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2002-09-26-12 Answer to the case of the week #79 © Bircher

Answer to Case of the week # 79

September 13-26, 2002

Ana Maria BIrcher, MD, Philippe Jeanty, MD, PhD, Fernando Heredia, MD.

Nashville, TN

This fetus was scanned for "heart-tones difficult to hear" at 11 weeks 2 days.

These following images show a ventral wall defect. Note also a prominent but normal nuchal translucency. 

Color doppler exmination show the heart outside the chest.

Surface rendering 3D reconstructions show a protruding mass in the fetuses anterior wall.


The final diagnosis of course was Pentalogy of Cantrell .

If we compare these images with the ones provided by Dr. Machado, we can see how this mass is more cranially positiioned. Also note that the implantation area of the mass is much wider compared to the omphalocele.

 Now, lets compare all the above with Dr. Sepulveda"s Limb-body-wall complex:


Although surface rendering 3D looks so nice, it lacks something: If we had the fetus in our hands we would recognize the organs by their colors. In a pentalogy the herniated tip of the heart and the omphalocele have very different appearance under the thin layer of amnion/Wharton"s jelly. 3D surface rendering mask these details a little like a blanket of snow covers the details on the ground. This adds to the difficulty in identifying the abnormal structures.Gray scale 2D ultrasound would probably not have these problems since we can also "look what is inside" these protruding masses. If there was a way to assign a colour to each organ"s ultrasound reflectivity index, that would be a great improvement for 3D.

If you would like to view other cases of Pentallogy of Cantrell and related articles at, click over the following links:

Ectopia Cordis

Pentalogy of Cantrell 1

Pentalogy of Cantrell 2

Pentalogy of Cantrell 3

Pentalogy of Cantrell 4

These are some references about prenatal detection of Pentalogy of Cantrell.

1.Bonilla-Musoles F, Machado LE, Bailao LA, Osborne NG, Raga F. Abdominal wall defects: two- versus three-dimensional ultrasonographic diagnosis. J Ultrasound Med 2001 Apr;20(4):379-89.

2.Bognoni V, Quartuccio A, Quartuccio A. First-trimester sonographic diagnosis of Cantrell"s pentalogy with exencephaly. J Clin Ultrasound 1999 Jun;27(5):276-8.

3.Hsieh YY, Lee CC, Chang CC, Tsai HD, Hsu TY, Tsai CH. Prenatal sonographic diagnosis of Cantrell"s pentalogy with cystic hygroma in the first trimester. J Clin Ultrasound 1998 Oct;26(8):409-12.

4.Liang RI, Huang SE, Chang FM. Prenatal diagnosis of ectopia cordis at 10 weeks of gestation using two-dimensional and three-dimensional ultrasonography. Ultrasound Obstet Gynecol 1997 Aug;10(2):137-9.

5.Ghidini A, Sirtori M, Romero R, Hobbins JC. Prenatal diagnosis of pentalogy of Cantrell. J Ultrasound Med 1988 Oct;7(10):567-72.

6.Abu-Yousef MM, Wray AB, Williamson RA, Bonsib SM. Antenatal ultrasound diagnosis of variant of pentalogy of Cantrell. J Ultrasound Med 1987 Sep;6(9):535-8.

7.Baker ME, Rosenberg ER, Trofatter KF, Imber MJ, Bowie JD. The in utero findings in twin pentalogy of Cantrell. J Ultrasound Med 1984 Nov;3(11):525-7.


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