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Chirurgia 2017 February;30(1):28-32

DOI: 10.23736/S0394-9508.16.04592-7

Copyright © 2016 EDIZIONI MINERVA MEDICA

language: English

Youngest reported infant with incomplete Cantrell Syndrome: successful correction using combined repair

Shuna XIAO 1, Xuexiang LV 2, Rong LU 1, Jianli DENG 2, Lijun WANG 1, Changdong ZHANG 1, Xiaoxian DENG 1, Xin CHEN 3, Xiaoke SHANG 1

1 Cardiac Dynamics Laboratory, Wuhan Asia Heart Hospital, Wuhan, China; 2 Department of Cardiology, Wuhan Iron and Steel Group, Wuhan, China; 3 Department of Epigenetics and Molecular Carcinogenesis, M.D. Anderson Cancer Center, University of Texas, Houston, TX, USA


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Pentalogy of Cantrell, also known as ectopia cordis, is a rare syndrome that involves intracardiac abnormalities and major defects in the diaphragm, abdominal wall, pericardium, and sternum. The survival rate of patients with complete pentalogy of Cantrell is as low as 20%. Survival is determined by the severity of cardiac anomalies, the type of surgery performed, and the timing of surgery. We present herein the youngest known infant with incomplete pentalogy of Cantrell successfully corrected using combined repair. The 23-day infant was treated by a team of pediatricians, cardiac surgeons, and plastic surgeons and achieved a satisfactory result without complications. He continued to thrive over a year of follow-up. It is crucial to choose the appropriate surgical treatment, proper timing of repair, and to encourage multidisciplinary treatment of patients with pentalogy of Cantrell.


KEY WORDS: Pentalogy of Cantrell - Ectopia cordis - Congenital heart defects

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