Question:

Which anomalies correspond to a class II pentalogy of Cantrell, according to the classification proposed by Dr Toyama?
1. Intracardiac defect, sternal cleft, pericardial defect, diaphragmatic defect, and epigastric omphalocele.
2. Intracardiac defect, sternal cleft, pericardial defect, and epigastric omphalocele.
3. Sternal cleft, pericardial defect, and epigastric omphalocele.
4. Intracardiac defect, pericardial defect, diaphragmatic defect.
5. Diaphragmatic defect.





Answer:

The correct answer for the question "Which anomalies correspond to a class II pentalogy of Cantrell, according to the classification proposed by Dr Toyama?" is:

4. Intracardiac defect, pericardial defect, diaphragmatic defect.



Explanation
According to Toyama [4], pentalogy of Cantrell can be classified into different groups based on the expression of symptoms. (A) Class I presents with all 5 defects and is a definite diagnosis. The five defects are intracardiac defect, sternal cleft, pericardial defect, diaphragmatic defect, and epigastric omphalocele. (B) Class II presents with 4 of the 5 defects and is a probable diagnosis. (C)  Class III presents with varying combinations of defects and is considered an incomplete expression. (D) In patients with Class II, symptoms include the lack of the sternal defect, such as the patient presented in this case report.  (E) Congenital defects of the diaphragm alone give rise to eventration of the diaphragm, defects of the central tendon, and Morgagni hernias.



From the manuscript:
Pentalogy of Cantrell with Ectopia Cordis: CT Findings
Radiology Case. 2014 Dec; 8(12):29-34


This article belongs to the Pediatric section.




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From the manuscript

Pentalogy of Cantrell with Ectopia Cordis: CT Findings

Free full text article: Pentalogy of Cantrell with Ectopia Cordis:  CT Findings

Abstract
A 14-month-old girl with pentalogy of Cantrell, a very rare congenital syndrome characterized by an epigastric omphalocele and malformations of the heart, sternum, pericardium, and diaphragm, underwent echocardiography and multidetector computed tomography before surgical repair of these deformities was attempted. These tests revealed multiple cardiovascular and noncardiovascular abnormalities. After surgery, the patient`s cardiovascular status was stable. Although studies have shown that echocardiography, multidetector computed tomography, and magnetic resonance imaging may each play a role in the diagnosis and management of this condition, there are few data available to support the use of one imaging modality over another.






References



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