|Year : 2022 | Volume
| Issue : 4 | Page : 239-243
Fulminant acute fatty liver of pregnancy presenting with multi-organ failure: A case series
Sai Saran1, Saumitra Misra1, Suhail Sarwar Siddiqui1, Avinash Agrawal1, Mohan Gurjar2, Ajay Kumar Patwa3, Syed Nabeel Muzaffar1
1 Department of Critical Care Medicine, King George's Medical University, Lucknow, Uttar Pradesh, India
2 Department of Critical Care Medicine, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
3 Department of Medicine, Division of Gastromedicine, King George's Medical University, Lucknow, Uttar Pradesh, India
|Date of Submission||21-Apr-2022|
|Date of Acceptance||20-Jun-2022|
|Date of Web Publication||26-Dec-2022|
Dr. Syed Nabeel Muzaffar
Department of Critical Care Medicine, King George's Medical University, Lucknow - 226 003, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Liver disease in pregnancy can be classified into pregnancy-related, liver disease coincident with pregnancy or preexisting liver disease. Acute fatty liver of pregnancy (AFLP) is a rare liver disorder that is caused by defects in mitochondrial beta (β) oxidation of fatty acids. In view of its fulminant presentation and rapid progression to multiple organ failure (MOF), AFLP carries high maternal and fetal mortality. These patients are commonly present in the third trimester of pregnancy with gastrointestinal symptoms and complications such as hypoglycemia, lactic acidosis, hyperammonemia, leukocytosis, liver dysfunction, coagulopathy, and renal dysfunction. Diagnosis is mostly based on the Swansea diagnostic criteria and by excluding other etiologies of liver dysfunction. Liver biopsy is rarely performed owing to underlying coagulopathy and thrombocytopenia. In this case series, we intend to share our experience of managing four cases of AFLP that were admitted to the intensive care unit with fetal demise and MOF.
Keywords: Fetal death, liver steatosis, multiple organ dysfunction syndrome, pregnancy complications
|How to cite this article:|
Saran S, Misra S, Siddiqui SS, Agrawal A, Gurjar M, Patwa AK, Muzaffar SN. Fulminant acute fatty liver of pregnancy presenting with multi-organ failure: A case series. Int J Crit Illn Inj Sci 2022;12:239-43
|How to cite this URL:|
Saran S, Misra S, Siddiqui SS, Agrawal A, Gurjar M, Patwa AK, Muzaffar SN. Fulminant acute fatty liver of pregnancy presenting with multi-organ failure: A case series. Int J Crit Illn Inj Sci [serial online] 2022 [cited 2023 Jan 29];12:239-43. Available from: https://www.ijciis.org/text.asp?2022/12/4/239/364739
| Introduction|| |
Acute fatty liver of pregnancy (AFLP) is a rare and life-threatening, liver disorder that occurs in the third trimester of pregnancy with a prevalence of 1:7,000–1:20,000 pregnancies., Diagnosis of AFLP is based on the Swansea criteria, after excluding other potential etiologies of liver dysfunction in pregnancy. Liver biopsy, although confirmatory, is not feasible in clinical settings due to underlying coagulopathy. Early termination of pregnancy and intensive care unit (ICU) management is essential to ensure a better outcome. We hereby describe four cases of AFLP that presented to ICU with multi-organ failure (MOF).
| Case Series|| |
A 24-year-old female, primigravida, G3P1L0A1, with no comorbidities, was referred to ICU at 33 weeks of gestation with acute hepatic dysfunction, intrauterine fetal death (IUFD), postpartum hemorrhage (PPH), and MOF. In the third trimester, she had developed loose stools on and off, followed by fever, altered sensorium, and decreased fetal movements. Ultrasonography (US) pelvis revealed IUFD. Immediately after the termination of pregnancy, she developed PPH which was managed with multiple blood products and uterine packing. At ICU admission, she was afebrile, restless and agitated, tachypneic (Respiratory rate (RR) 35/min), blood oxygen saturation (SpO2 98% [on room air]), tachycardia (heart rate [HR] 114/min), and normotensive (blood pressure [BP] 106/67 mm Hg). Her abdomen was soft but distended, urine output was reduced, and bleeding was present from the episiotomy wound.
Laboratories at admission showed anemia, thrombocytopenia, leukocytosis, coagulopathy, hypoglycemia, and deranged liver and renal functions [Table 1].US abdomen revealed a bulky uterus and no significant retained products of conception. 2-D echocardiography was normal. The general blood picture showed anisocytosis, hypochromia, microcytosis, thrombocytopenia, severe leukocytosis, and neutrophilia with no evidence of hemolysis. Viral serology for hepatotropic viruses (Hepatitis A virus, hepatitis B virus, hepatitis C virus, and Hepatitis E virus), tropical causes for acute febrile illness (dengue, malaria, typhoid, scrub typhus, and leptospirosis) were ruled-out and workup for autoimmune hepatitis was also negative. Noncontrast computed tomography head showed diffuse cerebral edema secondary to acute hepatic dysfunction. Cerebral protection strategy (head-up position, deep sedation, mannitol, hypertonic saline, and normocarbia) was initiated along with invasive mechanical ventilation (MV), hemodynamic support (vasopressors), antimicrobials, and blood component therapy. She improved within 48–72 h of delivery, and tracheal extubation was done after 2 weeks of ICU stay.
A 22-year-old female, primigravida, with no comorbidities, was transferred to ICU from the labor room after vaginal delivery of intrauterine fetal demise (IUFD) at 35 weeks of gestation. Her antenatal course was uneventful till the third trimester when she developed jaundice for 10 days and headache for 2 days, followed by diarrhea, oral bleeding, and altered sensorium. At ICU admission, she was afebrile, sedated, hypotensive (BP 80/50 mm Hg), tachycardia (HR 130/min), oliguric, and had deranged liver and kidney functions [Table 1]. 2-D echocardiography was normal. Organ support therapies such as cerebral protection strategy, hemodynamic support, and invasive MV were administered. Over the next 48 h, there was progressive improvement in organ functions. She got extubated on ICU day 7 and was ultimately discharged home.
A 25-year-old female, G3P1 L0A2, with no comorbidities, was admitted to the labor room at 34 weeks of gestation with low-grade fever intermittently for a month with high-grade fever and scleral icterus for 1 week, multiple episodes of vomiting and abdominal pain for 2 days and altered behavior for 1 day. At ICU admission, she was disoriented, agitated, tachycardia (HR 105/min), BP 126/70 mmHg (no vasopressors), tachypneic (RR 30/min), SpO2 98% (without supplemental oxygen), and oliguric. US pelvis revealed IUFD. Postvaginal delivery of the fetus, she had PPH that was managed with uterine packing and blood products. Laboratories are mentioned in [Table 1]. In view of progressively worsening sensorium, the airway was secured, and a cerebral protection strategy was instituted along with invasive MV, hemodynamic optimization, and renal replacement therapy. Within 72–96 h posttermination of pregnancy, she improved clinically and tracheal extubation was done on the 10th day of ICU stay.
A 25-year-old female, a primigravida with twin pregnancy, presented to ICU following an emergency lower segment cesarean section at 36 weeks of gestation for fetal distress. She had a history of pain abdomen, vomiting, jaundice for 1 week, and altered sensorium for 2–3 days before getting admitted to the hospital. Issues at ICU admission were encephalopathy, hypoglycemia, and shock, for which supportive management was done. Thereafter, the patient improved clinically and was subsequently discharged home from ICU.
[Table 1] shows a description of these four cases.
| Discussion|| |
AFLP is a rare but lethal disorder that occurs in the third trimester of pregnancy., It is a medical and obstetric emergency that leads to high fetal and maternal mortality. The pathophysiological basis of AFLP is defective mitochondrial beta (β) oxidation of fatty acids, which results in microvesicular fatty infiltration of hepatocytes. AFLP is generally diagnosed by excluding other etiologies of liver dysfunction in pregnancy, especially those presenting in the third trimester. [Figure 1] depicts an algorithmic approach to tackle varied etiologies of liver dysfunction in pregnancy and [Table 2] shows the common third-trimester etiologies of liver dysfunction in pregnancy.
|Figure 1: Algorithmic approach to liver dysfunction in pregnancy. HAV: Hepatitis A virus, HEV: Hepatitis E virus, HCV: Hepatitis C virus, IPC: intrahepatic cholestasis of pregnancy, HELLP: Hemolytic anemia, Elevated Liver Enzymes, and Low Platelets syndrome, AFLP: Acute fatty liver of pregnancy, GBP: General blood picture, CBC: Complete blood count, BMP: Basic metabolic panel, LFT: Liver function test, RBS: Random blood sugar, LDH: Lactate dehydrogenase, INR: International normalized ratio, USG: Ultrosonongraphy, ICP: Intracranial pressure, PPH: Postpartum hemorrhage, IUFD: Intrauterine fetal death, DIC: Disseminated intravascular coagulation, MODS: Multiorgan dysfunction syndrome|
Click here to view
|Table 2: Common third trimester etiologies of liver dysfunction in pregnancy|
Click here to view
Hemolysis, elevated liver enzymes, and low platelets syndrome is an important differential diagnoses of AFLP. Points in favor of AFLP include gastrointestinal (GI) symptoms at the onset of disease, fulminant course with MOF including encephalopathy, acute kidney injury, disseminated intravascular coagulation, hypoglycemia, hyperammonemia, and ascites., Liver biopsy is rarely feasible in clinical settings due to underlying coagulopathy. Hence, AFLP is usually diagnosed by Swansea criteria (with 100% sensitivity, 57% specificity, 100% negative predictive value, and 85% positive predictive value),,, ≥6 Swansea criteria qualify for the diagnosis. Clues favoring AFLP in our cases were third-trimester onset, initial GI symptoms, fulminant presentation with MOF, and ≥ 6 Swansea criteria [Table 3]. Aggressive ICU care and early termination of pregnancy are quintessential for the management of AFLP.
|Table 3: Diagnosis of acute fatty liver of pregnancy using Swansea criteria|
Click here to view
| Conclusion|| |
AFLP is a fulminant medical and obstetric condition that presents in the third trimester of pregnancy and culminates in MOF and fetal demise. Swansea criteria are helpful in the timely diagnosis of AFLP. Early diagnosis and ICU management with a multidisciplinary approach are pivotal to improving the outcome of patients.
Research quality and ethics statement
This case series did not require approval by the Institutional Review Board/Ethics Committee. The authors followed applicable EQUATOR Network (http://www.equator-network.org/) guidelines, specifically the CARE guidelines, during the conduct of this research project.
Declaration of patient consent
The authors certify that each subject provided written informed consent for the publication of de-identified findings, but that anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Ibdah JA. Acute fatty liver of pregnancy: An update on pathogenesis and clinical implications. World J Gastroenterol 2006;12:7397-404.
Sheehan HL. The pathology of acute yellow atrophy and delayed chloroform poisoning. J Obstet Gynecol Br Emp 1940;47:49-62.
Joshi D, James A, Quaglia A, Westbrook RH, Heneghan MA. Liver disease in pregnancy. Lancet 2010;375:594-605.
Westbrook RH, Dusheiko G, Williamson C. Pregnancy and liver disease. J Hepatol 2016;64:933-45.
Yadav A, Sharma C, Kathpalia SK, Singh SS. Acute fatty liver of pregnancy: A clinical dilemma. Indian J Community Fam Med 2020;6:171. [Full text]
Knight M, Nelson-Piercy C, Kurinczuk JJ, Spark P, Brocklehurst P, UK Obstetric Surveillance System. A prospective national study of acute fatty liver of pregnancy in the UK. Gut 2008;57:951-6.
Gami N, Singhal S, Puri M, Dhakad A. An approach to diagnosis and management of acute fatty liver of pregnancy. Int J Reprod Contracept Obstet Gynecol 2013;2:104-8.
Usta IM, Barton JR, Amon EA, Gonzalez A, Sibai BM. Acute fatty liver of pregnancy: An experience in the diagnosis and management of fourteen cases. Am J Obstet Gynecol 1994;171:1342-7.
Dwivedi S, Runmei M. Retrospective study of seven cases with acute Fatty liver of pregnancy. ISRN Obstet Gynecol 2013;2013:730569.
Goel A, Ramakrishna B, Zachariah U, Ramachandran J, Eapen CE, Kurian G, et al.
How accurate are the Swansea criteria to diagnose acute fatty liver of pregnancy in predicting hepatic microvesicular steatosis? Gut 2011;60:138-9.
[Table 1], [Table 2], [Table 3]