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Review
. 2019 Apr;28(2):106-110.
doi: 10.1053/j.sempedsurg.2019.04.006. Epub 2019 Apr 9.

Pentalogy of Cantrell

Adele P Williams  1 , Raoud Marayati  1 , Elizabeth A Beierle  2
Affiliations
Review

Pentalogy of Cantrell

Adele P Williams et al. Semin Pediatr Surg. 2019 Apr.

Abstract

Pentalogy of Cantrell is a constellation of five congenital defects that pose a unique challenge for surgeons. Defects of the heart, pericardium, diaphragm, sternum, and anterior abdominal wall are pathognomonic. Although the incidence is low, it is critical to identify it in a timely fashion in order to adequately address all aspects. Early diagnosis, supportive care, and strategic surgical planning with a multidisciplinary team are all key components in managing patients with Pentalogy of Cantrell. In this text we sought to explore the evolution of both the understanding and treatment for this complex entity and provide current recommendations to today's pediatric caregivers.

Keywords: Congenital diaphragmatic hernia; Ectopia cordis; Omphalocoele; Pentalogy of Cantrell; Sternal defect; Ventral wall defect.

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Figures

Fig. 1.
Fig. 1.
(A) Sagittal CT scan of a patient with Pentalogy of Cantrell demonstrates a large omphalocoele (open arrow) accompanied by the loss of diaphragm and diaphragmatic pericardium anteriorly (closed arrow). These are three key features of the disease. (B) An axial view showing the loss of diaphragm and pericardium between the heart and liver.
Fig. 2.
Fig. 2.
Axial CT angiography of the thorax showing (A) the sternum is present at the level of the aortic arch but (B) is absent inferiorly allowing cardiac protrusion and mild ectopia cordis.
See this image and copyright information in PMC

References

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