Clinical opinion
Obstetrics
Diagnosis and management of atypical preeclampsia-eclampsia

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Preeclampsia, eclampsia, and hemolysis, elevated liver enzymes, and low platelets syndrome are major obstetric disorders that are associated with substantial maternal and perinatal morbidities. As a result, it is important that clinicians make timely and accurate diagnoses to prevent adverse maternal and perinatal outcomes associated with these syndromes. In general, most women will have a classic presentation of preeclampsia (hypertension and proteinuria) at > 20 weeks of gestation and/or < 48 hours after delivery. However, recent studies have suggested that some women will experience preeclampsia without ≥ 1 of these classic findings and/or outside of these time periods. Atypical cases are those that develop at < 20 weeks of gestation and > 48 hours after delivery and that have some of the signs and symptoms of preeclampsia without the usual hypertension or proteinuria. The purpose of this review was to increase awareness of the nonclassic and atypical features of preeclampsia-eclampsia. In addition, a stepwise approach toward diagnosis and treatment of patients with these atypical features is described.

Section snippets

Definition of classic preeclampsia

The so-called classic triad of preeclampsia includes hypertension, proteinuria, and edema. However, there is now general agreement that edema should not be considered as part of the diagnosis of preeclampsia.1, 2, 3, 4, 5, 6 Indeed, edema is neither sufficient nor necessary to confirm the diagnosis of preeclampsia, because edema is a common finding in normal pregnancy, and approximately one-third of women with eclampsia never demonstrate the presence of edema.7

Hypertension is defined as

Gestational hypertension without proteinuria

The pathophysiologic abnormalities in preeclampsia are viable and can manifest as either 1 organ or multiorgan dysfunction. As a result, the signs and symptoms will reflect the organs involved. Proteinuria in preeclampsia is a manifestation of renal involvement that results from glomerulo endothelial injury (altered permeability to proteins) and abnormal tubular handling of filtered proteins. Traditionally, proteinuria was considered the hallmark for the diagnosis of preeclampsia, because it

Capillary leak syndrome: facial edema, ascites and pulmonary edema, gestational proteinuria

Hypertension is considered to be the hallmark for the diagnosis of preeclampsia; however, recent evidence suggests that, in some patients with preeclampsia, the disease may manifest itself in the form of either a capillary leak (proteinuria, ascites, pulmonary edema), excessive weight gain, or a spectrum of abnormal hemostasis with multiorgan dysfunction (Figure 1).5, 8, 18 These patients usually experience clinical manifestations of atypical preeclampsia (ie, proteinuria with or without facial

Gestational proteinuria

Gestational proteinuria is defined as urinary protein excretion of ≥ 300 mg/24-hour timed collection or persistent proteinuria (≥ 1+ on dipstick on at least 2 occasions at least 4 hours apart but no more than 1 week apart).19, 20 The exact incidence of gestational proteinuria is unknown. Two prospective studies in healthy nulliparous women found that approximately 4% of women who remained normotensive had gestational proteinuria; however, neither of these studies reported the percentage of

Preeclampsia-eclampsia at < 20 weeks of gestation

Preeclampsia and/or eclampsia that occurs at < 20 weeks of gestation has been reported with molar or hydropic degeneration of the placenta with or without a coexistent fetus.7, 8, 9, 25, 26, 27 Additionally, although exceedingly rare, preeclampsia-eclampsia can occur during the first half of pregnancy without molar degeneration of the placenta.9, 28, 29, 30 On the other hand, the presence of hypertension, proteinuria, and abnormal laboratory tests at < 20 weeks of gestation may be due to lupus

Late postpartum preeclampsia-eclampsia and HELLP syndrome

Late postpartum preeclampsia-eclampsia is defined as the development of signs and symptoms of preeclampsia-eclampsia for the first time at > 48 hours but < 4 weeks after delivery.34, 35 Historically, preeclampsia and eclampsia were believed to occur only < 48 hours from delivery. However, several reports have confirmed the existence of late postpartum preeclampsia-eclampsia.7, 34, 35, 36, 37, 38, 39, 40, 41 Table 4 provides a summary of the incidence of postpartum eclampsia in the last 2

Comment

Preeclampsia is a syndrome that is characterized by heterogenous clinical and laboratory findings for which the pathogenesis can differ. The traditional teaching states that preeclampsia is defined as hypertension plus proteinuria that develops at > 20 weeks of gestation and/or within 48 hours after delivery. However, based on our experience and review of literature, we suggest that the aforementioned criteria for defining preeclampsia should be revisited, because preeclampsia, like many other

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    Cite this article as: Sibai BM, Stella CL. Diagnosis and management of atypical preeclampsia-eclampsia. Am J Obstet Gynecol 2009;200:481.e1-481.e7.

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