2nd Year Resident

Professor and Head

Professor

Assistant Professor

Senior Resident

Dept. of Medicine

Shri MP Shah Govt. Medical College, Jamnagar, Gujarat

Keywords

Acute fatty liver, jaundice, encephalopathy, ascites, DIC, HELLP syndrome

Abstract: Acute fatty liver of pregnancy (AFLP) is a rare complication of full-term pregnancy and presents with jaundice and hepatic encephalopathy in the third trimester. Maternal and fetal mortality is increased and diagnosis should not be confused with HELLP (hemolysis, elevated liver enzymes and low platelet count) syndrome, disseminated intravascular coagulation (DIC) and fulminant viral hepatitis occurring in pregnancy as those conditions are usually self-limiting, whereas AFLP should be treated with utmost caution and care. Jaundice during pregnancy can be as simple as viral hepatitis or as serious as acute fatty liver of pregnancy (AFLP), which presents as acute liver cell failure and severe jaundice and is usually associated with pregnancy-induced hypertension (PIH), acute kidney injury and disseminated intravascular coagulation (DIC). This patient had all these associated problems but eventually fully recovered.

Case Report

A 28-year-old female was admitted with twin pregnancy around 36 weeks of gestation with PIH. She gave a history of lower-segment cesarean section (LSCS) 9 years earlier. Clinically, there was PIH, nausea, jaundice and altered coagulation profile with low platelet and raised prothrombin time (PT) and evidence of DIC.

She was taken up for emergency cesarean section and was given whole blood and fresh-frozen plasma. On 2nd post-op day, she developed renal failure and later hepatic encephalopathy with DIC and ascites. When the ascites was aspirated it was found to be hemorrhagic.

Investigations

(Initial sample and repeat samples)Serum bilirubin 8.6 | 7.3 | 2.4 | 1.4 mg/dLProthrombin time (PT) = >60 sec | 16.2 secActivated partial thromboplastin time (APTT) = >60 sec | 38.5 secSerum glutamic-pyruvic transaminase (SGPT) = 98 | 69 | 43 | 33 units/LViral markers = A | B | C | E negativeHemoglobin - 5.6 | 9.5 | 11.1 g/dLD-dimer - positiveWBC - 21,700 | 15,600 | 10,000 | 6800/mm3Platelets - 65,000 | 1,10,000 | 1,50,000/LBlood urea - 62 | 38 mg/dLSerum creatinine - 3.0 | 1.8 | 0.7 mg/dLUSG abdomen = Liver size normal with coarse echoes with septate ascites. No evidence of subcapsular hematoma.

Discussion

Jaundice in pregnancy has three different pregnancies: It may be peculiar to the pregnant state, it may be unrelated to pregnant state or it may be when pregnancy is superimposed on chronic liver disease (Table 1). Acute fatty liver of pregnancy (AFLP) presents with a spectrum ranging from modest hepatic dysfunction to hepatic failure, coma and death. It affects about 1 in 13,000 deliveries. Affected women present usually in third trimester, symptoms include bleeding due to altered coagulation factors, vomiting, jaundice and coma. In about 30% of cases, the presenting symptoms may be those of coexistent pre-eclampsia. It is more common in twin pregnancy.

The liver cells contain diffuse microvesicular fat droplets with widespread hepatic and metabolic, disturbances involving mitochondria and ribosomes. Ascites is found in 50% of patients. Severe bleeding is frequent and DIC is found in 10% of cases. Leukocytosis is usual with neutrophilia and thrombocytopenia, raised PT, APTT and raised ammonia. There is raised serum bilirubin and liver enzymes. Ultrasound of liver shows increased echoes in 50% of cases.

AFLP is a catastrophic illness of third trimester with maternal and fetal mortality of 80-90%. Death due to DIC, hepatorenal failure, hepatic encephalopathy and gastrointestinal hemorrhage is common. AFLP is often confused with HELLP (hemolysis, elevated liver enzymes and low platelet count) syndrome. In HELLP syndrome, there is no evidence of DIC, deep jaundice or hepatic encephalopathy. It is a self-limiting disease and should not be confused with AFLP.

Our patient came in third trimester of twin pregnancy around 36 weeks. She had associated evidence of PIH and was taken up for cesarean section. She also had nausea, jaundice, ascites, DIC, renal failure, hepatic encephalopathy and hemoperitoneum. Although her serum bilirubin was very high and she was bleeding into peritoneum as PT was very high and prognosis was very guarded, she finally rallied around after 1 month of treatment, came out of encephalopathy. PT got corrected, DIC and renal failure become normal. Ascites is very rare in acute liver cell failure and hemoperitoneum is even more rare in AFLP.

The usual cause of hemoperitoneum is rupture of subcapsular hematoma of liver. This patient did not have a subcapsular hematoma of liver as confirmed by USG abdomen. She also had severe hypoprothrombinemia, which usually presents as upper gastrointestinal bleeding but she presented as hemoperitoneum.

Conclusion

This case is being presented for its rarity and full recovery of the patient although she had a very rare complication of hemorrhagic ascites, which is usually unheard of as a complication of AFLP.

Suggested Reading

1. Manual of Practical Problems in Obstetrics - Donald School. 1st Edition, 2012. pp. 188-9.
2. Disease of the Liver and Biliary System – Sheila Sherlock. 9th Edition, 1993. pp. 452-5.
3. Obstetrics-Normal and Problem Pregnancies. 3rd Edition, 1996 p. 1119.
4. Pathologic Basis of Disease – Robbins and Contran. 3rd Edition, 2010.