Pentalogy of Cantrell

Changed by Tristan Skalina, 12 Jul 2019

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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.

omphalocoele
ectopia cordis (abnormal location of heart)
diaphragmatic defect
pericardial defect or sternal cleft
cardiovascular malformations

Pathology

It is thought to result from an abnormal migration of the sternal anlage and myotomes in the early embryonic stage ( ~ 6^th to 7^th 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.

~~-<li><a href="/articles/omphalocoele">omphalocoele</a></li>~~
+<li><a href="/articles/omphalocele-1">omphalocoele</a></li>
~~-<li><a href="/articles/encephalocoele">encephalocoele</a></li>~~
+<li><a href="/articles/encephalocele-1">encephalocoele</a></li>
-</ul><h4>Treatment and prognosis</h4><p>Most cases carry a very poor prognosis</p><h4>History and etymology</h4><p>It was first described by <strong>James R Cantrell</strong> in 1958</p><h4>See also</h4><ul><li><a href="/articles/pentalogy-of-fallot">pentalogy of Fallot</a></li></ul>
+</ul><h4>Treatment and prognosis</h4><p>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 <sup>9</sup>. Surgical management aims to <sup>9</sup>:</p><ul>
+<li>correct cardiac malformations</li>
+<li>ensure adequate space for return of the heart to the mediastinum</li>
+<li>protect the heart from compression and distortion via repair thoracoabdominal wall defects</li>
+</ul><h4>History and etymology</h4><p>It was first described by <strong>James R Cantrell</strong> in 1958.</p><h4>See also</h4><ul><li><a href="/articles/pentalogy-of-fallot">pentalogy of Fallot</a></li></ul>

References changed:

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. <a href="https://doi.org/10.1016/j.jpedsurg.2014.06.003">doi:10.1016/j.jpedsurg.2014.06.003</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/25092102">Pubmed</a> <span class="ref_v4"></span>