Asplenia is a congenital or acquired pathology characterized by the absence of a spleen. This condition is manifested by increased thrombosis and a decrease in the body’s resistance to infections. In the presence of a congenital combined defect, cardiac symptoms prevail: cyanosis of the skin and lips, shortness of breath, tachycardia, hyper- or hypotension. The diagnosis is established on the basis of ultrasound data, spleen scintigraphy, blood analysis. Asplenia after splenectomy does not require treatment. With a combination of abnormalities of the development of the spleen and other organs, surgical intervention is indicated, depending on the type of defect.
Q89.0 Congenital anomalies of the spleen
Asplenia is a pathological condition resulting from primary agenesis or surgical removal of the spleen. Organ agenesis is observed in less than 0.1% of cases of all diseases of the spleen, affecting mainly boys. Congenital pathology is often accompanied by underdevelopment of other organs (heart, main vessels, intestines, liver, etc.). Postoperative asplenia is more common in young and middle-aged men (30-45 years). For several months after undergoing splenectomy, patients are at risk for the occurrence of severe infectious diseases.
Causes of asplenia
The etiology of the disease is different. Pathology can be congenital and form already in the prenatal period or arise as a result of surgical removal of an organ as a result of various diseases. In modern gastroenterology , there are two groups of causes of asplenia:
- Abnormalities of intrauterine development. Fetal tissue differentiation disorders occur on the 31-36 day of embryogenesis. The cause of congenital asplenia can be negative environmental factors (radiation exposure, poor ecology), infectious diseases suffered by the mother during pregnancy (syphilis, gonorrhea, influenza, etc.), taking certain medications (antibiotics, tranquilizers). With a slight influence of teratogenic factors, isolated underdevelopment of the spleen develops, with prolonged exposure – severe combined disorders in the laying of the organs of the thoracic and abdominal cavity (heart, blood vessels, intestines, etc.).
- Surgical intervention. Organ removal operations are indicated for severe congenital and acquired anemia (hereditary hemolytic jaundice, sickle cell and autoimmune hemolytic anemia), idiopathic thrombocytopenic purpura, lymphogranulomatosis, chronic leukemia. Splenectomy is performed for traumatic rupture of the organ, large cysts and abscesses, neoplasms, spleen infarction.
The spleen performs important immunological, hematopoietic, metabolic and filtration functions in the body. Despite the fact that in the absence of an organ, the liver and lymph nodes take over part of its functions, it takes 2-3 months to rebuild the body. With asplenia, the inhibitory effect of the spleen on hematopoiesis stops, resulting in an increase in the number of all blood cells (erythrocytes, leukocytes, platelets). Against the background of alenia, there is an increase in the number of reticulocytes, the appearance of Jolly bodies, a change in the shape of blood cells. Increased platelet count increases the risk of thromboembolic complications. Of great importance in aging is the reduction of the body’s defenses. The weakening of immunity is associated with inhibition of immunogenic and phagocytic functions, which leads to increased susceptibility of the patient to infectious diseases.
Symptoms of asplenia
Isolated congenital absence of an organ is a rare disease and leads to the development of serious infections in infants. Asplenia in newborns is often combined with congenital heart defects (Fallot’s notebook, ventricular septal defect, etc.) and is manifested by severe heart failure, shortness of breath, cyanosis of the lips, nasolabial triangle and skin, increased or decreased blood pressure, respiratory failure. In children with alienia, there is a persistent suppression of immunity, a tendency to septic complications, anemia and thrombosis.
After splenectomy for several weeks / months, there is a high probability of developing infectious diseases (meningitis, pneumonia, etc.) with a lightning-fast unfavorable course. In patients after removal of the spleen, susceptibility to pneumococcal, staphylococcal, meningococcal, campylobacter infection increases. As a result of an increase in blood clotting, thrombosis of the vessels of the brain and lower extremities may occur. The increased number of white blood cells persists for several months after surgery.
Congenital asplenia is a severe, often incompatible with life pathology. In the absence of surgical treatment, mortality from asplenia, combined with combined heart defects, reaches 95% in the first year of life. With an isolated anomaly of the spleen, the probability of developing purulent-infectious complications (septicopiemia, meningitis) increases. After splenectomy, ischemic stroke, pulmonary embolism, thrombosis of the vessels of the lower extremities, myocardial infarction may occur due to increased thrombosis. Asplenia worsens the course of all infectious diseases, leading to the development of sepsis, encephalitis, total pneumonia, which can cause death.
Diagnosis of postoperative asplenia does not cause difficulties. The disease is detected during examination, collection of anamnesis and ultrasound of the abdominal organs. Diagnosis of congenital alienia is of great practical importance. If an organ agenesis is suspected, a specialist (neonatologist, pediatrician, pediatric gastroenterologist) prescribes the following examinations:
- Ultrasound examination. Ultrasound of the spleen allows you to identify asplenia. When examining other organs of the abdominal cavity, an abnormal location of the liver and intestines is often determined.
- Scintigraphy of the spleen. With the help of radioisotope scanning, it is possible to detect Alienia. This method allows for differential diagnosis with other abnormalities of intrauterine development.
- Blood test. With asplenia, patients have persistent leukocytosis, acceleration of ESR, thrombocytosis. In the red blood, an increased number of reticulocytes, erythrocytes, Howell—Jolly corpuscles, intracellular Heinz inclusions are detected.
Differential diagnosis of asplenia is carried out with a complete transposition of organs, which is easily detected using instrumental research methods. The disease should be distinguished from microsplenia, and in old age – from senile organ atrophy.
All patients after splenectomy are shown preventive measures and a protective regime. Seasonal vaccination against influenza, pneumococcal and meningococcal infections is recommended. With high thrombocytosis, the appointment of antiplatelet agents is recommended for the prevention of complications.
Newborns with isolated asplenia are monitored by a district pediatrician and a pediatric cardiologist. In case of inflammation combined with cardiac defects, surgical treatment is indicated. The choice of surgery depends on the severity of the concomitant pathology of the heart, the age of the patient and his somatic condition. In some cases, at the first stage, a shunt is applied between the aorta and the pulmonary artery to reduce heart failure. When the condition is stabilized, surgical treatment of the main congenital heart defect is started. In addition to surgical manipulations, symptomatic therapy is performed during asplenia: inhalation with moistened oxygen, intravenous administration of hemodynamic stabilizing drugs.
Prognosis and prevention
The prognosis for asplenia depends on the presence of abnormalities in the development of other organs, symptoms and complications. With severe combined defects, the prognosis is unfavorable. Even after the operation, there is a possibility of death from septic complications and acute heart failure. In patients after splenectomy, the prognosis is satisfactory if the protective regime is observed.
Prevention of congenital asplenia is pregnancy planning, rehabilitation of chronic foci of inflammation in the mother. During the period of carrying a child, a woman is recommended to lead a healthy lifestyle, adhere to the basics of rational nutrition. After splenectomy, patients need to exclude heavy physical exertion, harmful food and alcohol, avoid contact with infectious patients, undergo timely medical examination and vaccination. Patients with asplenia are not recommended to visit tropical countries because of the risk of infection with serious diseases (malaria, trypanosomiasis, etc.).