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Pentalogy of Cantrell and ectopia cordis,a familial developmental field complex |
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| Authors: | Rick A Martin Christopher Cunniff Lars Erickson Kenneth Lyons Jones |
| Institution: | 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 |