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

The exact cause of Ivemark syndrome is not known.

Inheritance

Most cases are sporadic (isolated and seemingly random). Unlike some other heterotaxy disorders, causative gene mutations have not been identified.

Signs and symptoms

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

Treatment

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

• NIH info on Ivemark syndrome

Ivemark syndrome is a rare disease.

Ivemark syndrome Resources
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