Overview
Definition
The term "asplenia" literally means absent spleen. However, in the condition asplenia, the spleen is not always absent. Sometimes the spleen is present, but not fully developed (hypoplastic). In asplenia, the spleen is typically not the only organ affected. Individuals with this condition often have problems with other organs and organ systems. A related condition is polysplenia.
The term "polysplenia" literally means multiple spleens. Both of these conditions affect the placement and development of the organs inside the body. There is controversy over whether asplenia and the other syndromes, like polysplenia, that affect the position of the internal organs are actually different aspects of the same condition, referred to as Heterotaxy syndrome, or separate and distinct syndromes. As of 2001, this issue has not been resolved.Asplenia is just one of the names used to refer to this condition. Other names include Ivemark syndrome, right isomerism sequence, bilateral right-sideness sequence, splenic agenesis syndrome, and asplenia with cardiovascular anomalies.
Signs and Symptoms
Almost all individuals with asplenia have an abnormal or absent spleen. However, there are other organs and organ systems that can be affected.
Abdominal Organs
SPLEEN As the name of the condition implies, the spleen is always affected in asplenia. The spleen in individuals with asplenia is either absent or does not develop completely (hypoplastic spleen). Since the spleen is involved in the body's immune system, these infants can have an abnormal immune system, which increases their risk for developing aninfection.
DIGESTIVE TRACT DISORDERS: There are several abnormalities that can occur with the digestive tract in individuals with asplenia. The most common digestive tract disorder associated with asplenia is malrotation of the intestine. Sometimes a digestive tract problem will present with symptoms of an obstruction in the digestive system, requiring emergency surgery.
STOMACH: Most individuals with asplenia have their stomach located on the right side or in the center of the body instead of the left. In addition, individuals with asplenia can have a "twisted" stomach that could result in an obstruction in their digestive system and impair the blood supply to the stomach (gastric volvulus).
LIVER :Normally, the liver is located on the right side of the body and the shape of the liver is not symmetrical. In asplenia, there can be isomerism of the liver—it can be located in the middle of the body, or located on the left side with the larger half of the liver located in the upper left side of the abdominal area.
GALLBLADDER: The gallbladder may also be located in the middle of the body in individuals with asplenia.
Treatment and Management
Surgery can sometimes be performed on the heart to repair the defect or defects. There are limitations to heart surgery and it cannot always be performed. Additionally, heart surgery is not always successful. Surgery can also be used to correct many of the digestive tract disorders.
Additionally, because the spleen is involved in the body's immune system, it is recommended that all patients with the diagnosis of asplenia be given antibiotics and pneumococcal vaccination.
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