Asplenia syndrome, also known as right isomerism or Ivemark syndrome, is a type of heterotaxy syndrome.
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Epidemiology
There is an increased male predilection. Asplenia syndrome is usually diagnosed in neonates 4.
Associations
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severe/complex congenital heart disease (50%), especially cyanotic congenital cardiac anomalies
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gastrointestinal
intestinal malrotation (up to 100% in small series) 3
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genitourinary
fused / horseshoe adrenal gland or absent left adrenal gland
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vascular
absent coronary sinus
juxtaposition of the IVC in front (usually) of the abdominal aorta (piggyback configuration)
Clinical presentation
In contrast to polysplenia syndrome, most patients die before 1-year-old because of severe/complex congenital heart disease. Most patients are immunocompromised due to absent spleen.
Pathology
Howell-Jolly and Heinz bodies are seen on H&E blood smear 6.
Radiographic features
General
Main characteristic radiographic features include:
bilateral eparterial bronchi
bilateral trilobed lungs
bilateral right atria
transverse liver
Treatment and prognosis
It carries poor prognosis than left isomerism (polysplenia syndrome) as it has more association with congenital heart diseases. 1-year mortality is approximately 85% with right isomerism and more than 50% with polysplenia syndrome 7.
See also
asplenia: not to be confused with asplenia syndrome