Diagnostic challenges in the evaluation of hepatic grafts from donors with HELLP syndrome: case report and review of the literature

HELLP syndrome (hemolysis, elevated liver function tests, low platelets) is a rare complication of pregnancy that can result in severe complications such as hepatic infarction, subcapsular liver hematomas, and maternal brain death from cerebral hemorrhage. Recently, several investigators have described cases of successful transplantation using livers procured from donors who suffered brain death as a result of HELLP syndrome. However, this new class of marginal liver donors must be approached with caution. We report the case of a 28-year-old pregnant woman who suffered brain death due to HELLP syndrome and was subsequently evaluated for potential liver donation. Although her transaminitis and other liver function tests were markedly improving during the final days of her hospital course, her liver demonstrated segments of necrosis during attempted procurement, and the histology revealed extensive centrilobular necrosis. This case suggests that peak values of serum transaminases, as well as partial resolution of transaminitis, appear to have limited predictive ability in determining the suitability of the hepatic graft for transplantation. Thus, donors with HELLP syndrome should be approached with caution, even in the setting of laboratory values suggesting minimal or resolving hepatic injury. Furthermore, there should be an additional emphasis on obtaining and reviewing histology of the potential graft to determine its suitability for transplantation.     

Outcome of pregnancies with HELLP syndrome complicated by acute renal failure (1989-1999)

HELLP syndrome, a syndrome of hemolysis, elevated liver enzymes and low platelets may occur in pregnancy with pre-eclampsia/eclampsia, and its a significant complication is acute renal failure (ARF). The aim of study was to determine frequency and outcome of HELLP syndrome complicated by ARF. Thirty-nine patients with pregnancy-related ARF were treated between Jan 1, 1989 and Jan 1, 1999. In these patients, the most frequent causes were HELLP syndrome (n = 14; 36%), postpartum hemorrhage (n = 10; 26%), pre-eclampsia/eclampsia (n = 6; 15%) and abruptio placenta (n = 4; 10%). Seven of the patients with HELLP syndrome had impairment of consciousness during hospitalization. Of these patients, coma in 5, stupor in 1, confusion in 1 were diagnosed. Twelve of the patients with HELLP syndrome and 14 of the other patients were treated by dialysis. Mann-Whitney U test and chi2 test(corrected by Yates and Fisher exact) were used for statistical analysis. Although serious clinical findings, with supportive treatment, 12 patients with HELLP syndrome and 21 other patients were fully recovered. One patient both with and without HELLP syndrome could not recovered due to diffuse cortical necrosis. Moreover, one patient with HELLP syndrome and 3 other patients were died. Mortality rate of the patients with HELLP syndrome was not found different from those of the other patients (p = 0.544). The causes of death were cerebral hemorrhage in patient with HELLP syndrome and disseminated intravascular coagulation (n = 1), cerebral emboli (n = 1), adult respiratory distress syndrome (n = 1). Fetal death occurred in 4 patients with HELLP syndrome (28.5%) and 7 other patients (28%), and rates were similar (p > 0.5). Finally, HELLP syndrome was the most frequent cause leading to ARF in pregnancy and their prognosis was not different from those of the other patients.     

OUTCOME OF PREGNANCIES WITH HELLP SYNDROME COMPLICATED BY ACUTE RENAL FAILURE (1989–1999)

HELLP syndrome, a syndrome of hemolysis, elevated liver enzymes and low platelets may occur in pregnancy with pre-eclampsia/eclampsia, and its a significant complication is acute renal failure (ARF).

The aim of study was to determine frequency and outcome of HELLP syndrome complicated by ARF.

Thirty-nine patients with pregnancy-related ARF were treated between Jan 1, 1989 and Jan 1, 1999. In these patients, the most frequent causes were HELLP syndrome (n = 14; 36%), postpartum hemorrhage (n = 10; 26%), pre-eclampsia/eclampsia (n = 6;15%) and abruptio placenta (n = 4;10%). Seven of the patients with HELLP syndrome had impairment of consciousness during hospitalization. Of these patients, coma in 5, stupor in 1, confusion in 1 were diagnosed. Twelve of the patients with HELLP syndrome and 14 of the other patients were treated by dialysis. Mann-Whitney U test and X² test(corrected by Yates and Fisher exact) were used for statistical analysis.

Although serious clinical findings, with supportive treatment, 12 patients with HELLP syndrome and 21 other patients were fully recovered. One patient both with and without HELLP syndrome could not recovered due to diffus cortical necrosis. Moreover, one patient with HELLP syndrome and 3 other patients were died. Mortality rate of the patients with HELLP syndrome was not found different from those of the other patients (p = 0.544). The causes of death were cerebral hemorrhage in patient with HELLP syndrome and disseminated intravascular coagulation (n = 1), cerebral emboli (n = 1), adult respiratory distress syndrome (n = 1). Fetal death occured in 4 patients with HELLP syndrome (28.5%) and 7 other patients (28%), and rates were similar(p > 0.5).

Finally, HELLP syndrome was the most frequent cause leading to ARF in pregnancy and their prognosis was not different from those of the other patients.     

Obstetrical anaesthesia for a parturient with preeclampsia, HELLP syndrome and acute cortical blindness

Purpose

To report the management of a multigravida presenting with preeclampsia, HELLP syndrome and acute cortical blindness for Caesarean section.

Clinical features

A 39-yr-old woman, with three past uncomplicated pregnancies presented at 33 wk with acute cortical blindness. Based on clinical and laboratory assessment, a diagnosis of preeclampsia with HELLP syndrome was made. A CT scan of her head demonstrated ischaemic lesions of her basal ganglia, extending superiorly to involve both posterior parietal and occipital regions. Infusions of magnesium sulphate and hydralazine were started and an urgent Caesarean section was performed under subarachnoid anaesthesia after insertion of an arterial line and intravenous hydration. The course of anaesthesia and surgery was uneventful and she delivered a live 1540 g female infant. By the following morning, she had recovered some vision and visual recovery was complete by 72 hr postpartum. She underwent an MRI with angiography on the first postpartum day. Ischaemic lesions were confirmed in the same sites identified on CT scan but all major cerebral vessels were patent and no significant vascular abnormality was noted. Her postoperative course was uneventful and she was discharged home seven days postpartum after being prescribed labetalol for continued hypertension.

Conclusion

The anaesthetic management of a parturient with acute cortical blindness and HELLP syndrome is modeled on the underlying preeclamptic condition. Invasive monitoring is not routinely indicated but is specifically indicated in some cases. Provided that it is not contraindicated because of prohibitive risk to the mother, regional anaesthesia has particular advantage in these patients. In particular, the use of spinal anaesthesia, which has been discouraged by some for this patient population, should be re-evaluated.     

Anesthesia in three women with HELLP syndrome

Three pregnant women with diagnosis of HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets), received emergency cesarean section in our hospital. Considering low platelet counts, in all three patients, operations were performed under general anesthesia using sevoflurane without epidural or spinal anesthesia. Special attention was paid to management of blood pressure, especially intra-operative hypertension. Moreover, if necessary, platelet and fresh frozen plasma were transfused, and therapy to prevent disseminated intravascular coagulation (DIC) and to protect liver and renal function, was performed perioperatively. As a result, laboratory data of all three patients recovered to almost within normal ranges after operation, and they were discharged without untoward complications. HELLP syndrome is a severe complication of pregnancy. Complications of this syndrome were severe including acute renal failure, DIC, pulmonary edema, cerebral hemorrhage and liver rupture. It is reported that maternal mortality is 2-24%. In the management of pregnant women complicated with HELLP syndrome, early diagnosis and adequate therapy, including preventive therapy for complications, are necessary.     

Liver transplantation for HELLP syndrome.

Hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome is a rare complication of pregnancy that is associated with preeclampsia and may result in rupture of the liver. Although there have been case reports of liver transplantation for HELLP syndrome, the outcomes of transplantation for this rare indication have not been reported. Furthermore, the optimal management of complicated HELLP syndrome and indications for liver transplantation are unclear. Our objective was to review the national experience with liver transplantation for HELLP syndrome and to develop a comprehensive algorithm for the management of liver complications of HELLP syndrome, including indications for transplantation. A recent case from our institution is reported and the literature is reviewed. The results of liver transplantation for HELLP syndrome were analyzed from the United Network for Organ Sharing database. Between October 1987 and November 2003 there have been 8 deceased donor liver transplants performed for complications related to HELLP syndrome. As of the most recent follow-up, 6 of the 8 patients are alive, with both deaths occurring within 1 month of transplantation, and 2 patients have required retransplantation. This review supports that good results can be obtained with liver transplantation for patients with complicated HELLP syndrome that have either ongoing, uncontrolled hemorrhage or liver necrosis and failure. Patients with complicated HELLP syndrome are best managed at a center with expertise in liver transplantation.     

Dissection artérielle carotidienne bilatérale dans un contexte de prééclampsie sévère : une cause inhabituelle de céphalées du post-partum

A 30-year-old woman with severe preeclampsia presented at 27 weeks of amenorrhea with left headache, neck pain, blurred vision and numbness of left hemiface that resolved spontaneously within 2 hours. A week later, hypertension remained poorly controlled despite combination of nicardipine and labetalol intravenous therapy; an urgent caesarean section was eventually performed due to onset of HELLP syndrome. At day 5 postpartum, the patient had a Horner syndrome with right ipsilateral disabling tinnitus. A CT-angiography of supra-aortic trunks was performed urgently; it showed a bilateral carotid arterial dissection without stroke, which was subsequently confirmed by MRI angiography. The patient was transferred in neurovascular intensive care unit. Anticoagulant therapy was implemented to prevent cerebral and retinal ischemic lesions. Symptoms resolved quickly and the patient was discharged at day 7 postpartum. MR-angiography performed 4 months later showed a full resolution of the bilateral carotid dissection. Anticoagulant therapy was therefore discontinued.     

Neonatal nephrotic syndrome associated with placental transmission of proinflammatory cytokines

Although there are clinical data suggesting a direct relationship between neonatal nephrotic syndrome and placental transfer of proinflammatory cytokines from mothers with HELLP syndrome, there is no direct evidence that these inflammatory cytokines are pathogenic. Here, the first human model of placental transfer of proinflammatory cytokines from a mother with HELLP syndrome to a newborn, resulting in neonatal nephrotic syndrome is described. Forty-eight hours after delivery, the neonate developed nephrotic syndrome and abnormalities in renal function which resolved completely during the 5 days following the initiation of therapy with hydrocortisone, albumin, and furosemide. The newborn’s cord blood showed increased concentrations of interleukin (IL)-1β, IL-6, and tumor necrosis factor alpha that were identical to those found in the mother’s serum. Hydrocortisone therapy was discontinued after a 2-week course. Clinical and laboratory improvements were associated with a marked decline in serum cytokine levels, indicating that the proinflammatory cytokines were pathogenic. The neonate remained in remission with no recurrence of nephrotic syndrome during 12 months of follow-up. These findings demonstrate that the placental transmission of circulating cytokines causing HELLP syndrome occurred during pregnancy and may have resulted in nephrotic syndrome in the neonate.     

General anesthesia with remifentanil for Cesarean section in a patient with HELLP syndrome

HELLP syndrome is a severe complication of pre-eclampsia characterised by hemolysis, elevated liver enzymes and a low platelet count. It is associated with an increased risk of adverse outcome for both the mother and the fetus. Patients with HELLP syndrome are also at greater risk of pulmonary edema, adult respiratory distress syndrome, abruptio placentae, intracerebral hemorrhage, eclamptic convulsions, disseminated intravascular coagulation, ruptured liver hematomas and acute renal failure. Perinatal mortality is equally high. Before delivery, aggressive obstetric management is directed toward stabilization of the affected organ systems, if possible, and interruption of the pregnancy in the early phase of the accelerated disease progression. Definitive therapy is delivery. Parturients HELLP syndrome often require general anesthesia for Cesarean section delivery. The anesthetic technique is critical for these patients with a high risk of uncontrollable hypertension, bleeding and multiple organ failure. Remifentanil is increasingly used as a very short analgesic agent providing cardiovascular stability in high-risk patients. We report the management of a patient presenting in labor with HELLP syndrome, and describe the successful use of remifentanil as part of the anesthetic technique for her subsequent Cesarean section.     

Hématome sous-capsulaire du rein compliquant une prééclampsie sévère : cas clinique et revue de la littérature

Renal haematoma during severe preeclampsia is a rare uneventful event. It is usually associated with other organ injury such as cerebral or liver haematoma. Imaging (ultrasound or tomodensitometry examination) plays an important role in detecting this complication and following its evolution. In the current case report, we describe an isolated renal haematoma during a severe preeclampsia complicated by a HELLP syndrome. This patient was managed with a conservative treatment (control of arterial pressure and induction of delivery) and an imaging follow-up.     

Third nerve palsy associated with preeclampsia and HELLP syndrome

Preeclampsia can cause myriad organ dysfunction, including cranial nerve palsies that pose diagnostic and management dilemmas. We present an unusual case of third nerve palsy, (presenting as diplopia, ptosis) with hypertension, hyperreflexia, proteinuria, easy bruising in a parturient at 34 + 6/52 weeks of twins gestation. She was treated as for severe preeclampsia and HELLP syndrome; intravenous magnesium sulphate and labetalol commenced and emergent cesarean delivery performed under general anesthesia due to concerns of low platelets and for airway protection should her glascow coma scale (GCS) deteriorate. Postoperatively, stroke, aneurysm and intra-cerebral causes of third nerve palsy were excluded, with subsequent recovery of symptoms upon blood pressure normalization. The eye signs are postulated to be due to two preeclamptic mechanisms involving disordered cerebral autoregulation: (1) hyperperfusion and breakdown of the blood–brain barrier that occurs with rising hypertension, causing fluid/blood product extravasation into brain parenchyma, or (2) focal reactive vasoconstriction and local hypoperfusion, contributed to by endothelial dysfunction.     

Hellp syndrome and subcapsular liver haematoma

HELLP syndrome is a disease of variable presentation with high mortality and morbidity. Haemolysis in HELLP syndrome is microangiopathic. Obstruction of hepatic blood flow by fibrin deposits in sinusoids results in elevated liver enzymes. It also leads to periportal necrosis and, in severe cases, intrahepatic haemorrhage, subcapsular haematoma formation or hepatic rupture. This case report describes a patient presenting with mild hypertension with abruptio-placentae. She developed HELLP syndrome, subcapsular liver haematoma and pleural effusion after delivery.     

The management of the surgical complications of HELLP syndrome

Introduction

Complications from HELLP (Haemolysis, Elevated Liver enzymes and Low Platelet) syndrome may present as an emergency to any surgeon. We review the ten-year experience of a tertiary hepatobiliary centre managing HELLP patients. Three selected cases are described to highlight our management strategy and a systematic review of the recent literature is presented.

Methods

All patients with HELLP syndrome were identified from a prospectively maintained database and their details collated. Subsequently, a detailed search of PubMed was carried out to identify all case series of HELLP syndrome in the literature in the English language since 1999.

Results

On review of 1,002 cases, 10 patients were identified with surgical complications of HELLP syndrome. Seven of these patients had a significant liver injury. Only three of these required surgical intervention for liver injury although four other patients required surgical intervention for other complications. There was no maternal mortality in this series. Review of the literature identified 49 cases in 31 publications. The management approaches of these patients were compared with ours.

Conclusions

We have presented a large series of patients with surgical complications resulting from HELLP syndrome managed without maternal mortality. This review has confirmed that haemodynamically stable patients with HELLP syndrome associated hepatic rupture can be conservatively treated successfully. However, in unstable patients, perihepatic packing and transfer to a specialist liver unit is recommended.     

HELLP综合征30例临床分析

目的总结HELLP综合征的诊治体会。方法对我院2001年1月至2007年7月间收治的30例HELLP综合征进行回顾性分析。结果HELLP综合征占同期分娩产妇的0．38％、重度子痫前期的7．61％、子痫的10％。2例双胎孕妇外院转入时子痫合并颅内出血和小脑疝,迅速死亡;25例在诊断HELLP后（8．9±1．1）h内终止妊娠;3例〈32孕周者病情较平稳,应用地塞米松72h后终止妊娠。孕产妇死亡4例（死亡率13．3％）。分娩时孕龄（36．3士3．4）周,新生儿出生体重（2,590．5±620．9）g,新生儿32例、存活22例、死亡10例,围生儿死亡率35．3％,孕妇死亡2例。结论HELLP综合征是一种严重威胁母儿安全的并发症,除早期诊断、早期治疗、及时终止妊娠外,还应该切实做好产前保健、提高危重儿的抢救水平,才能有效改善母儿预后。     

Postpartale Eklampsie und fulminantes HELLP-Syndrom

Postpartum onset of eclampsia and HELLP (hemolysis, elevated liver enzymes, low platelet count) syndrome is a rare but life-threatening complication for both mother and fetus. A case of a 38-year-old parturient (gravida 2, para 1) who was asymptomatic prior to delivery is reported. Emergency caesarean section had to be performed due to sudden onset of fetal bradycardia as a result of partial placental separation. The perioperative course was characterized by new onset hypertension, nausea and restlessness; within 2 h the patient suffered a generalized seizure which was treated with magnesium sulfate and hydralazine. Despite management in accordance with current guidelines, the condition deteriorated with hypotension, anemia and renal failure. On further examination hematomas in the abdominal cavity and walls were identified and laboratory tests confirmed HELLP syndrome with severe coagulopathy. Explorative laparotomy revealed diffuse bleeding without a significant isolated source or postpartum uterine hemorrhage. Retrospectively, the anemia could be ascribed to severe hemolysis and diffuse bleeding from coagulopathy. The patient required packed red cells, platelets, fresh frozen plasma and prothrombin complex. After admission to the intensive care unit persistent diffuse bleeding mainly caused by hyperfibrinolysis and renal failure occurred, which required blood transfusion, antifibrinolytic (tranexamic acid) and renal replacement therapy (continuous veno-venous hemodiafiltration with citrate) for 6 days. The patient recovered without any sequelae and was discharged 26 days later. Placental separation with new onset peripartum hypertension is to be interpreted as a precursor of severe gestosis and associated complications, especially disseminated intravascular coagulation (DIC), acute renal failure and pleural effusion. A differentiation between a rapid drop in hemoglobin concentration secondary to hemolysis in postpartum HELLP syndrome rather than postpartum hemorrhage can be challenging. In addition, HELLP syndrome can lead to rapidly developing, fulminant hyperfibrinolysis in the context of DIC. Keys to successful management of postpartum gestosis and associated complications are early detection and perception of clinical and laboratory warning signs, a multidisciplinary approach with rapid and consistent targeted symptomatic therapy to save the mother and fetus.     

HELLP综合征合并肝包膜下血肿1例

HELLP综合征全称溶血-肝脏转氨酶增高-血小板减少综合征,是指妊娠期高血压综合症伴有溶血、肝酶升高以及血小板减少的一组临床综合征。是一种急危重症,妇科、肝胆外科和影像科医生应熟悉其病变特征,以免在临床工作中漏诊、误诊。我们临床上诊治了1例36岁在产后出现HELLP综合征,且合并肝包膜下血肿的病例。在多学科诊治,密切观察肝包膜下血肿,血流动力学稳定情况下采取保守治疗后,最终患者病情逐渐好转。     

Liver transplantation using an organ donor with HELLP syndrome

BACKGROUND: The shortage of organs for liver transplantation has forced transplant centers to expand the donor pool by using donors traditionally labeled as marginal. One such example is liver transplantation using a donor with HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets), a disorder of late pregnancy that involves the liver as one of the target organs. METHODS: Two patients who died from complications of HELLP syndrome were evaluated for attempted multi-organ procurement. Donor characteristics, gross and microscopic liver findings, and procurement and transplant outcomes were reviewed. RESULTS: One of the liver allografts was successfully transplanted; the other was not procured because of poor macroscopic appearance. CONCLUSION: It is possible to successfully transplant the liver from a donor that succumbs to HELLP syndrome, provided there is adequate recovery of liver function before procurement.     

Plasma exchange in severe postpartum HELLP syndrome

We present a case of extremely severe postpartum HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome, associated with activation of coagulation, massive recurrent intra-abdominal bleeding requiring two laparotomies, renal failure, and central nervous system symptoms. This case underlines the complexity of pregnancy-related thrombotic microangiopathies regarding their differential diagnosis, multiple organ dysfunction, as well as management. Systemic endothelial cell injury plays a central role in the pathogenesis of thrombotic microangiopathies. Treatment of HELLP syndrome usually consists of administration of antihypertensive therapy and magnesium sulfate. No consensus exists regarding the use of plasma exchange or corticosteroids. Plasma exchange was a major part of the treatment regime in our patient. Early plasma exchange may be considered as an adjuvant therapy in severe and progressive postpartum HELLP syndrome.     

Caesarean section in a patient with a history of HELLP syndrome]

A 36-year-old woman underwent emergency caesarean section following the diagnosis of HELLP syndrome. Four years earlier, after having undergone the same procedure for HELLP syndrome, she had experienced hypovolemic shock, renal failure, and disseminated intravascular coagulopathy during the postoperative period. This time, the patient showed bleeding, elevation of liver enzymes (ALT, AST, LDH) and a reduction of antithrombin III activity in the 36th week of pregnancy. Anesthesia was induced by thiamylal 4 mg.kg-1 and suxamethonium 1 mg.kg-1 and after delivery maintained by oxygen-nitrous oxide-isoflurane, and all procedures were performed without any incident. No major complications such as intraperitoneal bleeding, renal failure, or disseminated intravascular coagulopathy occurred during the postoperative period. It is suggested that caesarean section should be carried out as soon as possible after the diagnosis of HELLP syndrome is confirmed.