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05-2-2009 - Keep him in your prayers. please!

My mood while writing this blog: sad

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I have been blessed by God with a beautiful nephew name Tuyeng. Sad thing about it, is that he is born with pentalogy of cantrell. The complete syndrome is characterized by two major defects: ectopia cordis and an abdominal wall defect (most commonly an omphalocele, but gastroschisis can also be present). The other three defects of the pentalogy are disruption of all the interposing structures: the distal sternum, anterior diaphragm and diaphragmatic pericardium. Incomplete expressions have also been reported. I'm trying to do research on this diagnosis before his heart surgery on monday the 9th of febuary. If anyone can help me out that would be greatly appreciated. Any information would be helpful at this stage. There's only been 90 cases ever filed, and I wanted numbers of how many survived this. Please remember to help keep him in your prayers! Thank you!

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2 Comments on Keep him in your prayers. please!


lisamarie8503 - Friday, 6 Feb
I am so sorry! :( Your family will be in my prayers...

I found this online... Prognosis
In a review of the literature in 1972, Toyama reported a survival rate of 20%. This observation included cases with mild defects and incomplete expressions of the syndrome, and all cases were diagnosed after delivery1. In Ghidini"s 1988 report of 17 prenatally diagnosed cases, 6 patients opted for termination, 4 infants were stillborn, 4 infants died in the first four days after delivery, and the remaining 3 died at one, four, and four months. This gave a survival rate of 0%8. These cases were prenatally diagnosed, so the extent of anomalies could have been more severe than those cases detected at birth. Three of the five patients Cantrell reported in 1958 survived, but none of the five had true ectopia cordis3. Overall the prognosis appears dismal, but may be related to the extent of the ventral wall, sternal, and cardiac defects.

So sorry :(


Blessing8168 - Thursday, 5 Feb
I will do some research and see what I can find. Praying for him!!