Spontaneous Hepatic Rupture Research Articles

Hereditary lysozyme amyloidosis – phenotypic heterogeneity and the role of solid organ transplantation

Lysozyme amyloidosis (ALys) is a form of hereditary systemic non-neuropathic amyloidosis, which is inherited in an autosomal dominant fashion. Lysozyme, which is the amyloidogenic precursor protein in ALys, is a ubiquitous bacteriolytic enzyme synthesized by hepatocytes, polymorphs and macrophages. The aim of this study is to describe the phenotype and outcome of patients with ALys including the role of solid organ transplantation. Retrospective evaluation of patients with ALys. UK National Amyloidosis Centre. All 16 patients with ALys followed at the centre. A family history of amyloidosis was present in every affected individual. Although the phenotype was broadly similar amongst those from the same kindred, there were marked phenotypic differences between kindreds who possessed the same amyloidogenic mutation. Symptomatic gastrointestinal (GI) amyloid was prevalent, and macroscopically visible amyloidotic lesions were present in nine of 10 patients who underwent GI endoscopy. All symptomatic ALys individuals had hepatic amyloid. Four patients received orthotopic liver transplants (OLT), three for spontaneous hepatic rupture and one case, who had extensive hepatic amyloid and a strong family history of hepatic rupture, pre-emptively. All of the liver grafts were functioning at censor 1.7, 5.8, 9.0 and 11.0 years after OLT. Five patients had progressive amyloidotic renal dysfunction culminating in end-stage renal failure, three of whom underwent renal transplantation (RTx). There was no evidence of renal allograft dysfunction at censor 6.6, 1.8 and 0.8 years after RTx. Lysozyme amyloidosis is a disease of the GI tract, liver and kidneys, which has a slow natural history. There was a clear family history in all cases within this cohort, demonstrating a high clinical penetrance in the presence of an amyloidogenic lysozyme mutation. There is currently no amyloid-specific therapy for the condition which is managed symptomatically. OLT and RTx appear to be successful treatments for patients with liver rupture or end-stage renal disease, respectively, with excellent outcomes in terms of medium-term graft function and patient survival.

Spontaneous Hepatic Rupture with Intraperitoneal Hemorrhage without Underlying Etiology: A Report of Two Cases

Spontaneous hepatic rupture is a rare event most often occurring in association with underlying liver disease or pregnancy. We report two unusual cases of hepatic rupture without any identifiable pathology, trauma, or comorbid conditions.

Hemobilia caused by a ruptured hepatic cyst: a case report

IntroductionHemobilia is a rare cause of upper gastrointestinal bleeding. More than 50% of hemobilia cases are related to iatrogenic trauma from hepatobiliary procedures, and needle biopsy of the liver represents the most common cause. A minority of hemobilia cases are due to hepatobiliary disorders such as cholangitis, hepatobiliary cancers, choledocholithiasis, and vascular abnormalities in the liver. The classic presentation of hemobilia is the triad of right upper quadrant (biliary) pain, obstructive jaundice, and upper gastrointestinal bleeding. We report a rare case of hemobilia caused by a spontaneous hepatic cyst rupture, where our patient presented without the classical symptoms, in the absence of therapeutic or pathological coagulopathy, and in the absence of spontaneous or iatrogenic trauma.Case presentationA 91-year-old African-American woman was referred to our out-patient gastroenterology clinic for evaluation of mild epigastric pain and intermittent melena. An abdominal computed tomography scan was remarkable for multiple hepatic cysts. Esophagogastroduodenoscopy revealed multiple blood clots at the ampulla of Vater. Endoscopic retrograde cholangiopancreatography showed a single 18 mm-sized filling defect in the common hepatic duct wall at the junction of the right and left hepatic duct, adjacent to one of the hepatic cysts. The ruptured hepatic cyst communicated to the bile ducts and was the cause of hemobilia with an atypical clinical presentation.ConclusionHemobilia is an infrequent cause of upper gastrointestinal bleeding and rarely occurs due to hepatic cyst rupture. To the best of our knowledge, this is only the second case report in the literature that describes hemobilia due to hepatic cyst rupture. However, it is the first case in the literature of hemobilia due to hepatic cyst rupture in the absence of iatrogenic or spontaneous trauma, and in the absence of a spontaneous or pathological coagulopathy.

Rupture hépatique spontanée au cours d’une grossesse gémellaire non compliquée

Liver subcapsular haematoma and its consequence, spontaneous hepatic rupture, are very rare complications of pregnancy. They are mainly associated with pre-eclampsia. The diagnosis is difficult and the maternal and fetal mortality rates are high. We report the case of a spontaneous hepatic rupture on a normal liver during an uncomplicated twin pregnancy with a favorable outcome for both the mother and the newborns.

Spontaneous hepatic rupture: 13-year experience of a single center

Spontaneous hepatic rupture is a rare clinical event associated with various pathologies of the liver. Most series to date reported the incidence and characteristics of a single etiology. Data were collected for all patients admitted with spontaneous hepatic rupture from 1995 to 2007. Ten patients met the study criteria. Hepatocellular adenoma was the cause of the rupture in six female patients, in their second to fourth decade. In the remaining patients, the ruptures were because of hepatocellular carcinoma in two, metastatic gastrointestinal stromal tumor in one, and HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count) in one. Nine out of 10 patients were treated surgically. Spontaneous hepatic rupture requires a high index of suspicion for a correct and timely diagnosis. Outcome is potentially grave and greatly depends on the underlying condition.

Liver transplantation for spontaneous hepatic rupture associated with HELLP syndrome

Liver transplantation for spontaneous hepatic rupture associated with HELLP syndrome

Hematoma subcapsular hepático en el síndrome de HELLP

We present a case of spontaneous hepatic rupture after the formation of a subcapsular hematoma in a patient with a previously normal pregnancy and no previous diagnosis of severe hypertension. The patient was treated conservatively through hepatic packing. Maternal and fetal outcomes were optimal.

A very severe case of feline amyloidosis with spontaneous hepatic rupture and chronic renal failure

A 3.5-year-old neutered male domestic shorthaired cat presenting with inappetence, depression, polydisia, and severe icteric mucous membranes was diagnosed as having systemic amyloidosis with spontaneous hemorrhage from the liver and chronic renal failure. Laboratory findings were remarkable for anemia, thrombocytopenia, mature neutrophilia, hyperbilirubinemia, azotemia, and hyperphosphatemia. Little treatment was possible and the cat was euthanased later. At necropsy, the entire abdominal cavity was filled with intra-abdominal hemorrhage. Histopathological examination revealed extensive deposition of eosinophilic homogenous material in the parenchyma of the liver and sinusoids. Hepatic amyloid was detected primarily in periacinar regions associated with atrophy of adjacent hepatocytes. In the kidney, marked eosinophilic homogenous material was found in numerous glomeruli and outer medullary area. Amyloid deposits were demonstrated by the Congo red stain. Whether underlying diseases present as risk factors for the severity of clinical and biochemical picture of generalized amyloidosis in this case is unclear.

Rotura hepática espontánea en el síndrome HELLP: tratamiento mediante packing

Spontaneous hepatic rupture associated with HELLP syndrome (hemolysis, elevated liver enzyme levels and low platelet count) is a very rare phenomenon that is frequently associated with a substantial rate of complications and mortality. In these cases, urgent management based on prompt delivery, blood transfusion and hepatic hemostasis is required. We report the case of a patient with spontaneous hepatic rupture caused by HELLP syndrome, which was treated with urgent cesarean section and hepatic packing.

Spontaneous Hepatic Rupture Associated with Preeclampsia: Treatment with Hepatic Artery Embolization

Spontaneous rupture of the liver due to preeclampsia is a rare condition of pregnant women, and it can be very dangerous if not treated. We report here on a case of successfully treating spontaneous liver rupture associated with preeclampsia by performing transcatheter hepatic arterial embolization. A 41-year-old woman with spontaneous rupture of the liver associated with preeclampsia was treated by hepatic arterial embolization.

Splenic tear causing a hemoperitoneum after cardiac surgery

Hemoperitoneum after cardiac surgery is a very rare but life-threatening complication. We have only found a few cases described in the literature, in which the intra-abdominal hemorrhages were caused by liver bleeding, due to direct hepatic trauma or spontaneous hepatic rupture. We describe the first case of hemoperitoneum caused by a spontaneous rupture of the spleen.

Diagnosis and Surgical Management of Spontaneous Hepatic Rupture Associated with HELLP Syndrome

Spontaneous hepatic rupture with hemoperitoneum is an uncommon but devastating complication of hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome. Although the syndrome has been considered a variant of preeclampsia/eclampsia, its pathogenesis is not completely understood. Hepatic rupture increases the perinatal and maternal morbidity and mortality. This report describes 3 cases of extensive spontaneous hepatic rupture in pregnant women with HELLP syndrome. Early diagnosis and adequate tertiary management of this rare but life-threatening condition reduce the high maternal and newborn mortality.

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.

Spontaneous rupture of the liver in a patient with systemic AL amyloidosis undergoing treatment with high-dose melphalan and autologous stem cell transplantation: A case report with literature review

A 55-year-old woman with primary Immunoglobulin light chain (AL) systemic amyloidosis died due to spontaneous rupture of her liver following treatment with high-dose melphalan and autologous stem cell transplant (HDM/SCT). She was first diagnosed after developing nephrotic-range proteinuria. Spontaneous rupture of her liver occurred 10 days after treatment with HDM/SCT and was complicated by septic shock. She was not eligible for surgical intervention and died shortly after. Amyloid fibrils were extracted from the autopsied liver sample (05-135L) and the biochemical nature of the fibrils was analyzed using electrophoretic and immunohistochemical techniques. Our testing showed that the fibrils were composed of immunoglobulin lambda light chains that were not glycosylated.While the liver is often involved in AL amyloidosis, this is the first documented case of a spontaneous hepatic rupture in a patient during treatment with HDM/SCT. A literature review of spontaneous liver rupture in patients with amyloidosis is presented.

Liver Transplantation From a Donor Affected by Marfan's Syndrome

Marfan's Syndrome (MS) is a systemic inheritable connective tissue (CT) disease related to fibrillin-1 genetic mutation, which gives an abnormal consistency to the CT. This compromised structure of the CT is related to cardiovascular, skeletal, and ocular system complications (1, 2). A 49-year-old patient underwent liver transplantation (LT) for hepatitis C virus-related end-stage liver disease and hepatocellular carcinoma. The donor was a 17-year-old female with MS pronounced brain dead from postanoxic brain injury. Her clinical findings from MS were characterized by severe mitral valve prolapse and bone-joint abnormalities. She was not affected from any vascular manifestation of arterial degeneration; however, the liver was the only organ harvested. Histological hepatic artery analysis, performed over the procurement, appeared normal; therefore, the organ was considered suitable. Before procurement, the recipient received a thorough informed-consent about MS. During posttransplant follow-up, the graft hepatic artery was checked by serial ultrasound-Doppler scans, weekly for the first month and subsequently once per month; angio-computed tomography scan was performed on the sixth month. The vascular changes from MS mostly involve the main vessels (incidence ranged between 30% and 50%) because of the greater content of compromised CT within the vessel walls and to the higher pressure and blood flow the arteries are exposed to. The incidence of visceral artery changes is approximately 0.4−2% and is usually manifested with cystic medionecrosis (1–3). In the literature, only two hepatic artery complications are described in patients with MS. Santiago-Delpin et al. (4) described a case of incomplete MS with multiple aneurysms of the aorta and its branches, in which a hepatic artery aneurysm perforated into the common bile duct. Ruschen et al. (5) in 1984 published a case in which a spontaneous hepatic artery rupture occurred in a patient with MS. The FBN1-gene mutation persists in the transplanted graft and, theoretically, may increase the risk of vascular complications such as dilation, aneurysmal changes, dissection, and intimal tears (6). Our rationale to accept a donor with MS was based on the low incidence of hepatic artery complications and total absence of hepatic dysfunction in patients with MS. The normal hepatic artery biopsy also gave a crucial piece of information in the acceptance process. Farese et al. (6) suggested another potential complication occurring in this setting: the possibility of antibodies developed against mutated fibrillin-1 donor liver cells. To date, we have not detected any posttransplant liver dysfunction and the recipient has not presented graft rejection. In conclusion, LT from MS donors may represent an opportunity to expand the potential donor pool. Moreover, the mean age at death of patients affected from MS is 45 (±16) years old; therefore, the liver status of such a young donor should be better than common marginal donors (7, 8). The biopsy at the time of procurement should be the first step in accepting an organ from a patient with MS. Furthermore, we advocate a closer follow-up of the graft vessels by means of Doppler-ultrasound in patients receiving such a graft. After a follow-up of 10 months our recipient did not show signs of graft complications or hepatic artery changes. Fabrizio Di Benedetto Stefano Di Sandro Nicola De Ruvo Michele Masetti Liver and Multivisceral Transplant Center University of Modena and Reggio Emilia Modena, Italy Cristiano Quintini Department of General Surgery Cleveland Clinic Foundation Cleveland, OH Roberto Montalti Roberto Ballarin Giorgio E. Gerunda Liver and Multivisceral Transplant Center University of Modena and Reggio Emilia Modena, Italy

Hepatic rupture after cesarean section in a patient with HELLP syndrome: a case report and review of the literature

Spontaneous hepatic rupture in pregnancy is rare and carries a high maternal and perinatal mortality. We describe a case of hepatic rupture that occurred after emergency caesarean section performed in a pregnant woman at 38 weeks of gestation with Hemolysis, Elevated Liver enzymes and Low Platelet count (HELLP) syndrome. The patient was discharged after 30 days of intensive therapy and four explorative laparotomies. Today, 2 years after delivery, the patient and her baby are doing well. Hepatic rupture in pregnancy is a rare event that may complicate HELLP syndrome. In this case, we were able to achieve an excellent clinical outcome by the performance of an immediately explorative laparotomy.

HELLP syndrome et hématome sous-capsulaire du foie rompu. Stratégie thérapeutique à partir d'un cas clinique

We report a case of spontaneous hepatic rupture secondary to HELLP syndrome. A favourable evolution was observed after massive transfusion and surgical management limited to hepatic packing. Subcapsular hepatic haematoma is a rare complication of preeclampsia occurring mainly in the context of HELLP syndrome. A high maternal and foetal mortality is observed. Different therapeutic options are presented including medical, surgical and radiological interventions. A unique strategy cannot be defined. Multidisciplinary approach seems mandatory. Surgery should remain as less aggressive as possible.

Liver capsule rupture in eclampsia: treatment with hepatic artery embolisation

Spontaneous hepatic rupture associated with pregnancy is a rare and very serious complication associated with eclampsia or pre-eclampsia. We report a case of hepatic rupture following eclampsia at 27 weeks gestation which was managed successfully by selective hepatic artery embolisation as an adjunct to liver packing. Selective embolisation of the hepatic artery is an effective means of therapy for hepatic artery bleeding and is an adjunct to liver packing in spontaneous hepatic rupture in eclamptic patients.

Hereditary lysozyme amyloidosis: Spontaneous hepatic rupture (15 years apart) in mother and daughter. role of emergency liver transplantation

Hepatic rupture is a rare condition, and treatment options are very limited. We report a case of hepatic rupture secondary to hereditary lysozyme amyloidosis that was successfully treated by liver transplantation. The mother of this patient had presented in an identical fashion 15 years earlier in the pretransplant era and died very rapidly.

Spontaneous Hepatic Rupture due to Metastatic Tumor of Lung Adenocarcinoma

A 64-year-old man diagnosed as lung adenocarcinoma with hepatic tumor was admitted to our hospital. He carried the hepatitis B virus but was negative for PIVKA-II and alpha-fetoprotein, and hence we diagnosed a case of stage IV lung adenocarcinoma. We planned to administer systemic chemotherapy, but he experienced sudden-onset abdominal discomfort accompanied with decreased blood pressure. We diagnosed hemorrhagic ascites due to spontaneous rupture of the liver tumor. Emergency angiography and therapeutic embolization stabilized his clinical condition. Hemorrhagic ascites due to metastatic liver tumor is rare and the sudden onset of abdominal symptoms is an indicator of rupture.