jellyfish head sign
Introduction
Introduction Sea snake head sign ("Jellyfish head" sign) - cerebral venous malformation (brain developmental venous abnormality), abnormally dilated medullary vein leads into 1 or 2 large drainage veins, forming a "sea snake head" sign, without blood supply Arterial and direct arteriovenous short circuit. When the portal vein is high, the umbilical vein is reopened, and it is connected with the secondary umbilical vein and the abdominal wall vein. A curved vein is formed on the abdominal wall of the umbilicus. The direction of blood flow is: the umbilicus is upward and the umbilicus is downward. This direction of blood flow can be differentiated from the collateral circulation caused by upper or lower vena cava obstruction. The prominent varicose veins of the abdominal wall can be called the head of the jellyfish. It is a clinical manifestation of portal hypertension. Portal hypertension is a group of syndromes caused by a persistent increase in portal pressure. The vast majority of patients are caused by cirrhosis, and a small number of patients are secondary to portal vein or hepatic vein obstruction and some unexplained factors.
Cause
Cause
The causes of portal hypertension are different, and the cause of the disease has not been fully elucidated so far, but the blockage of portal vein blood flow is the root cause of its disease, but it is not the only cause. In the past, the etiology of this disease has been classified into many categories, such as Whipple, Leevy, Sherlock, Dolle, Friedel, and so on. According to the research progress of the pathogenesis of this disease in recent years, combined with its etiology and anatomy, and considering the simple and practical, the Bass & Sombry classification method is introduced.
1. Primary blood flow increase
(1) Arterial-portal fistula (including intrahepatic, intrasplenic and other internal organs).
(2) spleen capillary hemangioma.
(3) cavernous hemangioma of the portal vein.
(4) Non-hepatic splenomegaly (such as polycythemia vera, leukemia, lymphoma, etc.).
2. Primary blood flow resistance increase
(1) Pre-liver type: Incidence rate <5%.
1 thrombosis: portal vein thrombosis; splenic vein thrombosis; portal vein sponge-like changes.
2 portal vein or splenic vein is compressed or infiltrated by foreign tumor or pseudo-pancreatic cyst, or portal vein tumor thrombus.
(2) intrahepatic type: the incidence rate accounts for 90%.
1 anterior sinus type: early schistosomiasis, congenital liver fibrosis, idiopathic portal hypertension, early primary biliary cirrhosis, cholangitis, hepatolenticular degeneration, arsenic poisoning, azathioprine hepatotoxicity, bone marrow fiber (early), sarcoidosis, myeloproliferative diseases, etc.
2 sinus type / mixed type: hepatitis cirrhosis, alcoholic cirrhosis, fatty liver, incomplete septal fibrosis, hepatocyte nodular regenerative hyperplasia, vitamin A poisoning, methotrexate poisoning, advanced schistosomiasis and cholangitis.
3 posterior sinus type: hepatic vein thrombosis or embolism, Budd-Karst syndrome.
(3) Post-hepatic type: 1%. Inferior vena cava occlusive disease, constrictive pericarditis, chronic right heart failure, tricuspid dysfunction (congenital, rheumatic).
Examine
an examination
Related inspection
Blood routine gastroscope
The typical "Jellyfish" sign, we usually call it venous tumor, DSA is the gold standard for its diagnosis. This kind of vascular malformation is quite common. Any MR image with good image quality can be easily observed.
Diagnostic points: arterial phase, capillary phase normal, medullary vein expansion & contrast agent retention, typical manifestations of the sea snake head sign / "jelly head" sign. In fact, some cases in the so-called "hidden vascular malformations" may have such missed diagnosis. MR enhancement is of great value in the diagnosis of cerebral developmental venous anomalies.
The diagnosis of portal hypertension is generally not difficult, mainly based on splenomegaly, hypersplenism, lower esophageal varices or upper gastrointestinal bleeding and ascites. In addition to the diagnosis of portal hypertension, it should be judged whether it is intrahepatic or extrahepatic; what is the cause of obstruction. Auxiliary examination: blood routine test, gastroscope or barium meal, color Doppler B-ultrasound, portal venography and liver biopsy. Sometimes it needs to be differentiated from other diseases with similar symptoms and signs.
Diagnosis
Differential diagnosis
1. Idiopathic portal hypertension (Banti syndrome): In 1882, Italian scholar Banti first noticed anemia associated with known hematological diseases and other diseases with splenomegaly, so it was called Banti syndrome. Its etiology and pathogenesis are still unclear, and may be related to exposure to toxicants, infections, immunity, genetics and other factors. The liver histology showed "liver occlusive portal vein disease", and the large and medium branches of the intrahepatic portal vein showed obvious subendothelial thickening and fibrosis around the bile duct. Clinically, it is common to have insidious onset, mostly with left upper abdominal mass as the main complaint, but also gastrointestinal bleeding, anemia, edema, etc. Physical examination shows splenomegaly, obvious anemia, liver is not large, a few can see abdominal wall venous engorgement, jaundice and ascites Rare, hepatic encephalopathy is rare. Anemia is positive cell positive pigmentation or positive cell hypochromia, and it can also be seen that whole blood cells are reduced, liver function is normal or mild abnormality. The disease is better with shunt or interrupted surgery, and the prognosis is good. The disease needs to be differentiated from cirrhotic portal hypertension. The pathological examination of liver tissue is confirmed to have no diffuse regenerative nodules, and all kinds of cirrhosis, schistosomiasis liver fibrosis and extrahepatic portal vein obstruction are excluded.
2. Budd-Chiari syndrome: due to hepatic vein, hepatic inferior vena cava thrombosis or tumor thrombosis, membranous stenosis or occlusion and certain heart diseases can cause obstruction of hepatic vein outflow, making the liver Pathological changes such as hepatic sinus congestion, hemorrhage, necrosis, etc., eventually lead to a group of clinical syndromes of post-sinus portal hypertension. Pathologically, it is divided into three types: thrombosis, membranous, and fibrous stenosis. The clinical manifestations were firstly related to the obstruction site. The patients with hepatic vein obstruction mainly showed abdominal pain, hepatomegaly, tenderness and ascites. The inferior vena cava obstruction still had lower extremity edema, ulcer, pigmentation and even varicose veins. Proteinuria or nephrotic syndrome may occur in the lesion involving the renal vein. Abdominal ultrasound, Doppler, CT, magnetic resonance imaging, liver or inferior vena cava angiography can help to confirm the diagnosis. Surgical and non-surgical treatments are still effective, which can significantly improve the prognosis of patients.
3. Hepatic venular occlusive disease: due to factors such as monocrotaline, chemotherapy drugs, poisons, radiotherapy and other factors caused by intrahepatic central vein and sublobular vein endothelial swelling or fibrosis, caused by stenosis or even occlusion. The clinical manifestations are very similar to Bu-ka syndrome. Due to hepatomegaly, ascites and edema due to hepatic venous outflow obstruction, the patient has a sharp onset, severe pain in the upper abdomen, abdominal distension, rapid ascites, hepatomegaly, tenderness and so on. Most patients may have prodromal symptoms in the respiratory tract, gastrointestinal tract or system before the onset, but also may have symptoms such as fever, loss of appetite, nausea, vomiting, diarrhea, etc., but jaundice, splenomegaly and lower extremity edema are rare, and more often in the acute phase. There is obvious abnormal liver function. About half of the disease recovered within 2 to 6 weeks, 20% died of liver failure, and a few developed cirrhotic portal hypertension. The diagnosis of this disease mainly depends on liver biopsy, and the biopsy under laparoscopy is the most diagnostic.
4. Splenic diseases: Many diseases, especially blood and lymphatic diseases and some infectious diseases, may have splenomegaly, or secondary to portal hypertension; some diseases of the spleen itself also show splenomegaly;
(1) Hodgkin's disease and other lymphomas: These diseases are malignant tumors originating from lymphatic network, including Hodgkin's disease, lymphosarcoma, reticuloma.
1 systemic manifestations: fever, night sweats, weight loss, fatigue and anemia, especially early misdiagnosis.
2 liver, spleen, lymph node enlargement: about 20% to 50% have splenomegaly, especially the spleen of spleen-type Hodgkin's disease can be extremely swollen; superficial lymph node enlargement, cervical lymph node enlargement (accounting for 60 More than %); liver is large, accounting for about 10%.
3 bone and skin damage (such as intradermal nodules and mycosis fungoides, erythema and eczema, etc.).
4X-ray examination, such as lung, mediastinum and bone damage, can have a positive finding.
5 blood, early red blood cells only decreased, in the late stage, whole blood cells decreased.
6 bone marrow puncture and lymph node puncture smear or biopsy If you find Li-Shi (Reed-Sternderg) cells can confirm the disease.
(2) Leukemia:
1 systemic manifestations: fever, anemia and bleeding tendency, late stage have cachexia.
2 liver, spleen, lymph nodes, large spleen, can be extremely swollen.
3 skin, bone and gastrointestinal involvement can cause the corresponding symptoms.
4 leukocytosis is a feature of this disease, most of which are above 10.0 × 109 / L, can be as high as 100.0 × 109 / L.
5 bone marrow: diffuse hyperplasia, red blood cells and megakaryocyte system decreased.
(3) hereditary spherocytosis: also known as familial hemolytic anemia or chronic hereditary hemolytic jaundice. Its characteristics:
1 obvious family history: more than 10 years old before the onset.
2 clinical manifestations: mild anemia, jaundice may be optional (for hemolytic), hepatosplenomegaly with splenomegaly (70% to 80%).
3 If the mood is fluctuating or infection is prone to hemolytic crisis: the sudden onset of anemia, hemoglobin drops to 30g / L, accompanied by fever, chills, vomiting, liver and spleen pain, jaundice deepening, can last for several days More than 10 days.
4 blood: red blood cells and hemoglobin decreased, reticulocyte count increased significantly (5% ~ 20%), showing small spherical cells increased, red blood cell osmotic fragility test increased, anti-human globulin test negative.
5 bone marrow showed that the red blood cell system proliferated actively, and the main yolk and young erythrocytes increased.
6X line examination showed that the skull and hand bones became thinner and the medullary cavity was widened. The splenectomy effect of this disease is good, and surgery is more suitable for 4 years old.
(4) Autoimmune hemolytic anemia: for some reasons, autoantibodies are produced in the blood, and the antigen-antibody complex is formed on the surface of the red blood cells, so that the red blood cells are destroyed to cause hemolysis. The disease is divided into acute and chronic. Acute is primary, more common in children. Chronic people are more common in adult women, often secondary, mild anemia.
1 performance: dizziness, headache, fatigue, loss of appetite and so on.
2 blood examination: red blood cells and hemoglobin decreased to varying degrees, reticulocytes significantly increased, up to 50%.
3 anti-human globulin test (Coombs test) positive for the diagnosis of this disease.
(5) Idiopathic thrombocytopenic purpura: The etiology of this disease has not been clarified so far. Divided into two categories of acute and chronic.
1 acute seen in children. 1 to 3 weeks before onset, there is often a history of upper respiratory tract infection, chickenpox, measles and viral infection; acute onset; bleeding spots on the skin mucosa; history of bleeding from the nose and mouth and gums, and even gastrointestinal, urinary and intracranial hemorrhage; The spleen is often swollen.
2 Chronic patients are more common in young women, slow onset, long-term menorrhagia, bleeding gums, subcutaneous ecchymosis; bleeding is not serious, but often repeated episodes, mild splenomegaly.
3 bone marrow examination found that platelets, megakaryocytes decreased or disappeared can be diagnosed.
(6) Black fever:
1 The spleen is markedly enlarged, and the liver can be slightly enlarged. If the reticuloendothelial cells proliferate and compress the small arteries, a focal infarction can occur, and the spleen area can be painful and can hear the rubbing sound.
2 The skin pigmentation is deepened, the superficial lymph nodes are swollen, and the reticuloendothelial cells containing the protozoa can be found in the lymph nodes. The protozoa are contained in the skin and subcutaneous nodules.
3 blood. Complete blood cell reduction, especially neutrophils, can be <2.0 × 109 / L, bone marrow puncture to find the pathogen can be diagnosed.
(7) Chronic schistosomiasis:
1 There is a history of life in the epidemic area of the disease. Patients often have intestinal lesions and chronic diarrhea, which may have pus and blood.
2 spleen can be very large, hard; more with ascites.
3 sigmoid colonoscopy, in the sigmoid colon and rectum at the junction of the intestinal wall to do biopsy, found that the positive rate of eggs is quite high, you can identify.
(8) Chronic malaria:
1 has a history of malaria or a recent history of recurrent episodes.
2 spleen is significantly associated with hypersplenism.
3 bone marrow puncture smear is higher than the blood smear positive rate, can be identified.
(9) Rheumatoid arthritis:
More than 1 is 15 years old, more women, mainly joint disease, manifested as centripetal or peripheral joint pain, swelling, effusion, late joint stiffness, deformity and functional limitations.
2 The disease may have splenomegaly in the advanced stage, but it is mostly in the active period of the disease.
5. Upper gastrointestinal bleeding: When patients are treated with acute upper gastrointestinal bleeding, they should be differentiated from diseases such as peptic ulcer, gastric cancer and esophageal cancer.
6. Ascites: Typical cirrhosis ascites is leakage. A small number of patients may have atypical manifestations due to the cause of liver disease or complications. Very few are exudates, more are exudate and leakage. Occasionally bloody between the liquids. Ascites of cirrhosis must be differentiated from ascites caused by diseases such as cardiogenic, renal, dystrophic, and cancerous. Ascites itself has no differential diagnostic value and needs to be identified in combination with medical history, physical signs and other data.
