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Ectopia cordis is defined as a congenital malposition of the heart outside of the thoracic cavity. It is a rare condition, and complete ectopia cordis can be a fatal condition. Successful surgical reconstruction of this defect has been reported but is uncommon. The general approach to reconstructing the chest wall involves repositioning the heart and providing adequate coverage of the chest wall defect.
We describe our experience with a patient who had complete thoracic ectopia cordis treated with staged chest wall reconstruction. The first stage involved temporary closure with synthetic material, and the second stage involved definitive reconstruction with autologous bone and cartilage grafts supported with plates. The patient has been active and without complaints since the second stage and is awaiting tracheal decannulation.
There have been a few descriptions of how to approach chest wall reconstruction in patients with ectopia cordis. The 2 stage method described can be considered to repair the chest wall defect in complete thoracic ectopia cordis.
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