Hot Case #14 – I’m pregnant … HELLP!

I recently had cause to revise a bit about HELLP syndrome, so I’ve put together a pop quiz for your brain-scratching pleasure

What is the incidence of HELLP syndrome in developed nations?

a) 1 in 10 – 20 pregnancies

b) 1 in 100 – 200 pregnancies

c) 1 in 1000 – 2000 pregnancies

d) 1 in 10000 – 20000 pregnancies

b) 1 in 100 – 200 pregnancies

What is the mortality incidence of HELLP syndrome?

a) 0.01 – 0.02%

b) 0.1% – 0.2%

c) 1 – 2%

d) 10 – 20%

c) 1 – 2%

HELLP syndrome is typically a third trimester condition, which may occur up to 7 days after delivery – True or false?

True

List 6 complications of the HELLP syndrome

Complications of the HELLP syndrome

• Abruptio placentae
• Severe post partum haemorrhage
• Subcapsular liver haematoma
• Liver infarct
• Intracerebral or brainstem haemorrhage
• Cerebral infarct
• Visual impairment due to retinopathy
• Pulmonary oedema – higher risk in post partum onset of HELLP
• Acute kidney injury – higher risk in post partum onset of HELLP
• DIC
• Foetal demise in utero

List 5 differentials for HELLP syndrome

Differentials for HELLP syndrome

• Pre-ecclampsia / ecclampsia
• Acute fatty liver of pregnancy
• HUS
• TTP (rare in pregnancy)
• ITP
• Acute hepatitis

Corticosteroid therapy has demonstrated benefit for promoting foetal lung maturity for deliveries between 26 and 34 weeks of gestation. Are there any maternal benefits for corticosteroid therapy in HELLP syndrome?

It is uncertain as to whether there are any maternal benefits for corticosteroid therapy in HELLP syndrome. There is some weak evidence for shortened duration of thrombocytopaenia and a shorter duration of hospital stay. However, evidence for a mortality benefit or a reduction in the occurrence of complications is mixed to the point that steroids are not currently recommended as a standard therapy when managing HELLP.

While delivering the infant is recognised as the key intervention in managing HELLP syndrome, all efforts should be made to allow the infant to remain in utero until as close as possible to the expected date of delivery – True or false?

False

The critical gestation time here is about 34 weeks. After this time, foetal lung maturity is adequate to proceed with urgent delivery, at which time the mortality risk to the infant is about 8%.

Before 34 weeks of gestation, the mortality risk to the infant during HELLP syndrome is about 34%, so there is an argument for trying to delay delivering the infant until close to 34 weeks or at least until the mother has received 2 doses of either dexamethasone or betamethasone.

However, the longer delivery is delayed, the greater this risk of complications. Furthermore, if the mother’s condition is deteriorating, systolic BP is > 160mmHg, there is DIC or organ impairment such as worsening pulmonary oedema or kidney injury, then delivery should be expedited.

Vaginal delivery, including an induced delivery, is considered preferable to Caesarian section for HELLP syndrome – True or false?

True

Which of the following therapies are not used in the treatment of HELLP?

• Antithrombin
• Glutathione
• Exchange transfusion
• Labetolol
• Octreotide

• Antithrombin – has been trialled with some benefit demonstrated, but has not yet been subjected to any high quality trial
• Glutathione – has been trialled with some benefit demonstrated, but has not yet been subjected to any high quality trial
• Exchange transfusion – considered in situations of progressive elevation of bilirubin or falling Hb or PLTs and ongoing deterioration in maternal condition.
• Labetolol – or hydralizine or nifedipine, as hypertension is often associated
• Octreotide – No role in HELLP syndrome