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Pediatric and Developmental Pathology
Published in: Pediatric and Developmental Pathology 6/2004

01-12-2004 | Case report

Pentalogy of Cantrell: A Case Report with Pathologic Findings

Authors: María S. Correa-Rivas, Isabel Matos-Llovet, Lourdes García-Fragoso

Published in: Pediatric and Developmental Pathology | Issue 6/2004

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Abstract

We present the case of a 28-h-old female infant born at 37 weeks of gestation with a rare congenital malformation consisting of a pentad of findings: ectopia cordis, a midline supraumbilical wall defect, a defect of the lower sternum, absent pericardium, and an anterior diaphragmatic defect. This constellation of defects is known as the pentalogy of Cantrell. Additional autopsy findings included a bilateral cleft lip and palate, bilateral pulmonary hypoplasia, an atrial septal defect, and a patent ductus arteriosus. We present this case because of its rarity and discuss the pathologic findings.
Literature
1.
Cantrell, JR, Haller, JA, Ravitch, MM 1958A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heartSurg Gynecol Obstet.602614PubMed
2.
Carmi, R, Boughman, JA 1992Pentalogy of Cantrell and associated midline anomalies: a possible ventral midline developmental fieldAm J Med Genet429095PubMed
3.
Morales, JM, Patel, SG, Duff, JA, Villareal, RL, Simpson, JW 2000Ectopia cordis and other midline defectsAnn Thorac Surg70111114PubMed
4.
Martin, RA, Cunniff, C, Erickson, L, Jones, KL 1992Pentalogy of Cantrell and ectopia cordis a familial ventral field complexAm J Med Genet42839841PubMed
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Sepulveda, W, Weiner, E, Bower, S,  et al. 1994Ectopia cordis in a triploid fetus: first trimester diagnosis using transvaginal color Doppler ultrasonography and chorionic villous samplingJ Clin Ultrasonogr22573575
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Bick, D, Markowitz, RI, Horwich, A 1988Trisomy 18 associated with ectopia cordis and occipital meningoceleAm J Med Genet30805810
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Fox, JE, Gloster, ES, Mirchandani, R 1988Trisomy 18 with Cantrell pentalogy in a stillborn infantAm J Med Genet31391394PubMed
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Ghidini, A, Sirtori, M, Romero, R, Hobbins, JC 1988Prenatal diagnosis of pentalogy of CantrellJ Ultrasound Med7567572PubMed
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Metadata
Title
Pentalogy of Cantrell: A Case Report with Pathologic Findings
Authors
María S. Correa-Rivas
Isabel Matos-Llovet
Lourdes García-Fragoso
Publication date
01-12-2004
Publisher
Springer-Verlag
Published in
Pediatric and Developmental Pathology / Issue 6/2004
Print ISSN: 1093-5266
Electronic ISSN: 1615-5742
DOI
https://doi.org/10.1007/s10024-004-9104-5

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