What is asplenia syndrome?
Asplenia syndrome is a syndrome of right isomerism or attempted bilateral right sidedness. It includes congenital asplenia in association with complex congenital cyanotic heart disease and situs anomalies of the other thoracoabdominal organs.
What is heterotaxy syndrome?
Heterotaxy syndrome is a rare birth defect that involves the heart and other organs. The beginning of the word (hetero-) means “different” and the end (–taxy) means “arrangement.” There are different forms of heterotaxy syndrome. All usually involve heart defects of varying types and severity.
What is left right isomerism?
Abnormal cardiac development typically leads to atrial appendage isomerism, resulting in either bilateral paired right atria (right atrial isomerism) or paired left atria (left atrial isomerism). This topic will review the anatomical variation, clinical manifestations, and diagnosis of heterotaxy (isomerism).
What is the deficiency of the congenital absence of a spleen?
Isolated congenital asplenia is a condition in which affected individuals are missing their spleen (asplenia) but have no other developmental abnormalities. While most individuals with this condition have no spleen at all, some people have a very small, nonfunctional spleen (hyposplenism).
How is asplenia diagnosed?
The identification of Howell-Jolly bodies on peripheral blood film is an important clue to the diagnosis of asplenia or hyposplenia. Management of patients with these conditions includes a combination of immunization, antibiotic prophylaxis and patient education.
How do you test for functional asplenia?
Functional hyposplenism is diagnosed by the presence of Howell-Jolly bodies and pitted erythrocytes in the peripheral blood smear, and by nuclear imaging modalities such as spleen scintigraphy with the use of Technetium-99m and/or spleen scintigraphy with the use of heat-damaged Technetium-99m labeled erythrocytes.
What is Situs Ambiguus?
Heterotaxy (or situs ambiguus) is defined as the abnormal arrangement of thoracic or abdominal organs, or both, across the left-right axis (Video 90.1.
What is polysplenia?
Polysplenia is a presence of two or more spleens in a patient and polysplenia syndrome refers to its association with various organ abnormalities in abdomen and chest.
What is heterotaxy with asplenia?
Heterotaxy syndrome (HS) is a rare multiorgan disorder characterized by congenital heart defects (CHDs) and abnormal arrangement of the internal organs of chest and abdomen. Asplenia, polysplenia, hyposplenia, or ectopic spleens are frequently observed.
Is asplenia and immunodeficiency?
Asplenia is a form of immunodeficiency, increasing the risk of sepsis from polysaccharide encapsulated bacteria, and can result in overwhelming post-splenectomy infection, often fatal within a few hours. In particular, patients are at risk from Streptococcus pneumoniae, Haemophilus influenzae, and meningococcus.
What is the treatment for asplenia?
Antibiotic prophylaxis should be initiated immediately upon the diagnosis of asplenia because these patients are at significant risk of pneumococcal infections. For children younger than 2 years, oral penicillin V may be given twice a day. Amoxicillin has also been recommended as an appropriate prophylactic antibiotic.
What infections are Asplenic patients at risk for?
Asplenia is a form of immunodeficiency, increasing the risk of sepsis from polysaccharide encapsulated bacteria, and can result in overwhelming post splenectomy infection (OPSI), often fatal within a few hours. In particular, patients are at risk from Streptococcus pneumoniae, Haemophilus influenzae, and meningococcus.