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2014-03-11-09 Answer to the case of the week #368 © Bronshtein www.TheFetus.net 

 Answer to the case of the week #368

May 01, 2014 - May 15, 2014

Moshe Bronshtein, MD*, Kidron Dvora, MD**

* Haifa, Rambam, Israel.
** Meir Hospital, Israel.

 

Case report

Following images and video show a 16 weeks old fetus with Scimitar syndrome. Our examination revealed:

- Dextroposition of the heart;
- Narrow right pulmonary artery;
- Anomalous curved vein entering the inferior vena cava.

The couple opted for the termination of the pregnancy at 19 weeks (male fetus, 300 g., 25 cm). The autopsy confirmed Scimitar syndrome with following findings:

- Anomalous right pulmonary venous drainage from aberrant veins in diaphragmatic aspect of the right lung leading to the inferior vena cava;
- Anomalous bronchial artery arising from the abdominal aorta (above the celiac trunk) leading to the diaphragmatic aspect of the right lung;
- Hypoplasia of the right lung with prominent edematous fibrous septa.

Images 1, 2, 3, and videos 1, 2, and 3
Dextroposition of the fetal heart at the level of the four-chamber view (image 1 and video 1); hypoplastic right branch of the pulmonary artery (image 2, video 2); and aberrant scimitar vein entering the inferior vena cava (image 3, video 3) can be seen.

 


 


 

Images 4, 5: The images show dextroposition of the fetal heart at the level of the four-chamber view of the heart.

 

Images 6, 7: The images show transverse scan of the fetal chest at the level of the branching of the pulmonary artery - the right branch of the pulmonary artery is almost not visible (it should be visible in the position depicted by yellow lines on the image 7). PA - pulmonary artery; Ao - aorta. 

 

Images 8, 9: Sagittal scans of the fetal thorax and abdomen showing inferior vena cava entering the right atrium. Aberrant scimitar vein can be seen entering the inferior vena cava (depicted in red color on the image 9). RA - right atrium; IVC - inferior vena cava.

 

Images 10, 11, 12: Pathological and histological images showing hypoplastic right lung (image 10), a
nomalous right pulmonary venous drainage from aberrant veins in diaphragmatic aspect of the right lung to the inferior vena cava and anomalous bronchial artery arising from abdominal aorta (above celiac trunk) leading to the diaphragmatic aspect of the right lung (image 11).

 




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