J Pediatr Surg. 2011 Jul;46(7):1455-8.

Cantrell syndrome with complex cardiac malformations: a case report.

Wen L, Jun-lin L, Jia H, Dong Z, Li-guang Z, Shu-hua D, Wei-jin L, Yun-hua G.

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Department of Radiology, XinQiao Hospital, Third Military Medical University, ChongQing 400037, PR China.

Abstract

Cantrell syndrome is a rare condition of congenital defects of 5 developmentally associated structures: the abdominal wall, sternum, diaphragm, pericardium, and heart. Few patients survive, and even fewer survive with good outcomes of quality of life. We present a case with the pentalogy and profound cardiac malformations. Despite repeated life-threatening conditions, the boy survived to have his first operation at 5 months old. The operation reduced the heart within the thoracoabdominal area, repaired the abdominal wall defect, and released multiple stenoses of the heart and great vessels. The patient recovered uneventfully. At 4.5 years old, he is healthy and developed normally.

PMID:: 21763854; [PubMed - indexed for MEDLINE]

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