“Hepatic rupture in preeclampsia and HELLP syndrome: A catastrophic presentation”

The objective of index study is to review the available literature on hepatic rupture or hematoma in hypertensive disorders of pregnancy to find the incidence, associated risk factors, clinical presentation, mode of management and feto-maternal outcome. Electronic database was searched using hepatic rupture or hematoma in pregnancy, preeclampsia, eclampsia, and HELLP syndrome (Hemolysis, EL: elevated liver enzymes, LP: low platelet count) as key words and literature published since January, 2000 to December, 2018 which met the inclusion criteria was reviewed. A total of 56 articles were reviewed describing 93 cases of hepatic hemorrhage in hypertensive disorders of pregnancy. Treatment varied from conservative management to abdominal packing, hepatic artery embolization, and partial hepatectomy to liver transplantation. Seven out of 93 patients with liver rupture met mortality and in one of them diagnosis was established on autopsy. Unawareness of the hepatic rupture in pregnancy by an obstetrician demands high index of suspicion for diagnosis and requires specialized, focused and exhaustive management for optimal feto-maternal outcome. Laparotomy and perihepatic packing is a viable option in patients with unstable vitals and is feasible even in limited resource settings.Short interval between diagnosis and management may enhance the feto-maternal survival rate and prevent further morbidity or mortality.     

Hepatic rupture associated with preeclampsia,report of three cases and literature review

Background: Hepatic rupture is a complication during pregnancy that, although rare, accounts for high morbidity and mortality rates. It is mainly associated with severe preeclampsia and HELLP syndrome. Incidence is estimated to be at one per 67,000 births or one per 2000 patients with preeclampsia/eclampsia/HELLP, mainly in multiparous women; women in their 40s; after 32 weeks of gestation; and during the first 15?h postpartum.

Cases: This article exposes the institutional experience at Fundación Valle del Lili in Cali, Colombia, in managing and treating hepatic rupture associated with severe preeclampsia and HELLP syndrome in three patients in the 30th, the 26th, and the 27th week of gestation, not resulting in maternal death.

Discussion: A search in Pubmed, Embase, and Ovid from 2000 to 2017 resulted in 35 cases reported in either pregnant or puerperal women. Hepatic rupture is a rare complication in pregnancy associated with preeclampsia and HELLP syndrome. Its pathophysiology is attributed to the presence of vasospasm due to an increase in concentration and sensitivity to circulating vasopressors during pregnancy. There is no standard management, but surgery reduces mortality significantly. It includes endovascular management, partial hepatectomy, or transplant (only one patient required a liver transplant in our search). The most used techniques have been ligation of the hepatic artery, embolization of the hepatic artery, and examination, packing, and drainage of hepatic lesion for bleeding control (27 cases were treated with laparotomy with evacuation of hematoma and hemostasis and four cases were treated with embolization of the hepatic artery). Hepatic artery occlusion both by surgery ligation and by embolization through interventional radiology has reported successful and failing results during pregnancy

Conclusion: Management of pathologies as hepatic rupture associated with severe preeclampsia and HELLP syndrome has to be clearly protocolized for prompt diagnosis and early management. Furthermore, it has to be carried out through multidisciplinary teams in high-complexity obstetrics scenarios.     

Pre-eclampsia/eclampsia and hepatic rupture

ObjectiveTo review case reports of hepatic hematoma/rupture in women with pre-eclampsia/eclampsia.MethodsMEDLINE, SciELO, and LILACS databases were searched for case reports of pre-eclampsia/eclampsia with hepatic hematoma/rupture. Only articles written in English, Spanish, French, or Portuguese and published between 1990 and 2010 were reviewed.ResultsIn total, 180 cases of hepatic hematoma or rupture were identified: 18 (10.0%) with subcapsular hematoma without hepatic rupture; and 162 (90.0%) with capsule rupture. Twelve (6.7%) cases were associated with eclampsia plus hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome. Average age was 30.9 ± 5.0 years, 74/129 (57.4%) women were parous, and cesarean delivery was performed in 132/162 (81.5%) cases. The right lobule was the most frequently affected 77/100 (77.0%). The total maternal mortality rate was 22.2% during the 21 years; however, it decreased to 16.4% in the last decade studied. The perinatal mortality rate was 30.7% and was very similar during the 2 decades.ConclusionHELLP syndrome is a frequent diagnosis (92.8%) in hepatic hemorrhage/rupture. The major reduction in maternal mortality rate was probably associated with advances in resuscitation, intensive-care medicine, and surgical intervention, including liver transplantation and arterial embolization.     

Prediction of HELLP syndrome with assessment of maternal dual hepatic blood supply by using Doppler ultrasound

Objective: Early structural and functional changes in the systemic vasculature have been proposed to play a major pathogenetic role in HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome. Our objective was to assess whether the evaluation of maternal hepatic blood supply is instructive to the prediction of onset of HELLP syndrome. Design: Prospective observation study. Population: Fifty-eight women with severe preeclampsia and 60 healthy pregnant controls at 25–36 weeks gestation. Methods: Angle-corrected time-averaged flow velocity and the cross-sectional area of common hepatic artery and portal vein were measured by using Doppler ultrasonography in 58 women with severe preeclampsia and in 60 healthy pregnant controls at 25–36 weeks gestation. Intravascular flow volumes were calculated from the product of the time-averaged velocity and the cross-sectional area. The total liver blood flow was taken as the sum of flow volumes in the hepatic artery and portal vein. Results: The total liver blood flow decreased significantly to about 40% of control in 9 women with severe preeclampsia who developed HELLP syndrome within 4 days after the examination, but not in 49 women with severe preeclampsia without HELLP syndrome. Conclusion: The results indicated that the decrease in dual hepatic blood supply preceded the onset of HELLP syndrome.     

Hepatic rupture associated with pregnancy: treatment with transcatheter embolotherapy

Spontaneous rupture of the liver associated with pregnancy is a rare and very serious complication, usually occurring in association with eclampsia or preeclampsia. Survival has generally been dependent on early recognition of characteristic signs and symptoms and prompt surgical intervention. Even with surgery, maternal mortality approaches 40% and fetal mortality is even higher. The diagnosis can usually be firmly established based on the clinical and radiographic findings presented in this article. Reported is a patient with hepatic rupture successfully treated by transcatheter embolization of the hepatic artery. It is the authors' belief that if such a patient is clinically stable enough to undergo angiography, then transcatheter embolotherapy is a reasonable alternative to surgery.     

Modified ISTH pregnancy-specific DIC score in parturients with liver rupture: population-based case series

Objectives: Liver rupture and hematoma are rare life-threatening complications of pregnancy. The aims of the current study are to: (1) characterize in a population-based study all cases of liver hematoma and/or rupture; and (2) validate the utility of the International Society on Thrombosis and Haemostasis (ISTH) modified pregnancy specific disseminated intravascular coagulation (DIC) score in those cases.

Study design: A retrospective cohort study including all patients with liver subcapsular hematoma or rupture between the years 1996 and 2012 was conducted. Information on maternal characteristics, clinical presentation, diagnostic studies, therapeutic modalities, as well as maternal and fetal outcomes was collected. The pregnancy-specific modified ISTH DIC scores were calculated from admission to discharge, a score >26 is suggestive of DIC.

Results: Out of 175,000 births in our database, seven patients were identified with liver rupture or subcapsular hematoma, representing a prevalence of 4:100,000 deliveries. Of those, six had liver rupture and one had subcapsular liver hematoma. One patient died of hemorrhagic shock. Four patients underwent surgical liver packing and one also underwent hepatic artery ligation. Four out of seven patients were diagnosed during the immediate postpartum period with severe features of preeclampsia or with hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome. Modified ISTH pregnancy-specific DIC scores were calculated for five out of seven patients, and three (60%) had a score higher than 26. Patients with higher scores received more blood product transfusions, had longer hospitalizations, and their neonates had lower 1 and 5?minutes Apgar scores.

Conclusions: Elevated pregnancy-specific modified ISTH DIC score (>26) in patients with liver hematoma or rupture was associated with adverse maternal and neonatal outcomes and appeared to perform well in distinguishing high and low-risk cases. Postpartum preeclampsia may be associated with severe features and a more complicated disease course.     

HELLP syndrome complicated by liver rupture

The object of our study was the liver rupture as a complication of HELLP syndrome. We described a case of 27-year-old primigravida who underwent the cesarean section because of HELLP syndrome, followed by four laparotomies, due to capsular hematoma and liver rupture. Surgery with packing, argon coagulation or embolization of hepatic artery remain the only treatment for subcapsular hematoma and liver rupture. The main issue is to state the early diagnosis of HELLP syndrome because its clinical presentation at the beginning is very unclear and diagnosis is often delayed. It is important because the morbidity and mortality rates associated with the syndrome have been reported to be as high as 25 percent.     

Assessment of the hepatic arterial and portal venous blood flows during pregnancy with Doppler ultrasonography

Purpose: The aim of this study was to evaluate whether the dual hepatic blood supply is altered in healthy pregnant women compared with that in nonpregnant women. Materials and methods: Flow waveforms in common hepatic artery and portal vein were obtained in 67 healthy pregnant women at 10–40 weeks gestation and 22 nonpregnant women by using Doppler ultrasonography. Results: In the nonpregnant group, the mean (SD) hepatic arterial blood flow, portal venous blood flow, and total liver blood flow were 0.57 (0.31) L/min, 1.25 (0.46) L/min, and 1.82 (0.63) L/min, respectively. In the healthy pregnant group, the portal venous blood flow and total liver blood flow significantly increased after 28 weeks gestation. However, the hepatic arterial blood flow remained unchanged during pregnancy. There was no relationship between the hepatic arterial blood flow and the portal venous blood flow. Conclusion: The results demonstrated that the hepatic perfusion increased during third trimester compared to nonpregnant level. Because the hepatic arterial blood flow remained unchanged during pregnancy, major determinant of the increase in the hepatic perfusion was the portal venous return. The data suggest that the hepatic arterial and portal venous vascular territories have regulatory mechanisms that allow for independent changes during pregnancy. Received: 8 January 2001 / Accepted: 22 February 2001     

Spontaneous rupture of liver during pregnancy: current therapy

Spontaneous hepatic rupture secondary to severe pregnancy-induced hypertension is associated with a high rate of maternal and fetal mortality. Numerous types of surgical management have been described, but a uniform surgical approach has not been accepted. The purpose of this review was to examine modes of surgical therapy reported in the literature since 1976, as well as the 11-year experience at our institution. Twenty-eight cases were extracted from the literature and seven more were identified at our institution. The incidence in our population was one per 45,145 live births. Among 27 cases managed by packing and drainage, an 82% overall survival was achieved, whereas only 25% of eight patients undergoing hepatic lobectomy survived (P = .006). Hepatic hemorrhage with persistent hypotension unresponsive to blood products should be managed by evacuating the hematoma, packing the damaged liver, and draining the operative site. More aggressive surgical techniques, such as hepatic artery ligation or hepatic lobectomy, should be reserved for refractory cases.     

The pathology of eclampsia: An autopsy series

Objective: We describe the main lesions in the liver, brain, and kidney from autopsies of women who died of eclampsia and characterize the endothelial injury.

Methods: Cases were identified from a study involving 317 maternal deaths (2003–2006) conducted at the Maputo Central Hospital (Maputo, Mozambique) in association with ISGlobal (Barcelona, Spain). Histology slides along with stains for endothelial, histiocyte, and platelet markers (CD31, CD34, CD68, CD42B) were reviewed to identify the relevant lesions. Malondialdehyde stain was performed to demonstrate free radical generation.

Results: Brain lesions were characterized by perivascular “edema” (68.4%), hemorrhage (36.8%), hemosiderin (31.6%), small vessel thrombosis (10.5%), and parenchymal necrosis (15.8%). Liver sections showed periportal/portal necrosis and sinusoidal fibrin (72.2%) with associated hepatic arterial medial necrosis (44.4%). Kidneys showed glomerular endotheliosis. Endothelial, histiocytic, and platelet markers highlighted capillary injury in the otherwise intact brain parenchyma. Stains for free radical formation were positive predominantly in the areas of tissue injury, but intact glial/neuronal elements were focally positive as evidence of widespread oxidative stress.

Conclusion: Pathological changes in cases of eclampsia include widespread endothelial/vascular injury in vulnerable organ beds.     

The Importance of Parity to Major Maternal Morbidity in the Eclamptic Mother with Hellp Syndrome

Objective: To determine if the rate of major morbidity from severe preeclampsia with/without hemolypis, elevated liver enzymes and low platelets (HELLP) syndrome differs by parity. Methods: Retrospective investigation of 970 gravidas with severe preeclampsia with and without HELLP syndrome analyzed according to parity. Results: Altogether 609 (63%) patients were nulliparous and 361 (37%) parous. Between groups there was no significant difference in the incidence of overall major morbidity (21% vs 19%, p = 0.467), or specific morbidities including hematologic/coagulopathic (13.6% vs 11.9%, p = 0.442), cardiopulmonary (8.9% vs 7.2%, p = 0.362), CNS/visual (1.8% vs 2.8%, p = 0.319), or hepatorenal (0.8% vs 2.2%, p = 0.068). Although eclampsia was significantly more common in nulliparas (10.2%) than in parous patients (5.5%, p = 0.012), the latter significantly more often demonstrated major maternal morbidity associated with eclampsia (50%) than did nulliparous patients (25%, p = 0.043). Conclusions: Unless parous patients with severe preeclampsia with or without HELLP syndrome develop eclampsia, their disease acuity does not differ significantly from their nulliparous counterparts.     

Maternal deaths associated with hypertensive disorders of pregnancy: a population-based study.

There were 507 deaths associated with hypertensive disorders of pregnancy (eclampsia, preeclampsia, and chronic hypertension) in South Africa over the triennium 1999-2001. Eclampsia was associated with 289 deaths, preeclampsia with 139, and the remaining 79 with chronic hypertension, hemolysis, elevated lever enzymes, and low platelet count (HELLP) syndrome, liver rupture and acute fatty liver. The major final cause of death was intracranial hemorrhage. Other causes included HELLP syndrome and liver rupture. Contributory causes include pulmonary edema, renal failure/impairment, and disseminated intravascular coagulation. Deaths from eclampsia occurred at all levels of health care, in particular, there was still a considerable number of deaths at level I hospitals. Most deaths from eclampsia occurred at low parity (parity 0 = 51%), while 13% of deaths in noneclamptics occurred in women of parity > or = 5. Similarly, most deaths from eclampsia occurred in women aged < or = 24 years, while most in the noneclamptic group were aged 25 years and greater. The most common avoidable factors were patent-oriented problems--women who either presented late for antenatal care or late to hospital when symptomatic. Administrative factors also played a major role, in that there was a delay in referral due to the unavailability of transport. The lack of protocols of management or failure to follow clinical protocols of care contributed towards avoidable medical factors. Most women presented as an emergency event and failure of resuscitation/achievement of hemodynamic stabilization constituted a significant avoidable factor. Clear protocols for management of hypertension in pregnancy at all levels of health care are required.     

Pre-myomectomy uterine artery embolisation minimises operative blood loss

Women with massive fibroids (extending beyond the level of the umbilicus) are conventionally offered a hysterectomy, rather than myomectomy, which is considered too technically challenging, with risks of excessive haemorrhage. Some women desire fertility, or may simply wish to preserve their uterus. Uterine artery embolisation is a relatively new treatment for fibroids, and complication rates are thought to be high with massive fibroids. We have performed uterine artery embolisation immediately prior to myomectomy, and found a reduction in blood loss. Uterine artery embolisation may be a useful adjunct to surgery in women with massive fibroids or for whom uterine artery embolisation alone is considered inadequate primary treatment, those with previous myomectomy where surgery might be complicated by extensive adhesions, in Jehovah's Witnesses and in other women who refuse blood transfusion.     

Pre-eclampsia-eclampsia admitted to critical care unit

Objective: To evaluate women with hypertensive disorder admitted to critical care unit. Methods: This study was carried out in Cartagena, Colombia, between January 2006 and December 2009. Patients were divided into 4 groups; severe pre-eclampsia, eclampsia, HELLP syndrome and HELLP with eclampsia (HEEH). Result: A total of 217 cases were admitted. The admitting diagnoses were severe pre-eclampsia without HELLP syndrome (39.2%), HELLP syndrome without eclampsia (33.6%), eclampsia without HELLP syndrome (20.3%) and Eclampsia with HELLP syndrome or HEEH (6.9%). Groups were similar with respect to parity (p = 0.25), gestational age (p = 0.11), cesarean section (p = 0.58), mechanical ventilation (p = 0.54), level of systolic (p = 0.48) and diastolic blood pressure (p = 0.15) and inotropic support (p = 0.32). Average total duration of hospitalization was significantly different among groups, more time in women with HEEH (p = 0.001). Multiple organ dysfunctions was diagnosed > 70% of all women admitted to intensive care, but was significantly more frequent in patients with HELLP syndrome and HEEH (p = 0.001). There were 5 maternal deaths (2.3%). Causes of maternal death were intracranial hemorrhage (3), intra-abdominal bleeding (1) and pulmonary complications (1). Conclusion: Women with HELLP syndrome with or without eclampsia are associated with major morbidity and mortality. Therefore, the maternal outcome in eclampsia is influenced for HELLP syndrome.     

The effects of maternal weight and age on pre-eclampsia and eclampsia in Haiti

Objective: Patient data from Maison de Naissance (MN), a rural maternity clinic in Haiti, were analyzed to determine the prevalence of pregnancy-related hypertensive disorders and the extent to which maternal weight and age are associated with these disorders in the MN population.

Methods: A case-control study design was used with cases defined as pregnant women who were presented at MN with pregnancy-related hypertensive disorders (pregnancy-induced hypertension, pre-eclampsia or eclampsia) and controls defined as those women who delivered babies at MN and were not diagnosed with a pregnancy-related hypertensive disorder. The final cohort size was 622 controls and 67 cases. Odds ratios were calculated using multivariate logistic regression.

Results: The incidence of pre-eclampsia and eclampsia was 7.0%. Older maternal age at delivery (OR?=?3.18; 95%CI: 1.31, 7.76) and higher maternal weight (OR?=?3.24; 95%CI: 1.76, 5.98) measured during prenatal care were significantly associated with pregnancy-related hypertensive disorders. Prenatal care was not significantly associated with reduced risk of pre-eclampsia/eclampsia.

Conclusions: The prevalence of pregnancy-related hypertensive disorders was high relative to rates in other developing countries. More is required to reduce the rate of pre-eclampsia perhaps by targeting older and women with high weight for preconception and more intensive prenatal care.     

Comparison of outcomes after typical and atypical eclampsia: a retrospective study

Objective: To evaluate the characteristics, clinical features and maternal–perinatal outcomes after atypical eclampsia. Methods: In a retrospective study, we compared demographics, clinical characteristics and outcomes between typical and atypical eclampsia. Results: Of 90 eclamptic patients, 56 had typical eclamptic features and 34 had atypical features. Compared to typical eclampsia, atypical eclampsia had higher gestational age (37.6?±?3.3 vs. 34.6?±?4.2 weeks, p = 0.001), a higher incidence of no antenatal risk factors [25 (73.5%) vs. 12 (21.4%), p < 0.001], less antepartum seizures [11 (32.4%) vs. 45 (80.4%), p < 0.001], a lower incidence of prodromal symptoms [20 (58.5%) vs. 49 (87.5%), p = 0.002], and a higher incidence of no lesion in brain imaging [16 (47.1%) vs. 12 (21.4%), p = 0.010). Although atypical eclampsia was associated with a lower odd ratio (OR) in composite perinatal complications (OR = 0.22, 95% CI = 0.08–0.60, p = 0.003), composite maternal complications did not differ between the two groups (OR = 0.52, 95% CI = 0.08–0.60, p =0.191). Conclusions: Maternal outcomes did not differ between the two groups. Therefore, more attention should be focused on atypical eclampsia.     

Postpartum spontaneous rupture of a liver hematoma associated with preeclampsia and HELLP syndrome

We report the case of a 28-year-old nulliparous woman who presented at 36 weeks’ gestation with preeclampsia: high blood pressure, proteinuria and edema. Serum test results were normal and platelet count was 155 × 10³/mm³. After 30 h the patient initiated severe epigastric pain and vomits, and lab tests presented a slight increase in liver enzymes. The platelet count remained above 150,000/μL. Cesarean section (CS) was performed. Six hours after CS, she developed a hypovolemic shock and lab tests became increasingly abnormal (liver enzymes increased sharply and hemoglobin and platelet count decreased sharply). Abdominal ultrasound showed suggestive signs of hepatic rupture. The patient was stabilized and exploratory laparotomy was performed. Perihepatic packing was applied. The patient was discharged after 25 days of admission (15 days at the ICU) and three explorative laparotomies. This case highlights the unpredictability of this event based on the changes of liver enzyme values, LDH and platelet count. These changes only occurred when the rupture had already occurred and the patient was in hypovolemic shock.     

Severe Maternal Morbidity Associated with Hypertensive Disorders in Pregnancy in the United States

Objectives: This study was to report the incidence of severe maternal morbidity associated with hypertensive disorders of pregnancy in the United States. Study Design: We used data from the National Hospital Discharge Survey, a nationally representative sample of discharge records, from 1988 to 1997. The database consisted of approximately 300,000 deliveries, which represented 39 million births during the 10‐year period. Results: The overall incidence of hypertensive disorders in pregnancy was 5.9% [95% confidence interval (CI): 5.2 to 6.5%]. Eclampsia was reported at 1.0 per 1000 deliveries (95% CI: 0.8 to 1.2). The incidence of eclampsia, severe preeclampsia, and superimposed preeclampsia remained unchanged during the 10‐year period. Women with preeclampsia and eclampsia had a 3‐ to 25‐fold increased risk of severe complications, such as abruptio placentae, thrombocytopenia, disseminated intravascular coagulation, pulmonary edema, and aspiration pneumonia. More than half of women with preeclampsia and eclampsia had cesarean delivery. African American women not only had higher incidence of hypertensive disorders in pregnancy but also tended to have a greater risk for most severe complications. Preeclamptic and eclamptic women younger than 20 years or older than 35 years had substantially higher morbidity. Conclusion: Preeclampsia and eclampsia carry a high risk for severe maternal morbidity. Compared to Caucasians, African Americans have higher incidence of hypertensive disorders in pregnancy and suffer from more severe complications.     

Spontaneous liver hematoma in pregnancy not clearly associated with preeclampsia: A case presentation and literature review

Spontaneous liver hemorrhage with formation of subcapsular hematomas and rupture of Glissan's capsule is a rare but often lethal complication of pregnancy. This entity has usually been associated with severe preeclampsia or the HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome. A case of spontaneous subcapsular hematoma of the liver occurring in the third trimester is presented in which the patient probably had neither preeclampsia nor the HELLP syndrome. The literature on liver hematomas in pregnancy published since 1982 when the term HELLP syndrome was coined is reviewed with a focus on the association of liver hematomas with preeclampsia and the HELLP syndrome. Therapy and maternal and neonatal outcomes for this entity are reassessed. (Am J Obstet Gynecol 1997;176:1328-33)     

Diagnostic dilemma: hepatic rupture due to HELLP syndrome vs. trauma

Case report We herein present the first reported case of a pregnant patient with underlying pregnancy-induced hypertension and HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome who presented with hepatic rupture following a minor motor vehicle accident.Discussion The diagnostic dilemma between spontaneous HELLP syndrome-associated versus trauma-related hepatic rupture is discussed.