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Pentalogy of Cantrell and ectopia cordis,a familial developmental field complex
Authors:Rick A. Martin  Christopher Cunniff  Lars Erickson  Kenneth Lyons Jones
Affiliation:Division of Dysmorphology, Department of Pediatrics, University of California, San Diego, California
Abstract:The association of sternal fusion defects with various cardiac, diaphragmatic, and anterior body wall defects represents a developmental field complex that includes the Pentalogy of Cantrell and ectopia cordis. No familial cases have been reported previously. We present 3 consecutively born brothers with extensive diaphragmatic defects, 2 who had the Pentalogy of Cantrell. One of the 2 also had ectopia cordis.
Keywords:Pentalogy of Cantrell  ectopia cordis  anterior diaphragmatic hernia  midline  developmental field  X-linked
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