Chronic congestive splenomegaly in children
Introduction
Introduction to chronic congestive splenomegaly in children Chronic congestive splenomegaly (chronic congestives plenomegaly), also known as portal hypertension (portalhypertension) or Banti syndrome (Bantissyndrome), is an unexplained, congestive chronic progressive disease, more common in older children, chronic congestive splenomegaly The disease is mainly caused by portal hypertension. The clinical features are chronic progressive splenomegaly, progressive anemia, white blood cells and thrombocytopenia and gastrointestinal hemorrhage as the main manifestations. Late ascites, jaundice, liver dysfunction and cirrhosis . It has recently been found that this symptom is often complicated by portal hypertension. However, it is currently believed that splenomegaly and "spleen hyperfunction" are not caused only by hyperemia, and splenomegaly and hypersplenism can continue to exist after portal decompression. basic knowledge The proportion of illness: 0.005% Susceptible people: children Mode of infection: non-infectious Complications: cirrhosis, malnutrition, blood in the stool
Cause
Causes of chronic congestive splenomegaly in children
(1) Causes of the disease
The main cause of portal hypertension in childhood is portal vein and splenic vein embolism, splenic vein embolization may be associated with neonatal umbilical inflammation, neonatal sepsis, umbilical vein intubation complications, portal vein cavernous tumor, congenital splenic vascular malformation, abdominal mass Compression and other related, portal hypertension can be divided into two types of extrahepatic and intrahepatic.
1. Extrahepatic portal hypertension, portal vein congenital malformation, spongiform disease and portal vein obstruction and thrombosis can cause portal hypertension, splenic vein may be obstructed due to congenital valve malformation or due to neonatal umbilical inflammation, sepsis, umbilical vein insertion Splenic phlebitis and thrombosis occur during tube surgery.
2. Intrahepatic portal hypertension is seen in chronic hepatitis, congenital biliary stricture or atresia, schistosomiasis, galactosemia, hepatolenticular degeneration (Wilson's disease), cystic changes of the pancreas.
(two) pathogenesis
The splenic vein belongs to the portal vein system. 70% of the normal person's splenic venous blood is introduced into the vein. If the portal vein is obstructed, the spleen's blood returning disorder may cause congestive splenomegaly. When the cause of congestion is removed, the swollen spleen may be retracted. In advanced cases, due to the proliferation of fibrous tissue and reticuloendothelial tissue, even if the cause is removed, there is no obvious retraction of splenomegaly. It is currently considered that this is not an independent disease, but the portal vein caused by chronic portal vein obstruction or cirrhosis. High blood pressure, high splenomegaly, a group of symptoms of anemia and hypersplenism, there are three aspects of pathophysiology can form portal hypertension: 1 portal vein obstruction; 2 cirrhosis; 3 phlebitis or thrombosis, splenomegaly secondary spleen Hyperfunction, often manifestations of complete blood cell reduction, skin mucosal hemorrhage, hyperplasia of the bone marrow and other spleen hyperfunction, the common features of intrahepatic and extrahepatic portal hypertension are hematemesis (upper gastrointestinal bleeding), splenomegaly and ascites.
Prevention
Prevention of chronic congestive splenomegaly in children
Caused by the cause of this disease, there are liver factors as well as extra-hepatic factors, preventive measures are detailed in the relevant sections.
Complication
Chronic congestive splenomegaly complications in children Complications, cirrhosis, malnutrition, blood in the stool
Cause esophageal varices, rupture and bleeding, symptoms of cirrhosis often occur in the late stage, such as ascites, jaundice, severe malnutrition, lower extremity edema, and chest, abdominal subcutaneous vein dilatation (collateral circulation), hematemesis, blood in the stool, etc., ascites are generally not seen in Extrahepatic portal hypertension.
Symptom
Symptoms of chronic congestive splenomegaly in children Common symptoms Abdominal discomfort Abdominal indigestion Left upper abdominal mass accompanied by... Lower esophageal varices blood in the stool
The disease mostly occurs in older children, the incidence is slow, often caused by accidental splenomegaly caused by parents, children generally good state, no liver disease signs, some children with abdominal discomfort, indigestion, fatigue, pale, sputum And the left upper abdominal mass (splenomegaly) is the main manifestation. Therefore, splenomegaly is the main sign of pediatric portal hypertension. The spleen is moderately enlarged. The hardness depends mainly on the duration of portal hypertension. After the portal vein is high pressure, The collateral circulation is generated to ensure blood flow back into the heart, and the lower collateral circulation forms the lower esophageal and varicose veins. In the lower part, the varicose veins are formed. These varicose veins are often rubbed by food and feces, and are easily broken. Cause hematemesis and blood in the stool, esophageal varices bleeding is a risk factor for portal hypertension, hematemesis can occur in any age of children, but rare in 2 years old, hematemesis can be sudden, and there is a tendency to recurrent, bleeding often Occurrence of esophageal varices due to frequent cough after abdominal discomfort or fever and bronchitis or pneumonia , A large amount of vomiting in 80 ~ 200ml, heavy bleeding spleen may have some degree of retraction, however, the shape recovery may be further splenomegaly 48h after the bleeding stops.
1. Portal hypertension
(1) extrahepatic portal hypertension: the disease has upper gastrointestinal symptoms (hematemesis and melena) earlier; ascites is less common and easy to resolve; spleen is significantly enlarged with hypersplenism, may have neonatal sepsis, umbilical cord History of illness, or history of umbilical vein intubation, without a history of hepatitis.
(2) intrahepatic portal hypertension: common in chronic hepatitis cirrhosis, post-necrosis cirrhosis, advanced schistosomiasis cirrhosis, congenital biliary stricture, etc., hematemesis, blood in the stool, and other gastrointestinal symptoms appear more than the liver type Late, the disease occurs between 2 and 12 years old, gastrointestinal bleeding is often accompanied by malnutrition, more refractory ascites, abnormal liver function with coagulopathy, liver enlargement or shrinkage, hard texture and knot Section; significant splenomegaly is often accompanied by hypersplenism, portal venography is the main method for the diagnosis of this disease, individual cases of difficult diagnosis, need to be diagnosed by laparotomy.
2. Chronic congestive heart failure (chronic congestive heart failure) is more common in school-age children, long-term venous congestion caused by cardiogenic cirrhosis can lead to splenomegaly, but rare.
3. Constrictive pericarditis (85% of cases of chronic constrictive pericarditis) have splenomegaly, mostly mild.
4. Portal thrombosis is very rare, can be divided into acute and chronic two types, both types have splenomegaly, acute type often secondary to splenectomy, portal vein surgery, portal vein infection or trauma, its main Clinical manifestations of acute abdominal pain, abdominal distension, vomiting, hematemesis and blood in the stool, chronic portal vein thrombosis is more common than acute, common in cirrhosis, followed by liver cancer or other organs in the abdominal cavity, eroding the portal vein, children may have ascites, spleen Swelling and hypersplenism, the liver is rarely swollen, and the splenomegaly is obvious. This point can be distinguished from hepatic vein obstruction. Splenic portal vein angiography is the main method for diagnosing this disease. Some patients can be diagnosed by surgical exploration. .
5. Budd-Chiari syndrome is rare in clinical practice. Only a few cases are reported in China. Most of them are caused by thrombosis. The primary is rare, mostly secondary, acute and chronic. The main manifestations of acute type For abdominal pain, mild jaundice, liver, ascites, chronic type in addition to abdominal pain, liver and dyspepsia, there are still splenomegaly, ascites, inferior vena cava angiography to determine the diagnosis, the disease has a poor prognosis.
Examine
Examination of chronic congestive splenomegaly in children
1. Blood examination: showing different degrees of anemia, leukopenia, normal or slight reduction of platelets, poor blood clot dysfunction and beam arm test, typical cases can see obvious whole blood cell reduction, in the early stage of the disease, anemia is positive color, many times After blood loss, it turns into small cells with low color, reticulocytes after blood loss, white blood cells can be temporarily increased, the larger the spleen, the lower the white blood cells, often between (1.5 ~ 4) × 109 / L.
2. Bone marrow examination: There is no abnormal change in the early stage, sometimes hyperplasia, and there are increased nucleated red blood cells and megakaryocytes, and cell maturation disorders. The maturation of granulocytes and megakaryocytes may be restricted, and the late red blood cell maturation is affected.
3. Liver function test: Before liver cirrhosis, the liver function test is more normal.
4. Changes in coagulation factors: In the early stage, only splenomegaly and no liver dysfunction, there was no significant difference in various coagulation factors, and various clotting factors were significantly different from normal controls in advanced cirrhosis.
5. X-ray, ultrasound examination of the spleen: splenomegaly can not be touched under the rib arch, can be determined by X-ray, ultrasound to determine the size, location, nature of the spleen, to determine whether the spleen is a spleen, at the same time, ultrasonic examination to detect the size of the spleen And there is a certain value with or without ascites and ascites.
6. Esophagus gastrointestinal barium meal examination, pyelography: esophageal barium meal examination can be used to observe esophageal varices to understand the presence or absence of portal hypertension, and through gastrointestinal barium meal examination, pyelography, etc. help to identify the nature of abdominal masses.
7. Puncture and biopsy: The nature of the lesion can be directly understood, and the test should be used when the above examination is still undiagnosed.
(1) Liver biopsy: It is helpful for the identification of hepatosplenomegaly caused by fatty liver, glycogen metabolism, hepatoblastoma, cirrhosis and mucopolysaccharidosis.
(2) Spleen puncture examination: the risk of spleen puncture is large, especially the significant splenomegaly, due to fibrous tissue hyperplasia, the texture becomes hard and brittle, and it is easy to rupture and hemorrhage. Therefore, spleen puncture should not be easily adopted, and it is not often used in children. After surgical laparotomy or splenectomy, pathological examination can be performed to provide a basis for diagnosis.
8. Colloidal gold liver blood flow measurement: Help to identify chronic hepatitis, splenomegaly caused by portal cirrhosis.
9. Radionuclide scanning: Spleen scintigraphy The application of 99mTc or 113In colloidal injection after spleen scanning helps to estimate the size and shape of the spleen.
10. Hemolytic index in the spleen: 51Cr red blood cell life measurement, can determine whether the red blood cell destruction site is located in the spleen, providing indications for splenectomy.
11. splenic portal vein angiography: help to understand the presence or absence of deformity of the splenic vein, the obstruction of the splenic vein, to assist in the diagnosis of congestive splenomegaly.
Diagnosis
Diagnosis and diagnosis of chronic congestive splenomegaly in children
diagnosis
Diagnosis should be based on physical examination, blood and bone marrow, to exclude other diseases of splenomegaly and whole blood cell reduction, such as blood diseases, Gaucher disease and other congenital metabolic diseases, malignant tumors and various infections, age of patients, chronic spleen enlargement Progressive anemia, leukopenia is important for the diagnosis of this disease. Esophageal varices is an early manifestation of portal hypertension. Positive results can be obtained in about 40% or more of barium meal examinations, often in the lower third of the esophagus. On the X-ray, it appears as a filling defect shadow or a sputum-like shape. This examination can be used as a judgment index for efficacy before and after surgery.
Percutaneous splenic portal vein angiography can be used to understand the portal vein obstruction, varicose vein and collateral circulation in children with extrahepatic portal hypertension. It is a valuable test method and can also measure spleen pressure and portal pressure. It is helpful for the diagnosis of the cause and the choice of surgical methods. For those who do not cooperate, the operation should be carried out under the anesthesia, and there is generally no adverse reaction.
For the purpose of prognosis and treatment, the cause of the disease should be further sought to distinguish between intrahepatic or extrahepatic.
1. Intrahepatic diagnosis points
1 history of hepatitis;
2 liver enlargement (or shrink);
3 liver function test was positive; prothrombin complex decreased, prothrombin time prolonged, portal vein circulation time prolonged;
4 ascites;
5 liver biopsy has abnormal changes.
2. Extrahepatic diagnosis points
1 no history of hepatitis;
2 the liver is not large;
3 liver function test is normal;
4 There was no abnormal change in liver biopsy.
Differential diagnosis
In the diagnosis process, it must be differentiated from other giant spleen diseases such as chronic myeloid leukemia, kala-azar, Niemann-Pick syndrome, and subacute bacterial endocarditis.
1. Chronic infectious splenomegaly Congestive splenomegaly Non-infectious splenomegaly should first be differentiated from infectious spleen.
(1) chronic viral hepatitis (chronic virus hepatitis): more splenomegaly than acute, mostly mild swelling, hard, no tenderness, and a history of acute viral hepatitis.
(2) chronic schistosomiasis (chronic schistosomiasis).
(3) chronic malaria (chronic malaria): the spleen can be extremely swollen, the quality is hard, the peripheral blood is not easy to see the malaria parasite, the adrenaline priming test is often negative, according to the history of past malaria and epidemiological history, bone marrow examination sees the malaria parasite For diagnosis.
(4) Sarcoidosis: The cause is unknown, rare, can affect the systemic system, 50% to 60% involving the liver and spleen, so there is swollen liver and spleen.
(5) histoplasmosis (histoplasmosis): caused by capsular histoplasma, invading bone marrow, lung, liver, spleen, lymph nodes, deep mycosis, more common in 6 to 24 months of sick children, clinical A variety of manifestations, often liver and spleen lymph node enlargement, tissue cytoplasmic skin test positive, bone marrow smear found macrophage capsular histoplasmosis spores, blood, bone marrow, lymph node pus and sputum, etc. Fungal culture can help diagnose.
(6) Toxoplasmosis (toxoplasmosis): a systemic infectious disease caused by Toxoplasma gondii, which is subacute. The disease is divided into acute congenital toxoplasmosis and acquired toxoplasmosis, congenital Toxoplasma gondii. Sick, born with severe jaundice, skin rash, purpura and hepatosplenomegaly, accompanied by convulsions, chorioretinitis and other nervous system symptoms and signs, acquired infection early asymptomatic, infancy convulsions and developmental backward, liver and spleen Swollen.
(7) Brucellosis: caused by Brucella, is a zoonotic disease, often suffered by eating sick cow's milk, meat or close contact with diseased cattle and sheep. Sexual, wavy and repeated fever, if not treated for several months, there may be chills, sweating, joint neuralgia, lymph nodes and hepatosplenomegaly, 70% to 80% of cases of bone marrow examination can obtain pathogenic bacteria, Brucella test and serum agglutination test can be positive, streptomycin, sulfonamide has a good therapeutic effect on this disease.
2. The etiology of congestive splenomegaly identifies the differential diagnosis of extrahepatic portal hypertension and intrahepatic portal hypertension, chronic congestive heart failure, constrictive pericarditis, portal vein thrombosis, hepatic vein occlusion syndrome Identification is detailed in clinical manifestations.
3. Different from the cause of splenomegaly caused by blood diseases. A variety of blood cases can have splenomegaly, and often accompanied by different degrees of swelling of the liver and lymph nodes.
(1) hemolytic anemia: often family history, splenomegaly, hard texture, jaundice, liver enlargement.
(2) iron-deficiency anemia (iron-deficiency anemia): often mild to moderate hepatosplenomegaly.
(3) idiopathic thrombocytopenic purpura (idiopathic thrombocytopenic purpura): acute spleen is not large; chronic type often has mild splenomegaly.
(4) leukemia (leukemia): leukemia often accompanied by splenomegaly, the obvious enlargement of lymphocytic leukemia, followed by granulocyte type, monocytic leukemia mostly mild splenomegaly.
(5) primary myelofibrosis: a myeloproliferative disease in which diffuse fibrous tissue and bone hyperplasia with extramedullary hematopoiesis occur in the bone marrow. The cause is unknown, and children are very rare. In recent years, studies have shown that this disease is A malignant proliferative disease of bone marrow with fibroblasts and osteoblasts as the main proliferating cells.
(6) malignant lymphoma (malignant lymphoma): often accompanied by irregular periodic fever and different degrees of swelling of the liver and spleen, of which about 50% of Hodgkin's granuloma type has splenomegaly, follicular lymphoma also Often splenomegaly, Hodgkin's disease occasionally with splenomegaly as a prominent sign, the spleen can be extremely swollen without a superficial lymphadenopathy.
(7) malignant histiocytosis: about 90% of cases have splenomegaly, and rapidly increase, but there are also a few patients with liver and spleen lymph nodes are not large.
(8) familial eosinophilia: very rare, clinical manifestations of fever, hepatosplenomegaly, increased serum globulin, peripheral blood eosinophil count is often above 50 × 109 / L There are also increased eosinophils in the bone marrow.
(9) idiopathic eosinophilia syndrome (idiopathic eosinophilia syndrome): autosomal dominant hereditary disease, the age of onset is 20 to 40 years old, pediatric cases have also been reported, early symptoms of myocardial insufficiency The great heart is mostly caused by mitral regurgitation, fever, hepatosplenomegaly, peripheral blood and bone marrow eosinophilia as its main clinical manifestations.
(10) primary macroglobulinemia (primary macroglobulinemia): rare, is a lymphoplasmacytic disease, the cause is unknown, the patient is more elderly, more men than women, may have weight loss, fatigue, anemia, repeated infections, etc. Performance, hepatosplenomegaly, lymphoid tissue hyperplasia, especially plasma cell malignant hyperplasia, a large number of monoclonal IgM in serum, macroglobulinemia can make blood viscous, leading to heart failure, affecting the blood supply of major organs, the prognosis of this disease Poor, more than a few months or a few years of death, the use of plasma separation can reduce blood viscosity, penicillamine can decompose macroglobulin, temporarily relieve symptoms, in recent years advocate the use of chlorambucil (tumoren) and Cyclophosphamide treatment.
(11) polycythemia vera (polycythemia vera): also known as splenomegalic polycythemia (splenomegalic polycythemia), the cause of unexplained myeloproliferative syndrome, the number of peripheral blood red blood cells exceeds normal values, hemoglobin, hematocrit The corresponding increase, that is, the red blood cell count is higher than 6×109/L, the hemoglobin is more than 180g/L, and the hematocrit is more than 52%. The disease is divided into primary and secondary types, and the secondary is often secondary to the artery. Decreased blood oxygen saturation, occupants in high mountains, newborns, pulmonary heart disease, methemoglobinemia, etc., often have hepatosplenomegaly.
(12) von Jakschs syndrome: large liver, especially splenomegaly.
(13) Marble bones: Also known as stony bone or osteopetrosis, it is a hereditary disease that can be divided into heavy (autosomal recessive) and light (autosomal dominant). The clinical features are refractory anemia and the X-ray shows an increase in bone density, the medullary cavity almost disappears, and the excessive secretion of calcitonin in the thyroid may be the cause of the disease. In severe cases, there is paleness, liver and spleen and swollen lymph nodes in one month after birth. In addition, there are special faces, which are characterized by large head, forehead protrusion, widened eye distance, flat nose; decreased vision, increased intracranial pressure, etc., mild symptoms are similar to heavy, but not obvious in childhood, often appear after puberty Signs, peripheral blood is positive cells positive pigment anemia, there are different degrees of whole blood cell reduction, blood samples can be seen immature granulocytes and immature red blood cells, bone marrow puncture is difficult, cortical bone is hard, often dry pumping, bone marrow is aplastic anemia bone marrow, severe Children with anemia need blood transfusion therapy, and antibiotics to prevent infection, adrenal cortical hormone treatment, can improve blood routine and electrolyte metabolism disorders, can be used prednisone 7.5 ~ 10mg Day orally, splenomegaly or hypersplenism splenectomy possible cases, reduce the symptoms of hemolysis and thrombocytopenia.
4. Identification with reticuloendothelial cell disease
(1) Langhans cell histiocytosis.
(2) familial reticuloendotheliosis (familial reticuloendotheliosis): often a positive family history, can be expressed as developmental disorders, eczema, hepatosplenomegaly, can be repeatedly infected, peripheral blood, complete blood cell reduction, serum gamma globulin Increased, advanced bone marrow plasma cells, tissue cells and eosinophilia, X-ray chest showed lung infiltration shadow, no special treatment.
(3) sea-blue histiocyte syndrome: can be divided into primary and secondary (acquired) two types, a large number of cytoplasm in the bone marrow stained blue opaque tissue cells, Patients with primary blue-blue histiocytosis often have hepatosplenomegaly, thrombocytopenia, and hemolytic anemia. This disease may be caused by abnormal accumulation of mucopolysaccharide in the body. Acquired people are often secondary to idiopathic thrombocytopenia. Purpura, chronic granuloma, hyperlipoproteinemia, Niemann-Pick disease, thalassemia, polycythemia vera, etc., secondary cases of bone marrow in addition to sea blue tissue cells, as well as the symptoms and signs of primary disease, Such as mild anemia, purpura, hepatosplenomegaly (mainly splenomegaly), lymph nodes are not swollen, a few may have jaundice, laboratory examination of peripheral blood leukocyte count decreased, platelet count decreased, a large number of sea blue tissue cells are visible in the bone marrow The main basis for the diagnosis of this disease, but still need to further find the cause, except for acquired, can be diagnosed as primary navy cell syndrome, the primary has no satisfactory treatment, The author believes splenectomy may improve symptoms, patients should be secondary to treatment of primary disease, infection, bleeding, liver dysfunction symptomatic treatment.
5. Identification of connective tissue diseases and allergic diseases
(1) Systemic lupus erythematosus: 1.7% to 8.1% of cases may have mild splenomegaly.
(2) Dermatomyositis: proliferation and infiltration of mononuclear macrophages can cause splenomegaly.
(3) polyarteritis nodose (polyarteritis nodose): rare in children, mononuclear macrophage hyperplasia, spleen infarction or arteritis can cause splenomegaly.
(4) Juvenile rheumatoid arthritis: There may be swelling of the liver and spleen and lymph nodes.
(5) Felty syndrome: There may be splenomegaly, anemia and neutropenia.
(6) idiopathic pulmonary hemosiderosis (idiopathic pulmonary hemosiderosis): clinical manifestations of recurrent fever, cough and hemoptysis, and pale, fatigue, palpitations, mild swelling of the liver and spleen.
6. Identification with metabolic diseases
(1) Gauchers disease: also known as cerebroside reticulum endothelial cell disease, caused by glucocerebrosidase deficiency, is an autosomal recessive hereditary disease, glucocerebroside is in mononuclear macrophages A large number of cells are deposited. The disease is divided into infant type, juvenile type and adult type. The three types have hepatosplenomegaly and Gaussian cell infiltration in the bone marrow.
(2) Niemann-Pick disease (Niemann-Pick disease): also known as neuropeptide-containing reticulocyte endothelial cell disease, is a familial lipid-like metabolic disorder, is autosomal recessive, due to neurophospholipidase Defects cause neurolipids to accumulate in the mononuclear macrophage system, and there are a large number of neuropeptide-containing reticulocytes in the liver, spleen, lymph nodes, and bone marrow.
(3) cystineosis (cystinosis): this disease is an autosomal recessive hereditary disease characterized by the deposition of cystine in the liver, spleen, bone marrow, lymph nodes, granulocytes, kidneys, cornea and thyroid gland. The acid is mainly deposited in the cell lysosome, causing the cells to be damaged or even die.
(4) Mucopolysaccharidosis type I (Hurler syndrome): Hepatosplenomegaly can occur in the early stage.
7. It is rare to identify primary spleen malignant tumors with splenic tumors. It is also rare for malignant tumors to metastasize to the spleen. Spleen tumors can cause splenomegaly, hard and unsmooth performance.
8. It is rare to distinguish from splenic cysts. It can be divided into true spleen cysts and pseudo splenic cysts. The true cysts are further divided into epidermis (such as dermoid cysts), endothelium (such as lymphatic cysts) and parasitism. There are 3 kinds of insects (such as echinococcosis). The pseudocysts are divided into hemorrhagic, serum and inflammatory. The children with spleen cysts can be seen in the left upper quadrant and cystic mass during the examination. It is soft and smooth. Fluctuation, no movement, ultrasound examination can be found in cystic masses in the spleen.
