal pain drome can develop for the first time post- • Persistent or in third partum in women who had no evidence of trimester preeclampsia before or during labor. Thus, • Bleeding from mucosal surfaces it is important to educate all postpartum • Unexplained hematuria or proteinuria women to report new symptoms (listed in TABLE 3) as soon as possible. When these • Petechial hemorrhages or ecchymosis symptoms develop, evaluate the patient for both preeclampsia and HELLP syndrome. TABLE 3 Signs and symptoms Risk for life-threatening maternal complications CONDITION FREQUENCY (%) When all components of HELLP syndrome Hypertension 85 are present in a woman with preeclampsia, FAST TRACK Proteinuria 87 the risk of and serious HELLP syndrome Right upper quadrant 40–90 maternal morbidities increases substantial- or epigastric pain ly (TABLE 5). The rate of these complica- sometimes appears Nausea or vomiting 29–84 tions depends on gestational age at onset, for the first time 33–60 presence of associated obstetric complica- postpartum Visual changes 10–20 tions (eclampsia, abruptio placentae, peri- Mucosal bleeding 10 partum hemorrhage, or fetal demise) or in women who had preexisting conditions (lupus, renal disease, Jaundice 5 no evidence chronic hypertension, or type 1 diabetes). of preeclampsia Abruptio placentae increases the risk of before or during syndrome have hypertension. In 15% to disseminated intravascular 50% of cases, the hypertension is mild, but (DIC), as well as the need for blood trans- labor it may be absent in 15%. fusions. Proteinuria. Most patients also have pro- Marked ascites (>1 L) leads to higher rates teinuria by dipstick (≥1+). Proteinuria may of cardiopulmonary complications. be absent in approximately 13% of women with HELLP syndrome, although they will likely have many of the symp- When diagnosing HELLP syndrome, con- toms reported by women with severe firm or exclude the conditions listed in preeclampsia. TABLE 6, since the presenting symptoms TABLE 3 lists the signs and symptoms and clinical and laboratory findings in to be expected in these patients, along with women with HELLP syndrome overlap their frequency. those of several microangiopathic disor-

56 OBG MANAGEMENT • April 2005 ▲ A practical plan to detect and manage HELLP syndrome

sudden deterioration in maternal and fetal TABLE 4 conditions, patients should be hospitalized Usual times of onset* and observed in a labor and delivery unit. Initially, assume the patient has severe RELATION TO DELIVERY PERCENTAGE preeclampsia and treat her with intra- Antepartum 72 venous magnesium sulfate to prevent con- Postpartum 28 vulsions and antihypertensive medications ≤48 hours 80 as needed to keep systolic >48 hours 20 below 160 mm Hg and diastolic blood GESTATIONAL AGE (WEEKS) PERCENTAGE pressure below 105 mm Hg. 17–20 2 Blood tests should include: • complete blood count with platelet count, 21–27 10 • peripheral smear evaluation, 28–36 68 • serum AST, >37 20 • lactate dehydrogenase, * Based on 700 cases • creatinine, • bilirubin, and TABLE 5 • studies. Maternal complications These tests help confirm the diagnosis and check for the presence of DIC, massive FREQUENCY (%) hemolysis, severe anemia, or renal failure. Death 1 The first priority is to assess the patient Adult respiratory 1 for the presence of cardiovascular complica- distress syndrome tions, signs of liver hematoma or hemor- Laryngeal 1–2 rhage, and abruptio placentae. If any is pres- or hemorrhage 1–2 ent—particularly hypotension, hypovolemia, Acute renal failure 5–8 DIC, or —make every Pulmonary edema 6–8 effort to stabilize the maternal condition. Pleural effusions 6–10 Can delivery wait 48 hours FAST TRACK Abruptio placentae 10–15 for corticosteroids? In women Disseminated intravascular 10–15 coagulopathy Evaluate fetal status by heart rate monitor- with true HELLP, ing or biophysical profile, and confirm ges- Marked ascites 10–15 delivery can only tational age. Then decide whether delivery be delayed is indicated or can be delayed for 48 hours ders that can develop during so that corticosteroids can be given. for up to 48 hours and/or postpartum. In some women, No room for expectant management. Do not for preeclampsia may be superimposed on one consider expectant management in women administration— of these disorders, further confounding an with true HELLP syndrome. Delivery can and then only if already difficult differential diagnosis. only be delayed for a maximum of 48 Because of the remarkably similar clin- hours—and only when both mother and both mother ical and laboratory findings of these dis- are stable, at 24 to 34 weeks’ gestation, and fetus are stable eases, make every effort to achieve an accu- and awaiting the benefit of corticosteroids. rate diagnosis, since management and out- Corticosteroid dosing. My practice is to comes may differ among these conditions. give 2 doses of either betamethasone 12 mg intramuscularly every 12 hours or dexamethasone 12 mg intravenously every 12 hours. This is to improve mater- ❚ Initial management nal status, at least temporarily. Hospitalize the patient Initiate delivery within 24 hours after Because HELLP syndrome usually is char- the last steroid dose, with continuous acterized by progressive and sometimes monitoring in the labor and delivery unit. CONTINUED

58 OBG MANAGEMENT • April 2005 A practical plan to detect and manage HELLP syndrome ▲

Although some women may demon- TABLE 6 strate transient improvement in their Differential diagnosis blood tests (eg, increased platelet count or decreased AST levels), delivery is still indi- • Acute fatty liver of pregnancy cated. Conversely, in some cases, maternal • Acute pancreatitis and fetal conditions may deteriorate, man- • Antiphospholipid syndrome dating delivery before the 2 doses of • Cholecystitis steroids are completed. • Disseminated herpes simplex • Fulminant • Hemolytic uremic syndrome ❚ Delivery considerations • Hemorrhagic or septic shock HELLP syndrome does not justify • Immune thrombocytopenic purpura immediate cesarean • Stroke Patients with HELLP syndrome in labor or • Systemic lupus erythematosus with rupture of membranes can deliver • Thrombotic thrombocytopenic purpura vaginally in the absence of obstetric com- plications. In addition, induction or aug- mentation of labor is acceptable with either TABLE 7 oxytocin infusion or prostaglandins if the Cesarean delivery: fetal gestational age is 32 weeks or more Indications and management and the cervical Bishop score exceeds 5. Indications for cesarean TABLE 7 lists the indications for elec- tive cesarean delivery and summarizes • Nonreassuring fetal status management during surgery. It is important • Abnormal fetal presentation to stabilize the maternal condition, correct • <30 weeks’ gestation and Bishop score <5 coagulopathy, and have blood or blood • <32 weeks’ gestation with intrauterine products available before initiating surgery. growth restriction or and Bishop score <5 FAST TRACK Watch for oozing from surgical sites • Known subcapsular liver hematoma Avoid In a cesarean section, generalized oozing • Suspected abruptio placentae from the surgical site can occur during the Management during cesarean pudendal block operation or immediately postpartum • General anesthesia for platelet because of the risk because of the continued drop in platelet count <75,000/mm3 for bleeding count in some of these patients. Thus, it is • Transfuse 6 units of platelets if and hematoma advisable to insert a subfascial drain and count <40,000/mm3 to leave the skin incision open for at least • Insert subfascial drain formation 48 hours to avoid hematoma formation in • Secondary skin closure or leave in this area these areas (FIGURE 1). subcutaneous drain • Observe for bleeding from Small doses of systemic upper abdomen prior to closure opioids are best For maternal analgesia during labor, give small, intermittent doses of systemic opi- anesthesiologist if the platelet count oids. For repair of episiotomy or vulvar or exceeds 75,000/mm3. vaginal lacerations, use local infiltration Some authors report rising platelet anesthesia. counts after intravenous dexamethasone Avoid pudendal block because of the and, with the improved platelets, greater use potential for bleeding and hematoma for- of epidural anesthesia, especially in women mation in this area. Epidural anesthesia who achieved a 24-hour latency period may be used after consultation with the before delivery. However, since the platelet

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FIGURE 1 Early signs and symptoms Insert subfascial drain Liver can develop antepartum, at cesarean section during labor, or in the postpartum period. Presenting symptoms may include severe epigastric or retrosternal pain in associa- tion with breathing difficulty (pain on inspiration), with or without shoulder or neck pain. When profound hypovolemic shock occurs in a previously hypertensive patient, suspect rupture of a liver hematoma. Diagnosis can be made by ultrasound or computed tomography (CT) imaging of the liver, both of which can also confirm Because generalized oozing from the surgical site intraperitoneal bleeding. can occur intraoperatively or immediately postpar- tum, insert a subfascial drain and leave the skin In most cases, rupture involves the incision open for at least 48 hours to avoid right lobe of the liver and is preceded by a hematoma formation. parenchymal liver hematoma.

FIGURE 2 Mortality can exceed 50% Rare but life-threatening: Maternal and fetal mortality increase sub- Subcapsular liver hematoma stantially when a subcapsular liver hematoma is present. In fact, mortality may exceed 50% when frank rupture of the capsule involves liver tissue.

Choose conservative management whenever possible FAST TRACK Management of subcapsular liver Profound hematoma depends on maternal hemody- namic status, integrity of the capsule (rup- hypovolemic shock tured or intact), and the fetal condition. in a previously Conservative management is prefer- hypertensive Liver hematomas can develop antepartum, able in hemodynamically stable women intrapartum, or postpartum. Presenting symptoms with an unruptured hematoma. It consists patient may be may include severe epigastric or retrosternal pain of close monitoring of the patient’s hemo- in association with respiratory difficulty (pain on a sign of rupture inspiration), with or without shoulder or neck pain. dynamic and coagulation status and serial of a liver hematoma assessment of the hematoma with ultra- sound or CT scan. count may drop again, insert the epidural Avoid exogenous trauma to the liver, such catheter once the desired platelet level (with as frequent abdominal palpation, emesis, anesthesiologist approval) is reached. or convulsions. Any sudden increase in intraabdominal pressure can led to rupture of the hematoma.

❚ Suspected liver hematoma When rupture occurs A rare and potentially life-threatening This surgical emergency requires an acute complication of HELLP syndrome is sub- multidisciplinary team, including an capsular liver hematoma (FIGURE 2). Ob/Gyn, anesthesiologist, highly qualified Unfortunately, the rarity of this complica- surgeon, and a representative of the hospi- tion sometimes causes it to be overlooked. tal’s blood bank.

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64 OBG MANAGEMENT • April 2005 ▲ A practical plan to detect and manage HELLP syndrome

Maternal resuscitation should include: pletely recover within 72 hours, while oth- • transfusion of packed red blood cells ers deteriorate further or fail to recover for to maintain blood pressure and tissue as long as 1 week after delivery. Thus, perfusion, some women may require intensive moni- • correction of coagulopathy with fresh toring for several days because of the risk frozen plasma and platelets, and of pulmonary edema, renal failure, or • laparotomy, preferably using a cell adult respiratory distress syndrome. saver. Keep in mind that, in some of these Options at laparotomy include: women, the cause of the postpartum dete- • packing and drainage (preferred), rioration may be something other than • ligation of the hepatic lacerations, HELLP syndrome (TABLE 6). • embolization of the hepatic artery to the affected liver segment, and Watch for sudden hypotension • loosely suturing omentum or surgical A sudden drop in blood pressure to mesh to the liver surface. hypotensive levels can be an early sign of severe hemolysis or unrecognized intraperitoneal blood loss (from surgical sites or ruptured liver hematoma), as well ❚ Postpartum care as sepsis. In women who develop HELLP prior to In a woman with severe hemoconcen- delivery, closely monitor postpartum vital tration (ie, severe vasoconstriction), sud- signs, intake and output, and symptoms in den hypotension also may indicate exces- intensive care or a similar facility for at sive vasodilation from antihypertensive least 48 hours. drugs such as hydralazine or nifedipine, During this time, my practice is to give resulting in relative hypovolemia. the patient intravenous magnesium sulfate Such a case requires volume resuscita- and antihypertensive medications as need- tion, (if indicated), and ed to keep systolic blood pressure below evaluation for unrecognized bleeding. FAST TRACK 155 mm Hg (the standard is 160 mm Hg) A sudden drop and diastolic blood pressure below 105 Use of steroids mm Hg. Some authors recommend giving intra- in blood pressure The rationale for this treatment is to venous dexamethasone (5 to 10 mg every to hypotensive prevent bleeding in the brain if the woman 12 hours) for approximately 48 hours after levels can signify has . delivery in women who develop antepar- tum or postpartum HELLP. They claim this severe hemolysis, When HELLP appears treatment improves maternal blood tests, unrecognized in the postpartum period shortens recovery, and reduces maternal intraperitoneal Several maternal complications from morbidity. blood loss, HELLP syndrome may not appear until However, at present, no data indicate immediately postpartum. Thus, all this approach has clinical benefit—and the or sepsis women with preeclampsia require close risks are unknown. For these reasons, monitoring of vital signs, fluid intake and treatment with intravenous dexametha- output, laboratory values, and pulse sone after delivery remains empiric. ■ oximetry for at least 48 hours. Also continue magnesium sulfate in the postpartum period and keep maternal BIBLIOGRAPHY Abramovici D, Friedman SA, Mercer BM, Audibert F, Kao L, blood pressure below 155 mm Hg systolic Sibai BM. Neonatal outcome in severe preeclampsia at 24 and 105 mm Hg diastolic. to 36 weeks’ gestation. Does HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome matter? Am J Obstet Gynecol. 1999;180:221–225. Time to recovery Audibert F, Friedman SA, Frangieh AY, Sibai BM. Clinical Most patients begin to improve or com- utility of strict diagnostic criteria for the HELLP (hemolysis,

66 OBG MANAGEMENT • April 2005 A practical plan to detect and manage HELLP syndrome ▲

elevated liver enzymes, and low platelets) syndrome. Am J Friedman SA. Maternal morbidity and mortality in 442 Obstet Gynecol. 1996;175:460–464. with hemolysis, elevated liver enzymes, and Egerman RS, Sibai BM. Recognizing and managing HELLP low platelets (HELLP syndrome). Am J Obstet Gynecol. syndrome and its imitators. Contemporary Ob/Gyn. 1997; 1993;169:1000–1006. (October):129–149. Sibai BM. The HELLP syndrome (hemolysis, elevated liver Magann EF, Perry KG Jr, Meydrech EF, Harris RL, Chauchan enzymes, and low platelets): much ado about nothing? Am SP, Martin JN Jr. Postpartum corticosteroids: accelerated J Obstet Gynecol. 1990;162:311–316. recovery from the syndrome of hemolysis, elevated liver Sibai BM. Diagnosis, controversies, and management of enzymes, and low platelets (HELLP). Am J Obstet Gynecol. HELLP syndrome. Obstet Gynecol. 2004;103:981–991. 1994;171:1154–1158. Tompkins MJ, Thiagarajah S. HELLP (hemolysis, elevated Martin Jr JN, Thigsen BD, Rose CH, et al. Maternal benefit liver enzymes, and low platelet count) syndrome: the bene- of high-dose intravenous corticosteroid therapy for HELLP. fit of corticosteroids. Am J Obstet Gynecol. 1999; Am J Obstet Gynecol. 2003;189:830–834. 181:304–309. O’Brien JM, Milligan DA, Barton JR. Impact of high-dose VanPampus MG, Wolf H, et al. Maternal and perinatal out- corticosteroid therapy for patients with HELLP (hemolysis, come after expectant management of the HELLP syndrome elevated liver enzymes, and low platelet count) syndrome. compared with preeclampsia without HELLP syndrome. Am J Obstet Gynecol. 2000;183:921–924. Eur J Obstet Gynecol Reprod Biol. 1998;76:31. O’Brien JM, Shumate SA, Satchwell SL, Milligan DA, Weinstein L. Syndrome of hemolysis, elevated liver enzymes, Barton JR. Maternal benefit to corticosteroid therapy in and low platelet count: A severe consequence of hyperten- patients with HELLP (hemolysis, elevated liver enzymes, sion in pregnancy. Am J Obstet Gynecol. 1982;142:159–167. and low platelets) syndrome: impact on the rate of region- Weinstein L. Preeclampsia/eclampsia with hemolysis, ele- al anesthesia. Am J Obstet Gynecol. 2002;186:475–479. vated liver enzymes and thrombocytopenia. Obstet Rinehart BK, Terrone DA, Magann EF, Martin RW, May WL, Gynecol. 1985;66:657–660. Martin JN Jr. Preeclampsia-associated hepatic hemorrhage and rupture: mode of management related to maternal and perinatal outcome. Obstet Gynecol Surv. 1999;196–202. The author reports no financial relationships relevant to this article. Sibai BM, Ramadan MK, Usta I, Salama M, Mercer BM,

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