Ivemark syndrome
Other Names: Asplenia with cardiovascular anomalies; Asplenia syndrome; Bilateral right-sidedness sequence; Splenic agenesis syndrome
Ivemark syndrome is a rare congenital condition that affects multiple organ systems of the body. Ivemark syndrome is classified as a heterotaxy disorder or a laterality disorder. These terms refer to the failure of the internal organs of the chest and abdomen to be arranged in the proper location within the body.
Cause[edit | edit source]
The exact cause of Ivemark syndrome is not known.
Inheritance[edit | edit source]
Most cases are sporadic (isolated and seemingly random). Unlike some other heterotaxy disorders, causative gene mutations have not been identified.
Signs and symptoms[edit | edit source]
It is characterized by the absence (asplenia) or underdevelopment (hypoplasia) of the spleen, heart malformations, and the abnormal arrangement of the internal organs of the chest and abdomen.
For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 1%-4% of people have these symptoms
- Aortopulmonary collateral arteries
- Asplenia(Absent spleen)
- Common atrium
- Inguinal hernia
- Situs inversus totalis(All organs on wrong side of body)
- Tetralogy of Fallot
- Total anomalous pulmonary venous return
- Transposition of the great arteries
Diagnosis[edit | edit source]
Treatment[edit | edit source]
There is no cure for Ivemark syndrome. Treatment might include surgical repair of heart malformations when appropriate and prophylactic antibiotic therapy to reduce the incidence of infection due to the absence or poor function of the spleen.
NIH genetic and rare disease info[edit source]
Ivemark syndrome is a rare disease.
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