|Year : 2021 | Volume
| Issue : 2 | Page : 124-126
Acute fatty liver of pregnancy leading to a delayed hepatic failure necessitating liver transplantation: A case report
Patriot Yang, Rutuja R Sikachi, Madina Gerasimov, Judith Aronsohn, Gregory Palleschi
Department of Anesthesiology, Zucker School of Medicine at Hofstra/Northwell Program, Hempstead, NY, United States
|Date of Submission||16-Feb-2021|
|Date of Acceptance||05-Aug-2021|
|Date of Web Publication||01-Oct-2021|
Dr. Patriot Yang
300 Community Drive, Manhasset, NY - 1103
Source of Support: None, Conflict of Interest: None
Acute fatty liver of pregnancy (AFLP) is a potentially fatal metabolic disorder in pregnant patients that requires urgent delivery and aggressive medical and aesthetic management of maternal complications associated with acute liver failure. A 41-year-old female (79 kg) G1P0 at 31 weeks gestation presented with nausea, vomiting, pruritus, and jaundice. A diagnosis of severe liver dysfunction secondary to AFLP was made. We proceeded with urgent delivery under general anaesthesia. The patient had an uncomplicated caesarean section and gave birth to female infant with Apgar scores of 7 and 8. The patient remained stable for the following 2 weeks, however, given the lack of further recovery of hepatic function, a transjugular liver biopsy was performed, revealing persistent AFLP. She received N-acetylcysteine infusion and 4 cycles of plasma exchange with no improvement. Over the next few days her mental status worsened and her liver functions further deteriorated. She was listed for a deceased liver donor transplant and underwent successful orthotopic liver transplantation. She was discharged on post-operative day (POD) 14 of liver transplant.
Keywords: Acute fatty liver of pregnancy, liver failure, liver transplant
|How to cite this article:|
Yang P, Sikachi RR, Gerasimov M, Aronsohn J, Palleschi G. Acute fatty liver of pregnancy leading to a delayed hepatic failure necessitating liver transplantation: A case report. J Obstet Anaesth Crit Care 2021;11:124-6
|How to cite this URL:|
Yang P, Sikachi RR, Gerasimov M, Aronsohn J, Palleschi G. Acute fatty liver of pregnancy leading to a delayed hepatic failure necessitating liver transplantation: A case report. J Obstet Anaesth Crit Care [serial online] 2021 [cited 2021 Nov 28];11:124-6. Available from: https://www.joacc.com/text.asp?2021/11/2/124/327402
| Introduction|| |
Acute fatty liver of pregnancy (AFLP) is a potentially fatal metabolic disorder with a reported incidence of 1 to 3 cases per 10,000 deliveries. One of the most common findings in patients with AFLP is multiorgan fatty infiltration, which is theorized to increase fatty acids within the placenta, resulting in impaired oxygen delivery to the foetus.
This case report describes the medical and aesthetic management of a patient diagnosed with AFLP that rapidly progressed to acute decompensated liver failure, requiring emergent delivery and liver transplantation two weeks later.
| Case Description|| |
A 41-year-old female G1P0 at 31 weeks gestation, with past medical history of Factor V Leiden and gestational diabetes mellitus, presented with nausea and jaundice for 3 days. Her medication list included aspirin and enoxaparin in addition to prenatal vitamins and H-2 blockers.
Her initial set of labs showed haemoglobin (hgb) 13.8 g/dL, platelet count 289,000, total bilirubin 20.0 mg/dL, aspartate transaminase (AST) 2595 u/L, alanine transaminase (ALT) 1999 u/L, alkaline phosphatase 227 u/L, international normalized ratio (INR) 2.5, prothrombin time (PT) 30.1, and fibrinogen levels 314 mg/dL. Serum Acetaminophen level, a common cause of drug-induced liver injury, was normal. Viral hepatitis panel and autoimmune hepatitis markers were negative. A Doppler ultrasound study confirmed fatty infiltration with patent hepatic vasculature.
Based on the patient's normotensive blood pressure and normal platelet count, pre-eclampsia and HELLP Syndrome (Haemolysis/Elevated Liver Enzymes/Low Platelets) were unlikely. In addition, with normal platelet count and fibrinogen levels that were only mildly reduced, Disseminated Intravascular Coagulopathy (DIC) was less likely. Drug-induced liver injury from acetaminophen was ruled out from the negative history of acetaminophen ingestion and normal serum acetaminophen levels on the day of presentation Liver injury from hepatitis was less likely based on negative markers. Due to the patient's history of Factor V Leiden, thrombosis was also on the differential; however, the ultrasound showed patency of hepatic vasculature. Instead, the ultrasound showed fatty infiltration. This finding, along with clinical signs and symptoms, led to the diagnosis of severe liver dysfunction secondary to AFLP.
Following a multidisciplinary discussion that included obstetrician, herpetologist neonatologist and anaesthesiologist, the decision was made to proceed with urgent delivery via caesarean section.
Due to an elevated INR, the patient received 2 units of FFP (Fresh Frozen Plasma) preoperatively and general anaesthesia, rather than neuraxial anaesthesia, was induced followed by uneventful endotracheal intubation using propofol, fentanyl and succinylcholine. The patient was maintained on sevoflurane with oxygen and hydromorphone. Hemodynamic stability was measured with standard monitors. She delivered a 1.5 Kg female neonate with Apgar scores of 7 and 8 at 1 and 5 minutes respectively. The neonate was admitted to NICU for thermal dysregulation and poor feeding and discharged home after 3 weeks in care of father and maternal grandmother. Post-operative analgesia for mother was accomplished with intermittent boluses of hydromorphone.
Over the next 2 weeks, the patient remained stable but the hepatic function did not show a significant recovery. A transjugular liver biopsy demonstrated extensive microvesicular steatosis with centrilobular hepatocyte dropout, indicating persistent AFLP.
On Post-operative day (POD) 15 of caesarean section, the patient experienced further worsening of hepatic function with MELD-Na score of 37 and INR of 4.58. She received N-Acetylcysteine infusions, 24g over 24 hours and 4 cycles of plasma exchange with no improvement. Due to further worsening of mental status and liver function parameters (serum ammonia levels 131 μmol/L, INR 5.5, lactate 4.2), she was listed in a diseased donor transplant program as a high priority on POD 19. On the same day the patient underwent deceased donor orthotopic liver transplantation.
Briefly, general anaesthesia was induced with propofol and succinylcholine. Radial arterial line and a 9 Fr multi-lumen left internal Jugular catheter were inserted. In anticipation of intraoperative veno-venous bypass, 16Fr venous cannulas were inserted in the right Internal Jugular and left Femoral veins. In order to reduce intracerebral pressure and the risk of cerebral enema, mannitol was administered. Anaesthesia was maintained with fentanyl, propofol and rocuronium infusions. Arterial blood gas analysis was performed at frequent intervals and electrolytes were repleted as needed. Thromboelastography (TEG) was used to guide blood product replacement. Over the 12 hour of operating time, the patient received 2800 ml of crystalloids, 750 ml of 5% albumin, 8 units Packed Red Blood Cells plus 300 ml of blood from cell saver, 8 units FFPs, 3 units platelets and 4 units cryoprecipitate. The total urine output was 2700 mL and estimated blood loss was approximately 3L. Intraoperative surgical findings included 3 litres of yellow coloured ascites and small, firm and shrunken liver, thus confirming the diagnosis. She was successfully extubated the following morning and discharged home on POD 14 of liver transplant.
| Discussion|| |
Risk factors for AFLP are thought to include multiple gestation, male foetus, fatty acid oxidation disorders in the foetus or previous episode of AFLP. The mortality rate for AFLP has significantly improved over the last decades. In the 1980's, these rates were estimated to be up to 70%, but more recent reports are closer to 2%, likely secondary to early diagnosis, improved medical, surgical and aesthetic management.
The pathophysiology of AFLP is related to hormonal changes during normal pregnancy that lead to a physiologic decrease in the oxidation of long and medium chain fatty acids. This results in an increase in maternal serum fatty acids over the course of gestation, increasing the maternal susceptibility to a burden of free fatty acids that act as hepatotoxins. One of the most common findings in patients with AFLP is multiorgan fatty infiltration. Widespread microvesicular fatty steatosis of the liver impairs hepatic production of cholesterol, fibrinogen and coagulation factors and decreases bilirubin conjugation and clearance.
This process results in abnormalities seen in acute liver failure: elevated liver enzymes, hyperbilirubinemia, elevated ammonia. Other abnormalities include hypofibrinogenemia, hypocholesterolaemia, hypoglycaemia, hyperuricemia, elevated creatinine and blood urea nitrogen. Though thrombocytopenia can be seen in both AFLP and HELLP Syndrome, hypoglycaemia, absence of hypertension, and coagulopathy are clinical features that can help distinguish the former from the latter. In addition, acute liver failure is rarely seen in other differential diagnoses like hyperemesis gravidarum and intrahepatic cholestasis of pregnancy; liver dysfunction is often mild.
Our patient satisfied ten features on the Swansea criteria. A minimum of six of the following features in the absence of another explanation suggests the diagnosis of AFLP: vomiting, abdominal pain, polydipsia/polyuria, encephalopathy, elevated bilirubin, hypoglycaemia, elevated urate, leucocytosis, ascites or bright liver on ultrasound, elevated transaminases, elevated ammonia, renal impairment, coagulopathy, and/or microvesicular steatosis on liver biopsy.
The patient successfully delivered via caesarean section under general anaesthesia, but her liver function failed to improve, and she progressed to fulminant liver failure in the following two weeks, necessitating emergent liver transplantation that was successfully performed at our institution. Without a liver transplant, the patient would likely not have survived as other bridging treatments like N-acetylcysteine infusion and plasma exchange were already used and exhausted. This timeline of disease progression sets our case apart from other reports of AFLP leading to liver transplantation., According to published case reports, patients with AFLP experience complete recovery of liver function within a few days of delivery with no evidence of cirrhosis or chronic hepatitis. Rather, a majority of patients will have normal liver function tests by 4 to 8 weeks post-delivery.
Management focuses on prevention of hypoglycaemia, correction of coagulopathy, and prevention and treatment of cerebral oedema/intra-cranial hypertension.
Acute liver failure from any cause is associated with decreased production of coagulation factors as well as a decrease in procoagulant proteins, leading to coagulopathy in coexistence with hypercoagulability. A functional platelet defect may also occur secondary to uraemia and endothelial abnormalities and is related to the degree of liver dysfunction. Therefore, excessive bleeding should be anticipated during any surgical procedure and adequate vascular access and appropriate repletion of blood products are crucial when taking care of these patients.
Hepatic encephalopathy is frequently seen in AFLP and can range from mild to severe with increased intracranial pressure. Cerebral oedema and intracranial hypertension contribute significantly to the morbidity and mortality associated with AFLP.
Management of this patient presented several challenges. Neuraxial anaesthesia is typically favoured for the operative delivery but the coagulopathy and increased intracranial pressure presented potential risks associated with this approach in our patient. On the other hand, general anaesthesia carries the risk of difficult airway, pulmonary aspiration, exacerbation of hepatic dysfunction and intracranial hypertension.
In conclusion, a parturient with AFLP who developed hepatic encephalopathy with profound coagulopathy and underwent a orthotopic liver transplantation is described here. This case report highlights the need for close observation and continuous vigilance when taking care of patients with AFLP.
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Conflicts of interest
There are no conflicts of interest.
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