Journal of the Bahrain Medical Society

Year 2021, Volume 33, Issue 1, Pages 47-50

Case Report

Ivemark Syndrome: Syndrome of Right Isomerism, a Case Report and Review of Literature

Razan Abdulrahim Alsayed, Jemila James, Emad Shatla, Neale Nicola Kalis, Ahmed Fawzy Elbehery, Abdulaziz Abdulrahman Shehab

Author Affiliation

Department of Pediatrics, King Hamad University Hospital, Bahrain

*Corresponding author: Razan Abdulrahim Alsayed, Department of Pediatrics, King Hamad University Hospital, Bahrain; Email: 

Received date: March 31, 2020; Accepted date: December 14, 2020; Published date: March 31, 2021


Right isomerism (Ivemark syndrome) is a rare disorder associated with multiple congenital malformations. It is the result of embryological anatomical disturbances, characterized by absence of spleen, malformations of the heart and abnormal arrangement of the internal organs of the chest and abdomen. This case report describes a rare occurrence in a newborn, presented at 4 hours of age with bluish discoloration of the extremities and low oxygen saturation and diagnosed with complex cyanotic congenital heart defect with situs ambiguous, which was undetected in the antenatal period.

Keywords: Asplenia; Heterotaxy; Isomerism; Ivemark; Situs ambiguous.