The patient’s mental status began to improve, and by postop day 6, she was able to follow commands and engage in brief conversations. By postop day 9, she had returned almost completely to her baseline mental status.
The patient’s liver function test results and total bilirubin, ammonia, and creatinine levels all improved over the first few postoperative days but began to rise again by day 6. In response to worsening renal and hepatic functioning, the decision was made on postop day 9 to transfer the patient to a hospital with liver transplantation capabilities, should this procedure become necessary.
Discussion
AFLP is a rare condition specific to pregnancy, affecting 1/7,000 to 1/20,000 pregnancies. Due to the low incidence of this disease, randomized controlled trials to study it are not possible. Instead, clinicians must learn either from individual case studies or from retrospective syntheses of cases reported over time.1,2,7 Fortunately, the wealth of information gleaned over the past 30 years has significantly reduced AFLP-associated maternal and fetal mortality and morbidity rates. In the 1980s, maternal and fetal mortality rates as high as 85% were reported.3 Worldwide, maternal mortality associated with AFLP has decreased significantly to 7% to 18%, whereas the fetal mortality rate has fallen to between 9% and 23%.1,16,17
Common trends among women who have developed AFLP include nulliparity, multiparity, and advanced maternal age. One retrospective study of 57 women who had developed AFLP revealed that 35 cases (61%) involved first-time pregnancies. It also showed that 10 (18%) of the women had twins, and 14 (25%) were older than 35.2 In another study of 35 cases of AFLP, 40% of the women were nulliparous, and 11.4% were multiparous, including one triplet gestation.12 In a third, smaller study, 80% of women affected by AFLP were multiparous.10 Currently, there is no known evidence linking any maternal behavior to development of AFLP.
Presentation
Women who present with AFLP often experience vague, nonspecific symptoms, leading to misdiagnosis or delayed diagnosis. Objective measurements, including physical exam findings, laboratory studies, and other diagnostic tests, will help with a diagnosis. The most frequent initial symptoms are nausea and vomiting (in 70% of patients) and abdominal pain (50% to 80%), epigastric or right upper-quadrant.3 Other common symptoms include fatigue, malaise, anorexia, weight gain, polyuria, and polydipsia.2,3,9,18,19
Because the presenting symptoms in AFLP can be vague, clinicians should complete a thorough physical exam to differentiate accurately among conditions associated with pregnancy. Physical signs present in women with AFLP can include jaundice, ascites, edema, confusion, abdominal tenderness, and fever. More severe cases can present with multisystem involvement, including acute renal failure, gastrointestinal bleeding, pancreatitis, coagulopathy, and hepatic encephalopathy.3,4,9,18
Diagnostic Tests
Relevant laboratory tests include a complete blood count (CBC), liver studies, chemistry, coagulation studies, and urinalysis (see Table 1). Viral causes should be ruled out by way of a hepatitis panel.3 In AFLP, the CBC may show elevated white blood cells, decreased hemoglobin and hematocrit, and decreased platelets. Liver studies show elevated hepatic aminotransferase, bilirubin, LDH, and ammonia levels. Chemistry results show elevated blood urea nitrogen and creatinine, and decreased blood glucose. Coagulation factors are affected, and prolonged PTT, decreased fibrinogen, and proteinurea may also be found.9
Though invasive and not often necessary4,13 (and not possible for the case patient), the definitive diagnostic test for AFLP is liver biopsy.13,14 Biopsy reveals a microvesicular fatty infiltration of the hepatocytes as minute fat droplets surrounding a centrally located nucleus. These fatty infiltrates stain with oil red O, specific for fat. Inflammation is present in 50% of cases. There may also be a picture similar to cholestasis with bile thrombi or deposits within the hepatocytes.20
Due to the risk for hemorrhage and the critical status of women with AFLP, biopsy is often not possible. Ultrasonography may show increased echogenicity; CT may show decreased or diffuse attenuation in the liver. These imaging studies, though possibly helpful in severe cases, often yield false-negative results.3,20
In the absence of another explanation for the patient’s symptoms, the Swansea criteria are used for diagnosis of AFLP.1 Six or more of the following criteria must be present to confirm this diagnosis: vomiting, abdominal pain, polydipsia or polyuria, encephalopathy, leukocytosis, elevated bilirubin, elevated liver enzymes, elevated ammonia, hypoglycemia, renal dysfunction, coagulopathy, elevated uric acid, ascites on ultrasound, and microvesicular steatosis on liver biopsy.1,2,5
Pathophysiology
Normal functions of the liver include metabolism, protein synthesis, and manufacturing of blood coagulation proteins. These functions are disturbed in the presence of AFLP. Thus, women with this disease experience signs and symptoms related directly to the dysfunction of these processes.20-22