What is the management for severe right upper quadrant (RUQ) pain with Hemolysis, Elevated Liver enzymes, and Low Platelet count (HELLP) syndrome?

Management of Severe Right Upper Quadrant Pain with HELLP Syndrome

Women with HELLP syndrome experiencing severe right upper quadrant pain should be delivered promptly once maternal coagulopathy and severe hypertension have been corrected, as this approach improves maternal outcomes. 1

Initial Assessment and Diagnosis

When a pregnant woman presents with severe right upper quadrant pain and suspected HELLP syndrome:

1. Immediate laboratory evaluation:

   • Complete blood count with platelets
   • Liver function tests
   • Renal function tests
   • Coagulation profile including fibrinogen levels 2

2. Imaging:

   • Abdominal ultrasound should be performed if there are symptoms suggestive of hepatic hematoma (abdominal, epigastric, or right shoulder pain) 1
   • Be particularly vigilant in women with markedly reduced platelet counts (<20×10⁹/L) who have higher risk of hepatic hemorrhage 1

Acute Management

1. Blood Pressure Control

• For severe hypertension (systolic BP >160 mmHg or diastolic BP >110 mmHg):

  • Urgent treatment in a monitored setting with:
    • IV labetalol (10-20 mg initially, then 20-80 mg every 10-30 minutes)
    • IV hydralazine (5-10 mg every 15-30 minutes)
    • Oral nifedipine 1, 2
  • Target diastolic BP of 85 mmHg (systolic 110-140 mmHg) 2

• For non-severe hypertension (systolic BP 140-159 mmHg and/or diastolic BP 90-109 mmHg):

  • Oral labetalol, nifedipine, or methyldopa 1

2. Seizure Prophylaxis

• Administer magnesium sulfate to prevent eclamptic seizures:
  • Loading dose: 4-5g IV over 15-20 minutes
  • Maintenance: 1-2g/hour continuous infusion
  • Monitor in high-dependency or intensive care setting for at least 24-48 hours 1, 2
  • Also provides neuroprotection if delivery is required before 32 weeks' gestation 1

3. Management of Coagulopathy

• Platelet transfusion is recommended if platelet count is less than 50,000/mm³, especially prior to Caesarean section 2
• Whole blood or packed red cells if hemoglobin is less than 10 g/dL 2
• Correct coagulopathy before proceeding to delivery 1

4. Corticosteroid Administration

• Do not give corticosteroids to improve maternal outcomes in HELLP syndrome 1
• Do give high-dose dexamethasone or betamethasone to improve fetal lung maturity if delivery will occur before 35 weeks' gestation 1

Definitive Management: Delivery

• Prompt delivery is the definitive treatment once maternal coagulopathy and severe hypertension have been corrected 1, 3
• The time interval between diagnosis and delivery significantly impacts outcomes; shorter intervals result in better prognosis 3
• Mode of delivery should be determined based on:
  • Maternal condition
  • Gestational age
  • Fetal status
  • Cervical favorability 2

Special Considerations

• If signs of hepatic failure develop, early referral to a transplant center should be made 1
• General anesthesia may be recommended if contraindications to regional anesthesia exist, using drugs with minimal hepatic/renal metabolism 2
• Monitor closely for complications such as:
  • Disseminated intravascular coagulation
  • Acute kidney injury
  • Pulmonary edema
  • Placental abruption 2

Post-Delivery Management

• Continue close monitoring for at least 48-72 hours post-delivery as HELLP syndrome can worsen after delivery 2
• Continue blood pressure monitoring every 4-6 hours for at least 3 days 2
• Taper antihypertensive medications slowly after days 3-6 2
• Follow-up within 1 week if still on antihypertensives at discharge 2

Common Pitfalls to Avoid

• Delayed diagnosis: All pregnant women with upper abdominal pain should be screened for HELLP syndrome with platelet count and liver enzymes, regardless of whether classic preeclampsia symptoms are present 4, 5
• Misdiagnosis: HELLP syndrome can mimic other conditions such as acute fatty liver of pregnancy, viral hepatitis, gallbladder disease, or appendicitis 6, 4
• Delayed delivery: Attempting prolonged conservative management can lead to deterioration of maternal condition and worse outcomes 3
• Inadequate monitoring: HELLP syndrome patients should be treated in tertiary care centers with multidisciplinary expertise 6, 7