Hepatic manifestations of HELLP syndrome

An otherwise healthy 24-year-old patient, 36-week pregnant, was admitted to obstetrics/gynaecology for severe preeclampsia (arterial blood pressure: 140/100 mmHg, 3+ proteinuria) and epigastric pain, scotomas, and headache. Labs before delivery showed high AST/ALT (306/422 units/litre [normal: 9–25/6–30]) and low platelets (78x10⁹ platelets/litre [normal: 130–380x10⁹]). Epigastric pain and lab abnormalities persisted 3-day postpartum with new-onset jaundice.

Imaging was first obtained 3-day postpartum with an abdominal ultrasound. The liver parenchyma was heterogeneous, and several distinct hypoechoic lesions were identified within the liver (Figure 1). Doppler findings were unremarkable, showing normal colour and flow signals. The lesions were suspected to be acute changes, given the patient’s bloodwork and jaundice, consistent with HELLP syndrome.

Computed tomography (CT) imaging was then obtained for further characterisation of the liver and to strengthen the diagnosis. It demonstrated hepatomegaly and heterogeneous attenuation of its parenchyma. There were multiple, irregular heterogeneous, predominantly hypodense lesions with geographical outlines distributed in both hepatic lobes (Figures 2a and 2b). Some of these extended peripherally into the subcapsular location. Mild periportal oedema was also noted (Figure 3).

Background

HELLP syndrome is a rare, life-threatening complication of pregnancy, which presents between the second trimester to shortly after delivery [1]. It is characterised on bloodwork by haemolysis, elevated liver enzymes and thrombocytopenia. The pathogenesis involves endothelial damage triggering a proinflammatory state and hyperactivation of the coagulation cascade [2,3]. Liver damage occurs secondary to factors including hepatic sinusoidal obstruction, intrahepatic vascular congestion, reduced portal blood flow, fibrin deposits and placenta-derived factors [1–4]. Intrahepatic lesions may cause ischemia and stasis within surrounding biliary tributaries [5]. Long-term, this can result in bile duct dilation, inflammation, fibrosis and stricturing (ischemic cholangiopathy) [6].

Clinical Perspective

Clinical symptoms of HELLP syndrome are nonspecific. Patients frequently present with colicky epigastric or right upper quadrant pain with nausea and/or vomiting [4]. Other symptoms include headache, scotomas, and jaundice [1,7]. It more commonly affects multiparous or primiparous women > 34 years old. Additional risk factors include pre-existing and gestational diabetes and/or hypertension [8].

Imaging Perspective

Imaging findings in HELLP syndrome are nonspecific and may include subcapsular or perihepatic haematoma(s), hepatic haemorrhage/rupture and/or hepatic infarct(s) [9]. On ultrasound, haematomas are usually hyperechoic acutely in the clotting phase [10]. A more hypoechoic appearance may manifest by days 4–5 [10–12]. On CT with intravenous contrast, haematomas typically appear as non-enhancing diffuse or wedge-shaped regions demonstrating reduced attenuation relative to adjacent parenchyma [11,13]. Active haemorrhage is usually easily detectable. In contrast, hepatic infarcts present peripherally as sizable but ill-defined hypoechoic lesions on ultrasound and low attenuation lesions on CT, without mass effect [11,14–16]. Subtle focal enhancement can be seen secondary to hepatic vasculature coursing through the lesions [11,14]. Occasionally, more pronounced hypoattenuation is seen on post-contrast CT imaging.

Outcome

HELLP syndrome may be life-threatening for the mother and neonate, with a maternal and perinatal death rate of up to 24% and 37%, respectively [17]. Early detection is crucial to mitigate outcomes. Following attempted delivery, supportive measures include respiratory support, hypertension management, analgesia, and fluid resuscitation [1]. In cases of liver rupture, surgical intervention or transplant may be indicated [18].

Despite the resolution of the initial hepatic haematomas (confirmed by ultrasound), our patient developed chronic ischemic cholangiopathy with multiple ischemic strictures involving the intra/extra hepatic biliary tree leading to recurrent attacks of cholangitis (Figures 4a, 4b and 5). This resulted in cirrhosis (Figure 6) and portal hypertension. She underwent a liver transplant two years after the initial presentation.

Take Home Message / Teaching Points

HELLP syndrome is a rare, potentially fatal outcome of pregnancy. Clinical presentation is non-specific. Therefore, if clinically suspected, prompt imaging of the liver can be useful in diagnosis and facilitating timely management.

All patient data have been completely anonymised throughout the entire manuscript and related files.