Correct answer to Q2 is: Nasal glioma
Discussion:
To diagnose tumors of the facial middle line, different imaging must be performed pre-natal and post-natal. These include pre-natal ultrasound and post-natal MRI and CT. The final diagnosis requires histology after surgical resection.
A congenital nasal mass, in the midline, is a rare developmental abnormality. Nasal glioma or heterotopic nasal glial is a rare benign congenital tumor. Its origin is in the ectopic glial cells located at the level of the nasal root with no communication with the central nervous system. They represent approximately 5% of all congenital nasal abnormalities and may present as intranasal masses (30%), extranasal masses (60%) or mixed (10%). With a predominance of 3:1 in male sex.
The encephaloceles are herniations of the contents of the cranium that maintain communication with the brain through a bone defect, they are characterized by being pulsatile, increasing in size with weeping with Valsalva or jugular compression (Furstemberg’s sign). Illumination through the tumor is possible.3-4
Dermoid cysts are the most frequent congenital midline nasal masses, with an incidence of 61% with a higher frequency in boys .3,5,6-9
Hemangiomas are vascular lesions. They are frequent in infancy, especially located in the head and neck. We ruled out this diagnosis, since there was no Doppler signal during the ultrasound evaluation
CT is useful to define bone anatomy, and MRI is essential to determine the content of the mass. It will show a fluid signal in an epidermoid cyst, fat in a dermoid and encephaloceles are isointense masses with the gray matter, contiguous with the brain parenchyma that extends through a bone defect. While in the case of gliomas a well circumscribed mass hypointense or isointense with the grey matter is seen. 3,7,10,11 The last features coincide with the findings in the post-natal imaging studies in our case.
Conclusion
Congenital midline nasal tumors are rare entities, but susceptible to prenatal diagnosis. If there is no compromise of the central nervous system, the prognosis is generally favorable. Nasal gliomas should be treated by complete excision to prevent recurrence. Prenatal follow up should be with ultrasound. After birth all imaging findings and clinical findings must be assessed by a multidisciplinary team. The treatment of choice is surgical resection, and the definitive diagnosis is made by histology.
Conflicts of interest
“The authors declare no conflict of interest.”
References
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