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Table of Contents
CASE REPORT
Year : 2020  |  Volume : 6  |  Issue : 2  |  Page : 171-174

Acute fatty liver of pregnancy: A clinical dilemma


1 Department of Obstetrics and Gynaecology, AIIMS, Nagpur, Maharashtra, India
2 Department of Obstetrics and Gynaecology, AIIMS, Jodhpur, Rajasthan, India
3 Department of Obstetrics and Gynaecology, Dr. D.Y. Patil Medical College, Hospital and Research Centre, Pimpri, Pune, Maharashtra, India
4 Department of Medicine, Andaman and Nicobar Islands Institute of Medical Sciences, Port Blair, India

Date of Submission25-Mar-2020
Date of Decision13-May-2020
Date of Acceptance10-Oct-2020
Date of Web Publication24-Dec-2020

Correspondence Address:
Dr. Charu Sharma
Department of Obstetrics and Gynaecology, AIIMS, Jodhpur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJCFM.IJCFM_22_20

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  Abstract 

Acute Fatty Liver of Pregnancy (AFLP) is a rare and life-threatening obstetric emergency. Although the exact pathogenesis is unknown, the cases of AFLP are believed to be associated with abnormal fatty acid oxidation. The diagnosis of AFLP is a challenging task for the clinicians because of the nonspecific clinical presentation which may mimic conditions such as acute viral hepatitis, preeclampsia or Hemolysis, Elevated Liver Enzymes, and Low Platelet Count (HELLP) syndrome. Early diagnosis, prompt obstetric management, intensive supportive care, and a multidisciplinary approach are the key to a good outcome. We here present two similar cases of AFLP with two different outcomes managed in a tertiary care center of the Andaman and Nicobar Islands.

Keywords: Acute fatty liver of pregnancy, disseminated intravascular coagulopathy, hepatic failure, jaundice


How to cite this article:
Yadav A, Sharma C, Kathpalia SK, Singh SS. Acute fatty liver of pregnancy: A clinical dilemma. Indian J Community Fam Med 2020;6:171-4

How to cite this URL:
Yadav A, Sharma C, Kathpalia SK, Singh SS. Acute fatty liver of pregnancy: A clinical dilemma. Indian J Community Fam Med [serial online] 2020 [cited 2021 Jan 25];6:171-4. Available from: https://www.ijcfm.org/text.asp?2020/6/2/171/304792


  Introduction Top


Acute fatty liver of pregnancy (AFLP) is one of the rare and potentially fatal complications of late pregnancy. The incidence ranges between 1 in 7000 to 1 in 16,000 pregnancies.[1],[2] It is an autosomal recessive disorder with inherited mitochondrial abnormalities of fatty acid oxidation. The most common mutations are the G1528C and E474Q mutations of the gene on chromosome 2 that code for long-chain-3 hydroxy acyl-CoA dehydrogenase (LCHAD).[2] The clinical presentation is vivid and may range from a mild asymptomatic case to a severe variety resulting in overt hepatic failure with encephalopathy leading to high maternal and perinatal morbidity and mortality. However, the updated guidelines stressing on early termination of pregnancy and more intensive supportive care protocols have led to the decrease in maternal mortality from 80%–85% to 7%–18% and the fetal mortality rate from 50% to 9%–23%.[1] We, hereby, present two cases of AFLP in which we could save one mother but lost the other one despite intensive obstetric management and support.


  Case Reports Top


Case 1

A 20-year-old primigravida was admitted at 34 weeks gestation with preterm labor pains along with fever and malaise for 2 days. On examination, she was conscious and well oriented. The temperature was 99.2°F, pulse rate (PR): 88 beats/min, blood pressure (BP): 110/70 mmHg, and sclera was yellow. Systemic examination was within the normal limits and there was no organomegaly. Obstetric examination revealed a single viable fetus of around 32 weeks. Her laboratory investigations are shown in [Table 1].[3] Abdominal ultrasound showed fatty changes with bright echotexture in the liver with ascites. Initially, hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome was suspected, but on account of hypoglycemia, hypofibrinogenemia, raised bilirubin, normal BP, and ascites, the diagnosis of AFLP was made. The patient underwent emergency cesarean section for fetal distress. Live male child of 2600 g was delivered. Hemostasis was secured and a prophylactic abdominal drain was kept. The patient received two units of packed cell infusion and four units of fresh frozen plasma (FFP). She was shifted to the intensive care unit (ICU) and managed by critical care specialist and gynecologist. She was shifted to the ward on the 7th postoperative day and discharged on day 12. The mother and baby were healthy on follow-up on day 28. Unfortunately, we could not screen the mother and baby for LCHAD mutation, as this facility was not available in the institute or anywhere else in the island.
Table 1: On admission laboratory parameters of cases with acute fatty liver of pregnancy

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Case 2

A 32-year-old primigravida with 37-week gestation was referred to our hospital with complaints of yellow discoloration of urine and eyes and vomiting for 3–4 days. There was no history of pain in the abdomen, leaking or bleeding per the vagina. On examination, she was drowsy. Her BP was 160/100 mmHg, PR: 84 beats/min, and temperature: 99°F. She had icterus and mild pedal edema. Abdominal examination revealed relaxed uterus of 36 week size with the fetus in cephalic presentation and normal fetal heart rate. Her investigations on admission are shown in [Table 1]. Blood gas analysis revealed metabolic acidosis with respiratory alkalosis. Ultrasound abdomen showed grade 1 fatty changes with moderate ascites.

The patient had spontaneous vaginal delivery of a 2540 g male, stillborn baby, but her condition deteriorated after delivery with rising liver enzymes and deranged coagulation profile despite correction with fresh frozen plasma. She was shifted to the ICU in view of hepatic encephalopathy with coagulopathy and hepatorenal shutdown. She had to be dialyzed and blood component therapy was given to correct coagulation profile. The patient was intubated and started on inotropes later. Despite vigorous management, she could not be revived and expired on the 5th day.


  Discussion Top


AFLP was first described by Stander and Cadden in 1934[4] and later, Sheehan[5] in 1940 gave it the name of acute yellow atrophy – where postmortem examination was performed on 400 obstetric patients and six were found to have significant liver pathology. This entity has a high case fatality rate unless managed intensively and promptly.

The majority of cases of AFLP occur in the late third trimester, commonly in primigravida, multifetal gestation, and with a male fetus.[6] Both our cases were primigravida and had male babies.

The literature suggests that AFLP usually occurs due to the deficiency of LCHAD or mutations in the genes coding for this enzyme. There is not much effect on fatty acid oxidation mechanism under normal physiological conditions; however, when both the parents are heterozygous for this abnormality and the fetus is homozygous, it will not be able to oxidize long-chain fatty acids. The un-metabolized free fatty acids traverse the placenta and saturate the maternal circulation overwhelming the mothers' mitochondrial capacity to oxidize excess fatty acids.[7] Termination of pregnancy will break this chain of fatty acid circulation and diminish the hepatic stress, thereby normalizing the condition postpartum.[8] Newborns with defective mitochondrial fatty acid oxidation may present with hypoglycemia, metabolic acidosis, hepatic failure, and cardiomyopathy and are associated with increased perinatal morbidity and mortality.[1] In an ideal scenario, it is always better to search for this mutation in the mother and the newborn so that we can prognosticate the case and predict about the recurrence in a future pregnancy. However, in our case, we could not get the mutation testing done as it was not available in our setup. Andaman and Nicobar Islands Institute of medical Sciences is the only tertiary referral hospital of the island catering the entire population. The patients who are difficult to be managed due to lack of infrastructure or resources are referred to Chennai or Kolkata on government expense for further management. Our first case, who survived, was given the option of molecular testing at a higher center, but she refused for that.

AFLP is a diagnosis of exclusion. A high index of suspicion is required to diagnose AFLP, as there are no straight forward noninvasive diagnostic tests to make a confirmatory diagnosis. The clinical presentation may be nonspecific. Many case reports have been published describing the myriad presentation of this condition.[9],[10],[11] The patient commonly presents with malaise, epigastric pain, nausea, and vomiting followed by jaundice. On laboratory tests, bilirubin levels may be high but are usually not more than 10 mg per dl, liver transaminases may be elevated but not more than 1000 U/L, and renal dysfunction, coagulopathy, hypofibrinogenemia, and hypoglycemia may be present.[6] Both our cases had hyperbilirubinemia, hypoglycemia, hypofibrinogenemia, and raised LDH which favored AFLP. Although one should have a high index of suspicion for AFLP, other pregnancy-related liver conditions that mimic AFLP such as acute viral hepatitis, preeclampsia, HELLP syndrome, intrahepatic cholestasis of pregnancy (IHCP), and cholelithiasis must also be taken into consideration. Differentiation between preeclampsia with liver involvement, HELLP syndrome, and AFLP is a challenging task because of similar clinical and laboratory picture. However, there are some specific patterns which can aid in differentiating them. Women with HELLP usually have high BP and do not have hypoglycemia. Women with AFLP can have preeclampsia, but females with preeclampsia alone are not usually jaundiced and do not usually have hypoglycemia. Severe coagulopathy, jaundice, hepatic encephalopathy, ascites, hypoglycemia, and a mild-to-moderate elevation of transaminase levels are the key features of AFLP and were present in our cases also.

Women with acute viral hepatitis in pregnancy also present with fever, nausea, vomiting, fatigue, and jaundice, but the aminotransferase values are markedly elevated (>1000 U/L) and serology test will be positive. IHCP can cause jaundice as well, but itching is the characteristic symptom with raised serum bile acid levels. After excluding these cases, a clinical impression of AFLP was made in our case.

The specificity and sensitivity of the imaging studies in diagnosing AFLP are insufficient, and the likelihood of false-negative results is high.[6] Liver biopsy is the gold standard test, but it is invasive, risky, and requires a normal coagulation status.

The optimal management of AFLP is prompt delivery and supportive care of the mother. However, many patients may deteriorate even after delivery,[10] as it was seen in our second case where the patient died on the 5th day. The relatives denied postmortem examination. Such severely ill patients may require ICU care including assisted ventilation and dialysis. Aggressive correction of hypoglycemia and coagulation abnormality is the mainstay of treatment. Maternal deaths usually occur due to hemorrhage, gastrointestinal bleeding, sepsis, aspiration, pancreatitis, or renal failure. Surviving patients generally recover with no hepatic sequel.[6]


  Conclusion Top


AFLP is a medical and obstetrical emergency. Although in low-resource settings, molecular testing cannot be done, but with a high index of suspicion, early recognition, timely referral, prompt delivery, and aggressive management, morbidity and mortality of AFLP can be greatly reduced.

Acknowledgment

We acknowledge the ICU team and nursing staff of the Department of Obstetrics and Gynecology, G.B. Pant Hospital, ANIIMS Port Blair, where these patients were managed.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Flint Porter T. Acute fatty liver of pregnancy. In: Michael Belfort. Critical Care Obstetrics, 5th ed. Oxford: Blackwell Publishing Ltd.; 2010. p. 385-90.  Back to cited text no. 1
    
2.
Cunningham FG, Leveno KJ, Bloom SL, Hauth JC, Rouse DJ, Spong CY, editors. Hepatic, Gall Bladder and Pancreatic Disorders. Williams Obstetrics. 25th ed. New York: Mc Graw Hill; 2018. p. 1060-2.  Back to cited text no. 2
    
3.
Cunningham FG, Leveno KJ, Bloom SL, Hauth JC, Rouse DJ, Spong CY, editors. Appendix 1. Williams Obstetrics. 25th ed. New York: Mc Graw Hill; 2018. p. 1255-60.  Back to cited text no. 3
    
4.
Stander HJ, Cadden JF. Acute yellow atrophy of the liver in pregnancy. Am J Obstet Gynecol 1934;28:61-9.  Back to cited text no. 4
    
5.
Sheehan H. The pathology of acute yellow atrophy and delayed chloroform poisoning. Int J Obstetrics Gynecol 1940;47:46-62.  Back to cited text no. 5
    
6.
Dey M, Kumar R, Narula GK, Vadhera A. Acute fatty liver of pregnancy. Med J Armed Forces India 2014;70:392-93.  Back to cited text no. 6
    
7.
Ko HH, Yoshida E. Acute fatty liver of pregnancy. Canadian J Gastroenterol Hepatol 2006;20:25-30.  Back to cited text no. 7
    
8.
Gregory TL, Hughes S, Coleman MA, De Silva A. Acute fatty liver of pregnancy; three cases and discussion of analgesia and anaesthesia. Int J Obstetric Anesth 2007;16:175-9.  Back to cited text no. 8
    
9.
Ziki E, Bopoto S, Madziyire MG, Madziwa D. Acute fatty liver of pregnancy: A case report. BMC Pregnancy Childbirth 2019;19:259.  Back to cited text no. 9
    
10.
Myers J, Wu G, Shapiro RE, Vallejo MC. Preeclampsia induced liver dysfunction complicated by disseminated intravascular coagulopathy and placental abruption: A case report and review of the literature. Case Rep Anesthesiol 2019;2019:4305849.  Back to cited text no. 10
    
11.
Dwivedi S, Runmei M. Retrospective study of seven cases with acute fatty liver of pregnancy. ISRN Obstetrics Gynecol 2013;2013:730569.  Back to cited text no. 11
    



 
 
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