A 36-year-old woman, weighing 110 kg and gravida 3, para 2, presented to the labor and delivery unit at 34 weeks gestation with epigastric pain radiating to the right shoulder, severe nausea and vomiting, headache and thrombocytopenia.
The patient was diagnosed with HELLP syndrome (hemolysis, elevated liver enzymes and low platelet count). It was decided to proceed with cesarean delivery. An arterial line and two large-bore peripheral IV lines were placed. General anesthesia was induced with propofol 200 mg and succinylcholine 200 mg. Remifentanil 60 mcg IV was administered to blunt hemodynamic response at induction. A 6.5-mm endotracheal tube was inserted using a Macintosh laryngoscope on the first attempt. Anesthesia was maintained with 50:50 nitrous oxide and oxygen with sevoflurane to a minimum alveolar concentration of 0.7.
After delivery of a boy with APGAR scores of 7 and 9, fentanyl 100 mcg, rocuronium 20 mg and oxytocin 5 IU were administered. Approximately 15 minutes after delivery, severe postpartum bleeding was noted. A massive transfusion protocol was activated with red blood cell units and fresh frozen plasma in a 1:1 ratio, together with platelets to correct thrombocytopenia. The estimated blood loss was approximately 3 L. The patient received 6 units of red blood cells, 6 units of fresh frozen plasma, 4 units of platelets, 2,000 mL of Ringer’s lactate and 1,000 mL of 0.9% saline. At the end of the procedure, the patient was transported intubated to the ICU. The next day, the patient was extubated and laboratory values had significantly improved.
A life-threatening, multisystem complication of pregnancy, HELLP syndrome has a reported incidence of 0.5% to 0.9% among all pregnancies.1 Approximately 1% to 20% of patients who present with preeclampsia with severe features develop HELLP syndrome.2 It is not clear whether HELLP is a distinct entity or a severe subtype of preeclampsia. There are various diagnostic criteria to define HELLP syndrome, which can be classified as “true/complete” if all of the following are present:
|Table 1. Laboratory Diagnostic Signs of HELLP Syndrome|
|Elevated liver enzymes||
HELLP syndrome is “partial/incomplete” when only some of these criteria are met.3 HELLP syndrome usually develops in the antepartum period; however, onset in the immediate postpartum period can occur in 15% to 20% of patients.3,4 Clinical symptoms are epigastric or right upper quadrant pain, shoulder pain, malaise, flu-like symptoms, nausea and vomiting, headache and edema.
Hypertension may be absent in 12% to 18% or mild in 15% to 50% of patients, and proteinuria is absent in approximately 15% of patients.1,5 The differential diagnosis is very broad and includes acute fatty liver of pregnancy, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, idiopathic thrombocytopenia, systemic lupus erythematosus, cholecystitis, cholangitis and appendicitis.1,3,6
Complications associated with HELLP syndrome are multisystemic and affect hematologic, cardiovascular, cerebral, gastrointestinal and renal systems.1,3,7,8 Because of the multisystemic nature of the syndrome, these patients should be overseen by a specialized multidisciplinary team in a tertiary care center.
|Table 2. Serious Complications Associated With HELLP Syndrome|
|Acute renal failure|
|Acute respiratory distress syndrome|
|Disseminated intravascular coagulopathy|
|Subcapsular liver hematoma|
|Based on references 1, 3, 7 and 8.|
Subcapsular liver hematoma and hepatic rupture are rare, potentially catastrophic complications of HELLP syndrome, with high maternal and fetal mortality rates. The pathogenesis is not fully understood. Liver parenchymal necrosis and fibrin obstruction of hepatic sinusoids due to vascular endothelial damage can cause infarction, ischemia, liver swelling and distention of the Glisson’s capsule surrounding the liver. Sudden abdominal pain can be the first clinical sign of subcapsular hepatic hematoma or hepatic rupture.9,10 Surgical treatment along with intensive supportive treatment, including implementation of a massive transfusion protocol, is usually required.10
Definitive treatment of HELLP syndrome necessitates prompt delivery of the infant after maternal stabilization.4 High-dose corticosteroids are considered beneficial for improving both maternal and fetal outcomes.3 A meta-analysis compared corticosteroids with placebo or no treatment, and found an increase in platelet count with corticosteroids administration, but no benefit for major clinical outcomes.11 Peripartum administration of dexamethasone was more efficient than betamethasone for increasing the platelet count.11 Magnesium sulfate is administered to prevent eclampsia and for fetal neuroprotection.
Long-term complications reported to occur in patients with HELLP syndrome are recurrent HELLP syndrome, preeclampsia, new-onset hypertension, depression and anxiety.12
Maternal or fetal deterioration can occur very quickly. Cesarean delivery is required in the majority of patients. Generalized edema may exacerbate the progressive airway changes during pregnancy and further increase the risk for a difficult airway. Given the increased risk for peripartum hemorrhage, large-bore IV access needs to be established and blood products made immediately available. Blood pressure should be maintained at lower than 140 to 150 mm Hg for systolic and 90 to 100 mm Hg for diastolic values. Controlling systolic hypertension is more important than controlling diastolic pressure in preventing intracranial bleeding. Point-of-care transthoracic echography can be a valuable tool to guide administration of IV fluids.
The main concerns regarding neuraxial anesthesia are decreased platelets and hepatic alterations contributing to coagulopathy.13 The decrease in platelet count is attributed to increased platelet consumption due to vascular endothelium damage. The severity of thrombocytopenia correlates with severity of disease. The decision to proceed with neuraxial anesthesia is based on the downward trend in platelet count rather than an arbitrary value as well as clinical signs of bleeding or other coexisting coagulation abnormalities. A recent retrospective study of pregnant patients with thrombocytopenia showed a low risk for spinal/epidural hematoma when platelets are between 70,000 and 900,000/mcL.14 Most anesthesiologists will not place an epidural if the platelet count is less than 75,000/mcL.
Rapid sequence induction for cesarean delivery can cause an additional increase in blood pressure due to exaggerated pressor responses during laryngoscopy and tracheal intubation, which can significantly increase the risk for intracranial hemorrhage.
Traditionally, opioid administration is postponed to after the delivery to avoid neonatal respiratory depression. However, given the potentially devastating consequences from an abrupt and severe increase in blood pressure, patients presenting with increased blood pressure will benefit from administration of short-acting opioids (e.g., remifentanil) before delivery. Dexmedetomidine has been used safely to blunt hormonal and hemodynamic changes before endotracheal intubation in preeclamptic patients.14
Esmolol (selective beta-1 receptor antagonist) and labetalol (beta-1 and beta-2 receptor antagonist) can be used safely, but maternal and fetal bradycardia should be monitored. Nitroglycerin, a vasodilator mainly used in obstetric anesthesia to facilitate uterine relaxation, provides good hemodynamic control if administered before induction of general anesthesia.15
Magnesium sulfate is key for the prophylaxis, treatment and prevention of seizures (eclampsia) and should be administered regardless of the presence of seizures. Dosing should be 4 to 5–g administered over 5 minutes, followed by 1–g per hour for 24 hours.
Dr. Prozesky is an associate professor of anesthesia, Department of Anesthesiology and Perioperative Medicine, and associate vice chair for quality and quality physician leader. Dr. Vaida is a professor of anesthesiology and perioperative medicine, and obstetrics and gynecology; the vice chair of research; and the director of obstetric anesthesia at Penn State College of Medicine, Penn State Health Milton S. Hershey Medical Center, in Hershey, Pa. The author and reviewer reported no relevant financial disclosures.