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2010-01-24-16 Answer to the case the week #266 © Manohar

Answer to the case of the week #266

April 15, 2010 - April 29 2010


 S.Manohar, M.D; M.Mohan Karthikeyan, DMRDD.

Doppler Scans, Madurai, Tamilnadu, India. 


The following images represent an interesting case of the Cystic hygroma diagnosed at the 34 weeks of gestation.

Case report


The ultrasound examination performed at 34 weeks of otherwise uncomplicated pregnancy of a healthy primigravida showed a huge cystic lesion located at the right half of the fetal face. The anechoic cystic lesion measured 80 by 90 mm. It did not show any septations and was located in the right parietal and paraorbital region. We did not detect any intracranial or other anomalies. The baby was delivered at term via cesarean section.


The postnatal findings confirmed our diagnosis. There was a large round mass protruding to the right side of the neonate"s  face. Deforming the right cheek and eye.


The Magnetic Resonance Imaging performed after delivery, described a very large cystic mass, 93x84x84 mm, located at the right side of the neck and facio-maxillary region. There was a notable thinning of the greater and lesser wings of the sphenoid bone, temporal, frontal bone and maxillary and zygomatic arch with excentric proptosis. There was no evidence of the intraorbital or intracranial expansion. The differential diagnosis according to the MRI was lymphangioma, specifically cystic hygroma.


The neonate underwent the surgery. The mass was removed and send for the pathology examination. The pathologist confirmed the diagnosis of the cystic hygroma.


Images 1,2: Image 1 shows an axial view of the fetal head. Image 2 shows a round mass on the side of the head.




Image 3-6: Images show both orbits and large anechoic mass protruding on the right side of the head.






Images 7,8: 3D images of the fetal face, note the right cheek with the protruding mass. 




Images 9,10,11: MRI images showing the cystic mass located at the facio-maxillary region. Image 11 shows a proptosis of the right eye.






Images 12,13,14: Images show a neonate with a large mass deforming the right side of the face.






Images 15,16: Images show a neonate after the surgery, removal of the lymphangioma.






Cystic hygromas result from the blockage of the lymphatic vessels. They are located in the regions which contain lymphatic tissue. Most common location for cystic hygroma is a neck region, head and axilla. The location of the cystic hygroma at the presented case is a very unusual because there are no lymphatic vessel in the central nervous system or meninges. Lymphatic drainage of the brain is via perivascular space along the basal membranes in the walls of capillaries and cerebral arteries. Lymphatics are present within the nasal cavity, orbits, around the jugular foramen and leptomeningeal sheaths around cranial nerves. One of the above mentioned locations maybe on origin of the cystic hygroma in the presented case.




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