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.     

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.     

Liver transplantation for HELLP syndrome

Of the approximately one in 1000 pregnant women who develop the syndrome of hemolysis, elevated liver enzymes, and low platelets (HELLP), 2 to 3 per cent develop hepatic complications, including liver failure for which liver transplantation (LT) may be required. Between February 1, 1984, and December 31, 2006, eight women without a history of liver disease underwent LT for complications of HELLP syndrome. All received cadaveric grafts with a mean interval from delivery to LT of 7 days. The mean admission Child-Turcotte-Pugh score was 13.1 (class C), and the mean model for end-stage liver disease score was 40. Manifestations of liver failure included encephalopathy (seven patients), renal failure (four), disseminated intravascular coagulation (three), and respiratory failure (one). There were no intraoperative deaths. Complications of LT included biliary leaks (three patients), reoperation (three), and retransplantation (two). There was one death from sepsis on postoperative day 91 and one death from cholangitis/sepsis more than 5 years postoperatively. After LT, 1-, 5-, and 10-year patient survival rates were 88 per cent, 88 per cent, and 65 per cent; 1-, 5-, and 10-year graft survival rates were 64 per cent, 64 per cent, and 48 per cent. This is the largest single-center report of LT for HELLP. Early recognition and transfer to a transplant center will yield best results with this challenging complication of pregnancy.     

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.     

Late occurrence of diffuse cerebral swelling after intracerebral hemorrhage in a patient with the HELLP syndrome--Case report

Hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome can occur at any time in the course of pregnancy and is associated with many complications including fatal stroke. A 37-year-old female presented with HELLP syndrome causing an intracerebral hematoma, which was treated by evacuation and mild hypothermia. Unexpected diffuse cerebral swelling occurred on the 15th day of the initially favorable postoperative course. Considerable impairment of consciousness persisted despite conservative therapy. Serial computed tomographic findings indicated delayed cerebral vasospasm as the cause of the swelling. Particularly careful management is required even beyond the first 2 weeks for patients with stroke as a complication of HELLP syndrome.     

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.     

HELLP syndrome--a severe form of pregnancy-induced hypertension. A case report

HELLP syndrome consists in serious liver alterations during pregnancy induced hypertension, that causes hemolysis, elevated liver enzymes and low platelet count. It is frequently accompanied by evidence of severe kidney and brain involvement. Our paper aims to stress the importance of an early accurate diagnosis and an adequate management by presenting an illustrative case.     

Thrombocytopenia during pregnancy: from etiologic diagnosis to therapeutic management

Thrombocytopenia complicates 10% of all pregnancies. It has many potential causes, but three are responsible for almost all cases: incidental gestational thrombocytopenia (IGT) (74%), preeclampsia and HELLP (hemolysis, elevated liver function tests, low platelet count) syndrome (21%) and immune thrombocytopenic purpura (ITP) (4%). Although there is no risk of maternal or fetal hemorrhage with IGT, a benign disorder, preeclampsia, HELLP syndrome and ITP expose mother and child to potentially life-threatening complications. Other rare causes are also associated with severe complications: thrombotic thrombocytopenic purpura, hemolytic and uremic syndrome, disseminated intravascular coagulation and von Willebrand disease type IIB. Because risks for mother and child vary so greatly according to the cause of thrombocytopenia, an accurate etiologic diagnosis is essential to ensure optimal therapeutic management.     

Unusual presentation of leptospirosis in the late stage of pregnancy

Here we report a case of leptospirosis without fever during the late stage of pregnancy in which the initial clinical presentation was more suggestive of a pregnancy-related liver dysfunction rather than an infectious disease. A 32-year-old primipara at 37 week of gestation was hospitalised with a 10-day history of nausea, vomiting, and abdominal pain without fever. Initial routine blood tests showed hyperbilirubinemia, a moderate increase in transaminase levels, severe coagulopathy and an increased creatinine level. On clinical suspicion of pregnancy-related liver dysfunction such as HELLP syndrome (hemolysis, elevated liver enzyme levels, low platelet count) or acute fatty liver of pregnancy (AFLP), emergency caesarean section was performed and a healthy baby was delivered. Postoperatively, the patient was stable, but 5 days later she developed clouding of consciousness, severe jaundice and respiratory failure. At this time, an infectious disease was considered and leptospirosis was confirmed by serological tests. In conjunction with intensive care management, antibiotic therapy was given; the patient was discharged in good condition and her baby did not develop signs of active leptospirosis. While leptospirosis is rare in pregnancy, this is the first report of acute infection without fever mimicking the clinical pattern of HELLP syndrome or AFLP     

Renal histopathology of prolonged acute kidney injury in HELLP syndrome: a case series and literature review

International Urology and Nephrology - Acute kidney injury (AKI) is a severe complication of hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome. However, renal pathological...     

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.     

Spontaneous acute subdural haematoma and intracerebral haemorrhage in patient with HELLP syndrome: case report

HELLP (haemolysis, elevated liver enzymes, and low platelet count) syndrome can result in a fatal intracranial haemorrhage during the perinatal period. We report treatment of a 32-year-old primigravida who fell into a deep coma during labour with fetal distress, complicated by a spontaneous acute subdural haematoma and intracerebral haemorrhage. Simultaneous emergency operations, evacuation of the acute subdural haematoma and a caesarean section, were performed, during which a diagnosis of HELLP syndrome with disseminated intravascular coagulation was made. Both mother and infant recovered, though hemiparesis persisted in the mother. Patients with HELLP syndrome should be managed as high-risk, which requires an excellent working relationship of the physicians involved. Prompt recognition of intracranial haemorrhagic complications and neurosurgical intervention are particularly important.     

Second trimester hepatic rupture in a 35 year old nulliparous woman with HELLP syndrome: a case report

The HELLP syndrome (haemolysis, elevated liver blood tests and low platelets) is a serious complication in pregnancy characterized by haemolysis, elevated liver enzymes and low platelet count occurring in 0.5 to 0.9% of all pregnancies and in 10–20% of cases with severe preeclampsia. Hepatic capsular rupture is a rare yet dramatic complication of HELLP syndrome. The majority of cases occur in multiparous women over the age of 30. Classically it presents with acute onset right upper quadrant pain in the presence of constitutional symptoms such as vomiting and pyrexia. However, symptoms and signs are usually non specific. Spontaneous hepatic rupture can be preceded by signs of hypovolaemic shock; yet the diagnosis is infrequently made prior to emergent laparotomy. We present the case of a 35 year old nulliparous woman with a second trimester gestational hepatic rupture associated with HELLP syndrome. We briefly discuss the aetiology, diagnostic difficulties and treatment options associated with this rare presentation.     

Results of the pregnancies with HELLP syndrome

In this study, clinical features, developing complications, and results of thirty-six patients, which were followed up in our Obstetrics and Gynecology and Nephrology departments between 1997 and 2001, with the diagnosis of HELLP syndrome were searched retrospectively. The mean age of the cases followed up with diagnosis of HELLP syndrome were 30.2 +/- 5.9 (17-46) years. HELLP syndrome was diagnosed on average in the 32.6 +/- 4.8th (23-41) week of gestations. Seventy percent of the cases were with severe preeclampsia and 30% of the cases were with mild preeclampsia. Eleven cases (30%) were nullipara and twenty-five cases (70%) multipara. The average of arterial systolic blood pressure of the cases were 161.6 +/- 26 mmHg, and that of diastolic blood pressure was 98.5 +/- 16.8 mmHg. In thirteen cases (36%) acute renal failure (ARF), six cases (17%) placenta detachment, two cases disseminate intravascular coagulation (DIC), one case Adult Respiratory Distress Syndrome (ARDS) were developed. In seven cases (19%) intrauterine dead fetuses were detected. In twenty-three cases by cesarian section (64%), in thirteen cases by induction (36%) the pregnancies were terminated in 72 h after diagnosing HELLP syndrome. Birth weights of eleven babies (30%) were below 1500 g. Five of the eleven babies were dead in the neonatal period. Six of the thirteen patients who had ARF were given hemodialysis. Two patients died because of the development of ARF + DIC and ARDS. No predicting factors for the development of HELLP syndrome could be detected, but severe preeclampsia. Therefore we think that preeclamptic pregnancies must be followed up very closely and if HELLP syndrome develops, termination of the pregnancy would be proper as soon as possible.     

Results of the Pregnancies with HELLP Syndrome

In this study, clinical features, developing complications, and results of thirty-six patients, which were followed up in our Obstetrics and Gynecology and Nephrology departments between 1997 and 2001, with the diagnosis of HELLP syndrome were searched retrospectively. The mean age of the cases followed up with diagnosis of HELLP syndrome were 30.2 ± 5.9 (17–46) years. HELLP syndrome was diagnosed on average in the 32.6 ± 4.8th (23–41) week of gestations. Seventy percent of the cases were with severe preeclampsia and 30% of the cases were with mild preeclampsia. Eleven cases (30%) were nullipara and twenty-five cases (70%) multipara. The average of arterial systolic blood pressure of the cases were 161.6 ± 26 mmHg, and that of diastolic blood pressure was 98.5 ± 16.8 mmHg. In thirteen cases (36%) acute renal failure (ARF), six cases (17%) placenta detachment, two cases disseminate intravascular coagulation (DIC), one case Adult Respiratory Distress Syndrome (ARDS) were developed. In seven cases (19%) intrauterine dead fetuses were detected. In twenty-three cases by cesarian section (64%), in thirteen cases by induction (36%) the pregnancies were terminated in 72 h after diagnosing HELLP syndrome. Birth weights of eleven babies (30%) were below 1500 g. Five of the eleven babies were dead in the neonatal period. Six of the thirteen patients who had ARF were given hemodialysis. Two patients died because of the development of ARF + DIC and ARDS. No predicting factors for the development of HELLP syndrome could be detected, but severe preeclampsia. Therefore we think that preeclamptic pregnancies must be followed up very closely and if HELLP syndrome develops, termination of the pregnancy would be proper as soon as possible.     

Spinal subarachnoid hematoma following spinal anesthesia in a patient with HELLP syndrome

A case of subarachnoid hematoma following spinal anesthesia for cesarean section in a patient with HELLP syndrome is reported. A 39-year-old woman underwent cesarean section under spinal anesthesia for worsening preeclampsia with HELLP syndrome. Despite full recovery from the spinal anesthetic, on the second postoperative day she felt numbness on the posterior aspect of her right leg, noticed she was insensitive to bladder fullness and had mild flaccid paraparesis. Magnetic resonance imaging revealed a spinal subarachnoid hematoma with cauda equina compression. With conservative management she made an almost complete recovery within three months. Serial magnetic resonance imaging showed spontaneous regression of the hematoma. The risk of spinal subarachnoid hematoma following obstetric regional anesthesia is exceedingly small even in a patient with coagulopathy and, to our knowledge, this is only the second reported case following obstetric regional anesthesia. Anesthesia for HELLP syndrome in patients with an adequate platelet count but without disseminated intravascular coagulation is controversial. It is therefore important for clinicians to recognize the symptoms and signs of spinal subarachnoid hematoma to avoid delay in treatment that might result in severe neurological deficit.     

Unusual presentation of mesangial proliferative glomerulonephritis in HELLP syndrome associated with acute renal failure

Acute renal failure in pregnancy is not common in industrialized countries. HELLP syndrome (hemolysis, elevated liver enzyme, and low platelets) was one of the causes of acute renal failure in pregnancy, but renal pathological findings in case of acute renal failure had rarely been reported. We reported an unusual case of HELLP syndrome with acute renal failure requiring renal replacement therapy and which histopathologic findings of kidney biopsy showed mesangial proliferative glomerulonephritis and her renal function completely recovered after immediate artificial abortion, supportive management, transfusion of blood products, and hemodialysis.     

UNUSUAL PRESENTATION OF MESANGIAL PROLIFERATIVE GLOMERULONEPHRITIS IN HELLP SYNDROME ASSOCIATED WITH ACUTE RENAL FAILURE

Acute renal failure in pregnancy is not common in industrialized countries. HELLP syndrome (hemolysis, elevated liver enzyme, and low platelets) was one of the causes of acute renal failure in pregnancy, but renal pathological findings in case of acute renal failure had rarely been reported. We reported an unusual case of HELLP syndrome with acute renal failure requiring renal replacement therapy and which histopathologic findings of kidney biopsy showed mesangial proliferative glomerulonephritis and her renal function completely recovered after immediate artificial abortion, supportive management, transfusion of blood products, and hemodialysis.     

Obstetrical anesthesia for parturient patients with HELLP syndrome

In the period from April 1995 to March 2000, 11 parturient patients were diagnosed as having HELLP syndrome and underwent Caesarian section at our institution. All of the patients also had eclampsia or preeclampsia. Six of the operations were performed under general anesthesia and 5 were performed under regional anesthesia (one epidural and 4 spinal blocks). Subarachnoid anesthesia was well tolerated and clinically serious decreases in blood pressure with onset of block were not common. In the cases in which general anesthesia was used, control of blood pressure was difficult and the Apgar scores of the babies were low. No major complications occurred during the postoperative periods. Anesthetic management of a parturient patient with HELLP syndrome is modeled on the underlying preeclamptic condition. Determination of the appropriate anesthetic should be based on the patient's condition, condition of the fetus, and the urgency of the situation.     

Pulmonary embolism in the setting of HELLP syndrome

HELLP syndrome (hemolysis, elevated liver enzymes and low platelets) complicates 0.5–0.9% of pregnancies and is frequently associated with multiorgan dysfunction. Treatment relies on prompt diagnosis, delivery and supportive care. The clinical presentation may make the concurrent diagnosis and management of other disease entities challenging. This case report describes a patient with postpartum HELLP syndrome complicated by severe multiorgan dysfunction and pulmonary embolism.