April 2013
A 29-day-old infant with a lump at the root of the nose, present since birth. No change in the size of the lump since first noticed, no pulsatility felt or change in size with crying.
Heterotopic Central Nervous System Tissue
- Also known as a nasal glioma (which is essentially a misnomer because there are no tumoral elements present in this heterotopic tissue)
- A rare congenital abnormality. 60% are extranasal, 30% intranasal, and 10% are mixed. Usually presents with a purplish mass at the root of the nose.
- Key Diagnostic Features: Ultrasound is the first-line investigation to exclude a vascular lesion. Minimal vascularity is normal with low arterial velocity in the end diastolic phase. The mass should follow the same signal intensity as glial tissue on all MRI sequences, although can be gliotic. The diagnosis is usually made out of exclusion from other differential diagnosis listed below.
- DDx: Hemangioma, encephalocele, dermoid/epidermoid, teratoma
- Rx: Surgical removal
