HELLP (haemolysis, elevated liver enzymes and low platelet count) syndrome is one of the presentations of pre-eclampsia and may occur before or soon after delivery (usually within 48 hours). Severe HELLP syndrome is associated with disseminated intravascular coagulation (DIC) and placental abruption, and may progress to multi-organ failure. It is associated with a high fetal and maternal morbidity and mortality.
Problems and special considerations
The mother with HELLP syndrome may not necessarily have presented with other symptoms or signs of pre-eclampsia. The main presenting feature may be abdominal pain, perhaps with nausea and vomiting, so a high index of suspicion is needed. The first presentation may be a seizure.
Mild changes in liver function tests have been reported in up to 50% of women with preeclampsia, but more serious dysfunction may occur, including periportal haemorrhage and hepatic infarction. There have also been several reported cases of liver rupture associated with severe HELLP syndrome. Acute fatty liver of pregnancy is also considered by many authorities to be part of the spectrum of pre-eclampsia/eclampsia.
Other complications of HELLP syndrome include renal failure, DIC, pulmonary oedema, pleural effusions, acute respiratory distress syndrome, retinal detachment and cerebral oedema. About 16% of women who are undelivered and who develop HELLP syndrome present with placental abruption.
Management options
The treatment of HELLP syndrome is supportive; delivery of the placenta is the definitive treatment.
Women should be delivered as soon as the maternal condition has been optimised, usually by caesarean section. Recommendations vary regarding pre- or perioperative platelet transfusion, with some centres suggesting platelet transfusion if the platelet count is below 50 x 109/l and others not giving platelets until the count falls below 20 x 109/l.
The benefits of invasive pressure monitoring must be balanced against the potential hazards. Careful fluid balance is important, and may be guided with central venous pressure monitoring and urine output. However, many anaesthetists believe that the hazards of central venous lines and their use on the labour ward for fluid balance outweigh the benefits, and there is little evidence in the literature supporting an improved outcome.
Regional anaesthesia is relatively contraindicated in the presence of thrombocytopenia, but ultimately the anaesthetist must choose the anaesthetic technique that he or she judges to be the safest in the circumstances. The choice of regional anaesthesia in a woman with a platelet count of less than 75 x 109/1 should only be made by an experienced consultant obstetric anaesthetist. In women with progressive thrombocytopenia, a platelet count should be checked immediately before performing neuraxial anaesthesia. Thromboelasto- graphy/thromboelastometry has been used to aid management.
If general anaesthesia is used, attempts must be made to attenuate the hypertensive response to intubation, usually by use of an intravenous opioid as part of the induction sequence (see Chapter 86, Hypertension, pre-eclampsia and eclampsia). Tracheal intubation should be carried out as atraumatically as possible, and difficult intubation secondary to airway oedema should be anticipated.
There is no specific treatment for HELLP syndrome other than symptomatic treatment of the associated complications, although there is some evidence to support steroid therapy, e.g. two doses of dexamethasone 10 mg 12 hours apart followed by 5 mg at 24 and 36 hours. Plasma exchange has been anecdotally reported to be useful, although the evidence for it is weak. Magnesium sulfate is indicated for seizure prophylaxis. Women with HELLP syndrome should be managed in a high-dependency or intensive care environment. Postnatal management is entirely supportive.
Key points
• HELLP syndrome is part of the spectrum of pre-eclampsia/eclampsia and may present without prodromal symptoms and signs of pre-eclampsia.
• It may present before or after delivery, and is associated with significant maternal and fetal morbidity and mortality.
• Epigastric pain, nausea and vomiting are common presenting features, and a high index of suspicion is essential in every pregnant woman presenting with abdominal pain.
• Treatment consists of delivery and supportive management of the associated complications.
Further reading
del-Rio-Vellosillo M, Garcia-Medina JJ. Anesthetic considerations in HELLP syndrome. Acta Anesthesiol Scand 2016; 60: 144-57.
Fitzpatrick KE, Hinshaw K, Kurinczuk JJ, Knight M. Risk factors, management, and outcomes of hemolysis, elevated liver enzymes, and low platelets syndrome and elevated liver enzymes, low platelets syndrome. Obstet Gynecol 2014; 123: 618-27.
Haram K, Svendsen E, Abildgaard U. The HELLP syndrome: clinical issues and management.
A review. BMC Pregnancy Childbirth 2009; 9: 8.