Citation, DOI, disclosures and article data
Citation:
Weerakkody Y, Yap J, Bell D, et al. Pentalogy of Cantrell. Reference article, Radiopaedia.org (Accessed on 21 Jan 2025) https://doi.org/10.53347/rID-7742
The pentalogy of Cantrell is an extremely rare phenomenon with an incidence estimated at around 6 per million live births 3.
It encompasses the following 5 main features 1.
- omphalocele
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ectopia cordis (abnormal location of heart)
- diaphragmatic defect
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pericardial defect or sternal cleft
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cardiovascular malformations
It is thought to result from an abnormal migration of the sternal anlage and myotomes in the early embryonic stage ( ~ 6th to 7th week of gestation).
Associations
Treatment and prognosis
Most cases carry a very poor prognosis. The diversity and complexity of pentalogy of Cantrell results in a variable clinical course requiring unique surgical approaches 9. Surgical management aims to 9:
- correct cardiac malformations
- ensure adequate space for return of the heart to the mediastinum
- protect the heart from compression and distortion via repair thoracoabdominal wall defects
History and etymology
It was first described by James R Cantrell in 1958.
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1. Emanuel PG, Garcia GI, Angtuaco TL. Prenatal detection of anterior abdominal wall defects with US. Radiographics. 1995;15 (3): 517-30. Radiographics (abstract) - Pubmed citation
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2. Daltro P, Fricke BL, Kline-Fath BM et-al. Prenatal MRI of congenital abdominal and chest wall defects. AJR Am J Roentgenol. 2005;184 (3): 1010-6. doi:10.2214/ajr.184.3.01841010 - Pubmed citation
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3. Van Hoorn JH, Moonen RM, Huysentruyt CJ et-al. Pentalogy of Cantrell: two patients and a review to determine prognostic factors for optimal approach. Eur. J. Pediatr. 2007;167 (1): 29-35. Eur. J. Pediatr. (full text) - doi:10.1007/s00431-007-0578-9 - Free text at pubmed - Pubmed citation
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4. Di Bernardo S, Sekarski N, Meijboom E. Left ventricular diverticulum in a neonate with Cantrell syndrome. Heart. 2004;90 (11): 1320. Heart (full text) - doi:10.1136/hrt.2004.035451 - Free text at pubmed - Pubmed citation
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5. Peixoto-filho FM, Do cima LC, Nakamura-pereira M. Prenatal diagnosis of Pentalogy of Cantrell in the first trimester: is 3-dimensional sonography needed? J Clin Ultrasound. 2009;37 (2): 112-4. doi:10.1002/jcu.20498 - Pubmed citation
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6. Mcmahon CJ, Taylor MD, Cassady CI et-al. Diagnosis of pentalogy of cantrell in the fetus using magnetic resonance imaging and ultrasound. Pediatr Cardiol. 28 (3): 172-5. doi:10.1007/s00246-006-0032-1 - Pubmed citation
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7. Polat I, Gül A, Aslan H et-al. Prenatal diagnosis of pentalogy of Cantrell in three cases, two with craniorachischisis. J Clin Ultrasound. 33 (6): 308-11. doi:10.1002/jcu.20134 - Pubmed citation
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8. Entezami M, Albig M, Knoll U et-al. Ultrasound Diagnosis of Fetal Anomalies. Thieme. (2003) ISBN:1588902129. Read it at Google Books - Find it at Amazon
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9. Zhang X, Xing Q, Sun J, Hou X, Kuang M, Zhang G. Surgical treatment and outcomes of pentalogy of Cantrell in eight patients. (2014) Journal of pediatric surgery. 49 (8): 1335-40. doi:10.1016/j.jpedsurg.2014.06.003 - Pubmed
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