Search :     
2010-06-16-13 Answer to the case of the week #281 © Quiroga
Answer to the case of the week #281

November 18, 2010 - December 2, 2010.

Héctor Gonzalo Quiroga Pacheco, MD.

Centro Medico Profesional Rosancar. Barquisimeto. Venezuela.

Case report

This is a case 32-year-old G2 P1 who was referred to our department at 14 weeks of gestation for a finding of a large cystic hygroma of the neck. Her family and personal history were unremarkable.

Ultrasound examination revealed the generalized fetal hydrops and echogenic lungs. The examination of the fetal heart showed interruption of the aortic arch. We could see at least one artery branching to the fetal neck before the aortic interruption (either brachiocephalic artery or left carotid artery as well). The descending aorta was connected to the ductal arch. There was no apparent evidence of the ventricular septal defect but the examination was quite technically difficult considering the gestational age.

Parents wished for the pregnancy termination based on the ultrasound findings and very poor prognosis for the fetus. The pathological examination was not performed.

The fetal hydrops and interrupted aortic arch could be part of several syndromes including: Turner, Di George (del 22q11.2), Fryns or Noonan syndrome.

Images 1,2: Image 1 shows a sagittal view of the fetus with a large cystic hygroma. Image 2 shows a transverse view of the thorax, note echogenic lungs and hydrops.

Images 3,4: Images are showing the straight course of the ascending aorta towards the head. The transverse aortic arch is missing.

Images 5,6: Image 5 shows Doppler imaging of the ascending aorta, it's branch,
brachiocephalic or left carotid artery is indicated by arrow. The arrow on image 6 shows the aortic arch interruption.

Images 9,10: Images show pulmonary artery and ductal arch.

Video 1,2: Video 1 shows the transverse view of the fetal thorax, note the echogenic lungs, hydrothorax, hydrops, left ventricle is smaller comparing to the right. Video 2 shows sagittal view of the fetal thorax with interrupted aortic arch. Ascending aorta continues straight towards the head, no aortic arch is visible.

Video 3: Doppler imaging showing the interruption of the aortic arch.


Back to case
Help Support :