Aspleniya - the congenital or acquired pathology which is characterized by lack of a spleen. This state is shown by the raised tromboobrazovaniye and decrease in resistance of an organism to infections. With the congenital combined defect kardialny symptoms prevail: cyanosis of skin and lips, short wind, tachycardia, hyper - or hypotension. The diagnosis is established on the basis of data of ultrasonography, a stsintigrafiya of a spleen, blood test. Aspleniya after a splenektomiya does not demand treatment. At a combination of anomaly of development of a spleen and other bodies surgical intervention depending on a type of defect is shown.
Aspleniya (aliyeniye) – the pathological state resulting from primary agenesia or surgical removal of a spleen. The agenesia of body is observed less than in 0,1% of cases of all diseases of a spleen, affecting mainly boys. Congenital pathology often is followed by an underdevelopment of other bodies (heart, the main vessels, intestines, liver, etc.). The postoperative aspleniya meets more often at men of young and middle age (30-45 years). Within several months after the postponed splenektomiya patients are in risk group on developing of serious infectious diseases.
The etiology of a disease is various. Pathology can be congenital and be formed already in the prenatal period or arise owing to surgical removal of body as a result of various diseases. In gastroenterology allocate two groups of the reasons of an aspleniya:
- Anomalies of pre-natal development. Violations of a differentiation of fabrics of a fruit arise on 31-36 day of an embryogenesis. As the reason of a congenital aspleniya negative factors of the external environment (radiation exposure, bad ecology) transferred mother during pregnancy infectious diseases (syphilis, gonorrhea, flu, etc.), reception of some medicines (antibiotics, tranquilizers) can serve. At insignificant influence of teratogenny factors the isolated spleen underdevelopment develops, at long influence - the heavy combined violations in laying of bodies of a chest and abdominal cavity (heart, vessels, intestines, etc.).
- Surgical intervention. Operations on removal of body are shown at the heavy congenital and acquired anemias (hereditary hemolytic jaundice, serpovidnokletochny and autoimmune hemolytic anemias), idiopathic trombotsitopenichesky purple, a limfogranulematoza, a chronic leukosis. To Splenektomy carry out at a traumatic rupture of body, big cysts and abscesses, new growths, a spleen heart attack.
The spleen carries out important immunological, haematogenic, exchange and filtrational functions in an organism. In spite of the fact that in the absence of body a part of its functions undertakes a liver and lymph nodes, on reorganization of an organism 2-3 months leave. At an aspleniye the braking influence of a spleen on a hematopoiesis therefore there is a growth of quantity of all blood cells stops (erythrocytes, leukocytes, platelets). Against the background of an aliyeniya increase in number of retikulotsit, emergence of little bodies of Joly, change of a form of blood cages is observed. The increased quantity of platelets increases risk of tromboembolichesky complications. At an aliyeniye decrease in protective forces of an organism is of great importance. Easing of immunity is connected with braking of immunogene and fagotsitarny functions that leads to increase in a susceptibility of the patient to infectious diseases.
The isolated congenital absence of body is a rare disease and leads to development of serious infections in babies. Aspleniya at newborns is quite often combined with congenital heart diseases (Fallo's tetrad, defect of an interventricular partition, etc.) and HELL, respiratory insufficiency is shown by the expressed heart failure, short wind, cyanosis of lips, a nasolabial triangle and integuments, increase or decrease. Children from aliyeniye have a permanent oppression of immunity, tendency to septic a complication, to anemias and thromboses.
After a splenektomiya for several weeks/months the probability of development of infectious diseases (meningitis, pneumonia, etc.) with a lightning adverse current is high. At patients after removal of a spleen the susceptibility to a pnevmokokkovy, staphylococcal, meningococcal, kampilobakterny infection raises. Thrombosis of vessels of a brain, the lower extremities can result from increase in coagulability of blood. The increased quantity of leukocytes remains several months after operation.
The congenital aliyeniya is heavy, pathology often incompatible with life. In the absence of surgical treatment mortality from the aspleniya which is combined with the combined heart diseases on the first year of life reaches 95%. At the isolated anomaly of a spleen the probability of development increases it is purulent - infectious complications (a septikopiyemiya, meningitis). After a splenektomiya in connection with the raised tromboobrazovaniye there can be an ischemic stroke, a thrombembolia of a pulmonary artery, thromboses of vessels of the lower extremities, a myocardial infarction. Aspleniya worsens the course of all infectious diseases, leading to development of sepsis, encephalitis, total pneumonia which can become the reason of a lethal outcome.
Diagnostics of a postoperative aspleniya does not cause difficulties. The disease comes to light at survey, collecting the anamnesis and ultrasonography of abdominal organs. Diagnostics of a congenital aliyeniya has important practical value. At suspicion of an agenesia of body the expert (, the pediatrician, the children's gastroenterologist) appoints the following inspections:
- Ultrasonography of a spleen. Allows to reveal an aspleniya. At a research of other abdominal organs the abnormal arrangement of a liver, intestines often is defined.
- To Stsintigrafy of a spleen. By means of radio isotope scanning the aliyeniye manages to find. This method allows to carry out differential diagnostics with other anomalies of pre-natal development.
- General blood test. At an aspleniye at patients it is observed resistant , acceleration of SOE, . In red blood the increased quantity of retikulotsit, erythrocytes, Howell's little bodies — Joly, intracellular inclusions of Heinz is found.
Differential diagnostics of an aspleniya is carried out with a full transposition of bodies that easily comes to light by means of tool methods of a research. The disease should be distinguished from a mikrospleniya, and at advanced age – from a senile atrophy of body.
Treatment of an aspleniya
Preventive actions and the guarding mode are shown to all patients after a splenektomiya. Performing seasonal vaccination against flu, a pnevmokokkovy and meningococcal infection is recommended. At a high trombotsitoz for the purpose of prevention of complications purpose of antiagregant is recommended.
For newborns with the isolated aspleniya observation of the local pediatrician and children's cardiologist is carried out. At the aliyeniya which is combined with kardialny defects surgical treatment is shown. The choice of operation depends on weight of the accompanying pathology of heart, age of the patient and his somatic state. In certain cases at the first stage carry out imposing of the shunt between an aorta and a pulmonary artery for reduction of heart failure. At stabilization of a state start expeditious treatment of the main congenital heart disease. Besides surgical manipulations at an aspleniye carry out symptomatic therapy: inhalations by the moistened oxygen, intravenous administration of the medicines stabilizing haemo dynamics.
Forecast and prevention
The forecast of pathology depends on existence of anomalies of development of other bodies, symptomatology and complications. At the heavy combined defects the forecast adverse. Even after the performed operation there is a probability of a lethal outcome from septic complications and an acute heart failure. At patients is after a splenektomiya at observance of the guarding mode the forecast satisfactory.
Prevention of a congenital aspleniya is pregnancy planning, sanitation of the chronic centers of an inflammation at mother. During incubation of the child it is recommended to woman to lead a healthy lifestyle, to adhere to bases of a balanced diet. After a splenektomiya patients need to exclude heavy physical activities, junk food and alcohol, to avoid contacts with infectious patients, in due time to undergo medical examination and vaccination. With an aspleniya it is not recommended to patient to visit the tropical countries because of danger of infection with a serious illness (malaria, tripanosomozy, etc.).