Elevation of intracranial pressure following transjugular intrahepatic portosystemic stent-shunt for variceal haemorrhage

Increased intracranial pressure and cerebral oedema in patients with chronic liver disease is rare and is more typical of acute liver failure. Transjugular intrahepatic portosystemic stent-shunt is being increasingly used in the management of uncontrolled variceal haemorrhage in patients with cirrhosis. In our institution, a total of 160 patients has undergone transjugular intrahepatic porto-systemic stent-shunt for variceal haemorrhage; 56 of these procedures were emergencies for uncontrolled variceal haemorrhage. Four of these 56 patients developed features of acute liver failure, with marked deterioration in liver function tests and elevated intracranial pressure. This unusual but important complication of transjugular intrahepatic portosystemic stent-shunt has not been reported in the literature previously, and may have important consequences both for clinical practice and in the provision of further clues to understanding the pathogenesis of increased intracranial pressure in patients with liver diseases.     

Management of hepatic encephalopathy

Among the general principles of the therapy of hepatic encephalopathy the authors discuss the intensive care of patients, maintenance of their volume and electrolyte balance, treatment of coagulation defect, therapy of gastrointestinal bleeding and portal hypertension, provision of central renal catheter, infection prophylaxis, monitoring of intracranial pressure, and if necessary, respiration and intubation of patients. The study also deals with possibilities of treatment based on the toxic hypothesis and on the theory of neurotransmitters. Attention is paid to the therapy of brain oedema and to the significance of hemodialysis, hemoperfusion, plasmapheresis and hybrid bioartificial liver cell treatment. The authors deal with the indications of glucagon-insulin therapy and emphasize the importance of liver transplantation in the treatment of hepatic encephalopathy.     

Antibodies to streptococcal cell wall in psoriatic arthritis and cutaneous psoriasis

Increased intracranial pressure is a risk factor which may result in secondary brain damage, and affect neurological outcome in head injured patients. In case of diffuse brain lesions, elevated intracranial pressure is characterised by two important features. First, it results from vasogenic or cellular oedema, or from an increase in cerebral blood volume. Second, it is strongly associated to biochemical disorders. The latter may be considered as a direct consequence of the initial traumatic impact, mediating factors of the secondary neurological lesion and the biochemical result of cerebral ischaemia. They contribute to increased intracranial pressure and ischaemia by inducing physiological disorders and cell lesions. They also reflect the degree of cerebral ischaemia. Cerebral acidosis, free radicals and excitatory amicoacids are the main biochemical disorders implicated in this vicious circle leading to neuronal death.     

Liver transplantation resolves the hyperdynamic circulation in hereditary hemorrhagic telangiectasia with hepatic involvement

BACKGROUND & AIMS: Hepatic involvement in hereditary hemorrhagic telangiectasia is common but often asymptomatic. However, in some cases, the vascular lesions that involve the liver may lead to high-output cardiac failure and pulmonary hypertension that is predominant over hepatobiliary manifestations. Liver transplantation and treatment of these complications are described and discussed in this article. METHODS: Three patients with hereditary hemorrhagic telangiectasia and hepatic involvement received transplants. They had pulmonary hypertension and chronic right-sided heart failure caused by disseminated intrahepatic telangiectasias with shunts between the hepatic artery and hepatic veins or portal vein. Left-to-right intrahepatic shunt output was estimated to range between 51% and 57.5% of cardiac output. RESULTS: Hyperdynamic circulation disappeared after liver transplantation in all patients. Results of computed tomography and right-sided heart catheterization performed 6 months later were normal. Follow-up periods currently are 65, 53, and 29 months, and each patient continues to be asymptomatic. CONCLUSIONS: This report suggests that liver transplantation can be considered as an alternative and successful curative treatment that may prevent the irreversible evolution of cardiopulmonary disease.     

Experimental study of venous circulatory disturbance by dural sinus occlusion

Using a newly devised model of dural sinus occlusion, we investigated the pathophysiology of venous haemorrhage as well as venous circulatory disturbance. The superior sagittal sinus (SSS) and diploic veins (DV) were occluded in 16 cats. Intracranial pressure (ICP), cerebral blood volume (CBV) and regional cerebral blood flow (rCBF) were measured for 12 hours after the occlusion. At the end of the experiment, cerebral water content was estimated. In another 8 cats additional occlusions of cortical veins were carried out. In both groups, the blood-brain barrier permeability was evaluated with Evans blue or horseradish peroxidase. The SSS and DV occlusion produced a significant increase in ICP and CBV concomitant with a significant decrease in rCBF. Cerebral water content also increased significantly. However, there was no transition of Evans blue and horseradish peroxidase through the cerebral vessels, and no haemorrhages could be observed. In contrast, the additional occlusion of cortical veins produced haemorrhagic infarctions with Evans blue extravasation in 6 out of the 8 cats. These data suggest that dural sinus occlusion may lead to an increase in CBV and cerebral water content resulting in intracranial hypertension and decreased rCBF. The brain oedema in this model seemed to be mainly hydrostatic oedema, and might also be contributed by cytotoxic oedema. The additional occlusion of cortical veins might be essential in the development of haemorrhage in this model, and the blood-brain barrier was also disrupted in these areas.     

Dieldrin in fish and shellfish from the Mersey Estuary and Liverpool Bay

OBJECT: The authors studied the reliability of a new method for noninvasive assessment of cerebral perfusion pressure (CPP) in head-injured patients in which mean arterial blood pressure (ABP) and transcranial Doppler middle cerebral artery mean and diastolic flow velocities are measured. METHODS: Cerebral perfusion pressure was estimated (eCPP) over periods of continuous monitoring (20 minutes-2 hours, 421 daily examinations) in 96 head-injured patients (Glasgow Coma Scale score < 13) who were admitted to the intensive care unit. All patients were sedated, paralyzed, and ventilated. The eCPP and the measured CPP (ABP minus intracranial pressure, measured using an intraparenchymal microsensor) were compared. The correlation between eCPP and measured CPP was r=0.73; p < 10(-6). In 71% of the examinations, the estimation error was less than 10 mm Hg and in 84% of the examinations, the error was less than 15 mm Hg. The method had a high positive predictive power (94%) for detecting low CPP (< 60 mm Hg). The eCPP also accurately reflected changes in measured CPP over time (r > 0.8; p < 0.001) in situations such as plateau and B waves of intracranial pressure, arterial hypotension, and refractory intracranial hypertension. A good correlation was found between the average measured CPP and eCPP when day-by-day variability was assessed in a group of 41 patients (r=0.71). CONCLUSIONS: Noninvasive estimation of CPP by using transcranial Doppler ultrasonography may be of value in situations in which monitoring relative changes in CPP is required without invasive measurement of intracranial pressure.     

Increased restenosis in diabetes mellitus after coronary interventions is due to exaggerated intimal hyperplasia. A serial intravascular ultrasound study

Hepatic hydrothorax is a rare complication of portal hypertension. Conservative therapy may be successful but refractory hepatic hydrothorax is not uncommon. Management of refractory hydrothorax is usually ineffective and can result in a worsened clinical status. Transjugular intrahepatic portosystemic shunts (TIPS) lower portal pressure and have been used in the treatment of refractory ascites. The aim of this study was to determine the efficacy of TIPS in the treatment of symptomatic refractory hepatic hydrothorax. A TIPS was placed in 24 consecutive cirrhotic patients with symptomatic refractory hepatic hydrothorax. Five patients (20.8%) were Child's/Pugh class B and 19 (79.2%) were class C. All had undergone multiple thoracenteses and were hypoalbuminemic. Mean follow-up was 7.2 months (range, 0.25-49 months). Fourteen (58.3%) of 24 patients had complete relief of symptoms after shunt placement and did not require further thoracentesis. Five (20.8%) additional patients required fewer thoracenteses. Five (20.8%) patients developed worsening liver function and died within 45 days. In eight (66.7%) of 12 patients with > or = 60 days of follow-up, the serum albumin increased by a mean of 1.2 g/dL (range, 0.1-2.2 g/dL). The Child's-Pugh score improved in 7 (58.3%) of these 12 patients and two patients improved from class C to class A. These two patients no longer require liver transplantation. This study shows that TIPS can be effective in the management of symptomatic, refractory hepatic hydrothorax. Clinical and laboratory improvement may be seen and liver transplantation may become unnecessary.     

To be born, to live and to die with lead

Fulminant hepatic failure is a severe impairment of liver function in someone who has had previously normal liver function. The patients presenting to intensive therapy units have generally had a rapid deterioration which requires prompt intervention and treatment. The first part of this paper gives a detailed review of the aetiology, physiology and management of the disease process. Medical and nursing care of these patients is critical; any adverse intervention may affect outcome. A literature review and small study of the intervention of endotracheal suctioning and its effects on intracranial pressure and cerebral perfusion pressure is described.     

Retrograde transport of nerve growth factor from olfactory bulb to olfactory epithelium

OBJECTIVE: The purposes of this study were to determine if splenic perfusion measurements obtained using dynamic CT are useful in the evaluation of portal hypertension. MATERIALS AND METHODS: Forty-four patients with chronic liver disease (29 men and 15 women, 49-81 years old) and 38 control subjects (17 men and 21 women, 21-79 years old) underwent dynamic CT of the spleen. Regions of interest were drawn on images of the spleen and aorta, and splenic perfusion was calculated by dividing the peak gradient of the splenic time-attenuation curve by the peak aortic CT measurement increase. In 11 patients with chronic liver disease and three patients with normal livers, we measured the wedged hepatic vein pressure (WHVP) of the right or right accessory hepatic vein to estimate portal vein pressure. RESULTS: Splenic perfusion was less in patients with chronic liver disease (0.894 +/- 0.324 ml/min) than in the control group (1.299 +/- 0.429 ml/min; p < .0001). We found a significant negative correlation between splenic perfusion and WHVP (r = .741; p = .0024). CONCLUSION: A significant decrease in splenic perfusion in patients with chronic liver disease negatively correlated with WHVP. Measurement of splenic perfusion may be useful in the evaluation of portal hypertension.     

Coexistence of migraine and idiopathic intracranial hypertension without papilledema

Eighty-five patients with refractory transformed migraine type of chronic daily headache (CDH) had spinal tap as a part of diagnostic work-up. Twelve had increased intracranial pressure without papilledema, transient visual obscurations, or visual field defects. The headache profile of these 12 patients was not different from that of transformed migraine type of CDH. Acute headache exacerbations responded to specific antimigraine agents such as ergotamine, dihydroergotamine (DHE), and sumatriptan, whereas prophylactic antimigraine medications were only partially helpful. Addition of agents such as acetazolamide and furosemide, after the diagnosis of increased intracranial pressure, resulted in better control of symptoms. These observations suggest a link between migraine and idiopathic intracranial hypertension that needs further research. In refractory CDH with migrainous features, a spinal tap to exclude coexistent idiopathic intracranial hypertension without papilledema may be indicated.     

Liver transplantation for Wilson's disease

BACKGROUND/AIMS: As has been the case with other metabolic diseases of the liver in the last decade, orthotopic liver transplantation has been applied to the treatment of Wilson's disease with increasing frequency. The experience at the University of Pittsburg with orthotopic liver transplantation for Wilson's disease is reported. METHODS: Between February 1981 and December 1991, 51 orthotopic liver transplants were performed on 39 patients (16 pediatric, 23 adults) with Wilson's disease. Twenty-two patients were transplanted because of a presentation co-existent with fulminant hepatic failure. Seventeen presented with chronic advanced liver disease with (n=9) or without (n=8) associated neurologic dysfunction. RESULTS: The rate of primary graft survival (n-39) was 73% and patient survival was 79.4%. No patient mortality occurred beyond 3 weeks post-orthotopic liver transplantation. Survival was butter for those with a chronic advanced liver disease presentation (90%) than it was for those with a fulminant hepatic failure (73%) presentation, but the difference was not statistically significant. CONCLUSIONS: 1) Currently, orthotopic liver transplantation is the treatment of choice for Wilson's disease presenting as fulminant hepatic hepatic failure; 2) orthotopic liver transplantation should be considered for patients with Wilson's disease with advanced, chronic liver disease for whom no other therapy is possible; 3)orthotopic liver transplantation only partially corrects the underlying metabolic defect of patients with Wilson's disease and converts the copper kinetics from that characteristic of an individual affected with a homozygous disease to that of an individual who is an obligate heterozygote, thereby effecting a phenotypic cure.     

Optimal recovery of cytomegalovirus from urine as a function of specimen preparation

BACKGROUND/AIMS: Early liver transplantation is crucial in children with liver disease and pulmonary artery hypertension. Some severe pulmonary vascular anomalies associated with portal hypertension disappear after isolated liver transplantation. Evolution of pulmonary artery hypertension due to plexogenic arteriopathy is controversial, as this association is still considered a contraindication to isolated liver transplantation. Outcome of pulmonary hypertension after isolated liver transplantation is reported in three patients with portal hypertension. METHODS: After echocardiographic diagnosis, the patients had a complete hemodynamic exploration, and two had a lung biopsy. After liver transplantation, the survivors had echocardiographic follow up and a second hemodynamic exploration. RESULTS: In two children, pulmonary pressures and resistances returned to near-normal values 1 and 6 years after successful isolated liver transplantation. The third patient, with the most severe arteriopathy, had to wait 1 year for a donor, and the attempted transplantation was complicated by ventricular tachycardia; death occurred 2 days after surgery. CONCLUSIONS: Liver transplantation can reverse pulmonary artery hypertension due to high pulmonary resistances complicating liver disease with portal hypertension, provided it is carried out at an early stage. Early detection of pulmonary hypertension by systematic echocardiography may thus be crucial in these children with portal hypertension.     

Age characteristics of formation of the hematopoietic microenvironment by stromal progenitors from the bone marrow of thymectomized mice

BACKGROUND: Portopulmonary hypertension, defined as mean pulmonary artery pressure >25 mmHg in the presence of a normal pulmonary capillary wedge pressure and portal hypertension, is a known complication of end-stage liver disease that has been associated with high morbidity and mortality at the time of liver transplantation. We have recently reported the successful treatment of portopulmonary hypertension with chronic intravenous epoprostenol and now report the first patient with severe portopulmonary hypertension successfully treated with epoprostenol who subsequently underwent successful liver transplantation. METHODS: A patient with severe portopulmonary hypertension was treated with intravenous epoprostenol, 23 ng/kg/min, for a 4-month period, after which the portopulmonary hypertension resolved and the patient underwent successful liver transplantation. RESULTS: The patient was discharged, continues to do well, and at 3 months is off epoprostenol with near normal pulmonary artery pressures. CONCLUSIONS: Chronic epoprostenol, in conjunction with a multidisciplinary, well-planned perioperative evaluation and treatment plan, may be the answer to a heretofore untreatable disease.     

Epidemiology, appropriateness, and cost of vancomycin use

OBJECTIVE: Our purpose was to compare the estimated maternal cerebral perfusion pressure and an index of vascular resistance, the resistance area product, in nonpregnant women with hypertensive pregnant women. STUDY DESIGN: The maternal middle cerebral artery was evaluated by transcranial Doppler ultrasonography in 17 nonpregnant women, 17 pregnant normotensive patients, 20 pregnant patients with chronic hypertension, and 21 pregnant patients with pre-eclampsia (defined by The American College of Obstetricians and Gynecologists criteria) and cerebral blood flow velocities were determined. We calculated estimated cerebral perfusion pressure as [Estimated cerebral perfusion pressure = V mean/(V mean = V diastolic) (Mean blood pressure - Diastolic blood pressure)] modified from Aaslid et al, 1986. Because the diameter of the vessels could not be measured directly, an index of resistance, the resistance area product, was calculated. Resistance area product = Mean blood pressure/mean velocity (Evans et al, 1988). We calculated an index of cerebral blood flow (Cerebral blood flow index) = Estimated cerebral perfusion pressure/resistance area product. RESULTS: Women who were chronically hypertensive and those with pre-eclampsia showed a significant increase in estimated cerebral perfusion pressure and resistance area product compared with nonpregnant and pregnant normotensive women. An estimate of cerebral blood flow (cerebral blood flow index) in nonpregnant women showed that pregnancy resulted in a nonsignificant 18% increase in cerebral blood flow. CONCLUSIONS: Women with chronic hypertension and pre-eclampsia behave similarly by demonstrating significant increases in cerebral perfusion pressure (estimated cerebral perfusion pressure) and cerebrovascular resistance (resistance area product) compared with normotensive and nonpregnant women. Pregnant patients have a minimal increase in cerebral blood flow (18%).     

Systemic lupus erythematosus: description of a pediatric series

OBJECTIVE: To analyze possible causative mechanisms for intracranial hemorrhage after orthotopic liver transplantation. DESIGN: We conducted a retrospective survey of medical records and a case-control comparison in patients who had undergone liver transplantation during the period 1986 through 1992. MATERIAL AND METHODS: In a group of 8 patients with intracranial hemorrhage after orthotopic liver transplantation and a control series of 207 patients who had undergone liver transplantation but did not have intracranial hemorrhage, we summarized pertinent clinical and laboratory data and statistically analyzed potential risk factors for hemorrhage. RESULTS: In the eight study patients, intracerebral hematomas were located in the parietal or frontal lobe in six, the cerebellum in one, and the putamen in one. Autopsy demonstrated a Candida-associated mycotic aneurysm in one of the eight patients, and one had disseminated aspergillosis. No statistically significant differences in thrombocytopenia, hypertension, extracranial bleeding sites, or cyclosporine-related neurotoxicity were found when these patients were compared with the control series. Bacteremia or fungemia was found in five of the eight patients with intracerebral hemorrhages (62%) but in only 11% of the control group (P = 0.03; Fisher's exact test). CONCLUSION: Overwhelming infections, thrombocytopenia, or both may have a role in intracerebral hemorrhage after liver transplantation.     

Disorders of the cognitive functions in those who worked in the cleanup of the aftermath of the accident at the Chernobyl Atomic Electric Power Station

The potentialities of cerebral oximetry in the para-infrared band as a means of neuromonitoring are assessed. The method is highly informative for selecting the treatment strategy during the acute period of craniocerebral injury and cerebrovascular diseases. Simultaneous analysis of intracranial pressure, central hemodynamics, and cerebral oximetry permits differentiation of mechanisms of intracranial hypertension and its compensation. Increase of rSO2 in increased intracranial pressure corresponds to brain hyperemia, its decrease to cerebral ischemia. A long trend of rSO2 changes is informative for disease prediction.     

Epidermal growth factor receptor immunoreactivity in gallbladder and extrahepatic biliary tract tumours

BACKGROUND: Brain dysfunction is common in patients with advanced liver disease; it is often manifested as hepatic encephalopathy, but its cause is not clearly understood. METHODOLOGY: Intracranial blood flow velocity parameters, including peak systolic velocity, end diastolic velocity and mean velocity of both middle cerebral arteries were measured by transcranial Doppler ultrasonography in 37 patients with cirrhosis without encephalopathy (16 Child's A, 10 Child's B and 11 Child's C) and 12 normal controls. The cause was alcohol-related in 24 and non-alcohol-related in 13. RESULTS: No significant differences in any of the Doppler parameters were detected in Child's group A when compared with controls. However, a statistically significant decrease in middle cerebral artery blood flow velocity was evident when Child's B and C patients without clinically apparent encephalopathy were compared with controls irrespective of the cause. Our results demonstrate that intracranial blood flow is abnormal in patients with advanced liver disease without clinically apparent encephalopathy.     

Sickle cell anemia and internal cerebral vein thrombosis

Cerebrovascular disorders are frequently associated with sickle cell disease, mainly in homozygous children. We report the case a 25-old-patient with known sickle cell disease who presented with coma inaugurated by manifestations of intracranial hypertension. CT revealed bilateral thalamic infarcts and angiography confirmed the thrombosis of internal cerebral veins. Treatment included heparin and blood transfusion. Severe cerebral oedema resulted in the lethal outcome three days later.     

Effects of induced hypertension on transcranial Doppler ultrasound velocities in patients after subarachnoid hemorrhage

BACKGROUND AND PURPOSE: Transcranial doppler ultrasound (TCD) is used after subarachnoid hemorrhage to detect cerebral vasospasm and is often treated with induced hypertension. Cerebral autoregulation, however, may be disturbed in this population, raising the possibility that TCD velocities may be elevated by induced hypertension. To study this possibility, we performed continuous TCD monitoring of the middle cerebral artery during the induction and withdrawal of induced hypertension in patients after subarachnoid hemorrhage. METHODS: Twenty-eight patients were studied during the induction and withdrawal of hypertension using primarily phenylephrine. Continuous monitoring was performed on the middle cerebral artery with the highest flow velocity. Treatment was based on rising TCD velocities or clinical evidence for cerebral vasospasm. Mean arterial pressure and mean TCD velocities were recorded every minute. A change of > 15% from starting TCD values was considered significant. Cerebral autoregulation was calculated as a percentage of intact autoregulation. Patients were subsequently divided into groups of disturbed and intact autoregulation. RESULTS: In 10 of 19 patients (53%), TCD velocities changed by > 15% and paralleled changes in mean arterial pressure. This directly altered the TCD interpretation of the grade of vasospasm in 7 of 19 patients (36%). Three additional patients had smaller absolute changes in TCD velocities. No clinical difference could be identified between patients with disturbed and intact autoregulation. CONCLUSIONS: In patients with disturbed autoregulation after subarachnoid hemorrhage, induced hypertension can alter cerebral blood flow velocities. The level of autoregulation needs to be considered when interpreting TCD velocities in patients after subarachnoid hemorrhage.     

Fulminant hepatic failure

Fulminant hepatic failure is a rare disorder with a high lethality. It is characterized by the development of acute hepatic symptoms and encephalopathy, in the absence of underlying chronic liver disease. The main causes are viruses, adverse drug effects and toxins. The major causes of death in patients with fulminant hepatic failure are cerebral edema, infections, gastrointestinal hemorrhage and renal failure, or a combination of these. Treatment of fulminant hepatic failure with a variety of medical therapies has hitherto been found to have little overall influence on outcome. With high volume plasmapheresis the clinical condition of the patients improves, and if there is no sign of spontaneous regeneration, emergency liver transplantation is the only solution.