The HELLP syndrome: clinical issues and surgical management. A Case Experience.

AIM: This study aims to challenge the current know-how in patients with spontaneous rupture of a liver hematoma, to differentiate amongst patients requiring such specific surgical therapy and avoiding mistakes during surgical operations, in order to terminate pregnancy with beneficial effects on the mother and fetus. MATERIALS AND METHODS: In a emergency scenario we admitted a 37-year-old woman at 35+4 weeks of gestation for emergency cesarean section after the onset of right hypochondrium pain. A diagnosis of hemoperitoneum and severe preeclampsia with liver and splenic bleeding was done and managed with packing of hepatic and splenic hematomas and according to her haemo-dynamic clinical conditions, done in different time. RESULTS: A diagnosis of hemoperitoneum and severe pre-eclampsia with liver and splenic bleeding was done and managed it with 3 xypho-pubic-laparatomy in different time with haemostatic packing. DISCUSSION: In this case report, the patient underwent an emergency caesarean section and was managed with packing of hepatic and splenic hematomas and according to her haemodynamic clinical conditions was operated in different time. The choice of laparotomy and hepatic packing has proved to be a viable option in patients with unstable vital signs and is feasible even in limited resource settings. CONCLUSION: Short interval between diagnosis and management may enhance the feto-maternal survival rate and prevent further morbidity or mortality. The choice of laparotomy and hepatic packing has proved to be a viable option in patients with unstable vital signs and is feasible even in limited resource settings. KEY WORDS: HELLP syndrome, Liver hematoma rupture, Packing.

Ruptura hepática espontánea en paciente con síndrome HELLP.

Hemorrhagic liver rupture is a rare and deadly complication. The pathogenesis is unknown. This situation forces the multidisciplinary team, the immediate termination of pregnancy, the treatment and management of the patient in an intensive care unit (ICU). We report the results of two patients with spontaneous rupture of the liver during pregnancy and HELLP (hemolysis, elevated liver enzymes, low platelet count) syndrome, asymptomatic, during cesarean section, with management in ICU, poor evolution without adequate response; one died and the other leaves hospital. Liver rupture requires high suspicion and timely, aggressive multidisciplinary management in all cases and surgical intervention in those who develop liver ruptura, to improve survival.

La hemorragia por ruptura hepática es una rara y letal complicación, de etiología desconocida. Obliga al equipo multidisciplinario a la interrupción del embarazo, al tratamiento agresivo y al manejo de la paciente en una unidad de cuidado intensivo (UCI). Se presentan dos pacientes con embarazo de término con ruptura de hematoma hepático subcapsular asociado a síndrome HELLP (hemolysis, elevated liver enzymes, low platelet count), asintomáticas, durante operación cesárea, con manejo en UCI, ambas con evolución tórpida; una fallece y la otra se egresa. La ruptura hepática requiere una alta sospecha y un manejo multidisciplinario oportuno, agresivo en todos los casos y de intervención quirúrgica en quienes desarrollen ruptura hepática, para mejorar la supervivencia.

Acute colonic pseudo-obstruction with bowel rupture after caesarean section in HELLP syndrome: a case report.

BACKGROUND: Ogilvie syndrome, also known as acute colonic pseudo-obstruction (ACPO), can occur postpartum after caesarean section (C-section), often resulting in caecal dilatation. The incidence rate is approximately 100 cases in 100,000 patients per year (Ross et al., Am Surg 82:102-11, 2016). Without proper diagnosis and treatment, it may progress to intestinal perforation or other fatal complications. CASE PRESENTATION: A 39-year-old pregnant woman underwent emergency low-segment C-section due to complications of Haemolysis, Elevated Liver enzymes and Low Platelets syndrome (HELLP) syndrome. ACPO was suspected on the third day after C-section based on inability to pass flatus, evident abdominal distension, slight abdominal pain, and computed tomography (CT) scan revealing severe, diffuse colonic distention with caecal dilatation of approximately 9 cm. Based on these findings, conservative treatment was implemented. However, 6 days after C-section, her symptoms worsened, and CT showed possible intestinal perforation; thus, an emergency laparotomy was performed. Due to a 3-cm (diameter) laceration in the anterolateral wall of the ascending colon and a 5-cm tear in the ileocecal junction, in combination with mucosal eversion in the colon, resection of the ileocecum, distal closure of the ascending colon, and a terminal ileostomy were performed. The patient was discharged 2 weeks post-laparotomy and continued to undergo nursing care for the incision and stoma. Ileostomy was performed 4 months later. CONCLUSION: Ogilvie syndrome after C-section is an extremely rare but severe condition, which warrants early recognition and treatment to prevent potentially fatal complications, especially in patients with poor health status.

Liver Transplantation for Hemolysis, Elevated Liver Enzymes, and Low Platelet Count Syndrome: A Propensity-score Matched Long-term Survival of the Scientific Registry of Transplant Recipients Dataset.

BACKGROUND: Hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome is a life-threatening form of pre-eclampsia/eclampsia, with liver transplantation being the life-saving option for treatment for those who develop severe hepatic complications. METHODS: We retrospectively analyzed data from the Scientific Registry of Transplant Recipients (SRTR) through October 1, 1987, to December 31, 2014. Forty patients were listed for liver transplantation, with 18 patients finally receiving their transplant. These 18 patients were matched with 1:10 patients using a propensity-score matched approach. RESULTS: Baseline demographics were comparable between HELLP syndrome patients who received a transplant and those who did not. The 10-year intent-to-treat survival was 63.6% for non-transplanted HELLP patients, 64.2% for transplanted HELLP patients, and 61.8% for matched patients (P = .369). Overall survival was also similar between HELLP patients and matched patients, with 10-year survival at 64.2% and 61.8% respectively (P = .985). However, death-censored graft survival was inferior in HELLP patients, with 10-year survival at 63.4% compared with 75.4% in the matched patients (P = .044). CONCLUSIONS: Patients with HELLP syndrome who did not receive a transplant achieved similar intent-to-treat survival with those transplanted patients, while the death-censored graft survival of transplanted patients was inferior to matched patients. This might guide improved liver donor allocation in clinical practice.

[Case Report of Liver Rupture with Fulminant HELLP Syndrome in the 37th Gestational Week]. / Fallbericht Leberruptur bei fulminantem HELLP-Syndrom in der 37. SSW.

HELLP syndrome, characterized by the triad of hemolysis, elevated liver enzymes due to liver impairment, and low platelet count, is a hypertensive disorder in pregnancy. Although it is said to be caused by disturbed placentation in the first trimester, its clinical presentation can be seen mostly in the third trimester, but never before the completed 20th gestational week. Predictive for its diagnosis is the reported upper abdominal pain that normally is localized under the right arc of ribs. With the aid of laboratory examination, the suspected diagnosis can be confirmed or excluded. Therapeutic options are observational treatment with the prophylaxis of respiratory distress syndrome and attempting to prolong pregnancy with the help of steroids like dexamethasone, or delivering the infant by inducing labor or performing a primary caesarian section, depending on the gestational week. Delivery is the unique causal therapy of HELLP syndrome. Clinical management is mainly influenced by the course of HELLP syndrome. There are mild forms that allow prolonging the pregnancy for several days or sometimes weeks, but also foudroyant courses with acute liver damage. We report the case of a 40-year-old, gravida 1 woman in gestational week 36+1 who was brought to our hospital in hemorrhagic shock caused by a rupture of the liver due to acute HELLP-syndrome.

Hematoma After Transversus Abdominis Plane Block in a Patient With HELLP Syndrome: A Case Report.

We herein present a case of intramuscular hematoma that developed after transversus abdominis plane block in a patient undergoing cesarean delivery. The patient had HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count) preoperatively. Ultrasonography-guided transversus abdominis plane block was performed at the end of surgery. Postoperatively, the platelet count and antithrombin III level decreased, and computed tomography revealed intramuscular hematomas that possibly were related to vascular injury and potential disseminated intravascular coagulation. We should be mindful of the possibility of intramuscular hematoma formation in patients with HELLP syndrome, even when using ultrasound guidance.

A HELLP syndrome complicates a gestational trophoblastic neoplasia in a perimenopausal woman: a case report.

BACKGROUND: HELLP syndrome is a combination of symptoms described as hemolysis, elevated liver enzymes and low platelets, that complicates 0.01-0.6 % of pregnancies. HELLP syndrome has been scarcely reported associated with partial moles, another rare complication of pregnancy. This manuscript describes the only reported case of HELLP syndrome associated with a complete invasive hydatiform mole. CASE PRESENTATION: We report a perimenopausal patient in prolonged remission from an uncommon high-risk invasive complete mole. The diagnosis was set in a context of early onset preeclampsia and HELLP syndrome. The development of life-threatening complications required primary hysterectomy. Postoperative hCG quickly returned to normal with EMA/CO multi-agent chemotherapy. CONCLUSION: Our patient is in prolonged remission from a complete mole complicated with EOP and HELLP syndrome. This exceptional case of complicated gestational trophoblastic neoplasia reflects a very rare condition in which several risk factors for placental ischemia are associated. Emergency hysterectomy should be considered as salvage initial treatment in such life-threatening situations.

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.

Haemolytic anaemia after nitrofurantoin treatment in a pregnant woman with G6PD deficiency.

We present a normotensive, pregnant woman with severe haemolytic anaemia in the third trimester of pregnancy. Owing to normal platelet count diagnoses other than HELLP syndrome were considered and investigated. The patient was treated with nitrofurantoin 3 weeks before presentation and she turned out to have a deficiency of glucose-6-phosphate dehydrogenase. After treatment with blood transfusion, vitamin B12 and folic acid the patient recovered completely. Caesarean delivery was performed because of maternal hypertension and fetal distress at 33 weeks' gestation.

Tratamiento quirúrgico conservador del síndrome de HELLP (ELLP) asociado a hematoma hepático subcapsular / Conservative surgical treatment of HELLP syndrome (ELLP variant) associated with a subcapsular liver hematoma

Las complicaciones hemorrágicas graves asociadas al embarazo suelen ocurrir en el tercer trimestre del mismo y se relacionan frecuentemente con situaciones de preeclampsia y síndrome HELLP. El síndrome HELLP solo incide en el 0,5-0,9% de las gestaciones, pero su elevada morbimortalidad maternofetal nos obliga a tener en cuenta su diagnóstico en sus diversas formas de presentación clínica. Aunque la gran mayoría de las alteraciones hepáticas que ocurren en el embarazo tienen relación con las escasas hepatopatías inherentes al mismo, la duda diagnóstica ocurre con cierta frecuencia. Presentamos un caso de síndrome HELLP incompleto (variante ELLP) tratado efectivamente mediante actitud quirúrgica conservadora y cuyas peculiaridades inciden en diversos aspectos de esta entidad(AU)

Severe hemorrhagic complications associated with pregnancy usually occur within the third trimester and are frequently linked to preeclampsia and HELLP syndrome. HELLP syndrome affects only 0.5-0.9% of pregnancies but, because it causes high maternal-fetal mortality, a correct diagnosis of the various forms of presentation of this syndrome is essential. Although most of the liver alterations observed during pregnancy are related to pregnancy itself, diagnostic doubts are not infrequent. We present a case of incomplete HELLP syndrome (ELLP variant) associated with a subcapsular liver hematoma, which was successfully treated with a conservative surgical approach(AU)

[A case of HELLP syndrome resulting in eclampsia with non-aneurysmal subarachnoid hemorrhage].

It is known that hemorrhagic stroke at the perinatal period are caused by specifics conditions like eclampsia as well as by the existing abnormal vessels. We treated a case of HELLP syndrome resulting in eclampsia with non-aneurysmal, convexity subarachnoid hemorrhage. A 34-year-old female, who had been pointed out to have a high level of urinal protein at the 37th week, was seen in the emergency department because of severe headache, vomiting and respiratory discomfort. Her systolic blood pressure was over 190mmHg, and caesarean section was selected. On the way to the operating room, she had a generalized convulsion with loss of consciousness. The delivery was carried out. The CT immediately after the caesarean section revealed faint and localized subarachnoid hemorrhage in the bilateral convexity areas. Additionally, the FLAIR image of MRI demonstrated increased intensity in the bilateral cerebellar hemispheres, basal ganglion and subcortical area, suggesting vasogenic edema. The patient had a good clinical course and the abnormal signal of MRI also recovered by treatment with oral iron and zinc. Here, we report a speculation for the mechanism of this case and precautions against stroke in the perinatal period.

[Hepatic subcapsular hematoma: a case report and management update]. / Hématome sous-capsulaire du foie : cas clinique et mise au point actualisée sur la prise en charge.

Hepatic subcapsular hematoma (HSH) is a rare complication of pregnancy that keeps a materno-fetal high mortality rate. Given this severity, HSH management requires a prompt diagnosis and an appropriate care. Before focusing on the current management strategy of HSH, we illustrate this article with a clinical case presenting an original method of abdominal exploration, which has not been described to our knowledge. Thus, taking into account the presence of a moderate hemoperitoneum during a caesarean section procedure for HSH, introduction of laparoscopy through the same incision allowed a satisfactory evaluation of the abdominal cavity and hepatic area without performing an invasive midline laparotomy.

Apoptosis, proliferation and Fas ligand expression in placental trophoblast from pregnancies complicated by HELLP syndrome or pre-eclampsia.

OBJECTIVE: To investigate apoptosis, proliferation and Fas ligand expression of placental trophoblast in the hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome and in pre-eclampsia (PE), and to compare this with normal pregnancies. DESIGN: Prospective study. SETTING: University hospital in Croatia. SAMPLE: Placentae from women with HELLP syndrome (n=10), PE (n=10) and normal pregnancies (n=10). METHODS: The HELLP syndrome was diagnosed with platelets <100×10(9) /L, aspartate aminotransferase (AST) and alanine transaminase (ALT) >70 U/L and lactic acid dehydrogenase (LDH) > 600 U/L. Pre-eclampsia was diagnosed at blood pressure >140/90 mmHg, with proteinuria >300 mg/L/24 hours. For detection of apoptosis and proliferation in villous trophoblast, antibodies M30 and Ki-67 were used. Expression of Fas ligand was assessed using immunohistochemistry and the semiquantitative HSCORE method. MAIN OUTCOME MEASURES: Apoptosis, proliferation and Fas ligand expression in villous trophoblast. RESULTS: Apoptosis, proliferation and Fas ligand expression were higher in villous trophoblast in HELLP syndrome than in the PE group (p=0.015, p=0.018 and p=0.002, respectively) and the control group (p=0.000, p=0.012 and p=0.049, respectively). Placentae from the PE group had higher levels of apoptosis (p=0.019), lower Fas ligand expression (p=0.029) and no difference in proliferation (p=0.887) compared with the control group. CONCLUSIONS: There is an increase in apoptosis, proliferation and Fas ligand expression in placentae from women with HELLP syndrome compared with placentae from PE and normal pregnancies. Our findings indicate the possibility of differential mechanisms behind HELLP syndrome and PE.

Parathyroid adenoma with hypertensive crisis and intracerebral hemorrhage mimicking hemolysis, elevated liver enzymes, low platelets syndrome.

BACKGROUND: Hyperparathyroidism is seldom encountered during pregnancy. Moreover, when the disease does occur, it is typically masked until late pregnancy or after delivery. CASE: A previously healthy multiparous woman presented with sudden-onset severe preeclampsia with hemolysis, elevated liver enzymes, low platelets syndrome at 37 weeks of gestation. Acute intracerebral hemorrhage and disseminated intravascular coagulapathy developed 24 hours after cesarean delivery and persisted after craniotomy. Hypercalcemia and hyperparathyroidism were noted, and imaging studies revealed parathyroid tumor. The patient recovered from severe preeclampsia after resection of a hemorrhagic parathyroid adenoma and was fully rehabilitated after 3 months. CONCLUSION: This patient exhibited a concealed hyperparathyroidism with acute hypertensive crisis, probably attributable to hemorrhagic parathyroid adenoma. The presentation mimics acute late-onset preeclampsia and requires vigilant diagnosis followed by surgery.