Muna Tahlak, MD, FACOG Latifa Hospital

Objectives
Update on the disease
focus on diagnosis Complications

timing and mode of delivery

mortality and morbidity controversial aspects of corticosteroid use

Latifa Hospital is a tertiary center on average 6000 deliveries per year >80% high risk obstetric care 465 pregnant women had hypertensive disorder (8%)

Causes of maternal death in developed countries

Other direct causes of deaths Hypertensive disorders Embolism 21.3 16.1 14.9

Haemorrhage Abortion
Ectopic pregnancy Unclassified deaths Sepsis/infection

13.4 8.2
4.9 4.8 2.1

WHO data on maternal mortality

Team Work

HELLP Syndrome
Weinstein regarded signs and symptoms to constitute

an entity separate from severe preeclampsia and in 1982 named the condition HELLP H = Haemolysis EL = Elevated Liver enzymes LP = Low Platelets currently regarded as a variant of severe preeclampsia or a complication .

 The HELLP syndrome occurs in about 0.5 to 0.9% of

all pregnancies and in 10 to 20% of cases with severe preeclampsia

 70% of the cases develops before delivery with a peak

frequency between the 27th and 37th gestational weeks 10% occur before the 27th week 20% beyond the 37th gestational week

HELLP syndrome usually develops within the first 48 hours in women who have had proteinuria and hypertension prior to delivery

hypertension and proteinuria absent in 1020% of the cases

Symptoms
right upper abdominal quadrant
epigastric pain nausea and vomiting

3060% of women have headache

20% visual symptoms partial HELLP syndrome fewer symptoms less complications

Reported frequency of signs and symptoms of HELLP syndrome

Sign/symptom Frequency, percent

Proteinuria
Hypertension Right upper quadrant/epigastrict pain Nausea, vomiting Headache Visual changes Jaundice

86 to 100
82 to 88 40 to 90 29 to 84 33 to 61 10 to 20 5

Haemolysis, one of the major characteristics of the disorder, is due to a microangiopathic haemolytic anaemia

Normal peripheral blood smear

Microangiopathic smear

H(hemolysis)
high LDH concentration
unconjugated bilirubin low or undetectable haptoglobin concentration is a

more specific indicator. Low haptoglobin concentration (< 1 g/L < 0.4 g/L)

Elevated Liver enzymes(EL)

Elevation of liver enzymes may reflect the haemolytic

process as well as liver involvement. Haemolysis contributes to the elevated levels of LDH enhanced asparate aminotransferase (AST) and alanine aminotransferase (ALAT) levels are mostly due to liver injury

Low platelet(LP)
Thrombocytopenia < 150109/L)
caused by gestational thrombocytopenia (GT) (59%) immune thrombocytopenic purpura (ITP) (11%)

preeclampsia (10%)
HELLP syndrome (12%). PLTs < 100109/L are relatively rare in preeclampsia and

gestational thrombocytopenia, frequent in ITP and obligatory in the HELLP syndrome (according to the Sibai definition)

Diagnosis
Many different criteria
Biochemical markers

Clinical
Preeclampsia ELLP

Diagnostic criteria
two major definitions for diagnosing the HELLP

syndrome

Professor Baha Sibai

Professor and Chairman of the Department of Obstetrics and Gynecology at the University of Cincinnati College of Medicine leading authority in the care and treatment of women with preeclampsia and eclampsia, has published more than 500 peer-reviewed articles

Tennessee Classification System

Platelets 100109/L
AST 70 IU/L LDH 600 IU/L

Sibai has proposed strict criteria for "true" or

"complete" HELLP syndrome Intravascular haemolysis is diagnosed by abnormal peripheral blood smear, increased serum bilirubin ( 20.5 mol/L or 1.2 mg/100 mL) and elevated LDH levels (> 600 units/L (U/L)

Mississippi classification Class 1

Platelets 50109/L
AST 70 IU/LAST LDH 600 IU/L

Mississippi classification Class 2

Platelets 100109/L

50109/L AST or ALT 70 IU/L LDH 600 IU/L

Mississippi classification Class 3

Platelets 150109/L

100109/L AST or ALT 40 IU/L LDH 600 IU/L

Differential diagnosis
viral hepatitis
cholangitis and other acute disease ITP

acute fatty liver of pregnancy (AFLP)

haemolytic uremic syndrome (HUS) thrombotic thrombocytopenic purpura (TTP) systemic lupus erythematosus (SLE)

Complications reported in the HELLP syndrome

Maternal complications Eclampsia Abruptio placentae Occurrence (%) 49 920

DIC Acute renal failure

Severe ascites Cerebral oedema Pulmonary oedema

556 736
411 18 310

Complications reported in the HELLP syndrome

Maternal complications Subcapsular liver hematoma Liver rupture Occurrence (%) Between 0.9% and <2% >200 cases or about 1.8%

Cerebral infarction Cerebral Haemorrhage

Maternal death Wound hematoma/infection2 Retinal detachment

Few case reports 1.540

125 714 1

Maternal Mortality
Stroke Cardiac Arrest DIC ARDS Renal failure Sepsis Hepatic Rupture 45% 40% 39% 27% 27% 24% 20%

Hypoxic encephalopathy 15% Contributing factors to deaths in 54 women with HELLP syndrome From Isler and co-authors,1999

Complications reported in the HELLP syndrome

Foetal/neonatal complications Perinatal death IUGR 7.434 3861

Preterm delivery Neonatal thrombocytopenia

RDS

70 (15% < 28 gestational weeks) 1550

5.740

Management of pregnant women with HELLP syndrome

Immediate delivery
> 34 weeks' gestation or later Nonreassuring tests of fetal status

Presence of severe maternal disease: multiorgan

dysfunction, DIC, liver infarction or hemorrhage, renal failure, or abruptio placenta

 27 to 34 weeks of gestation
Delivery within 48 hours evaluation

stabilization
steroid treatment for fetal lung maturity

Steroid use
no clear evidence of any effect of corticosteroids on

substantive clinical outcomes. insufficient evidence for the routine use The use of corticosteroids only to increase rate of recovery in platelet count if considered clinically worthwhile.
Cochrane Review of 11 trials comparing corticosteroids

with placebo/no treatment

 before 24 weeks' gestation, termination of pregnancy

should be strongly considered

Method of Delivery
Vaginal
Cesarean section

Anesthesia Choice
According to ACOG
Regional anesthesia is preferred for women with

preeclapmsia and eclampsia General anesthesia carries more risk than regional

Anesthesia Choice
What platelet count is adequate for regional

anesthesia? No absolute answer Platelet counts >100,000/ul are acceptable to most anesthesiologists Platelet counts in 50,000-100,000 range are potential candidates according to ACOG

 Risk of spinal or epidural hematoma

Paralysis

Frederick P. Zuspan, M.D. 19222009

An internationally recognized authority in the field of maternal-fetal medicine An expert on preeclampsia In the 1960s, Zuspan pioneered the use intravenous magnesium sulfate to prevent convulsions in women with preeclampsia. His treatment protocol was adopted internationally and is still used to treat preeclampsia nearly 50 years later

 there's an empty plaque at Chicago's famous Lying-in

Hospital waiting for the engraved name of the person who discoveres the cause.

Summary
HELLP syndrome is unique to pregnancy
HELLP syndrome develops in approximately 1 of 1000

pregnancies overall and 10 to 20 percent of pregnancies with severe preeclampsia/eclampsia Delivery and supportive management is cure Multidisciplinary approach Tertiary center

Summary
outcome for mothers with HELLP syndrome is

generally good, but serious complications can occur Recommendations are against giving dexamethasone for treatment