Buga syndrome is a series of syndromes caused by obstruction of the hepatic vein or its adjacent inferior vena cava, resulting in obstruction of hepatic venous blood flow. The result is hepatosplenomegaly, hepatic impairment and ascites, and eventually cirrhosis. This disease belongs to the category of “bulging” in Chinese medicine, which is mostly caused by the obstruction of inferior vena cava.
1.Etiology and pathology.
(1) Etiology.
(1) Congenital obstruction of the inferior vena cava septum: During embryonic development, the inferior vena cava is formed by the fusion of five parts, namely the renal segment, the inferior renal segment, the superior renal segment, the hepatic segment and the superior hepatic segment. The hepatic segment is formed by the fusion of the sinusoidal gap and the inferior main vein, and the superior hepatic segment is derived from the yolk vein. If the hepatic and superior hepatic segments develop abnormally, a septum may form in the proximal inferior vena cava. The exact mechanism of septum formation is not known.
(ii) Inferior vena cava or hepatic vein thrombosis: The cause of thrombosis is unknown, but it is associated with diseases that predispose the blood to a hypercoagulable state. For example, true erythrocytosis, paroxysmal nocturnal hemoglobinuria, systemic lupus erythematosus, leukoaraiosis, chronic leukemia, long-term oral contraceptive pills, etc.
(3) Tumor: If hepatocellular carcinoma compresses or invades hepatic vein or inferior vena cava of hepatic segment, it may also form cancer thrombus along hepatic vein; in case of kidney cancer, cancer thrombus may extend along inferior vena cava to proximal side, resulting in venous obstruction.
(2) Pathology: The location of diaphragmatic obstruction of the inferior vena cava is relatively fixed, often located below the diaphragm to near the opening of the hepatic vein, which is equivalent to the level of the 8th-9th thoracic vertebrae. The diaphragm is often thin (but some can be several centimeters thick), composed of collagen fibers and a few elastic fibers, without muscular components. The septum is smooth and elastic, and some septa have a central hole or are sieve-shaped, which may later become fibrotic and close the hole, a phenomenon that may explain why congenital lesions develop in adulthood. The septum can be located above or below the opening of the hepatic vein, and if it is above the opening of the hepatic vein, it can lead to obstruction of blood flow in the inferior vena cava and the hepatic vein regardless of obstruction of the hepatic vein, resulting in inferior vena cava hypertension and post-hepatic portal hypertension. The inferior vena cava distal to the septum is often prone to thrombosis due to obstruction of blood flow.
Hepatic vein thrombosis occurs mostly near the confluence of the inferior vena cava and rarely within the lobules and central hepatic veins.
The pathologic changes in the liver are mainly due to extensive hepatic stasis caused by obstruction of hepatic venous blood flow. The entire liver is enlarged with blunt edges and a maroon appearance. A deep red area of stasis is visible in the liver section, next to which is a yellow color, which is the result of impaired fat metabolism. Because of its red and yellow color, it is called betel liver. The central hepatic vein is dilated, with thickened walls and sinusoidal gaps filled with red blood cells and infiltrated with necrotic and inflammatory cells. If the hepatic hypertension and stasis persist for a long time, the liver may develop fibrosis and hyperplasia, leading to hepatic sclerosis and portal hypertension, splenomegaly, hypersplenism, and esophageal varices. Increased pressure due to stasis in the hepatic veins and sinusoidal gaps increases the formation of hepatic lymphatic fluid, which overflows from the surface of the liver and forms ascites, which is high in protein and sometimes contains red blood cells.
Inferior vena cava obstruction causes obstruction of venous blood return to the lower extremities, resulting in varicose veins, edema, hyperpigmentation, and ulcers in both lower extremities. Varicose veins also appear in the chest and abdominal walls.
(3) Etiology and pathogenesis.
① Congenital deficiency: the spleen and stomach are weak, the spleen is not healthy, the dampness and turbidity are stagnant, the clear yang does not rise when it rises, the turbid yin does not descend when it descends, so that the clear and turbid are mixed and yong in the middle jiao. If the spleen and earth are stagnant, the liver loses its control, the qi and blood are stagnant and do not work, the water gradually accumulates and increases, coupled with the lack of kidney qi, the openness is not conducive, the water cannot be drained, so it becomes bulging.
②Emotional and moral depression: Qi is not regulated, the veins are damaged, the blood does not run smoothly, causing the liver veins to be depressed by blood stasis, and the liver is involved. On the other hand, liver qi is depressed and does not relax, crosses the stomach, the spleen and stomach are restrained, the transportation and transformation are not normal, water and dampness stay, water and dampness and blood stop and stagnate, and do not transform over time, and the middle jiao is blocked, the liver and spleen are sick together, and gradually affect the kidney, then all three organs are sick and become bloated.
2.Clinical manifestations and signs
Buga syndrome mostly occurs in young adults, with a higher incidence in men than in women. The onset of the disease is mostly slow, but there are occasional acute onset cases. The symptoms include abdominal distension, abdominal pain, nausea, loss of appetite and general weakness. Both lower limbs are swollen and have varicose veins. Examination may reveal hepatosplenomegaly, ascites, and occasionally mild jaundice. Longitudinal dilated veins are seen in the lateral thoracoabdominal wall and even in the lumbar back, and the direction of blood flow is from bottom to top. Significant concave edema and extensive varicose veins in the lower extremities, sometimes varicose veins of the spermatic cord and scrotal edema are seen. Pigmentation and ulceration of the lower legs are also seen. Sometimes the rupture of esophageal varices causes massive vomiting of blood and black stools. Due to the obstruction of the inferior vena cava, the amount of blood returned to the heart is reduced, so the heart function is poor, and the heart often feels short of breath after activity.
3.Auxiliary examination.
In the early stage of the disease, liver function is mostly normal, but in the later stage, there may be a decrease in serum albumin, mild increase in bilirubin and prolonged prothrombin time. Ultrasound can show the morphology, diameter, narrowing or obstruction of the inferior vena cava and hepatic vein, and whether there is thrombosis. This is a non-invasive test and can be repeated before and after the procedure for comparison. Imaging can show the site, length, morphology, presence of thrombus, degree of dilatation of the right hepatic vein and collateral circulation of the inferior vena cava stenosis or obstruction. Venous pressure measurement is feasible on both sides of the obstruction site, and the pressure on the distal side of the obstruction is significantly higher.
4.Cognitive treatment.
Buga syndrome belongs to the category of “bulging”, the occurrence of this disease is due to congenital deficiency, weak spleen and stomach, the spleen is not healthy, the clear yang does not rise, the essence of water and grain can not be transported to feed other organs; turbid yin does not descend, water and dampness can not be transferred to excrete outside the body, so the clear and turbid are mixed. At the same time, liver qi stagnation, blood cohesion, water-dampness and blood stasis congestion, can form a bulge. If the disease is prolonged for a long time, the liver and spleen will become deficient, and then the kidneys will be involved. The kidney yang is insufficient to warm the spleen and the kidney yin is deficient and the liver water is less nourishing, which makes the liver and spleen more exhausted and deficient. On the other hand, the kidney and the bladder are on the same side, so the deficiency of the kidney and the bladder is not conducive to qi-transformation, and the congestion of water and blood stasis is even worse, resulting in a critical condition.
Blood stasis in the liver and spleen: abdominal distention and fullness, angry veins, pain in the abdomen, dark face, blood mole on the head, neck and chest, filiform, purple-brown lips, thirst, inability to drink water, black stool, purple-red tongue or purple spots, thin and astringent or hollow pulse; treatment is recommended to activate blood stasis, move qi and promote water, which is an urgent remedy. If the water is too full, the pulse is stringent and strong, the physique is still good, can be attacked and expelled, can be used to attack and expel water qi, water qi reduction is still treating its stasis. However, attention must be paid to the spleen and stomach qi, not to attack and attack, it is appropriate to both attack and supplement, not to force quick results. We can add and subtract the following: Curcuma longa, Rhizoma Chuanxiong, Radix Angelicae Sinensis, Yuan Hu, Radix Paeoniae Alba, Qu Mai, Rhizoma Rheum, Betel nut, Chen Pi, Daggerbelly, Scapularia Scopariae, Poria, Mulberry Bark, Papaya, Fang Qi, etc.
Dampness and heat accumulation evidence: abdomen is large and full, the stomach and abdomen support urgent, face and skin yellow, irritable heat and bitterness, urine red and astringent, constipated stool, or pond scale deep yellow, tongue tip side red, yellow greasy moss or with gray and black, pulse string count. Treatment is appropriate to clear heat and water, attack and expel water, clear heat and dampness can be used in the full of the sub-consumption pill, attack and expel water temporarily with the boat car pill, the formula is as follows: thick park, Citrus aurantium, Poria, Chen Pi, Zelia, Huang Lian, rhubarb, Gansui, Daxi, papaya, Fangqi, etc..
Spleen and kidney yang deficiency: abdomen is large, but not very full, wide in the morning and urgent in the evening, pale yellow face, or white in the evening, chest stuffiness and dullness, tiredness and coldness, cold limbs or swelling of the lower limbs, white urine and short and unfavorable, light purple tongue, thin pulse. The treatment is to warm the kidney yang, promote water retention and reduce swelling, and the formula is as follows: Gui Zhi, Bai Zhu, Fu Ling, Pig Ling, Ze Xie, Pei Zi, Gan Jiang, Huo Xiang and Clove. Sometimes the Kidney Qi Pill is added to help warm the kidneys, transform the Qi and move water.
Yin deficiency of liver and kidney: abdominal distension, obscure face, purple lips, dry mouth, irritable heart, epistaxis or epistaxis, short urine, red tongue with less fluid, thin pulse. Treatment should be to nourish liver and kidney yin and blood, accompanied by moving qi and promoting water, using Dihuang Wan with the following formula: Sheng Di, Shan Yu Fei, Fu Ling, Dan Pi, Ze Di, Fructus Lycii, Shou Wu, etc. In case of epistaxis, add Cynthia sagittata and foxglove; in case of scanty urination, add Poria ling and slippery stone; in case of hot flushes and dry mouth, add Mai Dong, Xuan Shen, Chai Hu and Dibao Pi.
5.Western medicine treatment
Patients with Buga syndrome should actively seek surgical treatment as early as possible, otherwise they often die of liver failure, gastrointestinal bleeding or sepsis at a later stage. Commonly used surgical methods are.
(1) Direct surgery on inferior vena cava lesions.
(1) Balloon catheter dilation: for perforated membranous obstruction of the inferior vena cava. It is contraindicated in the presence of thrombosis in the inferior vena cava.
(2) Finger rupture via the right atrium: applicable to those with septal thickness less than 2 cm.
(③Septal dissection: Applicable to those with thick septum and stenosis in the inferior vena cava.
(2) Diversion procedure to reduce the pressure in the inferior vena cava and portal vein.
①Inferior vena cava-right atrium diversion: applicable to those with thicker inferior vena cava septum and unobstructed hepatic vein, which can directly reduce inferior vena cava pressure and indirectly reduce portal vein pressure. After the operation, the liver shrinks, ascites disappears, esophageal varices are reduced, and varices in the abdominal abdominal wall and lower limbs are significantly reduced. The long-term efficacy depends on the prevention of thrombosis, and postoperative anticoagulation therapy is required.
②Superior mesenteric vein-right atrial diversion: It is suitable for those who have a long segment of obstruction in the inferior vena cava of the posterior hepatic segment or complete obstruction in the hepatic vein, mainly to reduce portal hypertension. This procedure is effective for esophageal varices and intractable ascites, but postoperative hepatic encephalopathy is its disadvantage. To prevent the occurrence of hepatic encephalopathy, the anastomosis can be appropriately narrowed to reduce the amount of bypass blood, and the postoperative diet can be controlled to reduce the protein intake.
(③) Superior mesenteric vein-inferior vena cava diversion: it is only applicable to those who have hepatic vein obstruction while the inferior vena cava is patent.
Splenopulmonary fixation: the purpose of this operation is to establish portal pulmonary shunt through splenopulmonary fixation to relieve portal hypertension, which is effective for esophageal varices and ascites. Therefore, the surgery is not a direct venous diversion, so the effect of lowering the pressure is limited.
There are many other surgical options for Buga syndrome, and the choice of which to use depends on the overall condition of the patient in addition to the obstruction of the inferior vena cava and hepatic veins.