Hepatic rupture from haematomas in patients with pre-eclampsia/eclampsia: a case series

Hepatic rupture from haematomas is a rare complication of severe preeclampsia/eclampsia especially when complicated with the haemolysis, elevated liver enzymes and low platelet count (HELLP) syndrome. It is associated with poor maternal and foetal outcomes as demonstrated by three cases we describe. The first case had eclampsia at 31 weeks gestation with features of abruptio placentae and at caesarean section we found haemoperitoneum of 1.5 litres, a 10cm liver rupture and a still birth. She subsequently died in ICU within 24 hours of surgery; the second case had eclampsia at 35 weeks and ended up as a table death during emergency caesarean section. She had 4 litres of haemoperitoneum, hepatic rupture, placental abruption and a stillbirth; the third case had pre-eclampsia at 33 weeks with markedly elevated liver enzymes. She had one litre haemoperitoneum, right lobe hepatic rupture and a stillbirth. She recovered after conservative management. Severe pre-eclampsia/eclampsia associated hepatic rupture calls for rapid and aggressive intervention with prompt multidisciplinary management to avert adverse outcomes.


Introduction
Rupture of a subcapsular hepatic haematoma is a rare complication that occurs in patients with severe preeclampsia or eclampsia especially when complicated with haemolysis, elevated liver enzymes and low platelet syndrome (HELLP). Its incidence ranges between 1:250,000 and 1:40,000 pregnancies but in women with HELLP syndrome its occurrence may be as high as 0.9%-2% [1,2].
It results from liver engorgement due to extravasation of fluid from porous hepatic microvasculature as a result of abnormal fibrin deposition in the hepatic sinusoids. Sensitization of the reticuloendothelial system of the liver by preeclampsia may render it unable to clear the fibrin thrombi from the circulation resulting in infarction with vascular disruption leading to intrahepatic hemorrhage and parenchymal destruction [3]. Abdominal palpation, manual removal of the placenta, or even uterine contractions have been reported to cause sudden rupture of the subcapsular haematoma.

Methods
A critical descriptive study of the presentation and management of consecutive patients who presented with hepatic rupture from subcapsular haematoma complicating preeclampsia/eclampsia. This is done through case summaries followed finally with a discussion on the similarities and differences between cases together with how the diagnosis, management and outcomes of these patients compare with global patterns.

Case 1:
A 29 year old para 2 gravida 4 at 31+3 weeks gestation was referred from a district hospital with a diagnosis of eclampsia.
She was referred with blood pressure (BP) of 179/111mmHg and proteinuria of 2+, and had been commenced on magnesium sulphate and anti-hypertensives. On arrival at the tertiary hospital she had epigastric pain, backache and was drowsy with marked conjunctival pallor. Her BP was 99/77mmHg with a weak thready pulse of 63 beats/minute which was associated with cold peripheries. The uterine fundus was small for gestational age, at 26 weeks size, with marked generalised abdominal tenderness and absent foetal heart tones. She had haemoglobin concentration of 9.3g/dl and thrombocytopaenia of 95*10 9

Discussion
We have presented 3 cases with hepatic rupture from subcapsular haematomas. Two of them had eclampsia associated with maternal deaths while all of them had still births and massive haemorrhage.
These findings are consistent with those reported in literature were hepatic rupture from haematomas has been largely reported in patients with preeclampsia and eclampsia leading to severe maternal and neonatal morbidity reported to be 60-86% of cases [4]. HELLP syndrome is a clinical syndrome associated with preeclampsia and is characterised by intravascular haemolysis (H), elevated liver enzymes (EL) and low platelet count (LP). The most sensitive marker for haemolysis is reduced serum haptogobulin though peripheral blood smear, serum LDH and elevated indirect bilirubin may all suggest the presence of haemolysis. Some of these tests were not done in our patients partly due to cost and availability but also due to the acuity of the presentation of the three cases. Coagulation tests are important to undertake as they help in ruling our disseminated intravascular coagulation which is an important differential diagnosis of HELLP syndrome. HELLP syndrome commonly occurs at preterm gestations with peak incidence being between 27 and 37 weeks resulting in poor neonatal outcomes due to prematurity and placental abruption [5].
Similarly the 3 cases we described had some features of HELLP syndrome and presented at preterm gestational ages (31-35 weeks). Sibai et al reported of high transfusion rates (55%), disseminated intravascular coagulation (21%), acute kidney injury (7.7%) and laparotomy rate of 2% in a prospective study of patients with HELLP syndrome [6]. This is similar to the findings in visual changes, epigastric pain and vomiting have better predictive value for poor maternal outcome [8]. Evidence on the management of patients with hepatic haematoma rupture is largely from a small number of case reports and they all show the importance of multidisciplinary team care involving obstetricians, paediatricians, intensivists, hepato-biliary surgeons, haematologists and physicians.
For haemodynamically stable patients with radiologically confirmed unruptured haematoma as in our third case, conservative nonoperative management is appropriate. This requires serial imaging by CT or ultrasound scan together with correction of bleeding disorders often associated with HELLP syndrome through transfusion of blood or its components depending on the clinical scenario [1,9]. Follow up should be long as spontaneous rupture has been reported to occur six weeks after initial presentation. The clinically unstable patient requires primarily operative management with the options of definitive procedure ranging from peri-hepatic packing and haemostatic angio-embolization to hepatorrhaphy and hepatectomy. A systematic review of case reports conducted by Vigil-De Gracia highlighted a significant improvement in maternal mortality owing to advanced resuscitation, intensive care medicine, surgical intervention, liver transplant and arterial embolization [10].

Conclusion
The three cases demonstrate the high morbidity and mortality associated with hepatic rupture from subcapsular haematoma, the need for urgent resuscitation, termination of pregnancy, high care and multi-disciplinary care in improving clinical outcomes. This condition is closely associated with pre-eclampsia/eclampsia and HELLP syndrome which all call for urgent haematologic evaluation for end organ dysfunction.
What is known about this topic  Hepatic rupture from subcapsular haematoma has high morbidity and mortality;  Occurrence of hepatic subcapsular haematoma is closely associated with preeclampsia.

What this study adds
 Early multi-disciplinary care is associated with increased survival chance;  Care of patients with hepatic subcapsular haematoma is largely governed by clinical features and these should be closely monitored;  Due to paucity of evidence guiding diagnosis and management, this series adds to the pool of published cases and ultimately may assist in future systematic reviews on this subject.