The Deadly Masquerade: Unravelling Acute Fatty Liver of Pregnancy

Abstract

Background: Acute fatty liver of pregnancy (AFLP) is a rare but life-threatening obstetric emergency that typically occurs in the third trimester or early postpartum period. Its early clinical manifestations are often non-specific and may overlap with infectious, hepatic, and other pregnancy-related disorders, making timely diagnosis challenging. Delayed recognition can lead to rapidly progressive hepatic failure, coagulopathy, renal impairment, and multi-organ dysfunction. Aims: To report a rare case of fulminant acute fatty liver of pregnancy (AFLP) with an atypical presentation, highlighting diagnostic challenges and the importance of early recognition. Presentation of Case: A 33-year-old primigravida at 32 weeks' gestation presented with a persistent pruritic maculopapular rash, initially managed as an allergic condition. She subsequently developed fever, vomiting, diarrhoea and arthralgia. Initial investigations suggested sepsis with acute kidney injury, hepatic dysfunction and coagulopathy. Despite antibiotic therapy and supportive management, she rapidly deteriorated with progressive liver failure, metabolic acidosis and multi-organ dysfunction. AFLP was diagnosed on day four of admission based on the Swansea criteria following multidisciplinary discussion. Unfortunately, during pre-operative stabilisation, intrauterine death with abruptio placenta was detected, necessitating emergency caesarean section. Postpartum, she developed disseminated intravascular coagulation, haemorrhagic pancreatitis, pulmonary haemorrhage, bilateral pleural effusions and refractory acute kidney injury requiring continuous renal replacement therapy. Plasma exchange was attempted but discontinued because of haemodynamic instability. Liver histopathology confirmed diffuse panlobular microvesicular steatosis consistent with AFLP. Despite aggressive multidisciplinary management, she succumbed to multi-organ failure. Discussion: AFLP is a life-threatening obstetric emergency whose non-specific early manifestations frequently delay diagnosis. This case demonstrates that AFLP may be obscured by competing clinical features. Prolonged pruritus in late pregnancy should prompt consideration of pregnancy-related hepatic disorders, particularly when accompanied by abnormal liver biochemistry. Conclusion: Early recognition of AFLP requires a high index of suspicion. Once AFLP is suspected, prompt multidisciplinary management and immediate delivery are essential; however, severe disease may progress despite optimal treatment, highlighting the importance of timely diagnosis and intervention.