Atraumatic spontaneous liver rupture is a very rare occurrence. Most case reports and case series focus on patients during pregnancy, conditions associated with malignancy, hepatomegaly/hepatic pathology, benign masses/lesions, or infectious etiologies. This case report presents a unique circumstance where none of the above-mentioned etiologies were evident at the initial presentation or during the clinical workup. The patient presented with some non-specific symptoms of biliary colic without a conclusive diagnosis before the hepatic rupture. Given the high morbidity and mortality associated with spontaneous liver rupture, we believe this case allows for a closer look at the pre-rupture presentation and eventual sequelae not mentioned elsewhere in the literature.

Hepatic rupture is a rare complication of severe preeclampsia. A high index of suspicion is required in the presence of abdominal pain accompanied by hemodynamic decompensation in a pregnant woman. Hepatic rupture constitutes a medical emergency that demands immediate intervention, often with the support of other medical disciplines, in a highly specialized hospital setting. Unruptured hepatic hematomas can be managed conservatively. Immediate delivery and surgical repair of the liver are necessary for maternal survival. Spontaneous liver rupture in pregnancy is often unrecognized, highly lethal, and not completely understood with few cases having been reported in the literature. Therefore, we present two cases of HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome with hepatic rupture, emphasizing their clinical presentation and therapeutic approaches.

A 32-year-old woman with preeclampsia who presented with persistent severe hypertension and epigastric pain underwent an emergency cesarean section for fetal distress and was diagnosed with hepatic rupture and HELLP (hemolysis, elevated liver enzymes, and a low platelet) syndrome. After the operation, the patient was transferred to the intensive care unit for supportive treatment and management of complications. Diagnosis and treatment decisions were made through multidisciplinary management. The patient received plasma exchange and continuous renal replacement therapy. One week after the operation, the patient developed deep vein thrombosis and received anticoagulant therapy, which triggered rebleeding. Conservative treatment was taken, including halving the dosage of anticoagulant medication and performing a blood transfusion, and the patient\'s condition gradually stabilized. The patient was discharged 44 days after the operation. Early diagnosis, effective treatment, and multidisciplinary management can help patients with this critical presentation achieve good clinical outcomes.

A 2-year-old spayed female Siberian Husky was presented with a history of acute onset lethargy, collapse, haematochezia and vomiting. The patient was severely tachycardic and hypotensive. Point-of-care ultrasound revealed gallbladder wall thickening and peritoneal effusion consistent with haemorrhage on subsequent abdominocentesis. Despite attempted medical stabilization over the course of several hours, including blood products and multiple autotransfusions, the patient progressed to cardiopulmonary arrest. The dog was successfully resuscitated but was subsequently euthanized. Necropsy revealed a severe, acute hemoperitoneum secondary to rupture of the left lateral liver lobe. A tear in the hepatic capsule was identified along with a large hematoma. A single adult nematode, consistent with Dirofilaria immitis, was found in a pulmonary vessel in the right caudal lung lobe. The remaining necropsy findings were supportive of the clinical diagnosis of anaphylaxis. This report details a case, with necropsy findings, supporting a diagnosis of anaphylaxis and severe, refractory hemoperitoneum resulting from hepatic rupture. Acute hepatic rupture should be considered in cases of anaphylaxis-related hemoperitoneum.

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.

BACKGROUND: Nodular regenerating hyperplasia (NRH) is the most common liver involvement in common variable immunodeficiency (CVID). Most patients are asymptomatic with gradually increasing alkaline phosphatase (ALP) and mildly elevated transaminase enzymes over the years. We report the first case of fatal liver mass rupture in a CVID patient with probable NRH.
METHODS: A 24-year-old man was diagnosed with CVID at the age of 1.25 years. Genetic testing revealed a transmembrane activator and calcium-modulator and cyclophilin-ligand interactor (TACI) mutation. He had been receiving intravenous immunoglobulin (IVIg) replacement therapy ever since then. The trough level of serum IgG ranged between 750-1200 mg/dL. However, he still had occasional episodes of lower respiratory tract infection until bronchiectasis developed. At 22 years old, computed tomography (CT) chest and abdomen as an investigation for lung infection revealed incidental findings of numerous nodular arterial-enhancing lesions in the liver and mild splenomegaly suggestive of NRH with portal hypertension. Seven months later, he developed sudden hypotension and tense bloody ascites. Emergency CT angiography of the abdomen showed NRH with intrahepatic hemorrhage and hemoperitoneum. Despite successful gel foam embolization, the patient died from prolonged shock and multiple organ failure.
CONCLUSIONS: Although CVID patients with NRH are generally asymptomatic, late complications including portal hypertension, hepatic failure, and hepatic rupture could occur. Therefore, an evaluation of liver function should be included in the regular follow-up of CVID patients.

OBJECTIVE: Most spontaneous hepatic rupture cases are associated with a pregnancy-induced hypertensive disorder like preeclampsia and HELLP syndrome. Although it is a rare complication, it is still associated with high maternal and fetal morbidity and mortality rates. With this study, we aim to present a case report and review the available literature on hepatic rupture associated with hypertensive disorders of the pregnancy.
METHODS: We present a case report and a review of the literature of the last 20 years on hepatic rupture associated with pregnancy-induced hypertensive disorders. The selected cases were reviewed to collect information on maternal characteristics, clinical presentation, diagnostic studies, therapeutic modalities and maternal and fetal outcomes.
RESULTS: Our review has found 57 publications describing a total of 93 cases of hepatic hemorrhage with capsule rupture associated with pregnancy-induced hypertensive disorders. Most of the patients were less than 35 years old and primiparous and the first symptoms of hepatic rupture included epigastric and right upper abdominal pain. Most of the diagnoses were made during surgery without previous diagnosis and, in the majority of cases, a surgical approach was necessary to achieve hemostasis. Perihepatic packing was the most used surgical method.
CONCLUSIONS: Our clinical case and literature review reinforces the importance of closely monitoring all pregnancies complicated with hypertensive disorders, including in the postpartum period. Although hepatic rupture accounts for high maternal and fetal morbidity and mortality rates, it is possible to keep a conservative approach with good maternal and fetal outcomes, with a high index of suspicious, an early diagnosis and a multidisciplinary approach.

A 67-year-old man with a low-grade fever was found to have a 25-mm diameter tumor of the left hepatic umbilical portion. The tumor was accompanied by occlusion of the left portal vein. Positron emission tomography using fluorodeoxyglucose showed that the tumor had abnormally high metabolic activity. Magnetic resonance imaging revealed the left hepatic duct segmental narrowing. There was a mild elevation in serum IgG4 (206 mg/dL). Intrahepatic cholangiocarcinoma was suspected. Instead of planned hepatectomy, the patient was forced to undergo emergency surgery for biliary panperitonitis caused by intrahepatic bile duct rupture. Intraoperative ultrasonography revealed a hypoechoic tumor-like thickened Glissonean sheath and needle biopsy was performed. Histologic examination confirmed fibrous tissue with IgG4-positive plasma cell infiltration without neoplastic proliferation. He was diagnosed with IgG4-related sclerosing cholangitis (IgG4-SC) presenting hepatic inflammatory pseudotumor. After his general condition improved, he underwent left hepatectomy. Macroscopic findings showed extreme fibrosis of the Glissonean sheath of the umbilical portion, and diffuse granular lesion aggregated in the left lateral segment. Microscopic examination confirmed chronic cholangitis and dense portal fibrosis in the umbilical portion and diffuse xanthogranulomatous inflammation. This is the first case report of spontaneous rupture of the intrahepatic bile duct in patient with IgG4-SC.

We report the case of a 34-year-old woman with a 32-week pregnancy complicated by recurrent severe preeclampsia, HELLP Class I syndrome, and an intact hepatic hematoma of the right lobe detected by ultrasound. During the cesarean section, the rupture of the hematoma occurred and a gastroesophageal probe of the Sengstaken-Blakemore type was placed to occlude the bleeding cavity and the exit tunnel. The balloons were deflated gradually and the probe was removed on the 10th day without complications. The Sengstaken-Blakemore probe can be an effective remedy to control liver bleeding in selected cases.
Reportamos el caso de una mujer de 34 años con embarazo de 32 semanas complicado con preeclampsia grave recurrente, síndrome HELLP de clase I y hematoma hepático intacto del lóbulo derecho detectado por ultrasonido. Durante la operación cesárea se rompió el hematoma, por lo que se colocó una sonda gastroesofágica de tipo Sengstaken–Blakemore para ocluir la cavidad sangrante y el túnel de salida. Los balones fueron desinflados paulatinamente y la sonda se retiró el décimo día sin complicaciones. La sonda de Sengstaken–Blakemore puede ser un recurso efectivo para controlar el sangrado hepático en casos seleccionados.

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.