Question:

What is the reason why it is advisable to wait until 2 years of age to repair the defects of Pentalogy of Cantrell?
1. This delay allows for some growth of the thoracic cavity.
2. This delay allows for the maturity of the respiratory system.
3. This delay allows the heart to reach its maximum size.
4. The longer the duration of ventilation (>100 days) improves outcome.
5. Due to the to rarity of pentalogy of Cantrell, it has not been possible to determine the timing for surgery that improves outcome.





Answer:

The correct answer for the question "What is the reason why it is advisable to wait until 2 years of age to repair the defects of Pentalogy of Cantrell?" is:

1. This delay allows for some growth of the thoracic cavity.



Explanation
Evidence suggests that a judicious application of palliation or open repair using can lead to reduced overall mortality in patients with Pentalogy of Cantrell. [If delay of operative repair is favorable, it is advisable to wait until 2 years of age. This delay allows for some growth of the thoracic cavity and avoidance of high intrathoracic pressures during repositioning of the heart]. (D) Several reports indicate that patients requiring more than 100 days of ventilation present more complications, such as sepsis, multiorgan failure, and cardiac arrest.



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.






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