Preeclampsia and HELLP syndrome complicated by subcapsular liver hematoma and rupture

Discussion

Hypertensive disorders of pregnancy, including severe manifestations such as preeclampsia, are increasingly common, with a reported doubling in prevalence between 2000 and 2018 in the United States.1 These disorders exist on a spectrum, including chronic or pre-existing hypertension, gestational hypertension and preeclampsia, with further characterization based on associated complications such as HELLP syndrome. According to the most recent guideline from the Society of Obstetricians and Gynecologists of Canada (SOGC), preeclampsia is diagnosed based on when hypertension is present with any one or more of new proteinuria; an adverse condition affecting the central nervous system, cardiorespiratory, hematologic, renal, hepatic or fetoplacental systems; or severe end-organ complications (SOGC Guideline No. 426 includes full diagnostic criteria).2 The syndrome of HELLP is an uncommon complication in pregnancy, affecting 1% of pregnancies but occurring in 10%–20% of patients who develop preeclampsia.3 It is typically associated with hypertension; however, 10%–20% of patients are normotensive. 4 Our patient met criteria for both preeclampsia (based on her elevated blood pressure and multiple adverse conditions and complications) and HELLP (based on evidence of hemolysis, elevated liver enzymes and low platelets), complicated by subcapsular liver hematoma. The initial biochemical abnormalities may have been explained by hemorrhage and prerenal acute kidney injury with intravascular depletion. However, the evolution of worsened anemia, thrombocytopenia, elevated liver enzymes and evidence of hemolysis, in parallel with development of hypertension, was most consistent with a unifying diagnosis of severe preeclampsia and HELLP syndrome, with associated complications of each including acute kidney injury, subcapsular liver hematoma and thrombosis.

Subcapsular liver hematoma is an uncommon complication of HELLP syndrome, reported in less than 2% of pregnancies with HELLP syndrome.5 It can progress to liver rupture or infarct, which can have devastating consequences for both the patient and baby if not promptly recognized and managed.5 Patients can present with dyspnea; shoulder, back or epigastric pain; anorexia; or nausea and vomiting, as was the case for our patient.5 Our patient was managed collaboratively by specialists in obstetrics and gynecology, general surgery, interventional radiology and obstetric internal medicine from an operative and medical perspective, given the atypical presentation, severe manifestations requiring emergent intervention and incidental finding of partially occlusive gonadal vein thrombosis.

The management options for subcapsular liver hematoma range from a conservative approach (i.e., hemodynamic support, transfusion and reversal of coagulopathy), to minimally invasive transcatheter hepatic artery embolization, to surgical intervention with packing, parenchymal suturing, resection or hepatic artery interruption.6 Some patients require liver transplantation.6 A recent systematic review reported maternal and fetal survival rates of 85% and 59% respectively, suggesting that these rates have remained largely unchanged over the last decade.7 However, this is an improvement when compared with data collected before 2003, which estimated a maternal survival rate of 61%.7 In a 2021 review of 73 patients with subcapsular liver hematoma, the best maternal and fetal survival rates were observed among those treated with hepatic artery embolization. 7 A minimally invasive approach with embolization should therefore be strongly considered as the first intervention if clinical stability allows, highlighting the importance of performing CT angiography in patients with HELLP syndrome with abdominal pain or elevated liver enzymes to facilitate timely diagnosis and management. 7 Computed tomography angiography provides both diagnostic information (particularly among patients who present with abdominal pain and abnormal liver enzymes, but who do not have indication for urgent cesarian delivery) and opportunity for minimally invasive therapy, and can be used to identify other pathology that may explain or be associated with the patient’s presentation. Open surgical intervention has been suggested as a rescue option for patients who are clinically unstable or when CT angiography is unavailable.7

Gonadal vein thrombosis is rare, with an incidence of 0.05%–0.16% in all pregnancies, most commonly in the postpartum period. Of these thromboses, 90% occur in the right gonadal vein because of its length and dextrorotation of the gravid uterus, resulting in compression. 8 Risk factors include infection and cesarian delivery, particularly with the birth of multiples.9 No consensus exists in the literature to guide treatment, and a recent review found no statistically significant correlation between use of anticoagulation and resolution of thrombosis.8 The authors of the review suggested treatment only in the presence of symptoms, such as septic thrombophlebitis and concurrent deep vein thrombosis or pulmonary embolism. Current SOGC recommendations presume an infectious trigger for gonadal vein thrombosis and support the use of broad-spectrum antibiotics, as well as consideration of therapeutic anticoagulation.9 The decision to treat with anticoagulation must be made on an individual basis, depending on the provoking factor, location and extent of thrombosis. For our patient, the left-sided location could suggest that the inflammatory and hypercoagulable states of preeclampsia and HELLP syndrome were more influential predisposing factors than anatomic changes associated with pregnancy. Based on the risk of recurrent bleeding in the setting of subcapsular liver hematoma and rupture, multifactorial anemia with evidence of hemolysis, the partially occlusive clot burden, the lack of extension into the inferior vena cava and the lack of evidence for an infectious trigger, we decided not to treat with anticoagulation or antibiotics.

We present a severe case of a patient with preeclampsia and HELLP — complicated by acute kidney injury, subcapsular hematoma and thrombosis — who initially presented with normal blood pressure, tachycardia and nausea and vomiting, and was found to have a hemoperitoneum intraoperatively. This case highlights the importance of having a high index of suspicion for preeclampsia or HELLP syndrome for patients with red-flag symptoms such as upper abdominal pain, headache, visual changes, altered mental status or dyspnea, and early biochemical signals, even in the absence of hypertension at presentation. Clinicians should consider prompt imaging and minimally invasive intervention if patient stability, resources and time permit.