Budd-Chiari Syndrome
Radiology, Interventional
Portasystemic Shunt, Transjugular Intrahepatic
Syringomyelia
Encephalocele
Platybasia
Cranial Fossa, Posterior
Meningomyelocele
Decompression, Surgical
Subarachnoid Space
Decompressive Craniectomy
Down Syndrome
Pelvic Bones
Cerebrospinal Fluid Pressure
Metabolic Syndrome X
Managing Budd-Chiari syndrome: a retrospective review of percutaneous hepatic vein angioplasty and surgical shunting. (1/234)
BACKGROUND: The role of percutaneous hepatic vein angioplasty in the management of Budd-Chiari syndrome has not been well defined. Over a 10 year period at our unit, we have often used this technique in cases of short length hepatic vein stenosis or occlusion, reserving surgical mesocaval shunting for cases of diffuse hepatic vein occlusion or failed angioplasty. AIMS: To review the outcome of angioplasty and surgical shunting to define their respective roles. PATIENTS: All patients treated by angioplasty or surgical shunting for non-malignant hepatic vein obstruction over a ten year period from 1987 to 1996. METHODS: A case note review of pretreatment features and clinical outcome. RESULTS: Angioplasty was attempted in 21 patients with patent hepatic vein branches and was successful in 18; in three patients treatment was unsuccessful and these patients had surgical shunts. Fifteen patients were treated by surgical shunting only. Mortality according to definitive treatment was 3/18 following angioplasty and 8/18 following surgery; in most cases this reflected high risk status prior to treatment. Venous or shunt reocclusion rates were similar for both groups and were associated with subtherapeutic warfarin in half of these cases. Most surviving patients in both groups are asymptomatic although one surgical patient has chronic hepatic encephalopathy. CONCLUSION: With appropriate case selection, many patients with Budd-Chiari syndrome caused by short length hepatic vein stenosis or occlusion may be managed successfully by angioplasty alone. Medium term outcome is good following this procedure provided that anticoagulation is maintained. Further follow up is required to assess for definitive benefits but we suggest that this should be included as a valid initial approach in the algorithm for management of Budd-Chiari syndrome. (+info)Results of surgical treatment (modified Sugiura-Futagawa operation) of portal hypertension associated to complete splenomesoportal thrombosis and cirrhosis. (2/234)
BACKGROUND: Hemorrhagic portal hypertension, secondary to both intrahepatic and extrahepatic portal hypertension, is an uncommon entity. In this condition, the extrahepatic and the intrahepatic obstruction of the portal vein, due to chronic liver disease, produce a more severe form of hemorrhagic portal hypertension that is more difficult to control. The results of surgical treatment (modified Sugiura-Futagawa operation) in this subset of patients is analyzed. METHODS: Among 714 patients with a history of hemorrhagic portal hypertension, 14 cases were found with histologically proven liver cirrhosis and complete splenomesoportal thrombosis demonstrated by means of preoperative angiography. Patients with incomplete (partial) splenomesoportal thrombosis were excluded. There were nine males and 5 females with a mean age of 51 years. Alcoholic cirrhosis was demonstrated in 50% of the cases, post hepatitic cirrhosis in 28%, primary biliary cirrhosis in 7%, and cryptogenic cirrhosis in 14%. There were nine Child-Pugh A and 5 B cases. All cases were treated by means of our modified Sugiura-Futagawa procedure. RESULTS: Bleeding recurrence from esophagogastric varices was shown in one case, colonic varices in one case and hypertensive gastropathy in another of the survivors. Post operative encephalopathy was shown in 3 of the cases. The thirty-six month survival rate was 30% (Kaplan-Meier). CONCLUSIONS: The combination of intrahepatic plus extrahepatic portal hypertension has a worse prognosis. Treatment options are limited (sclerotherapy and/or devascularization), because shunt surgery, TIPS and liver transplantation have a very restricted role and postoperative outcome is poor. (+info)Successful twin pregnancy in a dual-transplant couple resulting from in-vitro fertilization and intracytoplasmic sperm injection: case report. (3/234)
There are numerous reports of successful pregnancy following liver transplantation. Little information is available regarding the incidence and management of infertility in transplant recipients, particularly the use of artificial reproductive technologies. We present a case of a successful twin pregnancy resulting from in-vitro fertilization with intracytoplasmic sperm injection (IVF/ICSI) in a liver transplant recipient, whose partner was a renal transplant recipient with severe oligozoospermia. With careful evaluation and monitoring, and the involvement of appropriate consultants, artificial reproductive technologies can be safely used in transplant recipient couples experiencing infertility. (+info)A 27-year experience with surgical treatment of Budd-Chiari syndrome. (4/234)
OBJECTIVE: To determine the effects of surgical portal decompression in Budd-Chiari syndrome (BCS) on survival, quality of life, shunt patency, liver function, portal hemodynamics, and hepatic morphology during periods ranging from 3.5 to 27 years. SUMMARY BACKGROUND DATA: Experiments in the authors' laboratory showed that surgical portal decompression reversed the deleterious effects of BCS on the liver. This study was aimed at determining whether similar benefit could be obtained in patients with BCS. METHODS: From 1972 to 1999, the authors conducted prospective studies of the treatment of 60 patients with BCS who were divided into three groups: the first had occlusion confined to the hepatic veins treated by direct side-to-side portacaval shunt (SSPCS); the second had occlusion involving the inferior vena cava (IVC) treated by a portal decompressive procedure that bypassed the obstructed IVC; and the third group, who had advanced cirrhosis and hepatic decompensation and were referred too late for treatment by portal decompression, required orthotopic liver transplantation. RESULTS: In the 32 patients with BCS resulting from hepatic vein occlusion alone, SSPCS had a surgical death rate of 3%, and 94% of the patients were alive 3.5 to 27 years after surgery. All 31 survivors remained free of ascites and almost all had normal liver function. No patient with a patent shunt had encephalopathy. The SSPCS remained patent in all but one patient. Liver biopsies showed no evidence of congestion or necrosis, and 48% of the biopsies were diagnosed as normal. Mesoatrial shunt was performed in eight patients with BCS caused by IVC thrombosis. All patients survived surgery, but five subsequently developed thrombosis of the synthetic graft and died. Because of the poor results, mesoatrial shunt was abandoned. Instead, a high-flow combination shunt was introduced, consisting of SSPCS combined with a cavoatrial shunt (CAS) through a Gore-Tex graft. There were no surgical or long-term deaths among 10 patients who underwent combined SSPCS and CAS, and the shunts functioned effectively during 4 to 16 years of follow-up. Ten patients with advanced cirrhosis were referred too late to benefit from surgical portal decompression, and they were approved and listed for orthotopic liver transplantation. Three patients died of liver failure while awaiting a transplant, and four patients died after the transplant. The 1- and 5-year survival rates were 40% and 30%, respectively. CONCLUSIONS: SSPCS in BCS with hepatic vein occlusion alone results in reversal of liver damage, correction of hemodynamic disturbances, prolonged survival, and good quality of life when performed early in the course of BCS. Similarly good results are obtained with combined SSPCS and CAS in patients with BCS resulting from IVC occlusion. In contrast, mesoatrial shunt has been discontinued in the authors' program because of an unacceptable incidence of graft thrombosis and death. In patients with advanced cirrhosis from long-standing, untreated BCS, orthotopic liver transplantation is the only hope of relief and results in the salvage of some patients. The key to long survival in BCS is prompt diagnosis and treatment by portal decompression. (+info)Successful liver transplantation in a patient with Budd-Chiari syndrome caused by homozygous factor V Leiden. (5/234)
Budd-Chiari syndrome (BCS) is a rare form of portal hypertension characterized by hepatic venous outflow obstruction. Although hematologic disorders are the most common cause of this syndrome, to date, 30% of the cases have been classified as idiopathic. Resistance to activated protein C caused by factor V Leiden is the most common cause of thrombophilia; its role in the pathogenesis of BCS is now becoming apparent. We report successful liver transplantation in a patient with BCS caused by homozygous factor V Leiden. The patient was administered standard heparin anticoagulation until activated protein C resistance was normalized by the liver allograft. Liver transplantation corrected the thrombophilic state. The patient has excellent graft function, is not on anticoagulation therapy, and has had no recurrent venous thrombosis at 5 months posttransplantation. Activated protein C resistance caused by the factor V Leiden mutation may be responsible for idiopathic cases of BCS. To avoid unnecessary long-term anticoagulation after liver transplantation, factor V Leiden should be considered as a pathogenic factor in BCS. In addition, because of the high prevalence of factor V Leiden in the world population, cadaveric organ donors with a history of venous thrombosis should be screened for activated protein C resistance lest thrombophilia be transmitted to the recipient. (+info)Factor V Leiden mutation, prothrombin gene mutation, and deficiencies in coagulation inhibitors associated with Budd-Chiari syndrome and portal vein thrombosis: results of a case-control study. (6/234)
In a collaborative multicenter case-control study, we investigated the effect of factor V Leiden mutation, prothrombin gene mutation, and inherited deficiencies of protein C, protein S, and antithrombin on the risk of Budd-Chiari syndrome (BCS) and portal vein thrombosis (PVT). We compared 43 BCS patients and 92 PVT patients with 474 population-based controls. The relative risk of BCS was 11.3 (95% CI 4.8-26.5) for individuals with factor V Leiden mutation, 2.1(95% CI 0.4-9.6) for those with prothrombin gene mutation, and 6.8 (95% CI 1.9-24.4) for those with protein C deficiency. The relative risk of PVT was 2.7 (95% CI 1.1-6.9) for individuals with factor V Leiden mutation, 1.4 (95% CI 0.4-5.2) for those with prothrombin gene mutation, and 4.6 (95% CI 1.5-14.1) for those with protein C deficiency. The relative risk of BCS or PVT was not increased in the presence of inherited protein S or antithrombin deficiency. Concurrence of either acquired or inherited thrombotic risk factors was observed in 26% of the BCS patients and 37% of the PVT patients. We conclude that factor V Leiden mutation and hereditary protein C deficiency appear to be important risk factors for BCS and PVT. Although the prevalence of the prothrombin gene mutation was increased, it was not found to be a significant risk factor for BCS and PVT. The coexistence of thrombogenic risk factors in many patients indicates that BCS and PVT can be the result of a combined effect of different pathogenetic mechanisms. (+info)Successful outcome of orthotopic liver transplantation in patients with preexisting malignant states. (7/234)
Preexisting malignancy is considered a relative contraindication to orthotopic liver transplantation (OLT) because of the risk of tumor recurrence. The purpose of this study is to assess the outcome of OLT in patients with a preexistent malignant state. Of 1,097 OLTs performed between 1989 and 1999 at King's College Hospital (London, UK), 18 patients had a pretransplantation malignant state, including 6 cases of myeloproliferative disorder (MPD) presenting as Budd-Chiari syndrome. Those patients with solid-organ malignancies had their tumor detected at an early stage and underwent curative treatment before or during OLT. Patients were followed up for a median of 71 months (range, 1 to 119 months) post-OLT, and the rates of rejection and malignancy were compared with those of transplant recipients without preexisting malignancy during the same period. One patient had a recurrence of his primary malignancy (non-Hodgkin's lymphoma) after 27 months, whereas another patient developed a de novo posttransplant lymphoproliferative disorder after 57 months. One patient with MPD developed acute leukemia 72 months after OLT. In comparison, of 1,079 OLTs performed in patients without preexisting malignancy during the same period, there were 34 cases of de novo malignancies. The rate of rejection in patients with and without preexisting malignancy was similar. Successful medium-term outcome after OLT can be achieved in carefully selected patients with preexisting malignancy providing the malignancy is amenable to curative treatment before or at OLT. Primary MPDs responsible for Budd-Chiari syndrome should not be considered a contraindication to OLT. (+info)Factor V Leiden related Budd-Chiari syndrome. (8/234)
BACKGROUND: The role of factor V Leiden as a cause of Budd-Chiari syndrome has only recently been described. AIMS: To assess the specific features of factor V Leiden related Budd-Chiari syndrome. PATIENTS: Sixty three consecutive patients with hepatic vein or terminal inferior vena cava thrombosis. METHODS: Standardised chart review. RESULTS: Factor V Leiden was found in 20 patients (31% (95% CI 20-43)). In the subgroup of patients with, compared with the subgroup without, factor V Leiden, a combination of prothrombotic states was more common (70% (95% CI 50-90) v 14% (95% CI 3-24)); inferior vena cava thrombosis was more frequent (40% (95% CI 19-61) v 7% (95% CI 0-14)); and distribution of initial alanine aminotransferase values was bimodal (almost normal or extremely increased) versus unimodal (p=0.003). Factor V Leiden accounted for four of five cases of massive ischaemic necrosis (transaminases >50-fold the upper limit of normal values) (p=0.014), and also for all three cases developing during pregnancy. Patients with and without factor V Leiden did not differ with regard to mortality, portosytemic shunting, or listing for liver transplantation. Hepatocellular carcinoma developed in two patients; both had factor V Leiden and indolent obstruction of the inferior vena cava. CONCLUSIONS: In patients with Budd-Chiari syndrome, factor V Leiden (a) is common; (b) precipitates thrombosis mostly when combined with another risk factor; (c) is associated with one of two contrasting clinical pictures: indolent thrombosis-particularly of the inferior vena cava-or massive ischaemic necrosis; and (d) is a major cofactor of Budd-Chiari syndrome developing during pregnancy. (+info)Budd-Chiari syndrome is a rare condition characterized by the obstruction of the hepatic veins, which are the blood vessels that carry blood from the liver to the heart. This obstruction can be caused by blood clots, tumors, or other abnormalities, and it can lead to a backflow of blood in the liver, resulting in various symptoms such as abdominal pain, swelling, and liver enlargement. In severe cases, Budd-Chiari syndrome can cause liver failure and other complications if left untreated. The diagnosis of this condition typically involves imaging tests such as ultrasound, CT scan, or MRI, and treatment may include anticoagulation therapy, thrombolytic therapy, or surgical intervention to remove the obstruction.
Interventional radiology (IR) is a subspecialty of radiology that uses minimally invasive image-guided procedures to diagnose and treat various medical conditions. The main goal of interventional radiology is to offer patients less invasive options for treatment, which can result in smaller incisions, reduced recovery time, and fewer complications compared to traditional open surgeries.
Interventional radiologists use a variety of imaging techniques, such as X-rays, fluoroscopy, computed tomography (CT), magnetic resonance imaging (MRI), and ultrasound, to guide catheters, wires, needles, and other small instruments through the body to target specific areas. These targeted interventions can be used for both diagnostic and therapeutic purposes, including:
1. Biopsies: Obtaining tissue samples from organs or tumors to determine a diagnosis.
2. Drainage procedures: Removing fluid from abscesses, cysts, or blocked areas to alleviate symptoms and promote healing.
3. Stent placements: Opening narrowed or obstructed blood vessels, bile ducts, or airways using small mesh tubes called stents.
4. Embolization: Blocking abnormal blood vessels or reducing blood flow to tumors, aneurysms, or other problematic areas.
5. Tumor ablation: Destroying tumors using heat (radiofrequency ablation, microwave ablation), cold (cryoablation), or other energy sources.
6. Pain management: Treating chronic pain by targeting specific nerves and blocking their transmission of pain signals.
7. Vascular access: Creating secure pathways to blood vessels for dialysis, chemotherapy, or other long-term treatments.
8. Aneurysm repair: Reinforcing weakened or bulging blood vessel walls using coils, stents, or flow diverters.
9. Vertebroplasty and kyphoplasty: Stabilizing fractured vertebrae in the spine to alleviate pain and improve mobility.
10. Uterine fibroid embolization: Reducing the size and symptoms of uterine fibroids by blocking their blood supply.
These are just a few examples of interventional radiology procedures. The field is constantly evolving, with new techniques and technologies being developed to improve patient care and outcomes. Interventional radiologists work closely with other medical specialists to provide minimally invasive treatment options for a wide range of conditions.
A Transjugular Intrahepatic Portosystemic Shunt (TIPS) is a medical procedure that creates an alternative pathway for blood flow from the portal vein to the hepatic vein within the liver. This shunt is composed of a stent, which is a small metal tube that is inserted into the liver using a long needle that is passed through a vein in the neck (jugular vein).
TIPS is typically used to treat complications of portal hypertension, such as variceal bleeding, ascites, and hepatic hydrothorax. By creating a shunt that bypasses the liver, TIPS reduces the pressure in the portal vein, which can help to alleviate these symptoms. However, because the shunt allows blood to bypass the liver, it can also impair liver function and lead to other complications, such as hepatic encephalopathy.
It is important to note that TIPS is a complex procedure that should only be performed by experienced interventional radiologists in a hospital setting with appropriate medical backup and monitoring capabilities.
Syringomyelia is a medical condition characterized by the formation of a fluid-filled cavity or cavities (syrinx) within the spinal cord. This syrinx can lead to various symptoms depending on its size and location, which may include pain, muscle weakness, numbness, and stiffness in the neck, back, shoulders, arms, or legs. In some cases, it may also affect bladder and bowel function, sexual performance, and the ability to maintain normal body temperature. Syringomyelia is often associated with Chiari malformation, a condition where the lower part of the brain extends into the spinal canal. However, other conditions such as spinal cord injuries, tumors, or infections may also cause syringomyelia.
A syndrome, in medical terms, is a set of symptoms that collectively indicate or characterize a disease, disorder, or underlying pathological process. It's essentially a collection of signs and/or symptoms that frequently occur together and can suggest a particular cause or condition, even though the exact physiological mechanisms might not be fully understood.
For example, Down syndrome is characterized by specific physical features, cognitive delays, and other developmental issues resulting from an extra copy of chromosome 21. Similarly, metabolic syndromes like diabetes mellitus type 2 involve a group of risk factors such as obesity, high blood pressure, high blood sugar, and abnormal cholesterol or triglyceride levels that collectively increase the risk of heart disease, stroke, and diabetes.
It's important to note that a syndrome is not a specific diagnosis; rather, it's a pattern of symptoms that can help guide further diagnostic evaluation and management.
The foramen magnum is the largest opening in the human skull, located at the base of the skull, through which the spinal cord connects to the brain. It is a crucial structure for the transmission of nerve impulses between the brain and the rest of the body. The foramen magnum also provides passage for blood vessels that supply the brainstem and upper spinal cord.
An Encephalocele is a type of neural tube defect that occurs when the bones of the skull do not close completely during fetal development. This results in a sac-like protrusion of the brain and the membranes that cover it through an opening in the skull. The sac may be visible on the scalp, forehead, or back of the head, and can vary in size. Encephaloceles can cause a range of symptoms, including developmental delays, intellectual disabilities, vision problems, and seizures, depending on the severity and location of the defect. Treatment typically involves surgical repair of the encephalocele soon after birth to prevent further damage to the brain and improve outcomes.
Platybasia is a medical term that refers to a condition where the base of the skull is flattened or broadened, resulting in an abnormal increase in the angle between the clivus (a part of the sphenoid bone) and the posterior aspect of the upper surface of the palatine bone. This condition can be congenital or acquired and is often associated with other skeletal abnormalities. In some cases, platybasia may lead to neurological symptoms such as headaches, neck pain, or even brainstem compression.
The posterior cranial fossa is a term used in anatomy to refer to the portion of the skull that forms the lower, back part of the cranial cavity. It is located between the occipital bone and the temporal bones, and it contains several important structures including the cerebellum, pons, medulla oblongata, and the lower cranial nerves (IX-XII). The posterior fossa also contains the foramen magnum, which is a large opening through which the spinal cord connects to the brainstem. This region of the skull is protected by the occipital bone, which forms the base of the skull and provides attachment for several neck muscles.
Meningomyelocele is a type of neural tube defect that affects the development of the spinal cord and the surrounding membranes known as meninges. In this condition, a portion of the spinal cord and meninges protrude through an opening in the spine, creating a sac-like structure on the back. This sac is usually covered by skin, but it may be open in some cases.
Meningomyelocele can result in various neurological deficits, including muscle weakness, paralysis, and loss of sensation below the level of the lesion. It can also cause bladder and bowel dysfunction, as well as problems with sexual function. The severity of these symptoms depends on the location and extent of the spinal cord defect.
Early diagnosis and treatment are crucial for managing meningomyelocele and preventing further complications. Treatment typically involves surgical closure of the opening in the spine to protect the spinal cord and prevent infection. Physical therapy, occupational therapy, and other supportive care measures may also be necessary to help individuals with meningomyelocele achieve their full potential for mobility and independence.
Surgical decompression is a medical procedure that involves relieving pressure on a nerve or tissue by creating additional space. This is typically accomplished through the removal of a portion of bone or other tissue that is causing the compression. The goal of surgical decompression is to alleviate symptoms such as pain, numbness, tingling, or weakness caused by the compression.
In the context of spinal disorders, surgical decompression is often used to treat conditions such as herniated discs, spinal stenosis, or bone spurs that are compressing nerves in the spine. The specific procedure used may vary depending on the location and severity of the compression, but common techniques include laminectomy, discectomy, and foraminotomy.
It's important to note that surgical decompression is a significant medical intervention that carries risks such as infection, bleeding, and injury to surrounding tissues. As with any surgery, it should be considered as a last resort after other conservative treatments have been tried and found to be ineffective. A thorough evaluation by a qualified medical professional is necessary to determine whether surgical decompression is appropriate in a given case.
The subarachnoid space is the area between the arachnoid mater and pia mater, which are two of the three membranes covering the brain and spinal cord (the third one being the dura mater). This space is filled with cerebrospinal fluid (CSF), which provides protection and cushioning to the central nervous system. The subarachnoid space also contains blood vessels that supply the brain and spinal cord with oxygen and nutrients. It's important to note that subarachnoid hemorrhage, a type of stroke, can occur when there is bleeding into this space.
A decompressive craniectomy is a neurosurgical procedure in which a portion of the skull is removed to allow the swollen brain to expand and reduce intracranial pressure. This surgical intervention is typically performed as a last resort in cases where other treatments for increased intracranial pressure, such as hyperosmolar therapy or drainage of cerebrospinal fluid, have been unsuccessful.
During the procedure, the surgeon creates an opening in the skull (craniectomy) and removes a piece of bone (bone flap). This exposes the brain and creates additional space for it to expand without being compressed by the rigid skull. The dura mater, the outermost protective layer surrounding the brain, may also be opened to provide further room for brain swelling.
Once the swelling has subsided, a second procedure known as cranioplasty is performed to replace the removed bone flap or use an artificial implant to restore the skull's integrity and protect the underlying brain tissue. The timing of cranioplasty can vary depending on individual patient factors and clinical conditions.
Decompressive craniectomy is most commonly used in the management of traumatic brain injuries, stroke-induced malignant cerebral edema, and intracranial hypertension due to various causes, such as infection or inflammation. While this procedure can be lifesaving in some cases, it may also lead to complications like seizures, hydrocephalus, or neurological deficits. Therefore, the decision to perform a decompressive craniectomy should be made carefully and on an individual basis, considering both the potential benefits and risks.
Down syndrome is a genetic disorder caused by the presence of all or part of a third copy of chromosome 21. It is characterized by intellectual and developmental disabilities, distinctive facial features, and sometimes physical growth delays and health problems. The condition affects approximately one in every 700 babies born in the United States.
Individuals with Down syndrome have varying degrees of cognitive impairment, ranging from mild to moderate or severe. They may also have delayed development, including late walking and talking, and may require additional support and education services throughout their lives.
People with Down syndrome are at increased risk for certain health conditions, such as congenital heart defects, respiratory infections, hearing loss, vision problems, gastrointestinal issues, and thyroid disorders. However, many individuals with Down syndrome live healthy and fulfilling lives with appropriate medical care and support.
The condition is named after John Langdon Down, an English physician who first described the syndrome in 1866.
The pelvic bones, also known as the hip bones, are a set of three irregularly shaped bones that connect to form the pelvic girdle in the lower part of the human body. They play a crucial role in supporting the spine and protecting the abdominal and pelvic organs.
The pelvic bones consist of three bones:
1. The ilium: This is the largest and uppermost bone, forming the majority of the hip bone and the broad, flaring part of the pelvis known as the wing of the ilium or the iliac crest, which can be felt on the side of the body.
2. The ischium: This is the lower and back portion of the pelvic bone that forms part of the sitting surface or the "sit bones."
3. The pubis: This is the front part of the pelvic bone, which connects to the other side at the pubic symphysis in the midline of the body.
The pelvic bones are joined together at the acetabulum, a cup-shaped socket that forms the hip joint and articulates with the head of the femur (thigh bone). The pelvic bones also have several openings for the passage of blood vessels, nerves, and reproductive and excretory organs.
The shape and size of the pelvic bones differ between males and females due to their different roles in childbirth and locomotion. Females typically have a wider and shallower pelvis than males to accommodate childbirth, while males usually have a narrower and deeper pelvis that is better suited for weight-bearing and movement.
The spinal canal is the bony, protective channel within the vertebral column that contains and houses the spinal cord. It extends from the foramen magnum at the base of the skull to the sacrum, where the spinal cord ends and forms the cauda equina. The spinal canal is formed by a series of vertebral bodies stacked on top of each other, intervertebral discs in between them, and the laminae and spinous processes that form the posterior elements of the vertebrae. The spinal canal provides protection to the spinal cord from external trauma and contains cerebrospinal fluid (CSF) that circulates around the cord, providing nutrients and cushioning. Any narrowing or compression of the spinal canal, known as spinal stenosis, can cause various neurological symptoms due to pressure on the spinal cord or nerve roots.
Cerebrospinal Fluid Pressure (CSFP) is the pressure exerted by the cerebrospinal fluid (CSF), a clear, colorless fluid that surrounds and protects the brain and spinal cord. CSF acts as a cushion for the brain, allowing it to float within the skull and protecting it from trauma.
The normal range of CSFP is typically between 6 and 18 cm of water (cm H2O) when measured in the lateral decubitus position (lying on one's side). Elevated CSFP can be a sign of various medical conditions, such as hydrocephalus, meningitis, or brain tumors. Conversely, low CSFP may indicate dehydration or other underlying health issues.
It is important to monitor and maintain normal CSFP levels, as abnormal pressure can lead to serious neurological complications, including damage to the optic nerve, cognitive impairment, and even death in severe cases. Regular monitoring of CSFP may be necessary for individuals with conditions that affect CSF production or absorption.
Metabolic syndrome, also known as Syndrome X, is a cluster of conditions that increase the risk of heart disease, stroke, and diabetes. It is not a single disease but a group of risk factors that often co-occur. According to the American Heart Association and the National Heart, Lung, and Blood Institute, a person has metabolic syndrome if they have any three of the following five conditions:
1. Abdominal obesity (waist circumference of 40 inches or more in men, and 35 inches or more in women)
2. Triglyceride level of 150 milligrams per deciliter of blood (mg/dL) or greater
3. HDL cholesterol level of less than 40 mg/dL in men or less than 50 mg/dL in women
4. Systolic blood pressure of 130 millimeters of mercury (mmHg) or greater, or diastolic blood pressure of 85 mmHg or greater
5. Fasting glucose level of 100 mg/dL or greater
Metabolic syndrome is thought to be caused by a combination of genetic and lifestyle factors, such as physical inactivity and a diet high in refined carbohydrates and unhealthy fats. Treatment typically involves making lifestyle changes, such as eating a healthy diet, getting regular exercise, and losing weight if necessary. In some cases, medication may also be needed to manage individual components of the syndrome, such as high blood pressure or high cholesterol.
Osteotomy is a surgical procedure in which a bone is cut to shorten, lengthen, or change its alignment. It is often performed to correct deformities or to realign bones that have been damaged by trauma or disease. The bone may be cut straight across (transverse osteotomy) or at an angle (oblique osteotomy). After the bone is cut, it can be realigned and held in place with pins, plates, or screws until it heals. This procedure is commonly performed on bones in the leg, such as the femur or tibia, but can also be done on other bones in the body.
Budd-Chiari syndrome
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Budd-Chiari syndrome - Wikipedia
Budd-Chiari syndrome - Wikipedia
Budd-Chiari Syndrome: Practice Essentials, Background, Pathophysiology
Survival and prognostic indicators of Budd-Chiari syndrome: a systematic review of 79 studies
Nuclear medicine dynamic investigations in the diagnosis of Budd-Chiari syndrome
Budd-Chiari syndrome
Budd-Chiari Syndrome: Practice Essentials, Background, Pathophysiology
Changing spectrum of Budd-Chiari syndrome in India with special reference to non-surgical treatment
Placement of a caudal vena cava stent for treatment of Budd-Chiari-like syndrome in a 4-month-old Ragdoll cat | Advanced...
Hepatic vein obstruction (Budd-Chiari): MedlinePlus Medical Encyclopedia
Budd-Chiari Syndrome Differential Diagnoses
Budd-Chiari syndrome CT - wikidoc
Budd-Chiari syndrome electrocardiogram - wikidoc
Budd-Chiari syndrome - Atlas of Ultrasound
Budd-Chiari Syndrome: Practice Essentials, Background, Pathophysiology
Budd-Chiari Syndrome | 5-Minute Clinical Consult
Budd-Chiari syndrome associated to antiphospholipid syndrome
Budd-Chiari syndrome - An overview | Capsule Health
The Diagnosis and Management of Budd-Chiari Syndrome
Budd-Chiari syndrome-UKMLA PLAB AMC USMLE Prometric MCQ
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Budd-Chiari Syndrome as A Manifestation of Right Atrium Myxoma
All You Need to Know About Pediatric Budd- Chiari Syndrome
Budd-Chiari Syndrome - Hepatic and Biliary Disorders - MSD Manual Professional Edition
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Pregnancy Outcomes in Women with Budd Chiari Syndrome or Portal Hypertension - ISTH Congress Abstracts
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Antiphospholipid syndrome6
- Other acquired hypercoagulable disorders that may result in Budd-Chiari syndrome include antiphospholipid syndrome and paroxysmal nocturnal hemoglobinuria, which are responsible for 10-12% and 7-12% of Budd-Chiari syndrome cases, respectively. (wikipedia.org)
- This study aims to report a clinical case of Budd-Chiari syndrome secondary to an antiphospholipid syndrome, a rather unusual association in literature. (scielo.edu.uy)
- Diagnosis of antiphospholipid syndrome. (scielo.edu.uy)
- Underlying prothrombotic disorders included myeloproliferative neoplasms (N=4), protein C deficiency (N=2), antiphospholipid syndrome (N=1) and cirrhosis (N=5) [Table 1]. (isth.org)
- An 18-year-old female with history of autoimmune hepatitis and triple positive antiphospholipid syndrome (APS) under Aco treatment with acenocoumarol, presented at the emergency room with acute abdominal pain. (journalmc.org)
- Nick has Antiphospholipid Syndrome and Budd Chiari. (caringbridge.org)
Thrombosis7
- Primary Budd-Chiari syndrome occurs due to thrombosis of the hepatic vein. (wikipedia.org)
- Inherited disorders of hypercoagulability may lead to thrombosis of the hepatic vein and Budd-Chiari syndrome. (wikipedia.org)
- Budd-Chiari syndrome is hepatic venous outflow tract obstruction due to either primary vascular occlusion (thrombosis) or secondary occlusion from extrinsic compression (eg, malignancy). (logicalimages.com)
- Alternatively, this syndrome is characterized by occlusion due to hepatic vein thrombosis or mechanical venous obstruction. (fortunejournals.com)
- Budd-Chiari syndrome (BCS) and portal vein thrombosis have been reported to be associated with JAK2V617F-positive hematopoiesis. (uludag.edu.tr)
- Prevalence of the JAK2V617F mutation in Chinese patients with Budd-Chiari syndrome and portal vein thrombosis: a prospective study. (hjradiology.org)
- Complications include iron deficiency (from chronic haemoglobinuria), progressive renal impairment (from haemoglobinuria), and the Budd Chiari syndrome (hepatic vein thrombosis). (lu.se)
Cause of Budd-Chiari s3
- The cause of Budd-Chiari syndrome can be found in more than 80% of patients. (wikipedia.org)
- Hepatic vein blockage is the most common cause of Budd-Chiari syndrome. (medlineplus.gov)
- We conclude that right atrial myxoma is a rare cause of Budd-Chiari syndrome. (fortunejournals.com)
Diagnosis of Budd-Chiari s1
- CT scan may be helpful in the diagnosis of Budd-Chiari syndrome. (wikidoc.org)
Pediatric Budd-Chiari s1
- Chaudhuri M, Jayaranganath M, Chandra VS. Percutaneous recanalization of an occluded hepatic vein in a difficult subset of pediatric Budd-Chiari syndrome. (medscape.com)
Prothrombotic2
- Hematologic diseases are the prothrombotic states more frequently associated to Budd-Chiari syndrome in the western world. (scielo.edu.uy)
- Prothrombotic conditions associated with Budd-Chiari syndrome. (medscape.com)
Occlusion2
- Overt Budd-Chiari syndrome generally requires the occlusion of at least 2 hepatic veins. (medscape.com)
- Budd-Chiari is a rare condition characterized by the occlusion of the hepatic vein flow out. (scielo.edu.uy)
Ascites5
- The fulminant syndrome presents early with encephalopathy and ascites. (wikipedia.org)
- Budd-Chiari syndrome is an uncommon condition induced by thrombotic or nonthrombotic obstruction of the hepatic venous outflow and is characterized by hepatomegaly, ascites, and abdominal pain. (medscape.com)
- CLINICAL RELEVANCE: Budd-Chiari-like syndrome is a rare phenomenon in veterinary medicine, and congenital malformations should be considered in young feline patients with ascites. (avmi.net)
- Ascites secondary to portal hypertension was suspected, probable Budd Chiari syndrome. (scielo.edu.uy)
- In 1899, Chiari described an "obliterating endophlebitis of the hepatic veins" and its association with hepatomegaly, ascites and abdominal pain. (medscape.com)
Obstruction of hepatic venous2
- Budd-Chiari syndrome is characterized by obstruction of hepatic venous flow at the level of right atrium, inferior vena cava, large hepatic veins, or hepatic venules [1]. (fortunejournals.com)
- Budd-Chiari syndrome is obstruction of hepatic venous outflow that originates anywhere from the small hepatic veins inside the liver to the inferior vena cava and right atrium. (msdmanuals.com)
Acute3
- The acute syndrome presents with rapidly progressive severe upper abdominal pain, yellow discoloration of the skin and whites of the eyes, liver enlargement, enlargement of the spleen, fluid accumulation within the peritoneal cavity, elevated liver enzymes, and eventually encephalopathy. (wikipedia.org)
- For patients with acute Budd-Chiari syndrome and fulminant liver failure, urgent liver transplant evaluation should be completed. (logicalimages.com)
- Budd-Chiari syndrome (BCS) has a wide spectrum of presentations, from an asymptomatic status to acute liver failure (ALF). (journalmc.org)
Venous obstruction2
- Tian ZL, Jia GL, Xi HL, Feng S, Wang XK, Li R. Investigation on etiology of hepatic venous obstruction Budd-Chiari syndrome. (medscape.com)
- Budd-Chiari syndrome (BCS) is a rare condition marked by a number of symptoms due to hepatic venous obstruction. (fortunejournals.com)
Hypercoagulable3
- Budd-Chiari syndrome may be the presenting sign of these hypercoagulable disorders. (wikipedia.org)
- Patients with underlying hypercoagulable states, most frequently myeloproliferative neoplasms, are at highest risk for primary Budd-Chiari syndrome, and multiple genes have been associated. (logicalimages.com)
- Several factors predispose to the development of Budd-Chiari syndrome, including hypercoagulable, inherited, and acquired conditions, as well as a variety of other causes, can be identified in approximately 75% of patients. (fortunejournals.com)
Portal hypertension2
- Women with Budd Chiari Syndrome (BCS) and/or portal hypertension are at high risk of pregnancy complications and adverse outcomes. (isth.org)
- Hamulyák EN, Wiegers HMG, van Duuren JR, Middeldorp S, Ganzevoort W. Pregnancy Outcomes in Women with Budd Chiari Syndrome or Portal Hypertension [abstract]. (isth.org)
Cirrhosis3
- Chronic Budd-Chiari syndrome can present with cirrhosis , which often results from months or years of outflow tract obstruction and subsequent ischemic hepatic injury. (logicalimages.com)
- Chronic Budd-Chiari syndrome can often be asymptomatic, and cirrhosis can be detected incidentally on imaging or laboratory testing demonstrating synthetic liver dysfunction. (logicalimages.com)
- Cutaneous manifestations of cirrhosis include jaundice, spider angiomata, skin telangiectasias ("paper money skin"), palmar erythema, white nails, disappearance of lunulae, and finger clubbing, especially in the setting of hepatopulmonary syndrome. (medscape.com)
Ultrasound2
- Budd-Chiari syndrome is most commonly diagnosed using ultrasound studies of the abdomen and retrograde angiography. (wikipedia.org)
- Budd-Chiari syndrome is readily diagnosed via abdominal ultrasound with Doppler flow demonstrating occlusive clot in the hepatic venous outflow tracts. (logicalimages.com)
Symptoms3
- Herein, our authors present a case of a 40-year-old female patient with Budd-Chiari syndrome in whom the right atrium myxoma was manifested with worsening of clinical symptoms despite medical treatment therapy. (fortunejournals.com)
- Symptoms of Allergic Tension Fatigue Syndrome can include edema , especially puffiness of the eyelids and fullness and discoloration of the infraorbital (below-eye) areas. (diagnose-me.com)
- Symptoms of tumor lysis syndrome such as muscle cramping, muscle weakness, confusion, visual loss or disturbances and shortness of breath. (medicines.org.uk)
Systematic Review2
- Qi X, Ren W, Wang Y, Guo X, Fan D. Survival and prognostic indicators of Budd-Chiari syndrome: a systematic review of 79 studies. (medscape.com)
- Redefining Budd-Chiari syndrome: A systematic review. (hjradiology.org)
Transjugular intra2
- Budd-Chiari syndrome: long-term effect on outcome with transjugular intrahepatic portosystemic shunt. (medscape.com)
- The Budd-Chiari syndrome: Outcome after treatment with the transjugular intrahepatic portosystemic shunt. (hjradiology.org)
Vascular1
- The clinical presentation of Budd-Chiari syndrome varies based on the acuity of vascular compromise. (logicalimages.com)
20181
- Byerly, SI 2018, Budd-chiari syndrome . (elsevierpure.com)
Thrombus1
- Multifactorial genesis of thrombus formation in Budd-Chiari syndrome. (raredis.org)
Mortality2
- Budd-Chiari syndrome may be associated with high mortality. (logicalimages.com)
- Rotterdam score predicts early mortality in Budd-Chiari syndrome, and surgical shunting prolongs transplant-free survival. (medscape.com)
Secondary3
- Secondary Budd-Chiari syndrome, which is very rare compared to the primary variant, is due to compression of the hepatic vein by an outside structure (such as a tumor or polycystic kidney disease). (wikipedia.org)
- Patients with malignancies or inflammatory conditions involving the gastrointestinal tract are at highest risk for development of secondary Budd-Chiari syndrome. (logicalimages.com)
- A diagnosis of Budd-Chiari-like syndrome secondary to a membranous obstruction of the caudal vena cava was made. (avmi.net)
Epidemiology1
- Budd-Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival - an 18-year experience. (medscape.com)
Hepatocellular2
- Hepatocellular carcinoma in Budd-Chiari syndrome: characteristics and risk factors. (medscape.com)
- Liu FY, Wang MQ, Duan F, Fan QS, Song P, Wang Y. Hepatocellular carcinoma associated with Budd-Chiari syndrome: imaging features and transcatheter arterial chemoembolization. (medscape.com)
Liver failure1
- The prognosis is poor in patients with Budd-Chiari syndrome who remain untreated, with death resulting from progressive liver failure in 3 months to 3 years from the time of the diagnosis. (medscape.com)
Paroxysmal nocturnal hemo1
- People who have paroxysmal nocturnal hemoglobinuria (PNH) appear to be especially at risk for Budd-Chiari syndrome, more than other forms of thrombophilia: up to 39% develop venous thromboses, and 12% may acquire Budd-Chiari. (wikipedia.org)
Asymptomatic1
- Sometimes Budd-Chiari syndrome begins during pregnancy and unmasks a previously asymptomatic hypercoagulability disorder. (msdmanuals.com)
Intrahepatic1
- To investigate long-term efficacy of transjugular intrahepatic porto-systemic shunt (TIPS) creation for the management of symptomatic Budd Chiari Syndrome (BCS) refractory to drug therapy. (hjradiology.org)
Idiopathic1
- These cases are known as idiopathic Budd-Chiari syndrome. (wikipedia.org)
Venules2
- Any obstruction of the venous vasculature of the liver is referred to as Budd-Chiari syndrome, from the venules to the right atrium. (wikipedia.org)
- Budd-Chiari syndrome (BCS) is a rare condition that refers to any obstruction of the hepatic venous outflow from the hepatic venules to the junction of the inferior vena cava (IVC) with the right atrium [ 1 , 2 ]. (journalmc.org)
Veno-occlusi2
- Budd-Chiari syndrome should be considered separate from veno-occlusive disease (VOD), also known as sinusoidal obstruction syndrome, which is characterized by toxin-induced, nonthrombotic obstruction of prehepatic veins (see the images below). (medscape.com)
- Outflow obstruction caused by the sinusoidal obstruction syndrome (formerly 'veno-occlusive disease') and cardiac disorders is excluded. (medscape.com)
Uncommon2
- Budd-Chiari syndrome is an uncommon disorder resulting from obstruction of the large hepatic veins or inferior vena cava at the suprahepatic level. (fortunejournals.com)
- Mirizzi syndrome refers to an uncommon phenomenon that results in extrinsic compression of an extrahepatic biliary duct from one or more calculi within the cystic duct or gallbladder . (radiopaedia.org)
Disorders1
- We searched PubMed without time restriction using key words: bone marrow and fibrosis as the main stem against the terms: growth factors, cytokines and chemokines, morphology, megakaryocytes and platelets, myeloproliferative disorders, myelodysplastic syndrome, collagen biosynthesis, mesenchymal stem cells, vitamins and minerals and hormones, and mechanism of tissue fibrosis. (bvsalud.org)
Biliary1
- It is a functional hepatic syndrome but can often present with biliary duct dilatation and can mimic other hepatobiliary pathologies such as cholangiocarcinoma 2 . (radiopaedia.org)
Clinical3
- Medicine Central , im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688517/0.2/Budd_Chiari_Syndrome. (unboundmedicine.com)
- [ 1 ] Budd did not associate any clinical features with this finding. (medscape.com)
- The description of the clinical features of hepatic vein outflow obstruction is generally attributed to a pathologist, Hans Chiari (although he was not the first). (medscape.com)
Therapeutic1
- Aiming at minimal invasiveness as a therapeutic strategy for Budd-Chiari syndrome. (hjradiology.org)
Survival1
- This paper aimed to systematically review the survival of Budd-Chiari syndrome and to identify the most robust prognostic predictors. (nih.gov)
Treatment2
- Intracardiac tumors have rarely been reported as a predisposing cause for the development of Budd-Chiari syndrome, and surgical removal of these tumors is generally a curative treatment for this syndrome [1, 3-6]. (fortunejournals.com)
- Expandable venous stents for treatment of the Budd-Chiari Syndrome. (hjradiology.org)
Outcome1
- Etiology, management, and outcome of the Budd-Chiari syndrome. (medscape.com)
Patients5
- The majority of patients have a slower-onset form of Budd-Chiari syndrome. (wikipedia.org)
- In the United States, Budd-Chiari syndrome typically presents in patients in their 30s or 40s with a slight female predominance, but it can occur in patients of all ages. (logicalimages.com)
- The diagnosis, initial management and long-term follow-up of patients with Budd-Chiari syndrome is reviewed. (medscape.com)
- TIPS for Budd-Chiari syndrome: Long-term results and prognostics factors in 124 patients. (hjradiology.org)
- The syndrome occurs in approximately 1 in every 1000 patients with gallstones 8 . (radiopaedia.org)
Diseases of the Liver1
- Budd G. On diseases of the liver. (hjradiology.org)
Prevalence1
- Incidence, prevalence, and complications of Budd-Chiari syndrome in South Korea: a nationwide, population-based study. (medscape.com)
Tuberculosis1
- Budd-Chiari syndrome is also seen in tuberculosis, congenital venous webs and occasionally in inferior vena caval stenosis. (wikipedia.org)
Acquire1
- up to 39% develop venous thromboses, [17] and 12% may acquire Budd-Chiari. (wikipedia.org)
Occurs1
- Usually, this syndrome occurs in 1/100,000 children in the general population worldwide. (tpaf.in)
Condition1
- Budd-Chiari syndrome is a very rare condition, affecting one in a million adults. (wikipedia.org)
Rare2
- Budd-Chiari syndrome is a rare disorder caused by hepatic venous outflow obstruction and resulting hepatic dysfunction. (medscape.com)
- Budd-Chiari syndrome is a rare liver disease and rarer in children. (tpaf.in)