*   Institute of Chemical Biology and Fundamental Medicine, Russia, Novosibirsk.
**  Municipal Medical Genetic Centre, Russia, Novosibirsk.
*** Novosibirsk State Medical University, Russia, Novosibirsk.

Case report

A 21-year old healthy primigravida with unremarkable family and personal history was referred to our department at 21 weeks of her otherwise uncomplicated pregnancy. The reason for her referral was a facial anomaly suspicious of teratoma. Our ultrasound examination revealed following findings:

• Soft tissue tubular structure, attached to the right medial canthal region; 13 mm in length, 8,2 mm in diameter; the tubular tissue had an internal canal
• Heminasal hypoplasia and complete closure of the nasal opening on the side of the proboscis
  
• Microphthalmia on the affected side
  
• Cleft of the lower lid on the affected side
  
  

The diagnosis of  proboscis lateralis was suggested. Parents opted for the pregnancy termination. The fetal karyotype was normal, 46 XY. The pathological study confirmed the diagnosis. The ethmoidal cells were absent at the site of the lesion, the internal canal within the proboscis had a blind end at the level of dura mater. According to the classification of the lateral proboscis suggested by Koo, this case belongs to the most common group III, which includes lateral proboscis with ipsilateral deformity of the nose, eye and or ocular adnex.

Images 1,2

: 2D, 3D images of the fetal profile from left side and affected right side, deformed by proboscis.

Images 3,4: 3D images of the proboscis. Image 4 shows also a deformed left eye.

Images 5,6: Axial and sagittal view of the proboscis in tomographic ultrasound imaging.

Images 7,8: Image 7 shows microphtalmia of the right eye. Image 8 shows an axial view of the intact maxilla and palate with no signs of clefting.

Images 9,10: Image 9 shows a central canal inside the proboscis. Image 10 shows a narrow lumen of the canal within the proboscis which ends blindly at the level of dura mater (arrow).

Images 11,12: 3D-images of the face. Image 11 shows a inferior opening of the proboscis and left nostril (arrows). Image 12 shows a cleft of the right lower lid (arrow).

Images 13,14: Image of the baby after pregnancy termination, the postnatal findings correspond with the ultrasound. The right eye is smaller with the cleft of the lower lid and there is a proboscis nect to the hypoplastic nose with only a left nasal opening. The image 14 shows a blind end of the proboscis at the level of dura matter (arrow), ethmoidal cells were absent on the right side.