Bulga syndrome



Overview

Definition

Budd-Chiari syndrome (BCS), also known as hepatic vein obstruction syndrome, is a group of disorders characterized by portal hypertension or portal and inferior vena cava hypertension caused by obstruction of the hepatic vein or the inferior vena cava above its opening.

Most of the patients are caused by thrombosis or septum of the inferior vena cava or hepatic vein. Typical manifestations are ascites, lower limb edema, hepatosplenomegaly, and varicose veins of the abdominal wall.

Classification

The classification of Buga’s syndrome is very inconsistent, and currently the more recognized classification is hepatic vein obstruction type, inferior vena cava obstruction type and mixed type.

Incidence

Studies have shown that in non-Asian countries, Buchanan syndrome is more common in women, usually in their 20s or 30s, but also in children or older adults. In Asia, it is slightly more common in men, with a median age of onset of 45 years.

The prevalence of Buga syndrome is reported to be 2.4/1 million in foreign countries and 4~11/1 million in China.

With the development of modern medicine, especially diagnostic imaging technology, the number of newly discovered cases of Buga syndrome in China has been increasing, especially in China’s Yellow River and Huaihe River Basin in Shandong, Henan, Jiangsu and other high prevalence areas.

Questions you may be concerned about

What is Bugatti syndrome?

Buchanan syndrome is a group of diseases characterized by obstruction of the hepatic vein and the inferior vena cava, leading to portal hypertension and impaired vena cava return.

Clinical symptoms may include enlarged liver and spleen, ascites, varicose veins in the esophagogastric fundus, as well as lower limb edema, hyperpigmentation, lower limb ulcers and other symptoms.

In daily life, attention should be paid to a reasonable diet and adequate nutrition, and care should be taken to avoid excessive activity and overwork, which may lead to liver function damage.

The prognosis of Buga syndrome is poor. Therefore, patients should go to the hospital for examination and treatment in time, and choose the appropriate treatment method under the guidance of doctors. At the same time, patients who are not hospitalized for treatment should also be vigilant and pay attention to observe their own situation.

Causes

The cause of Buga syndrome is not very clear.

Causes

Congenital factors

Abnormal development of inferior vena cava, such as inferior vena cava septum, nonunion and atresia.

Hypercoagulability of blood

Certain congenital abnormalities of blood clotting substances, oral contraceptives, thrombocytosis, pregnancy and postpartum period.

Intravascular luminal non-thrombotic obstruction

Intravascular endothelioma, smooth muscle tumor/sarcoma, metastatic tumor in the lumen of inferior vena cava, etc.

Extravascular compression

Amebic liver abscess, hepatocellular carcinoma, hypertrophy of the caudal lobe of the liver and benign or malignant tumors of adjacent organs, etc.

Abdominal trauma

Abdominal trauma involving the inferior vena cava may cause stenosis, occlusion or thrombosis of the inferior vena cava vessels.

Unknown cause

The cause cannot be identified due to the complexity of the condition, also known as idiopathic Buga syndrome.

Symptoms

Main Symptoms

The underlying lesion of Bugart’s syndrome is stenosis or obstruction of the hepatic veins and/or the inferior vena cava, and its clinical manifestations can be categorized into two areas.

Hepatic vein obstruction syndrome

Symptoms and signs such as hepatosplenomegaly, ascites, hypersplenism, and gastrointestinal bleeding.

Hepatomegaly and liver function impairment

Siltation enlargement of the liver and absence of hepatic-jugular venous return phenomenon are typical signs of Budd-Chiari syndrome.

In the advanced stage, the liver begins to shrink, and most patients have cirrhosis and hepatic function impairment, such as spider nevus, jaundice, nosebleed, gum bleeding, emaciation, weakness, fatigue, loss of appetite and abdominal distension.

Most chronic patients have normal or mildly impaired liver function, while acute patients have obvious liver function impairment.

Intractable ascites

As ascites appears early, with the worsening of obstruction, the emergence of portal hypertension and the prolongation of the course of the disease, ascites gradually increases, and its degree is significantly heavier than that caused by cirrhosis, and the therapeutic effect of various diuretics is poor.

Acute patients more quickly produce a large number of persistent ascites, dyspnea, can not lie down, a significant reduction in urine output, abdominal distension is obvious patients can not tolerate, insomnia and a significant loss of appetite.

Upper gastrointestinal bleeding

Mainly due to esophagogastric fundus varices rupture bleeding, also seen in portal hypertension gastropathy, a few for ectopic varices rupture.

In mild cases, fecal occult blood, in severe cases, vomiting blood, tarry stools, is often the main cause of death of Bugart’s syndrome. Hepatic encephalopathy may be induced.

Splenomegaly

Splenomegaly is usually mild or moderate. Secondary hypersplenism may occur when the spleen is moderately enlarged.

Superficial abdominal wall varicose veins

The most common sign in patients with Buchanan’s syndrome and is more prominent than in general portal hypertension.

Hepatic vein obstruction alone causes only superficial varicose veins in the abdominal wall, radiating upward and downward from the umbilicus, the so-called “sea serpent’s head sign”.

Liver pain

Acute or chronic hepatic stasis and increased hepatic periosteal tension may cause persistent distension and pain in the upper abdomen or hepatic region, which is more obvious in the early stage, and reduced or not obvious in the late stage due to cirrhosis.

Inferior vena cava obstruction syndrome

The main manifestations of inferior vena cava obstruction syndrome include panic, chest tightness, shortness of breath, which is aggravated by activities; swelling, varicose veins, hyperpigmentation of both lower limbs, recurrent or intractable ulcers of unilateral or bilateral lower limbs, varicose veins of perineum or spermatic cord.

Lower extremity edema and varicose veins

Double lower extremity edema appears early, is bilateral, worsens after activity, and is accompanied by a feeling of distension.

The severity of varicose veins in the lower extremities is usually positively correlated with the duration and degree of inferior vena cava obstruction.

Perineal or spermatic varicose veins

In women, varicose veins of the perineum and labia majora are more common and often more severe.

In men, varicose veins of the spermatic cord and scrotal edema are common.

Superficial varicose veins of the upper trunk

Superficial varicose veins of the anterior and lateral walls of the chest and abdomen and the lumbar back are obvious, among which the superficial varicose veins of the lateral chest and abdominal walls and the lumbar region are the most common and characteristic manifestations with the most diagnostic and differential diagnostic value.

Gonadal function changes

Due to pelvic stasis and edema, the ovaries or testes are hypogonadal or degenerative atrophy.

Women manifest hypogonadism, menstrual disorders or amenorrhea, infertility; men manifest hypogonadism and impotence.

Impairment of renal function

Impairment of renal venous blood return, causing renal venous hypertension.

Sedimentary portal hypertension leads to the formation of a large amount of ascites, and the pressure in the abdominal cavity rises, compressing the renal veins and affecting renal blood perfusion.

Hepatic function is impaired, toxic products, vasoactive substances and pseudo-neuromediators accumulate and endotoxemia is formed.

The main manifestations are oliguria or anuria, hypoproteinemia, azotemia, elevated blood creatinine and blood urea nitrogen, and acute renal failure can be induced in severe cases.

Complications

Ascites.

Liver failure.

Upper gastrointestinal bleeding.

Spontaneous bacterial peritonitis.

Hypersplenism.

Infertility.

Consultation

Department of Medicine

Gastroenterology

Gastroenterology is recommended for symptoms such as abdominal pain, abdominal distension, hepatosplenomegaly, ascites, bilateral lower extremity edema, jaundice, and blood in stool.

Hepatobiliary Surgery

If the above symptoms occur due to abdominal trauma or imaging tests suggesting abnormal development or blockage of veins, we recommend seeking treatment from the Department of Hepatobiliary Surgery or Vascular Surgery as soon as possible.

Preparation

Consultation: Registration, Preparation of documents, Frequently Asked Questions

Tips for medical treatment

Try to record the symptoms and duration of symptoms, and whether there were any triggering events before the onset of symptoms.

Bring all images and labs from previous visits.

Preparation Checklist

Symptom Checklist

Pay particular attention to the time of onset of symptoms, specific presentations, etc.

Any epigastric pain or bloating?

Are both legs swollen?

Is there blood in the stool?

Is your skin yellow?

Do you feel irritable?

How long have the above symptoms lasted?

List of medical history

Any trauma to the stomach?

Any anxiety, depression, insomnia?

Any long-term use of birth control pills?

Any blood disease?

Any history of malignant tumor?

Checklist

Test results in the past six months, which can be brought to the doctor’s office

Laboratory tests: blood routine, biochemistry, tumor markers, etc.

Imaging examination: abdominal ultrasound, abdominal CT, abdominal MRI, etc.

Pathological histology: liver tissue biopsy.

Medication list

Medication used in the last 3 months, if available, bring the box or package to the doctor’s appointment

Contraceptives: e.g. medroxyprogesterone acetate, norethindrone, levonorgestrel, etc.

Diagnosis

Diagnosis based on

medical history

The following medical history may be present:

History of abdominal trauma.

Long-term use of oral contraceptives.

There is a history of malignancy.

History of hematologic disorders.

Clinical manifestations

Symptoms

Abdominal pain, abdominal distension, lower extremity edema, jaundice, and blood in stool may occur.

Physical signs

Abdominal pressure, rebound pain, muscle tension; spider nevus, liver palms may be present.

Laboratory Tests

Routine blood tests

If there is upper gastrointestinal bleeding or splenomegaly or hypersplenism, there may be anemia or concomitant decrease in platelet and white blood cell counts.

Biochemistry

There may be an increase in serum bilirubin, elevation of alanine aminotransferase, glutamine aminotransferase, alkaline phosphatase, and a decrease in serum albumin.

Coagulation

Prolonged prothrombin time may be seen.

Imaging

Abdominal ultrasound

Doppler ultrasound is widely used and does not require contrast or radiation exposure.

Ultrasound findings include hepatomegaly, splenomegaly, ascites, and compression or obstruction of the hepatic and inferior vena cava.

Abdominal CT

CT scans of Bugatti syndrome may indicate delayed filling or filling defects of the main hepatic veins, a patchy “flea-bite” appearance of the liver, rapid clearance of contrast from the caudate lobe, narrowing of the inferior vena cava, and/or contrast opacification.

Abdominal MRI

MRI of the abdomen may show low intensity signal in the liver parenchyma, openness of the hepatic veins and inferior vena cava, and typical “comma-shaped” twisting of the intrahepatic venous collateral vessels.

Hepatic venography

Venography should be performed if non-invasive tests are negative or non-diagnostic, but the disease is strongly suspected clinically.

In addition, venography can identify the involved vessels and thus can also be used to guide subsequent treatment.

Liver tissue biopsy

Histologic features include central zone bruising, necrosis, and hemorrhage. Large regenerative nodules, obstructive portal vein lesions and fibrosis/cirrhosis may also be found.

Differential Diagnosis

Peptic ulcer

Similarities: Both may present with epigastric pain, vomiting blood, black stools, anemia and other GI symptoms.

Differences: Buerger’s syndrome is often accompanied by ascites, bilateral lower extremity edema, and jaundice, while peptic ulcers often have periodic episodes of rhythmic epigastric pain.

Cirrhosis portal hypertension

Similarities: Both can present with esophagogastric fundal varices. Hepatocellular necrosis, hepatitis, and fibrosis may be present on liver tissue biopsy.

Differences: Bugart’s syndrome may reveal obstruction of the hepatic veins or the inferior vena cava above their openings, whereas cirrhotic portal hypertension alone usually does not. Patients with cirrhotic portal hypertension tend to have a history of chronic hepatitis, and laboratory tests often show hepatitis viral markers, impairment of liver function, and atrophic sclerosis of the liver.

Constrictive pericarditis

Similarities: Both may present with clinical symptoms such as hepatomegaly, ascites, lower limb edema, decreased activity tolerance and dyspnea.

Differences: Cardiac ultrasound in patients with constrictive pericarditis may show thickening and adhesion of the pericardium, deformation of the heart, and weakening of ventricular wall movement.

Treatment

Treatment purpose: improve liver and kidney function, eliminate ascites, prevent and treat lethal complications such as gastrointestinal hemorrhage, prolong patient’s life sludge liver decompression as well as prevent or treat complications.

Treatment principle: make hepatic vein and inferior vena cava recanalization, strengthen nutritional support and symptomatic treatment measures.

General treatment

Bed rest, appropriate elevation of the head of the bed, double lower limb massage to avoid the formation of deep vein thrombosis in the lower limbs.

Strengthen the nutrition, with high protein and high calorie diet.

Keep the bowel clear, avoid constipation and excessive force during bowel movement.

Correct the underlying diseases that may lead to Bugart’s syndrome as much as possible.

Fasting is required when vomiting blood and black stools.

If there is a clear cause or trigger, it should be removed, e.g. oral contraceptives should be stopped.

Medication

Hepatoprotection, protein infusion, diuretic drugs for symptomatic treatment, strengthen nutritional support.

Patients with hypercoagulable state can be treated with anticoagulation therapy, including warfarin, low molecular heparin, etc. Thrombolytic therapy can be adopted for Buga syndrome caused by acute thrombosis.

For patients with gastrointestinal bleeding combined with portal hypertension, growth inhibitors, acid-suppressing drugs, hemostatic drugs, etc. can be used.

Interventional therapy

Interventional therapy has become the first-line treatment option for BCS.

Indications

Membranous or segmental obstruction at the opening of the hepatic vein.

Membranous or segmental obstruction of the inferior vena cava.

Combination of old thrombus distal to the obstruction in the inferior vena cava and hepatic vein.

Ineffective pharmacologic treatment.

Restenosis after hepatic and inferior vena cava angioplasty.

Obstruction of the shunt tract after surgical shunt surgery.

Treatment

Mainly include balloon dilatation (PTA), endovascular stenting (EMS), percutaneous or transjugular intrahepatic portosystemic shunt (TIPS).

Postoperative precautions

Anticoagulant or antiplatelet drugs, such as low molecular heparin and aspirin, should be applied for at least 3~6 months after the intervention.

Regularly review ultrasound, pay attention to the presence of thrombosis, stent restenosis and stent displacement.

Surgery

With the development of interventional techniques, surgery is now less frequently used as the treatment of choice for Buga syndrome.

Commonly used surgical procedures include lateral-lateral portacaval shunt, intestinal-venous vena cava shunt, portacaval-atrial and intestinal-atrial shunts and other portacaval shunts, and liver transplantation.

Questions you may be concerned about

Does Bugart’s syndrome require surgery once every three years?

There are no clinical studies to prove that Buga syndrome requires triennial surgery. Treatment options for Buga syndrome include general treatment, surgery, and interventional therapy, with the specific treatment plan and duration depending on the patient’s specific condition.

Bugart’s syndrome can be cured or clinically cured after appropriate treatment under the guidance of doctors, so it does not need surgery once every three years.

1. General treatment: patients need to keep bed rest, massage both lower limbs to avoid the formation of deep vein thrombosis in the lower limbs; strengthen the nutrition; avoid too much fluid rehydration.

2. Surgical treatment: The principle of surgical treatment for Buga syndrome is to relieve portal and inferior vena cava hypertension at the same time, and when it is impossible to take care of both, portal hypertension and its complications should be targeted first. The main surgical methods include simple septal resection, transabdominal radical surgery, enteroatrial diversion and so on.

3. Interventional therapy: it is the first choice of treatment for Buga syndrome, which has the advantages of small trauma and fast recovery. Commonly used interventional treatments include percutaneous endoluminal angioplasty, endovascular stent implantation, etc. After the operation, patients should pay special attention to close observation and regular review. After intervention, the survival rate of patients can be significantly improved. However, some patients may still have a relapse, which requires prompt treatment.

If the diagnosis of Bugatti syndrome is confirmed, the patient should not be too anxious, but should go to the hospital in time and actively cooperate with the doctor’s treatment, so as to avoid delaying the condition.

Prognosis

Cure

The prognosis of patients with Bugatti syndrome depends on the extent of the disease and whether the treatment is timely.

The 5-year survival rate is more than 90% after recanalization of the obstructed vessel; although restenosis may occur in about 10% of the patients, the 5-year survival rate is still more than 85% after re-intervention. Primary liver cancer occurs in about 3.5% of patients during the course of the disease.

Hazards

Symptomatic Buga syndrome has a high mortality rate if left untreated. Studies have shown that 90% of patients die within 3 years. Patients often die of intractable ascites with wasting, gastrointestinal bleeding, and liver failure.

Survival rates are high with treatment of Buga syndrome. Factors associated with a poorer prognosis in treated patients include older age at diagnosis, chronic disease at presentation, severe liver failure, and recalcitrant ascites. In addition, the presence of portal vein obstruction in patients with Bugart’s syndrome limits treatment options and has a poorer prognosis.

Daily

Daily Management

Dietary management

Eat a diet appropriately rich in high-quality protein, highly nutritious, low-fat, and non-scummy foods.

Avoid rough and hard foods as much as possible.

Life Management

Exercise appropriately, but should avoid doing strenuous sports, it is advisable to combine work and rest.

Avoid prolonged bed rest.

Psychological support

Regulate emotions and keep a positive and optimistic mind.

Disease monitoring

Laboratory examination (blood routine, blood biochemistry, coagulation function) every 3 months. Gastroscopy every 1-2 years for those with combined varicose veins, and liver puncture biopsy when necessary for those with severe liver function abnormalities; if the results are stable, the frequency of examination can be reduced to once every 2-3 years.

Non-invasive methods for detecting liver fibrosis (e.g., ultrasound-based transient elastography) are regularly used to assess liver fibrosis or cirrhosis.

Patients with compensated cirrhosis due to Buga syndrome should be monitored for complications of portal hypertension.

Patients with cirrhosis should be monitored for the development of primary liver cancer.

Monitor patients for myeloproliferative tumor transformation.

Prevention

There is no specific method of prevention for Bugart’s syndrome, but the following can reduce the risk of Bugart’s syndrome

Modify poor eating habits and maintain a light diet.

Avoid abuse of sex hormone-containing drugs, such as birth control pills.

Actively treat related diseases that predispose to Bugart’s syndrome, such as cirrhosis of the liver and chronic hepatitis.

Conduct regular health checkups.