Anesthetic considerations for liver diseases unique to pregnancy

Table 1 The incidence, etiology, symptoms, diagnosis and treatment of the liver disease unique to pregnancy

Disease	Incidence	Etiology	Symptoms	Diagnosis	Prognosis	Treatment
Hyperemesis gravidarum[3-17]	1-20/1000 pregnancies (< 2%)[5]	Psychological predisposition Hormones (human chorionic gonadotropin, estradiol)[7]	Severe nausea and vomiting Dehydration Malnutrition Poor weight gain[1,7]	Diagnosis by clinical presentation (persistent vomiting, acute starvation and weight loss) Increaed levels of liver enzymes (aminotransferase, alkaline phosphatase and amylase)[1,3,8-12] Rarely, liver biopsy is needed[1,11,12]	Unchanged maternal and fetal outcomes after use of safe antiemetics Increased risk of low-birth-weight infants, preterm birth, preeclampsia, and placental abruption in the 2nd trimester[8,16,17]	Avoid nausea triggering substances Medical and supportive therapy (ginger, multivitamin or Vit B6 with H1 receptor antagonist doxylamine) Treatment of dehydration (intravenous infusion of fluids, metoclopramide or promethazine with another H1 receptor antagonist dimenhydramine)[1,8,13-15]
Acute fatty liver of pregnancy[18-29]	1/10000-15000 pregnancies[18]	Mutations in LCHAD[1,21]	Nausea, vomiting, anorexia lethargy, abdominal pain, ascites, progressive jaundice Polyuria and polydipsia due to transient diabetes insipidus Acute renal failure Hepatic encephalopathy Hypertension, proteinuria and edema[1,4,22,23]	Diagnosis by clinical and laboratory findings (increased levels of aminotransferases, ammonia, bilirubin, leukocytosis, hypoglycemia, thrombocytopenia, neutrophilia, coagulopathy, renal dysfunction)[20,22,24] Tomography and ultrasonography are unremarkable[7] Liver biopsy reveals microvesicular steatosis[25]	Liver function improves within a week to months[27] Preterm delivery (75%) approximately at 34 wk gestation Check all mothers with AFLP for defects in fatty acid oxidation[1]	Immediate hospitalization Supportive measures (glucose infusion, readily availbale blood products) Prompt delivery
Intrahepatic cholestasis of pregnancy[1,30-51]	1-2/1000 pregnancies[1]	Multifactorial genetic (mutations in the MDR3 gene) Hormonal Exogenous factors (e.g., progesterone) Abnormal biliary transport[25,33-35]	Generalized peripheral pruritus (1st sign) Chills and abdominal pain Diarrhea or steatorrhea[36,37]	Diagnosis by clinical symptoms and/or laboratory tests (increased levels of fasting serum bile acid and elevated bilirubin and transaminase levels)[38] Liver biopsy is needed only in severe cases and biopsy reveals cholestasis with minimal or no inflammatory changes[39]	Good maternal outcome (laboratory results resolve within 2-8 wk postpartum)[7] Compromised fetal outcome (spontaneous preterm labor, meconium-stained fluid with some perinatal mortality) in moderate and severe forms[1,42-46]	Symptomatic medical treatment (ursodeoxycholic acid: UDCA which is B Class safe drug for pregnancy and breastfeeding by FDA)[40,41] Optimal timing of delivery at the best possible fetal maturity[45]
HELLP syndrome[1,51-62]	0.1%-0.6%[1,52]	Usually presents with preeclampsia (4%-12% are with severe preeclampsia) Endothelial injury with fibrin deposit is the underlying mechanism of the disease[54]	Right upper quadrant or epigastric pain Nausea and vomiting Malaise Nonspecific viral-like symptoms headache visual symptoms[55]	Liver biopsy is not necessary but if performed sinusoidal fibrin thrombin, hemorrhage, and hepatocellular necrosis might be observed[25] Laboratory findings Platelet count < 100000/μL Serum aspartate aminotransferase > 70 U/L Serum lactic dehydrogenase > 600 U/L[56]	Maternal death (1%) Perinatal death (7.4%-20.4%) Pulmonary edema Acute renal failure DIC Abruptio placenta Liver hemorrhage or failure ARDS Retinal detachment Stroke Blood transfusion related complications	Provide transfer to tertiary care center after confirmed diagnosis Delivery > 34 weeks’ gestation is recommended if possible Prophylaxis of seizures with magnesium during labor and 24 h postpartum[58]

HELLP: Hemolysis, elevated liver enzymes and low platelets; LCHAD: Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase; ARDS: Acute respiratory distress syndrome; AFLP: Acute fatty liver of pregnancy; FDA: Food and Drug Administration.